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Sample records for primary cutaneous lymphomas

  1. Primary cutaneous lymphoma

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    Nguyen, M. Connie; Cleary, Sean F.; Hoppe, Richard T.

    1995-01-01

    Purpose: A retrospective review analyzed the survival and freedom from relapse of patients with stage IE or IIE primary cutaneous lymphoma (non mycosis fungoides) after treatments with radiation therapy alone (XRT), chemotherapy alone (RX) or combined modality therapy (CMT). Methods and Materials: Fifty two patients with stage IE-IIE cutaneous lymphoma treated at Stanford University Hospital were reviewed. The median age was 57, with a range of 26 to 94 and a male to female ratio of 1.21:1. Patients were staged according to the Ann Arbor System. Pathology was classified according to the Working Formulation. Treatment outcomes were compared using Kaplan-Meier survival curves with a Gehan p-value test. Results: The follow up range was 6 months to 22 years (median 7 years.) Twenty one percent of patients had low grade, 63% had intermediate grade and 15% had high grade lymphoma. The most common histologic subtype was diffuse large cell lymphoma Thirty two patients received radiation alone as initial treatment and sixteen patients received combined modality as initial treatment. Four patients received chemotherapy alone. The only significant prognostic factor for survival was the stage at diagnosis. Patients with stage IE disease had a longer actuarial survival (5-yr=79%, 10-yr=71%), as compared to those with stage IIE (5-yr=49%, 10-yr=33%), (p=0.029). The only significant prognostic factor for freedom from relapse was the initial treatment. Initial combined modality treatment lead to a longer freedom from relapse compared to patients treated with radiation alone (p=0.002), (median 5 years vs. 1.2 years). Despite this, the actuarial overall survival in the combined modality group and the radiation alone group are similar (median survival 7.7 and 8 years). The efficacy of either radiation or chemotherapy as salvage treatment after radiation failure was equivalent and both salvage treatments lead to equally long survival and freedom from second relapse. Conclusion

  2. Primary cutaneous lymphoma

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    Nguyen, M Connie; Cleary, Sean F; Hoppe, Richard T

    1995-07-01

    Purpose: A retrospective review analyzed the survival and freedom from relapse of patients with stage IE or IIE primary cutaneous lymphoma (non mycosis fungoides) after treatments with radiation therapy alone (XRT), chemotherapy alone (RX) or combined modality therapy (CMT). Methods and Materials: Fifty two patients with stage IE-IIE cutaneous lymphoma treated at Stanford University Hospital were reviewed. The median age was 57, with a range of 26 to 94 and a male to female ratio of 1.21:1. Patients were staged according to the Ann Arbor System. Pathology was classified according to the Working Formulation. Treatment outcomes were compared using Kaplan-Meier survival curves with a Gehan p-value test. Results: The follow up range was 6 months to 22 years (median 7 years.) Twenty one percent of patients had low grade, 63% had intermediate grade and 15% had high grade lymphoma. The most common histologic subtype was diffuse large cell lymphoma Thirty two patients received radiation alone as initial treatment and sixteen patients received combined modality as initial treatment. Four patients received chemotherapy alone. The only significant prognostic factor for survival was the stage at diagnosis. Patients with stage IE disease had a longer actuarial survival (5-yr=79%, 10-yr=71%), as compared to those with stage IIE (5-yr=49%, 10-yr=33%), (p=0.029). The only significant prognostic factor for freedom from relapse was the initial treatment. Initial combined modality treatment lead to a longer freedom from relapse compared to patients treated with radiation alone (p=0.002), (median 5 years vs. 1.2 years). Despite this, the actuarial overall survival in the combined modality group and the radiation alone group are similar (median survival 7.7 and 8 years). The efficacy of either radiation or chemotherapy as salvage treatment after radiation failure was equivalent and both salvage treatments lead to equally long survival and freedom from second relapse. Conclusion

  3. Modern radiation therapy for primary cutaneous lymphomas

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    Specht, Lena; Dabaja, Bouthaina; Illidge, Tim

    2015-01-01

    Primary cutaneous lymphomas are a heterogeneous group of diseases. They often remain localized, and they generally have a more indolent course and a better prognosis than lymphomas in other locations. They are highly radiosensitive, and radiation therapy is an important part of the treatment......, either as the sole treatment or as part of a multimodality approach. Radiation therapy of primary cutaneous lymphomas requires the use of special techniques that form the focus of these guidelines. The International Lymphoma Radiation Oncology Group has developed these guidelines after multinational...... meetings and analysis of available evidence. The guidelines represent an agreed consensus view of the International Lymphoma Radiation Oncology Group steering committee on the use of radiation therapy in primary cutaneous lymphomas in the modern era....

  4. Primary cutaneous anaplastic large-cell lymphoma.

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    Perry, Edward; Karajgikar, Jay; Tabbara, Imad A

    2013-10-01

    Since the recognition of the anaplastic large-cell lymphomas in the 1980s, much has been learned about the diagnosis, clinical presentation, and treatment of these malignant conditions. The systemic and primary cutaneous types of anaplastic large cell lymphomas have been differentiated on clinical and immunophenotypical findings, but further research is required to elucidate their exact etiologies and pathogeneses. Primary cutaneous anaplastic large-cell lymphoma has a 95% disease-specific 5-year survival, owing partly to the relatively benign course of the disease and partly to the variety of effective treatments that are available. As with many other oncological diseases, new drugs are continually being tested and developed, with immunotherapy and biological response modifiers showing promise.

  5. Primary cutaneous lymphoma with involvement of external genitalia

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    Viscandi, C.; Campo, L. del

    1998-01-01

    We describe the radiological findings recorded in a case of primary B cells cutaneous lymphoma that presented with involvement of penis and scrotum. The patient was referred to our center to undergo an ultrasonographic study. (Author) 7 refs

  6. The First Year of the AEVD Primary Cutaneous Lymphoma Registry.

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    Peñate, Y; Servitje, O; Machan, S; Fernández-de-Misa, R; Estrach, M T; Acebo, E; Mitxelena, J; Ramón, M D; Flórez, A; Blanes, M; Morillo, M; Medina, S; Bassas, J; Zayas, A; Espinosa, P; Pérez, A; Gónzalez-Romero, N; Domínguez, J D; Muniesa, C; López Robles, J; Combalia, A; Yanguas, I; Suh, H; Polo-Rodríguez, I; Bielsa, I; Mateu, A; Ferrer, B; Descalzo, M A; García-Doval, I; Ortiz-Romero, P L

    2018-04-18

    Primary cutaneous lymphomas are uncommon. This article describes the Primary Cutaneous Lymphoma Registry of the Spanish Academy of Dermatology and Venereology (AEDV) and reports on the results from the first year. Disease registry for patients with primary cutaneous lymphoma. The participating hospitals prospectively recorded data on diagnosis, treatment, tests, and disease stage for all patients with primary cutaneous lymphoma. A descriptive analysis was performed. In December 2017, the registry contained data on 639 patients (60% male) from 16 university hospitals. The most common diagnoses, in order of frequency, were mycosis fungoides/Sézary syndrome (MF/SS) (348 cases, 55%), primary cutaneous B-cell lymphoma (CBCL) (184 cases, 29%), primary cutaneous CD30 + T-cell lymphoproliferative disorder (CD30 + CLPD) (70 cases, 11%), and other types of T-cell lymphoma (37 cases, 5%). In total, 105 (16.5%) of the cases recorded were incident cases. The most common diagnosis in the MF/SS group was classic MF (77.3%). Half of the patients with MF had stage IA disease when diagnosed, and the majority were either in partial remission (32.5%) or had stable disease (33.1%). The most widely used treatments were topical corticosteroids (90.8%) and phototherapy. The most common form of primary CBCL was marginal zone lymphoma (50%). Almost all of the patients had cutaneous involvement only and nearly half had stage T1a disease. Most (76.1%) were in complete remission. The main treatments were surgery (55.4%) and radiotherapy (41.9%). The most common diagnosis in patients with CD30 + CLPD was lymphomatoid papulosis (68.8%). Most of the patients (31.4%) had stage T3b disease and half were in complete remission. The most common treatments were topical corticosteroids (68.8%) and systemic chemotherapy (32.9%). The characteristics of patients with primary cutaneous lymphoma in Spain do not differ from those described in other series in the literature. The registry will facilitate

  7. [Primary cutaneous B-cell lymphomas: study of 22 cases].

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    Martín Carrasco, Pablo; Morillo Andújar, Mercedes; Pérez Ruiz, Carmen; de Zulueta Dorado, Teresa; Cabrera Pérez, Rocío; Conejo-Mir, Julián

    2016-09-02

    Primary cutaneous B-cell lymphoma (CBCL) is a very low prevalence neoplasm and constitutes 25% of all primary cutaneous lymphomas. Our objective was to discover the epidemiological, clinic and histologic characteristics of CBCL in our area. Retrospective descriptive study with patients with histologic diagnosis of CBCL followed up in our department between 2004 and 2015. Twenty-two patients with CBCL were included; 65% were men and 35% were women. Follicle centre lymphoma was the most common subtype (41%). Only 3 cases presented with node involvement and one with bone marrow invasion. Five recurrences were detected and one patient died because of the CBCL. This is one of the first CBCL series in theSpanish population. The incidence, sex, age, subtype distribution, clinical features and immunohistochemical patterns are very similar to those of the other series. Copyright © 2016 Elsevier España, S.L.U. All rights reserved.

  8. Primary cutaneous smoldering adult T-cell leukemia/ lymphoma.

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    Gittler, Julia; Martires, Kathryn; Terushkin, Vitaly; Brinster, Nooshin; Ramsay, David

    2016-12-15

    HTLV-1 is a virus that is endemic in southwesternJapan and the Caribbean and has been implicatedin the development of ATLL. ATLL, which is anuncommon malignant condition of peripheralT-lymphocytes, is characterized by four clinicalsubtypes, which include acute, lymphomatous,chronic, and smoldering types, that are based onLDH levels, calcium levels, and extent of organinvolvement. We present a 52-year- old woman withpruritic patches with scale on the buttocks and withtender, hyperpigmented macules and papules oftwo-years duration. Histopathologic examinationwas suggestive of mycosis fungoides, laboratoryresults showed HTLV-I and II, and the patient wasdiagnosed with primary cutaneous ATLL. We reviewthe literature on HTLV-1 and ATLL and specifically theprognosis of cutaneous ATLL. The literature suggeststhat a diagnosis of ATLL should be considered amongpatients of Caribbean origin or other endemicareas with skin lesions that suggest a cutaneousT-cell lymphoma, with clinicopathologic features ofmycosis fungoides. Differentiation between ATLLand cutaneous T-cell lymphoma is imperative as theyhave different prognoses and treatment approaches.

  9. Posttransplantation primary cutaneous CD30 (Ki-1)-positive large-cell lymphoma.

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    Seçkin, D; Demirhan, B; Oğuz Güleç, T; Arikan, U; Haberal, M

    2001-12-01

    We describe the case of a 51-year-old female renal transplant recipient with primary cutaneous CD30-positive large-cell lymphoma of T-cell origin. Cutaneous T-cell lymphomas are rarely reported in organ transplant recipients, and we believe they should be considered in the differential diagnosis of cutaneous neoplastic and infectious diseases affecting this patient group.

  10. Rituximab in the treatment of primary cutaneous B-cell lymphoma: a review.

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    Fernández-Guarino, M; Ortiz-Romero, P L; Fernández-Misa, R; Montalbán, C

    2014-06-01

    Rituximab is a chimeric mouse-human antibody that targets the CD20 antigen, which is found in both normal and neoplastic B cells. In recent years, it has been increasingly used to treat cutaneous B-cell lymphoma and is now considered an alternative to classic treatment (radiotherapy and surgery) of 2 types of indolent lymphoma, namely, primary cutaneous follicle center lymphoma and primary cutaneous marginal zone B-cell lymphoma. Rituximab is also administered as an alternative to polychemotherapy in the treatment of primary cutaneous large B-cell lymphoma, leg type. Its use as an alternative drug led to it being administered intralesionally, with beneficial effects. In the present article, we review the literature published on the use of rituximab to treat primary cutaneous B-cell lymphoma. Copyright © 2012 Elsevier España, S.L. and AEDV. All rights reserved.

  11. Primary cutaneous marginal zone B-cell lymphoma: clinical and histological aspects.

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    Khaled, A; Sassi, S; Fazaa, B; Ben Hassouna, J; Ben Romdhane, K; Kamoun, M R

    2009-02-01

    According to the WHO-EORTC classification of cutaneous lymphomas, primary cutaneous marginal zone B-cell lymphoma are now well characterized. We report here a case of primary cutaneous marginal zone B-cell lymphoma in a 51 year-old man in which the diagnosis was made using both histology and immunopathology. The patient had no remarkable medical history, no history of either acute inflammation or insect bite, and presented with a 5 cm solitary asymptomatic erythematous firm, multinodular and infiltrated plaque on the back for 12 months. Histological examination and immunohistochemical study of a cutaneous biopsy provided a differential diagnosis between B cell lymphoma and lymphocytoma cutis. Full body work up revealed no signs of extracutaneous dissemination. The patient underwent surgical excision of the nodule. Histological examination showed a histological and immunophenotyping profile typical of primary cutaneous marginal zone B-cell lymphoma. The lesion was completely excised with clear margins and no recurrence occurred after a 12 month-follow-up period. Primary cutaneous marginal zone B-cell lymphoma are low-grade lymphomas that have an indolent course and a high tendency to recur. They should be differentiated from lymphocytoma cutis and from the other types of cutaneous B cell lymphomas that have a different course and prognosis.

  12. Primary cutaneous peripheral T-cell lymphoma, unspecified with an indolent clinical course: a distinct peripheral T-cell lymphoma?

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    Ryan, A J A

    2012-02-01

    Primary cutaneous peripheral T-cell lymphomas (PTL), unspecified, are rare lymphomas, with a poor prognosis. They grow and disseminate rapidly, leading to widespread disease. We report a case of PTL, unspecified occurring on the nose. Despite its aggressive histology, this tumour behaved indolently. It is remarkably similar, clinically and histologically, to four recently described cases that occurred on the ear.

  13. Primary cutaneous anaplastic large cell lymphoma masquerading as large pyogenic granuloma

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    Anupama Bains

    2016-01-01

    Full Text Available Primary cutaneous anaplastic large cell lymphoma (pcALCL forms 9% of the cutaneous T-cell lymphomas. It usually presents as solitary reddish brown ulcerating nodule or indurated plaque. Sometimes, it mimics other dermatological diseases such as eczema, pyoderma gangrenosum, pyogenic granuloma, morphea, and squamous cell carcinoma. Our case presented with large pyogenic granuloma like lesion with regional lymphadenopathy. Since pcALCL is rare, one can misdiagnose such cases and therefore high index of suspicion is necessary.

  14. Primary Cutaneous Diffuse Large B-Cell Lymphoma – a Case Report

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    Milovanović Milena

    2017-06-01

    Full Text Available In 2005, the World Health Organization - European Organization for Research and Treatment of Cancer (WHOEORTC classified cutaneous B-cell lymphomas into 4 categories: primary cutaneous marginal zone B-cell lymphoma (PCMZL, primary cutaneous follicle center lymphoma (PCFCL, primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL-LT, and primary cutaneous diffuse large B-cell lymphoma, other (PCDLBCL-O. The absence of evident extra-cutaneous disease is a necessary condition for the diagnosis of primary cutaneous B-cell lymphomas, because they have a completely different clinical behavior and prognosis from their nodal counterparts. PCDLBCL-O basically represents a morphological variation, lacking the typical features of PCDLBCLLT, neither confirming the definition of PCFCCL, but on the clinical ground, its behavior seems at least to partially overlap the indolent course of PCFCCL. In fact, the present WHO lymphoma classification from 2008 overcame the previous WHO-EORTC classification, including at least a part of PCDLBCL-O within the spectrum of PCFCCL. However, owing to the rarity and heterogeneity of the PCDLBCL-O, the precise clinicopathological characteristics have not been well characterized and the optimal treatment for this group of lymphomas is yet to be defined. Nevertheless, dermatologists and pathologists should be aware of this entity in order to avoid unnecessary aggressive treatment. We present a case of a 46-year-old Caucasian male with one large round-shaped tumor and a few scattered nodules localized on the back. The histopathological features of the lesion corresponded to PCDLBCL-O. The patient follow-up showed that he was disease-free three months after surgical excision of the lesions and adjuvant local radiotherapy. No additional therapy was introduced, including chemotherapy with rituximab, cyclophosphamide, doxorubicin hydrochloride, oncovin, prednisolone (R-CHOP.

  15. Radiation Therapy for Primary Cutaneous Anaplastic Large Cell Lymphoma: An International Lymphoma Radiation Oncology Group Multi-institutional Experience

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    Million, Lynn, E-mail: lmillion@stanford.edu [Stanford Cancer Institute, Stanford, California (United States); Yi, Esther J.; Wu, Frank; Von Eyben, Rie [Stanford Cancer Institute, Stanford, California (United States); Campbell, Belinda A. [Department of Radiation Oncology and Cancer Imaging, Peter MacCallum Cancer Centre, East Melbourne (Australia); Dabaja, Bouthaina [The University of Texas MD Anderson Cancer Center, Houston, Texas (United States); Tsang, Richard W. [Department of Radiation Oncology, Princess Margaret Cancer Centre, Toronto, Ontario (Canada); Ng, Andrea [Department of Radiation Oncology, Dana-Farber Cancer Institute, Boston, Massachusetts (United States); Wilson, Lynn D. [Department of Therapeutic Radiology/Radiation Oncology, Yale School of Medicine, Yale Cancer Center, New Haven, Connecticut (United States); Ricardi, Umberto [Department of Oncology, University of Turin, Turin (Italy); Kirova, Youlia [Institut Curie, Paris (France); Hoppe, Richard T. [Stanford Cancer Institute, Stanford, California (United States)

    2016-08-01

    Purpose: To collect response rates of primary cutaneous anaplastic large cell lymphoma, a rare cutaneous T-cell lymphoma, to radiation therapy (RT), and to determine potential prognostic factors predictive of outcome. Methods and Materials: The study was a retrospective analysis of patients with primary cutaneous anaplastic large cell lymphoma who received RT as primary therapy or after surgical excision. Data collected include initial stage of disease, RT modality (electron/photon), total dose, fractionation, response to treatment, and local recurrence. Radiation therapy was delivered at 8 participating International Lymphoma Radiation Oncology Group institutions worldwide. Results: Fifty-six patients met the eligibility criteria, and 63 tumors were treated: head and neck (27%), trunk (14%), upper extremities (27%), and lower extremities (32%). Median tumor size was 2.25 cm (range, 0.6-12 cm). T classification included T1, 40 patients (71%); T2, 12 patients (21%); and T3, 4 patients (7%). The median radiation dose was 35 Gy (range, 6-45 Gy). Complete clinical response (CCR) was achieved in 60 of 63 tumors (95%) and partial response in 3 tumors (5%). After CCR, 1 tumor recurred locally (1.7%) after 36 Gy and 7 months after RT. This was the only patient to die of disease. Conclusions: Primary cutaneous anaplastic large cell lymphoma is a rare, indolent cutaneous lymphoma with a low death rate. This analysis, which was restricted to patients selected for treatment with radiation, indicates that achieving CCR was independent of radiation dose. Because there were too few failures (<2%) for statistical analysis on dose response, 30 Gy seems to be adequate for local control, and even lower doses may suffice.

  16. Double-hit or dual expression of MYC and BCL2 in primary cutaneous large B-cell lymphomas.

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    Menguy, Sarah; Frison, Eric; Prochazkova-Carlotti, Martina; Dalle, Stephane; Dereure, Olivier; Boulinguez, Serge; Dalac, Sophie; Machet, Laurent; Ram-Wolff, Caroline; Verneuil, Laurence; Gros, Audrey; Vergier, Béatrice; Beylot-Barry, Marie; Merlio, Jean-Philippe; Pham-Ledard, Anne

    2018-03-26

    In nodal diffuse large B-cell lymphoma, the search for double-hit with MYC and BCL2 and/or BCL6 rearrangements or for dual expression of BCL2 and MYC defines subgroups of patients with altered prognosis that has not been evaluated in primary cutaneous large B-cell lymphoma. Our objectives were to assess the double-hit and dual expressor status in a cohort of 44 patients with primary cutaneous large B-cell lymphoma according to the histological subtype and to evaluate their prognosis relevance. The 44 cases defined by the presence of more than 80% of large B-cells in the dermis corresponded to 21 primary cutaneous follicle centre lymphoma with large cell morphology and 23 primary cutaneous diffuse large B-cell lymphoma, leg type. Thirty-one cases (70%) expressed BCL2 and 29 (66%) expressed MYC. Dual expressor profile was observed in 25 cases (57%) of either subtypes (n = 6 or n = 19, respectively). Only one primary cutaneous follicle centre lymphoma, large-cell case had a double-hit status (2%). Specific survival was significantly worse in primary cutaneous diffuse large B-cell lymphoma, leg type than in primary cutaneous follicle centre lymphoma, large cell (p = 0.021) and for the dual expressor primary cutaneous large B-cell lymphoma group (p = 0.030). Both overall survival and specific survival were worse for patients belonging to the dual expressor primary cutaneous diffuse large B-cell lymphoma, leg type subgroup (p = 0.001 and p = 0.046, respectively). Expression of either MYC and/or BCL2 negatively impacted overall survival (p = 0.017 and p = 0.018 respectively). As the differential diagnosis between primary cutaneous follicle centre lymphoma, large cell and primary cutaneous diffuse large B-cell lymphoma, leg type has a major impact on prognosis, dual-expression of BCL2 and MYC may represent a new diagnostic criterion for primary cutaneous diffuse large B-cell lymphoma, leg type subtype and further identifies patients with

  17. Treatment of primary cutaneous anaplastic large cell lymphoma with superficial x-rays

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    Jepsen, Malene E; Gniadecki, Robert

    2015-01-01

    The optimal radiation schedule for primary cutaneous anaplastic lymphoma (PCALCL) has not been investigated. We report here satisfactory outcomes of low-dose (16-20 Gy, 3-5 fractions), superficial X-ray radiation (40-50 kV) in a series of 10 patients with PCALCL. Only 1 patient developed a local...

  18. Primary cutaneous large B-cell lymphoma of scalp: Case report of a rare variant

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    Yasmeen Khatib

    2017-01-01

    Full Text Available Primary cutaneous large B-cell lymphoma (Bcl is defined as a lymphoma composed of large cells constituting more than 80% of the infiltrate and absence of extracutaneous involvement after staging investigations. In the new World Health Organization/European Organization for Research and Treatment of Cancer classification, cutaneous Bcls with large cells are of three types - primary cutaneous large Bcl leg type (PCLBCLLT, primary cutaneous follicle center lymphoma diffuse type (PCFCLDT, and primary cutaneous large Bcls other (PCLBCLO. These three different types are distinct in terms of their clinicopathological features and survival. The PCLBCLO has intermediate features between those of PCLBCLLT and PCFCLDT. We present a case of PCLBCLO in a 57-year-old male who presented with a scalp swelling. Ultrasonography examination was suggestive of a sebaceous cyst. Computed tomography scan revealed the presence of an ill-defined hyperdense region in the soft tissue of the scalp region extending into the deeper layers of the scalp. Fine-needle aspiration cytology (FNAC revealed the presence of atypical lymphoid cells. Diagnosis was confirmed by biopsy and immunohistochemistry. Patient received rituximab combined with doxorubicin, vincristine, cyclophosphamide, and prednisolone regimen with complete resolution of the lesion. We present this case for its rarity, the utility of FNAC in early diagnosis, and to discuss the differential diagnosis.

  19. Radiotherapy of primary cutaneous follicle center lymphoma: case report and review of literature

    International Nuclear Information System (INIS)

    Ceovic, Romana; Jovanovic, Ivana; Kostovic, Kresimir; Rados, Jaka; Dotlic, Snjezana; Radman, Ivo; Kulisic, Sandra Marinovic; Loncaric, Davorin

    2013-01-01

    Primary cutaneous follicle center lymphoma is an indolent primary cutaneous B-cell lymphoma originating from the follicle center cells, composed of a combination of centrocytes (small and large cleaved cells) and centroblasts (large noncleaved cells) with a follicular, follicular/diffuse, or diffuse growth pattern. Lesions are mostly located on the head, neck and trunk. A case is presented of a 56-year-old male patient with primary cutaneous follicle center lymphoma, with lesions involving the skin of the back, shoulders, presternal area and right forearm. As the patient presented a disseminated cutaneous form of the disease that involved several anatomical regions, complete work-up was followed by superficial fractionated radiotherapy of eight fields in VI expositions, with total irradiation dose of 1400 cGy upon the following fields: right and left pectoral region, left and right shoulders, right suprascapular region, and proximal third of the right forearm. Total irradiation dose applied upon each field for the lesions located on the left and right side of the back was 1500 cGy. This therapy resulted in significant reduction of visible tumor. The patient was regularly followed up on outpatient basis for 12 months of radiotherapy, being free from local recurrence and systemic spread of the disease

  20. Primary Cutaneous CD 30+ Anaplastic Large Cell Lymphoma. A Case Report and Literature Review

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    Miguel Ángel Serra Valdés

    2014-03-01

    Full Text Available Primary cutaneous CD 30+ anaplastic large cell lymphoma is part of the spectrum of primary cutaneous CD30 + lymphoproliferative disorders, together with lymphomatoid papulosis. Its frequency is less than 0.5 x 100 000 inhabitants per year. It accounts for a very small proportion of non-Hodgkins lymphomas. The case of an 80-year-old female patient whose diagnosis was established in 2006 because of lesions on the face and neck is presented. The lesions continued to grow in an exaggerated fashion lately leading to deformity of her face. She was admitted due to neurological manifestations unrelated to the lesions. The presentation of this case is necessary because it requires performing differential diagnosis in clinical practice. Given its rarity, it is of interest to the medical community, especially trainees.

  1. Primary cutaneous lymphomas: A clinical and histological study of 99 cases in Isfahan, Iran

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    Farahnaz Fatemi Naeini

    2015-01-01

    Full Text Available Background: Primary cutaneous lymphomas (PCLs represent a heterogeneous group of T- and B-cell lymphomas that present in the skin with no evidence of extracutaneous disease at the time of diagnosis. The aim of this study was to assess and report the epidemiological characteristics of PCLs in Isfahan, Isfahan Province, Iran - as a main province of Iran. Materials and Methods: A total of 99 patients were recruited over a recent 10-year period (2003-2013 with diagnosis of PCLs; the patients were classified according to the The World Health Organization/European Organization for Research and Treatment of Cancer (WHO-EORTC criteria. Mean and standard deviations (SDs were used to describe continuous data, numbers, and percentages for categorical data. Statistical significance was defined as P < 0.05. Results: The patients comprised 45 men and 54 women aged 5-80 years (median 36 at diagnosis. The male-to-female ratio was 1:1.2. Histological examination showed features of primary cutaneous B-cell lymphomas (PCBCLs in four cases. The mean ± SD age in primary cutaneous T-cell lymphomas (PCTCLs and PCBCLs was 37.9 ± 16.5 years and 39.7 ± 9.1 years, respectively (P = 0.72. The mean ± SD latent period between the time of diagnosis and initiation of skin lesions in men and women was 2.3 ± 4.1 years and 5.9 ± 10.1 years, respectively (P = 0.02. The most frequent subtypes were mycosis fungoides (MFs (86.9% followed by Sιzary syndrome (SS (4%. Five patients died from PCL-related deaths. Conclusion: The distinguishing epidemiologic characteristics of PCL in Iran are the absence of a male predominance and a lower age of diagnosis. The study highlights the ethnic or regional variations in the clinicoepidemiological characteristics of PCLs.

  2. MicroRNA profiling of primary cutaneous large B-cell lymphomas.

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    Lianne Koens

    Full Text Available Aberrant expression of microRNAs is widely accepted to be pathogenetically involved in nodal diffuse large B-cell lymphomas (DLBCLs. However, the microRNAs profiles of primary cutaneous large B-cell lymphomas (PCLBCLs are not yet described. Its two main subtypes, i.e., primary cutaneous diffuse large B-cell lymphoma, leg type (PCLBCL-LT and primary cutaneous follicle center lymphoma (PCFCL are characterized by an activated B-cell (ABC-genotype and a germinal center B-cell (GCB-genotype, respectively. We performed high-throughput sequencing analysis on frozen tumor biopsies from 19 cases of PCFCL and PCLBCL-LT to establish microRNA profiles. Cluster analysis of the complete microRNome could not distinguish between the two subtypes, but 16 single microRNAs were found to be differentially expressed. Single microRNA RT-qPCR was conducted on formalin-fixed paraffin-embedded tumor biopsies of 20 additional cases, confirming higher expression of miR-9-5p, miR-31-5p, miR-129-2-3p and miR-214-3p in PCFCL as compared to PCLBCL-LT. MicroRNAs previously described to be higher expressed in ABC-type as compared to GCB-type nodal DLBCL were not differentially expressed between PCFCL and PCLBCL-LT. In conclusion, PCFCL and PCLBCL-LT differ in their microRNA profiles. In contrast to their gene expression profile, they only show slight resemblance with the microRNA profiles found in GCB- and ABC-type nodal DLBCL.

  3. Distinct types of primary cutaneous large B-cell lymphoma identified by gene expression profiling.

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    Hoefnagel, Juliette J; Dijkman, Remco; Basso, Katia; Jansen, Patty M; Hallermann, Christian; Willemze, Rein; Tensen, Cornelis P; Vermeer, Maarten H

    2005-05-01

    In the European Organization for Research and Treatment of Cancer (EORTC) classification 2 types of primary cutaneous large B-cell lymphoma (PCLBCL) are distinguished: primary cutaneous follicle center cell lymphomas (PCFCCL) and PCLBCL of the leg (PCLBCL-leg). Distinction between both groups is considered important because of differences in prognosis (5-year survival > 95% and 52%, respectively) and the first choice of treatment (radiotherapy or systemic chemotherapy, respectively), but is not generally accepted. To establish a molecular basis for this subdivision in the EORTC classification, we investigated the gene expression profiles of 21 PCLBCLs by oligonucleotide microarray analysis. Hierarchical clustering based on a B-cell signature (7450 genes) classified PCLBCL into 2 distinct subgroups consisting of, respectively, 8 PCFCCLs and 13 PCLBCLsleg. PCLBCLs-leg showed increased expression of genes associated with cell proliferation; the proto-oncogenes Pim-1, Pim-2, and c-Myc; and the transcription factors Mum1/IRF4 and Oct-2. In the group of PCFCCL high expression of SPINK2 was observed. Further analysis suggested that PCFCCLs and PCLBCLs-leg have expression profiles similar to that of germinal center B-cell-like and activated B-cell-like diffuse large B-cell lymphoma, respectively. The results of this study suggest that different pathogenetic mechanisms are involved in the development of PCFCCLs and PCLBCLs-leg and provide molecular support for the subdivision used in the EORTC classification.

  4. Spontaneous regression of primary cutaneous diffuse large B-cell lymphoma, leg type with significant T-cell immune response

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    Paul M. Graham, DO

    2018-05-01

    Full Text Available We report a case of histologically confirmed primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL-LT that subsequently underwent spontaneous regression in the absence of systemic treatment. The case showed an atypical lymphoid infiltrate that was CD20+ and MUM-1+ and CD10–. A subsequent biopsy of the spontaneously regressed lesion showed fibrosis associated with a lymphocytic infiltrate comprising reactive T cells. PCDLBCL-LT is a cutaneous B-cell lymphoma with a poor prognosis, which is usually treated with chemotherapy. We describe a case of clinical and histologic spontaneous regression in a patient with PCDLBCL-LT who had a negative systemic workup but a recurrence over a year after his initial presentation. Key words: B cell, lymphoma, primary cutaneous diffuse large B-cell lymphoma, leg type, regression

  5. Primary cutaneous CD30+ anaplastic large-cell lymphoma in a young patient with psoriasis

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    Ming-Chun Chen

    2013-03-01

    Full Text Available Psoriasis is a common chronic inflammatory cutaneous disease, while primary cutaneous CD30+ anaplastic large-cell lymphoma (PC-ALCL is a rare T-cell lymphoma which always has an excellent prognosis, although multifocal PC-ALCL tends to relapse after systemic chemotherapy. Psoriasis associated with PC-ALCL is exceptionally rare. We report a 29-year-old Chinese female with a 5-year history of psoriasis treated with Chinese herbs alone, who was referred to our institution with a tumor on the left clavicular region for 1 year and another one on the left palm for 2 months. Skin biopsies of both lesions showed diffuse infiltration of tumor cells, composed of large atypical cells with marked nuclear pleomorphism, prominent nucleoli, and eosinophilic cytoplasm. Large numbers of neutrophilic infiltrations were also noted in the lesion. Immunostaining revealed the lesion to be positive for CD30, vimentin, CD45, and CD68, and weakly positive for epithelial membrane antigen, but negative for anaplastic lymphoma receptor tyrosine kinase. The patient was diagnosed to have psoriasis associated with PC-ALCL; she died 18 months after the final diagnosis with unknown cause. We consider that immune dysregulation and/or Chinese herbs may play roles in the development of the present PC-ALCL.

  6. Importance of gallium-67 scintigraphy in primary cutaneous B-cell lymphoma: report of two cases

    International Nuclear Information System (INIS)

    Attab, Cyomara Sanches; Moriguchi, Sonia Marta; Rocha, Euclides Timoteo da

    2010-01-01

    The authors describe two cases of cutaneous B-cell lymphoma where correct staging, treatment and follow-up could be achieved through a combination of conventional imaging studies and gallium-67 scintigraphy. (author)

  7. Patients' Illness Perception as a Tool to Improve Individual Disease Management in Primary Cutaneous Lymphomas.

    Science.gov (United States)

    Porkert, Stefanie; Lehner-Baumgartner, Eva; Valencak, Julia; Knobler, Robert; Riedl, Elisabeth; Jonak, Constanze

    2018-02-07

    The Revised Illness Perception Questionnaire (IPQ-R) has been shown to assess illness perception reproducibly in primary cutaneous T-cell lymphomas (CTCL). Illness perception reflects patients' individual concepts of understanding and interpretation of the disease, influencing illness behaviour and health-related quality of life (HRQOL). This study investigated the clinical relevance of the relationships between illness perception, illness behaviour, and HRQOL in CTCL and cutaneous B-cell lymphomas (CBCL). A total of 92 patients completed the IPQ-R, the Scale for the Assessment of Illness Behavior (SAIB), and a skin-specific HRQOL tool (Skindex-29). Data on illness behaviour were not evidently related to illness perception, whereas illness perception was significantly associated with HRQOL. Both, IPQ-R and HRQOL results correlated with disease entity, stage, and socio-demographics. Only IPQ-R results provided practical information on patients' needs to train personal coping strategies. IPQ-R assessment in CTCL and CBCL might be a useful instrument to improve individual disease management.

  8. Primary Cutaneous CD4-Positive Small/Medium Pleomorphic T-cell Lymphoma – A Case Report

    Directory of Open Access Journals (Sweden)

    Micković Milena

    2016-12-01

    Full Text Available Primary cutaneous CD4-positive small- to medium-sized pleomorphic T-cell lymphoma is a provisional entity in the 2005 WHO-EORTC classification for cutaneous lymphomas. It is a rare condition and, in most cases, it has a favorable clinical course and prognosis. Primary cutaneous CD4-positive small/medium pleomorphic T-cell lymphoma (PCSM-TCL is defined as a cutaneous T-cell lymphoma with predominantly small- to medium-sized CD4-positive pleomorphic T-cells without a history of patches and plaques typical of mycosis fungoides. PCSM-TCL usually presents as a solitary plaque or tumor on the head, neck, trunk or upper extremities and it is considered to have indolent clinical behavior. Histologically, it is characterized by a dense infiltration of small/medium-sized pleomorphic T-cells that involves the entire dermal thickness, often with nodular extension into the hypodermis. Using immunohistochemical staining, the majority of the reported cases proved to be CD3, CD4 positive and CD8, CD30 negative. However, due to the rarity and heterogeneity of the PCSM-TCL, precise clinicopathologic characteristics of PCSM-TCL have not been well characterized and the optimal treatment for this group of lymphomas is yet to be defined. Dermatologists and pathologists should be aware of this entity in order to avoid unnecessary aggressive treatments.

  9. Advanced-Stage Primary Cutaneous T-Cell Lymphoma Treated with Bexarotene and Denileukin Diftitox

    Directory of Open Access Journals (Sweden)

    Iván Cervigón-González

    2011-02-01

    Full Text Available Advanced-stage primary cutaneous T-cell lymphoma has an unfavorable prognosis and low survival rates. Aggressive treatment with chemotherapy is not curative and causes considerable side effects. The combination of bexarotene and denileukin diftitox is associated with an acceptable safety profile and a likely synergistic effect because bexarotene is capable of modulating expression of IL-2 receptor and enhance the susceptibility of T-cell leukemia cells to denileukin diftitox. In the case reported here, the response to this combined treatment was satisfactory and well tolerated. The patient showed a complete regression of pruritus, restlessness, and insomnia. Skin lesions improved partially, and lymphadenopathy was reduced and finally disappeared completely.

  10. [Primary cutaneous T-cell lymphoma of the penis complicated by Fournier gangrene: a case report].

    Science.gov (United States)

    Zhou, Zun-lin; Wang, Chuan-yun; Xu, Zhi-shun; Zheng, Bao-zhong

    2008-06-01

    To explore the clinical presentation, pathologic characteristics, diagnosis and treatment of cutaneous T-cell lymphoma of the penis. A 49-year-old man presented with painful swelling and inflammation of the foreskin, failed to respond to antibiotic treatment and dorsal incision, and was instead complicated by Fournier gangrene. Then he underwent debridement and pathological examination. Pathological results indicated cutaneous T-cell lymphoma of the penis. Immunohistochemistry showed CD3 and CD45 RO to be positive, but CD30, CD79a, CD20 and HMB negative. The patient was treated by interferon alpha and ultraviolet B for 2 weeks, followed by total removal of the external genitalia because of necrosis of the corpus spongiosum, which involved the scrotum and right testis on pathological examination. Cutaneous T-cell lymphoma of the penis is a rare condition and easily mis diagnosed in the early phase. Definitive diagnosis depends on pathological study.

  11. Primary cutaneous follicle center lymphoma in the setting of chronic lymphocytic leukemia

    Directory of Open Access Journals (Sweden)

    S Konda

    2011-01-01

    Full Text Available Primary cutaneous malignancies arising in association with chronic lymphocytic leukemia (CLL are notable for their atypical clinical and histological presentation. We report a 69-year-old man with a 17-year history of CLL who presented for evaluation of a well-defined red to violaceous nodule with a central depressed scar on the left lower extremity. Microscopic examination of a punch biopsy revealed an infiltrate of predominantly small lymphocytes with scattered large, atypical epithelioid cells. Immunohistochemical stains revealed diffuse positive staining of the lesional cells with CD20+ and bcl-6+ and focal positive staining with bcl-2+ (negative CD10 and CD23, findings which, in conjunction with the histology, were most compatible with a diagnosis of primary cutaneous follicle center lymphoma (PCFCL. A review of the clinical charts revealed several prior biopsies with varied diagnoses. In light of the most recent biopsy findings, all previous biopsies were re-reviewed and interpreted as PCFCL arising in the setting of CLL. Features contributing to the diagnostic conundrum in this case included an atypical clinical and histological presentation, lack of pertinent clinical history and multiple presentations at different institutions.

  12. [Role of stem cell transplantation in treatment of primary cutaneous T‑cell lymphoma].

    Science.gov (United States)

    Stranzenbach, R; Theurich, S; Schlaak, M

    2017-09-01

    Within the heterogeneous group of cutaneous T‑cell lymphomas (CTCL) the therapeutic options for advanced and progressive forms are particularly limited. The therapeutic value of hematopoietic stem cell transplantation in CTCL was analyzed. A literature search using the keywords "hematopoietic stem cell transplantation" and "cutaneous T‑cell lymphoma" was performed in PubMed. Studies between 1990 and 2017 were taken into account. The studies identified were analyzed for relevance and being up to date. After reviewing the currently available literature no prospective randomized studies were found. Wu et al. showed a superiority of allogeneic transplantation in a comparison of autologous and allogeneic stem cell transplantation for cutaneous lymphoma. The graft-versus-lymphoma effect plays a significant role in a prolonged progression-free survival after allogeneic transplantation. By using a non-myeloablative conditioning regimen, stem cell transplantation can also be an option for elderly patients. The most extensive long-term data after allogeneic stem cell transplantation were reported by Duarte et al. in 2014. Autologous stem cell transplantation does not currently represent a therapeutic option, whereas allogeneic stem cell transplantation for advanced cutaneous T‑cell lymphoma, using a non-myeloablative conditioning scheme, does represent a therapeutic option. However, there is no consensus on the appropriate patients and the right timing. Morbidity and mortality of complications should be taken into account. Thus, this procedure is currently subject to an individual case decision.

  13. Expression of MCM-3 and MCM-7 in Primary Cutaneous T-cell Lymphomas.

    Science.gov (United States)

    Jankowska-Konsur, Alina; Kobierzycki, Christopher; Reich, Adam; Grzegrzolka, Jedrzej; Maj, Joanna; Dziegiel, Piotr

    2015-11-01

    Primary cutaneous T-cell lymphomas is a group of rare non-Hodgkin lymphomas, originally affecting the skin. Increased proliferation activity is a hallmark of diverse tumors and the proliferation rate, measured by the expression of various markers has a predictive value regarding the malignancy course. The aim of the present study was to evaluate the prognostic value and the potential correlation between the expression of proliferation markers Ki-67, MCM-3 and MCM-7, and clinicopathological data for different types of primary cutaneous T-cell lymphomas. Immunohistochemical reactions were performed on paraffin blocks obtained from 90 patients with mycosis fungoides (MF) and 21 patients with other CTCL (oCTCL), in comparison to 19 patients with benign inflammatory dermatosis (lichen planus, eczema), serving as control. Statistically significant differences in the expression of Ki-67, MCM-3 and MCM-7 were observed between oCTCL vs. the control group (29% vs. 5%; 17% vs. 5%; 13% vs. 1.5%, respectively, ANOVA with Scheffé post-hoc test: pMCM-3 (r=0.83; pMCM-7 (r=0.84; pMCM-3 and MCM-7 (r=0.81, pMCM-3 expression was significantly higher in advanced compared to early stages (11% vs. 3% and 15.5% vs. 5.0%, respectively, Student's t-test: pMCM-3 and MCM-7 compared to benign inflammatory dermatoses (Student's t-test: pMCM-3 and MCM-7 than T1a (pMCM-3 expression in MF showed a positive relationship with both nodal and distant metastases (ANOVA with Scheffé post hoc test: pMCM-3 expression correlated with shorter survival in MF, although the latter did not reach statistical significance (10-year survival 0.38 vs. 0.82, p=0.02, and 0.46 vs. 0.81, p=0.06, respectively; log rank test). All studied proliferation markers may had predictive values regarding the disease severity and prognosis. Further studies are required to analyze their implementation into patient stratification and treatment process such that will improve prognosis in CTCL. Copyright© 2015 International

  14. Primary cutaneous follicle center lymphoma associated with an extracutaneous dissemination: a cytogenetic finding of potential prognostic value.

    Science.gov (United States)

    Subramaniyam, Shivakumar; Magro, Cynthia M; Gogineni, Swarna; Tam, Wayne; Mathew, Susan

    2015-11-01

    Cytogenetic studies on cutaneous lymphomas are rare, and very little is known about their prognostic value. We present a rare case of primary cutaneous follicle center lymphoma (PCFCL) with a complex translocation presenting with cutaneous and extracutaneous dissemination in the lymph node. Morphologic, immunohistochemical, conventional cytogenetic, and fluorescence in situ hybridization (FISH) studies were performed on this patient. A combination of cytogenetic and FISH analysis identified a complex novel four-way t(2;14;9;3) (p11.2;q32;p13;q27) translocation involving rearrangements of BCL6, immunoglobulin light and heavy chain genes, and an unknown gene on 9p. Our report elaborates the morphologic and immunohistochemical features in combination with cytogenetic and molecular cytogenetic analysis of PCFCL, which provide additional insight into the clinical and biologic behavior of this lesion. Copyright© by the American Society for Clinical Pathology.

  15. Total Skin Electron Beam for Primary Cutaneous T-cell Lymphoma

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    Elsayad, Khaled; Kriz, Jan; Moustakis, Christos; Scobioala, Sergiu; Reinartz, Gabriele; Haverkamp, Uwe; Willich, Normann [Department of Radiation Oncology, University Hospital of Muenster, Muenster (Germany); Weishaupt, Carsten [Department of Dermatology, University Hospital of Muenster, Muenster (Germany); Stadler, Rudolf [Department of Dermatology, Johannes-Wesling-Klinikum Minden, Minden (Germany); Sunderkötter, Cord [Department of Dermatology, University Hospital of Muenster, Muenster (Germany); Eich, Hans Theodor, E-mail: Hans.Eich@ukmuenster.de [Department of Radiation Oncology, University Hospital of Muenster, Muenster (Germany)

    2015-12-01

    Purpose: Recent trials with low-dose total skin electron beam (TSEB) therapy demonstrated encouraging results for treating primary cutaneous T-cell lymphoma (PCTCL). In this study, we assessed the feasibility of different radiation doses and estimated survival rates of different pathologic entities and stages. Methods and Materials: We retrospectively identified 45 patients with PCTCL undergoing TSEB therapy between 2000 and 2015. Clinical characteristics, treatment outcomes, and toxicity were assessed. Results: A total of 49 courses of TSEB therapy were administered to the 45 patients. There were 26 pathologically confirmed cases of mycosis fungoides (MF) lymphoma, 10 cases of Sézary syndrome (SS), and 9 non-MF/SS PCTCL patients. In the MF patients, the overall response rate (ORR) was 92% (50% complete remission [CR]), 70% ORR in SS patients (50% CR), and 89% ORR in non-MF/SS patients (78% CR). The ORR for MF/SS patients treated with conventional dose (30-36 Gy) regimens was 92% (63% CR) and 75% (25% CR) for low-dose (<30-Gy) regimens (P=.09). In MF patients, the overall survival (OS) was 77 months with conventional dose regimens versus 14 months with low-dose regimens (P=.553). In SS patients, the median OS was 48 versus 16 months (P=.219), respectively. Median event-free survival (EFS) for MF in conventional dose patients versus low-dose patients was 15 versus 8 months, respectively (P=.264) and 19 versus 3 months for SS patients (P=.457). Low-dose regimens had shorter treatment time (P=.009) and lower grade 2 adverse events (P=.043). A second TSEB course was administered in 4 MF patients with 100% ORR. There is a possible prognostic impact of supplemental/boost radiation (P<.001); adjuvant treatment (P<.001) and radiation tolerability (P=.021) were detected. Conclusions: TSEB therapy is an efficacious treatment modality in the treatment of several forms of cutaneous T-cell lymphoma. There is a nonsignificant trend to higher and longer clinical benefit

  16. The role of 18F-FDG PET and PET/CT in the evaluation of primary cutaneous lymphoma.

    Science.gov (United States)

    Qiu, Lin; Tu, Guojian; Li, Jing; Chen, Yue

    2017-02-01

    Primary cutaneous lymphoma (PCL) is the second most common type of extranodal non-Hodgkin lymphoma, including both cutaneous T-cell and B-cell lymphomas. PCL comprises numerous subtypes and thus has myriad clinical presentations in the skin and subcutaneous tissues. Accurate classification and staging are important for making treatment recommendations for PCL and will further impact patient prognosis significantly. We review the role of fluorine-18-fluorodeoxyglucose (F-FDG) PET (F-FDG PET) and F-FDG PET with computed tomography (CT) in the diagnosis, staging, tumor biological evaluation, treatment response assessment, and early recurrence surveillance of PCL. Although F-FDG PET and PET/CT do not seem to adequately distinguish the plaque, patch, or erythroderma cutaneous lesions of PCL, the imaging modalities are superior to CT, MRI, and other nuclear medicine methods in detecting both the cutaneous and the extracutaneous lesions of PCL. The available literature addressing the clinical role of F-FDG PET and PET/CT in patients with PCL is promising for the use of the modalities in staging, tumor biological evaluation, biopsy guidance, early treatment response assessment, and recurrence surveillance. However, more data are needed to better specify the role of F-FDG PET and PET/CT in the management of PCL.

  17. Primary cutaneous marginal zone lymphoma associated with juxta-articular fibrotic nodules in a teenager.

    Science.gov (United States)

    Ghatalia, Pooja; Porter, Joanne; Wroblewski, Danielle; Carlson, John Andrew

    2013-05-01

    Primary cutaneous marginal zone lymphoma (PCMZL) has rarely been reported in teenagers and is occasionally associated with Borrelia burgdorferi infection. Juxta-articular fibrotic nodules represent a unique, localized fibrosing response to spirochete infections, namely Borreliosis. Herein, we report a 15-year-old healthy boy who presented with a 4-year history of progressive acquisition of asymptomatic, erythematous nodules, ≤ 3 cm, beginning with his right forearm (3), then right arm (1) and lastly his right inner thigh (1). Biopsy showed PCMZL in three of five samples, and inflamed, fibrotic nodules, near the elbow in two. The bottom heavy lymphomatous nodules consisted of mostly small CD20+ CD43+ lymphocytes, some with plasmacytoid features. Mature plasma cells were lambda light chain restricted by in situ hybridization. The juxta-articular fibrotic nodules were located in the deep dermis and subcutis, had peripheral plasma cell-rich infiltrates, and showed nodular sclerosis (morphea profunda-like) in one, and lamellar and angiocentric sclerosis in the other reminiscent of quiescent lesions of chronic localized fibrosing leukocytoclastic vasculitis. Immunohistochemistry for B. burgdorferi revealed rare positive organisms; however, polymerase chain reaction (PCR) and serology were negative for B. burgdorferi as were serologic and/or PCR assays for Bartonella henselae, Ba. quintana, Ehrlichia chaffeensis, Treponema pallidum, Helicobacter pylori and Babesia microti. No evidence of extracutaneous disease was found by the review of systems and imaging studies. A 4-week trial of doxycycline therapy failed, whereas intralesional (IL) corticosteroid therapy induced rapid regression of his nodules. After two local recurrences, also treated with IL corticosteroids, he is well, without cutaneous disease, 20 months later. A literature review of 19 pediatric cases PCMZL reveals a similar natural history as adult PCMZL. Despite negative serology and PCR for B. burgdorferi

  18. Is bone marrow biopsy always indicated in patients with primary cutaneous marginal zone B-cell lymphoma?

    Science.gov (United States)

    Muniesa, C; Hernández-Machín, B

    2013-10-01

    Bone marrow involvement at the time of diagnosis is uncommon in patients with primary cutaneous marginal zone B-cell lymphoma (PCMZL). Moreover, in these patients such involvement is rarely found in isolation on diagnosis. Typically the few patients with PCMZL who have early bone marrow involvement also present secondary nodal or visceral involvement, which is detected by other staging studies (usually computed tomography). In recent years, this has given rise to some debate about whether a bone marrow biopsy should be routinely performed in patients diagnosed with PCMZL in view of the good prognosis and low incidence of bone marrow infiltration and/or extracutaneous involvement in this type of lymphoma. Copyright © 2012 Elsevier España, S.L. and AEDV. All rights reserved.

  19. Allogeneic hematopoietic stem cell transplantation in Primary Cutaneous T Cell Lymphoma.

    Science.gov (United States)

    Cudillo, Laura; Cerretti, Raffaella; Picardi, Alessandra; Mariotti, Benedetta; De Angelis, Gottardo; Cantonetti, Maria; Postorino, Massimiliano; Ceresoli, Eleonora; De Santis, Giovanna; Nasso, Daniela; Pisani, Francesco; Scala, Enrico; Di Piazza, Fabio; Lanti, Alessandro

    2018-06-01

    In our retrospective study, 16 patients affected by advanced cutaneous T cell lymphoma (CTCL) underwent allogeneic hematopoietic stem cell transplantation (HSCT). Two patients (12.5%) were in complete remission (CR), nine (56.3%) in partial remission (PR), and five (31.2%) with active disease. The patients were transplanted from an HLA-identical (n = 7) from a mismatched (n = 1) or haploidentical (n = 1) sibling, from matched unrelated donor (n = 5), or from a single cord blood unit (n = 2). Conditioning regimen was standard myeloablative in 6 patients and at reduced intensity in 10. Seven patients died from non relapse mortality (NRM) and four patients relapsed or progressed, three of them achieved a second CR after donor lymphocyte infusion (DLI) or chemotherapy plus DLI. To date, with a median follow-up of 76 months (range 6-130), nine patients are alive, eight in CR, and one with active disease. Overall survival (OS) and disease-free survival (DFS) at 1 and 10 years are 61% (95% CI 40-91%) and 54% (95% CI 33-86%), 40% (95% CI 22-74%), and 34% (95% CI 16-68%), respectively. The time from diagnosis to transplant seems to influence negatively both OS (log-rank p < 0.04) and DFS (log-rank p < 0.05). Our results confirm on a long follow-up that CTCL appears particularly susceptible to the graft versus lymphoma (GVL) effect, so that allogeneic HSCT represents a possibility of cure for advanced CTCL. The timing of HSCT in the clinical course of disease remains an open issue.

  20. Molecular Mechanisms of Disease Progression in Primary Cutaneous Diffuse Large B-Cell Lymphoma, Leg Type during Ibrutinib Therapy

    Directory of Open Access Journals (Sweden)

    Lucy C. Fox

    2018-06-01

    Full Text Available Primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL-LT is one of the well-recognized extranodal lymphomas commonly addicted to the B-cell receptor-MYD88 superpathway. We aimed to describe the genomic changes in a patient who progressed through treatment with ibrutinib, a Bruton’s tyrosine kinase (BTK inhibitor. An 80-year-old woman presented with multiply relapsed PCDLBCL-LT after multiple lines of chemoimmunotherapy and radiotherapy. Pre-treatment testing of the localized cutaneous tumor lesion on a lymphoid amplicon panel demonstrated an MYD88 p.L265P mutation. Ibrutinib therapy was subsequently commenced, resulting in complete resolution of the skin disease. Despite an ongoing skin response, the patient developed progressive nodal disease at two months. Genomic analysis of the cutaneous tumor sample at baseline was compared to that of the inguinal lymph node upon progression, and revealed the acquisition of multiple genomic changes. These included several aberrations expected to bypass BTK inhibition, including two CARD11-activating mutations, and a deleterious mutation in the nuclear factor kappa B (NF-κB negative regulator, NFKBIE. In addition, an IgH-IRF8 translocation was detected (which brings the IRF8 transcription factor under control of the immunoglobulin heavy chain locus, representing a third plausible mechanism contributing to ibrutinib resistance. Several copy-number changes occurred in both samples, including an amplification of 18q, which encodes the anti-apoptotic protein BCL2. We describe the first case of novel genomic changes of PCDLBCL-LT that occurred while on ibrutinib, providing important mechanistic insights into both pathogenesis and drug resistance.

  1. Primary lymphoma of the brain

    Science.gov (United States)

    Brain lymphoma; Cerebral lymphoma; Primary lymphoma of the central nervous system; Lymphoma - brain ... The cause of primary brain lymphoma is not known. People with a weakened immune system are at high risk for primary lymphoma of the brain. ...

  2. Clinico-Radiologic Findings in Primary Cutaneous Extranodal Natural Killer/T-Cell Lymphoma, Nasal Type Mimicking Cellulitis of the Left Arm

    International Nuclear Information System (INIS)

    Kim, Soo Hyun; Seon, Hyun Ju; Choi, Yoo Duk; Yun, Sook Jung

    2015-01-01

    Extranodal natural killer (NK)/T-cell lymphoma is a very rare and aggressive disease characterized histopathologically by an Epstein-Barr virus (EBV)-positive atypical lymphoid cytotoxic infiltrate, extensive vascular destruction, and prominent tissue necrosis. It commonly shows cutaneous lesions that primarily or secondarily mimic cellulitis at the primary site. We report on a very rare case of extranodal NK/T-cell lymphoma, nasal type of skin/soft tissue, in a 64-year-old man, and describe the radiological findings. The condition was misdiagnosed as cellulitis of the left arm based on initial noninvasive clinical and radiologic work-up

  3. Very late onset small intestinal B cell lymphoma associated with primary intestinal lymphangiectasia and diffuse cutaneous warts

    OpenAIRE

    Bouhnik, Y; Etienney, I; Nemeth, J; Thevenot, T; Lavergne-Slove, A; Matuchansky, C

    2000-01-01

    As only a handful of lymphoma cases have been reported in conjunction with primary intestinal lymphangiectasia, it is not yet clear if this association is merely fortuitous or related to primary intestinal lymphangiectasia induced immune deficiency. We report on two female patients, 50 and 58 years old, who developed small intestinal high grade B cell lymphoma a long time (45 and 40 years, respectively) after the initial clinical manifestations of primary intestinal lymphangiectasia. They pre...

  4. Excellent outcome of immunomodulation or Bruton’s tyrosine kinase inhibition in highly refractory primary cutaneous diffuse large B-cell lymphoma, leg type

    Directory of Open Access Journals (Sweden)

    Eva Gupta

    2015-12-01

    Full Text Available Primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL-LT is a rare diffuse large B-cell lymphoma confined to the skin of the legs. The typical presentation is characterized by solitary or multiple growing plaques, usually confined to one leg. We report a case of PCDLBCL-LT of activated B-cell subtype characterized by multiple local relapses in the legs, initially, and systemic relapses about seven years after the diagnosis. Local relapses were sensitive to radiation therapy. Cutaneous and systemic relapses responded well to immunomodulatory therapy with lenalidomide followed by Bruton’s tyrosine kinase inhibition with ibrutinib. Ibrutinib is the only treatment that resulted in long-lasting complete remission. Lenalidomide and especially ibrutinib appear to have a significant activity against this lymphoma and should be incorporated in the treatment of this resistant and aggressive lymphoma. To our knowledge, this is the first case of PCDLBCL-LT reported in the literature exhibiting a complete response to ibrutinib.

  5. Primary cutaneous marginal zone B-cell lymphoma with amyloid deposition: report of two cases with review of literature.

    Science.gov (United States)

    Zhang, Hai-Yan; Liu, An-Li; Zhou, Ling-sheng; He, Miao-Xia; Wang, Jian-Xin

    2010-06-01

    Amyloid deposition is rare. If there was a great amount of amyloid depositions in the skin tissue, it would be considered to be amyloid deposition disease at first, and then primary cutaneous marginal zone B-cell lymphoma (PCMZL). This study was to analyze the diagnosis and differential diagnosis of two cases of PCMZL with amyloid deposition. Clinicopathologic characteristics and follow-up of two cases of PCMZL were analyzed. Immunohistochemical staining was performed by EnVision method using antibodies LCA, CD19, CD20, CD79a, CD3, CD7, MUM1, kappa, lambda, Ki-67. IgH and TCRgamma gene rearrangement was detected by polymerase chain reactive (PCR). Case 1, a 71-year-old Chinese male, had a subcutaneous mass on the right elbow that was initially diagnosed with "amyloidosis" in 2004. Three years after the initial diagnosis, he developed recurrences on the right para-auxillary that was still diagnosed with "probably amyloidosis". Four years after the first diagnosis, the patient presented a lesion on the right para-auxillary with a diameter of 2 cm and a lesion on the temporal-parietal dural with a size of 6.0 cmx3.0 cmx3.0 cm. Case 2, a 68-year-old Chinese male, had a subcutaneous mass next to back of the left ear with a size of 9.0 cmx5.0 cm, and he underwent a operation one year previously because of subcutaneous mass in the same site. Microscopically, the tumors of both cases were located in dermis and subcutaneous, tumor cells were medium size with a nodular or diffuse distribution, and some of tumor cells were plasmacytoid/plasma cells. Morphologically, the temporal-parietal dural lesion was similar to subcutaneous lesion and infiltrated into cranial (case 1). Juxtaposed the tumor cells of two cases, there were the large amyloid deposits of amorphous hyaline material and concentrically laminated hyaline spherules in case 1, while cord-like amyloid deposits in case 2. Reactive lymphoid follicles with germinal centers and foreign body giant cells in the stroma were

  6. Primary cutaneous B-cell lymphoma is associated with somatically hypermutated immunoglobulin variable genes and frequent use of VH1-69 and VH4-59 segments.

    Science.gov (United States)

    Perez, M; Pacchiarotti, A; Frontani, M; Pescarmona, E; Caprini, E; Lombardo, G A; Russo, G; Faraggiana, T

    2010-03-01

    Accurate assessment of the somatic mutational status of clonal immunoglobulin variable region (IgV) genes is relevant in elucidating tumour cell origin in B-cell lymphoma; virgin B cells bear unmutated IgV genes, while germinal centre and postfollicular B cells carry mutated IgV genes. Furthermore, biases in the IgV repertoire and distribution pattern of somatic mutations indicate a possible antigen role in the pathogenesis of B-cell malignancies. This work investigates the cellular origin and antigenic selection in primary cutaneous B-cell lymphoma (PCBCL). We analysed the nucleotide sequence of clonal IgV heavy-chain gene (IgVH) rearrangements in 51 cases of PCBCL (25 follicle centre, 19 marginal zone and seven diffuse large B-cell lymphoma, leg-type) and compared IgVH sequences with their closest germline segment in the GenBank database. Molecular data were then correlated with histopathological features. We showed that all but one of the 51 IgVH sequences analysed exhibited extensive somatic hypermutations. The detected mutation rate ranged from 1.6% to 21%, with a median rate of 9.8% and was independent of PCBCL histotype. Calculation of antigen-selection pressure showed that 39% of the mutated IgVH genes displayed a number of replacement mutations and silent mutations in a pattern consistent with antigenic selection. Furthermore, two segments, VH1-69 (12%) and VH4-59 (14%), were preferentially used in our case series. Data indicate that neoplastic B cells of PBCBL have experienced germinal centre reaction and also suggest that the involvement of IgVH genes is not entirely random in PCBCL and that common antigen epitopes could be pathologically relevant in cutaneous lymphomagenesis.

  7. Primary malignant intramedullary lymphoma

    International Nuclear Information System (INIS)

    Orrego P, E.; Heinicke Y, H.; Arbaiza A, D.; Yepez R, V.

    1999-01-01

    A case of primary malignant intramedullary lymphoma, localized in the dorsal part of the spinal cord is presented. The clinical symptoms were associated with motor and sensitive deficit. Clinical investigations excluded the presence of lymphoma in other locations in the central nervous system and the extra neural organs. Postoperative radiotherapy and chemotherapy improved relict neurological symptoms. (authors)

  8. An overview of cutaneous T cell lymphomas [version 1; referees: 2 approved

    Directory of Open Access Journals (Sweden)

    Nooshin Bagherani

    2016-07-01

    Full Text Available Cutaneous T cell lymphomas (CTCLs are a heterogeneous group of extranodal non-Hodgkin’s lymphomas that are characterized by a cutaneous infiltration of malignant monoclonal T lymphocytes. They typically afflict adults with a median age of 55 to 60 years, and the annual incidence is about 0.5 per 100,000. Mycosis fungoides, Sézary syndrome, and primary cutaneous peripheral T cell lymphomas not otherwise specified are the most important subtypes of CTCL. CTCL is a complicated concept in terms of etiopathogenesis, diagnosis, therapy, and prognosis. Herein, we summarize advances which have been achieved in these fields.

  9. Malignant T cells express lymphotoxin alpha and drive endothelial activation in cutaneous T cell lymphoma

    DEFF Research Database (Denmark)

    Lauenborg, Britt; Christensen, Louise; Ralfkiaer, Ulrik

    2015-01-01

    Lymphotoxin α (LTα) plays a key role in the formation of lymphatic vasculature and secondary lymphoid structures. Cutaneous T cell lymphoma (CTCL) is the most common primary lymphoma of the skin and in advanced stages, malignant T cells spreads through the lymphatic to regional lymph nodes...

  10. Cutaneous B-cell lymphoma : classification, prognostic factors and management recommendations

    NARCIS (Netherlands)

    Senff, Nancy Johanna

    2009-01-01

    The term primary cutaneous B-cell lymphomas refers to a heterogeneous group of B-cell non-Hodgkin lymphomas, that present in the skin without evidence of extracutaneous disease at the time of diagnosis. In recent years, there has been considerable debate regarding the classification and terminology

  11. Primary cutaneous b-cell lymphoma successfully treated with highly active antiretroviral therapy alone: A case report and review of the literature

    Directory of Open Access Journals (Sweden)

    María F Villafañe

    2011-01-01

    Full Text Available Cutaneous B-cell lymphoma (CBCL is an unusual skin neoplasm with a great range of clinical presentations. Here, we report a case of CBCL in an AIDS patient presented as a single and nodular/ulcerative lesion in the perianal area. The patient was started on highly active antiretroviral therapy alone with a good clinical and oncological response. Two years later, the patient is asymptomatic with undetectable viral load and immune reconstitution.

  12. Intravesicular taxane-induced dermatotoxicity in a 78-year-old man with urothelial carcinoma and primary cutaneous anaplastic large cell lymphoma.

    Science.gov (United States)

    J Pelletier, Daniel; O'Donnell, Michael; Stone, Mary Seabury; Liu, Vincent

    2018-06-01

    Patients treated with intravesical bacillus Calmette-Guérin therapy for urothelial carcinoma often become refractory and experience recurrent disease, thus necessitating alternative intravesical treatment modalities if the patient is to be spared the morbidities associated with radical cystectomy. Intravesical treatment with taxane-based chemotherapy, such as docetaxel, has gained traction in urologic oncology, proving to be an effective salvage therapy in such patients. Systemic taxane-based chemotherapeutic regimens have long been used in several advanced malignancies, and their systemic side-effects and associated histologic correlates have been extensively documented. In contrast to adverse effects associated with systemic administration, intravesical taxane administration has thus far proven to be well-tolerated, with little to no systemic absorption. To our knowledge, features of taxane-induced systemic effects have not been reported in this setting. Herein, we report a case of a patient with recurrent urothelial carcinoma treated with intravesical docetaxel, along with primary cutaneous anaplastic large cell lymphoma, who developed characteristic dermatotoxic histologic findings associated with intravenous taxane administration. As such histopathologic findings often represent close mimickers of neoplastic and infectious etiologies, knowledge of the potential for systemic manifestations of taxane therapy in patients treated topically may prevent potentially costly diagnostic pitfalls. © 2018 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  13. Human herpesvirus 8-associated lymphoma mimicking cutaneous anaplastic large T-cell lymphoma in a patient with human immunodeficiency virus infection.

    Science.gov (United States)

    Li, Meng-Fang; Hsiao, Cheng-Hsiang; Chen, Yi-Lin; Huang, Wen-Ya; Lee, Yi-Hsuan; Huang, Hsien-Neng; Lien, Huang-Chun

    2012-02-01

    Primary effusion lymphoma, a human herpesvirus 8 (HHV8)-associated lymphoma, is uncommon, and it is usually seen in human immunodeficiency virus (HIV)-infected patients. It presents as a body cavity-based lymphomatous effusion, but several cases of the so-called solid primary effusion lymphoma presenting as solid tumors without associated lymphomatous effusion have been reported. They have similar clinical, histopathological and immunophenotypical features. Most of them have a B-cell genotype. This suggests the solid variant may represent a clinicopathological spectrum of primary effusion lymphoma. We report a case of HHV8-associated lymphoma histopathologically and immunophenotypically mimicking cutaneous anaplastic large cell lymphoma. The patient was a 31-year-old HIV-seropositive man presenting with skin nodules over his right thigh. Biopsy of the nodules showed anaplastic large cells infiltrating the dermis. These malignant cells strongly expressed CD3, CD30 and CD43. Cutaneous anaplastic large T-cell lymphoma was initially diagnosed, but further tests, including immunoreactivity for HHV8 protein and clonal rearrangements of immunoglobulin genes, confirmed the diagnosis of HHV8-associated B-cell lymphoma with aberrant T-cell marker expression. This case provides an example of solid primary effusion lymphoma mimicking cutaneous anaplastic large T-cell lymphoma and highlights the importance of HHV8 immunohistochemistry and molecular tests in the diagnosis of HHV8-associated lymphoma with a cutaneous presentation. Copyright © 2011 John Wiley & Sons A/S.

  14. Current and Emerging Therapeutics for Cutaneous T-Cell Lymphoma: Histone Deacetylase Inhibitors

    OpenAIRE

    Annabelle L. Rodd; Katherine Ververis; Tom C. Karagiannis

    2012-01-01

    Cutaneous T-cell lymphoma is a term that encompasses a spectrum of non-Hodgkin’s T-cell lymphomas with primary manifestations in the skin. It describes a heterogeneous group of neoplasms that are characterised by an accumulation of malignant T cells of the CD4 phenotype that have the propensity to home and accumulate in the skin, lymph nodes, and peripheral blood. The two most common variants of cutaneous T-cell lymphoma include mycosis fungoides and the leukemic variant, the Sézary syndrome....

  15. Isolated cutaneous involvement in a child with nodal anaplastic large cell lymphoma

    Directory of Open Access Journals (Sweden)

    Vibhu Mendiratta

    2016-01-01

    Full Text Available Non-Hodgkin lymphoma is a common childhood T-cell and B-cell neoplasm that originates primarily from lymphoid tissue. Cutaneous involvement can be in the form of a primary extranodal lymphoma, or secondary to metastasis from a non-cutaneous location. The latter is uncommon, and isolated cutaneous involvement is rarely reported. We report a case of isolated secondary cutaneous involvement from nodal anaplastic large cell lymphoma (CD30 + and ALK + in a 7-year-old boy who was on chemotherapy. This case is reported for its unusual clinical presentation as an acute febrile, generalized papulonodular eruption that mimicked deep fungal infection, with the absence of other foci of systemic metastasis.

  16. Cutaneous manifestations of non-Hodgkin's lymphoma.

    Science.gov (United States)

    Kumar, S S; Kuruvilla, M; Pai, G S; Dinesh, M

    2003-01-01

    Thirty-two confirmed cases of non -Hodgkin's lymphoma (NHL) were examined for cutaneous manifestations for a period of 2 years from November 1998 in KMC Hospital Attavar, Mangalore. Cutaneous manifestations in the study group were compared to a control group of 32 patients. Specific infiltrates were present in all (5/5) CTCL patients and one out of twenty-seven patients with low grade NHL. Morphologically they presented as papules, plaques, nodules and erythroderma. Infective conditions seen in the study group were superficial fungal (7/32) and viral infections (2/ 32). Non-infective conditions were acquired ichthyosis (10/32), generalised pruritus (5/32), insect bite reaction (1/32) and drug eruption (1/32). When compared to control patients only acquired ichthyosis and generalised pruritus were found to be statistically significant. The study group also showed changes due to chemotherapy like diffuse alopecia (24/29), bluish pigmentation of proximal part of nail (4/29), localised pigmentation of palms and soles (1 /29), diffuse pigmentation at injection site (1 /29), pigmentation at scar site (1 /29) and stomatitis (4/29).

  17. Breast Cancer Mimic: Cutaneous B-Cell Lymphoma Presenting as an Isolated Breast Mass

    Directory of Open Access Journals (Sweden)

    Margaret Taghavi

    2014-10-01

    Full Text Available Background: Primary cutaneous B-cell lymphoma typically localizes to the skin, and dissemination to internal organs is rare. Lymphomatous involvement of the breasts is also rare. We describe the clinical and radiological findings of an unusual case of primary cutaneous B-cell lymphoma presenting as an isolated breast mass without associated skin changes. Case Presentation: The patient was a 55-year-old Caucasian female who initially presented with cutaneous B-cell lymphoma around her eyes and forehead with recurrence involving the skin between her breasts. Three years after terminating treatment due to a lack of symptoms, she presented for an annual screening mammogram that found a new mass in her upper inner right breast without imaging signs of cutaneous extension. On physical examination, there were no corresponding skin findings. Due to the suspicious imaging features of the mass that caused concern for primary breast malignancy, she underwent a core biopsy which revealed cutaneous B-cell lymphoma. Conclusion: When evaluating patients with a systemic disease who present with findings atypical for that process, it is important to still consider the systemic disease as a potential etiology, particularly with lymphoma given its reputation as a great mimicker.

  18. Characterization of primary cutaneous CD8+/CD30+ lymphoproliferative disorders.

    Science.gov (United States)

    Martires, Kathryn J; Ra, Seong; Abdulla, Farah; Cassarino, David S

    2015-11-01

    CD30 primary cutaneous lymphoproliferative diseases include both lymphomatoid papulosis (LyP) and primary cutaneous anaplastic large cell lymphoma (PCALCL). The neoplastic cell of most primary CD30 lymphoproliferative disorders is CD4 positive. The terminology LyP "type D" has been used to describe a growing number of cases of LyP with a predominantly CD8 infiltrate. PCALCL with a CD8 phenotype has also been described, which presents a particularly difficult diagnostic and management challenge, given the difficulty in distinguishing it histologically from other cytotoxic lymphomas such as primary cutaneous aggressive epidermotropic CD8 cytotoxic T-cell lymphoma and CD8 gamma/delta and natural killer/T-cell lymphoma. We report 7 additional cases of these rare cutaneous CD8/CD30 lymphoproliferative disorders. We also present a unique case of CD8/CD30 LyP with histologic similarities to LyP type B. In all 7 of our cases of CD8 LyP and CD8 anaplastic large cell lymphoma, we found focal to diffuse MUM-1 positivity. We propose that MUM-1 may represent an adjunctive marker for CD8 lymphoproliferative disease. Finally, we review the current literature on cases of CD8 LyP and PCALCL. For the 106 cases examined, we found similar clinical and histologic features to those reported for traditional CD4CD30 LyP and PCALCL.

  19. Molecular analysis of immunoglobulin variable genes supports a germinal center experienced normal counterpart in primary cutaneous diffuse large B-cell lymphoma, leg-type.

    Science.gov (United States)

    Pham-Ledard, Anne; Prochazkova-Carlotti, Martina; Deveza, Mélanie; Laforet, Marie-Pierre; Beylot-Barry, Marie; Vergier, Béatrice; Parrens, Marie; Feuillard, Jean; Merlio, Jean-Philippe; Gachard, Nathalie

    2017-11-01

    Immunophenotype of primary cutaneous diffuse large B-cell lymphoma, leg-type (PCLBCL-LT) suggests a germinal center-experienced B lymphocyte (BCL2+ MUM1+ BCL6+/-). As maturation history of B-cell is "imprinted" during B-cell development on the immunoglobulin gene sequence, we studied the structure and sequence of the variable part of the genes (IGHV, IGLV, IGKV), immunoglobulin surface expression and features of class switching in order to determine the PCLBCL-LT cell of origin. Clonality analysis with BIOMED2 protocol and VH leader primers was done on DNA extracted from frozen skin biopsies on retrospective samples from 14 patients. The clonal DNA IGHV sequence of the tumor was aligned and compared with the closest germline sequence and homology percentage was calculated. Superantigen binding sites were studied. Features of selection pressure were evaluated with the multinomial Lossos model. A functional monoclonal sequence was observed in 14 cases as determined for IGHV (10), IGLV (2) or IGKV (3). IGV mutation rates were high (>5%) in all cases but one (median:15.5%), with superantigen binding sites conservation. Features of selection pressure were identified in 11/12 interpretable cases, more frequently negative (75%) than positive (25%). Intraclonal variation was detected in 3 of 8 tumor specimens with a low rate of mutations. Surface immunoglobulin was an IgM in 12/12 cases. FISH analysis of IGHM locus, deleted during class switching, showed heterozygous IGHM gene deletion in half of cases. The genomic PCR analysis confirmed the deletions within the switch μ region. IGV sequences were highly mutated but functional, with negative features of selection pressure suggesting one or more germinal center passage(s) with somatic hypermutation, but superantigen (SpA) binding sites conservation. Genetic features of class switch were observed, but on the non functional allele and co-existing with primary isotype IgM expression. These data suggest that cell-of origin is

  20. New targeted treatments for cutaneous T-cell Lymphomas

    Directory of Open Access Journals (Sweden)

    Martine Bagot

    2017-01-01

    Full Text Available Cutaneous T-cell lymphomas (CTCLs represent a group of rare and heterogeneous diseases that are very difficult to treat at advanced stages. The development of monoclonal antibodies is a new hope for the treatment of these diseases. Alemtuzumab (Campath is a humanized IgG1 kappa monoclonal antibody specific for CD52, an antigen expressed by most T and B lymphocytes. Alemtuzumab may frequently induce long-term remissions in patients with Sezary syndrome but high-dose treatments lead to severe cytopenia, immune depletion, and opportunistic infections. This treatment is less efficient in mycosis fungoides (MF. Brentuximab vedotin is a chimeric anti-CD30 monoclonal antibody conjugated to monomethyl auristatin E, a cytotoxic antitubulin agent. Brentuximab vedotin is a very interesting new treatment for advanced tumor MF, Sezary syndrome, and primary cutaneous CD30+ lymphoproliferative disorders. The main limiting adverse event is neurosensitive peripheral neuropathy. Mogamulizumab is a humanized anti-C-C chemokine receptor Type 4 monoclonal antibody with a defucosylated Fc region leading to increased antibody-dependent cellular cytotoxicity. Mogamulizumab is very efficient on aggressive peripheral T-cell lymphomas, particularly adult T-cell leukemia/lymphoma and CTCLs, especially on the blood component of tumor cells. The main limiting events are related to the concomitant depletion of regulatory T-cells. IPH4102 is a humanized monoclonal antibody that targets the immune receptor KIR3DL2/CD158k. Preclinical results with this antibody offer proofs of concept for the clinical development of IPH4102 to treat patients with advanced CTCL.

  1. Importance of gallium-67 scintigraphy in primary cutaneous B-cell lymphoma: report of two cases;Importancia da cintilografia com galio-67 no linfoma cutaneo primario de celulas B: relato de dois casos

    Energy Technology Data Exchange (ETDEWEB)

    Attab, Cyomara Sanches, E-mail: rubinha.attab@terra.com.b [Centro Brasileiro de Medicina Nuclear (CEBRAMEN), Goiania, GO (Brazil); Moriguchi, Sonia Marta [Fundacao Pio XII, Barretos, SP (Brazil). Hospital de Cancer; Paton, Eduardo Jose Alencar [Fundacao Pio XII, Barretos, SP (Brazil). Cancer Hospital. Div. of Hemotherapy and Bone-Marrow Transplant; Alencar, Mario Henrique Leite de [Clinica de Prevencao em Cancer, Goiania, GO, (Brazil); Rocha, Euclides Timoteo da [Fundacao Pio XII, Barretos, SP (Brazil). Cancer Hospital. Dept. of Nuclear Medicine

    2010-05-15

    The authors describe two cases of cutaneous B-cell lymphoma where correct staging, treatment and follow-up could be achieved through a combination of conventional imaging studies and gallium-67 scintigraphy. (author)

  2. Primary intracranial malignant lymphoma

    International Nuclear Information System (INIS)

    Matsumoto, Mikiro; Ohtsuka, Takatsugu; Kuroki, Takao; Shibata, Iekado; Terao, Hideo; Kudo, Motoshige

    1988-01-01

    Nine cases of primary intracranial malignant lymphoma, which accounts for 3.3 % of all intracranial tumors seen in the authors' institution, were studied in terms of diagnostic computed tomographic (CT) features, the tumors' histologic appearance, treatment, post-treatment blood immunologic and cerebrospinal fluid (CSF) characteristics, and outcome. The patients were seven males and two females aged 42 to 67 years. Their chief signs and symptoms on admission were intracranial hypertension, focal signs, and disturbance of consciousness. CT, which proved the most useful preoperative diagnostic technique, demonstrated multiple lesions in seven cases and, in all cases, regions of isodensity or slight high density that were enhanced by contrast medium. According to the patterns of enhancement, the tumors were classed as diffuse (three cases) or nodular (six cases). The former is considered typical of malignant lymphoma, whereas the latter type was sometimes indistinguishable from metastatic tumor and meningioma. At surgery, one patient underwent radical tumor excision, two partial removal, and six biopsy only. Histologic examination revealed one tumor to be of the diffuse small cell type, three of the medium cell type, and five of the large cell type (Lymphoma Study Group classification). Of seven tumors in which lymphocytes were examined by peroxidase-antiperoxidase staining, four were of the B cell type. Postoperatively, whole brain irradiation with 29 to 46 Gy was followed by local irradiation with 15 to 50 Gy. If the tumor persisted, one of three chemotherapies was administered. In one case, methotrexate was given intrathecally. Seven patients were divided into two groups: long remission (three) and recurrence (four). These two groups were compared in terms of serum immunoglobulin levels, T and B cell ratios, CSF characteristics, CT features, tumor cell type, and treatment. No clear differences were found. (author)

  3. A novel xenograft model of cutaneous T-cell lymphoma

    DEFF Research Database (Denmark)

    Krejsgaard, Thorbjørn; Kopp, Katharina; Ralfkiaer, Elisabeth

    2010-01-01

    Cutaneous T-cell lymphomas (CTCLs) are characterized by accumulation of malignant T cells in the skin. Early disease resembles benign skin disorders but during disease progression cutaneous tumors develop, and eventually the malignant T cells can spread to lymph nodes and internal organs. However...... and lymphatic tumors, originated from the transplanted malignant T cells. In conclusion, we describe a novel mouse model of tumor stage CTCL for future studies of disease dissemination and preclinical evaluations of new therapeutic strategies....

  4. Primary and secondary cutaneous CD30(+) lymphoproliferative disorders : a report from the Dutch Cutaneous Lymphoma Group on the long-term follow-up data of 219 patients and guidelines for diagnosis and treatment

    NARCIS (Netherlands)

    Bekkenk, MW; Geelen, FAMJ; Vader, PCV; Heule, F; Geerts, ML; van Vloten, WA; Meijer, CJLM; Willemze, R

    2000-01-01

    To evaluate our diagnostic and therapeutic guidelines, clinical and long-term follow-up data of 219 patients with primary or secondary cutaneous CD30(+) lympho proliferative disorders were evaluated. The study group included 118 patients with lymphomatoid papulosis (LyP; group 1), 79 patients with

  5. Primary and secondary cutaneous CD30(+) lymphoproliferative disorders: a report from the Dutch Cutaneous Lymphoma Group on the long-term follow-up data of 219 patients and guidelines for diagnosis and treatment

    NARCIS (Netherlands)

    Bekkenk, M. W.; Geelen, F. A.; van Voorst Vader, P. C.; Heule, F.; Geerts, M. L.; van Vloten, W. A.; Meijer, C. J.; Willemze, R.

    2000-01-01

    To evaluate our diagnostic and therapeutic guidelines, clinical and long-term follow-up data of 219 patients with primary or secondary cutaneous CD30(+) lymphoproliferative disorders were evaluated. The study group included 118 patients with lymphomatoid papulosis (LyP; group 1), 79 patients with

  6. Cutaneous double-hit B-cell lymphoma: an aggressive form of B-cell lymphoma with a propensity for cutaneous dissemination.

    Science.gov (United States)

    Magro, Cynthia M; Wang, Xuan; Subramaniyam, Shivakumar; Darras, Natasha; Mathew, Susan

    2014-04-01

    Diffuse large cell B-cell lymphoma of the skin is most commonly represented by diffuse large cell variants of primary cutaneous follicle center cell lymphoma and the leg-type lymphoma. In a minority of cases, the infiltrates are an expression of stage 4 disease of established extracutaneous B-cell lymphoma. We describe 3 patients with an aggressive form of B-cell lymphoma secondarily involving the skin. Two of the patients were in the ninth decade of life, whereas 1 patient was 34 years of age. In the elderly patients, there was an antecedent and/or concurrent history of follicular lymphoma, whereas in the younger patient, the tumor was a de novo presentation of this aggressive form of lymphoma. The elderly patients succumbed to their disease within less than a year from the time of diagnosis, whereas 1 patient is alive but with persistent and progressive disease despite chemotherapeutic intervention. The infiltrates in all 3 cases were diffuse and composed of large malignant hematopoietic cells that exhibited a round nucleus with a finely dispersed chromatin. Phenotypically, the tumor cells were Bcl-2 and CD10 positive, whereas Bcl-6 and Mum-1 showed variable positivity. One case showed combined Mum-1 positivity along with an acute lymphoblastic lymphoma phenotype, including the absence of CD20 expression. In each case, there was a c-MYC and BCL2/IGH rearrangement diagnostic of double-hit lymphoma. In one case, there was an additional BCL6 rearrangement, defining what is in essence triple-hit lymphoma. In conclusion, double-hit lymphoma is an aggressive form of B-cell neoplasia resistant to standard chemotherapy regimens, which in many but not all cases represents tumor progression in the setting of a lower grade B-cell malignancy.

  7. Cutaneous lesions as presentation form of mantle cell lymphoma

    Directory of Open Access Journals (Sweden)

    Nayra Merino de Paz

    2011-12-01

    Full Text Available Mantle cell lymphoma is a type of no-Hodgkin lymphoma that affects extranodal areas, especially, bone narrow, digestive tract and Waldeyer ring. Here we report a case of mantle cell lymphoma IV Ann Arbor stage with cutaneous lesions on nasal dorsum and gland as the first manifestations. Skin involvement is a very rare manifestation and less than 20 cases have been reported in the literature. The importance of stablishing multidisciplinary relationships for a global approach has been shown by this clinical case.

  8. Primary intracerebral lymphoma: Case report

    Directory of Open Access Journals (Sweden)

    Olcay Eser

    2012-09-01

    Full Text Available We describe a case of primary central nervous lymphoma (PCNSL that may be confused with magnetic resonance imaging (MRI findings of high grade glioma. Primary central nervous lymphoma is a rare tumour and it account for 0.3-3% of intracranial tumours. A 61 year’s old woman was admitted to our clinic with a severe headache, vomiting, left hemiparesia and transient loss of consciousness. Primary central nervous lymphoma may show various biological and radiological characteristics. We herein emphasized being confused with MRI findings of PCNSL and high grade glioma. J Clin Exp Invest 2012; 3 (3: 409-411Key words: Primary central nervous lymphoma, high grade glioma, B-cell, diagnosis

  9. Cutaneous lymphoid hyperplasia mimicking cutaneous lymphoma in a hyperthyroid cat

    OpenAIRE

    Snead, Elisabeth; Kerr, Moira; MacDonald, Valerie

    2013-01-01

    A 12-year-old neutered male domestic shorthair cat presented for chronic, localized, swelling and crusting of the left upper lip, weight loss, sporadic vomiting, and focal alopecia between the scapulae was diagnosed with hyperthyroidism and regional eosinophilic lymphadenitis. Treatment with methimazole exacerbated an underlying hypersensitivity disorder leading to marked generalized lymphadenopathy that histologically mimicked lymphoma.

  10. Primary splenic lymphoma

    International Nuclear Information System (INIS)

    Aslam, M.; Salamat, N.; Mamoon, N.; Ahmed, M.

    2006-01-01

    A middle-aged lady presented with fever and splenomegaly and had been provisionally treated for malaria, typhoid and tuberculosis. Diagnostic splenectomy was performed which revealed diffuse large cell lymphoma, B type, localized to spleen. Patient had remission of disease after splenectomy. (author)

  11. Primary splenic lymphoma

    Energy Technology Data Exchange (ETDEWEB)

    Aslam, M [Combined Military Hospital, Multan (Pakistan). Dept. of Surgery; Salamat, N [Combined Military Hospital, Multan (Pakistan). Dept. of Pathology; Mamoon, N [Armed Forces Inst. of Pathology, Rawalpindi (Pakistan). Dept. of Histopathology; Ahmed, M [Combined Military Hospital, Multan (Pakistan). Dept. of Medicine

    2006-04-15

    A middle-aged lady presented with fever and splenomegaly and had been provisionally treated for malaria, typhoid and tuberculosis. Diagnostic splenectomy was performed which revealed diffuse large cell lymphoma, B type, localized to spleen. Patient had remission of disease after splenectomy. (author)

  12. Primary thyroid lymphoma: CT findings

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Hyo-Cheol; Han, Moon Hee E-mail: hanmh@radcom.snu.ac.kr; Kim, Keon Ha; Jae, Hwan Jun; Lee, Sang Hyun; Kim, Sam Soo; Kim, Kwang Hyun; Chang, Kee-Hyun

    2003-06-01

    Introduction: To evaluate the computed tomographic (CT) findings of primary thyroid lymphoma. Methods and material: The clinicopathological data and CT images of nine patients with primary thyroid lymphoma were retrospectively reviewed. The CT appearances were classified into three types: type 1, a solitary nodule surrounded by normal thyroid tissue; type 2, multiple nodules in the thyroid, and type 3, a homogeneously enlarged both thyroid glands with a reduced attenuation with or without peripheral thin hyperattenuating thyroid tissue. Results: All patients had a rapidly enlarging thyroid mass and coexistent Hashimoto's thyroiditis. One patient showed type 1 pattern, three type 2, and five type 3. Six patients had homogeneous tumor isoattenuating to surrounding muscles. The tumors had a strong tendency to compress normal remnant thyroid and the surrounding structure without invasion. Conclusion: Primary thyroid lymphoma should be included in the differential diagnosis when old female had a homogeneous thyroidal mass isoattenuating to muscles, which does not invade surrounding structures.

  13. Human immunodeficiency virus-positive secondary syphilis mimicking cutaneous T-cell lymphoma.

    Science.gov (United States)

    Yamashita, Michiko; Fujii, Yoshiyuki; Ozaki, Keiji; Urano, Yoshio; Iwasa, Masami; Nakamura, Shingen; Fujii, Shiro; Abe, Masahiro; Sato, Yasuharu; Yoshino, Tadashi

    2015-10-08

    Malignant syphilis or lues maligna is a severe form of secondary syphilis that was commonly reported in the pre-antibiotic era, and has now reemerged with the advent of the human immunodeficiency virus (HIV) epidemic. However, the characteristic histopathological findings of malignant syphilis remain controversial. The aim of this case report was to clarify the clinical and histopathological findings of HIV-positive malignant secondary syphilis. A Japanese man in his forties complained of fever, skin lesions, headache, and myalgia without lymphadenopathy during the previous 4 weeks. The skin lesions manifested as erythematous, nonhealing, ulcerated papules scattered on his trunk, extremities, palm, and face. Although the skin lesions were suspected to be cutaneous T-cell lymphomas on histological analyses, they lacked T-cell receptor Jγ rearrangement; moreover, immunohistochemical analyses confirmed the presence of spirochetes. The patient was administered antibiotics and anti-retroviral therapy, which dramatically improved the symptoms. On the basis of these observations of the skin lesions, we finally diagnosed the patient with HIV-associated secondary syphilis that mimicked cutaneous T-cell lymphoma. The patient's systemic CD4+ lymphocyte count was very low, and the infiltrate was almost exclusively composed of CD8+ atypical lymphocytes; therefore, the condition was easily misdiagnosed as cutaneous lymphoma. Although the abundance of plasma cells is a good indicator of malignant syphilis on skin histological analyses, in some cases, the plasma cell count may be very low. Therefore, a diagnosis of malignant secondary syphilis should be considered before making a diagnosis of primary cutaneous peripheral T-cell lymphoma or lymphoma associated with HIV infection.

  14. Allogeneic stem cell transplantation for advanced cutaneous T-cell lymphomas: a study from the French Society of Bone Marrow Transplantation and French Study Group on Cutaneous Lymphomas

    Science.gov (United States)

    de Masson, Adèle; Beylot-Barry, Marie; Bouaziz, Jean-David; de Latour, Régis Peffault; Aubin, François; Garciaz, Sylvain; d’Incan, Michel; Dereure, Olivier; Dalle, Stéphane; Dompmartin, Anne; Suarez, Felipe; Battistella, Maxime; Vignon-Pennamen, Marie-Dominique; Rivet, Jacqueline; Adamski, Henri; Brice, Pauline; François, Sylvie; Lissandre, Séverine; Turlure, Pascal; Wierzbicka-Hainaut, Ewa; Brissot, Eolia; Dulery, Rémy; Servais, Sophie; Ravinet, Aurélie; Tabrizi, Reza; Ingen-Housz-Oro, Saskia; Joly, Pascal; Socié, Gérard; Bagot, Martine

    2014-01-01

    The treatment of advanced stage primary cutaneous T-cell lymphomas remains challenging. In particular, large-cell transformation of mycosis fungoides is associated with a median overall survival of two years for all stages taken together. Little is known regarding allogeneic hematopoietic stem cell transplantation in this context. We performed a multicenter retrospective analysis of 37 cases of advanced stage primary cutaneous T-cell lymphomas treated with allogeneic stem cell transplantation, including 20 (54%) transformed mycosis fungoides. Twenty-four patients (65%) had stage IV disease (for mycosis fungoides and Sézary syndrome) or disseminated nodal or visceral involvement (for non-epidermotropic primary cutaneous T-cell lymphomas). After a median follow up of 29 months, 19 patients experienced a relapse, leading to a 2-year cumulative incidence of relapse of 56% (95%CI: 0.38–0.74). Estimated 2-year overall survival was 57% (95%CI: 0.41–0.77) and progression-free survival 31% (95%CI: 0.19–0.53). Six of 19 patients with a post-transplant relapse achieved a subsequent complete remission after salvage therapy, with a median duration of 41 months. A weak residual tumor burden before transplantation was associated with increased progression-free survival (HR=0.3, 95%CI: 0.1–0.8; P=0.01). The use of antithymocyte globulin significantly reduced progression-free survival (HR=2.9, 95%CI: 1.3–6.2; P=0.01) but also transplant-related mortality (HR=10−7, 95%CI: 4.10−8–2.10−7; P<0.001) in univariate analysis. In multivariate analysis, the use of antithymocyte globulin was the only factor significantly associated with decreased progression-free survival (P=0.04). Allogeneic stem cell transplantation should be considered in advanced stage primary cutaneous T-cell lymphomas, including transformed mycosis fungoides. PMID:24213148

  15. Cutaneous manifestations of primary immunodeficiency

    Directory of Open Access Journals (Sweden)

    Safa Abdelhakim

    2017-01-01

    Full Text Available Primary immunodeficiency diseases (PIDs are a group of rare, chronic disorders with deficient or malfunctioning immune system. It commonly affects the hematopoietic system, with skin the second most affected organ. Skin involvement is observed in half of pediatric PID cases and often precedes the final diagnosis. Skin infections and eczemas are the two most common manifestations in PID.[1] Skin manifestations associated with PIDs can be of infectious and noninfectious causes. Common noninfectious causes are eczema, erythroderma, cutaneous granulomas, dysplasia, vasculitis, and telangiectasia. It is important to be aware of skin manifestations in pediatric patients as early detection of PID may aid in the management of serious immunologic conditions and prevent associated morbidity and mortality.

  16. Imaging Cutaneous T-Cell Lymphoma with Optical Coherence Tomography

    Directory of Open Access Journals (Sweden)

    Hans Christian Ring

    2012-07-01

    Full Text Available Aim: To investigate the presentation of a patch-stage cutaneous T-cell lymphoma (CTCL using optical coherence tomography (OCT. Methods: A patient with a patch caused by CTCL was photographed digitally, OCT-scanned and biopsied. A normal skin area adjacent to the patch was OCT-scanned for comparison, but not biopsied. The OCT image and the histological image were compared. Results: The OCT images illustrated a thickened and hyperreflective stratum corneum. OCT also demonstrated several elongated hyporeflective structures in the dermis. The largest structure was measured to have a width of 0.13 mm. A good immediate correlation was found between histology and OCT imaging of the sample. Conclusion: The aetiology of the elongated structures is thought to be lymphomatous infiltrates. Similar findings have been described in ocular lymphoma and may therefore be an important characteristic of cutaneous lymphoma. It may further be speculated that the differences in OCT images may reflect the biological behaviour of the infiltrate. This observation therefore suggests that OCT imaging may be a relevant tool for the in vivo investigation of mycosis fungoides and other CTCLs, but in order to verify these observed patterns in OCT imaging, further investigations will be required.

  17. Malignant T Cells Secrete Galectins and Induce Epidermal Hyperproliferation and Disorganized Stratification in a Skin Model of Cutaneous T Cell Lymphoma

    DEFF Research Database (Denmark)

    Thode, Christenze; Andersen, Anders Woetmann; Wandall, Hans H

    2015-01-01

    Cutaneous T cell lymphomas (CTCL) are the most common primary skin lymphomas; which are characterized by an accumulation of malignant T cells in the skin. The early lesion resembles both clinically and histologically benign inflammatory disorders, which also presents with hyperproliferative epide...... in CTCL.Journal of Investigative Dermatology accepted article preview online, 09 July 2014; doi:10.1038/jid.2014.284....

  18. Primary cerebral lymphoma: radiological findings

    International Nuclear Information System (INIS)

    Ruiz, J.C.; Grandse, D.; Equidazu, J.; Elizagaray, E.; Grande, J.; Carrandi, J.

    1990-01-01

    We present four cases of primary cerebral lymphoma in non-immunodepressed adult patients. All cases were dsemonstrated with pathological study. CAT study showed solitary or multiple isodense lesions, which incorporated avidly and homoneneously the contrast. Arteriography performed in three patients and magnetic resonance, performed in one did not help for diagnosis. We also review the radiological findings obtained with different imaging methods, and suggest the criteria which could be useful for early diagnosis (Author)

  19. Primary Cutaneous Aspergillosis in an Immunocompetent Patient

    African Journals Online (AJOL)

    are the lungs, central nervous system (CNS), and sinuses, however, the rare cutaneous infection is usually associated with immunodeficiency. Primary cutaneous infection, especially in immunocompetent patients, is extremely rare, but an increase in prevalence has been noted in the last. 20 years. The predisposing factors ...

  20. Primary "cutaneous" T-cell anaplastic large cell lymphoma, CD30+, neutrophil-rich variant with subcutaneous panniculitic lesions, in a post-renal transplant patient: report of unusual case and literature review.

    Science.gov (United States)

    Salama, S

    2005-06-01

    Posttransplantation lymphoproliferative disorders (PTLD) presenting clinically in the skin are rare and usually of B-cell phenotype. Only 7 cases of cutaneous T-cell PTLD have been previously reported, mostly mycosis fungoides type, with no known cases of "cutaneous" presentation by CD30 (Ki-1) anaplastic large cell lymphoma (ALCL). The case reported is a 59-year-old male who developed multiple skin nodules on the right leg, 6 years following renal transplantation. Initial biopsy showed ALCL involving the dermis with a background rich in neutrophils. The neoplastic cells were of T-cell phenotype, strongly CD30 with typical staining, and BCL-2 positive, but P53 negative. No EBV was detected by IHC, ISH, or DNA analysis. One year later, he developed painful subcutaneous nodules with surrounding erythema, resembling deep pustules or panniculitis, which on biopsy showed preferential involvement of the subcutaneous fat and prominent component of neutrophils. Twenty-two months following diagnosis, he died of cardiac failure with terminal myocardial infarct. There was however no clinical evidence of systemic spread of the lymphoma.This report adds to the clinical and morphologic spectrum of these rare "cutaneous" lymphomas of T-cell lineage arising in the posttransplantation setting, and suggests that EBV does not play a role in their pathogenesis.

  1. A novel splice variant of the Fas gene in patients with cutaneous T-cell lymphoma.

    Science.gov (United States)

    van Doorn, Remco; Dijkman, Remco; Vermeer, Maarten H; Starink, Theo M; Willemze, Rein; Tensen, Cornelis P

    2002-10-01

    Defective apoptosis signaling has been implicated in the pathogenesis of primary cutaneous T-cell lymphomas (CTCLs), a group of malignancies derived from skin-homing T cells. An important mediator of apoptosis in T cells is the Fas receptor. We identified a novel splice variant of the Fas gene that displays retention of intron 5 and encodes a dysfunctional Fas protein in 13 of 22 patients (59%) in both early and advanced CTCL. Impairment of Fas-induced apoptosis resulting from aberrant splicing potentially contributes to the development and progression of CTCL by allowing continued clonal expansion of activated T cells and by reducing susceptibility to antitumor immune responses.

  2. Small cell lymphocytic variant of marginal zone lymphoma: A distinct form of marginal zone lymphoma derived from naïve B cells as a cutaneous counterpart to the naïve marginal zone lymphoma of splenic origin.

    Science.gov (United States)

    Magro, Cynthia M; Olson, Luke C

    2018-02-21

    Primary cutaneous marginal zone lymphoma most commonly represents an indolent form of cutaneous B cell lymphoma. However, epidermotropic marginal zone lymphoma, blastic marginal zone lymphoma and B cell dominant variants without isotype switching can be associated with extracutaneous dissemination. The presumptive cell of origin is a post germinal center B cell with plasmacytic features. In the extracutaneous setting, however, a naïve B cell origin has been proposed for a subset of marginal zone lymphomas, notably splenic marginal zone lymphoma. The author encountered 11 cases of atypical lymphocytic infiltration of the skin primarily occurring in older individuals with an upper arm and head and neck localization; there was a reproducible pattern of diffuse and nodular infiltration by small monomorphic-appearing B cells. Phenotypically, the infiltrate was one predominated by B cells exhibiting CD23 and IgD positivity without immunoreactivity for CD38 and there were either no plasma cells or only a few without light chain restriction. In cases presenting with a solitary lesion complete excision and/or radiation led to successful disease remission in all cases without recurrence or metastatic disease. Of three cases with multiple initial lesions, evidence of extracutaneous disease was seen in two cases and recurrence occurred in one case. No patients have died of lymphoma. Longer term follows up and additional cases are needed to determine if this subset of marginal zone lymphoma is associated with a worse prognosis. Copyright © 2018. Published by Elsevier Inc.

  3. Radiotherapy in primary cerebral lymphoma

    International Nuclear Information System (INIS)

    Legros, L.; Benezery, K.; Lagrange, J.L.

    1999-01-01

    Primary cerebral lymphoma is a rare disease with an unfavorable prognosis. Whole brain radiotherapy has been the standard treatment, but neither the optimal radiation fields nor optimal dose level of the regimen are as yet firmly establisheD. From this review of the literature, it seems that the whole brain must be treated, and a boost to the area of the primary site must be discussed. With regard to dose, the radiation dose-response relationship is not clearly proven. Yet, a minimum dose of 40 Gy is necessary, and the maximum dose is set at 50 Gy because of late neurological sequelae. Because of the poor prognosis of this disease and the risk of late sequelae, other avenues have been explored. Chemotherapy has been studied, seem to have a survival advantage and combinations of radiotherapy and chemotherapy, especially with high-dose methotrexate. Because primary cerebral lymphoma is an uncommon disease, randomized clinical trials that compare radiotherapy alone to chemotherapy plus radiotherapy may not be feasible. Finally, even if chemotherapy seems to have a survival advantage, the regimen of chemotherapy is still a matter of debate. (authors)

  4. IgV(H) and bcl6 somatic mutation analysis reveals the heterogeneity of cutaneous B-cell lymphoma, and indicates the presence of undisclosed local antigens.

    Science.gov (United States)

    Franco, Renato; Camacho, Francisca I; Fernández-Vázquez, Amalia; Algara, Patrocinio; Rodríguez-Peralto, José L; De Rosa, Gaetano; Piris, Miguel A

    2004-06-01

    Our understanding of the ontology of B-cell lymphomas (BCL) has been improved by the study of mutational status of IgV(H) and bcl6 genes, but only a few cases of cutaneous BCL have been examined for this status. We analyzed IgV(H) and bcl6 somatic mutations in 10 cutaneous BCL, classified as follicular (three primary and one secondary), primary marginal zone (two cases), and diffuse large BCL (three primary and one secondary). We observed a lower rate (IgV(H) mutation in all marginal zone lymphomas, and a preferential usage of V(H)2-70 (one primary follicular and two primary diffuse large BCL). Fewer than expected replacement mutations in framework regions (FR) were observed in three primary follicular lymphomas (FLs) and in all diffuse large BCL, indicating a negative antigen selection pressure. Ongoing mutations were observed in eight of 10 cases. Only two primary FLs and two diffuse large BCL showed bcl6 somatic mutation. These data support the heterogeneous nature of the different cutaneous BCL, and specifically the distinction between cutaneous follicular and marginal zone lymphomas. The biased usage of V(H)2-70, the low rate of replacement mutation in the FR, and the presence of ongoing mutation imply that local antigens could modulate the growth of primary cutaneous BCL.

  5. Primary periosteal lymphoma - rare and unusual

    Energy Technology Data Exchange (ETDEWEB)

    Abdelwahab, Ibrahim F. [Coney Island Hospital, Department of Radiology, New York, NY (United States); Hoch, Benjamin [Mount Sinai School of Medicine, CUNY, Department of Pathology, New York, NY (United States); Hermann, George [Mount Sinai School of Medicine, CUNY, Department of Radiology, New York, NY (United States); Bianchi, Stefano [Clinique et Fondation des Grangettes, Geneva (Switzerland); Klein, Michael J. [UAB School of Medicine, Department of Pathology, Birmingham, AL (United States); Springfield, Dempsey S. [Mount Sinai School of Medicine, CUNY, Department of Orthopedics, New York, NY (United States)

    2007-04-15

    We describe a primary periosteal lymphoma that involved only the periosteum without affecting the adjacent medulla or the regional lymph nodes. No other lymphomatous foci were found in either the distant lymph nodes or viscera. This unusual presentation simulates the imaging appearance of surface lesions of bone, namely benign and malignant tumors, and departs from the typical appearance of primary lymphoma of bone. Therefore, this rare type of lymphoma should be considered in the differential diagnosis of surface bone lesions. (orig.)

  6. Lymphoma of the eyelid

    DEFF Research Database (Denmark)

    Svendsen, Frederik Holm; Heegaard, Steffen

    2017-01-01

    Lymphoma of the eyelid constitutes 5% of ocular adnexal lymphoma. In previously published cases, 56% of lymphomas of the eyelid are of B-cell origin and 44% are of T-cell origin. The most frequent B-cell lymphomas are extranodal marginal zone lymphoma (27 cases-14%) and diffuse large B......-cell lymphoma (18 cases-9%). T-cell lymphomas are most frequently mycosis fungoides (25 cases-13%), extranodal natural killer/T-cell, nasal-type lymphoma (12 cases-6%), and primary cutaneous anaplastic large-cell lymphoma (12 cases-6%). This distribution differs from the distribution of ocular adnexal lymphoma...... and that of cutaneous lymphoma. The majority of subtypes occur in elderly patients, except for lymphoblastic lymphoma of B-cell and T-cell origin and Burkitt lymphoma, which occur in children and adolescents. Several subtypes have a male predominance, including peripheral T-cell lymphoma and Burkitt lymphoma. Only...

  7. Imaging of primary pediatric lymphoma of bone

    International Nuclear Information System (INIS)

    Milks, Kathryn S.; McLean, Thomas W.; Anthony, Evelyn Y.

    2016-01-01

    Primary pediatric bone lymphoma is a rare form of non-Hodgkin lymphoma. Unlike nodal forms of lymphoma, imaging abnormalities in lymphoma of bone do not resolve rapidly in conjunction with treatment and radiologic findings can remain abnormal for years, making it difficult to evaluate treatment response. To evaluate the utility of imaging in assessment of patients with primary pediatric bone lymphoma. At our institution between 2004 and 2013, six cases of pathology-proven primary pediatric bone lymphoma were diagnosed. Retrospective chart review was performed to assess imaging utilization. Our data were qualitatively compared with existing literature to construct an algorithm for imaging patients with primary lymphoma of bone. Imaging evaluation of patients with primary pediatric bone lymphoma was highly variable at our institution. Conventional imaging was routinely used to evaluate response to treatment, despite lack of appreciable osseous change. Imaging in the absence of symptoms did not alter clinical management. Only positron emission tomography CT (PET/CT) proved capable of demonstrating imaging changes from the pretreatment to the post-treatment scans that were consistent with the clinical response to treatment. Surveillance imaging is likely unnecessary in patients with a known diagnosis of pediatric lymphoma of bone. Pretreatment and post-treatment PET/CT is likely sufficient to assess response. There is little data to support the use of interim and surveillance PET/CT. (orig.)

  8. Spontaneous regression of primary diffuse large B-cell lymphoma, leg type.

    Science.gov (United States)

    Alcántara-González, J; González-García, C; Fernández-Guarino, M; Jaén-Olasolo, P

    2014-01-01

    Primary cutaneous diffuse large B-cell lymphoma, leg type (PCLBCL LT) accounts for approximately 20% of all primary cutaneous B-cell lymphomas and tends to present as infiltrated nodules, tumors, and plaques on the legs in the elderly. Unlike other primary cutaneous large B-cell lymphomas, it has a poor prognosis and tends to require treatment with systemic chemotherapy. We present the case of an 82-year-old patient with a 1-year history of nodules and plaques on her right leg. Biopsy led to a diagnosis of PCLBCL LT and the lesions resolved without treatment within 1 month of the first visit. This is an atypical course of PCLBCL LT and we believe that it is the first such case to be reported in the literature. Copyright © 2012 Elsevier España, S.L. and AEDV. All rights reserved.

  9. Transformation of a Cutaneous Follicle Center Lymphoma to a Diffuse Large B-Cell Lymphoma—An Unusual Presentation

    Directory of Open Access Journals (Sweden)

    J. Dias Coelho

    2010-01-01

    Full Text Available Primary cutaneous follicle center lymphoma (PCFCL is characterized by a proliferation of follicle center cells in the skin. A definitive diagnosis is frequently delayed because of difficulties in interpretation of the histopathologic findings. It has an excellent prognosis with a 5-year survival over 95% and its risk of transformation has not been established. We describe a case report of man with a gastric diffuse large B-cell lymphoma (DLBCL referred to our clinic because of nodules in the back that had gradually developed over a period of 10 years. A biopsy performed 3 years before was interpreted as reactive follicular hyperplasia. A new skin biopsy revealed a diffuse large B-cell lymphoma and immunoglobulin heavy chain gene rearrangements from the initial skin biopsy (PCBCL and the DLBCL gastric biopsy were studied by polymerase chain reaction and an identical clonal rearrangement was detected which was highly suggestive of a transformation lymphoma.

  10. Primary Hepatic Lymphoma Mimicking Cholangiocarcinoma

    Directory of Open Access Journals (Sweden)

    Foroogh Forghani1,

    2017-07-01

    Full Text Available Primary hepatic lymphoma (PHL presenting with obstructive jaundice is rare and can mimic a preoperative diagnosis of cholangiocarcinoma. We should consider PHL in patients with radiological hepatic disease with normal serum alpha-fetoprotein and carcinoembryonic antigen levels, and elevated lactate dehydrogenase. We present the case of a 67-year-old male with no significant medical history presented with abdominal pain, jaundice, fever, and abnormal liver function tests. Abdominal sonography and computed tomography scan suggested a diagnosis of obstructive jaundice and cholangitis due to cholangiocarcinoma (Klatskin tumor. A subsequent liver biopsy diagnosed PHL, and the patient was treated with combination chemotherapy, including rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP. PHL should be considered in patients presenting with biliary obstruction.

  11. Primary multifocal osseous lymphoma in a child

    Energy Technology Data Exchange (ETDEWEB)

    Sato, Takashi S.P. [University of Iowa, Carver College of Medicine, Iowa City, IA (United States); Ferguson, Polly J. [University of Iowa, Department of Pediatrics, Iowa City, IA (United States); Khanna, Geetika [Washington University, Mallinckrodt Institute of Radiology, St Louis, MO (United States)

    2008-12-15

    We report a case of primary multifocal osseous lymphoma in a 6-year-old girl presenting with multifocal osteolytic lesions without systemic symptoms or identifiable non-osseous primary tumor. The differential diagnoses for such a presentation include histiocytosis X, chronic recurrent multifocal osteomyelitis, acute lymphoblastic leukemia, metastatic disease, and primary bone lymphoma. Although non-Hodgkin lymphoma is common in the pediatric population, its presentation as a primary bone tumor, especially with multifocal disease, is extremely rare and is frequently misdiagnosed. We hope that awareness of this entity will help radiologists achieve timely diagnosis and intervention. (orig.)

  12. Primary Testicular B-cell Lymphoma

    Directory of Open Access Journals (Sweden)

    Aykut Buğra Şentürk

    2015-12-01

    Full Text Available Primary testicular lymphoma constitutes only 1-7% of all testicular neoplasms and less than 1% of all non-Hodgkin lymphoma. We report a 69-year-old man who presented with a painful right testicular mass. Treatment modalities consist of surgical excision, chemotherapy and radiation therapy, however there are no standardized treatment options.

  13. Clinicopathological study of primary gastric lymphoma

    International Nuclear Information System (INIS)

    Al-Shehabi, Zubeir A.; Saleh, Rana S.; Zezafon, Hassan B.

    2007-01-01

    Objective was to present a histopathologic and immunohistochemical analysis of primary gastric lymphomas that was reclassified according to the new World Health Organization classification of lymphoid neoplasms. We reviewed the morphological and immunohistochemical features of 28 patients with gastric lymphomas, diagnosed in the Department of pathology at the University Hospital of Tishreen University, Lattakia, Syria, during the period 1994-2003. Specimens were obtained from endoscopic and surgical biopsies. The immunohistochemical study was performed to analyze the immunophenotype of these lymphomas. Patients were aged 17-71 years. There was a slight predominance of females (male to female ratio, 13:15). Seventeen of the patients had tumors mainly located in the gastric antrum. Histologically, the most common lymphoma was of mucosa-associated lymphoid tissue (MALT) type (20 patients), also with diffuse large B-cell lymphoma (7 patients) and anaplastic large cell lymphoma (one patient). Our study demonstrates the different patterns of gastric lymphomas in Lattakia, Syria during a 10-year period in 28 Syrian patients, and reveals that the most primary gastric lymphomas are B-cell MALT lymphomas. (author)

  14. Primary lymphocytic lymphoma of lacrimal gland.

    Science.gov (United States)

    Romero-Caballero, M D; Lozano-García, I; Gómez-Molina, C; Gil-Liñán, A I; Arcas, I

    2017-02-01

    We report a case of primary small-cell lymphocytic lacrimal gland lymphoma in a male diagnosed with primary antiphospholipid syndrome. These rare lymphomas are usually presented in the clinic as disseminations secondary to chronic lymphocytic leukaemia, and the primary site is rare in the orbit. Non-Hodgkin lymphomas are a heterogeneous group of tumours. Although treatment in the IE stage is usually radiotherapy, due to its association with antiphospholipid syndrome, systemic treatment with rituximab was administered. Copyright © 2016 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L.U. All rights reserved.

  15. Radiotherapy of primary gastric malignant lymphoma

    International Nuclear Information System (INIS)

    Monzen, Yoshio; Mutsukura, Masahide; Moriuchi, Yukiyoshi

    2017-01-01

    Fifteen patients with primary gastric malignant lymphoma who underwent radiotherapy were examined. Median age was 68 years, and male to female ratio was 1:2. All the cases were stage I including 7 cases of diffuse large B-cell lymphoma (DLBCL), 7 cases of MALT lymphoma, and 1 case of follicular lymphoma. Therapy methods were as follows. For DLBCL, 30 Gy of radiotherapy was performed after chemotherapy. For six cases of MALT lymphomas, 30 Gy of radiotherapy was performed. For one patient diagnosed as high-grade gastric MALT lymphoma was treated in the same way as DLBCL. For one patient with follicular lymphoma, 30 Gy of radiotherapy was performed. The radiotherapy was applied with 3-dimensional fixed multi-portal irradiation, with the reduced irradiation of the liver and kidney. There was no recurrence of disease in all cases, and all patients have been alive, and no-recurrence living periods are 20 to 120 months. There was no harmful adverse event, and the tumor had disappeared with 30 Gy of radiation therapy in all cases. Considering the occurrence of secondary cancer, it was considered that a dosage of more than 30 Gy was not necessary for primary gastric malignant lymphoma. (J.P.N.)

  16. Bilateral primary malignant lymphoma of the breast.

    Science.gov (United States)

    Shpitz, B; Witz, M; Kaufman, Z; Griffel, B; Manor, Y; Dinbar, A

    1985-08-01

    A rare case of bilateral primary malignant lymphoma of breast in a 76 year old woman is presented. The lesion was examined by electron microscopy and immunochemistry. The diagnosis of primary malignant lymphoma remains a diagnosis by exclusion and requires extensive work-up to exclude widespread malignant process. The behaviour of this malignancy tends to be an aggressive one and the prognosis is generally poor.

  17. Primary Vitreoretinal Lymphoma Masquerading as Refractory Retinitis

    Directory of Open Access Journals (Sweden)

    Ofira Zloto

    2015-10-01

    Full Text Available Purpose: To report a case of a patient with primary vitreoretinal lymphoma masquerading as retinitis. Methods: Retrospective review of the patient's clinical, histopathological and imaging records. Results: Cytopathology was negative for malignancy, and preliminary polymerase chain reaction results supported the diagnosis of varicella zoster virus retinitis. Therefore, the patient was treated with antiviral therapy. However, under this treatment, the retinitis progressed. As a result, primary vitreoretinal lymphoma was suspected, and empirical treatment with intravitreal methotrexate injections was started. Under this treatment, the ocular features improved. Five months after initial ocular presentation and ocular resolution, the patient presented with central nervous system lymphoma. Conclusion: This case should raise the awareness of the variable clinical presentations, the challenging diagnosis and treatment of primary vitreoretinal lymphoma. All cases should be continuously systemically evaluated.

  18. Radiotherapy for the primary ocular adnexal lymphoma

    International Nuclear Information System (INIS)

    Yu Dahai; Sun Sanyuan; Zhuo Shichao; Wang Haiwei

    2007-01-01

    Objective: To study the pathological and clinical characteristics of primary lymphoma of ocular adnexae, analyze the treatment results and discuss the methods to prevent radiation complications. Methods: From Feb. 1995 to Feb. 2004, 25 patients with primary ocular adnexal lymphoma were treated in the second hospital and the forth hospital of Xuzhou, including 11 males and 14 females. The diagnosis was confirmed pathologically by biopsy in 19 patients and lumpectomy in 6 patients, including 22 mucosa-associated lymphoid tissue (MALT) lymphoma and 3 non-MALT lymphoma. According to the Ann Arbor Staging System, there were 21 patients with tumor in stage I E, 3 in stage II E and 1 in stage III E. The primary tumor was found in the eyelid or conjunctiva in 19 eyes and orbit in 9 eyes. Radiotherapy were given to 22 patients (25 eyes) by deep X-rays, 60 Co γ-rays or mixed beams. The total irradiation dose ranged from 30.0 to 57.6 Gy. Kaplan-Meier method was used to calculate the survival rate and Logrank test was used to detect the difference between the different groups. Results: The 5-, 10-year accumulated survival rates (SR) of the whole group were 90% and 82%. The 10-year SR of patients with primary, eyelid or conjunctiva tumor and orbit tumor were 100% and 58% (P=0.032). The local control rates of the radiotherapy group and non-radiotherapy group were 92% and 33 % (P=0.006). The 10-year SR of patients with tumor completely removed and those with residues were 83% and 82% (P=0.907). The 10-year SR of MALT lymphoma and non-MALT lymphoma were 90.0% and 33.3% (P=0.009). After radiotherapy, 8 eyes (36%) had cataract formation and 7 eyes (28%) had xerophalmic symptoms. Conclusions: The results of radiothera- py for the primary ocular adnexal lymphoma are satisactory. The prognosis of patients with primary, eyelid or conjunctiva tumor is better than those with orbit tumor. The vast majority of the primary ocular adnexal lymphomas are MALT lymphomas. The survival rate of

  19. Primary Hepatosplenic Large B-Cell Lymphoma

    Directory of Open Access Journals (Sweden)

    M.R. Morales-Polanco

    2008-03-01

    Full Text Available Diffuse large B-cell lymphoma is the most common form of lymphoma. It usually begins in the lymph nodes; up to 40% may have an extranodal presentation. According to a definition of primary extranodal lymphoma with presentation only in extranodal sites, there are reports of large B-cell lymphomas limited to liver or spleen as separate entities, and to date there have been only three documented cases of primary hepatosplenic presentation. This paper reports a fourth case. Due to a review of the literature and the clinical course of the case reported, we conclude that primary hepatosplenic large B-cell lymphoma has been found predominantly in females older than 60 years. The patients reported had <2 months of evolution prior to diagnosis, prominent B symptoms, splenomegaly in three and hepatomegaly in two, none with lymph node involvement. All had thrombocytopenia and abnormal liver function tests; three had anemia and elevated serum lactic dehydrogenase levels, two with hemophagocytosis in bone marrow. Because of the previously mentioned data, it can be stated that primary hepatosplenic lymphoma is an uncommon and aggressive form of disease that requires immediate recognition and treatment.

  20. Fundus autofluorescence patterns in primary intraocular lymphoma.

    Science.gov (United States)

    Casady, Megan; Faia, Lisa; Nazemzadeh, Maryam; Nussenblatt, Robert; Chan, Chi-Chao; Sen, H Nida

    2014-02-01

    To evaluate fundus autofluorescence (FAF) patterns in patients with primary intraocular (vitreoretinal) lymphoma. Records of all patients with primary intraocular lymphoma who underwent FAF imaging at the National Eye Institute were reviewed. Fundus autofluorescence patterns were evaluated with respect to clinical disease status and the findings on fluorescein angiography and spectral-domain optical coherence tomography. There were 18 eyes (10 patients) with primary intraocular lymphoma that underwent FAF imaging. Abnormal autofluorescence in the form of granular hyperautofluorescence and hypoautofluorescence was seen in 11 eyes (61%), and blockage by mass lesion was seen in 2 eyes (11%). All eyes with granular pattern on FAF had active primary intraocular lymphoma at the time of imaging, but there were 5 eyes with unremarkable FAF, which were found to have active lymphoma. The most common pattern on fluorescein angiography was hypofluorescent round spots with a "leopard spot" appearance (43%). These hypofluorescent spots on fluorescein angiography correlated with hyperautofluorescent spots on FAF in 5 eyes (36%) (inversion of FAF). Nodular hyperreflective spots at the level of retinal pigment epithelium on optical coherence tomography were noted in 43% of eyes. The hyperautofluorescent spots on FAF correlated with nodular hyperreflective spots on optical coherence tomography in 6 eyes (43%). Granularity on FAF was associated with active lymphoma in majority of the cases. An inversion of FAF (hyperautofluorescent spots on FAF corresponding to hypofluorescent spots on fluorescein angiography) was observed in less than half of the eyes.

  1. Notch 1 as a potential therapeutic target in cutaneous T-cell lymphoma

    DEFF Research Database (Denmark)

    Kamstrup, Maria Rørbæk; Gjerdrum, Lise Mette Rahbek; Biskup, Edyta Urszula

    2010-01-01

    Deregulation of Notch signaling has been linked to the development of T-cell leukemias and several solid malignancies. Yet, it is unknown whether Notch signalling is involved in the pathogenesis of mycosis fungoides and Sezary syndrome, the most common subtypes of cutaneous T cell lymphoma....... By immunohistochemistry of 40 biopsies taken from skin lesions of mycosis fungoides and Sezary syndrome we demonstrated prominent expression of Notch1 on tumor cells, especially in the more advanced stages. The gamma-secretase inhibitor I blocked Notch signaling and potently induced apoptosis in cell lines derived from...... mycosis fungoides (MyLa) and Sezary syndrome (SeAx, HuT-78)and in primary leukemic Sézary cells. Specific downregulation of Notch1 (but not Notch2 and Notch3) by siRNA induced apoptosis in SeAx. The mechanism of apoptosis involved the inhibition of NF-kappaB, which is the most important prosurvival...

  2. Management of cutaneous T cell lymphoma: new and emerging targets and treatment options

    Directory of Open Access Journals (Sweden)

    Li JY

    2012-03-01

    Full Text Available Janet Y Li1, Steven Horwitz2, Alison Moskowitz2, Patricia L Myskowski3, Melissa Pulitzer4, Christiane Querfeld31College of Physicians and Surgeons, Columbia University, 2Department of Medicine, Lymphoma Service, 3Department of Medicine, Dermatology Service, 4Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, NY, USAAbstract: Cutaneous T cell lymphomas (CTCL clinically and biologically represent a heterogeneous group of non-Hodgkin lymphomas, with mycosis fungoides and Sézary syndrome being the most common subtypes. Over the last decade, new immunological and molecular pathways have been identified that not only influence CTCL phenotype and growth, but also provide targets for therapies and prognostication. This review will focus on recent advances in the development of therapeutic agents, including bortezomib, the histone deacetylase inhibitors (vorinostat and romidepsin, and pralatrexate in CTCL.Keywords: novel targets, histone deacetylase inhibitors, pralatrexate, bortezomib, cutaneous T cell lymphoma

  3. Linfoma cutâneo primário de grandes células B de apresentação atípica: relato de caso Primary cutaneous large B-cell lymphoma of atypical presentation: case report

    Directory of Open Access Journals (Sweden)

    Antônio René Diógenes de Sousa

    2011-06-01

    Full Text Available Linfomas cutâneos primários são definidos como neoplasias linfocíticas que se apresentam clinicamente na pele sem doença extracutânea no momento do diagnóstico e até por 6 meses após. Os autores relatam o caso de um paciente masculino, idoso, com história de pápulas em axila, há 3 meses, que evoluíram para ulceração. Ao exame, úlcera profunda de bordos irregulares, infiltrados, em axila direita. Exames físico e complementares não evidenciaram doença à distância. O histopatológico mostra infiltrado dérmico denso e difuso de linfócitos atípicos. A imuno-histoquímica evidencia expressão de antígenos CD20 e bcl-2, com CD10 negativo, configurando diagnóstico de linfoma cutâneo difuso de grandes células B. Neste tipo de linfoma, é rara a manifestação cutânea primária, assim como a incidência é menor em homens e a localização, mais comum em membros inferioresPrimary cutaneous lymphomas are defined as lymphocytic neoplasias that present themselves clinically in the skin without extracutaneous disease at diagnosis and up to 6 months after it. The authors report the case of an elderly male patient, with a three- month-history of papules in the axilla which evolved into painful ulceration. Examination found deep ulcer with irregular borders ,infiltrates, in the right axilla. Physical and additional examinations did not evidence disease at distance. Histopathology revealed dense and diffuse dermic sample infiltrate of atypical lymphocytes. Imunohistochemistry shows expression of CD20 and bcl-2 antigens , with negative CD10, configuring diagnosis of cutaneous large B-cell lymphoma. In this type of cutaneous lymphoma, primary cutaneous manifestation is rare ,the incidence in men is lower and it is most commonly located in the lower limbs

  4. [Primary central nervous system lymphoma mimicking ventriculitis].

    Science.gov (United States)

    Yamamoto, Shiro; Nagano, Seiji; Shibata, Sumiya; Kunieda, Takeharu; Imai, Yukihiro; Kohara, Nobuo

    2013-01-01

    A 66-year-old man presented with deteriorated bradykinesia, gait disturbance, disorientation, and urinary incontinence for three weeks. Magnetic resonance imaging (MRI) showed dilatation of the ventricles. Cerebrospinal fluid (CSF) examination demonstrated lymphocytic pleocytosis, elevation of protein levels, and decreased of glucose levels. A gadolinium-enhanced MRI revealed lesions in the ventricular wall and choroid plexus, mimicking ventriculitis. No evidence of bacterial, fungal, mycobacterial, or viral infections were observed in the CSF. Flow cytometry of CSF showed predominance of CD20+, λ+ cells. PCR examination of CSF revealed positive IgH gene rearrangement, suggesting B cell lymphoma. Endoscopic brain biopsy showed diffuse large B cell lymphoma. As the patient had no evidence of lymphoma in the other organs, we made a diagnosed of primary central nervous system lymphoma (PCNSL). A limited intraventricular spread of PCNSL is rare but important as one of differential diagnosis of ventriculitis.

  5. Primary lymphoma of the testis

    International Nuclear Information System (INIS)

    Buskirk, S.J.; Evans, R.G.; Banks, P.M.; O'Connell, M.J.; Earle, J.D.

    1982-01-01

    Seventeen patients with initial presentation of lymphoma of the testis were evaluated at the Mayo Clinic between 1969 and 1979. The mean age of the patients was 69 years with 15 of the 17 patients age 60 and older at the time of diagnosis. All histologies were diffuse according to the Rappaport classification with 12 of 17 patients being histiocytic. Eleven of the 15 State I/sub E/A and II/sub E/A patients were treated with radiation therapy alone with doses ranging from 2,600 to 4,000 rad. Eight of these 11 Stage I/sub E/A patients experienced recurrence; in five of these eight, the first site of recurrence was Waldeyer's ring and adjacent structures. Four patients were treated initially with chemotherapy. In all four patients the lymphoma recurred, in two patients in the central nervous system (CNS). The survival rate at two years was 73% in Stage I/sub E/A patients and 25% in Stage II/sub E/A patients. There were no survivors at two years in those patients presenting with Stage IV disease. As patients with testicular lymphoma have a relatively high incidence of secondary involvement of Waldeyer's ring and the CNS, careful evaluation of these areas should be performed as part of the routine staging procedures. In view of the high incidence of secondary involvement of distant sites, systemic treatment should be given full consideration in addition to local irradiation as part of the initial treatment of patients with localized disease

  6. Primary thoracic epidural lymphoma: A rare cause of spinal cord ...

    African Journals Online (AJOL)

    Spinal epidural lymphoma is a rare entity that is not often considered in the differential diagnosis of an epidural mass in a previously healthy individual. Pfatients with Primary Spinal Epidural Lymphomas (PSELs) have negative diagnostic work up for systemic lymphoma and unlike disseminated lymphoma, they achieve ...

  7. Primary lymphoma of appendix: Ultrasound finding

    International Nuclear Information System (INIS)

    Sotillos Parra, V.E.; Belda Serrano, J.; Mota Castilla, A.; Falomir Gil, G.; Abreu Maqueda, V.; Trigueris Sanchez, M.; Hernandez Barcelo, J.E.; Martinez Diaz, F.

    1994-01-01

    We present an uncommon case of primary lymphoma of the appendix in a patient who complained of discomfort in lower right quadrant. The findings revealed by ultrasound, barium enema and CT scan are reported and the diagnostic aspects of this appendiceal tumor and others are discussed. (Author) 6 refs

  8. Hypercalcemia due to Primary Hepatic Lymphoma

    Directory of Open Access Journals (Sweden)

    Andrew Hsu

    2016-01-01

    Full Text Available A 65-year-old female with a history of mixed connective tissue disease and pulmonary fibrosis on azathioprine, hydroxychloroquine, and prednisone (osteoporosis on teriparatide presented with a 1-month history of hypercalcemia. After discontinuation of teriparatide, the patient’s hypercalcemia persisted. Further evaluation revealed primary hepatic lymphoma as the source of her hypercalcemia.

  9. A Case of Diffuse Large B-Cell Lymphoma Mimicking Primary Effusion Lymphoma-Like Lymphoma

    Directory of Open Access Journals (Sweden)

    Daisuke Usuda

    2017-11-01

    Full Text Available A 93-year-old female was transferred to the emergency ward of our hospital due to disturbance of consciousness and hypotension. Computed tomography showed bilateral pleural and pericardial effusion without evidence of tumor masses or lymphadenopathy. Cytodiagnosis of pleural effusion revealed proliferation of atypical lymphoid-like cells with pan-B surface markers. We suspected primary effusion lymphoma-like lymphoma; however, the monoclonality of these cells was not confirmed. Cytodiagnosis of bone marrow revealed lymphoma cells with monoclonal B-cell markers. These findings prompted a diagnosis of diffuse large B-cell lymphoma with bone marrow invasion. In the case of pericardial or pleural effusion, clinicians should consider carefully both hematological malignancy and its classification.

  10. Primary brain lymphoma presenting as Parkinson's disease

    International Nuclear Information System (INIS)

    Sanchez-Guerra, M.; Leno, C.; Berciano, J.; Cerezal, L.; Diez, C.; Figols, J.

    2001-01-01

    Neoplasm is an uncommon cause of a parkinsonian syndrome. We report a woman with primary brain B-cell lymphoma presenting as Parkinson's disease. After 1 year of the illness, CT and MRI showed lesions without mass effect in the basal ganglia and corpus callosum. The patient did not respond to levodopa and right cerebellar and brain-stem signs appeared, which prompted further neuroimaging, showing an increase in size of the lesions and a right cerebellar and pontine mass. Stereotactic biopsy of the basal ganglia showed high-grade B-cell lymphoma. Despite the basal ganglia frequently being involved in lymphoma of the brain, presentation with typical or atypical parkinsonism is exceptional. (orig.)

  11. Advances in Primary Central Nervous System Lymphoma.

    Science.gov (United States)

    Patrick, Lauren B; Mohile, Nimish A

    2015-12-01

    Primary central nervous system lymphoma (PCNSL) is a rare form of non-Hodgkin lymphoma that is limited to the CNS. Although novel imaging techniques aid in discriminating lymphoma from other brain tumors, definitive diagnosis requires brain biopsy, vitreoretinal biopsy, or cerebrospinal fluid analysis. Survival rates in clinical studies have improved over the past 20 years due to the addition of high-dose methotrexate-based chemotherapy regimens to whole-brain radiotherapy. Long-term survival, however, is complicated by clinically devastating delayed neurotoxicity. Newer regimens are attempting to reduce or eliminate radiotherapy from first-line treatment with chemotherapy dose intensification. Significant advances have also been made in the fields of pathobiology and treatment, with more targeted treatments on the horizon. The rarity of the disease makes conducting of prospective clinical trials challenging, requiring collaborative efforts between institutions. This review highlights recent advances in the biology, detection, and treatment of PCNSL in immunocompetent patients.

  12. Potential involvement of Notch1 signalling in the pathogenesis of primary cutaneous CD30-positive lymphoproliferative disorders

    DEFF Research Database (Denmark)

    Kamstrup, M.R.; Ralfkiaer, E.; Skovgaard, G.L.

    2008-01-01

    Background The central role of Notch signalling in T-cell development and oncogenesis raises the question of the importance of this pathway in cutaneous T-cell lymphomas. Objectives To investigate the pattern of expression of Notch and its ligands, Jagged and Delta, in skin samples of primary...... obtained from three patients with LyP and two patients with primary cutaneous ALCL. Results We identified single Notch1-positive cells or small clusters of atypical cells in LyP. Similarly, strongly positive Jagged1 cells tended to be localized in clusters. Primary cutaneous ALCL had higher expression...... of Notch1 and Jagged1 compared with LyP. Cells expressing Notch1 and Jagged1 were colocalized and a subset of cells expressed both the receptor and the ligand. The expression of the ligand Delta1 was low to undetectable in both types of lymphoproliferations. A subpopulation of lymphoma cells was found...

  13. Devastating posttraumatic primary cutaneous mucormycosis in a diabetic patient

    Directory of Open Access Journals (Sweden)

    Poongodi Lakshmi Santhana Kumarasamy

    2016-01-01

    Full Text Available Mucorales are saprophytic fungi causing mucormycosis, which is a life threatening infection manifested as rhinocerebral, pulmonary, gastrointestinal, cutaneous, and disseminated forms. The cutaneous form is further divided into primary and secondary forms. The major risk factors include uncontrolled diabetes mellitus with or without ketoacidosis, other forms of metabolic acidosis, and trauma. We report here a case of primary cutaneous mucormycosis caused by Rhizopus oryzae, in a diabetic after a road traffic accident.

  14. Radiation therapy for primary orbital lymphoma

    International Nuclear Information System (INIS)

    Chao, Cliff K.S.; Lin Hsiusan; Rao Devineni, V.; Smith, Morton

    1995-01-01

    Purpose: The influence of tumor size, grade, thoroughness of staging workup, and radiation dose on disease control, radiation-related complications, and incidence of systemic progression of primary orbital lymphoma is analyzed. Methods and Materials: Twenty patients with Stage I primary orbital lymphoma were treated from August 1976 through August 1991 at Mallinckrodt Institute of Radiology. Staging workups included physical examination, chest x-ray, complete blood count (CBC), liver function test, and computerized tomography (CT) scan of the orbit, abdomen, and pelvis. Nineteen patients had bone marrow biopsy. The histological types based on the National Cancer Institute working formulation were 9 low-grade and 11 intermediate-grade, including five lymphocytic lymphomas of intermediate differentiation. The extension of disease and the volume of tumor were evaluated by CT scan of the orbit. The most commonly used radiation therapy technique was single anterior direct field with 4 MV or 6 MV photons. Lens was shielded or not treated in eight patients. Dose ranged from 20 to 43.2 Gy. Thirteen of 20 patients received 30 Gy. Minimum follow-up was 24 months (median, 4 years). Results: Local control was achieved in all 20 patients. One patient with lymphocytic lymphoma with intermediate differentiation developed disseminated disease. Actuarial disease-free survival (DFS) was 100% and 90% at 2 and 5 years, respectively. No retinopathy was observed. Cataracts were noted in seven patients at 1 to 10 years following irradiation (median, 2 years). Three patients developed lacrimal function disorder, however, no corneal ulceration occurred. Conclusions: Thirty Gy in 15 fractions appears to be a sufficient dose for local control with acceptable morbidity, especially for low-grade, as well as certain types of intermediate-grade lymphomas, such as diffuse small cleaved cell and lymphocytic lymphoma of intermediate differentiation. Systemic dissemination is minimal, provided local

  15. Radiation therapy for primary orbital lymphoma

    Energy Technology Data Exchange (ETDEWEB)

    Chao, Cliff K.S.; Hsiusan, Lin; Rao Devineni, V; Smith, Morton

    1995-02-15

    Purpose: The influence of tumor size, grade, thoroughness of staging workup, and radiation dose on disease control, radiation-related complications, and incidence of systemic progression of primary orbital lymphoma is analyzed. Methods and Materials: Twenty patients with Stage I primary orbital lymphoma were treated from August 1976 through August 1991 at Mallinckrodt Institute of Radiology. Staging workups included physical examination, chest x-ray, complete blood count (CBC), liver function test, and computerized tomography (CT) scan of the orbit, abdomen, and pelvis. Nineteen patients had bone marrow biopsy. The histological types based on the National Cancer Institute working formulation were 9 low-grade and 11 intermediate-grade, including five lymphocytic lymphomas of intermediate differentiation. The extension of disease and the volume of tumor were evaluated by CT scan of the orbit. The most commonly used radiation therapy technique was single anterior direct field with 4 MV or 6 MV photons. Lens was shielded or not treated in eight patients. Dose ranged from 20 to 43.2 Gy. Thirteen of 20 patients received 30 Gy. Minimum follow-up was 24 months (median, 4 years). Results: Local control was achieved in all 20 patients. One patient with lymphocytic lymphoma with intermediate differentiation developed disseminated disease. Actuarial disease-free survival (DFS) was 100% and 90% at 2 and 5 years, respectively. No retinopathy was observed. Cataracts were noted in seven patients at 1 to 10 years following irradiation (median, 2 years). Three patients developed lacrimal function disorder, however, no corneal ulceration occurred. Conclusions: Thirty Gy in 15 fractions appears to be a sufficient dose for local control with acceptable morbidity, especially for low-grade, as well as certain types of intermediate-grade lymphomas, such as diffuse small cleaved cell and lymphocytic lymphoma of intermediate differentiation. Systemic dissemination is minimal, provided local

  16. Cutaneous T-cell lymphoma in a patient infected with human immunodeficiency virus type 1. Use of radiation therapy

    International Nuclear Information System (INIS)

    Goldstein, J.; Becker, N.; DelRowe, J.; Davis, L.

    1990-01-01

    A patient with cutaneous T-cell lymphoma (CTCL) and acquired immune deficiency syndrome (AIDS) is presented. The patient had a localized lesion on his scalp. Evaluation for systemic lymphoma was negative. A biopsy specimen showed superficial and deep dermal infiltrates of pleomorphic lymphocytes. Immunohistochemistry was consistent with T-cell lymphoma. The patient was treated successfully with local irradiation. He remained free of further systemic and cutaneous recurrences of the lymphoma until he died 8 months after treatment of pneumonia. This case is the first to our knowledge to describe a localized CTCL in a patient infected with human immunodeficiency virus type 1 (HIV-1)

  17. CT findings in primary pulmonary lymphomas

    International Nuclear Information System (INIS)

    Cardinale, Luciano; Allasia, Marco; Cataldi, Aldo; Ferraris, Fabrizio; Fava, Cesare; Parvis, Guido

    2005-01-01

    Purpose. To describe the CT findings of pathologically confirmed primary pulmonary lymphomas. Materials and methods. The CT examinations of 11 patients with pathologically proven primary pulmonary lymphoma (9 BALT lymphomas and 2 non-BALT lymphomas) were retrospectively reviewed by three radiologists. Evaluated findings included morphology (consolidation, mass, nodule), number and distribution of lesions. Other CT findings such as air bronchogram, lymphadenopathy atelectasis and pleural effusion were also assessed. Results. Pulmonary lesions were depicted as air-space consolidation (pneumonia-like) in 5 patients (45%), tumour-like rounded opacity in 4 (36%), and nodules in 4 (36%). Multiple and bilateral long lesions were seen in 3 patients (27%). Air bronchogram was present in 7 patients (63%), lymphadenopathy in 3 (27%), atelectasis in 4 (36%) and pleural effusion in only 1 (9%). Conclusions. Our results agree with previous studies regarding lesion patterns and their relative frequency. A smaller number of nodules and of multiple lesions were found compared with some previous studies. The most frequent pattern was airspace consolidation [it

  18. Primary immunodeficiencies and B-cell lymphomas.

    Science.gov (United States)

    Martín-Mateos, María Anunciación; Piquer Gibert, Mónica

    In primary immunodeficiencies there is a failure in the anti-tumor defense. Common variable immunodeficiency (CVID) is one of the most common primary immunodeficiencies characterized by an alteration in the differentiation of B lymphocytes (BL). Epstein-Barr virus (EBV) is an ubiquitous virus that selectively infects the BL. In patients with immunodeficiency, uncontrolled proliferation of infected BL and the action of viral proteins promote the development of lymphomas. At the University Hospital Sant Joan de Deu, Barcelona, 28 patients were diagnosed with CVID from 2000 to 2013. This paper describes four patients who developed non-Hodgkin's lymphoma (NHL). The lymphoma was associated with EBV in two of the cases. Patients were<18 years old, diagnosed with lymphoma between 4 and 13 years old. Two patients were treated with rituximab as monotherapy and achieved complete remission. Two patients were treated with CHOP (cyclophosphamide, doxorubicin, vincristine and prednisolone) and radiotherapy or rituximab and achieved complete remission. Early detection of EBV infections and NHL in all patients diagnosed with CVID is recommended, regardless of age at diagnosis. Copyright © 2016 Hospital Infantil de México Federico Gómez. Publicado por Masson Doyma México S.A. All rights reserved.

  19. Primary thyroid lymphoma: a rare thyroid malignancy

    International Nuclear Information System (INIS)

    Mukhtar, R.; Khattak, R.; Mateen, A.

    2011-01-01

    Incidence of thyroidal masses is very high with predominance in females. Most of these masses are benign. Malignancy can be of thyroid or non-thyroid origin. Surgery is treatment of choice in the former but not in latter. Primary thyroid lymphoma (PTL) is a rare cause of thyroid malignancy of non-thyroid origin which is highly treatable without surgery but early diagnosis is important requisite to have better outcome. Fine needle aspiration cytology (FNAC) is an important tool in early diagnosis of PTL, which enables doctors to treat patient better without major surgery and its morbidity. Two cases of primary thyroid lymphoma are hereby presented. The patient diagnosed on FNAC had better outcome than the one who had major surgery. (author)

  20. Treatment options for Primary CNS Lymphoma.

    Science.gov (United States)

    Laghari, Altaf Ali; Ahmed, Syed Ijlal; Jabbar, Adnan; Shamim, Muhammad Shahzad

    2018-03-01

    Primary CNS lymphoma (PCNSL) is a rare and aggressive brain tumour that is uniformly fatal. The rarity of the disease and the poor response to treatment makes it difficult to reach a consensus with regards to treatment options. In this review, the authors have discussed different treatment modalities used in the management of PCNSL including chemotherapy, surgery and radiation, as well as the results of recent clinical trials on treatment options for PCNSL.

  1. Low-dose total skin electron beam therapy for cutaneous lymphoma : Minimal risk of acute toxicities.

    Science.gov (United States)

    Kroeger, Kai; Elsayad, Khaled; Moustakis, Christos; Haverkamp, Uwe; Eich, Hans Theodor

    2017-12-01

    Low-dose total skin electron beam therapy (TSEBT) is attracting increased interest for the effective palliative treatment of primary cutaneous T‑cell lymphoma (pCTCL). In this study, we compared toxicity profiles following various radiation doses. We reviewed the records of 60 patients who underwent TSEBT for pCTCL between 2000 and 2016 at the University Hospital of Munster. The treatment characteristics of the radiotherapy (RT) regimens and adverse events (AEs) were then analyzed and compared. In total, 67 courses of TSEBT were administered to 60 patients. Of these patients, 34 (51%) received a standard dose with a median surface dose of 30 Gy and 33 patients (49%) received a low dose with the median surface dose of 12 Gy (7 salvage low-dose TSEBT courses were administered to 5 patients). After a median follow-up of 15 months, the overall AE rate was 100%, including 38 patients (57%) with grade 2 and 7 (10%) with grade 3 AEs. Patients treated with low-dose TSEBT had significantly fewer grade 2 AEs than those with conventional dose regimens (33 vs. 79%, P dose regimen compared to those with the conventional dose regimens (6 vs. 15%, P = 0.78). Multiple/salvage low-dose TSEBT courses were not associated with an increased risk of acute AEs. Low-dose TSEBT regimens are associated with significantly fewer grade 2 acute toxicities compared with conventional doses of TSEBT. Repeated/Salvage low-dose TSEBT, however, appears to be tolerable and can even be applied safely in patients with cutaneous relapses.

  2. Mantle cell lymphoma of the larynx: Primary case report

    Directory of Open Access Journals (Sweden)

    Naciri Sarah

    2012-07-01

    Full Text Available Abstract Introduction Primary laryngeal lymphomas are exceedingly rare. Only about a hundred cases have been reported. They consist mainly of non-Hodgkin lymphoma, especially of diffuse large B-cell lymphoma and mucosa-associated lymphoid tissue. We report the first case of a primary laryngeal mantle cell lymphoma. Case presentation We report a case of a primary mantle cell lymphoma of the larynx in a 70-year-old North African non-smoker male. We present a detailed report of his clinical and paraclinical data as well as treatment options. Conclusions Mantle cell lymphoma is a very aggressive lymphoma subset associated with poor prognosis. Laryngeal mantle cell lymphoma is exceedingly rare. To the best of our knowledge, this is the first case to ever be reported.

  3. Apresentação cutânea inicial de linfomas na infância Initial cutaneous manifestation of lymphomas in children

    Directory of Open Access Journals (Sweden)

    Maria Christina Lopes Araujo de Oliveira

    2011-08-01

    Full Text Available Os linfomas cutâneos compreendem um grupo heterogêneo de desordens linfoproliferativas que envolvem a pele e são classificados como um subgrupo dos linfomas não Hodgkin. No período de 1981 a 2007, 100 casos de linfomas em crianças foram admitidos no Serviço de Hematologia, do Hospital das Clínicas da Universidade Federal de Minas Gerais, sendo que nove apresentaram manifestação cutânea inicial. Três pacientes foram classificados como linfoma cutâneo primário e seis como sistêmicos. Sete pacientes apresentaram linfoma de células T, um, linfoma linfoblástico B e um, imunofenótipo indefinido. Nenhum óbito ocorreu nos pacientes com linfoma cutâneo primárioCutaneous lymphomas comprise a heterogeneous group of lymphoproliferative disorders with skin involvement and are classified as a subgroup of non-Hodgkin lymphomas. From 1981 to 2007, 100 children with non-Hodgkin lymphomas were admitted to the Hematology Unit of the Federal University of Minas Gerais Teaching Hospital. In nine of these children, the skin was involved at the onset of the disease. Three patients were classified as having primary cutaneous lymphoma, while in six the disease was systemic with cutaneous involvement. In seven patients, the immunophenotype was T-cell, in one it was B-cell, and in the remaining case the immunophenotype was indefinable. No deaths occurred in any of the children with primary cutaneous lymphoma

  4. Stenotrophomonas maltophilia with histopathological features mimicking cutaneous gamma/delta T-cell lymphoma

    Directory of Open Access Journals (Sweden)

    Natalie Kash

    2015-01-01

    Full Text Available We report a case of cutaneous Stenotrophomonas maltophilia infection which presented with clinical and histopathological findings that mimicked a gamma/delta (γδ T-cell lymphoma. In this case, tissue culture of the biopsy specimen was key to determining the diagnosis and allowing appropriate treatment with oral trimethoprim–sulfamethoxazole and topical silvadene. A prompt complete resolution of lesions was observed following antibiotic treatment, with no recurrence of disease over the last 5 years, supporting an infectious rather than malignant etiology. In our patient, radiation therapy was indicated based on the misdiagnosis of γδ T-cell lymphoma, which was supported both clinically and histopathologically. However, tissue culture in this case avoided unnecessary radiation exposure and highlights the role of tissue culture in the evaluation of the biopsy of an undiagnosed cutaneous lesion.

  5. Curative radiotherapy for primary orbital lymphoma

    International Nuclear Information System (INIS)

    Bhatia, Sudershan; Paulino, Arnold C.; Buatti, John M.; Mayr, Nina A.; Wen, B.-C.

    2002-01-01

    Purpose: To review our institutional experience with primary orbital lymphoma and determine the prognostic factors for survival, local control, and distant metastases. In addition, we also analyzed the risk factors for complications in the radiotherapeutic management of this tumor. Methods and Materials: Between 1973 and 1998, 47 patients (29 women [62%] and 18 men [38%], median age 69 years, range 32-89) with Stage IAE orbital lymphoma were treated with curative intent at one department. Five had bilateral orbital involvement. The tumor was located in the eyelid and extraocular muscles in 23 (44%), conjunctiva in 17 (33%), and lacrimal apparatus in 12 (23%). The histologic features according to the World Heath Organization classification of lymphoid neoplasms was follicular lymphoma in 25, extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue type in 8, diffuse large B-cell lymphoma in 12, mantle cell lymphoma in 6, and peripheral T-cell lymphoma in 1. For the purposes of comparison with the existing literature on orbital lymphomas, the grading system according to the Working Formulation was also recorded. The histologic grade was low in 33 (63%), intermediate in 18 (35%), and high in 1 (2%). All patients were treated with primary radiotherapy alone. The median dose for low-grade tumors was 3000 cGy (range 2000-4020); the median dose for intermediate and high-grade tumors was 4000 cGy (range 3000-5100). A lens-sparing approach was used in 19 patients (37%). Late complications for the lens and cornea were scored according to the subjective, objective, management, and analytic (SOMA) scale of the Late Effects of Normal Tissue (LENT) scoring system. The median follow-up was 55 months (range 6-232). Results: The local control rate was 100% in the 52 orbits treated. The 5-year overall survival and relapse-free survival rate was 73.6% and 65.5%, respectively. Tumor grade and location did not predict for overall survival or relapse-free survival

  6. Alisertib in Combination With Vorinostat in Treating Patients With Relapsed or Recurrent Hodgkin Lymphoma, B-Cell Non-Hodgkin Lymphoma, or Peripheral T-Cell Lymphoma

    Science.gov (United States)

    2018-04-10

    Adult B Acute Lymphoblastic Leukemia; Adult T Acute Lymphoblastic Leukemia; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-Cell Lymphoma; Chronic Lymphocytic Leukemia; Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue; Hepatosplenic T-Cell Lymphoma; Intraocular Lymphoma; Lymphomatous Involvement of Non-Cutaneous Extranodal Site; Mature T-Cell and NK-Cell Non-Hodgkin Lymphoma; Nodal Marginal Zone Lymphoma; Primary Cutaneous B-Cell Non-Hodgkin Lymphoma; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult T-Cell Leukemia/Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides and Sezary Syndrome; Recurrent Non-Hodgkin Lymphoma; Recurrent Primary Cutaneous T-Cell Non-Hodgkin Lymphoma; Recurrent Small Lymphocytic Lymphoma; Refractory Chronic Lymphocytic Leukemia; Refractory Hairy Cell Leukemia; Small Intestinal Lymphoma; Splenic Marginal Zone Lymphoma; T-Cell Large Granular Lymphocyte Leukemia; Testicular Lymphoma; Waldenstrom Macroglobulinemia

  7. MULTICENTRIC T-CELL LYMPHOMA AND CUTANEOUS HEMANGIOSARCOMA IN A CAPTIVE CHEETAH (ACINONYX JUBATUS).

    Science.gov (United States)

    Lindemann, Dana M; Carpenter, James W; Nietfeld, Jerome C; Gonzalez, Estehela; Hallman, Mackenzie; Hause, Ben M

    2015-12-01

    A 13-yr-old intact male cheetah (Acinonyx jubatus) presented for evaluation after a 4-mo history of intermittent lethargy and increased expiratory effort. The clinical signs were initially noted after the diagnosis and death of its 13-yr-old male sibling with solitary hepatic T-cell lymphoma. Physical examination findings included thin body condition, harsh lung sounds, peripheral lymphadenopathy, and a cutaneous mass on the right medial tarsus and scrotum. Excisional biopsies diagnosed well-differentiated cutaneous hemangiosarcomas. Thoracic radiographs revealed a cranial mediastinal mass. Complete blood count and serum biochemical analyses showed a leukocytosis with persistent lymphocytosis, progressive azotemia, and markedly elevated alkaline phosphatase. Because of the cheetah's declining quality of life, euthanasia was elected. Postmortem examination, histopathology, and immunohistochemical staining revealed multicentric T-cell lymphoma. Feline leukemia virus (FeLV) enzyme-linked immunosorbent assay, FeLV polymerase chain reaction (whole blood), and viral metagenomic analysis were negative. This is the first case of cutaneous hemangiosarcoma and multicentric T-cell lymphoma reported in a FeLV-negative cheetah.

  8. Sonographic Findings of Primary Tracheal Lymphoma: Case Report

    International Nuclear Information System (INIS)

    Kim, Min Sung; Ahn, Kook Jin; Kim, Jee Young; Kim, Hyo Lim; Park, Jeong Mi

    2010-01-01

    Primary lymphoma of the trachea is extremely rare and clinical presentation is nonspecific. CT findings are focal tracheal narrowing caused by a solitary mass or polypoid thickening of the tracheobronchial wall caused by diffuse infiltration of the submucosa. However, US finding of primary lymphoma of the trachea has been not reported. We experienced a case of primary lymphoma of the trachea presenting as a homogenous hypoechoic mass, and discuss ultrasonographic and CT findings of the case

  9. Vorinostat in Treating Patients With Metastatic or Unresectable Solid Tumors or Lymphoma and Liver Dysfunction

    Science.gov (United States)

    2014-02-21

    Adult Grade III Lymphomatoid Granulomatosis; Adult Nasal Type Extranodal NK/T-cell Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Primary Central Nervous System Hodgkin Lymphoma; Primary Central Nervous System Non-Hodgkin Lymphoma; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides/Sezary Syndrome; Recurrent Small Lymphocytic Lymphoma; Small Intestine Lymphoma; Splenic Marginal Zone Lymphoma; Stage III Adult Burkitt Lymphoma; Stage III Adult Diffuse Large Cell Lymphoma; Stage III Adult Diffuse Mixed Cell Lymphoma; Stage III Adult Diffuse Small Cleaved Cell Lymphoma; Stage III Adult Hodgkin Lymphoma; Stage III Adult Immunoblastic Large Cell Lymphoma; Stage III Adult Lymphoblastic Lymphoma; Stage III Adult T-cell Leukemia/Lymphoma; Stage III Cutaneous T-cell Non-Hodgkin Lymphoma; Stage III Grade 1 Follicular Lymphoma; Stage III Grade 2 Follicular Lymphoma; Stage III Grade 3 Follicular Lymphoma; Stage III Mantle Cell Lymphoma; Stage III Marginal Zone Lymphoma; Stage III Mycosis Fungoides/Sezary Syndrome; Stage III Small Lymphocytic Lymphoma; Stage IV Adult Burkitt Lymphoma; Stage IV Adult Diffuse Large Cell Lymphoma; Stage IV Adult Diffuse Mixed Cell Lymphoma; Stage

  10. Pomalidomide and Dexamethasone in Treating Patients With Relapsed or Refractory Primary Central Nervous System Lymphoma or Newly Diagnosed or Relapsed or Refractory Intraocular Lymphoma

    Science.gov (United States)

    2017-08-28

    B-Cell Lymphoma, Unclassifiable, With Features Intermediate Between Diffuse Large B-Cell Lymphoma and Burkitt Lymphoma; Central Nervous System Lymphoma; Intraocular Lymphoma; Primary Diffuse Large B-Cell Lymphoma of the Central Nervous System; Recurrent Adult Diffuse Large Cell Lymphoma; Retinal Lymphoma

  11. Vorinostat approved in Japan for treatment of cutaneous T-cell lymphomas: status and prospects

    Directory of Open Access Journals (Sweden)

    Sato A

    2012-04-01

    Full Text Available Akinori SatoDepartment of Urology, National Defense Medical College, 3-2 Namiki, Tokorozawa, Saitama 359-8513, JapanAbstract: Histone acetylation and deacetylation play important roles in the regulation of gene transcription and in the modulation of chromatin structure. The levels of histone acetylation are determined by the activities of histone acetyltransferases and histone deacetylases (HDACs. HDACs are associated with a number of oncogenes and tumor suppressor genes and can be aberrantly expressed and/or inappropriately activated in cancer cells. HDAC inhibitors have therefore recently emerged as a novel treatment modality against malignancies. They regulate gene expression by enhancing the acetylation of not only histones but also nonhistone proteins, including transcription factors, transcription regulators, signal transduction mediators, and DNA repair enzymes, and they inhibit cancer growth. Vorinostat (suberoylanilide hydroxamic acid is one of the most potent HDAC inhibitors, and was approved in Japan in 2011 for the treatment of cutaneous T-cell lymphoma. Numerous clinical trials have shown it to be effective against cutaneous T-cell lymphoma but less so against other types of cancer. Because vorinostat can overcome resistance to or enhance the efficacy of other anticancer agents, such as 5-fluorouracil, carboplatin, paclitaxel, bortezomib, and tamoxifen, combination therapies using vorinostat and these agents have been investigated. This review introduces the background and mechanism of action of vorinostat and describes the results of clinical trials using vorinostat, both as a single agent and in combination with other anticancer agents, against cutaneous T-cell lymphoma and other malignancies.Keywords: vorinostat, T-cell lymphoma, cancer, novel treatment

  12. Primary colorectal lymphoma: spectrum of imaging findings with pathologic correlation

    International Nuclear Information System (INIS)

    Lee, Hyun Ju; Han, Joon Koo; Kim, Tae Kyoung; Kim, Young Hoon; Kim, Ah Young; Kim, Kyoung Won; Choi, Ja Young; Choi, Byung Ihn

    2002-01-01

    Primary colorectal lymphoma is a very uncommon disease; therefore, it has received little attention in the radiology literature. Moreover, imaging features of newly described pathologic subtypes have not been reported such as low-grade B-cell lymphoma arising from mucosa-associated lymphoid tissue and peripheral T-cell lymphoma that involves colorectal area. We retrospectively reviewed double-contrast barium enema and CT scans in the patients with primary colorectal lymphoma. In this article the radiologic appearances of primary colorectal lymphoma are categorized into focal lesion and diffuse lesion. Focal lesion includes polypoid mass, circumferential infiltration with smooth mucosal surface, circumferential infiltration with extensive ulceration, cavitary mass, mucosal nodularity, and mucosal fold thickening. Diffuse lesion includes diffuse ulcerative lesion and diffuse nodular lesion. Peripheral T-cell lymphomas that involve the colon manifested as either a diffuse or focal segmental lesion and showed extensive mucosal ulceration. These findings are similar to those of Crohn's disease or tuberculous colitis and are different from those of previously reported colorectal lymphoma. Low-grade B-cell lymphoma arising from mucosa-associated lymphoid tissue manifest as multiple mucosal nodularity. The imaging features of primary colorectal lymphoma are quite variable and overlap with other colonic pathology; however, it is important for radiologists to know the imaging features of primary colorectal lymphoma with their pathologic correlation. (orig.)

  13. Primary effusion lymphomas in AIDS: CT findings in two cases

    International Nuclear Information System (INIS)

    Ferrozzi, F.; Tognini, G.; Mulonzia, N.W.; Pavone, P.; Bova, D.

    2001-01-01

    Primary effusion lymphomas represent an unusual subset of AIDS-related non-Hodgkin's lymphomas. They are associated with herpes virus 8 and Epstein-Barr virus and characterized by predominant involvement of the serous body cavities (pleura, pericardium, peritoneum) as lymphomatous effusion without any identifiable tumour mass. We report herein CT findings in two patients with primary effusion lymphoma emphasizing the possible neoplastic nature of a pleural effusion in a patient with AIDS. (orig.) (orig.)

  14. Primary effusion lymphomas in AIDS: CT findings in two cases

    Energy Technology Data Exchange (ETDEWEB)

    Ferrozzi, F.; Tognini, G.; Mulonzia, N.W.; Pavone, P. [Ist. di Scienze Radiologiche, Univ. di Parma (Italy); Bova, D.

    2001-04-01

    Primary effusion lymphomas represent an unusual subset of AIDS-related non-Hodgkin's lymphomas. They are associated with herpes virus 8 and Epstein-Barr virus and characterized by predominant involvement of the serous body cavities (pleura, pericardium, peritoneum) as lymphomatous effusion without any identifiable tumour mass. We report herein CT findings in two patients with primary effusion lymphoma emphasizing the possible neoplastic nature of a pleural effusion in a patient with AIDS. (orig.) (orig.)

  15. CUTANEOUS EPITHELIOTROPIC T-CELL LYMPHOMA WITH METASTASES IN A VIRGINIA OPOSSUM (DIDELPHIS VIRGINIANA).

    Science.gov (United States)

    Higbie, Christine T; Carpenter, James W; Choudhary, Shambhunath; DeBey, Brad; Bagladi-Swanson, Mary; Eshar, David

    2015-06-01

    A 2-yr-old, captive, intact female Virginia opossum ( Didelphis virginiana ) with a 7-mo history of ulcerative dermatitis and weight loss was euthanatized for progressive worsening of clinical signs. Initially the opossum was treated with several courses of antibiotics, both topically and systemically; systemic nonsteroidal anti-inflammatory medication; and, later, systemic glucocorticoids, with no improvement in clinical signs. Histopathologic samples of skin lesions taken 3 mo into the course of disease revealed no evidence of neoplasia; however, cytologic samples of a skin lesion taken 5 mo into the course of disease revealed mature lymphocytes, and were suggestive of cutaneous lymphoma. Postmortem histopathology revealed neoplastic cells consistent with lymphoma; these were found in the haired skin of the forearm, axilla, hind limb, face, and lateral body wall, as well as the liver, kidney, axillary lymph node, heart, and spleen. Multifocal neutrophilic and eosinophilic ulcerative and necrotizing dermatitis and folliculitis of the haired skin were also present. To the authors' knowledge, this is the first documented case of cutaneous lymphoma in a Virginia opossum and the first documented case with visceral metastases in a marsupial.

  16. Imaging of primary central nervous system lymphoma

    Energy Technology Data Exchange (ETDEWEB)

    Tang, Y.Z., E-mail: yenzhitang@doctors.net.uk [Royal Free Hospital, London (United Kingdom); Booth, T.C.; Bhogal, P.; Malhotra, A.; Wilhelm, T. [Royal Free Hospital, London (United Kingdom)

    2011-08-15

    Primary central nervous system lymphoma (PCNSL) comprises 5% of all primary brain tumours. PCNSL demonstrates a variety of well-documented imaging findings, which can vary depending on immune status and histological type. Imaging features of PCNSL may overlap with other tumours and infection making definitive diagnosis challenging. In addition, several rare variants of PCNSL have been described, each with their own imaging characteristics. Advanced imaging techniques including 2-[{sup 18}F]-fluoro-2-deoxy-D-glucose ({sup 18}FDG) and {sup 11}C positron-emission tomography (PET), {sup 201}Tl single-photon emission computed tomography (SPECT), {sup 1}H-magnetic resonance spectroscopy (MRS), and MR perfusion, have been used to aid differentiation of PCNSL from other tumours. Ultimately, no imaging method can definitively diagnose PCNSL, and histology is required.

  17. Cerebellar T-cell lymphoma: an unusual primary intracranial neoplasm

    International Nuclear Information System (INIS)

    Knorr, J.R.; Ragland, R.L.; Stone, B.B.; Woda, B.A.; Gelber, N.D.

    1992-01-01

    Primary T-cell lymphoma within the central nervous system is extremely rare. Imaging characteristics appear indistinguishable from the more common B-cell lymphoma. A case of such a primary tumor is discussed and the MRI and CT findings presented. (orig.)

  18. Radiotherapeutic management of primary thyroid lymphoma

    International Nuclear Information System (INIS)

    Blair, T.J.; Evans, R.G.; Buskirk, S.J.; Banks, P.M.; Earle, J.D.

    1985-01-01

    The purpose of this study was to evaluate the radiotherapeutic management of 38 patients, with malignant lymphoma of the thyroid, seen at the Mayo Clinic between 1965 and 1979. There were 8 males and 30 females with ages ranging from 34 to 90 years. A tissue diagnosis was made in all patients and tissue was available for reclassification under the Working Formulation in 31 of the 38 patients. Twenty-six patients had intermediate grade histology, four low grade and one indeterminate. All patients were treated with approximately 4000 rad megavoltage irradiation (range 2400-6000 rad) to the neck only (10 patients) or neck and mediastinum (28 patients). Twenty patients received subdiaphragmatic radiotherapy and four patients received adjuvant chemotherapy. Patients receiving radiation treatment to the neck and mediastinum and those with no gross residual disease at the initation of radiotherapy were less likely to develop a recurrence. Patients receiving a planned break during the course of therapy did not have reduced overall disease-free survival. Only 1 of 4 patients (25%) receiving adjuvant chemotherapy survived one year. Side effects of radiotherapy were minimal. The authors believe the radiotherapeutic management of clinical Stage IE and IIE primary thyroid lymphoma should include treatment of the neck, axillae and mediastinum to a dose of approximately 4000 rad using a continuous course technique. Additionally, gross total removal of the disease surgically may be beneficial

  19. Low-dose (10-Gy) total skin electron beam therapy for cutaneous T-cell lymphoma

    DEFF Research Database (Denmark)

    Kamstrup, Maria R; Gniadecki, Robert; Iversen, Lars

    2015-01-01

    a total dose of 10 Gy in 10 fractions. Data from 10 of these patients were published previously but were included in the current pooled data analysis. Outcome measures were response rate, duration of response, and toxicity. RESULTS: The overall response rate was 95% with a complete cutaneous response......PURPOSE: Cutaneous T-cell lymphomas (CTCLs) are dominated by mycosis fungoides (MF) and Sézary syndrome (SS), and durable disease control is a therapeutic challenge. Standard total skin electron beam therapy (TSEBT) is an effective skin-directed therapy, but the possibility of retreatments...... or a very good partial response rate (response was 174 days (5.8 months; range: 60-675 days). TSEBT-related acute adverse events (grade 1 or 2) were observed in 60% of patients. CONCLUSIONS...

  20. A CASE OF PRIMARY UTERINE LYMPHOMA

    Directory of Open Access Journals (Sweden)

    Parikshit Sanyal

    2013-06-01

    Full Text Available A post menopausal lady presented with lump lower abdomen and bleeding per vaginum. USG revealed diffuse enlargement of the uterus. On hysterectomy, a grossly enlarged uterus with cystic left ovary were found. Hysterectomy was done and uterus with bilateral adnexa submitted for histopathological examination. Microscopic examination of the body of uterus revealed sheets of small lymphoid cells were found to replace the endo- and myo-metrium. These cells have small nuclei with clumped chromatin, and no prominence of nucleoli. They are not forming lymphoid follicles or germinal centers. Similar lymphoid cells were also found in the left ovary admixed with ovarian stroma. On IHC these cells were found to be CD45, CD20, CD23 positive, and negative for CD3 , CK and SMA. The case is diagnosed as a primary small lymphocytic lymphoma of uterus with left ovarian spread.

  1. Anti-ICOS Monoclonal Antibody MEDI-570 in Treating Patients With Relapsed or Refractory Peripheral T-cell Lymphoma Follicular Variant or Angioimmunoblastic T-cell Lymphoma

    Science.gov (United States)

    2018-05-09

    Follicular T-Cell Lymphoma; Grade 1 Follicular Lymphoma; Grade 2 Follicular Lymphoma; Grade 3a Follicular Lymphoma; Recurrent Angioimmunoblastic T-Cell Lymphoma; Recurrent Follicular Lymphoma; Recurrent Mature T- and NK-Cell Non-Hodgkin Lymphoma; Recurrent Mycosis Fungoides; Recurrent Primary Cutaneous T-Cell Non-Hodgkin Lymphoma; Refractory Angioimmunoblastic T-Cell Lymphoma; Refractory Follicular Lymphoma; Refractory Mature T-Cell and NK-Cell Non-Hodgkin Lymphoma; Stage IB Mycosis Fungoides AJCC v7; Stage II Mycosis Fungoides AJCC v7; Stage III Cutaneous T-Cell Non-Hodgkin Lymphoma; Stage III Mycosis Fungoides AJCC v7; Stage IV Cutaneous T-Cell Non-Hodgkin Lymphoma; Stage IV Mycosis Fungoides AJCC v7

  2. Mechanisms of lymphocytotoxicity induced by extracorporeal photochemotherapy for cutaneous T cell lymphoma

    International Nuclear Information System (INIS)

    Marks, D.I.; Rockman, S.P.; Oziemski, M.A.; Fox, R.M.

    1990-01-01

    Extracorporeal photochemotherapy is an effective treatment for cutaneous T cell lymphoma but its mode of action is uncertain. The reduction in viability of patients' photoirradiated buffy coat lymphocytes was correlated with a 35% increase in DNA single-strand breaks and marked decreases in cellular ATP and NAD levels (to 58 and 34% of control, respectively) immediately after photoirradiation. Complementary in vitro studies were conducted with normal human peripheral blood lymphocytes using a Therakos ultraviolet A (UVA) light box. UVA light was cytotoxic on its own but was potentiated by 8-methoxysporalen. 3-aminobenzamide, a poly (ADP-ribose) synthetase inhibitor, mitigated the cytotoxic effect of ultraviolet A light in the presence of 8-methoxypsoralen in lymphocytes and reduced the amount of nucleotide depletion they caused. 10 J/cm2 of UVA light in the presence of 300 ng/ml 8-methoxypsoralen increased the poly (ADP-ribose) synthetase activity of peripheral blood lymphocytes. Exposing lymphocytes to deoxycoformycin and deoxyadenosine was found to induce biochemical and physical effects similar to those of photochemotherapy. In summary, we have shown that the lymphocytotoxic effect of extracorporeal photochemotherapy for cutaneous T cell lymphoma is apparently mediated by DNA damage, subsequent poly (ADP-ribosyl)ation and adenine nucleotide depletion. It is not known how the DNA damage and resultant biochemical effects relate to the possible immunological mechanism of extracorporeal photochemotherapy; however, it is possible that its effects can be mimicked by other DNA-damaging agents

  3. Neurolymphomatosis: An International Primary CNS Lymphoma Collaborative Group report

    NARCIS (Netherlands)

    S. Grisariu (Sigal); B. Avni (Batia); T.T. Batchelor (Tracy); M.J. van den Bent (Martin); F. Bokstein (Felix); D. Schiff (David); O. Kuittinen (Outi); M.C. Chamberlain (Marc C.); P. Roth (Patrick); A. Nemets (Anatoly); E. Shalom (Edna); D. Ben-Yehuda (Dina); T. Siegal (Tali)

    2010-01-01

    textabstractNeurolymphomatosis (NL) is a rare clinical entity. The International Primary CNS Lymphoma Collaborative Group retrospectively analyzed 50 patients assembled from 12 centers in 5 countries over a 16-year period. NL was related to non-Hodgkin lymphoma in 90% and to acute leukemia in 10%.

  4. Primary Lymphoma of the Thyroid: Diagnostic and Therapeutic Considerations

    Directory of Open Access Journals (Sweden)

    Basro Sarinah

    2010-01-01

    Conclusion: The diagnosis of primary thyroid lymphoma should be considered when dealing with rapidly growing goitres. The role of FNAC in diagnosing thyroid lymphoma is limited but it is still useful in the initial work-up. Nevertheless, surgical intervention is often required to establish the diagnosis and relieve critical airway compression. A combination of chemotherapy and irradiation is the mainstay of management.

  5. Unusual presentation of rare primary lymphoma of bone ...

    African Journals Online (AJOL)

    Primary bone lymphoma is a distinct disease. It represents only 3% of all malignant bone tumours and less than 1% of non-Hodgkin's lymphoma. It is essential to differentiate it from other tumours because of its good prognosis. We report a case of 45 years old male who presented one year ago with a painful left arm.

  6. Spontaneous interleukin-5 production in cutaneous T-cell lymphoma lines is mediated by constitutively activated Stat3

    DEFF Research Database (Denmark)

    Nielsen, Mette; Nissen, Mogens H; Gerwien, Jens

    2002-01-01

    Mycosis fungoides is a low-grade cutaneous T-cell lymphoma (CTCL) of unknown etiology. In advanced stages of CTCL, a shift in cytokine profile from T(H)1 to T(H)2 is observed, which coincides with eosinophilia, high levels of immunoglobulin E, and increased susceptibility to bacterial infections....

  7. Loss of SHP-1 tyrosine phosphatase expression correlates with the advanced stages of cutaneous T-cell lymphoma

    DEFF Research Database (Denmark)

    Witkiewicz, Agnieszka; Raghunath, Puthiyaveettil; Wasik, Agnieszka

    2007-01-01

    Cutaneous T-cell lymphoma (CTCL) comprises distinct and often progressive stages of skin involvement by patches, plaques, and tumors. We have previously demonstrated that CTCL-derived malignant T-cell lines display loss of a tumor suppressor SHP-1 tyrosine phosphatase because of epigenetic...

  8. Malignant cutaneous T-cell lymphoma cells express IL-17 utilizing the Jak3/Stat3 signaling pathway

    DEFF Research Database (Denmark)

    Krejsgaard, Thorbjørn Frej; Ralfkiær, Ulrik; Clasen-Linde, Erik

    2011-01-01

    IL-17 is a proinflammatory cytokine that is crucial for the host's protection against a range of extracellular pathogens. However, inappropriately regulated expression of IL-17 is associated with the development of inflammatory diseases and cancer. In cutaneous T-cell lymphoma (CTCL), malignant T...

  9. Disseminated primary cutaneous histoplasmosis successfully treated with itraconazole

    Directory of Open Access Journals (Sweden)

    Singhi M

    2003-11-01

    Full Text Available A 60-year-old immunocompetent lady with disseminated primary cutaneous histoplasmosis is reported. Histology showed a granulomatous skin infiltrate with numerous intracellular PAS positive rounded yeast cells within macrophages. Culture on Sabouraud′s dextrose agar yielded a typical cottony white colony characteristic of Histoplasma capsulatum. Treatment with itraconazole showed an excellent response.

  10. Primary Gallbladder Small Lymphocytic Lymphoma as a Rare Postcholecystectomy Finding

    Directory of Open Access Journals (Sweden)

    Kyriakos Psarras

    2014-01-01

    Full Text Available Introduction. Primary lymphoma of the gallbladder is an extremely rare entity with approximately 50 cases reported so far. In many of these cases the presenting symptoms were mimicking symptomatic gallstone disease and the diagnosis was made postoperatively, especially when the preoperative imaging results were far from suspicious for malignant disease. Patients and Methods. We report a case of primary lymphoma of the gallbladder in an 85-year-old man with gallstone disease, who was admitted for elective cholecystectomy 2 months after an episode of acute cholecystitis and pancreatitis. Histological evaluation of the specimen revealed a small lymphocytic lymphoma of the gallbladder. This type of primary gallbladder lymphoma has not been previously reported. Discussion. The most common primary lymphomas of the gallbladder are MALT lymphomas and diffuse large B-cell lymphomas, although a variety of other histological types have been reported. The association of these lesions with chronic inflammation is the most convincing theory for their pathogenesis. For lesions confined to the gallbladder, cholecystectomy is considered to be sufficient, while supplementary chemotherapy significantly improves prognosis in more advanced disease.

  11. Notch signalling in primary cutaneous CD30+ lymphoproliferative disorders: a new therapeutic approach?

    DEFF Research Database (Denmark)

    Kamstrup, M R; Biskup, E; Gniadecki, R

    2010-01-01

    The oncogenic potential of deregulated Notch signalling has been described in several haematopoietic malignancies. We have previously reported an increased expression of Notch1 in primary cutaneous CD30+ lymphoproliferative disorders, lymphomatoid papulosis and primary cutaneous anaplastic large...

  12. Primary bone lymphoma with multiple vertebral involvement

    Directory of Open Access Journals (Sweden)

    Showkat Hussain Dar

    2013-01-01

    Full Text Available A 20-year-old student presented with 2 months history of fever and night sweats, 15 days history of low backache, progressive weakness of both limbs of 7 days duration, and urinary retention for last 24 h. Examination revealed a sensory level at D 10 dermatome and grade two power in both the lower limbs with absent reflexes. Examination of spine revealed a knuckle at T8 level, which was tender on palpation. MRI spine showed erosion of D11-12 and L1 in vertebral bodies with destruction of left pedicles, transverse processes and lamina, and a prominent psoas abscess. Post gadolinium study revealed ring-enhancing lesions in the D11-12 and L1 vertebrae as well as the dural sac. Fine needle aspiration cytology (FNAC and bone biopsy demonstrated a non-Hodgkin′s lymphoma (NHL, large cell high-grade of the spine (primary, which as per age is the youngest case of NHL ever reported in literature with multiple vertebral involvement.

  13. The management of primary lymphoma of bone

    International Nuclear Information System (INIS)

    Mendenhall, N.P.; Jones, J.J.; Kramer, B.S.; Carter, R.L.; Enneking, W.F.; Marcus, R.B. Jr.; Million, R.R.; Hudson, T.M.

    1987-01-01

    From October 1962 through to April 1982, 21 patients with the diagnosis of primary lymphoma of bone (18 monostotic, stages I E and II E ; 3 polyostotic) were treated with curative intent. A combination of chemotherapy and radiation therapy was used in 11 patients, local treatment alone in 9 patients, and chemotherapy alone in one patient. Overall 5-year survival for the patients treated with curative intent was 56%. Standard work-up has changed over the 20-year study period. Five-year survival for the subset of eight stage I and II patients with full pretherapy staging was 83%. Prognosis was significantly correlated with extent of pretherapy staging. Treatment parameters that also seemed to predict outcome were the aggressiveness of chemotherapy and the use of irradiation or surgery for local-regional disease; the only local failure occurred in the patient who received chemotherapy alone. Complications of radiation therapy alone and in combination with chemotherapy are discussed and correlated with irradiation dose. Radiation therapy techniques are described, and a management approach is recommended. (Auth.)

  14. Atypical Primary Cutaneous Rosai Dorfman Disease: A Case Report.

    Science.gov (United States)

    Kinio, Anna E; Sawchuk, Michael A; Pratt, Melanie

    Rosai Dorfman disease (RDD) is a rare disorder that typically presents with bilateral cervical lymphadenopathy and follows a benign course. We present a case of late-onset atypical primary cutaneous RDD that is resistant to treatment modalities described in the literature. Case report. An 84-year-old woman presented with a 7-year history of cutaneous lesions histologically consistent with RDD. She later failed initial treatments of acitretin and thalidomide. Physicians must be aware of unusual presentations of RDD. Also, further treatment options must be explored for patients resistant to classical management of RDD.

  15. Low-dose total skin electron beam therapy for cutaneous lymphoma. Minimal risk of acute toxicities

    Energy Technology Data Exchange (ETDEWEB)

    Kroeger, Kai; Elsayad, Khaled; Moustakis, Christos; Haverkamp, Uwe; Eich, Hans Theodor [University Hospital of Muenster, Department of Radiation Oncology, Muenster (Germany)

    2017-12-15

    Low-dose total skin electron beam therapy (TSEBT) is attracting increased interest for the effective palliative treatment of primary cutaneous T-cell lymphoma (pCTCL). In this study, we compared toxicity profiles following various radiation doses. We reviewed the records of 60 patients who underwent TSEBT for pCTCL between 2000 and 2016 at the University Hospital of Munster. The treatment characteristics of the radiotherapy (RT) regimens and adverse events (AEs) were then analyzed and compared. In total, 67 courses of TSEBT were administered to 60 patients. Of these patients, 34 (51%) received a standard dose with a median surface dose of 30 Gy and 33 patients (49%) received a low dose with the median surface dose of 12 Gy (7 salvage low-dose TSEBT courses were administered to 5 patients). After a median follow-up of 15 months, the overall AE rate was 100%, including 38 patients (57%) with grade 2 and 7 (10%) with grade 3 AEs. Patients treated with low-dose TSEBT had significantly fewer grade 2 AEs than those with conventional dose regimens (33 vs. 79%, P < 0.001). A lower grade 3 AE rate was also observed in patients who had received the low-dose regimen compared to those with the conventional dose regimens (6 vs. 15%, P = 0.78). Multiple/salvage low-dose TSEBT courses were not associated with an increased risk of acute AEs. Low-dose TSEBT regimens are associated with significantly fewer grade 2 acute toxicities compared with conventional doses of TSEBT. Repeated/Salvage low-dose TSEBT, however, appears to be tolerable and can even be applied safely in patients with cutaneous relapses. (orig.) [German] Eine niedrigdosierte Ganzhautelektronenbestrahlung (TSEBT) wird vermehrt zur effektiven palliativen Behandlung von Patienten mit primaer kutanen T-Zell-Lymphomen (pCTCL) eingesetzt. In dieser Studie vergleichen wir die Toxizitaetsprofile verschiedener Dosiskonzepte. Untersucht wurden 60 zwischen 2000 und 2016 am Universitaetsklinikum Muenster mittels TSEBT

  16. Importância da cintilografia com gálio-67 no linfoma cutâneo primário de células B: relato de dois casos Importance of gallium-67 scintigraphy in primary cutaneous B-cell lymphoma: report of two cases

    Directory of Open Access Journals (Sweden)

    Cyomara Sanches Attab

    2010-06-01

    Full Text Available Os autores relatam dois casos de linfoma cutâneo de células B, nos quais o correto estadiamento, tratamento e seguimento foram possíveis graças à combinação de exames convencionais e a cintilografia com gálio-67.The authors describe two cases of cutaneous B-cell lymphoma where correct staging, treatment and follow-up could be achieved through a combination of conventional imaging studies and gallium-67 scintigraphy.

  17. Importância da cintilografia com gálio-67 no linfoma cutâneo primário de células B: relato de dois casos Importance of gallium-67 scintigraphy in primary cutaneous B-cell lymphoma: report of two cases

    OpenAIRE

    Cyomara Sanches Attab; Sônia Marta Moriguchi; Eduardo José Alencar Paton; Mário Henrique Leite de Alencar; Euclides Timóteo da Rocha

    2010-01-01

    Os autores relatam dois casos de linfoma cutâneo de células B, nos quais o correto estadiamento, tratamento e seguimento foram possíveis graças à combinação de exames convencionais e a cintilografia com gálio-67.The authors describe two cases of cutaneous B-cell lymphoma where correct staging, treatment and follow-up could be achieved through a combination of conventional imaging studies and gallium-67 scintigraphy.

  18. Primary conjunctival follicular lymphoma mimicking chronic conjunctivitis.

    Science.gov (United States)

    Labrador Velandia, S; García Lagarto, E; Saornil, M A; García Álvarez, C; Cuello, R; Diezhandino, P

    2016-02-01

    The case is presented of a 43 year-old male patient with chronic follicular conjunctivitis, negative bacterial serology, and refractory to local treatment. The incisional biopsy performed showed to be consistent with reactive lymphoid hyperplasia. A year later, a new incisional biopsy showed follicular lymphoma, with no systemic involvement, and he was treated with local radiotherapy. When a chronic follicular conjunctivitis is refractory to treatment, it is essential to perform an incisional biopsy to establish the histopathological diagnosis that can range from chronic inflammation, reactive lymphoid hyperplasia to lymphoma. Follicular lymphoma is rare among conjunctival lymphomas, and the staging is indispensable for the correct therapeutic approach. Copyright © 2014 Sociedad Española de Oftalmología. Published by Elsevier España, S.L.U. All rights reserved.

  19. Primary parotid gland lymphoma: a case report

    Directory of Open Access Journals (Sweden)

    Paraskevas Katsaronis

    2011-08-01

    Full Text Available Abstract Introduction Mucosa associated lymphoid tissue lymphomas are the most common lymphomas of the salivary glands. The benign lymphoepithelial lesion is also a lymphoproliferative disease that develops in the parotid gland. In the present case report, we describe one case of benign lymphoepithelial lesion with a subsequent low transformation to grade mucosa associated lymphoid tissue lymphoma appearing as a cystic mass in the parotid gland. Case presentation A 78-year-old Caucasian female smoker was referred to our clinic with a non-tender left facial swelling that had been present for approximately three years. The patient underwent resection of the left parotid gland with preservation of the left facial nerve through a preauricular incision. The pathology report was consistent with a low-grade marginal-zone B-cell non-Hodgkin lymphoma (mucosa associated lymphoid tissue lymphoma following benign lymphoepithelial lesion of the gland. Conclusions Salivary gland mucosa associated lymphoid tissue lymphoma should be considered in the differential diagnosis of cystic or bilateral salivary gland lesions. Parotidectomy is recommended in order to treat the tumor and to ensure histological diagnosis for further follow-up planning. Radiotherapy and chemotherapy should be considered in association with surgery in disseminated forms or after removal.

  20. STAT3-mediated constitutive expression of SOCS-3 in cutaneous T-cell lymphoma

    DEFF Research Database (Denmark)

    Brender, C; Nielsen, M; Kaltoft, K

    2001-01-01

    ) obtained from affected skin from a patient with mycosis fungoides (MF) and from peripheral blood from a patient with Sezary syndrome (SS). In contrast, constitutive SOCS-3 expression is not found in the leukemic Jurkat T-cell line, the MOLT-4 acute lymphoblastic leukemia cell line, and the monocytic......, it has been hypothesized that an aberrant SOCS expression plays a role in neoplastic transformation. This study reports on a constitutive SOCS-3 expression in cutaneous T-cell lymphoma (CTCL) cell lines. SOCS-3 protein is constitutively expressed in tumor cell lines (but not in nonmalignant T cells...... leukemic cell line U937. Expression of SOCS-3 coincides with a constitutive activation of STAT3 in CTCL tumor cells, and stable transfection of CTCL tumor cells with a dominant negative STAT3 strongly inhibits SOCS-3 expression, whereas transfection with wild-type STAT3 does not. Moreover, the reduced SOCS...

  1. Optical perception for detection of cutaneous T-cell lymphoma by multi-spectral imaging

    International Nuclear Information System (INIS)

    Hsiao, Yu-Ping; Wang, Hsiang-Chen; Chen, Shih-Hua; Tsai, Chung-Hung; Yang, Jen-Hung

    2014-01-01

    In this study, the spectrum of each picture element of the patient’s skin image was obtained by multi-spectral imaging technology. Spectra of normal or pathological skin were collected from 15 patients. Principal component analysis and principal component scores of skin spectra were employed to distinguish the spectral characteristics with different diseases. Finally, skin regions with suspected cutaneous T-cell lymphoma (CTCL) lesions were successfully predicted by evaluation and classification of the spectra of pathological skin. The sensitivity and specificity of this technique were 89.65% and 95.18% after the analysis of about 109 patients. The probability of atopic dermatitis and psoriasis patients misinterpreted as CTCL were 5.56% and 4.54%, respectively. (paper)

  2. Malignant inflammation in cutaneous T-cell lymphoma-a hostile takeover

    DEFF Research Database (Denmark)

    Krejsgaard, Thorbjørn; Lindahl, Lise M; Mongan, Nigel P

    2017-01-01

    Cutaneous T-cell lymphomas (CTCL) are characterized by the presence of chronically inflamed skin lesions containing malignant T cells. Early disease presents as limited skin patches or plaques and exhibits an indolent behavior. For many patients, the disease never progresses beyond this stage......, but in approximately one third of patients, the disease becomes progressive, and the skin lesions start to expand and evolve. Eventually, overt tumors develop and the malignant T cells may disseminate to the blood, lymph nodes, bone marrow, and visceral organs, often with a fatal outcome. The transition from early...... of the inflammatory environment, suppressing cellular immunity and anti-tumor responses while promoting a chronic inflammatory milieu that fuels their own expansion. Here, we review the inflammatory changes associated with disease progression in CTCL and point to their wider relevance in other cancer contexts. We...

  3. The role of cytokine signaling in the pathogenesis of cutaneous T-cell lymphoma

    DEFF Research Database (Denmark)

    abraham, Robert; Zhang, Qiang; Ødum, Niels

    2011-01-01

    Cutaneous T-cell lymphoma (CTCL) displays immunosuppressive properties and phenotypic plasticity. The malignant T cells in CTCL can possess features of immunomodulating regulatory T cells (Treg) and IL-17-producing helper T cells (Th17) depending on the stimuli they receive from antigen presenting...... therapeutic agents may potentially exploit the phenotypic plasticity of CTCL such that the malignant T cells become vulnerable to antitumor immunity....... cells and other sources. IL-2-type cytokines activate STAT5 to promote expression of Treg-related FoxP3, while various cytokines can activate STAT3 to induce synthesis of IL-10 and IL-17. When the Treg phenotype is activated in the early stages of CTCL, “immune evasion” can occur, allowing the clonal T...

  4. Primary Follicular Lymphoma of the Common Bile Duct Mimicking Cholangiocarcinoma

    Directory of Open Access Journals (Sweden)

    Khaled Youssef Elbanna

    2014-01-01

    Full Text Available Primary non-Hodgkin′s lymphoma of the common bile duct is extremely rare. We present a case with history of inflammatory bowel disease and clinical manifestations of obstructive jaundice. Abdominal magnetic resonance imaging with magnetic resonance cholangiopancreatography (MRCP was done and demonstrated tight stricture at the middle part of common bile duct, and radiological findings were supportive of extra-hepatic cholangiocarcinoma. Whipple′s procedure was performed and the case was histopathologically proven to be non-Hodgkin′s lymphoma of follicular subtype involving the common bile duct. Lymphoma of the hepatobiliary system is usually present as secondary manifestation of systemic malignant lymphoma. However, primary malignant lymphomas arising from the hepatobiliary tree are extremely rare. The radiological appearance of common bile duct lymphoma is very similar to cholangiocarcinoma, making preoperative diagnosis very difficult, as in our present case. We also compare the imaging findings of our case to those seen in reported cases of follicular lymphoma of the common bile duct.

  5. Non-Hodgkin's lymphoma - Part II: Management of primary extranodal lymphomas, generalized disease and salvage treatment

    International Nuclear Information System (INIS)

    Gospodarowicz, Mary K.; Sutcliffe, Simon B.

    1996-01-01

    Objective: To review the approach to the diagnosis, classification, assessment, treatment and continuing management of patients with primary extranodal non-Hodgkin's lymphoma, and the management of generalized disease with the emphasis on the current role of salvage treatment with high dose chemotherapy and stem cell/bone marrow support strategies. Non-Hodgkin's lymphoma may involve any part of the body. Many lymphomas, such as MALT, angiocentric T-cell, etc., commonly present in extranodal sites. Lymphomas presenting in the GI tract, and head and neck, are most common with the single most common site being the stomach. Gastric lymphoma is associated with Helicobacter pylorii and is most common in areas endemic for Helicobacter pylorii infection. Recent advances in the understanding of the etiology of gastric MALT, thyroid, and intestinal lymphomas present new opportunities for the application of novel therapeutic approaches e.g. antibiotic therapy for Helicobacter pylori and early stage IPSID. Lymphomas presenting in the orbit, thyroid, breast, bone, extradural and skin are of interest because of the importance of expert RT in securing local control. Primary brain lymphomas present a particular challenge to the radiation oncologist. Although localized, primary brain lymphomas are extremely difficult to control. Rare sites of extranodal lymphoma include testis, female genital tract, and lung. Extranodal lymphomas are often localized and cure with RT or CMT is possible. They represent a assorted group of diseases with diverse presentations, prognosis, sensitivity to RT and expected outcome. They are of particular importance to radiation oncologists as they require special attention to patterns of spread and treatment planning. The principles of management of primary extranodal lymphoma, however, follow those applicable to localized nodal presentations. Although primary extranodal lymphomas are highly curable, a proportion of patients will fail with disseminated

  6. Primary cardiac lymphoma in a patient with concomitant renal cancer.

    Science.gov (United States)

    Severino, Davide; Santos, Beatriz; Costa, Cátia; Durão, David; Alves, Miguel; Monteiro, Isabel; Pitta, Luz; Leal, Margarida

    2015-12-01

    Primary cardiac lymphoma is defined as non-Hodgkin lymphoma involving the heart and/or pericardium. It is a rare cancer that primarily affects the right heart and in particular the right atrium. By contrast, renal cell carcinoma is a relatively common cancer, which in rare circumstances can metastasize to the heart. It is now known that there is an association between non-Hodgkin lymphoma and renal cell carcinoma, although the underlying mechanisms are not fully understood. The authors present a case of primary cardiac non-Hodgkin lymphoma in a patient with concomitant renal cell carcinoma and explore the possible reasons for this association. Copyright © 2015 Sociedade Portuguesa de Cardiologia. Published by Elsevier España. All rights reserved.

  7. A Challenging Case of Primary Breast Hodgkin's Lymphoma.

    Science.gov (United States)

    Zarnescu, Narcis Octavian; Iliesiu, Andreea; Procop, Alexandru; Tampa, Mircea; Matei, Clara; Sajin, Maria; Costache, Mariana; Dumitru, Adrian; Lazaroiu, Anca Mihaela

    2015-03-01

    Primary breast lymphoma (PBL) is a rare entity accounting for less than 1% of all breast malignancies. Diagnostic criteria for primary Hodgkin's lymphoma of the breast are: the presence of sufficient tissue for diagnosis, close interaction between mammary tissue and lymphomatous infiltrate and no evidence or prior diagnosis of widespread lymphoma. Our case illustrates an unusual presentation of Hodgkin's lymphoma of the breast: clinically as inflammatory breast cancer and core biopsy as granulomatous mastitis, the final diagnosis requiring surgical biopsy. Current information regarding this entity is scant, mainly build upon its rarity. In this paper we assess the clinical presentation, the step-by-step diagnosis, the treatment and the importance of immunohistochemistry in this uncommon condition.

  8. Histone deacetylase inhibitors potentiate photochemotherapy in cutaneous T-cell lymphoma MyLa cells.

    Science.gov (United States)

    Sung, Jane J; Ververis, Katherine; Karagiannis, Tom C

    2014-02-05

    Cutaneous T cell lymphomas (CTCL) represent rare extranodal non-Hodgkin's lymphomas, which are characterised by pleomorphic skin lesions and distinct T-cell markers. CTCL is a relatively benign disease in its early stages, but survival rates decrease significantly with progression. Histone deacetylase inhibitors (HDACi) have recently emerged as a new class of targeted anticancer therapies for CTCL, which have been shown to induce growth inhibition, terminal differentiation and apoptosis in various cancers in vitro and in vivo. In addition to the intrinsic anticancer properties of HDACi, recent studies have demonstrated its ability to synergise with phototherapy. In particular, we examine the therapeutic potential of HDACi in combination with ultraviolet A (UV-A) phototherapy, employing a halogenated DNA minor groove binding ligand called UVASens as a photosensitiser. In vitro studies have demonstrated that UVASens is approximately 1000-fold more potent than current psoralens. The extreme photopotency of UVASens allows the use of lower radiation doses minimising the carcinogenic risks associated with the long-term use of phototherapy. Considering, previous findings using the photosensitiser UVASens and potential synergy of HDACi with phototherapy, it was hypothesised that HDACi will augment photochemotherapy-induced cytotoxicity in CTCL MyLa cells. The findings indicated that combinations of UVASens/UV-A photochemotherapy and HDACi significantly decreased cell viability and increased apoptosis and DNA double-strand breaks in MyLa cells. Crown Copyright © 2014. Published by Elsevier B.V. All rights reserved.

  9. A Case of Cutaneous Plasmablastic Lymphoma in HIV/AIDS with Disseminated Cryptococcus

    Directory of Open Access Journals (Sweden)

    Jun Gong

    2013-01-01

    Full Text Available We present a case of a patient with HIV/AIDS who presented with a tender left lower extremity cutaneous mass over a site of previous cryptococcal infection and was found to have plasmablastic lymphoma (PBL. The incidence of PBL is estimated to account for less than 5% of all cases of non-Hodgkin lymphoma (NHL in HIV-positive individuals. In fact, there were only two reports of extraoral PBL at the time of a 2003 review. PBL in HIV-positive individuals is an aggressive malignancy that tends to occur in middle-aged males with low CD4 counts, high viral loads, and chronic HIV infection. The definitive diagnosis can be made with biopsy which typically shows malignant lymphoid cells that stain positive for plasma cell markers and negative for B-cell markers. The most common treatment is chemotherapy with cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP or CHOP-like regimens, but the overall survival rate is poor despite its relative responsiveness to chemotherapy. This case highlights the challenges that remain in improving clinical outcomes, the importance of antiretroviral therapy and HIV disease control, and a potential association between a chronic inflammatory state caused by disseminated Cryptococcus and tumorigenesis in individuals with PBL.

  10. Role of denileukin diftitox in the treatment of persistent or recurrent cutaneous T-cell lymphoma

    International Nuclear Information System (INIS)

    Lansigan, Frederick; Stearns, Diane M; Foss, Francine

    2010-01-01

    Denileukin diftitox (Ontak ® ) is indicated for the treatment of patients with persistent or recurrent cutaneous T-cell lymphoma (CTCL), a rare lymphoproliferative disorder of the skin. Denileukin diftitox was the first fusion protein toxin approved for the treatment of a human disease. This fusion protein toxin combines the IL2 protein with diphtheria toxin, and targets the CD25 subunit of the IL2 receptor, resulting in the unique delivery of a cytocidal agent to CD-25 bearing T-cells. Historically, immunotherapy targeting malignant T-cells including monoclonal antibodies has been largely ineffective as cytocidal agents compared to immunotherapy directed against B-cells such as rituximab. This review will summarize the development of denileukin diftitox, its proposed mechanism of action, the pivotal clinical trials that led to its FDA approval, the improvements in quality of life, and the common toxicities experienced during the treatment of patients with CTCL. CTCL is often a chronic progressive lymphoma requiring the sequential use of treatments such as retinoids, traditional chemotherapy, or biological response modifiers. The incorporation of the immunotoxin denileukin diftitox into the sequential or combinatorial treatment of CTCL will also be addressed

  11. Primary localised cutaneous amyloidosis - a systematic review

    DEFF Research Database (Denmark)

    Kaltoft, Britta; Schmidt, Grethe; Lauritzen, Anne Falensteen

    2013-01-01

    Amyloidosis is defined as extracellular deposits of heterogenic, misfolded proteins, amyloid fibrils, in various tissues. The aim of our study was to review the literature and to evaluate the risk of developing systemic amyloidosis (SA) and the risk of local recurrence of primary localised...

  12. Primary periosteal lymphoma: an unusual presentation of non-Hodgkin's lymphoma with radiographic, MR imaging, and pathologic correlation

    Energy Technology Data Exchange (ETDEWEB)

    Campbell, Scot E.; Beall, Douglas P.; Sanders, Timothy G. [Department of Radiology, Wilford Hall Medical Center, 759th MDTS/MTRD, 2200 Bergquist Drive, Suite 1, Lackland AFB, TX 78236-5300 (United States); Filzen, Timothy W.; Parsons, Theodore W. [Department of Orthopedic Surgery, Wilford Hall Medical Center, 59th MDW/MCSO, 2200 Bergquist Drive, Suite 1, Lackland AFB, TX 78236-5300 (United States); Bezzant, Shane M. [Department of Radiology, Brooke Army Medical Center, 3851 Roger Brooke Drive, Bldg 3600, Fort Sam Houston, TX 78234-6200 (United States); Burton, Mark P. [Department of Pathology, Wilford Hall Medical Center, 59th MDW/MTLP, 2200 Bergquist Drive, Suite 1, Lackland AFB, TX 78236-5300 (United States)

    2003-04-01

    This report describes a primary periosteal location of non-Hodgkin's lymphoma, without nodal disease, and without adjacent intramedullary disease at presentation. The clinical and imaging appearance of periosteal lymphoma simulates other neoplastic osseous surface tumors more than that of lymphoma in other locations. Consideration of this rare presentation of non-Hodgkin's lymphoma in the differential diagnosis of periosteal bone lesions can be helpful to ensure proper diagnosis and treatment. (orig.)

  13. Primary brain lymphoma in a patient after renal transplantation

    International Nuclear Information System (INIS)

    Arteaga, Carlos; Duarte, Monica; Bayona, Hernan

    2009-01-01

    The incidence of primary central nervous system lymphoma (PCNSL) has increased during the past 40 years. This has been associated with immunodeficiency, mainly in patients infected with the human immunodeficiency virus (HIV) and in transplant patients. Tumor genesis is related with the Epstein-Barr virus (EBV). The most frequent PCNSL immuno phenotype is B-cell lymphoma. Clinical manifestations depend on tumor localization, and are usually behavior dysfunctions and intracranial hypertension syndrome. Differential diagnosis must take into consideration infectious processes, stroke, primary brain tumors, and metastases. The diagnosis of PCNSL requires brain MRI and brain biopsy. It is important to assess HIV infection when diagnosing PCNSL. This review reports a case of primary brain lymphoma in a patient who underwent renal transplantation due to polycystic kidney disease 8 years before.

  14. Primary intestinal hodgkin′s lymphoma: An uncommon presentation

    Directory of Open Access Journals (Sweden)

    Shruti Sharma

    2013-01-01

    Full Text Available Primary intestinal lymphoma is a rare lymphoproliferative neoplasm of the small intestine. The primary nature is established on the basis of lack of evidence of lymphoma on chest X-ray, computerized tomographic scan, peripheral blood or bone marrow puncture. Tumor involvement is limited to the gastrointestinal tract, the criteria for inclusion are that the symptoms related to the small intestine are predominant or the only symptoms at the time of laparotomy. Hodgkin′s lymphoma (HL primarily in the small intestine is a rare entity and an uncommon presentation of the disease. Ileum is the more common site of infliction than the jejunum because of its abundant lymphoid follicles. Here, we present a case of primary intestinal HL, in a 30-year-old male.

  15. [Primary cutaneous Nocardia brasiliensis cellulitis in immunocompetent child].

    Science.gov (United States)

    Shachor-Meyouhas, Yael; Ravid, Sarit; Suhair, Hanna; Kassis, Imad

    2012-08-01

    Primary cutaneous nocardiosis is an infrequent infection among children, generally affecting immunocompromised hosts. It is caused by Gram positive bacteria, partially alcohol and acid resistant which are saprophytes of the soil, water and organic matter. In most cases the causal agent enters through inhalation, and hematogenous dissemination may occur mainly among the immune compromised patients. Direct cutaneous inoculation is less frequent, especially among children. We report an 8-year old female who lives in an urban house with a small garden, who presented with an ulcer on her right shin accompanied by surrounding cellulitis, pain, swelling and fever. The patient's medical history was unremarkable, with no exposure to animals or travelling, except for rafting on the Jordan River the previous week. Culture from the ulcer grew Nocardia brasiliensis, and she recovered after 8 weeks of therapy with trimethoprim-sulphamethoxazole.

  16. Primary pancreatic non-Hodgkin's lymphoma

    Directory of Open Access Journals (Sweden)

    Čolović Nataša

    2005-01-01

    Full Text Available Diffuse large-cell B lymphoma of the pancreas is a rare disease, representing less than 1% of all non-Hodgkin's lymphomas and less than 0.9% of all malignant tumors of the pancreas. About 150 cases of the disease have been observed so far. The tumors are more frequent in the head of the pancreas then in other parts of the organ. They are usually larger (average size of 8 cm and are non-resectionable. As a rule, exact diagnosis is based on the histology and the immunohistology of the specimen taken during open surgery performed for general diagnosis of the pancreatic tumor. Very rarely can a very reliable and experienced cytopathologist establish a proper diagnosis based on material obtained from a fine needle biopsy. The disease usually responds positively to immunochemotherapy according to protocol R-CHOP. Occasionally, additional radiotherapy may be required. We present two women, 66 and 49 years old, in whom a diagnosis of large-cell B lymphoma of the pancreas was established, based on the histology and the immunohistochemistry of a specimen taken during open surgery performed in order to remove pancreatic tumors, which turned out to be non-resectionable. After immunochemotherapy, the symptoms disappeared and the tumors shrank, in one patient after additional radiotherapy. The authors would like to point out the importance of a proper histological diagnosis, which permitted the application of immunochemotherapy alone or together with additional radiotherapy with at least temporarily favorable results.

  17. A Rare Case of Primary Breast Mucosa- Associated Lymphoid Tissue Lymphoma

    Directory of Open Access Journals (Sweden)

    Marić Daliborka

    2016-12-01

    Full Text Available Breast involvement by lymphoma is uncommon and poses challenges in diagnosis. Breast involvement by malignant lymphoma, whether primary or secondary, is a rare event. Primary breast lymphomas account for 0.38% - 0.7% of all non-Hodgkin lymphomas, 1.7%-2.2% of all extranodal non-Hodgkin lymphomas, and only 0.04% - 0.5% of all breast cancer cases. Most frequent primary breast lymphomas are diffuse large B cell lymphomas (53%. Breast mucosa-associated lymphoid tissue (MALT lymphomas account for a small fraction of all the MALT lymphomas (1% - 2%. Herein we report a case of a patient with primary breast MALT lymphoma and its presentation on different imaging modalities. Two years after the presentation and treatment with eight cycles of chemotherapy, the patient is alive and well, without evidence of residual disease or recurrence.

  18. Primary CNS lymphoma in a patient treated with azathioprine

    DEFF Research Database (Denmark)

    Glesner, Matilde Kanstrup; Ocias, Lukas Frans; Larsen, Thomas Stauffer

    2014-01-01

    with surrounding oedema. There was cerebrospinal fluid pleocytosis, and Epstein-Barr virus (EBV) DNA was detected in the spinal fluid by PCR. A brain biopsy confirmed the suspicion of primary brain lymphoma. EBV-associated primary brain lymphoma is a relevant differential diagnosis in patients with long......A 33-year-old man treated with azathioprine for 12 years for Crohn's disease presented with headache, nausea and vomiting accompanied by difficulty in putting words together and slight mental confusion. Prednisolone and antibiotics were without effect. MRI of the brain showed multiple focal lesions...

  19. Primary orbital precursor T-cell lymphoblastic lymphoma

    DEFF Research Database (Denmark)

    Stenman, Lisa; Persson, Marta; Enlund, Fredrik

    2016-01-01

    Primary T-cell lymphoblastic lymphoma (T-LBL) in the eye region is very rare. The present study described a unique case of T-LBL involving the extraocular muscles. A 22-year-old male patient presented with a 3-week history of headache, reduced visual acuity and edema of the left eye. Clinical...... knowledge, this is the first report of a case of T-LBL involving the extraocular muscles. Although primary T-LBL in the eye region is very rare, our findings demonstrate that lymphoma should be considered in the differential diagnosis of patients with similar symptoms....

  20. Linfoma cutâneo de células B: relato de caso Cutaneous B-cell lymphoma: a case report

    Directory of Open Access Journals (Sweden)

    Mariana de Gusmão Nunes

    2004-12-01

    Full Text Available Os linfomas cutâneos são classificados em linfomas de células B ou de células T, sendo os primeiros menos freqüentes. O linfoma cutâneo de células B (LCCB pode ser primário ou secundário, tendo o último história natural mais agressiva, com pior prognóstico. Os autores apresentam um caso de LCCB secundário com três anos de evolução, curso indolente, sem envolvimento de outros órgãos além da pele, com ótima resposta ao tratamento quimioterápico. A distinção entre LCCB primário e secundário é muito difícil de ser realizada, uma vez que eles são clínica e histopatologicamente indistinguíveis. Deve-se sempre pesquisar o acometimento de órgãos internos para então definir o prognóstico.Cutaneous lymphomas are classified as either type B cell or T cell lymphoma, the former type being less frequent. Cutaneous B cell lymphoma (CBCL may be primary or secondary. The latter has a more aggressive natural history with a worse prognostic. In this paper, the authors present a secondary CBCL case with a three-year evolution, indolent course, without involvement of other organs beside the skin and with optimal response to chemotherapeutic treatment. The distinction between primary and secondary CBCL is very difficult to determine insofar as they are clinically and histopathologically indistinguishable. It is always necessary to investigate whether internal organs have been affected prior to defining the prognosis.

  1. Clinico pathological spectrum of mycosis fungoides type cutaneous t-cell lymphoma

    International Nuclear Information System (INIS)

    Shaikh, Z.I.; Rahman, S.B.

    2006-01-01

    Objective: To determine the clinical, histological, and immuno phenotypic characteristics of mycosis fungoides type cutaneous T-cell lymphoma. Design: Descriptive study. Place and Duration of Study: This study was conducted from January 2000 to December 2004 at the Department of Dermatology, Military Hospital and the Department of Dermatopathology, Armed Forces Institute of Pathology, Rawalpindi. Materials and Methods: The medical case records of patients with mycosis fungoides diagnosed during the period of study were surveyed. Data was collected pertaining to patients' characteristics, clinical descriptions, histopathological features, immuno phenotypic analysis and stage of disease at the time of diagnosis. Results: A total of 33 cases of mycosis fungoides were diagnosed between the years 2000 and 2004. There were 24 male and 9 female patients with male to female ratio of 2.6:1 The age ranged from 24 to 68 years and the duration of disease prior to diagnosis varied between 2 to 36 months. The number of skin biopsies performed for definite diagnosis ranged from 01 to 5. The various clinical presentations recorded in these patients were hypo pigmented patches in 7 (21.3%), infiltrated papules and plaques in 6 (18.2%), erythroderma in 5 (15.2%), psoriasiform lesions in 3 (9%), and nodular lesions in 3 (9%) patients. There were 2 (6%) cases respectively of noduloulcerative, ichthyosiform and poikilodermatous lesions, and 1 (3%) case each of follicular, morphoea-like and purpuric skin lesions. The predominant histological features were lymphocytic infiltrate in the upper dermis, epidermotropism, haloed lymphocytes in epidermis, Pautrier's micro abscesses, and interface dermatitis. The immunohistochemical studies (n=12) showed predominantly T helper cell immuno phenotype (CD3+, CD45RO+) in 11 (92%) cases and T suppressor cell immuno phenotype (CD3+, CD8+) in 1 (8%) patient. Conclusion: The mycosis fungoides type cutaneous T-cell lymphoma has a wide clinico

  2. Magnetic resonance imaging findings of primary bone lymphoma presenting with pathological fracture

    Directory of Open Access Journals (Sweden)

    Elif Karadeli

    2016-03-01

    Full Text Available Malignant bone lymphomas are uncommonly encountered clinically. Men, 77-years-old, was admitted to hospital because of pain in the left shoulder. After imaging and histological examination diagnosed primary bone lymphoma. Primary lymphoma of the bone is an extranodal lymphoma that arises from the medullary cavity and manifests as a localized, solitary lesion, which represents 1% of all malignant lymphomas. [Cukurova Med J 2016; 41(0.100: 103-104

  3. Primary Non-Hodgkin's Malignant Lymphoma of the Sinonasal Tract

    Directory of Open Access Journals (Sweden)

    Nitin Gupta

    2009-09-01

    Full Text Available Primary non-Hodgkin’s lymphomas (NHL of the sinonasal tract are rather uncommon entities. Morphologically and radiographically, sinonasal lymphomas are difficult to distinguish from other malignant neoplasms or non- neoplastic processes. They have a variable presentation from fulminant destructive manifestations to chronic indolent type of disease and may mimic as carcinomas and invasive fungal infection respectively. We report a case of primary NHL involving sinonasal tract in elderly female, which was clinically and radiologically mimicking as sinonasal malignany and was proven as NHL on histological examination and confirmed by immunohistochemistry. A high index of suspicion, appropriate histopathological examination and immunohistochemistry is necessary to differentiate sinonasal lymphomas from other possibilities. Failure to do so may miss the diagnosis and delay appropriate treatment

  4. Primary gastrointestinal non-Hodgkin's lymphoma in adults

    DEFF Research Database (Denmark)

    Hansen, P B; Vogt, K C; Skov, Robert L

    1998-01-01

    OBJECTIVES: To analyse the clinical course and the histopathology of primary gastrointestinal non-Hodgkin's lymphoma (GI-NHL) in adult patients and to investigate a possible impact of Helicobacter pylori. DESIGN/SETTING: Retrospective study of all adult patients in Copenhagen county diagnosed...... during a 6-year period with NHL. SUBJECTS: A total of 55 patients with GI-NHL diagnosed during the period from 1985 to the end of 1990. RESULTS: Twenty-eight patients had primary lymphoma in the stomach, 14 in the small intestine, 11 in the large intestine and two patients had multifocal involvement....... The dominant presenting symptoms were abdominal pain, weight loss, diarrhoea, constipation and fatigue. Acute emergency problems such as severe haemorrhage or perforation at initial presentation were unusual. According to the revised European-American lymphoma (REAL) classification, diffuse large B...

  5. Primary breast lymphoma in an immunocompromised male patient: A case report

    International Nuclear Information System (INIS)

    Yim, Bong Guk; Park, Jeong Seon; Koo, Hye Ryoung; Kim, Soo Yeon; Jang, Ki Seok; Kim, Jin Young; Choi, Yun Young

    2015-01-01

    Primary breast lymphoma in a male patient is extremely rare. We report a case of primary breast lymphoma in an immunocompromised male patient, after renal transplantation. The sonographic and histological features are described in depth

  6. Primary breast lymphoma in an immunocompromised male patient: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Yim, Bong Guk; Park, Jeong Seon; Koo, Hye Ryoung; Kim, Soo Yeon; Jang, Ki Seok [Hanyang University Medical Center, Hanyang University College of Medicine, Seoul (Korea, Republic of); Kim, Jin Young; Choi, Yun Young [Dept. of Hanyang University Guri Hospital, Hanyang University College of Medicine, Guri (Korea, Republic of)

    2015-10-15

    Primary breast lymphoma in a male patient is extremely rare. We report a case of primary breast lymphoma in an immunocompromised male patient, after renal transplantation. The sonographic and histological features are described in depth.

  7. Heart of Lymphoma: Primary Mediastinal Large B-Cell Lymphoma with Endomyocardial Involvement

    Directory of Open Access Journals (Sweden)

    Elisa Rogowitz

    2013-01-01

    Full Text Available Primary mediastinal B-cell lymphoma (PMBCL is an uncommon aggressive subset of diffuse large B-cell lymphomas. Although PMBCL frequently spreads locally from the thymus into the pleura or pericardium, it rarely invades directly through the heart. Herein, we report a case of a young Mexican female diagnosed with PMBCL with clear infiltration of lymphoma through the cardiac wall and into the right atrium and tricuspid valve leading to tricuspid regurgitation. This was demonstrated by cardiac MRI and transthoracic echocardiogram. In addition, cardiac MRI and CT scan of the chest revealed the large mediastinal mass completely surrounding and eroding into the superior vena cava (SVC wall causing a collar of stokes. The cardiac and SVC infiltration created a significant therapeutic challenge as lymphomas are very responsive to chemotherapy, and treatment could potentially lead to vascular wall rupture and hemorrhage. Despite the lack of conclusive data on chemotherapy-induced hemodynamic compromise in such scenarios, her progressive severe SVC syndrome and respiratory distress necessitated urgent intervention. In addition to the unique presentation of this rare lymphoma, our case report highlights the safety of R-CHOP treatment.

  8. Primary lymphoma of the liver - A complex diagnosis

    Science.gov (United States)

    Steller, Ernst JA; van Leeuwen, Maarten S; van Hillegersberg, Richard; Schipper, Marguerite EI; Rinkes, Inne HM Borel; Molenaar, Izaak Q

    2012-01-01

    A 59-year-old woman presented with the clinical symptoms and radiologic investigations of a liver lesion suspect of metastasis. However, postoperative histopathology revealed a primary hepatic lymphoma (PHL). The case of a patient with a solitary PHL, which was treated by resection and subsequent chemotherapy, will be discussed with a short overview of the literature. PMID:22423319

  9. [Thyroid and lipidic dysfunction associated with bexarotene in cutaneous T-cell lymphoma].

    Science.gov (United States)

    Rodriguez Suarez, Santiago; Pamies Andreu, Encarnación; Muñiz Grijalvo, Ovidio; Garcia Morillo, José Salvador

    2016-02-05

    Bexarotene is a synthetic selective X receptor rexinoide approved for the systemic treatment of cutaneous T-cell lymphoma. During treatment is very frequent the occurrence of hypothyroidism and severe mixed hyperlipidemia, both are reversibles and dose-dependent adverse events. Increase of triglycerides and LDL-cholesterol level (up to even higher levels have been associated with pancreatitis in some cases) is widely described (as is the case with other retinoids) but decrease in HDL-cholesterol is poored know. We review our experience with the use of bexarotene. We present a serie of 3 clinical report of patients treated with bexarotene in whose, in addition to these well-known adverse event, a serious lowering of HDL-cholesterol was observed. The 3 patients studied had metabolic complications like central hypothyroidism and severe mixed hyperlipidemia; with special emphasis on the sharp fall (mean decrease>83%) in the HDL-cholesterol level. Cholesterol lowering medication and substitutive hormonal replacement with levotiroxine resulted in an improvement of the biochimical parameters without reaching the correct goals. Bexarotene produce as predictable side effects severe mixed hyperlipidemia with marked decrease in HDL-cholesterol levels and central hypothyroidism, being the both reversible and dose-dependent. A reflection on the possible aetiological mechanisms and implications of this phenomenon are included. Copyright © 2015 Elsevier España, S.L.U. All rights reserved.

  10. A new protoparvovirus in human fecal samples and cutaneous T cell lymphomas (mycosis fungoides).

    Science.gov (United States)

    Phan, Tung G; Dreno, Brigitte; da Costa, Antonio Charlys; Li, Linlin; Orlandi, Patricia; Deng, Xutao; Kapusinszky, Beatrix; Siqueira, Juliana; Knol, Anne-Chantal; Halary, Franck; Dantal, Jacques; Alexander, Kathleen A; Pesavento, Patricia A; Delwart, Eric

    2016-09-01

    We genetically characterized seven nearly complete genomes in the protoparvovirus genus from the feces of children with diarrhea. The viruses, provisionally named cutaviruses (CutaV), varied by 1-6% nucleotides and shared ~76% and ~82% amino acid identity with the NS1 and VP1 of human bufaviruses, their closest relatives. Using PCR, cutavirus DNA was found in 1.6% (4/245) and 1% (1/100) of diarrhea samples from Brazil and Botswana respectively. In silico analysis of pre-existing metagenomics datasets then revealed closely related parvovirus genomes in skin biopsies from patients with epidermotropic cutaneous T-cell lymphoma (CTCL or mycosis fungoides). PCR of skin biopsies yielded cutavirus DNA in 4/17 CTCL, 0/10 skin carcinoma, and 0/21 normal or noncancerous skin biopsies. In situ hybridization of CTCL skin biopsies detected viral genome within rare individual cells in regions of neoplastic infiltrations. The influence of cutavirus infection on human enteric functions and possible oncolytic role in CTCL progression remain to be determined. Copyright © 2016 Elsevier Inc. All rights reserved.

  11. Burkitts primary thyroid lymphoma coexistence with Hashimoto's thyroiditis

    International Nuclear Information System (INIS)

    Higuera, A.; Vicente, J.; Lazaro, J. C.

    2000-01-01

    Th primary thyroid lymphoma is a rare neoplasm, above all in children. We present a case of a child with Burkitt's thyroid lymphoma as the only manifestation of this disease, associated to lymphocytic thyroiditis. Clinically, it initiated as a rapidly growing goiter with compressive symptomatology. The X-ray findings are described: hypoechoic and hypodense multiple nodes that affect the right thyroid lobe and isthmus, with extraglandular extension to the vascular space and to the mediastinum. The differential diagnosis is considered with other more frequent thyroid pathologies in this age group. (Author) 14 refs

  12. Primary bone lymphoma of the distal tibia mimicking brodie's abscess

    International Nuclear Information System (INIS)

    Park, Jina; Lee, Seung Hun; Joo, Kyung Bin; Park, Chan Kum

    2014-01-01

    The 'penumbra sign' on an unenhanced T1-weighted image is a well-known characteristic of Brodie's abscess, and this sign is extremely helpful for discriminating subacute osteomyelitis from other bone lesions. We present a case of primary bone lymphoma of the distal tibia mimicking subacute osteomyelitis with Brodie's abscess in a 50-year-old woman. Initial radiographs and MRI showed a lesion in the distal tibia consistent with Brodie's abscess with the penumbra sign. Histopathological examination of the surgical biopsy specimen confirmed the presence of a diffuse large B-cell lymphoma involving the bone.

  13. Unusual Origin and Rare Presentation of Primary Cardiac Lymphoma

    Science.gov (United States)

    Mohamed, Amir; Cherian, Sanjay; El-Ashmawy, Ahmed; Abdelmoneim, Salah Eldin; Soliman, Maher; Abu-Rayan, Mohamed; Kalangos, Afksendyios

    2011-01-01

    Non-Hodgkin lymphoblastic lymphomas are very uncommon tumors that rarely involve the heart; however, when they do, they typically cause cardiac symptoms. Herein, we describe the case of a young woman who presented with respiratory symptoms. These were caused by a high-grade lymphoblastic lymphoma, which originated in the left inferior pulmonary vein and extended into the left atrium. The tumor was surgically debulked, but it recurred in 1 month, and the patient underwent chemotherapy. Six months later, she had recurrent respiratory symptoms, and echocardiography revealed a persistent mass in the left lower lobar vein. A modified chemotherapy regimen led to complete resolution of the tumor within 2 months. We are unaware of other reports of a primary cardiac non-Hodgkin lymphoblastic lymphoma with this unusual site of origin and rare manifestation of symptoms. PMID:21841872

  14. Primary bone lymphoma: A report of two cases and review of the literature

    Directory of Open Access Journals (Sweden)

    Singh Tejinder

    2010-01-01

    Full Text Available Primary bone lymphoma (PBL is an uncommon tumor accounting for approximately 4-5% of extra nodal lymphoma and less than 1% of all non-Hodgkin′s lymphoma. Disease may be complicated at presentation by pathological fracture or spinal cord compression. Diffuse large-B-cell lymphoma (DLBCL accounts for the majority of cases of PBL. Owing to its rarity, only a few retrospective studies have been published addressing the prognosis and treatment of primary bone lymphoma. In this paper, we report our experience with two cases of PBL treated with chemotherapy and radiotherapy and review literature to elucidate the optimal treatment of primary bone lymphoma.

  15. Brentuximab Vedotin Treatment for Primary Refractory Hodgkin Lymphoma

    Directory of Open Access Journals (Sweden)

    Hung-Bo Wu

    2013-01-01

    Full Text Available Up to 40% of patients with advanced Hodgkin lymphoma (HL become refractory or relapsed after current standard chemotherapy, among which primary refractory HL confers a particularly poor outcome. With intensive salvage chemotherapy and autologous stem cell transplantation, the long-term remission rate for these patients was only 30%, but more selective treatments with higher therapeutic index are needed. We report the experience of using a new anti-CD30 immunotoxin, brentuximab vedotin, in salvage treatment of a 30-year-old woman with primary refractory Hodgkin lymphoma. The patient presented with SVC syndrome due to the bulky mediastinal tumor and was confirmed to have classical Hodgkin lymphoma, nodular sclerosis type, stage IIIA. The tumor responded to induction chemotherapy transiently, but local progression was noted during subsequent cycles of treatment. Salvage radiotherapy to the mediastinal tumor, obtained no remission but was followed by rapid in-field progression and then lung metastasis. She declined stem cell transplantation and received salvage brentuximab vedotin (BV therapy, which induced dramatic shrinkage of tumor without significant side effects. Serial followup of PET/CT imaging confirmed a rapid and continuous complete remission for 12 months. Although durability of the remission needs further observation, this case illustrates the excellent efficacy of brentuximab vedotin in primary refractory Hodgkin lymphoma.

  16. Primary breast lymphoma: A single-institute experience in Taiwan

    Directory of Open Access Journals (Sweden)

    Che-Wei Ou

    2014-10-01

    Full Text Available Background: Breast is an uncommon location of lymphoma involvement. The most common type of primary breast lymphoma (PBL is diffuse large B-cell lymphoma (DLBCL. Rituximab is the widely used monoclonal antibody against CD20+ B-cell lymphoma, especially DLBCL. We aimed to analyze the clinical features, prognostic factors, and treatment outcome with or without rituximab in primary breast DLBCL. Methods: We retrospectively analyzed patients diagnosed with PBL from October 1987 to March 2012 in our hospital, excluding metastasis by whole-body computed tomography and bone marrow study. Results: Twenty-three patients were diagnosed with PBL. All were females. Eighteen patients were stage IE and five were stage IIE according to the Ann Arbor staging system. Two patients had lymphoma other than DLBCL. The median age of primary breast DLBCL patients was 48 years (range 27-79. Two were excluded from the analysis due to refusal or ineligibility for chemotherapy. No significant prognostic factor was found. Patients receiving chemotherapy with (RC or without (C rituximab were not significantly different in the 5-year overall survival (RC: 57.1%; C: 58.3%; p = 0.457 or progression-free survival (RC: 57.1%; C: 50.0%; p = 0.456. A high incidence of relapse in the central nervous system (CNS (17.6% was observed. Conclusions: In accordance with prior literature reports, our Taiwanese cohort of primary breast DLBCL seemed younger than those reported in Japan, Korea, and Western societies. Relapse in the CNS was not uncommon. The benefit of rituximab in addition to chemotherapy was not statistically significant. Treatment modality remained to be defined by further large-scale studies.

  17. Primary mucosa-associated lymphoid tissue thyroid lymphoma: a rare thyroid neoplasm of extrathyroid origin

    Directory of Open Access Journals (Sweden)

    Dimitrios Hadjidakis

    2012-01-01

    Full Text Available Primary thyroid lymphoma is a rare malignancy, representing 2-8% of all thyroid malignancies and 1-2% of all extranodal lymphomas. The majority of cases concern non-Hodgkin`s lymphoma of B cell origin, following by Hodgkin’s disease, T cell lymphomas and rarely marginal zone B-cell mucosa-associated lymphoid tissue (MALT lymphomas. MALT lymphomas have been associated with long-standing autoimmune Hashimoto`s thyroiditis. We present the case of a 44-years-old woman with thyroid MALT lymphoma in the background of multinodular goiter of autoimmune origin.

  18. Bilateral primary lymphoma of kidney. Report of a case

    International Nuclear Information System (INIS)

    León Acosta, Pedro; Perdomo Hernando, Yaslin; Ceballos Nápoles, Yanis Janel; Pila Pérez, Rafael; Pila Peláez, Rafael

    2016-01-01

    Background: We present a case of primary renal lymphoma no bilateral Hodgkin of both kidneys which is uncommon Objective: To present the clinical case of a patient that dies four days of their entrance in which was discovered a bilateral renal mass with the anatomopathological diagnostic of primary renal lymphoma for necropsy stud . Case presentation: It is a 40 year-old patient, with family antecedents of neoplasias and personal of gastritis of seven months of evolution. The patient was entered for fever of 39 ℃ an important abdominal pain, in which was important the anemic and constitutional syndrome with loss of 11 kg in the last months; and diminished diuresis. A tumor was verified between 12 and 15 cm in both flanks of hard and not painful irregular surface. The patient presented unfavorable evolution for what was moved to room of cares intermissions. In the analytic study they were verified as exams of importance the Hb 89 g/L, the speed of globular sedimentation of 95 mm/first hour, the LDH 1495 UI/L, the creatinine of 537 mmol /L and several pathological glycemias one of them of 20 mmol/L. The x- ray results as much the ultrasonography as the contrasted on-line axial tomography guided the possibility to be a renal tumor. The medullogram and the laparoscopic were not useful for the diagnosis. The patient died and she was carried out histopathological necropsy study, which informed a lymphoma not bilateral Hodgkin of kidney of cells B, without an affectation to another organ Conclusions: They are uncommon the cases of primary renal lymphoma, since the renal affectation for a process lymphproliferative is in general secondary to a systemic illness. This case completes the fundamental approaches of Malbrain to consider it as a lymphoma non Hodgkin. The diagnosis is carried out by means of renal biopsy, during the surgery or necropsy for anatomopathological study. (author)

  19. Analysis of treatment outcomes for primary tonsillar lymphoma

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Yun Hee [Dept. of Radiation Oncology, Gyeongsang National University Hospital, Gyeongsang National University School of Medicine, Jinju (Korea, Republic of); Cho, Seok Goo; Jung, Seung Eun; Kim, Sung Hoon; O, Joo Hyun; Park, Gyeong Sin; Yang, Suk Woo; Lee, In Seok; Rhee, Chin; Kook; Choi, Byung Ock [Catholic University Lymphoma Group (CULG), Seoul St. Mary' s Hospital, College of Medicine, The Catholic University of Korea, Seoul (Korea, Republic of)

    2016-12-15

    Although each Waldeyer’s ring sub-site is considered an independent prognostic factor, few studies have assessed the prognosis and treatment of tonsillar lymphoma. Treatment outcomes were analyzed in patients with primary tonsillar lymphoma who were treated with chemotherapy and radiotherapy (RT). Nineteen patients with diffuse large B-cell lymphoma were evaluated, with a median follow-up of 53 months. Age, sex, and histology, amongst other factors, were reviewed. Progression-free survival (PFS) and overall survival (OS) rates were analyzed. Most patients had Ann Arbor stage I-II (94.7%), IPI score of 0 (89.5%), and complete remission after chemotherapy (89.5%). The 5-year PFS and OS rates were 74.6% and 80%, respectively. In univariate analysis, the rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) regimen resulted in a better PFS than the cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) regimen (88.9% vs. 50.0%; p = 0.053). RT dose was related to the survival outcome (p = 0.010 for PFS, p = 0.044 for OS). Patients were classified into the CHOP + RT (>40 Gy) group and R-CHOP + RT (≤40 Gy) group. The 5-year PFS rates were 50% in the CHOP + RT group, and 100 % in the R-CHOP + RT group (p = 0.018). The 5-year OS rates were 66.7% and 100%, respectively (p = 0.087). Primary tonsillar lymphoma patients typically have favorable outcomes. Chemotherapy (R-CHOP) combined with relatively lower dose consolidative RT may be safe and effective for primary tonsillar lymphoma.

  20. Hydroa vacciniforme-like lymphoma with primarily periorbital swelling: 7 cases of an atypical clinical manifestation of this rare cutaneous T-cell lymphoma.

    Science.gov (United States)

    Plaza, Jose A; Sangueza, Martin

    2015-01-01

    Hydroa vacciniforme-like lymphoma (HVL) is a rare cutaneous T-cell lymphoma that is usually seen in children of Hispanic or Asian origin. Association between chronic latent Epstein-Barr virus infection in both hydroa vacciniforme (HV) and HVL has been demonstrated and has recently been categorized by the World Health Organization as one of the Epstein Barr virus-positive lymphoproliferative disorders of childhood. Patients with HVL present with a cutaneous rash characterized by edema, blisters, ulcers, and scars mainly seen on the face and extremities that mimic HV; however, unlike in HV, the lesions tend to be extensive and deeper and are associated with severe scarring, necrosis, and systemic manifestations. We are reporting 7 cases of an unusual clinical variant of HVL with primarily periorbital edema. All of our patients in this series presented with progressive periorbital edema that was accompanied with systemic symptoms including fever, malaise, and lymphadenopathy. Most cases were initially misinterpreted as inflammatory processes including cellulitis, arthropod bite reactions, and periorbital lupus erythematosus. The biopsy of these lesions revealed an atypical lymphocytic infiltrate predominantly distributed in the deep dermis and in subcutaneous fat. Immunohistochemistry studies revealed a cytotoxic T-cell (CD8) profile. All cases were associated with Epstein-Barr virus infection. Our study presents a rare clinical variant of HVL with predominant periorbital edema. This variant could potentially be overlooked and misdiagnosed as an inflammatory condition; thus, it needs to be included in the differential diagnosis of periorbital edema in young patients.

  1. Total Skin Electron Therapy for Cutaneous T-Cell Lymphoma Using a Modern Dual-Field Rotational Technique

    Energy Technology Data Exchange (ETDEWEB)

    Heumann, Thatcher R. [Emory University School of Medicine, Emory University, Atlanta, Georgia (United States); Esiashvili, Natia [Department of Radiation Oncology, Emory University, Atlanta, Georgia (United States); Winship Cancer Institute (WCI), Emory University, Atlanta, Georgia (United States); Parker, Sareeta [Department of Dermatology, Emory University, Atlanta, Georgia (United States); Switchenko, Jeffrey M. [Biostatistics Shared Core Resource at WCI, Emory University, Atlanta, Georgia (United States); Dhabbaan, Anees [Department of Radiation Oncology, Emory University, Atlanta, Georgia (United States); Winship Cancer Institute (WCI), Emory University, Atlanta, Georgia (United States); Goodman, Michael [Department of Epidemiology, Rollins School of Public Health, Emory University, Atlanta, Georgia (United States); Lechowicz, Mary Jo; Flowers, Christopher R. [Department of Radiation Oncology, Emory University, Atlanta, Georgia (United States); Department of Hematology and Oncology, Emory University, Atlanta, Georgia (United States); Khan, Mohammad K., E-mail: drkhurram2000@gmail.com [Department of Radiation Oncology, Emory University, Atlanta, Georgia (United States); Winship Cancer Institute (WCI), Emory University, Atlanta, Georgia (United States)

    2015-05-01

    Purpose: To report our experience with rotational total skin electron irradiation (RTSEI) in cutaneous T-cell lymphoma (CTCL), and to examine response by disease stage and race. Methods and Materials: We reviewed our outcomes for 68 CTCL patients who received RTSEI (≥30 Gy) from 2000 to 2013. Primary outcomes were complete clinical response (CCR), recurrence-free survival (RFS), and overall survival (OS). Using log–rank tests and Cox proportional hazards, OS and RFS were compared across tumor stages at time of RTSEI with further racial subgroup analysis. Results: Median age at diagnosis and at time of radiation was 52 and 56 years, respectively. Median follow-up was 5.1 years, 49% were African American, and 49% were female. At time of treatment, 18, 37, and 13 patients were T stage 2, 3, and 4, respectively. At 6 weeks after RTSEI, overall CCR was 82% (88%, 83%, and 69% for T2, T3, and T4, respectively). Median RFS was 11 months for all patients and 14, 10, and 12 months for stage T2, T3, and T4, respectively. Tumor stage was not associated with RFS or CCR. Maintenance therapy after RTSEI was associated with improved RFS in both crude and multivariable analysis, controlling for T stage. Median OS was 76 months (91 and 59 months for T3 and T4, respectively). With the exception of improved OS in African Americans compared with whites at stage T2, race was not associated with CCR, RFS, or OS. Conclusions: These results represent the largest RTSEI clinical outcomes study in the modern era using a dual-field rotational technique. Our observed response rates match or improve upon the standard set by previous outcome studies using conventional TSEI techniques, despite a large percentage of advanced CTCL lesions in our cohort. We found that clinical response after RTSEI did not seem to be affected by T stage or race.

  2. A case of cutaneous large B-cell lymphoma of the legs appearing as chronic venous ulceration

    Directory of Open Access Journals (Sweden)

    Marta Carlesimo

    2012-04-01

    Full Text Available We report here a case of a woman with a cutaneous large B-cell lymphoma of the legs. She had a plaque lesion, superficially ulcerated and necrotized with tumorous borders situated on the posterior side of the right leg and two red or bluish-red nodular lesions. A skin biopsy from both nodular and plaque lesion showed a diffuse infiltrate of atypical large B cells CD20+ and CD79a+, spanning epidermis, dermis and subcutaneous tissue. A therapeutic approach containing anti-CD20 monoclonal antibody (rituximab was suggested.

  3. Total skin electron beam therapy for cutaneous T-cell lymphoma: a nationwide cohort study from Denmark

    DEFF Research Database (Denmark)

    Lindahl, Lise M; Kamstrup, Maria Rørbæk; Petersen, Peter M

    2011-01-01

    Background. Total skin electron beam therapy (TSEBT) is an effective palliative treatment for cutaneous T-cell lymphoma (CTCL). In the present study we reviewed the clinical response to TSEBT in Danish patients with CTCL. Material and methods. This retrospective study included 35 patients with CTCL...... to treatment compared to patients treated with high-dose. Consequently the study with low-dose was discontinued and published. In patients treated with high-dose the overall response rate was 100%. Complete response (CR) rate was 68% and CR occurred after a median time of 2.1 months (range 1.8 months-2.0 years...

  4. A unique case of primary effusion lymphoma-like lymphoma showing disappearance and recurrence of the body cavity effusion.

    Science.gov (United States)

    Koeda, Chikahiko; Sato, Takashi; Matsumoto, Yuki; Usui, Yuta; Kunugida, Fusanori; Ogawa, Muneyoshi

    2017-03-01

    Primary effusion lymphoma-like lymphoma (PEL-LL) is a rare B-cell lymphoma that the etiology remains unclear. We describe a case of PEL-LL with a pleuropericardial effusion. Diagnosis required long period of time as it followed a unique progress of disappearance and recurrence of the body cavity effusion. We finally had a diagnosis of B-cell lymphoma by the immunocytochemistry of effusion using the cell block procedure. Authors consider that it is valuable to actively try the cell block procedure at the time of the first drainage for early diagnosis, if the body cavity effusion due to the malignancy is suspected.

  5. Processos linfoproliferativos da pele: parte 2 - linfomas cutâneos de células T e de células NK Processos linfoproliferativos da pele: part 2 - cutaneous T-cell and NK-cell lymphomas

    Directory of Open Access Journals (Sweden)

    José Antonio Sanches Jr

    2006-02-01

    currently classified and subdivided based on their clinical behavior, according to a consensus reached between the World Health Organization and the European Organization for Research and Treatment of Cancer. The cutaneous NKT/cell lymphomas of indolent clinical behavior comprise the classical mycosis fungoides, folliculotropic mycosis fungoides, pagetoid reticulosis, granulomatous slack skin, primary cutaneous anaplastic large cell lymphoma, lymphomatoid papulosis, subcutaneous panniculitis-like T-cell lymphoma and primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma. The aggressive clinical behavior cutaneous NKT/cell lymphomas include Sézary syndrome, extranodal NK/T-cell lymphoma, nasal type, primary cutaneous aggressive epidermotropic CD8+T-cell lymphoma, cutaneous gamma-delta T-cell lymphoma and primary cutaneous peripheral T-cell lymphoma, unspecified. The adult T-cell leukemia lymphoma and CD4+ CD56+ hematodermic neoplasm are considered systemic lymphomas but are addressed in this article for their initial cutaneous manifestations in a significant number of patients. The diagnosis of these processes is based on histological examination complemented by phenotypic analysis of neoplastic cells, which is essential for classification. The recommended staging is based on type and extension of cutaneous involvement, clinical conditions and histological examination of lymph nodes and organs. Hematological assessment is fundamental to characterize Sézary syndrome. The recommended therapies include exclusively cutaneous treatment, biological response modifiers and systemic chemotherapy.

  6. Intralesional rituximab in primary conjunctival follicular lymphoma relapsed.

    Science.gov (United States)

    Rodríguez Villa, S; Ruiz Rodríguez, M J; Vargas Pabón, M

    2017-07-01

    A 49-year-old woman experienced a local relapse of a primary follicular lymphoma (FL) of the conjunctiva. She received 4 weekly intra-lesional injections followed by 6 monthly injections of rituximab (6mg/ml). A clinical response was achieved after first injection. No adverse ocular event or signs of lymphoma relapse were seen after 10 months of follow-up. Intralesional administration of rituximab for treating primary FL of the conjunctiva was an effective and safe therapeutic option; therefore it could be an alternative to other conventional treatments, such as radiotherapy or chemotherapy. Copyright © 2016 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L.U. All rights reserved.

  7. Real world data on primary treatment for mantle cell lymphoma

    DEFF Research Database (Denmark)

    Abrahamsson, Anna; Albertsson-Lindblad, Alexandra; Brown, Peter N

    2014-01-01

    to prognostic factors and first-line treatment in patients with MCL in a population-based data set. Data were collected from the Swedish and Danish Lymphoma Registries from the period of 2000 to 2011. A total of 1389 patients were diagnosed with MCL. During this period, age-standardized incidence MCL increased...... analysis. Hence, by a population-based approach, we were able to provide novel data on prognostic factors and primary treatment of MCL, applicable to routine clinical practice....

  8. Radiographically Negative, Asymptomatic, Sentinel Lymph Node Positive Cutaneous T-Cell Lymphoma in a 3-Year-Old Male: A Case Report

    Directory of Open Access Journals (Sweden)

    Jeffrey Carson

    2012-01-01

    Full Text Available We present a case of a 3-year-old male originally diagnosed with a CD30+ anaplastic cutaneous T-cell lymphoma with no evidence of systemic disease after CT scan, PET scan, and bone marrow aspiration. Sentinel lymph node biopsy (SLNB was performed as an additional step in the workup and showed microscopic disease. Current management/recommendations for cutaneous T-cell lymphoma do not include SLNB. Medical and surgical management of cutaneous malignancies is dramatically different for local versus advanced disease. Therefore adequate evaluation is necessary to properly stage patients for specific treatment. Such distinction in extent of disease suggests more extensive therapy including locoregional radiation and systemic chemotherapy versus local excision only. Two international case reports have described SLNB in cutaneous T-cell lymphoma with one demonstrating evidence of node positive microscopic disease despite a negative metastatic disease workup. This case is being presented as a novel case in a child with implications including lymphoscintigraphy and SLNB as a routine procedure for evaluation and staging of cutaneous T-cell lymphoma if the patient does not demonstrate evidence of metastatic disease on routine workup.

  9. Primary non-Hodgkin lymphoma of skeletal muscle: imaging findings

    International Nuclear Information System (INIS)

    Zhou Liangping; Peng Weijun; Tang Feng; Mao Jian; Yang Wentao

    2006-01-01

    Objective: To analyze the imaging manifestations of primary non-Hodgkin lymphoma of skeletal muscle and improve the recognition of this rare disease. Methods: Five cases of primary non- Hodgkin lymphoma of skeletal muscle proved pathologically underwent imaging exam, including MRI and CT in 3 cases, only MRI in 1 case, only CT in 1 case, X-ray in 2 cases and bone scintigraphy in 2 cases. Results: Diffuse enlargements of involved muscle with presentation of overall configuration were observed in all five cases. All 4 cases manifested as homogeneous soft masses, which is isoattenuating to normal muscle on unenhanced CT images. After intravenous injection of contrast media, the masses enhanced homogeneously and slightly (2 cases) or moderately (1 case) on CT images. The lesions were homogenous and had isointense or slightly low signal intensity compared with that of uninvolved muscle on T 1 -weighted images and high signal intensity on T 2 -weighted images. After intravenous injection of contrast media, all 2 cases enhanced homogeneously and moderately with the enhanced signal intensity of involved muscle greatly higher than that of uninvolved muscle on MR images. Two cases of X-ray plain showed no destruction of bone and 2 cases of bone scintigraphy exams showed increased radiotracer uptake of involved muscle with no infiltration of bone marrow. Conclusion: There are several characteristics on the imaging of primary non-Hodgkin lymphoma of skeletal muscle. MRI is the optimal imaging method for the diagnosis of this disease. (authors)

  10. Primary Lymphoma of the Gall Bladder

    African Journals Online (AJOL)

    1974-06-29

    Jun 29, 1974 ... phomas thus constitute about 10% of all primary sarcomas of the gall ... Department of Pathology, University of Cape Town. J B. C. BOTHA, M.B. CH.B. L. B. KAHN ... mon hepatic duct at its junction with the cystic duct. The.

  11. Primary Lymphoma of the Gall Bladder

    African Journals Online (AJOL)

    1974-06-29

    Jun 29, 1974 ... textbooks of pathology.'" We have been able to find only. 11 cases documented in the world literature; and only 4 of these in the English literature..·• They have appeared as isolated case reports or are mentioned in reviews deal- ing with primary sarcomas of the gall bladder. To the best of our knowledge ...

  12. Primary Breast Mucosa-Associated Lymphoid Tissue (MALT Lymphoma Transformation to Diffuse Large B-cell Lymphoma: A Case Report

    Directory of Open Access Journals (Sweden)

    Şerife Hülya Arslan

    2012-09-01

    Full Text Available Primary non-Hodgkin’s lymphoma (NHL of the breast constitutes 0.04%-0.53% of all malignancies and 2.2% of extra nodal lymphomas. In total, 7%-8% of all B-cell lymphomas are the mucosa-associated lymphoid tissue (MALT type, of which up to 50% of primary gastric MALT lymphoma. Herein we present a patient with breast MALT lymphoma that transformed to diffuse large B-cell lymphoma (DLBCL. A 69-year-old female presented with a mass on her left breast. Physical examination showed a 3 × 3-cm mass located 1 cm from the areola on the upper lateral quadrant of the breast at the 1 o’clock position, which was fixed and firm. Excisional biopsy was performed and pathologic examination of the specimen showed MALT lymphoma transformation to DLBCL. The patient was staged as II-EA. The rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone (R-CHOP protocol was scheduled as treatment. Following 6 courses of R-CHOP, 2 additional courses of rituximab were administered. Positron emission tomography (PET-CT was done at the end of the treatment. PET showed that the patient was in complete remission. At the time this report was written, the patient was being followed-up at the outpatient clinic on a regular basis. Lymphoma of the breast is a rarity among malignant tumors of the breast. The most common type of lymphoma is DLBCL. Breast MALT lymphoma is extremely rare. Primary MALT lymphoma of the breast can transform from low grade to high grade and recurrence is possible; therefore, such patients should be monitored carefully for transformation.

  13. MRI findings in primary brain lymphoma in immunocompetent patients

    Directory of Open Access Journals (Sweden)

    Saeed Nadhim Younis

    2017-08-01

    Full Text Available Background and objective: Primary brain lymphoma is an extranodal aggressive intracranial neoplasm of lymphocytic origin originating and confined to the brain parenchyma and meninges. It is rare in immune competent patients, but its incidence is increasing. This retrospective study was conducted to record the MRI features of primary brain lymphoma at the time of diagnosis in immunocompetent patients. Methods: Of the 450 patients diagnosed with the brain tumor during a period of five years from 2008 to 2013, the clinical features and MRI findings of 16 cases of pathologically proven to be non-Hodgkin’s lymphoma were studied. All the patients were tested negative for HIV and there was no history of immune suppression drugs or any other chronic illness. All the patients were examined with MRI observing the tumor location, multifocality, signal intensity in different sequences, enhancement patterns, peritumoral edema, the presence of hemorrhage and calcification. Results: Of the 16 patients, including the monofocal and multifocal cases, 30 lesions exhibited. The mean age at diagnosis was 53 years. Nine patients (56.25% found to have a multifocal disease. In more than 75% of lesions, MRI was hypo to iso signal on T1 and T2. Mild to moderate perilesional edema, strong contrast enhancement and restricted diffusion were seen in all cases. The hemorrhagic tumor was noticed in four lesions (13.3%. No calcification and no leptomeningeal lesions were noted. The MRI images in post steroid therapy were studied within one month of treatment. Tumour regression was noticed in 21/30 (70%, stable in 3/30 (10% and progressing in 6/30 (20%. Conclusion: MRI is a reliable imaging technique in the management of patients with primary brain lymphoma. Early accurate diagnosis is crucial to avoid the unnecessary operation and shift patients from extensive surgery to chemoradiotherapy.

  14. CT findings of primary thyroid lymphoma : report of two cases

    Energy Technology Data Exchange (ETDEWEB)

    Rhee, Ji Yong; Lee, Nam Joon; Seol, Hye Young; Kim, Jung Hyuk [Korea Univ. College of Medicine, Seoul (Korea, Republic of)

    1999-03-01

    We describe two cases of primary thyroid lymphoma, involving 64-and 74-year-old women who presented with a rapidly growing palpable mass in the anterior neck. In both patients, plain radiographs of this region revealed tracheal displacement and soft tissue mass, and CT scans demonstrated the presence of a large soft tissue mass with homogeneously low attenuation, predominantly located in the unilateral lobe of the thyroid gland. Within the masses there was no calcification, necrosis, or hemorrhage, though in one patient the trachea was involved posteriorly by the mass Both masses appeared as cold nodules on RI scan, and in both patients, the final diagnosis was diffuse large cell type non-Hodgkin's lymphoma with a background of Hashimoto's thyroiditis.

  15. Epigenetic profiling of cutaneous T-cell lymphoma: promoter hypermethylation of multiple tumor suppressor genes including BCL7a, PTPRG, and p73.

    Science.gov (United States)

    van Doorn, Remco; Zoutman, Willem H; Dijkman, Remco; de Menezes, Renee X; Commandeur, Suzan; Mulder, Aat A; van der Velden, Pieter A; Vermeer, Maarten H; Willemze, Rein; Yan, Pearlly S; Huang, Tim H; Tensen, Cornelis P

    2005-06-10

    To analyze the occurrence of promoter hypermethylation in primary cutaneous T-cell lymphoma (CTCL) on a genome-wide scale, focusing on epigenetic alterations with pathogenetic significance. DNA isolated from biopsy specimens of 28 patients with CTCL, including aggressive CTCL entities (transformed mycosis fungoides and CD30-negative large T-cell lymphoma) and an indolent entity (CD30-positive large T-cell lymphoma), were investigated. For genome-wide DNA methylation screening, differential methylation hybridization using CpG island microarrays was applied, which allows simultaneous detection of the methylation status of 8640 CpG islands. Bisulfite sequence analysis was applied for confirmation and detection of hypermethylation of eight selected tumor suppressor genes. The DNA methylation patterns of CTCLs emerging from differential methylation hybridization analysis included 35 CpG islands hypermethylated in at least four of the 28 studied CTCL samples when compared with benign T-cell samples. Hypermethylation of the putative tumor suppressor genes BCL7a (in 48% of CTCL samples), PTPRG (27%), and thrombospondin 4 (52%) was confirmed and demonstrated to be associated with transcriptional downregulation. BCL7a was hypermethylated at a higher frequency in aggressive (64%) than in indolent (14%) CTCL entities. In addition, the promoters of the selected tumor suppressor genes p73 (48%), p16 (33%), CHFR (19%), p15 (10%), and TMS1 (10%) were hypermethylated in CTCL. Malignant T cells of patients with CTCL display widespread promoter hypermethylation associated with inactivation of several tumor suppressor genes involved in DNA repair, cell cycle, and apoptosis signaling pathways. In view of this, CTCL may be amenable to treatment with demethylating agents.

  16. [A case of primary central nervous system anaplastic lymphoma kinase positive anaplastic large cell lymphoma manifested as a unilateral pachymeningits].

    Science.gov (United States)

    Fujisawa, Etsuco; Shibayama, Hidehiro; Mitobe, Fumi; Katada, Fumiaki; Sato, Susumu; Fukutake, Toshio

    2017-11-25

    There have been 23 reports of primary central nervous system anaplastic lymphoma kinase (ALK)-positive anaplastic large cell lymphoma in the literature. Here we report the 24th case of a 40-year-old man who presented with occipital headache for one month. His contrast-enhanced brain MRI showed enhancement around the right temporal lobe, which suggested a diagnosis of hypertrophic pachymeningitis. He improved with steroid therapy. After discharge, however, he was readmitted with generalized convulsive seizures. Finally, he was diagnosed as primary central nervous system ALK-positive anaplastic large cell lymphoma by brain biopsy. Primary central nervous system lymphoma invading dura matter can rarely manifests as a unilateral pachymeningitis. Therefore, in case of pachymeningitis, we should pay attention to the possibility of infiltration of lymophoma with meticulous clinical follow-up.

  17. Primary cutaneous malignancies in the Northern Cape Province of ...

    African Journals Online (AJOL)

    Results. A total of 4 270 biopsies (13 cutaneous malignancies) were identified. The commonest was squamous cell carcinoma (SCC), followed by basal cell carcinoma, Kaposi's sarcoma (KS), cutaneous malignant melanoma (CMM) and basosquamous carcinoma, in descending order. The odds of a white male developing ...

  18. Primary mantle cell lymphoma of tonsil: Case report

    Directory of Open Access Journals (Sweden)

    Knežević Snežana B.

    2017-01-01

    Full Text Available Introduction: Mantle cell lymphoma is rare type of the mature B cell lymphoma. It includes 4% - 6% of all Non Hodgkin's Lymphomas. Compared to the other subtypes of lymphoma it develops more often in older men, and the median age of patients is 65 years. Primary tonsillar lymphoma accounts for less than 1% of head and neck malignancies. Method: Data obtained from medical records of the patient. Objective: Emphasize the importance of early and accurate diagnosis and early treatment of malignant diseases. Case report: Patient RP, 63 years old, presents with difficult swallowing, hoarseness, enlarged tonsils, snoring. Left tonsil almost sets into the right tonsillar vine, displaces the uvula and covers the isthmus. Respiratory sound is normal, with rhythmic action of the heart and soft abdomen. Good general condition. Echo: enlarged and actively altered lymph glands of the middle right jugular chain, the largest 148x77 mm, on the left side lymph nodes are enlarged, the largest is 143x72 mm. Echo of the abdomen inconspicuous. Lab: WBC 5.9, RBC 5.2, Hb 152, Hct 0.44, SE 10, CK 129, LDH 331, CRP 4.6, ALP 61, fibrinogen 2.4, Ca2+ 2.3, phosphate 0.8; BK, HCV, HBsAg, EB, HIV negative. X-ray of the chest inconspicuous. Admitted to the hematology department of the General Hospital. PH: Immunoproliferative disease. Immunohistochemistry, at the institute of Pathology: IHH CK AE1-AE3, PAX5 +, CD20 +, CD3, bcl2 +, bcl6-, CyklinD1 +, CD23-, CD43 +, MUM1 - / +, Ki67 + in about 20% of the tumor cells. Morphological and immunohistochemical findings: Mantle cell lymphoma. MSCD of the neck, chest and upper abdomen: Left tonsil diameter is 28x32 mm and length is 36mm, with lobular contour and heterogeneous structure, asymmetrically narrowing lumen of the airways to 7 mm. pathologically enlarged submandibular and par jugular lymph nodes (10-15 mm diameter on the left. There were no pathological findings in the lung parenchyma. Abdominal and retroperitoneal lymph nodes

  19. MRI manifestations of primary muscle non-Hodgkin lymphoma

    International Nuclear Information System (INIS)

    Zhou Jianjun; Wang Jianhua; Zeng Mengsu; Ya Fuhua; Zhou Kangrong; Ding Jianguo; Ji Yuan

    2009-01-01

    Objective: To explore and evaluate MRI in diagnosing primary muscle non-Hodgkin lymphoma. Methods: Six surgically confirmed primary muscle non-Hodgkin lymphoma underwent MR examination including T 1 WI, T 2 WI and T 1 WI enhanced studies. The acquired images date was reviewed and analysed retrospectively in comparison with surgical and pathological results. Results: The locations of 6 cases were cervical part (2), upper extremity (1), lower extremity (3), respectively. All cases involved of more than one anatomical compartment with poorly defined solid masses in 5 cases and well defined in 1 cases, 5 extended to subcutaneous fat and 3 extended along the neurovascular bundle. The mean tumor diameter was 13.9 cm, ranging from 7.3 to 22.5 cm. One was well demarcated and 5 were ill-defined. On T 1 WI, 2 were slightly high signal intensity and 4 were slightly low signal intensity. On T 2 WI, 2 were slightly high signal intensity, 3 were intermediate signal intensity and 1 was high signal intensity. Five were inhomogeneous and 1 was homogeneous. The intrinsic structure such as muscle fiber, tendo, spatium intramuscular were detected on 5 cases. Of the 5 dynamic contrast-enhanced cases, it showed moderate enhancement during arterial phase, 2 were homogeneous and 3 were inhomogeneous. And it showed progressive enhancement during interstitial phase, 3 were homogeneous and 2 were inhomogeneous. Conclusions: Primary muscle lymphoma always originated deep to the fascia showing subcutaneous extension and multiple compartment invasion. Typically from poorly defined solid masses with slightly high in signal intensity on MR T 2 WI and middle degree dynamic delayed contrasted-enhanced in which intrinsic anatomic structure such as muscle fiber, tendo, spatium intramuscular and so on can be discerned, almost all cases involve more than one muscle compartment and some of tumor extend along the neurovascular bundle. (authors)

  20. ONC201 selectively induces apoptosis in cutaneous T-cell lymphoma cells via activating pro-apoptotic integrated stress response and inactivating JAK/STAT and NF-κB pathways

    OpenAIRE

    Ni, Xiao; Zhang, Xiang; Hu, Cheng-Hui; Langridge, Timothy; Tarapore, Rohinton S.; Allen, Joshua E.; Oster, Wolfgang; Duvic, Madeleine

    2017-01-01

    Cutaneous T-cell lymphomas (CTCLs) are extremely symptomatic and still incurable, and more effective and less toxic therapies are urgently needed. ONC201, an imipridone compound, has shown efficacy in pre-clinical studies in multiple advanced cancers. This study was to evaluate the anti-tumor activity of ONC201 on CTCL cells. The effect of ONC201 on the cell growth and apoptosis were evaluated in CTCL cell lines (n=8) and primary CD4+ malignant T cells isolated from CTCL patients (n=5). ONC20...

  1. Primary central nervous system B-cell lymphoma in a young dog

    Science.gov (United States)

    Kim, Na-Hyun; Ciesielski, Thomas; Kim, Jung H.; Yhee, Ji-Young; Im, Keum-Soon; Nam, Hae-Mi; Kim, Il-Hwan; Kim, Jong-Hyuk; Sur, Jung-Hyang

    2012-01-01

    This report describes a primary central nervous system B-cell lymphoma in a 3-year-old intact female Maltese dog. Canine primary central nervous system lymphomas constitute about 4% of all intracranial primary neoplasms, but comprehensive histopathologic classifications have rarely been carried out. This is the first report of this disease in a young adult dog. PMID:23115372

  2. Primary Lymphoma of the Liver: A Case Report and Review

    Directory of Open Access Journals (Sweden)

    Franzjosef Schweiger

    2000-01-01

    Full Text Available The case of a previously healthy man who developed primary non-Hodgkin’s lymphoma of the liver is presented. Biopsy confirmed that the tumour was of the diffuse large cell type and was of apparent T-cell origin. The diagnosis of these rare tumours is suggested by the presence of a hepatic mass without lymphadenopathy, splenomegaly or bone marrow involvement, as well as normal carcinoembryonic antigen and alpha-fetoprotein levels. However, histological examination of tissue is essential to confirm the diagnosis. The response to treatment varies, but surgical resection and/or chemotherapy can result in prolonged remissions. The literature on this topic is briefly reviewed.

  3. Masquerade Syndrome of Multicentre Primary Central Nervous System Lymphoma

    Directory of Open Access Journals (Sweden)

    Silvana Guerriero

    2011-01-01

    Full Text Available Purpose. In Italy we say that the most unlucky things can happen to physicians when they get sick, despite the attention of colleagues. To confirm this rumor, we report the sad story of a surgeon with bilateral vitreitis and glaucoma unresponsive to traditional therapies. Methods/Design. Case report. Results. After one year of steroidal and immunosuppressive therapy, a vitrectomy, and a trabeculectomy for unresponsive bilateral vitreitis and glaucoma, MRI showed a multicentre primary central nervous system lymphoma, which was the underlying cause of the masquerade syndrome. Conclusions. All ophthalmologists and clinicians must be aware of masquerade syndromes, in order to avoid delays in diagnosis.

  4. MRI appearance of primary non-Hodgkin's lymphoma of bone

    International Nuclear Information System (INIS)

    Hermann, G.; Abdelwahab, I.F.; Klein, M.J.; Kenan, S.

    1997-01-01

    Objective. To evaluate the signal characteristics of primary non-Hodgkin's lymphoma of bone on MRI. Designs and patients. Ten patients with primary non-Hodgkin's lymphoma of bone were included in the study. T1- and T2-weighted imaging was performed. The signal intensity of the lesions was compared with that of the surrounding muscle. Results. The results of the MRI were compared with the histological findings. In the majority of cases (5/10) the lesion involved the femur. In one case each the tibia, humerus, ileum, sacrum, and skull, respectively, were affected. A soft tissue mass was present in four cases. In nine of ten cases on T1-weighted imaging the lesion was hypointense. On T2-weighted imaging seven of ten lesions were hypointense compared with muscle, one isointense and, in two cases, part of the lesion showed slightly hyperintense signal. In all ten cases the signal pattern appeared inhomogeneous. Pathological examination showed extensive fibrosis in the majority of cases. Conclusion. According to our results there is decreased signal intensity of bone marrow on both T1- and T2-weighted imaging, unlike other primary round cell tumors of bone. Because the diagnoses were established with small tissue biopsies, the reason for these findings is speculative. (orig.)

  5. Increased risk of gastric adenocarcinoma after treatment of primary gastric diffuse large B-cell lymphoma

    International Nuclear Information System (INIS)

    Inaba, Koji; Morota, Madoka; Mayahara, Hiroshi; Ito, Yoshinori; Sumi, Minako; Uno, Takashi; Itami, Jun; Kushima, Ryoji; Murakami, Naoya; Kuroda, Yuuki; Harada, Ken; Kitaguchi, Mayuka; Yoshio, Kotaro; Sekii, Shuhei; Takahashi, Kana

    2013-01-01

    There have been sporadic reports about synchronous as well as metachronous gastric adenocarcinoma and primary gastric lymphoma. Many reports have dealt with metachronous gastric adenocarcinoma in mucosa-associated lymphoid tissue lymphoma of stomach. But to our knowledge, there have been no reports that document the increased incidence of metachronous gastric adenocarcinoma in patients with gastric diffuse large B-cell lymphoma. This retrospective study was conducted to estimate the incidence of metachronous gastric adenocarcinoma after primary gastric lymphoma treatment, especially in diffuse large B-cell lymphoma. The retrospective cohort study of 139 primary gastric lymphoma patients treated with radiotherapy at our hospital. Mean observation period was 61.5 months (range: 3.7-124.6 months). Patients profile, characteristics of primary gastric lymphoma and metachronous gastric adenocarcinoma were retrieved from medical records. The risk of metachronous gastric adenocarcinoma was compared with the risk of gastric adenocarcinoma in Japanese population. There were 10 (7.2%) metachronous gastric adenocarcinoma patients after treatment of primary gastric lymphomas. It was quite high risk compared with the risk of gastric carcinoma in Japanese population of 54.7/100,000. Seven patients of 10 were diffuse large B-cell lymphoma and other 3 patients were mixed type of diffuse large B-cell lymphoma and mucosa associated lymphoid tissue lymphoma. Four patients of 10 metachronous gastric adenocarcinomas were signet-ring cell carcinoma and two patients died of gastric adenocarcinoma. Metachronous gastric adenocarcinoma may have a more malignant potential than sporadic gastric adenocarcinoma. Old age, Helicobacter pylori infection and gastric mucosal change of chronic gastritis and intestinal metaplasia were possible risk factors for metachronous gastric adenocarcinoma. There was an increased risk of gastric adenocarcinoma after treatment of primary gastric lymphoma

  6. Cutaneous features seen in primary liver cell (Hepatocellular ...

    African Journals Online (AJOL)

    kemrilib

    features associated with the entity as a possible aid to diagnosis cutaneous features being considered a cheap tool that can help ... liver cell cancer (PLCC) and cancer of the breast and ... laboratory based -abdominal ultrasonography, liver.

  7. Primary cutaneous meningioma on the scalp of an infant; a case report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Su Jeong; Lim, Ji Yeon; Kim, Sung Mee; Yoo, Eun Ae [Presbyterian Medical Center, Chunju (Korea, Republic of)

    2001-02-01

    During childhood, meningioma is an uncommon tumor, and in infants, extracranial meningioma is very rare. We report a case of primary cutaneous menigioma occurring on the scalp of a ten-month-old girl.

  8. Primary diffuse large B-cell lymphoma of the corpora cavernosa presented as a perineal mass

    Directory of Open Access Journals (Sweden)

    González-Satué Carlos

    2012-01-01

    Full Text Available Primary male genital lymphomas may appear rarely in testis, and exceptionally in the penis and prostate, but there is not previous evidence of a lymphoma arising from the corpora cavernosa. We report the first case in the literature of a primary diffuse cell B lymphoma of the corpora cavernosa presented with low urinary tract symptoms, perineal pain and palpable mass. Diagnosis was based on trucut biopsy, histopathological studies and computed tomographic images.

  9. Primary CNS lymphoma as a cause of Korsakoff syndrome.

    Science.gov (United States)

    Toth, Cory; Voll, Chris; Macaulay, Robert

    2002-01-01

    Korsakoff syndrome presents with memory dysfunction with retrograde amnesia, anterograde amnesia, limited insight into dysfunction, and confabulation. The most common etiology of Korsakoff syndrome is thiamine deficiency secondary to alcoholism. There are limited case reports of structural lesions causing Korsakoff syndrome. A 46-year-old male with a long history of alcoholism presented with a history of confusion, amnesia, and confabulation with no localizing features on neurological examination. The patient showed no clinical change with intravenous thiamine. Computed tomography of the brain revealed a heterogenous, enhancing mass lesion centered within the third ventricle, with other lesions found throughout cortical and subcortical regions. The patient was given dexamethasone i.v. without noticeable clinical improvement but with marked radiological improvement with mass reduction. Stereotactic biopsy revealed a diagnosis of primary central nervous system (CNS) lymphoma. Most patients presenting with Korsakoff syndrome have thiamine deficiency; however, mass lesions can produce an identical clinical picture. This is the first case report of a patient with primary CNS lymphoma presenting as Korsakoff syndrome.

  10. FOXP3+ regulatory T cells in cutaneous T-cell lymphomas: association with disease stage and survival

    DEFF Research Database (Denmark)

    Gjerdrum, L M; Woetmann, A; Odum, Niels

    2007-01-01

    FOXP3 is a unique marker for CD4+CD25+ regulatory T cells (Tregs). In solid tumours, high numbers of Tregs are associated with a poor prognosis. Knowledge about the implications of Tregs for the behaviour of haematological malignancies is limited. In this study, skin biopsies from 86 patients...... with mycosis fungoides (MF) and cutaneous T-cell lymphoma (CTCL) unspecified were analysed for the expression of FOXP3 on tumour cells and tumour-infiltrating Tregs. Labelling of above 10% of the neoplastic cells was seen in one case classified as an aggressive epidermotropic CD8+ cytotoxic CTCL....... In the remaining 85 cases, the atypical neoplastic infiltrate was either FOXP3 negative (n=80) or contained only very occasional weakly positive cells (n=5). By contrast, all biopsies showed varying numbers of strongly FOXP3+ tumour-infiltrating Tregs. MF with early or infiltrated plaques had significantly higher...

  11. Trends in the treatment of cutaneous T-cell lymphoma – critical evaluation and perspectives on vorinostat

    Directory of Open Access Journals (Sweden)

    Zhang CL

    2012-02-01

    Full Text Available Sophia Rangwala, Madeleine Duvic, Chunlei ZhangDepartment of Dermatology, The University of Texas MD Anderson Cancer Center, Houston, Texas, USAAbstract: Epigenetic modification with small molecule histone deacetylase inhibitors has been a promising new anti-neoplastic approach for various solid and hematological malignancies, particularly cutaneous T-cell lymphoma (CTCL. Oral vorinostat was the first histone deacetylase inhibitor approved to enter the clinical oncology market for treating CTCL patients who have progressive, persistent, or recurrent disease after failing two systemic therapies. In two phase II clinical trials, oral vorinostat was found to be safe and effective at a dose of 400 mg/day, with an overall response rate of 24%–30% in heavily pretreated patients with advanced CTCL, including those with large-cell transformed mycosis fungoides and Sézary syndrome. About half of CTCL patients receiving vorinostat also experienced substantial relief in pruritus and thus a marked improvement in quality of life. A subsequent follow-up study reported long-term safety and clinical benefits of vorinostat in patients with refractory CTCL, regardless of previous treatment failures. The most frequent side effects of vorinostat include gastrointestinal symptoms, fatigue, and thrombocytopenia. These adverse reactions are dose-related and reversible upon cessation of therapy. Preclinical studies have supported the therapeutic potential of vorinostat by demonstrating in vitro and in vivo anti-tumor activities against CTCL, including selective induction of apoptosis in malignant T cells, inhibition of angiogenesis, suppression of signal transducer and activator of transcription proteins, and up-regulation of pro-apoptotic proteins. Identification of biomarkers of response and resistance will help select CTCL patients most likely to benefit from treatment and guide the design of effective combination therapies.Keywords: cutaneous T-cell lymphoma

  12. Primary intracranial malignant lymphoma. Report of nine cases

    Energy Technology Data Exchange (ETDEWEB)

    Matsumoto, Mikiro; Ohtsuka, Takatsugu; Kuroki, Takao; Shibata, Iekado; Terao, Hideo; Kudo, Motoshige

    1988-12-01

    Nine cases of primary intracranial malignant lymphoma, which accounts for 3.3 % of all intracranial tumors seen in the authors' institution, were studied in terms of diagnostic computed tomographic (CT) features, the tumors' histologic appearance, treatment, post-treatment blood immunologic and cerebrospinal fluid (CSF) characteristics, and outcome. The patients were seven males and two females aged 42 to 67 years. Their chief signs and symptoms on admission were intracranial hypertension, focal signs, and disturbance of consciousness. CT, which proved the most useful preoperative diagnostic technique, demonstrated multiple lesions in seven cases and, in all cases, regions of isodensity or slight high density that were enhanced by contrast medium. According to the patterns of enhancement, the tumors were classed as diffuse (three cases) or nodular (six cases). The former is considered typical of malignant lymphoma, whereas the latter type was sometimes indistinguishable from metastatic tumor and meningioma. At surgery, one patient underwent radical tumor excision, two partial removal, and six biopsy only. Histologic examination revealed one tumor to be of the diffuse small cell type, three of the medium cell type, and five of the large cell type (Lymphoma Study Group classification). Of seven tumors in which lymphocytes were examined by peroxidase-antiperoxidase staining, four were of the B cell type. Postoperatively, whole brain irradiation with 29 to 46 Gy was followed by local irradiation with 15 to 50 Gy. If the tumor persisted, one of three chemotherapies was administered. In one case, methotrexate was given intrathecally. Seven patients were divided into two groups: long remission (three) and recurrence (four). These two groups were compared in terms of serum immunoglobulin levels, T and B cell ratios, CSF characteristics, CT features, tumor cell type, and treatment. No clear differences were found.

  13. Immunohistochemical Characterization of Canine Lymphomas

    Directory of Open Access Journals (Sweden)

    Roxana CORA

    2017-11-01

    Full Text Available Lymphomas occur by clonal expansion of lymphoid cells and have distinctive morphological and immunophenotypic features. Determination of canine lymphoma immunophenotype is useful for accurate prognosis and further therapy. In the suggested study, we performed an immunohistochemical evaluation of some cases with canine lymphoma diagnosed in the Department of Pathology (Faculty of Veterinary Medicine, Cluj-Napoca, Romania, in order to characterize them. The investigation included 39 dogs diagnosed with different anatomical forms of lymphoma, following necropsy analysis or assessment of biopsies. The diagnosis of lymphoma was confirmed by necropsy and histopathology (Hematoxylin-eosin stain examinations. The collected specimens were analyzed by immunohistochemistry technique (automatic method using the following antibodies: CD3, CD20, CD21 and CD79a. The analyzed neoplasms were characterized as follows: about 64.10% of cases were diagnosed as B-cell lymphomas, 33.34% of cases as T-cell lymphomas, whereas 2.56% of cases were null cell type lymphomas (neither B nor T. Most of multicentric (80%, mediastinal (60% and primary central nervous system lymphomas (100% had B immunophenotype, while the majority of cutaneous (80% and digestive (100% lymphomas had T immunophenotype. Immunohistochemical description of canine lymphomas can deliver some major details concerning their behavior and malignancy. Additionally, vital prognosis and efficacy of some therapeutic protocols are relying on the immunohistochemical features of canine lymphoma.

  14. Primary non-Hodgkins lymphoma of eye and adnexa. Effect of method of treatment for prognosis

    Directory of Open Access Journals (Sweden)

    E. E. Grishina

    2013-01-01

    Full Text Available Purpose: To identify predictors of NHL of the vision associated with treatment choice for prognosis of primary non-Hodgkin’s lymphoma eye and adnexa.Methods: A retrospective and prospective study characteristics of the disease in 94 patients with primary lymphoma of the vision. Orbital lymphoma diagnosed in 35 patients (36 %, conjunctival — in 48 patients (52 %, the least damage observed age — 11 patients (12 %. Among the various types of malignant lymphomas morphologic immunological dominated B-cell lymphoma or marginal zone MALT-lymphoma, which were diagnosed in 70 (75 %. Patients were treated with radiation, chemotherapy and combined chemoradiotherapy.Results: During the follow-up period of 1 year to 26 years (median follow-up 5 years of the 94 patients the primary lymphoma of the vision in 22 cases (23 % relapses occurred in a period of 6 months to 10 years (median 2 years. In analyzing the data we found that the 5‑year disease-free survival with radiation therapy, chemotherapy, and combination therapy is the same and is equal to — 70 %.Conclusion: The treatment and monitoring of patients with primary non-Hodgkin’s lymphoma eye and adnexa should be implemented jointly ophthalmologist and oncology. The choice of treatment, with adequate treatment is prescribed, can not be associated with the weather and can not be a predictor of the primary non-Hodgkin’s lymphoma eye and adnexa.

  15. Primary intravascular large B-cell lymphoma of pituitary

    Directory of Open Access Journals (Sweden)

    K R Anila

    2012-01-01

    Full Text Available A 68-year-old retired nurse, who was a known hypertensive on medication, presented with prolonged fever of 2-month duration without any clinical evidence of infection. On examination she had altered mental status. She also had other nonspecific complaints such as sleep disturbances, loss of weight, etc. On investigation, she was found to have anemia, thrombocytopenia, raised erythrocyte sedimentation rate (ESR, C-reactive protein (CRP, and lactate dehydrogenase (LDH values. She also had electrolyte imbalance. Radiological evaluation of brain showed mass lesion in the sella turcica, suggestive of pituitary adenoma. Biochemical evaluation showed hypopituitarism. Trans-sphenoidal biopsy was done. Based on histopathological and immunohistochemical findings a diagnosis of intravascular large B-cell lymphoma (IVLBCL of pituitary was made. Our patient′s condition deteriorated rapidly and she succumbed to her illness before therapy could be initiated. We are reporting this case because of the rare subtype of large B-cell lymphoma presenting at an extremely unusual primary site.

  16. Primary B-cell Lymphoma of the Thyroid Featuring the Different Ultrasonographic Findings

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Ji Na; Choi, Yoon Jung; Kim, Dong Hoon [Kangbuk Samsung Medical Center, Seoul (Korea, Republic of)

    2009-06-15

    We review here 3 cases of primary thyroid lymphoma that we experienced during the past 5 years (age range: 39-55, all of the patients were female). The clinical and various ultrasonographic characteristics together with the other imaging modalities of primary thyroid lymphomas are described. The clinical features at presentation for one patient were a goiter with rapid growth and this was accompanied by compressive symptoms. The tumors of the other 2 patients were incidentally found during screening thyroid ultrasound exams. The pathologic studies of 2 cases showed a diffuse B-cell lymphoma with associated Hashimoto's thyroiditis and one case was a B-cell lymphoma of the MALT type. An extra-thyroid extension was shown in one case. The treatments included surgery alone for two cases, and chemotherapy and radiation therapy for one case. A US exam of thyroid lymphoma can show various morphological features, and US-CNB is helpful for diagnosing thyroid lymphoma.

  17. cMyc/miR-125b-5p signalling determines sensitivity to bortezomib in preclinical model of cutaneous T-cell lymphomas

    DEFF Research Database (Denmark)

    Manfè, Valentina; Biskup, Edyta; Willumsgaard, Ayalah

    2013-01-01

    Successful/effective cancer therapy in low grade lymphoma is often hampered by cell resistance to anti-neoplastic agents. The crucial mechanisms responsible for this phenomenon are poorly understood. Overcoming resistance of tumor cells to anticancer agents, such as proteasome inhibitors, could...... improve their clinical efficacy. Using cutaneous T-cell lymphoma (CTCL) as a model of the chemotherapy-resistant peripheral lymphoid malignancy, we demonstrated that resistance to proteasome inhibition involved a signaling between the oncogene cMyc and miR-125b-5p. Bortezomib repressed c...

  18. Primary central nervous system lymphoma: is absence of intratumoral hemorrhage a characteristic finding on MRI?

    Science.gov (United States)

    Sakata, Akihiko; Okada, Tomohisa; Yamamoto, Akira; Kanagaki, Mitsunori; Fushimi, Yasutaka; Dodo, Toshiki; Arakawa, Yoshiki; Takahashi, Jun C; Miyamoto, Susumu; Togashi, Kaori

    2015-06-01

    Previous studies have shown that intratumoral hemorrhage is a common finding in glioblastoma multi-forme, but is rarely observed in primary central nervous system lymphoma. Our aim was to reevaluate whether intratumoral hemorrhage observed on T2-weighted imaging (T2WI) as gross intratumoral hemorrhage and on susceptibility-weighted imaging as intratumoral susceptibility signal can differentiate primary central nervous system lymphoma from glioblastoma multiforme. A retrospective cohort of brain tumors from August 2008 to March 2013 was searched, and 58 patients (19 with primary central nervous system lymphoma, 39 with glioblastoma multiforme) satisfied the inclusion criteria. Absence of gross intratumoral hemorrhage was examined on T2WI, and an intratumoral susceptibility signal was graded using a 3-point scale on susceptibility-weighted imaging. Results were compared between primary central nervous system lymphoma and glioblastoma multiforme, and values of P central nervous system lymphoma and 23 patients (59%) with glioblastoma multiforme. Absence of gross intratumoral hemorrhage could not differentiate between the two disorders (P = 0.20). However, intratumoral susceptibility signal grade 1 or 2 was diagnostic of primary central nervous system lymphoma with 78.9% sensitivity and 66.7% specificity (P central nervous system lymphoma from glioblastoma multiforme. However, specificity in this study was relatively low, and primary central nervous system lymphoma cannot be excluded based solely on the presence of an intratumoral susceptibility signal.

  19. Primary orbital precursor T-cell lymphoblastic lymphoma

    DEFF Research Database (Denmark)

    Stenman, Lisa; Persson, Marta; Enlund, Fredrik

    2016-01-01

    Primary T-cell lymphoblastic lymphoma (T-LBL) in the eye region is very rare. The present study described a unique case of T-LBL involving the extraocular muscles. A 22-year-old male patient presented with a 3-week history of headache, reduced visual acuity and edema of the left eye. Clinical...... examination revealed left-sided exophthalmus, periorbital edema, chemosis, and reduced motility of the left eye. A magnetic resonance imaging scan revealed thickening of the left orbital muscles and a positron emission tomography-computed tomography scan also demonstrated activity in a subclavicular lymph....... There was no involvement of the bone marrow. Based on the clinical and histopathological findings, a diagnosis of T-LBL was made. There was no evidence of NOTCH1 mutation or rearrangements of the ETV6 and MLL genes and high-resolution array-based comparative genomic hybridization (arrayCGH) analysis revealed a normal...

  20. The role of surgery in primary central nervous system lymphomas

    Directory of Open Access Journals (Sweden)

    Juan Francisco Villalonga

    Full Text Available ABSTRACT Background Primary central nervous system lymphomas (PCNSL are infrequent. The traditional treatment of choice is chemotherapy. Complete resections have generally not been recommended, because of the risk of permanent central nervous system deficits with no proven improvement in survival. The aim of the current study was to compare survival among patients with PCNSL who underwent biopsy versus surgical resection. Methods A retrospective study was conducted on 50 patients with a confirmed diagnosis of PCNSL treated at our center from January 1994 to July 2015. Results Patients in the resection group exhibited significantly longer median survival time, relative to the biopsy group, surviving a median 31 months versus 14.5 months; p = 0.016. Conclusions In our series, patients who had surgical resection of their tumor survived a median 16.5 months longer than patients who underwent biopsy alone.

  1. Primary cardiac lymphoma: diagnostic tools and treatment challenges.

    LENUS (Irish Health Repository)

    Bambury, R

    2011-03-01

    Primary cardiac lymphoma (PCL) is a rare malignancy and the optimal treatment strategy remains uncertain. It appears to respond much better to systemic chemotherapy than to surgery and it should be considered in the differential diagnosis of all cardiac tumours before definitive management is undertaken. We report a case of this rare disorder treated successfully with a combination of rituximab and cyclophosphamide, adriamycin, vincristine and prednisolone. The patient developed recurrent unstable ventricular tachycardia (VT) post-chemotherapy secondary to extensive scarring at the tumour site. The tumour as well as the post-treatment scarring is well illustrated by cardiac magnetic resonance imaging highlighting its usefulness in this setting. An implantable cardioverter defibrillator (ICD) was placed. This is only the second case in the literature of PCL to have an ICD placed for recurrent VT. A brief literature review is included.

  2. Primary Cardiac Burkitt Lymphoma Presenting with Abdominal Pain

    Directory of Open Access Journals (Sweden)

    Dimitrios Tzachanis

    2014-01-01

    Full Text Available We describe the case of a 44-year-old woman with primary Burkitt lymphoma of the heart who presented with abdominal bloating and epigastric discomfort secondary to tamponade physiology caused by a large pericardial effusion. The pericardial fluid contained a large number of highly atypical lymphocytes with moderate basophilic cytoplasm, rare punched-out vacuoles, a vesicular nuclear chromatin, large nucleolus, and marginated chromatin that by FISH were positive for the 8;14 translocation. She had no other sites of disease. She was treated with four alternating cycles of modified CODOX-M and IVAC in combination with rituximab and remains in remission more than 5 years since diagnosis.

  3. Primary non-Hodgkin's lymphoma of breast – A rare cause of breast lump

    Directory of Open Access Journals (Sweden)

    Veena Gupta

    2017-03-01

    Full Text Available We, here, report a case of primary breast lymphoma in a 59 years old female. The diagnosis was suspected on fine needle aspiration cytology and confirmed on excision biopsy of the tumor. Histology and immunophenotyping were in accordance with non-Hodgkin's diffuse large B-cell lymphoma. The patient has been planned for adjuvant chemoradiation. The management and outcome of primary breast lymphoma and carcinoma are totally different. Early and prompt diagnosis of primary breast lymphoma is of utmost importance to avoid unnecessary mastectomies. Fine needle aspiration cytology supplemented by immuno-cytochemistry can be applied as a reliable and cost-effective tool in the early diagnosis of primary breast lymphomas, while histopathology and immunohistochemistry are conclusive.

  4. Primary intramedullary spinal cord lymphoma: a population-based study.

    Science.gov (United States)

    Yang, Wuyang; Garzon-Muvdi, Tomas; Braileanu, Maria; Porras, Jose L; Caplan, Justin M; Rong, Xiaoming; Huang, Judy; Jallo, George I

    2017-03-01

    Primary intramedullary spinal cord lymphoma (PISCL) is a rare diagnosis with poorly understood disease progression. Clarification of the factors associated with survival in PISCL patients is warranted. We conducted a population-based cohort study utilizing prospectively collected data from the Surveillance, Epidemiology, and End Results (SEER) database. Patients with histological diagnosis of primary lymphoma in spinal cord (C72.0) from 1973 to 2012 in the SEER database were included. Multivariable survival analysis between patient, lesion characteristics, and PISCL-related death was performed to adjust for confounding factors. We included 346 PISCL patients in our study. Average age was 56.5 ± 17.8 years, with 62.7% being male. Racial distribution of these patients was white (87.6%), black (8.0%), and other (4.3%). More than half (55.8%) of patients were married. The most prevalent histology of PISCL was diffuse B-cell (46.2%), and the majority (55.2%) were low stage (Ann Arbor stage I/II). Most patients (67.9%) received radiation therapy. Average survival interval of patients with PISCL-related death (n=135, 39.0%) was 27.8 months. General cumulative survival probability at 1 year, 2 years, and 5 years was 73.8%, 67.9%, and 63.1%, respectively. Multivariable accelerated failure time (AFT) regression showed follicular lymphoma (HR:0.25, P=.008) and more recent diagnosis (HR:0.96, P<.001) was positively associated with PISCL-related survival. Conversely, nonwhite race (HR:1.69, P=.046), older age (HR:1.02, P<.001), unmarried status (HR:2.14, P<.001), and higher stage (HR:1.54, P=.022) were negatively associated with survival. Age, race, marital status, tumor histology, tumor stage, and year of diagnosis were associated with survival of PISCL. While most PISCL-related deaths occur within a 1-year period, subsequent slow progression was observed after the first year of survival. © The Author(s) 2016. Published by Oxford University Press on behalf of the Society for

  5. Diagnostic microRNA profiling in cutaneous T-cell lymphoma (CTCL)

    DEFF Research Database (Denmark)

    Ralfkiaer, Ulrik; Hagedorn, Peter; Bangsgaard, Nannie

    2011-01-01

    from benign inflammation, we studied miRNA expression levels in 198 patients with CTCL, peripheral T-cell lymphoma (PTL), and benign skin diseases (psoriasis and dermatitis). Using microarrays, we show that the most induced (miR-326, miR-663b, and miR-711) and repressed (miR-203 and miR-205) mi...

  6. Anaplastic Large Cell Lymphoma

    Science.gov (United States)

    ... Non-Hodgkin Lymphoma Peripheral T-Cell Lymphoma Primary Central Nervous System Lymphoma T-Cell Lymphoma Transformed Mycosis Fungoides Waldenstrom Macroglobulinemia Young Adult Lymphoma Overview Treatment Options Relapsed/Refractory Long-term ...

  7. Mantle Cell Lymphoma

    Science.gov (United States)

    ... Non-Hodgkin Lymphoma Peripheral T-Cell Lymphoma Primary Central Nervous System Lymphoma T-Cell Lymphoma Transformed Mycosis Fungoides Waldenstrom Macroglobulinemia Young Adult Lymphoma Overview Treatment Options Relapsed/Refractory Long-term ...

  8. Marginal Zone Lymphoma

    Science.gov (United States)

    ... Non-Hodgkin Lymphoma Peripheral T-Cell Lymphoma Primary Central Nervous System Lymphoma T-Cell Lymphoma Transformed Mycosis Fungoides Waldenstrom Macroglobulinemia Young Adult Lymphoma Overview Treatment Options Relapsed/Refractory Long-term ...

  9. A Case of Primary Uterina Lymphoma Presenting with Bleeding, Pelvic Pain, and Dysmenorrhea

    Directory of Open Access Journals (Sweden)

    Lilian Yukari Miura

    2018-01-01

    Full Text Available Primary non-Hodgkin’s lymphoma (NHL can arise from lymphatic cells located in solid organs (extranodal and it accounts for 25 to 35% of all NHL. Primary lymphoma on the female genital tract (PLFGT is a rare disease, comprising 0.2 to 1.1% of all extranodal lymphomas in the female population. In this paper, the authors report an extremely rare case of a 48-year-old woman who exhibited an abnormal uterine bleeding, pelvic pain, and dysmenorrhea history. The transvaginal ultrasound showed an anteverted uterus measuring 153 cm3 in volume, with intramural leiomyomas. She underwent a total laparoscopic hysterectomy with bilateral salpingectomy. The histologic evaluation of the specimen showed a follicular lymphoma with diffuse pattern in the endometrium. This report illustrates the difficulty in the diagnosis of primary lymphomas of the female genital tract.

  10. Primary mucosa-associated lymphoid tissue lymphoma of the breast: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Park, Jong Heon; Kim, Dae Bong; Shin, Mi Kyung; Jang, Suk Ki; Kang, Su Min; Ahn, In Oak [Bundang Jesaeng General Hospital, Seongnam (Korea, Republic of)

    2007-11-15

    A primary mucosa-associated lymphoid tissue lymphoma (MALT lymphoma) of the breast is extremely rare. We report a case of a MALT lymphoma of the breast that presented as a palpable left breast mass in a 37-year-old woman. A physical examination revealed a large firm, and fixed mass in the left inner breast. Mammograms showed a large, isodense mass in the lower inner quadrant of the left breast and an enlarged lymph node in the axilla. A sonogram demonstrated a 5 cm sized, oval, circumscribed, and heterogeneously hypoechoic mass with posterior acoustic enhancement. A surgical biopsy was performed, and the pathology revealed a MALT lymphoma.

  11. Primary mucosa-associated lymphoid tissue lymphoma of the breast: a case report

    International Nuclear Information System (INIS)

    Park, Jong Heon; Kim, Dae Bong; Shin, Mi Kyung; Jang, Suk Ki; Kang, Su Min; Ahn, In Oak

    2007-01-01

    A primary mucosa-associated lymphoid tissue lymphoma (MALT lymphoma) of the breast is extremely rare. We report a case of a MALT lymphoma of the breast that presented as a palpable left breast mass in a 37-year-old woman. A physical examination revealed a large firm, and fixed mass in the left inner breast. Mammograms showed a large, isodense mass in the lower inner quadrant of the left breast and an enlarged lymph node in the axilla. A sonogram demonstrated a 5 cm sized, oval, circumscribed, and heterogeneously hypoechoic mass with posterior acoustic enhancement. A surgical biopsy was performed, and the pathology revealed a MALT lymphoma

  12. Primary thymic extranodal marginal zone B cell lymphoma as an incidental finding in a Caucasian woman

    DEFF Research Database (Denmark)

    Krogh Petersen, Jeanette; Larsen, Thomas Stauffer; Møller, Michael Boe

    2015-01-01

    Primary thymic extranodal marginal zone B cell lymphoma (TML) is an extremely rare lymphoma strongly associated with autoimmune disease. We report an exceedingly rare case of TML found in a non-Asian population. TML was found incidentally in a 60-year-old Caucasian woman with a short history...

  13. Helicobacter pylori and primary gastric lymphoma. A histopathologic and immunohistochemical analysis of 237 patients.

    Science.gov (United States)

    Nakamura, S; Yao, T; Aoyagi, K; Iida, M; Fujishima, M; Tsuneyoshi, M

    1997-01-01

    Few previous articles have analyzed the relation between infection with Helicobacter pylori (H. pylori) and primary gastric lymphoma in a large number of patients. Resected and biopsied specimens from 237 patients with primary gastric lymphoma were investigated for H. pylori using hematoxylin and eosin stain, modified Giemsa stain, and immunohistochemistry. These specimens were compared with specimens from 29 patients with chronic active gastritis, 33 with peptic ulcers, and 41 with gastric carcinoma. H. pylori was detected in 145 of 237 patients (61%) with gastric lymphoma. The frequency of H. pylori positivity was higher in patients with lymphoma restricted to the mucosa and submucosa (76%) than in those with lymphoma invading beyond the submucosa (48%) (P gastritis (100%) (P gastritis and peptic ulcer. The H. pylori grading score for patients with lymphoma (0.9 +/- 1.0) was lower than for those with chronic active gastritis (1.9 +/- 0.8) (P < 0.001), peptic ulcers (2.2 +/- 1.0) (P < 0.001), or gastric carcinoma (1.2 +/- 1.1) (P < 0.05). These results suggest that H. pylori is more likely to be associated with early states of primary gastric lymphoma than with advanced states. Thus, H. pylori may disappear during the progression of primary gastric lymphoma.

  14. Treatment Strategies in Primary Vitreoretinal Lymphoma A 17-Center European Collaborative Study

    NARCIS (Netherlands)

    Riemens, Anjo; Bromberg, Jacoline; Touitou, Valerie; Sobolewska, Bianka; Missotten, Tom; Baarsma, Seerp; Hoyng, Carel; Cordero-Coma, Miguel; Tomkins-Netzer, Oren; Rozalski, Anna; Tugal-Tutkun, Ilknur; Guex-Crosier, Yan; Los, Leonoor I.; Bollemeijer, Jan Geert; Nolan, Andrew; Pawade, Joya; Willermain, Francois; Bodaghi, Bahram; ten Dam-van Loon, Ninette; Dick, Andrew; Zierhut, Manfred; Lightman, Susan; Mackensen, Friederike; Moulin, Alexandre; Erckens, Roel; Wensing, Barbara; le Hoang, Phuc; Lokhorst, Henk; Rothova, Aniki

    IMPORTANCE The best treatment option for primary vitreoretinal lymphoma (PVRL) without signs of central nervous system lymphoma (CNSL) involvement determined on magnetic resonance imaging or in cerebrospinal fluid is unknown. OBJECTIVE To evaluate the outcomes of treatment regimens used for PVRL in

  15. Primary Diffuse Large Cell Lymphoma of the Bladder: Case Report and Literature Review

    Directory of Open Access Journals (Sweden)

    Mansour Ansari

    2017-01-01

    Full Text Available Most bladder tumors are epithelial in origin. Nonepithelial cancers are rarely located in the bladder. Sarcomas are the most common malignancies among nonepithelial cancers. Primary bladder lymphoma is rare and mostly low grade. Here, we have reported a case of diffuse large cell lymphoma of the bladder. The patient, a 64-year-old man, had urinary frequency for 18 months. Abdominal sonography indicated a thick bladder wall and transurethral biopsy showed diffuse large cell lymphoma. Immunohistochemistry (IHC results showed that the tumor was positive for CD20, CD45, and Pax-5 and negative for BCL-2, cytokeratin, and S100. He had a normal bone marrow biopsy, abdominal, pelvic and chest CT scans. He had no B symptoms. The patient received 6 cycles of R-CHOP followed by radiotherapy (36 Gy to the pelvis. Six months after treatment, the patient is well and has returned to work. We have searched PubMed for primary diffuse large cell lymphoma. Primary diffuse large cell lymphoma of the bladder is best treated according to treatment for diffuse large cell lymphoma of other sites, which includes chemotherapy and radiotherapy. As seen in our review, primary diffuse large cell lymphoma of the bladder has a similar clinical course to diffuse large cell lymphoma of other sites.

  16. False Negative Fine Needle Aspiration Biopsy Results in Primary Thyroid Lymphoma

    Energy Technology Data Exchange (ETDEWEB)

    Kim, In Joong; Kim, Eun Kyung; Koh, Myoung Ju; Kwak, Jin Young; Moon, Hee Jung [Yonsei University College of Medicine, Seoul (Korea, Republic of)

    2012-06-15

    Ultrasonography-guided fine needle aspiration biopsy (US-FNA) is one of the methods used to diagnose thyroid lymphoma, but it has a relatively high false-negative rate. The authors report a case of a primary thyroid lymphoma associated with underlying lymphocytic thyroiditis that was initially misdiagnosed as lymphocytic thyroiditis based on US-FNA findings

  17. MRI findings of primary intracranial lymphoma on immunologically normal patients

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Ho Chul; Chang, Kee Hyun; Han, Moon Hee; Choi, Choong Gon [Seoul National University College of Medicine, Seoul (Korea, Republic of); Cha, Sang Hoon [Chonbuk National University College of Medicine, Chungju (Korea, Republic of)

    1993-07-15

    Magnetic resonance (MR) images of 14 consecutive patients with pathologically proven primary intracranial lymphoma were reviewed. All patients had a brain MR imaging before any treatment and were immunologically competent. MR images were acquired using 2.0 T (n=6) or 0.5 T (n=8) machine. The MR images were reviewed regarding the location, multiplicity, size, signal intensity, margin, shape, and the extent of surrounding edema of the lesions. Seven patients had multi lesions, 2 to 4 in number. A total of 26 lesions was found; 25 were parenchymal lesions and one was dural lesion. The location of tumor was either central (n=11) or peripheral (n=14). The size of tumor was variable raging from 0.6 cm to 6.0 cm in its maximal diameter. The tumors were isointense (n=19) or hypointense (n=7) relative to gray matter om T1-weighted images, isointense (n=24) or hypointense (n=2) on proton-density weighted images, and isointense (n=21) or hypointense (n=5) on T2-weighted images. On gadolinium-enhanced T1- weighted images of 13 patients, strong enhancement was seen in 22 of 23 lesion. Nineteen lesions showed smooth, well-defined margin, whereas remaining 7 lesions showed irregular, ill-defined margin. The shape of the tumor was diverse; round of ovoid (n=15), lobulated (n=9), or short linear (n=2). These results suggest that one should consider the diagnosis of CNS lymphoma in case with single or multiple masses that abut CSF space and show iso- or similar intensity to gray matter with strong enhancement on MR images.

  18. A case of cutaneous scleroderma with primary sclerosing cholangitis

    Directory of Open Access Journals (Sweden)

    H P Nandeesh

    2014-01-01

    Full Text Available Sclerosing cholangitis comprises of a spectrum of cholestatic conditions that are characterized by patchy fibrosis, inflammation and destruction of intra hepatic and extrahepatic ducts. We report a case of a 42 year old woman who presented with darkening of skin with yellowish discolouration of the eyes. Clinical examination revealed icterus, taut skin with hepatosplenomegaly. Liver function tests showed a cholestatic picture. Skin biopsy showed features of cutaneous scleroderma. MRCP and Liver biopsy was suggestive of sclerosing cholangitis.

  19. Primary cutaneous Nocardia brasiliensis infection isolated in an immunosuppressed patient: a case report.

    Science.gov (United States)

    Lai, Kimberly W; Brodell, Lindsey A; Lambert, Emily; Menegus, Marilyn; Scott, Glynis A; Tu, John H

    2012-02-01

    Cutaneous nocardiosis is a rare infection that may manifest as a superficial skin lesion, lymphocutaneous infection, mycetoma, or diffuse cutaneous infection from a disseminated systemic infection. We report a case of a 65-year-old immunocompromised man with persistent primary cutaneous Nocardia brasiliensis infection following a motor vehicle collision. A high degree of suspicion is needed to diagnose Nocardia infection because of its resemblance to other bacterial infections. Nocardiosis should be included in the differential diagnosis of chronic cutaneous infections, especially when the response to antibiotics is inadequate or when the patient is immunocompromised. Because Nocardia may take several weeks to grow in standard bacterial culture media, laboratories should be notified of the suspicion so that culture plates are held for longer time periods. Long-term therapy, usually with sulfonamides, often is necessary.

  20. Non-Hodgkin's lymphoma presenting as a primary bladder tumor: a case report

    Directory of Open Access Journals (Sweden)

    Molinos-Castro Sonia

    2010-04-01

    Full Text Available Abstract Introduction Primary lymphoma of the bladder represents 0.2% of all bladder malignancies. Secondary involvement of the bladder by malignant lymphoma occurs in 10% to 50% of cases. Most lymphomas of the bladder are non-Hodgkin's lymphomas of the B-cell type, with preponderance among women. The impact of positron emission tomography (PET on tumor staging has recently become very important due to its use in the study of diagnosis extension and individual therapy design. Case presentation We report the case of a 79-year-old Caucasian man with intermittent haematuria as the presenting symptom of non-Hodgkin's lymphoma of the bladder. He was first diagnosed with primary lymphoma of the bladder using the current staging method, but a positron emission tomography study subsequently revealed that he instead had a secondary involvement of the bladder. Conclusion The staging of non-Hodgkin's lymphomas, which is useful in order to plan accurate therapy, has been changing since the introduction of positron emission tomography scanning. Primary lymphomas of the bladder, although very rare, may be even more uncommon when this imaging technique is used to assess the extension of the disease. Although the interpretation of this technique has some limitations that should be taken into account, the extensive use of positron emission tomography should nonetheless help improve the diagnosis of this disease.

  1. Vorinostat, Rituximab, Ifosfamide, Carboplatin, and Etoposide in Treating Patients With Relapsed or Refractory Lymphoma or Previously Untreated T-Cell Non-Hodgkin Lymphoma or Mantle Cell Lymphoma

    Science.gov (United States)

    2017-04-17

    Adult Nasal Type Extranodal NK/T-cell Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Contiguous Stage II Mantle Cell Lymphoma; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Nodal Marginal Zone B-cell Lymphoma; Noncontiguous Stage II Mantle Cell Lymphoma; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides/Sezary Syndrome; Recurrent Small Lymphocytic Lymphoma; Splenic Marginal Zone Lymphoma; Stage I Cutaneous T-cell Non-Hodgkin Lymphoma; Stage I Mantle Cell Lymphoma; Stage I Mycosis Fungoides/Sezary Syndrome; Stage II Cutaneous T-cell Non-Hodgkin Lymphoma; Stage II Mycosis Fungoides/Sezary Syndrome; Stage III Cutaneous T-cell Non-Hodgkin Lymphoma; Stage III Mantle Cell Lymphoma; Stage III Mycosis Fungoides/Sezary Syndrome; Stage IV Cutaneous T-cell Non-Hodgkin Lymphoma; Stage IV Mantle Cell Lymphoma; Stage IV Mycosis Fungoides/Sezary Syndrome; Waldenström Macroglobulinemia

  2. Primary Effusion Lymphoma without an Effusion: A Rare Case of Solid Extracavitary Variant of Primary Effusion Lymphoma in an HIV-Positive Patient

    Directory of Open Access Journals (Sweden)

    Hamza Hashmi

    2018-01-01

    Full Text Available Primary effusion lymphoma (PEL is a unique form of non-Hodgkin lymphoma, usually seen in severely immunocompromised, HIV-positive patients. PEL is related to human herpesvirus-8 (HHV-8 infection, and it usually presents as a lymphomatous body cavity effusion in the absence of a solid tumor mass. There have been very few case reports of HIV-positive patients with HHV-8-positive solid tissue lymphomas not associated with an effusion (a solid variant of PEL. In the absence of effusion, establishing an accurate diagnosis can be challenging, and a careful review of morphology, immunophenotype, and presence of HHV-8 is necessary to differentiate from other subtypes of non-Hodgkin lymphoma. Treatment involves intensive chemotherapy, and prognosis is usually poor. We present a rare case of a PEL variant in an HIV-positive patient who presented with extensive lymphadenopathy without any associated effusions.

  3. Primary Malignant Lymphoma of the Uterus: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Margriet Samama

    2011-12-01

    Full Text Available Primary malignant lymphomas in the female genital tract are rare. Most cases are non-Hodgkin lymphomas of which diffuse large B-cell lymphomas are most commonly seen. Symptoms are associated with other, more common diseases; therefore, a doctors’ delay can be expected. In this case a woman presented with complaints of urinary obstruction due to a large tumour in the pelvic area. A laparotomy was performed. A very large tumour of the uterus was found with adherence to the pelvic wall and urinary bladder. Diagnostic histological examination showed a diffuse large B-cell lymphoma. Treatment with R-CHOP chemotherapy was started shortly after the operation. The treatment of patients with a primary malignant lymphoma of the uterus should be individualized with the following options: surgery, radiotherapy and/or chemotherapy.

  4. Localized Orbital Mucosa-Associated Lymphoma Tissue Lymphoma Managed With Primary Radiation Therapy: Efficacy and Toxicity

    Energy Technology Data Exchange (ETDEWEB)

    Goda, Jayant Sastri [Radiation Medicine Program, Princess Margaret Hospital, University of Toronto, Toronto, Ontario (Canada); Le, Lisa W. [Biostatistics, Princess Margaret Hospital, University of Toronto, Toronto, Ontario (Canada); Lapperriere, Normand J.; Millar, Barbara-Ann; Payne, David; Gospodarowicz, Mary K.; Wells, Woodrow; Hodgson, David C.; Sun, Alexander [Radiation Medicine Program, Princess Margaret Hospital, University of Toronto, Toronto, Ontario (Canada); Simpson, Rand [Ocular Oncology, Princess Margaret Hospital, University of Toronto, Toronto, Ontario (Canada); Tsang, Richard W., E-mail: richard.tsang@rmp.uhn.on.ca [Radiation Medicine Program, Princess Margaret Hospital, University of Toronto, Toronto, Ontario (Canada)

    2011-11-15

    Purpose: To evaluate the clinical outcomes and late effects of radiation therapy (RT) in localized primary orbital mucosa-associated lymphoma tissue (MALT) lymphoma (POML). Methods and Materials: From 1989 to 2007, 89 patients with Stage IE POML received RT. The median age was 56 years old. Sites involved conjunctiva (59 patients [66%]), lacrimal gland (20 patients [23%]), and soft tissue (10 patients [11%]). Megavoltage beam(s) was used in 91%, electrons in 7%, and orthovoltage in 2% of cases. The dose given was 25 Gy in 97% and 30 Gy in 3% of patients. Lens shielding was possible in 57% of patients. Results: The median follow-up was 5.9 years. Complete response or unconfirmed complete response was seen in 88 patients (99%). Relapse occurred in 22 patients (25%). First relapse sites were local (2 patients [9%]), in the contralateral orbit (5 patients [23%]), and distant (15 patients [68%]). The 7-year overall survival (OS), cause-specific survival (CSS), relapse-free survival (RFS), and local control (LC) rates were 91%, 96%, 64%, and 97%, respectively. Radiation-related late sequelae were documented in 40 patients (45%). Cataracts were observed in 22 patients (Grade 1 in 2 patients; Grade 3 in 20 patients). The incidence of Grade 3 cataract at 7 years was 25%. Other late sequelae (n = 28) were dry eye(s) (22 patients [Grade 1 in 14 patients; Grade 2 in 2 patients; Grade 3 in 2 patients; n/s in 4 patients), keratitis (3 patients), macular degeneration/cystoid edema (2 patients), and vitreous detachment (1 patient). Five patients developed Grade 3 noncataract late effects. Lens shielding reduced the incidence of Grade 3 cataract and all Grade {>=}2 late sequelae. Seventeen patients (16 with cataracts) underwent surgery; 23 patients were treated conservatively. The outcome for managing late effects was generally successful, with 30 patients completely improved, and 9 patients with persisting late sequelae (10%). Conclusions: POML responds favorably to moderate doses

  5. Primary breast lymphoma: a consideration in an HIV patient when a mass is discovered by screening mammography: a case report.

    Science.gov (United States)

    Akinwande, Olagoke K; Paley, Robert

    2008-12-11

    Primary Breast lymphoma is a rare lesion that has been reported in patients without HIV. However, Primary Breast lymphoma occurring in a patient with HIV has rarely been reported despite the fact that HIV infection is known to increase the propensity to develop certain types of lymphoma. We report a case of an HIV patient with breast lymphoma that was discovered by screening mammography while presenting our argument for more cautionary management in this patient population.

  6. Primary cilium depletion typifies cutaneous melanoma in situ and malignant melanoma.

    Directory of Open Access Journals (Sweden)

    Jinah Kim

    Full Text Available Cutaneous melanoma is a lethal malignancy that arises spontaneously or via in situ precursor neoplasms. While melanoma in situ and locally invasive malignant melanoma can be cured surgically, these lesions can sometimes be difficult to distinguish from melanocytic nevi. Thus, the identification of histolopathologic or molecular features that distinguish these biologically distinct lesions would represent an important advance. To this end, we determined the abundance of melanocytic primary cilia in a series of 62 cases composed of typical cutaneous melanocytic nevi, melanoma in situ, invasive melanoma, and metastatic melanoma. Primary cilia are sensory organelles that modulate developmental and adaptive signaling and notably, are substantially depleted from the neoplastic epithelium of pancreatic carcinoma at a stage equivalent to melanoma in situ. In this series, we find that while nearly all melanocytes in 22 melanocytic nevi possessed a primary cilium, a near-complete loss of this organelle was observed in 16 cases of melanoma in situ, in 16 unequivocal primary invasive melanomas, and in 8 metastatic tumors, each associated with a cutaneous primary lesion. These findings suggest that the primary cilium may be used to segregate cutaneous invasive melanoma and melanoma in situ from melanocytic nevi. Moreover, they place the loss of an organelle known to regulate oncogenic signaling at an early stage of melanoma development.

  7. Malignant T cells exhibit CD45 resistant Stat 3 activation and proliferation in cutaneous T cell lymphoma

    DEFF Research Database (Denmark)

    Krejsgaard, T; Helvad, Rikke; Ralfkiær, Elisabeth

    2010-01-01

    CD45 is a protein tyrosine phosphatase, which is well-known for regulating antigen receptor signalling in T and B cells via its effect on Src kinases. It has recently been shown that CD45 can also dephosphorylate Janus kinases (Jaks) and thereby regulate Signal transducer and activator of transcr......CD45 is a protein tyrosine phosphatase, which is well-known for regulating antigen receptor signalling in T and B cells via its effect on Src kinases. It has recently been shown that CD45 can also dephosphorylate Janus kinases (Jaks) and thereby regulate Signal transducer and activator...... of transcription (Stat) activation and cytokine-induced proliferation in lymphocytes. Consequently, CD45 dysregulation could be implicated in aberrant Jak/Stat activation and proliferation in lymphoproliferative diseases. Despite high expression of the CD45 ligand, Galectin-1, in skin lesions from cutaneous T......-cell lymphoma (CTCL), the malignant T cells exhibit constitutive activation of the Jak3/Stat3 signalling pathway and uncontrolled proliferation. We show that CD45 expression is down-regulated on malignant T cells when compared to non-malignant T cells established from CTCL skin lesions. Moreover, CD45 cross...

  8. Primary breast lymphoma presenting as non-healing axillary abscess.

    Science.gov (United States)

    Anele, Chukwuemeka; Phan, Yih Chyn; Wong, Suanne; Poddar, Anil

    2015-10-07

    A 67-year-old woman with non-insulin dependent diabetes mellitus with a history consistent with a right axillary abscess, presented to her general practitioner (GP). A diagnosis of folliculitis was made and the GP started a course of flucloxacillin. Despite antibiotics, the patient's symptoms worsened and the abscess increased in size. This prompted her GP to perform an incision and drainage procedure of the abscess. The practice nurse subsequently oversaw the follow-up care of the wound. Two months after the incision and drainage, and after regular wound dressing, the patient was referred to the acute surgical team with a complicated, non-healing right axillary abscess cavity and associated generalised right breast cellulitis. There was no history of breast symptoms prior to the onset of the axillary abscess. The patient underwent wound debridement, washout and application of negative pressure vacuum therapy. Biopsies revealed primary breast lymphoma (B-cell). She underwent radical chemotherapy and is currently in remission. 2015 BMJ Publishing Group Ltd.

  9. Current Management of Primary Central Nervous System Lymphoma

    International Nuclear Information System (INIS)

    Schultz, Christopher J.; Bovi, Joseph

    2010-01-01

    Primary central nervous cell lymphoma (PCNSL) is an uncommon neoplasm of the brain, leptomeninges, and rarely the spinal cord. Initially thought to be characteristically associated with congenital, iatrogenic, or acquired immunosuppression, PCNSL is now recognized with increasing frequency in immunocompetent individuals. The role of surgery is limited to establishing diagnosis, as PCNSL is often multifocal with a propensity to involve the subarachnoid space. A whole-brain radiation volume has empirically been used to adequately address the multifocal tumor frequently encountered at the time of PCNSL diagnosis. Despite high rates of response after whole-brain radiotherapy (WBRT), rapid recurrence is common and long-term survival is the exception. Chemotherapy alone or in combination with WBRT has more recently become the treatment of choice. Most effective regimens contain high-dose methotrexate and or other agents that are capable of penetrating the blood-brain barrier. High response rates and improved survival with the use of chemotherapy has led to treatment strategies that defer or eliminate WBRT in hopes of lessening the risk of neurotoxicity attributed to WBRT. Unfortunately, elimination of WBRT is also associated with a higher rate of relapse. Combined chemotherapy and WBRT regimens are now being explored that use lower total doses of radiation and altered fractionation schedules with the aim of maintaining high rates of tumor control while minimizing neurotoxicity. Pretreatment, multifactor prognostic indices have recently been described that may allow selection of treatment regimens that strike an appropriate balance of risk and benefit for the individual PCNSL patient.

  10. Primary cutaneous mucormycosis caused by Mucor irregularis in an elderly person.

    Science.gov (United States)

    Yamaguchi, Sayaka; Okubo, Yuko; Katano, Azusa; Sano, Ayako; Uezato, Hiroshi; Takahashi, Kenzo

    2015-02-01

    Primary cutaneous mucormycosis is a rare but often lethal severe fungal infection, which usually occurs in immunocompromised patients. We report a case of primary cutaneous mucormycosis caused by Mucor irregularis in an elderly patient. Seven months after the surgical dissection of the involved skin, cutaneous mucormycosis recurred at the peripheral edge of the skin graft. Shortly subsequent to the administration of liposomal amphotericin B, the remaining skin lesion was excised again. M. irregularis is rarely but increasingly reported as a cause of mucormycosis in immunocompetent individuals, especially in Asian farmers. M. irregularis may be largely disseminated in the soils of Asia and thus the trivial trauma at the time of farm work may be a trigger for the onset. These cases tend to leave severe cosmetic damage even in healthy individuals, although the vital prognosis is not affected. © 2014 Japanese Dermatological Association.

  11. Treatment of primary parotid non-Hodgkin's lymphoma: an analysis of 29 patients

    International Nuclear Information System (INIS)

    Gu Wendong; Feng Yan

    2003-01-01

    Objective: To analyze the clinical characteristics, treatment and prognosis of primary parotid non-Hodgkin's lymphoma. Methods: From March 1988 to February 2001, twenty-nine patients with primary parotid non-Hodgkin's lymphoma treated in our hospital were retrospectively analyzed. The data were analyzed according to the following factors: sex, age, stage, pathologic classification, chemotherapy given or not, cycles of chemotherapy, radiotherapy given or not, and the dose at the parotid. Kaplan-Meier method and Log-rank method were used in the statistic analysis. Results: The overall 5-year and 10-year survival rates were 73.3% and 51.0%. Stage and pathologic classification were prognostic factors in our statistic analysis. The 5-year survival rates were 81.6% and 25.0% for early stage (I E + II E) and advanced stage (III E + IV E) patients, with the difference significant (P<0.01). The 5-year survival rate for patients with the pathologic classification of mucosa associated lymphoid tissue (MALT) was 100% as compared to 42.2% for patients with diffused large B cell lymphoma, with the difference also significant (P<0.05). Conclusions: The prognosis of primary parotid non-Hodgkin's lymphoma is satisfactory. Surgery should only be used as a diagnostic method. Radiotherapy should be the first choice for patients with MALT lymphoma and stage I E and II E follicular lymphoma, but comprehensive treatment including chemotherapy is necessary to the diffuse large B cell lymphoma

  12. Identification of genuine primary pulmonary NK cell lymphoma via clinicopathologic observation and clonality assay.

    Science.gov (United States)

    Gong, Li; Wei, Long-Xiao; Huang, Gao-Sheng; Zhang, Wen-Dong; Wang, Lu; Zhu, Shao-Jun; Han, Xiu-Juan; Yao, Li; Lan, Miao; Li, Yan-Hong; Zhang, Wei

    2013-08-19

    Extranodal natural killer (NK)/T-cell lymphoma, nasal type, is an uncommon lymphoma associated with the Epstein-Barr virus (EBV). It most commonly involves the nasal cavity and upper respiratory tract. Primary pulmonary NK/T cell lymphoma is extremely rare. If a patient with a NK or T-cell tumor has an unusual reaction to treatment or an unusual prognosis, it is wise to differentiate NK from T-cell tumors. The clinicopathologic characteristics, immunophenotype, EBV in situ hybridization, and T cell receptor (TCR) gene rearrangement of primary pulmonary NK cell lymphoma from a 73-year-old Chinese woman were investigated and the clonal status was determined using female X-chromosomal inactivation mosaicism and polymorphisms at the phosphoglycerate kinase (PGK) gene. The lesion showed the typical histopathologic characteristics and immunohistochemical features of NK/T cell lymphoma. However, the sample was negative for TCR gene rearrangement. A clonality assay demonstrated that the lesion was monoclonal. It is concluded that this is the first recorded case of genuine primary pulmonary NK cell lymphoma. The purpose of the present work is to recommend that pathologists carefully investigate the whole lesion to reduce the likelihood that primary pulmonary NK cell lymphoma will be misdiagnosed as an infectious lesion. In addition, TCR gene rearrangement and clonal analysis, which is based on female X-chromosomal inactivation mosaicism and polymorphisms at PGK and androgen receptor (AR) loci, were found to play important roles in differentiating NK cell lymphoma from T cell lymphoma. The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/5205300349457729.

  13. Cutaneous Pseudolymphomas.

    Science.gov (United States)

    Romero-Pérez, D; Blanes Martínez, M; Encabo-Durán, B

    2016-10-01

    The term cutaneous pseudolymphoma refers to benign reactive lymphoid proliferations in the skin that simulate cutaneous lymphomas. It is a purely descriptive term that encompasses various reactive conditions with a varied etiology, pathogenesis, clinical presentation, histology, and behavior. We present a review of the different types of cutaneous pseudolymphoma. To reach a correct diagnosis, it is necessary to contrast clinical, histologic, immunophenotypic, and molecular findings. Even with these data, in some cases only the clinical course will confirm the diagnosis, making follow-up essential. Copyright © 2016 AEDV. Publicado por Elsevier España, S.L.U. All rights reserved.

  14. Craniotomy and Survival for Primary Central Nervous System Lymphoma.

    Science.gov (United States)

    Rae, Ali I; Mehta, Amol; Cloney, Michael; Kinslow, Connor J; Wang, Tony J C; Bhagat, Govind; Canoll, Peter D; Zanazzi, George J; Sisti, Michael B; Sheth, Sameer A; Connolly, E Sander; McKhann, Guy M; Bruce, Jeffrey N; Iwamoto, Fabio M; Sonabend, Adam M

    2018-04-04

    Cytoreductive surgery is considered controversial for primary central nervous system lymphoma (PCNSL). To investigate survival following craniotomy or biopsy for PCNSL. The National Cancer Database-Participant User File (NCDB, n = 8936), Surveillance, Epidemiology, and End Results Program (SEER, n = 4636), and an institutional series (IS, n = 132) were used. We retrospectively investigated the relationship between craniotomy, prognostic factors, and survival for PCNSL using case-control design.  In NCDB, craniotomy was associated with increased median survival over biopsy (19.5 vs 11.0 mo), independent of subsequent radiation and chemotherapy (hazard ratio [HR] 0.80, P < .001). We found a similar trend with survival for craniotomy vs biopsy in the IS (HR 0.68, P = .15). In SEER, gross total resection was associated with increased median survival over biopsy (29 vs 10 mo, HR 0.68, P < .001). The survival benefit associated with craniotomy was greater within recursive partitioning analysis (RPA) class 1 group in NCDB (95.1 vs 29.1 mo, HR 0.66, P < .001), but was smaller for RPA 2-3 (14.9 vs 10.0 mo, HR 0.86, P < .001). A surgical risk category (RC) considering lesion location and number, age, and frailty was developed. Craniotomy was associated with increased survival vs biopsy for patients with low RC (133.4 vs 41.0 mo, HR 0.33, P = .01), but not high RC in the IS. Craniotomy is associated with increased survival over biopsy for PCNSL in 3 retrospective datasets. Prospective studies are necessary to adequately evaluate this relationship. Such studies should evaluate patients most likely to benefit from cytoreductive surgery, ie, those with favorable RPA and RC.

  15. Primary Diffuse Large B-Cell Lymphoma of the Liver in a Patient with Sjogren Syndrome

    Directory of Open Access Journals (Sweden)

    Vadim Gorodetskiy

    2016-01-01

    Full Text Available Sjögren’s syndrome (SS has the highest incidence of malignant lymphoproliferative disorders transformation among autoimmune diseases. We present a case of extranodal high grade lymphoma of the liver in a 52-year-old patient with long history of SS. Lymphoma manifested with sharp significant pain in the right hypochondrium, weakness, and profuse night sweats. Contrast-enhanced computed tomography scan (CT-scan of the abdomen revealed multiple low density foci with homogeneous structure and clear contours in both lobes of the liver. Histologically, proliferation of medium sized lymphoma cells with round-oval and slightly irregular nuclei with fine chromatin was shown. Immunohistochemical and molecular features of the tumors allowed diagnosis of diffuse large B-cell lymphoma (DLBCL. To exclude secondary liver lesion by non-Hodgkin lymphoma, chest and small pelvis CT-scan, endoscopy of upper and lower gastrointestinal tract and study of bone marrow were performed. After 8 cycles of R-CHOP chemotherapy (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone, the complete remission was achieved, which persists after 45 months of follow-up. Primary hepatic lymphomas are extremely rare, and previously only low-grade hepatic lymphomas have been described in SS. To our knowledge, the patient described here represents the first reported case of DLBCL with primary liver involvement in SS.

  16. A Case of Successful Remission of Extensive Primary Gastric Diffuse Large B Cell Lymphoma: Radiologic, Endoscopic and Pathologic Evidence

    Directory of Open Access Journals (Sweden)

    Mike M. Bismar

    2014-04-01

    Full Text Available Though rare amongst stomach neoplasms, primary gastric diffuse large B cell lymphoma is one of the commonest extranodal non-Hodgkin lymphomas. If left untreated, it can have a devastating progression and life-threatening consequences. We present the case of a successfully treated large antral ulcer confirmed to be large B cell lymphoma as evidenced by radiologic, endoscopic and histopathologic findings. A brief discussion about the types of gastric lymphoma, their Helicobacter pylori relation and therapeutic modalities follows.

  17. Patterns of care and treatment outcomes for primary thyroid lymphoma: A single institution study

    Energy Technology Data Exchange (ETDEWEB)

    Cha, Hye Jung; Kim, Jun Won; Suh, Chang Ok; Kim, Jin Seok; Cheong, June Won; Lee, Jeong Shim; Keum, Ki Chang; Lee, Chang Geol; Cho, Jae Ho [Yonsei University College of Medicine, Seoul (Korea, Republic of)

    2013-12-15

    The aim of this study was to analyze the patterns of care and treatment outcomes in patients with primary thyroid lymphoma (PTL) in a single institution. Medical records of 29 patients with PTL treated between April 1994 and February 2012 were retrospectively reviewed. Diagnosis was confirmed by biopsy (n = 17) or thyroidectomy (n = 12). Treatment modality and outcome were analyzed according to lymphoma grade. The median follow-up was 43.2 months (range, 3.8 to 220.8 months). The median age at diagnosis was 57 years (range, 21 to 83 years) and 24 (82.8%) patients were female. Twenty-five (86.2%) patients had PTL with stage IEA and IIEA. There were 8 (27.6%) patients with mucosa-associated lymphoid tissue (MALT) lymphoma and the remaining patients had high-grade lymphoma. Patients were treated with surgery (n = 2), chemotherapy (n = 7), radiotherapy (n = 3) alone, or a combination of these methods (n = 17). Treatment modalities evolved over time and a combination of modalities was preferred, especially for the treatment of high-grade lymphoma in recent years. There was no death or relapse among MALT lymphoma patients. Among high-grade lymphoma patients, 5-year overall survival (OS) and 5-year progression-free survival (PFS) were 75.6% and 73.9%, respectively. Complete remission after initial treatment was the only significant prognostic factor for OS (p = 0.037) and PFS (p = 0.003). Patients with PTL showed a favorable outcome, especially with MALT lymphoma. Radiotherapy alone for MALT lymphoma and chemotherapy followed by radiotherapy for high-grade lymphoma can be effective treatment options for PTL.

  18. Peripheral T cell lymphoma: Not otherwise specified

    Directory of Open Access Journals (Sweden)

    Anusha H Pai

    2015-01-01

    Full Text Available Peripheral T cell lymphoma (PTCL is a heterogeneous group of hematological tumors originating from mature T cells, which constitutes less than 15% of all non-Hodgkins lymphomas in adults. Primary cutaneous PTCL-not otherwise specified (NOS represent a subgroup of PTCLs with no consistent immunophenotypic, genetic or clinical features. PTCL-NOS frequently has an aggressive course with a tendency for systemic involvement, however, a well-defined therapeutic and prognostic approach has not been outlined yet. We report a case of PTCL-NOS with multiple cutaneous lesions in a young adult male with an emphasis on the treatment modality used.

  19. Treating Cutaneous T-cell Lymphoma with Highly Irregular Surfaces with Photon Irradiation Using Rice as Tissue Compensator

    Directory of Open Access Journals (Sweden)

    Lonika eMajithia

    2015-02-01

    Full Text Available Purpose: Cutaneous T-cell lymphoma (CTCL is known to have an excellent response to radiotherapy, an important treatment modality for this disease. In patients with extremity and digit involvement, the irregular surface and depth variations create difficulty in delivering a homogenous dose using electrons. We sought to evaluate photon irradiation with rice packing as tissue equivalence and determine clinical tolerance and response. Materials and Methods: Three consecutive CTCL patients with extensive lower extremity involvement including the digits were treated using external beam photon therapy with rice packing for tissue compensation. The entire foot was treated to 30-40 Gy in 2-3 Gy per fraction using 6 MV photons prescribed to the mid-plane of an indexed box filled with rice in which the foot was placed. Optically stimulated luminescence dosimeter (OSLD was used for dose measurement to determine the dose deposition to the skin surface. Treatment tolerance and response were monitored with clinical evaluation. Results: All patients tolerated the treatment without treatment breaks. Toxicities included grade 3 erythema and desquamation with resolution within 4 weeks. No late toxicities were observed. All four treated sites had partial response (PR by the end of the treatment course. All patients reported improved functionality after treatment, with less pain, drainage, or swelling. No local recurrence has been observed in these patients with a median follow-up time of 14 months. Conclusion: Tissue compensation with rice packing offers a convenient, inexpensive and reproducible method for the treatment of CTCL with highly irregular surfaces.

  20. Human anti-CCR4 minibody gene transfer for the treatment of cutaneous T-cell lymphoma.

    Directory of Open Access Journals (Sweden)

    Thomas Han

    Full Text Available Although several therapeutic options have become available for patients with Cutaneous T-cell Lymphoma (CTCL, no therapy has been curative. Recent studies have demonstrated that CTCL cells overexpress the CC chemokine receptor 4 (CCR4.In this study, a xenograft model of CTCL was established and a recombinant adeno-associated viral serotype 8 (AAV8 vector expressing a humanized single-chain variable fragment (scFv-Fc fusion (scFvFc or "minibody" of anti-CCR4 monoclonal antibody (mAb h1567 was evaluated for curative treatment. Human CCR4+ tumor-bearing mice treated once with intravenous infusion of AAV8 virions encoding the h1567 (AAV8-h1567 minibody showed anti-tumor activity in vivo and increased survival. The AAV8-h1567 minibody notably increased the number of tumor-infiltrating Ly-6G+ FcγRIIIa(CD16A+ murine neutrophils in the tumor xenografts over that of AAV8-control minibody treated mice. Furthermore, in CCR4+ tumor-bearing mice co-treated with AAV8-h1567 minibody and infused with human peripheral blood mononuclear cells (PBMCs, marked tumor infiltration of human CD16A+ CD56+ NK cells was observed. The h1567 minibody also induced in vitro ADCC activity through both mouse neutrophils and human NK cells.Overall, our data demonstrate that the in vivo anti-tumor activity of h1567 minibody is mediated, at least in part, through CD16A+ immune effector cell ADCC mechanisms. These data further demonstrate the utility of the AAV-minibody gene transfer system in the rapid evaluation of candidate anti-tumor mAbs and the potency of h1567 as a potential novel therapy for CTCL.

  1. An association between newly diagnosed cutaneous T cell lymphoma and prior impetigo: a nested case-control study.

    Science.gov (United States)

    Boursi, Ben; Haynes, Kevin; Mamtani, Ronac; Yang, Yu-Xiao

    2016-11-01

    Colonization with staphylococcus aureus (SA) is associated with disease activity and progression in patients with cutaneous T-cell lymphoma (CTCL) secondary to T-cell activation by bacterial superantigens. The aim of the current study was to evaluate the possible role of SA as an etiologic factor affecting CTCL initiation. We conducted a nested case-control study in a large population-representative database from the UK. Cases were defined as all patients with an incident diagnosis of mycosis fungoides (MF) or Sezary syndrome (SS) between 1995 and 2013. For every case, four eligible controls matched on age, sex, practice-site, and duration of follow-up were selected. Exposure of interest was clinical diagnosis of impetigo prior to CTCL diagnosis. Conditional logistic regression was used to calculate odds-ratio (ORs) and 95 % confidence-interval (CI) for CTCL risk. The results were further stratified according to age, sex and time interval between impetigo and CTCL diagnosis. The study population included 310 cases with MF or SS and 1223 matched controls. Among cases with CTCL 4.8 % (n = 15) had impetigo prior to cancer diagnosis compared to 2 % (n = 24) of controls. The adjusted OR for CTCL diagnosis among patients with prior impetigo was 2.33 (95 % CI 1.12-4.83). The risk was elevated among individuals with impetigo 1-5 years before cancer diagnosis (OR 3.33, 95 % CI 1.00-11.10). There was no change in risk among patients with impetigo more than 5 years before cancer diagnosis (OR 1.09, 95 % CI 0.35-3.37). Our results suggest a possible association between SA colonization and CTCL initiation that might serve as an important etiological factor for the disease.

  2. Outcome of Patients Treated With a Single-Fraction Dose of Palliative Radiation for Cutaneous T-Cell Lymphoma

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    Thomas, Tarita O.; Agrawal, Priya [Department of Radiation Oncology, Northwestern University, Robert H. Lurie Comprehensive Cancer Center, Chicago, Illinois (United States); Guitart, Joan [Department of Dermatology, Northwestern University, Robert H. Lurie Comprehensive Cancer Center, Chicago, Illinois (United States); Rosen, Steven T. [Division of Hematology/Oncology, Department of Medicine, Northwestern University, Robert H. Lurie Comprehensive Cancer Center, Chicago, Illinois (United States); Rademaker, Alfred W. [Department of Preventive Medicine, Northwestern University, Robert H. Lurie Comprehensive Cancer Center, Chicago, Illinois (United States); Querfeld, Christiane [Department of Medicine/Dermatology Service, Memorial Sloan-Kettering Cancer Center, New York, New York (United States); Hayes, John P. [Department of Radiation Oncology, Northwestern University, Robert H. Lurie Comprehensive Cancer Center, Chicago, Illinois (United States); Kuzel, Timothy M. [Division of Hematology/Oncology, Department of Medicine, Northwestern University, Robert H. Lurie Comprehensive Cancer Center, Chicago, Illinois (United States); Mittal, Bharat B., E-mail: bmittal@nmh.org [Department of Radiation Oncology, Northwestern University, Robert H. Lurie Comprehensive Cancer Center, Chicago, Illinois (United States)

    2013-03-01

    Purpose: Cutaneous T-cell lymphoma (CTCL) is a radiosensitive tumor. Presently, treatment with radiation is given in multiple fractions. The current literature lacks data that support single-fraction treatment for CTCL. This retrospective review assesses the clinical response in patients treated with a single fraction of radiation. Methods and Materials: This study reviewed the records of 58 patients with CTCL, primarily mycosis fungoides, treated with a single fraction of palliative radiation therapy (RT) between October 1991 and January 2011. Patient and tumor characteristics were reviewed. Response rates were compared using Fisher's exact test and multiple logistic regressions. Survival rates were determined using the Kaplan-Meier method. Cost-effectiveness analysis was performed to assess the cost of a single vs a multifractionated treatment regimen. Results: Two hundred seventy individual lesions were treated, with the majority (97%) treated with ≥700 cGy; mean follow-up was 41.3 months (range, 3-180 months). Response rate by lesion was assessed, with a complete response (CR) in 255 (94.4%) lesions, a partial response in 10 (3.7%) lesions, a partial response converted to a CR after a second treatment in 4 (1.5%) lesions, and no response in 1 (0.4%) lesion. The CR in lower extremity lesions was lower than in other sites (P=.0016). Lesions treated with photons had lower CR than those treated with electrons (P=.017). Patients with lesions exhibiting large cell transformation and tumor morphology had lower CR (P=.04 and P=.035, respectively). Immunophenotype did not impact response rate (P=.23). Overall survival was significantly lower for patients with Sézary syndrome (P=.0003) and erythroderma (P<.0001). The cost of multifractionated radiation was >200% higher than that for single-fraction radiation. Conclusions: A single fraction of 700 cGy-800 cGy provides excellent palliation for CTCL lesions and is cost effective and convenient for the patient.

  3. Outcome of Patients Treated With a Single-Fraction Dose of Palliative Radiation for Cutaneous T-Cell Lymphoma

    International Nuclear Information System (INIS)

    Thomas, Tarita O.; Agrawal, Priya; Guitart, Joan; Rosen, Steven T.; Rademaker, Alfred W.; Querfeld, Christiane; Hayes, John P.; Kuzel, Timothy M.; Mittal, Bharat B.

    2013-01-01

    Purpose: Cutaneous T-cell lymphoma (CTCL) is a radiosensitive tumor. Presently, treatment with radiation is given in multiple fractions. The current literature lacks data that support single-fraction treatment for CTCL. This retrospective review assesses the clinical response in patients treated with a single fraction of radiation. Methods and Materials: This study reviewed the records of 58 patients with CTCL, primarily mycosis fungoides, treated with a single fraction of palliative radiation therapy (RT) between October 1991 and January 2011. Patient and tumor characteristics were reviewed. Response rates were compared using Fisher's exact test and multiple logistic regressions. Survival rates were determined using the Kaplan-Meier method. Cost-effectiveness analysis was performed to assess the cost of a single vs a multifractionated treatment regimen. Results: Two hundred seventy individual lesions were treated, with the majority (97%) treated with ≥700 cGy; mean follow-up was 41.3 months (range, 3-180 months). Response rate by lesion was assessed, with a complete response (CR) in 255 (94.4%) lesions, a partial response in 10 (3.7%) lesions, a partial response converted to a CR after a second treatment in 4 (1.5%) lesions, and no response in 1 (0.4%) lesion. The CR in lower extremity lesions was lower than in other sites (P=.0016). Lesions treated with photons had lower CR than those treated with electrons (P=.017). Patients with lesions exhibiting large cell transformation and tumor morphology had lower CR (P=.04 and P=.035, respectively). Immunophenotype did not impact response rate (P=.23). Overall survival was significantly lower for patients with Sézary syndrome (P=.0003) and erythroderma (P 200% higher than that for single-fraction radiation. Conclusions: A single fraction of 700 cGy-800 cGy provides excellent palliation for CTCL lesions and is cost effective and convenient for the patient

  4. Primary Diffuse Large Cell Lymphoma of the Bladder: Case Report and Literature Review

    OpenAIRE

    Mansour Ansari; Hamid Nasrollahi; Majdaddin Rajaei; Maral Mokhtari; Seyed Hasan Hamedi; Mohammad Mohammadianpanah; Shapour Omidvari; Ahmad Mosalaei; Niloofar Ahmadloo

    2017-01-01

    Most bladder tumors are epithelial in origin. Nonepithelial cancers are rarely located in the bladder. Sarcomas are the most common malignancies among nonepithelial cancers. Primary bladder lymphoma is rare and mostly low grade. Here, we have reported a case of diffuse large cell lymphoma of the bladder. The patient, a 64-year-old man, had urinary frequency for 18 months. Abdominal sonography indicated a thick bladder wall and transurethral biopsy showed diffuse large cell lymp...

  5. Favourable response to rituximab by an ocular adnexal primary lymphoma.

    Science.gov (United States)

    Luque Valentin-Fernandez, M L; Alvarez Rodríguez, F; Rodríguez Jiménez, I

    2016-11-01

    A 70-year-old woman who presented with an extranodal marginal zone B-cell lymphoma in lacrimal gland and conjunctiva. Initial treatment with rituximab yielded an immediate good response. Five months later she showed lymphoid proliferation in her contralateral conjunctiva. Although no additional treatment was performed, the patient has been free of systemic symptoms and recurrences. Rituximab is a quite good therapeutic agent in low grade adnexal lymphomas. Copyright © 2016 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L.U. All rights reserved.

  6. Preliminary Clinical and Pharmacologic Investigation of Photodynamic Therapy with the Silicon Phthalocyanine Photosensitizer Pc 4 for Primary or Metastatic Cutaneous Cancers

    International Nuclear Information System (INIS)

    Kinsella, Timothy James; Baron, Elma D.; Colussi, Valdir C.; Cooper, Kevin D.; Hoppel, Charles L.; Ingalls, Stephen T.; Kenney, Malcolm E.; Li, Xiaolin; Oleinick, Nancy L.; Stevens, Seth R.; Remick, Scot C.

    2011-01-01

    Photodynamic therapy (PDT) for cutaneous malignancies has been found to be an effective treatment with a range of photosensitizers. The phthalocyanine Pc 4 was developed initially for PDT of primary or metastatic cancers in the skin. A Phase I trial was initiated to evaluate the safety and pharmacokinetic profiles of systemically administered Pc 4 followed by red light (Pc 4-PDT) in cutaneous malignancies. A dose-escalation study of Pc 4 (starting dose 0.135 mg/m 2 ) at a fixed light fluence (135 J/cm 2 of 675-nm light) was initiated in patients with primary or metastatic cutaneous malignancies with the aim of establishing the maximum tolerated dose (MTD). Blood samples were taken at intervals over the first 60 h post-PDT for pharmacokinetic analysis, and patients were evaluated for toxicity and tumor response. A total of three patients (two females with breast cancer and one male with cutaneous T-cell lymphoma) were enrolled and treated over the dose range of 0.135 mg/m 2 (first dose level) to 0.54 mg/m 2 (third dose level). Grade 3 erythema within the photoirradiated area was induced in patient 2, and transient tumor regression in patient 3, in spite of the low photosensitizer doses. Pharmacokinetic observations fit a three-compartment exponential elimination model with an initial rapid distribution phase (∼0.2 h) and relatively long terminal elimination phase (∼28 h), Because of restrictive exclusion criteria and resultant poor accrual, the trial was closed before MTD could be reached. While the limited accrual to this initial Phase I study did not establish the MTD nor establish a complete pharmacokinetic and safety profile of intravenous Pc 4-PDT, these preliminary data support further Phase I testing of this new photosensitizer.

  7. Preliminary clinical and pharmacologic investigation of photodynamic therapy with the silicon phthalocyanine photosensitizer Pc 4 for primary or metastatic cutaneous cancers

    Directory of Open Access Journals (Sweden)

    Timothy James Kinsella

    2011-06-01

    Full Text Available Photodynamic therapy (PDT for cutaneous malignancies has been found to be an effective treatment with a range of photosensitizers. The phthalocyanine Pc 4 was developed initially for PDT of primary or metastatic cancers in the skin. A Phase I trial was initiated to evaluate the safety and pharmacokinetic profiles of systemically administered Pc 4 followed by red light (Pc 4-PDT in cutaneous malignancies. A dose-escalation study of Pc 4 (starting dose 0.135 mg/m2 at a fixed light fluence (135 J/cm2 of 675-nm light was initiated in patients with primary or metastatic cutaneous malignancies with the aim of establishing the maximum tolerated dose (MTD. Blood samples were taken at intervals over the first 60 hours post-PDT for pharmacokinetic analysis, and patients were evaluated for toxicity and tumor response. A total of 3 patients (2 females with breast cancer and 1 male with cutaneous lymphoma were enrolled and treated over the dose range of 0.135 mg/m2 (first dose level to 0.54 mg/m2 (third dose level. Grade 3 erythema within the photoirradiated area was induced in patient 2, and transient tumor regression in patient 3, in spite of the low photosensitizer doses. Pharmacokinetic observations fit a 3-compartment exponential elimination model with an initial rapid distribution phase (~0.2 hrs and relatively long terminal elimination phase (~28 hrs, Because of restrictive exclusion criteria and resultant poor accrual, the trial was closed before MTD could be reached. While the limited accrual to this initial Phase I study did not establish the MTD nor establish a complete pharmacokinetic and safety profile of intravenous Pc 4-PDT, these preliminary data support further Phase I testing of this new photosensitizer.

  8. Cryotherapy with liquid nitrogen versus topical salicylic acid application for cutaneous warts in primary care : randomized controlled trial

    NARCIS (Netherlands)

    Bruggink, Sjoerd C.; Gussekloo, Jacobijn; Berger, Marjolein Y.; Zaaijer, Krista; Assendelft, Willem J. J.; de Waal, Margot W. M.; Bavinck, Jan Nico Bouwes; Koes, Bart W.; Eekhof, Just A. H.

    2010-01-01

    Background: Cryotherapy is widely used for the treatment of cutaneous warts in primary care. However, evidence favours salicylic acid application. We compared the effectiveness of these treatments as well as a wait-and-see approach. Methods: Consecutive patients with new cutaneous warts were

  9. Secondary cutaneous Epstein-Barr virus-associated diffuse large B-cell lymphoma in a patient with angioimmunoblastic T-cell lymphoma: a case report and review of literature

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    Yang Qing-Xu

    2012-01-01

    Full Text Available Abstract Only a few cases of extranodal Epstein-Barr virus (EBV-associated B-cell lymphomas arising from patients with angioimmunoblastic T-cell lymphoma (AITL have been described. We report a case of AITL of which secondary cutaneous EBV-associated diffuse large B-cell lymphoma (DLBCL developed after the initial diagnosis of AITL. A 65-year-old Chinese male patient was diagnosed as AITL based on typical histological and immunohistochemical characteristics in biopsy of the enlarged right inguinal lymph nodes. The patient initially received 6 cycles of chemotherapy with CHOP regimen (cyclophosphamide, vincristine, adriamycin, prednisone, but his symptoms did not disappear. Nineteen months after initial diagnosis of AITL, the patient was hospitalized again because of multiple plaques and nodules on the skin. The skin biopsy was performed, but this time the tumor was composed of large, polymorphous population of lymphocytes with CD20 and CD79a positive on immunohistochemical staining. The tumor cells were strong positive for EBER by in situ hybridization. The findings of skin biopsy were compatible with EBV-associated DLBCL. CHOP-R chemotherapy (cyclophosphamide, doxorubicin, vincristine, prednisone and rituximab was then administered, resulting in partial response of the disease with pancytopenia and suppression of cellular immunity. To our knowledge, this is the first case of cutaneous EBV-associated DLBCL originated from AITL in Chinese pepole. We suggest the patients with AITL should perform lymph node and skin biopsies regularly in the course of the disease to detect the progression of secondary lymphomas. Virtual slides The virtual slide(s for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1197421158639299

  10. Primary Central Nervous System Lymphoma: Incorporating MRI in the Planning of Treatment Strategies

    International Nuclear Information System (INIS)

    Eloraby, A.; Zaki, I.

    2001-01-01

    Primary lymphoma of the central nervous system is becoming increasingly encountered secondary to the acquired immune-deficiency disorders. MRI is rapidly evolving diagnostic tool in the management of the lymphomatous CNS primary infiltrates. Methods and materials: 40 patients of the National Cancer Institute of Cairo University were studied by medium and high power MRI machines before and after intra-venous contrast enhancement. Results: The cerebral lesions exhibited specific diagnostic criteria regarding the anatomical configuration, signal pattern, peri-focal oedema and response to steroids, such manifestations made. Conclusion: MRI a highly reliable tool in the management of the disease. The work proved that spinal cord primary lymphoma is a rare entity

  11. Primary central nervous system lymphoma in immunocompetent patients: spectrum of findings and differential characteristics.

    Science.gov (United States)

    Gómez Roselló, E; Quiles Granado, A M; Laguillo Sala, G; Pedraza Gutiérrez, S

    2018-02-23

    Primary central nervous system (CNS) lymphomas are uncommon and their management differs significantly from that of other malignant tumors involving the CNS. This article explains how the imaging findings often suggest the diagnosis early. The typical findings in immunocompetent patients consist of a supratentorial intraaxial mass that enhances homogeneously. Other findings to evaluate include multifocality and incomplete ring enhancement. The differential diagnosis of primary CNS lymphomas should consider mainly other malignant tumors of the CNS such as glioblastomas or metastases. Primary CNS lymphomas tend to have less edema and less mass effect; they also tend to spare the adjacent cortex. Necrosis, hemorrhage, and calcification are uncommon in primary CNS lymphomas. Although the findings in morphologic sequences are characteristic, they are not completely specific and atypical types are sometimes encountered. Advanced imaging techniques such as diffusion or especially perfusion provide qualitative and quantitative data that play an important role in differentiating primary CNS lymphomas from other brain tumors. Copyright © 2018 SERAM. Publicado por Elsevier España, S.L.U. All rights reserved.

  12. Primary gastrointestinal lymphoma: a review of 21 cases.

    Science.gov (United States)

    Kaufman, Z; Eliashiv, A; Shpitz, B; Witz, M; Griffel, B; Dinbar, A

    1984-05-01

    A retrospective study of 21 patients who had suffered from gastrointestinal lymphoma was carried out. Gastric involvement was more common than involvement of the small or large intestine and carried a better prognosis. Gastrointestinal lymphoma generally occurs most frequently during the fourth to seventh decades of life. In our study, however, five lymphomas occurred in patients under 20 years of age. Clinical symptoms were nonspecific, and abdominal mass was found in only 15% of the patients on clinical examination. All patients were explored, 17 underwent resection, and 4 laparotomy and biopsy. Five-year survival was much better for patients who had undergone resection. Survival was inversely proportionate to the extent of nodal spread. Multiple lesions on the same organ yielded a 5-year survival of 20%, while a single lesion offered a 55% chance of 5-year survival. Diffuse histiocytic lymphoma was the commonest type, followed by the poorly differentiated lymphocytic type. A longer survival rate was present in the lymphocytic type. However, the highest survival rate was in those patients in whom definite resections of the lesion were performed followed by radiation and chemotherapy, especially for gastric tumor.

  13. Angioimmunoblastic T-Cell Lymphoma

    Science.gov (United States)

    ... Non-Hodgkin Lymphoma Peripheral T-Cell Lymphoma Primary Central Nervous System Lymphoma T-Cell Lymphoma Transformed Mycosis Fungoides Waldenstrom Macroglobulinemia Young Adult Lymphoma Overview Treatment Options Relapsed/Refractory Long-term ...

  14. Peripheral T-Cell Lymphoma

    Science.gov (United States)

    ... Non-Hodgkin Lymphoma Peripheral T-Cell Lymphoma Primary Central Nervous System Lymphoma T-Cell Lymphoma Transformed Mycosis Fungoides Waldenstrom Macroglobulinemia Young Adult Lymphoma Overview Treatment Options Relapsed/Refractory Long-term ...

  15. Primary intestinal T cell lymphomas in Indian patients - In search of enteropathic T cell lymphoma

    Directory of Open Access Journals (Sweden)

    Shet Tanuja

    2010-07-01

    Full Text Available Objective: This series of six intestinal T cell lymphomas (ITCL attempts to document enteropathy-associated T cell lymphoma (EATCL in India. Materials and Methods: A total of six ITCL were selected from 170 gastrointestinal lymphomas in last 10 years. Results: The cases studied included EATCL (4, ITCL with a CD4 positive phenotype (1 and ITCL NK/T cell type (1. Of the four EATCL, two occurred in the ileum, one in right colon and one in duodenum. In three EATCL cases, there was history of celiac disease or lactose intolerance and enteropathic changes were noted in the adjacent mucosa. These tumors had CD3+/CD8+/CD56 (+/-/CD4-/ Granzyme B+ immunophenotype. One EATCL was monomorphic small cell type (type II EATCL with a CD3+/CD8-CD56+/CD4-/ Granzyme B+ phenotype. EBER- ISH (Epstein Barr virus coded RNA′s- in situ hybridization revealed positive tumor cells in ITCL NK/T cell type and in bystander cells in three EATCL. Conclusion: ITCL are rare in Indian patients but do occur and comprise a mixture of the enteropathic and non-enteropathic subtypes.

  16. Primary Pulmonary Mucosa-Associated Lymphoid Tissue Lymphoma with a Nodular Opacity: Report of a Case.

    Science.gov (United States)

    Yoshino, Naoyuki; Hirata, Tomomi; Takeuchi, Chie; Usuda, Jitsuo; Hosone, Masaru

    2017-01-01

    Herein, we describe our experience in treating a case of primary pulmonary mucosa-associated lymphoid tissue lymphoma detected as a nodular opacity. A 79-year-old man was referred to our hospital. Computed tomography showed a nodular opacity measuring 20 mm in diameter with regular margins in segment 5 of the right middle lobe of the lung. Although the bronchoscopic brush cytology result was class III, the patient was tentatively diagnosed with suspected mucosa-associated lymphoid tissue lymphoma. A thoracoscopic right middle lobectomy was performed. The pathological findings showed nodular proliferation of small to medium-sized, mature-appearing atypical lymphoid cells, lymphoepithelial lesions, and vague follicles suggesting follicular colonization in some areas. The patient was diagnosed with low-grade small B-cell lymphoma and mucosa-associated lymphoid tissue lymphoma. He has remained well to date, 23 months after surgery, without evidence of recurrence.

  17. Primary NK/T cell lymphoma nasal type of the colon

    Directory of Open Access Journals (Sweden)

    Ana María Chirife

    2013-02-01

    Full Text Available Since nasal NK/T-cell lymphoma and NK/T-cell lymphoma nasal type are rare diseases, colonic involvement has seldom been seen. We report a case of a patient with a primary NK/T-cell lymphoma nasal type of the colon. The patient had no history of malignant diseases and was diagnosed after exhaustive study in the context of fever of unknown origin. The first therapeutic approach followed the DAEPOCH-protocol: etoposide, prednisone, doxor-rubicin, vincristine and cyclophosphamide. The persistence of constitutional symptoms after the first treatment course motivated the switch to a second line following the SMILE-protocol: dexamethasone, metotrexate, ifosfamide, E.coli L-asparaginase, and etoposide. Despite intensive chemotherapy, the patient died 2 months after the diagnose of an extranodal NK/T-cell lymphoma of the colon and 4 months after the first symptomatic appearance of disease.

  18. Talimogene Laherparepvec and Nivolumab in Treating Patients With Refractory Lymphomas or Advanced or Refractory Non-melanoma Skin Cancers

    Science.gov (United States)

    2018-05-21

    Adenoid Cystic Carcinoma; Adnexal Carcinoma; Apocrine Carcinoma; Eccrine Porocarcinoma; Extraocular Cutaneous Sebaceous Carcinoma; Hidradenocarcinoma; Keratoacanthoma; Malignant Sweat Gland Neoplasm; Merkel Cell Carcinoma; Microcystic Adnexal Carcinoma; NK-Cell Lymphoma, Unclassifiable; Non-Melanomatous Lesion; Paget Disease; Papillary Adenocarcinoma; Primary Cutaneous Mucinous Carcinoma; Refractory Anaplastic Large Cell Lymphoma; Refractory Mature T-Cell and NK-Cell Non-Hodgkin Lymphoma; Refractory Mycosis Fungoides; Refractory Primary Cutaneous T-Cell Non-Hodgkin Lymphoma; Refractory T-Cell Non-Hodgkin Lymphoma; Sezary Syndrome; Signet Ring Cell Carcinoma; Skin Basal Cell Carcinoma; Skin Basosquamous Cell Carcinoma; Skin Squamous Cell Carcinoma; Spiradenocarcinoma; Squamous Cell Carcinoma of Unknown Primary Origin; Stage III Skin Cancer; Stage IV Skin Cancer; Sweat Gland Carcinoma; Trichilemmocarcinoma; Vulvar Squamous Cell Carcinoma

  19. STAT5 induces miR-21 expression in cutaneous T cell lymphoma

    DEFF Research Database (Denmark)

    Lindahl, Lise M; Fredholm, Simon; Joseph, Claudine

    2016-01-01

    was inhibited by Tofacitinib (CP-690550), a clinical-grade JAK3 inhibitor. Chromatin immunoprecipitation (ChIP) analysis showed direct binding of STAT5 to the miR-21 promoter. Cytokine starvation ex vivo triggered a decrease in miR-21 expression, whereas IL-2 induced an increased miR-21 expression in primary SS...

  20. Cutaneous features seen in primary liver cell (Hepatocellular ...

    African Journals Online (AJOL)

    Primary liver cell carcinoma (PLCC), predominantly hepatocellular carcinoma is a killer. In the southwestern region of Nigeria it occupies the second position, behind prostate cancer in males. Females account for about a third of diagnosed cases. Children are not spared. Over 80 % of PLCC cases present to the hospital at ...

  1. Primary lymphoma of the breast involving both axillae with bilateral breast carcinoma

    Directory of Open Access Journals (Sweden)

    Rubin Gary

    2008-05-01

    Full Text Available Abstract Background Primary Non-Hodgkin's Lymphoma (PHNL of the breast is a rare entity, while secondary involvement of the breast with diffuse disease of Non-Hodgkin's lymphoma (NHL is more common. However, PNHL is the most frequent haematopoietic tumour of the breast. Diagnostic criteria for PNHL of the breast are presence of technically adequate pathologic specimens, close association of mammary tissue and lymphomatous infiltrate, no prior diagnosis of an extarammamary lymphoma, and no evidence of concurrent widespread disease, except for ipsilateral axillary lymph nodes if concomitant with the primary lesion. Case presentation A 57-year-old woman was recalled because her screening mammograms revealed three separate lesions in her right breast and one in the left. Histology of the lesions confirmed lymphoma in one breast with ductal carcinoma in the other. Conclusion Most of reported cases in literature have been involving the right breast, and almost all the patients were females. NHLs of the breast typically present as unilateral mass; the frequency of bilateral disease at first presentation ranges from 5–25%. Our objective is to report a case of primary lymphoma of the breast involving both axillae with concomitant bilateral primary breast cancer which has not been reported yet to our best of knowledge in literature.

  2. Primary appendiceal lymphoma presenting as suspected perforated acute appendicitis: clinical, sonography and CT findings with pathologic correlation.

    Science.gov (United States)

    Guo, Jingjing; Wu, Gang; Chen, Xiaojun; Li, Xiaodong

    2014-01-01

    The gastrointestinal tract is the most common site for extranodal involvement by non-Hodgkin's lymphoma. However, primary appendiceal lymphomas presenting as perforated acute appendicitis are very rare: they occur in only 0.015% of all gastrointestinal lymphoma cases. The management of this condition is still controversial, and a multimodality approach (e.g., surgery, radiation therapy, and chemotherapy) is the optimal treatment. In these cases, appendiceal non-Hodgkin's lymphomas typically manifest with acute symptoms in patients with no prior lymphoma history. Additionally, we treated our patient with a right hemicolectomy and postoperative multiagent chemotherapy.

  3. A case of primary osseous malignant immunoblastic B-cell lymphoma with intracytoplasmic mu lambda immunoglobulin inclusions.

    Science.gov (United States)

    Fiche, M; Le Tourneau, A; Audouin, J; Touzard, R C; Diebold, J

    1990-02-01

    Primary malignant lymphoma of bone, so-called Parker-Jackson reticulosarcoma, is a rare form of extranodal lymphoma with a relatively good prognosis. It often corresponds to B-cell lymphoma of high-grade malignancy. We report a case of mu lambda immunoblastic lymphoma showing two distinctive features: an abundant reactive T-lymphocytic population and unusual intra-cytoplasmic inclusions. These inclusions were PAS positive and consisted of monotypic mu lambda immunoglobulin localized in peculiar aggregates of rough endoplasmic reticulum. Their morphological appearances resembled the well-documented inclusions described in some varieties of non-Hodgkin's lymphoma.

  4. Primary Bilateral Non-Hodgkin's Lymphoma of the Adrenal Gland: A Case Report

    Directory of Open Access Journals (Sweden)

    Ahmed Amine Bouchikhi

    2012-01-01

    Full Text Available Primary bilateral non-Hodgkin's lymphoma (NHL of the adrenal gland is a very rare entity. Indeed less than 60 cases have been reported in the literature. Hence, we report a case of high-grade lymphoma of both adrenal glands that was found in a young patient of 32 years of age. The patient was admitted in the emergency department of our hospital with a profile of hemorrhagic shock. After stabilization, the imaging investigations demonstrated large bilateral adrenal masses. The CT-scan guided biopsy of both adrenal glands allowed the diagnosis of primary bilateral adrenal NHL. The patient died after the first chemotherapy session. The presence of bilateral adrenal masses associated with a rapid increase of volume should raise the diagnosis of primary adrenal non-Hodgkin's lymphoma.

  5. Clinical potential of mechlorethamine gel for the topical treatment of mycosis fungoides-type cutaneous T-cell lymphoma: a review on current efficacy and safety data

    Directory of Open Access Journals (Sweden)

    Liner K

    2018-01-01

    Full Text Available Kendall Liner,1 Celeste Brown,2 Laura Y McGirt3 1Division of Dermatology, Medical College of Georgia at Augusta Health, Augusta, GA, USA; 2School of Medicine, University of North Carolina at Chapel Hill, Carolinas Medical Center, Charlotte, NC, USA; 3Department of Hematology/Oncology, Levine Cancer Institute, Carolinas Medical Center, Charlotte, NC, USA Abstract: Nitrogen mustard is a chemotherapeutic agent that has a well-documented safety and efficacy profile in the treatment of cutaneous T-cell lymphoma. Development of nitrogen mustard formulations and treatment regimens has been studied extensively over the last 40 years. In the last 5 years, a new gel formulation has been developed that is associated with a decrease in delayed hypersensitivity reactions. The authors in this review found that while the gel formulation may result in a decrease of allergic contact dermatitis, this advantage has been replaced by a higher number of irritant contact reactions and a decrease in complete response rate. The gel formulation has a complete response rate of 13.8%, which is a decrease in efficacy when compared to aqueous-based preparations of similar concentrations. Keywords: mycosis fungoides, nitrogen mustard, mechlorethamine gel, cutaneous T-cell lymphoma, CTCL, Valchlor®

  6. Primary CNS lymphoma in nonimmunocompromised patients magnetic resonance study

    International Nuclear Information System (INIS)

    Pena, J.; Fernandez, J.M.; Galarraga, M.I.; Pozo, A.; Montes, A.; Ablanedo, P.

    1995-01-01

    Prymary lymphoma of the CNS (PLCNS) is a relatively infrequent malignant tumor that has become increasingly common over the past decade. The radiological signs, although not pathognomonic, are quite specific and suggestive of the correct diagnosis, thus facilitating therapeutic management. We present six cases of PLCNS in nonimmunocopromised patients studied by MR in our hospital over the past two and a half years. We describe theradiological findings, correlating them with those mentioned in the literature. 14 refs

  7. Primary non-Hodgkin′s lymphoma of the salivary gland: A spectrum of lymphoepithelial sialadenitis, low-grade B-cell lymphoma of mucosa-associated lymphoid tissue with transformation to high-grade lymphoma

    Directory of Open Access Journals (Sweden)

    Agale Shubhangi

    2010-04-01

    Full Text Available Lymphoid infiltrates of the salivary gland can be either reactive or neoplastic. The reactive lesion, lymphoepithelial sialadenitis (LESA may be associated with Sjogren′s syndrome (SS or may occur as an isolated salivary gland enlargement. Patients with LESA/SS have a particularly high risk of subsequently developing lymphoma, which is a low-grade mucosa-associated lymphoid tissue (MALT type lymphoma of the salivary gland. We document a rare case of primary non-Hodgkin′s lymphoma of the parotid gland arising in the background of LESA and with a rare example of transformation from low grade to high-grade B cell lymphoma of MALT type.

  8. Total skin electron beam therapy for cutaneous T-cell lymphoma: A nationwide cohort study from Denmark

    International Nuclear Information System (INIS)

    Lindahl, Lise M.; Iversen, Lars; Kamstrup, Maria R.; Gniadecki, Robert; Petersen, Peter M.; Specht, Lena; Wiren, Johan; Fenger-Groen, Morten

    2011-01-01

    Background. Total skin electron beam therapy (TSEBT) is an effective palliative treatment for cutaneous T-cell lymphoma (CTCL). In the present study we reviewed the clinical response to TSEBT in Danish patients with CTCL. Material and methods. This retrospective study included 35 patients with CTCL treated with TSEBT in Denmark from 2001 to 2008 and followed for a median time of 7.6 months (range 3 days-3.7 years). Twenty five patients were treated with high-dose (30 Gy) and 10 patients in a protocol with low-dose (4 Gy) TSEBT. Results. Patients treated with low-dose therapy had inadequate response to treatment compared to patients treated with high-dose. Consequently the study with low-dose was discontinued and published. In patients treated with high-dose the overall response rate was 100%. Complete response (CR) rate was 68% and CR occurred after a median time of 2.1 months (range 1.8 months - 2.0 years). We found no difference in CR rate in patients with T2 (66.7%) and T3 disease (78.6%) (p = 0.64). Following CR 82.4% relapsed at a median time of four months (range 12 days-11.5 months). Relapse-free-survival was similar in patients with T2 and T3 disease (p 0.77). Progressive disease (PD) was experienced in 28.0% and the median time to PD was 9.0 months (range 4.6-44.3 months). Overall progression-free survival was 95.3%, 72.1% and 64.1% after 0.5-, 1- and 2-years. Effects of initial therapy on TSEBT treatment response and side effects to TSEBT were also analyzed. Conclusion. In conclusion, the present study confirms that high-dose TSEBT is an effective, but generally not a curative therapy in the management of CTCL. High-dose treatment yielded significantly better results than low-dose treatment with 4 Gy. TSEBT offers significant palliation in most patients when other skin-directed or systemic treatments have failed

  9. Primary versus secondary closure of cutaneous abscesses in the emergency department: a randomized controlled trial.

    Science.gov (United States)

    Singer, Adam J; Taira, Breena R; Chale, Stuart; Bhat, Rahul; Kennedy, David; Schmitz, Gillian

    2013-01-01

    Cutaneous abscesses have traditionally been treated with incision and drainage (I&D) and left to heal by secondary closure. The objective was to compare the healing rates of cutaneous abscesses following I&D after primary or secondary closure. This was a randomized, controlled, trial, balanced by center, with blocked randomization created by a random-number generator. One urban and one suburban academic emergency department (ED) participated. Subjects were randomized to primary or secondary wound closure following I&D of the abscess. Main outcome measures were the percentage of healed wounds (wound was completely closed by visual inspection; a 40% difference in wound healing was sought) and overall failure rate (need for additional intervention including suture removal, additional drainage, antibiotics, or admission within 7 days after drainage). Fifty-six adult patients with simple localized cutaneous abscesses were included; 29 were randomized to primary closure, and 27 were randomized to secondary closure. Healing rates at 7 days were similar between the primary and secondary closure groups (69.6%, 95% confidence interval [CI] = 49.1% to 84.4% vs. 59.3%, 95% CI = 40.7% to 75.5%; difference 10.3%, 95% CI = -15.8% to 34.1%). Overall failure rates at 7 days were also similar between the primary and secondary closure groups (30.4%, 95% CI = 15.6% to 50.9% vs. 28.6%, 95% CI = 15.2% to 47.1%; difference 1.8%, 95% CI = -24.2% to 28.8%). The rates of wound healing and treatment failure following I&D of simple abscesses in the ED are similar after primary or secondary closure. The authors did not detect a difference of at least 40% in healing rates between primary and secondary closure. © 2013 by the Society for Academic Emergency Medicine.

  10. Simultaneous Primary Hodgkin's Lymphoma of the Sigmoid Colon and Papillary Thyroid Carcinoma in an HIV-Positive Patient.

    Science.gov (United States)

    Liszewski, Walter; Sittig, Mark; Kandil, Emad; Van Sickels, Nicholas; Safah, Hana

    2015-01-01

    Primary Hodgkin's lymphoma of the colon is a rare phenomenon previously only reported in patients with chronic diverticulitis or inflammatory bowel disease. Herein we report a case of primary Hodgkin's lymphoma of the sigmoid colon in an HIV-positive patient without a history of inflammatory bowel disease or chronic diverticulitis that was later complicated by the discovery of concurrent papillary thyroid carcinoma.

  11. [Primary lymphoma of the central nervous system: 20 years' experience in a referral hospital].

    Science.gov (United States)

    Calderón-Garcidueñas, A L; Pacheco-Calleros, J; Castelán-Maldonado, E; Nocedal-Rustrián, F C

    Primary central nervous system lymphomas (PCNSL) are rare neoplasms. AIM. To study the clinical aspects and the immuno-phenotype of all cases of PCNSL in a 20 years lapse in a referral hospital in Northeastern Mexico. From January 1986 to December 2005 all PCNSL histologically confirmed were studied. The primary lymphomas were 1% of malignant central nervous system neoplasms. 21 cases were studied (ages from 9-70 years) with male predominance (2:1). 24% patients had immuno-suppression. The more frequent clinical data were: papilledema (71%), headache (62%), paresis (48%) and seizures (33%). 33% of patients died during the first six months after diagnosis. The T lymphomas were 19% of cases and corresponded to small cell type. PCNSL are still a diagnostic challenge. Multicenter studies are required in order to determine the best treatment protocol.

  12. Analysis of trends and seasonal variation in primary cutaneous melanoma: an Irish study.

    LENUS (Irish Health Repository)

    Downes, M R

    2010-11-10

    A seasonal variation in the presentation of cutaneous melanoma has been documented in several studies. We performed a retrospective review of primary cutaneous melanomas (n = 263) from our institution to examine whether the seasonal patterns of presentation noted in the literature would be similar in Ireland, a climate with low ambient sunshine. A summer : winter ratio was determined for age, gender, subtype, location and Breslow thickness. We found an increase in total numbers of melanomas, particularly in men. The summer : winter ratio was 2.39 for all patients (95% CI 1.60-3.57, P < 0.001), with seasonal variations noted for location, thickness and subtype (excluding lentigo). Melanomas presenting over the summer tended towards a greater Breslow thickness than did those presenting in winter. This subclassification of primary cutaneous melanoma with summer : winter ratios based on patient and tumour characteristics gave remarkably similar results to previously published reports, notwithstanding the low levels of annual ambient sunshine in Ireland.

  13. [Posttraumatic primary cutaneous aspergillosis with Candida guilliermondii infection in a healthy host].

    Science.gov (United States)

    Türkşen, Zeynep; Yağcı, Server; Karadağ, Ayşe Serap; Tezer, Ayla; Taner, Omer Faruk; Tekin, Fatih; Arıkan Akdağlı, Sevtap

    2010-10-01

    Opportunistic fungal infections are usually seen in immunocompromised patients. While Candida is the most prevalent agent in such infections, Aspergillus is at the second order. Primary cutaneous aspergillosis is most common in immunocompromised patients but can rarely be seen in healthy hosts as well. We report a case of posttraumatic primary cutaneous aspergillosis and Candida guilliermondii coinfection in a 70-years-old healthy man. The patient had an ulcerous lesion which developed in the site of a trauma on the middle finger of the right hand. Histopathological examination of the biopsy specimens revealed septate hyphae with dichotomous branching small circular blastospores. The cultures of the biopsy specimen yielded yellow-green colored, granular mold colonies and creamy white yeast colonies. Microscopic examination of the lactophenol cotton blue stained mold colonies indicated long conidiophores with vesicles surrounded by uniseriate phialides, compatible with Aspergillus flavus. Yeast colonies were identified as Candida guilliermondii by ID32C (BioMerieux, France) and by their microscopical morphology detected in corn meal-Tween 80 agar incubated at 25°C for 72 hours. The patient was treated properly with surgical debridement and itraconazole therapy. Since the immune system is compressed as a consequence of aging, cutaneous opportunistic fungal infections should be considered in the differential diagnosis of posttraumatic necrotic ulcers and black eschar in aged patients.

  14. Primary Cutaneous Carcinosarcoma of the Basal Cell Subtype Should Be Treated as a High-Risk Basal Cell Carcinoma.

    Science.gov (United States)

    Bourgeault, Emilie; Alain, Jimmy; Gagné, Eric

    2015-01-01

    Cutaneous carcinosarcoma is a rare primary tumor of the skin, characterized by biphasic epithelial and mesenchymal differentiation. Due to the limited number of cases reported, there is no consensus regarding treatment and prognosis. Some authors suggest that cutaneous carcinosarcomas should be viewed as aggressive tumors, with ancillary imaging used to evaluate potential metastatic disease. Other reports demonstrate an indolent disease course, especially with epidermal-type cutaneous carcinosarcomas. We report a case of cutaneous carcinosarcoma, which we treated with electrodessication and curettage following a shave biopsy. The tumor had an epithelial component resembling a basal cell carcinoma and a fibrosarcomatous stroma. At 1-year follow-up, our patient did not show evidence of recurrence or metastasis. Our case suggests that a cutaneous carcinosarcoma with an epithelial component composed of basal cell carcinoma can be regarded as a high-risk nonmelanoma skin cancer. © The Author(s) 2015.

  15. Isolated orbital mass as the primary presentation of a triple-hit lymphoma transformed from a systemic follicular lymphoma.

    Science.gov (United States)

    Zhou, Xiao Yi; Lu, Xinyan; Raparia, Kirtee; Chen, Yi-Hua

    2018-06-01

    Triple-hit lymphoma is a highly aggressive B-cell lymphoma. We report a case of triple-hit lymphoma transformed from systemic follicular lymphoma (FL) after 9-year remission and presented primarily as an isolated orbital mass without systemic symptoms or lymphadenopathy. A 58-year-old female presented with intermittent vertical binocular diplopia, left upper eyelid swelling and pain and was found to have a 2.9 cm orbital mass. Histological section revealed a CD10-positive large B-cell lymphoma, consistent with transformation of FL. Fluorescent in situ hybridization (FISH) analysis demonstrated rearrangements involving C-MYC, BCL-2 and BCL-6 genes, indicating a high grade, triple-hit lymphoma. Triple-hit lymphoma transformed from a low-grade lymphoma may initially present as an isolated orbital mass without systemic evidence of transformation. Early recognition of double or triple-hit lymphomas is important since these patients require aggressive chemotherapy.

  16. Radiotherapy for primary localized (stage I and II) non-Hodgkin's lymphoma of the oral cavity

    International Nuclear Information System (INIS)

    Sunaba, Kohji; Shibuya, Hitoshi; Okada, Norihiko; Amagasa, Teruo; Enomoto, Shoji; Kishimoto, Seiji

    2000-01-01

    Purpose: To assess the role of radiation therapy in the treatment of primary localized (Stage I: 24 cases and Stage II: 13 cases) non-Hodgkin's Lymphoma (NHL) of the oral cavity. Methods and Materials: In total, 37 patients (27 male, 10 female) with primary localized NHL of the oral cavity have been treated with radiotherapy alone (23 cases) or radiation with chemotherapy (14 cases). The age range was 29 to 86 years (median: 65). Clinical and treatment variables with potential prognostic significance for survival were evaluated by univariate and multivariate analysis. Of the 37 patients, 31 (84%) had intermediate-grade lymphomas and six (14%) had high-grade lymphomas. Four patients showed necrotic ulcer in the central portion of the hard palate. Results: The 5-year actuarial survival rate for all cases was 73%. The 5-year survival rates for intermediate-grade and high-grade lymphoma were 85% and 14%, respectively. Significant prognostic factors identified by the multivariate analysis were histologic grade of malignancy (p = 0.02) and central necrotic ulcer in the tumor (p = 0.02). Chemotherapy did not improve survival (p = 0.41). Conclusions: Our analysis suggests that radiotherapy alone may be approved as the treatment for localized oral NHL with no ulceration and intermediate histology. However, patients with high-grade lymphoma and/or necrotic ulcer are difficult to cure with radiation alone and aggressive treatment should be advocated to improve survival

  17. A Case of Primary Central Nervous System Lymphoma Located at Brain Stem in a Child.

    Science.gov (United States)

    Kim, Jinho; Kim, Young Zoon

    2016-10-01

    Primary central nervous system lymphoma (PCNSL) is an extranodal Non-Hodgkin's lymphoma that is confined to the brain, eyes, and/or leptomeninges without evidence of a systemic primary tumor. Although the tumor can affect all age groups, it is rare in childhood; thus, its incidence and prognosis in children have not been well defined and the best treatment strategy remains unclear. A nine-year old presented at our department with complaints of diplopia, dizziness, dysarthria, and right side hemiparesis. Magnetic resonance image suggested a diffuse brain stem glioma with infiltration into the right cerebellar peduncle. The patient was surgically treated by craniotomy and frameless stereotactic-guided biopsy, and unexpectedly, the histopathology of the mass was consistent with diffuse large B cell lymphoma, and immunohistochemical staining revealed positivity for CD20 and CD79a. Accordingly, we performed a staging work-up for systemic lymphoma, but no evidence of lymphoma elsewhere in the body was obtained. In addition, she had a negative serologic finding for human immunodeficient virus, which confirmed the histopathological diagnosis of PCNSL. She was treated by radiosurgery at 12 Gy and subsequent adjuvant combination chemotherapy based on high dose methotrexate. Unfortunately, 10 months after the tissue-based diagnosis, she succumbed due to an acute hydrocephalic crisis.

  18. The endoscopic spectrum of primary non-Hodgkin's lymphoma of the stomach

    NARCIS (Netherlands)

    Taal, B. G.; den Hartog Jager, F. C.; Tytgat, G. N.

    1987-01-01

    Thirty-one consecutive patients with primary non-Hodgkin's lymphoma of the stomach were studied to outline the spectrum of endoscopic abnormalities. The 17 men and 14 women had a median age of 65 years. There were 22 patients in stage I and 9 in stage II. Three endoscopic patterns were recognized:

  19. Cognitive functions in primary central nervous system lymphoma: Literature review and assessment guidelines

    NARCIS (Netherlands)

    D.D. Correa; L. Maron; H. Harder (Helena); M. Klein (Martin); C.L. Armstrong; P. Calabrese; J.E.C. Bromberg (Jacolien); L.E. Abrey (Lauren); T.T. Batchelor (Tracy); D. Schiff (David)

    2007-01-01

    textabstractBackground: Treatment-related neurotoxicity has been recognized as a significant problem in patients with primary central nervous system lymphoma (PCNSL) as effective treatment has increased survival rates. There is, however, a paucity of research on cognitive functions in this

  20. Primary diffuse large B-cell lymphoma of the prostate in a young patient

    Directory of Open Access Journals (Sweden)

    Carlos A. Alvarez

    2006-02-01

    Full Text Available We report a primary lymphoma of the prostate, which arose in a 29-year-old man with hematuria. Pathological evaluation of tissue fragments allowed us to choose appropriate medical management. A diagnosis of suspicion can be performed by urine cytology, and molecular techniques may be helpful. Emphasis in differential diagnosis is made.

  1. Primary Lymphoma of the Testis in Remission for more than Ten ...

    African Journals Online (AJOL)

    It is the most common testicular tumour in males between 60 and 80 years old OBJECTIVE: To report a case of primary testicular lymphoma in a young man who has done very well on surgery and chemotherapy. METHODS: The patient a Nigerian male civil servant who was single and aged 31 years presented to us with a ...

  2. Discrimination of different brain metastases and primary CNS lymphomas using morphologic criteria and diffusion tensor imaging

    Energy Technology Data Exchange (ETDEWEB)

    Bette, S.; Wiestler, B.; Huber, T.; Boeckh-Behrens, T.; Zimmer, C.; Kirschke, J. [Technical University Munich, Klinikum rechts der Isar (Germany). Dept. of Neuroradiology; Delbridge, C. [Technical University Munich, Klinikum rechts der Isar (Germany). Dept. of Neuropathology; Meyer, B.; Gempt, J. [Technical University Munich, Klinikum rechts der Isar (Germany). Dept. of Neurosurgery

    2016-12-15

    Brain metastases are a common complication of cancer and occur in about 15-40% of patients with malignancies. The aim of this retrospective study was to differentiate between metastases from different primary tumors/CNS lymphyomas using morphologic criteria, fractional anisotropy (FA) and apparent diffusion coefficient (ADC). Morphologic criteria such as hemorrhage, cysts, pattern of contrast enhancement and location were reported in 200 consecutive patients with brain metastases/primary CNS lymphomas. FA and ADC values were measured in regions of interest (ROIs) placed in the contrast-enhancing tumor part, the necrosis and the non-enhancing peritumoral region (NEPTR). Differences between histopathological subtypes of metastases were analyzed using non-parametric tests, decision trees and hierarchical clustering analysis. Significant differences were found in morphologic criteria such as hemorrhage or pattern of contrast enhancement. In diffusion measurements, significant differences between the different tumor entities were only found in ADC analyzed in the contrast-enhancing tumor part. Among single tumor entities, primary CNS lymphomas showed significantly lower median ADC values in the contrast-enhancing tumor part (ADC{sub lymphoma} 0.92 [0.83-1.07] vs. ADC{sub no} {sub lymphoma} 1.35 [1.10-1.64] P=0.001). Further differentiation between types of metastases was not possible using FA and ADC. There were morphologic differences among the main subtypes of brain metastases/CNS lymphomas. However, due to a high variability of common types of metastases and low specificity, prospective differentiation remained challenging. DTI including FA and ADC was not a reliable tool for differentiation between different histopathological subtypes of brain metastases except for CNS lymphomas showing lower ADC values. Biopsy, surgery and staging remain essential for diagnosis.

  3. Primary Adrenal Lymphoma Infiltrating in to Pancreas: A Rare Cause of Adrenomegaly

    Directory of Open Access Journals (Sweden)

    Lovelesh Kumar Nigam

    2017-03-01

    Full Text Available Primary adrenal lymphoma is a rare entity and may be suspected in patients having bilateral adrenal masses, with/without lymphadenopathy, and with/without adrenal insufficiency. We report a rare case of a 45-year-old man who presented with pain in the abdomen, with no signs of adrenal insufficiency and bilateral adrenal masses on imaging. Light microscopy findings with immunohistochemistry and flow cytometry confirmed the diagnosis of diffuse large B-cell lymphoma. The patient was offered cyclophosphamide, adriamycin, vincristine, and prednisolone chemotherapy regimen and doing well till the last follow-up. [J Interdiscip Histopathol 2017; 5(1.000: 25-28

  4. Differentiation of Enhancing Glioma and Primary Central Nervous System Lymphoma by Texture-Based Machine Learning.

    Science.gov (United States)

    Alcaide-Leon, P; Dufort, P; Geraldo, A F; Alshafai, L; Maralani, P J; Spears, J; Bharatha, A

    2017-06-01

    Accurate preoperative differentiation of primary central nervous system lymphoma and enhancing glioma is essential to avoid unnecessary neurosurgical resection in patients with primary central nervous system lymphoma. The purpose of the study was to evaluate the diagnostic performance of a machine-learning algorithm by using texture analysis of contrast-enhanced T1-weighted images for differentiation of primary central nervous system lymphoma and enhancing glioma. Seventy-one adult patients with enhancing gliomas and 35 adult patients with primary central nervous system lymphomas were included. The tumors were manually contoured on contrast-enhanced T1WI, and the resulting volumes of interest were mined for textural features and subjected to a support vector machine-based machine-learning protocol. Three readers classified the tumors independently on contrast-enhanced T1WI. Areas under the receiver operating characteristic curves were estimated for each reader and for the support vector machine classifier. A noninferiority test for diagnostic accuracy based on paired areas under the receiver operating characteristic curve was performed with a noninferiority margin of 0.15. The mean areas under the receiver operating characteristic curve were 0.877 (95% CI, 0.798-0.955) for the support vector machine classifier; 0.878 (95% CI, 0.807-0.949) for reader 1; 0.899 (95% CI, 0.833-0.966) for reader 2; and 0.845 (95% CI, 0.757-0.933) for reader 3. The mean area under the receiver operating characteristic curve of the support vector machine classifier was significantly noninferior to the mean area under the curve of reader 1 ( P = .021), reader 2 ( P = .035), and reader 3 ( P = .007). Support vector machine classification based on textural features of contrast-enhanced T1WI is noninferior to expert human evaluation in the differentiation of primary central nervous system lymphoma and enhancing glioma. © 2017 by American Journal of Neuroradiology.

  5. Treatment of Primary Cutaneous CD4 Small/Medium T cell Lymphoproliferative Disorder with Intralesional Triamcinolone Acetonide.

    Science.gov (United States)

    2018-02-15

    12. REPORT TYPE 02/15/2018 Poster 4. TITLE AND SUBTITLE Treatment of Primary Cutaneous CD4+ Small/Medium T- cell Lymphoproliferative Disorder with...cutaneous CD4+ small/medium T- cell lymphoproliferative disorder (LPD) is a generally indolent cutaneous T- cell proliferation. Most cases follow a benign...lmmunohistochemistry showed diffuse CD3+ CD4+ T- cells without CD30, TIA1 or CD10. A subset of medium to large cells expressed BCL-6. Small subsets of B- cells and CDB

  6. Primary hepatic peripheral T-cell lymphoma mimicking hepatocellular carcinoma: a case report.

    Science.gov (United States)

    Lee, Jisun; Park, Kil Sun; Kang, Min Ho; Kim, Yook; Son, Seung-Myoung; Choi, Hanlim; Choi, Jae-Woon; Ryu, Dong Hee

    2017-08-01

    Peripheral T-cell lymphomas (PTCLs) are aggressive neoplasms which may involve the liver. The imaging manifestations of hepatic lymphoma are highly variable and show overlapping appearances of numerous other hepatic diseases. As the management and prognosis of lymphoma differ markedly from those of other malignant diseases, prompt diagnosis and early effective treatment are very important. Here, we report an atypical case of primary PTCL not otherwise specified involving the liver that exhibited a solitary hepatic mass mimicking hepatocellular carcinoma (HCC) on CT. Liver biopsy is not commonly recommended in highly suspicious cases of HCC. However, in a patient without risk factors for HCC, consideration of other diagnostic possibilities is required and needle biopsy may be a more rational choice. An imaging approach, based on a careful review of clinical and laboratory findings is essential to prevent false-positive diagnosis of HCC and subsequent invasive treatment.

  7. A virtual tissue bank for primary central nervous system lymphomas in immunocompetent individuals.

    Science.gov (United States)

    Ponzoni, Maurilio; Kwee, Ivo; Mazzucchelli, Luca; Ferreri, Andrés J M; Zucca, Emanuele; Doglioni, Claudio; Cavalli, Franco; Bertoni, Francesco

    2007-01-01

    Primary central nervous system lymphoma (PCNSL) is a rare form of extranodal non-Hodgkin's lymphoma with continuously increasing incidence in both immunosuppressed and immunocompetent individuals. PCNSL is a very aggressive tumor with a poor outcome, and its clinical outcome is much worse than for nodal lymphomas. Differently from lymphomas arising in lymph nodes or in other extranodal sites, the treatment of PCNSL remains very unsatisfactory. Current biologic knowledge of PCNSL is still limited and several fundamental questions remain to be answered. This is mainly due to the paucity of PCNSL material for adequate translational research. With the aim of providing biologic material to investigators interested in PCNSL, we have implemented a virtual tissue bank (VTB) for PCNSL in immunocompetent patients. After registration, the VTB is accessible via any web browser at www.ielsg.org. Only anonymous data are centralized at the website of the International Extranodal Lymphoma Study Group, whilst the pathologic material is maintained at the local pathology institutes. (c) 2007 S. Karger AG, Basel.

  8. Primary hepatic lymphoma presenting as fulminant hepatic failure with hyperferritinemia: A case report

    Directory of Open Access Journals (Sweden)

    Haider Fyeza S

    2008-08-01

    Full Text Available Abstract Introduction Primary hepatic lymphoma is an unusual form of non-Hodgkin's lymphoma that usually presents with constitutional symptoms, hepatomegaly and signs of cholestatic jaundice. Diffuse hepatic infiltration is uncommon and presentation with acute hepatic failure even more rare. The presence of markedly elevated ferritin levels can complicate the evaluation process and suggest alternative diagnoses. We present the case of a middle-aged woman exhibiting pancytopenia, hyperferritinemia and rapidly deteriorating to develop acute hepatic failure. Her initial clinical picture led to a working diagnosis of adult onset Still's disease with probable hemophagocytic syndrome before her worsening liver function necessitated a percutaneous liver biopsy and establishment of the final diagnosis of primary hepatic lymphoma. Conclusion Primary hepatic lymphoma is an uncommon malignancy and its manifestation as progressive hepatitis or acute fulminant hepatic failure can be difficult to diagnose. The presence of constitutional symptoms, pancytopenia and high ferritin levels can complicate the evaluation process. A liver biopsy early in the course of liver dysfunction may establish the diagnosis without a higher risk of bleeding complications seen once liver failure sets in.

  9. Primary bony non-Hodgkin lymphoma of the cervical spine: a case report

    Directory of Open Access Journals (Sweden)

    Sedrak Mark F

    2010-02-01

    Full Text Available Abstract Introduction Non-Hodgkin lymphoma primarily originating from the bone is exceedingly rare. To our knowledge, this is the first report of primary bone lymphoma presenting with progressive cord compression from an origin in the cervical spine. Herein, we discuss the unusual location in this case, the presenting symptoms, and the management of this disease. Case presentation We report on a 23-year-old Caucasian-American man who presented with two months of night sweats, fatigue, parasthesias, and progressive weakness that had progressed to near quadriplegia. Magnetic resonance (MR imaging demonstrated significant cord compression seen primarily at C7. Surgical management, with corpectomy and dorsal segmental fusion, in combination with adjuvant chemotherapy and radiation therapy, halted the progression of the primary disease and preserved neurological function. Histological analysis demonstrated an aggressive anaplastic large cell lymphoma. Conclusion Isolated primary bony lymphoma of the spine is exceedingly rare. As in our case, the initial symptoms may be the result of progressive cervical cord compression. Anterior corpectomy with posterolateral decompression and fusion succeeded in preventing progressive neurologic decline and maintaining quality of life. The reader should be aware of the unique presentation of this disease and that surgical management is a successful treatment strategy.

  10. Sarcomas cutâneos primários Primary cutaneous sarcomas

    Directory of Open Access Journals (Sweden)

    Luiz Fernando Fróes Fleury Jr

    2006-06-01

    Full Text Available Os sarcomas com apresentação cutânea primária são tumores raros e de grande heterogeneidade histológica. Com a evolução da oncologia cutânea e da cirurgia dermatológica, os dermatologistas têm sido cada vez mais requisitados para o diagnóstico e orientação terapêutica de tumores menos freqüentes. Este artigo de revisão analisa os sarcomas cutâneos primários observando suas características clínicas, etiopatogênicas e histológicas, bem como aspectos do tratamento e evolução. Enfatiza os sarcomas de maior relevância para o dermatologista, como angiossarcoma, dermatofibrossarcoma protuberans, fibroxantoma atípico, leiomiossarcoma, lipossarcoma, tumor maligno de bainha de nervo periférico e sarcoma epitelióide. O sarcoma de Kaposi não é abordado devido a suas características individuais específicas.Soft tissue tumors represent a heterogeneous group of mesenchymal and neural lesions. The cutaneous presentation of these tumours is rare. With the evolution of dermatologic surgery and cutaneous oncology, dermatologists have emerged as specialists for skin cancer management. This article reviews primary cutaneous sarcomas with particular emphasis on the epidemiologic, clinical, and histological features of diagnosis, as well as treatment modalities and prognosis. The most frequent cutaneous sarcomas were reviewed, including angiosarcoma, dermatofibrosarcoma protuberans, atypical fibroxanthoma, leiomyosarcoma, liposarcoma, malignant nerve sheath tumor, and epithelioid sarcoma. Kaposi's sarcoma, due to specific characteristics, was omitted from this review.

  11. [Primary non-Hodgkin's lymphoma of the breast. A case report].

    Science.gov (United States)

    Villalón-López, José Sebastián; Souto-Del Bosque, Rosalía; Méndez-Sashida, Pedro Gonzalo

    Primary breast lymphomas, a rare subtype of non-Hodgkin's lymphoma, represent 0.04 to 0.5% of all breast cancers, 0.38 to 0.7% of all lymphomas, and 1.7 to 2.2% of extranodal lymphomas. The treatment choice is based on chemotherapy containing anthracycline and rituximab. Surgery is limited to being less invasive and only for diagnostic purposes. Radiotherapy has an important role as consolidation therapy, particularly in patients with negative nodes. A 70 year old woman with a breast nodule in the left upper outer quadrant, with slow growth, expansive, painless, and accompanied by skin changes, malaise, weight loss, fatigue, chill, and sweating. There was tissue replacement by the mammary gland tumour, skin changes due to invasion, and a 5cm axillary lymphadenopathy. The mammography showed skin thickening and a dense pattern of 80% of breast tissue replacement, and the lymphadenopathy with loss of radiolucent centre and soft tissue invasion. The biopsy confirmed a diffuse high grade large cell lymphoma. She received an Rituximab (R-CHOP) chemotherapy scheme and radiotherapy with tangential and supraclavicular and axillary fields. After completing the chemotherapy, the patient is on follow-up, and at 15 months she is alive without disease activity. Primary lymphoma of the breast is a rare entity. Multimodal treatment with combined chemo-radiotherapy is the cornerstone. Surgery is reserved only for diagnostic purposes. Copyright © 2015 Academia Mexicana de Cirugía A.C. Publicado por Masson Doyma México S.A. All rights reserved.

  12. Analysis of image findings in forty-one patients with primary lymphoma of the bone

    International Nuclear Information System (INIS)

    Yu Baohai; Liu Jie; Zhong Zhiwei; Zhao Jingpin; Peng Zhigang; Liu Jicun; Wu Wenjuan

    2011-01-01

    Objective: To analyze the imaging features of primary lymphoma of the bone, and discuss the special feature of the 'floating ice sign'. Methods: Forty-one cases of primary lymphoma of the bone in our unit from 1963.1-2009.6 were retrospectively studied. All 41 patients underwent X-ray examination, and 20 patients underwent CT examination, 12 patients underwent MR examination (3 cases simultaneously with enhancement). Results: Involvement of the flat bone was seen in 12 cases. Vertebral column was affected in 8 cases, and 17 cases showed lesions in long bones and irregular bones were involved in 4 cases. The most common location was the femur (10, 24.4%), followed by the ilium (8, 19.5%). Lesions were found in the metaphyses of the long bone in 11 cases (64.7%). 'Floating ice sign' was showed in the calcaneus of 2 patients and in the lumbar vertebra of 2 cases respectively, accounted for 9.8% of all cases. Slight bone destruction with soft tissue mass on CT image could be found in 12 cases, while obvious soft tissue mass was found in 9 cases. No periosteal reaction was found in 37 cases (90.2%). MRI examinations of 12 patients revealed soft tissue mass in 10 patients, and the extent of the lesion was larger in MR than CT. One case showed extensive bone destruction on MR but inconspicuous bone destruction on X-ray plain film and CT scan. Conclusion: Slight bone destruction with conspicuous soft tissue mass, conspicuous bone destruction on MR but slight or inconspicuous bone destruction on X-ray film and CT, could strongly imply the diagnosis of primary lymphoma of the bone. 'Floating ice sign' was a special imaging feature of primary lymphoma of the bone, which could be used as a clue for the diagnosis of lymphoma. (authors)

  13. Primary cutaneous aspergillosis due to Aspergillus niger in an immunocompetent patient

    Directory of Open Access Journals (Sweden)

    Mohapatra S

    2009-01-01

    Full Text Available Primary cutaneous aspergillosis is a rare entity, usually caused by A. fumigatus and A. flavus . Here, we present such a case, manifested by ulceration due to A. niger, which remained undiagnosed for a prolonged period. The immunological status was intact, although the patient had associated severe fungal infection. Recurrence of the lesion occurred despite repeated anti-fungal therapies. Anti fungal testing was done based on the broth dilution (M-38A, NCCLS, USA method. The culture isolate was found to be sensitive to fluconazole and amphotericin B. Continuation of antifungal therapy improved the symptoms, reducing the size of the lesion.

  14. PET/CT presentation of primary effusion lymphoma-like lymphoma unrelated to human herpes virus 8, a rare NHL subtype

    International Nuclear Information System (INIS)

    Patil, Vivek V; Sideras, Panagiotis; Machac, Josef

    2014-01-01

    We present a 71-year-old female with human herpes virus 8 (HHV8)-unrelated primary effusion lymphoma (PEL)-like lymphoma. Dyspnea and pericardial effusion led to pericardiocentesis, diagnosing diffuse large B-cell lymphoma. She underwent positron emission tomography/computed tomography (PET/CT), which demonstrated hypermetabolic pericardial, pleural, and ascites fluid without lymphadenopathy elsewhere. Malignant fluid in the absence of lymphadenopathy is a hallmark of PEL. PEL is associated with immunodeficiency states such as acquired immunodeficiency syndrome (AIDS) and infectious agents such as HHV8. Our patient had no such history and had not received immunosuppressive chemotherapy. We present the PET/CT findings of this rare case of HHV8-unrelated PEL-like lymphoma

  15. Isolated orbital mass as the primary presentation of a triple-hit lymphoma transformed from a systemic follicular lymphoma

    Directory of Open Access Journals (Sweden)

    Xiao Yi Zhou

    2018-06-01

    Full Text Available Purpose: Triple-hit lymphoma is a highly aggressive B-cell lymphoma. We report a case of triple-hit lymphoma transformed from systemic follicular lymphoma (FL after 9-year remission and presented primarily as an isolated orbital mass without systemic symptoms or lymphadenopathy. Observations: A 58-year-old female presented with intermittent vertical binocular diplopia, left upper eyelid swelling and pain and was found to have a 2.9 cm orbital mass. Histological section revealed a CD10-positive large B-cell lymphoma, consistent with transformation of FL. Fluorescent in situ hybridization (FISH analysis demonstrated rearrangements involving C-MYC, BCL-2 and BCL-6 genes, indicating a high grade, triple-hit lymphoma. Conclusions and importance: Triple-hit lymphoma transformed from a low-grade lymphoma may initially present as an isolated orbital mass without systemic evidence of transformation. Early recognition of double or triple-hit lymphomas is important since these patients require aggressive chemotherapy. Keywords: Lymphoma, Triple-hit lymphoma, Orbital mass

  16. Primary non-Hodgkin lymphoma of the cecum. A case report

    International Nuclear Information System (INIS)

    Kropivnik, M.; Jamar, B.; Cernelc, B.

    2002-01-01

    Background. Primary lymphoma of the colon is rare, constituting 0.4% of primary colonic malignancies and usually involves cecum or rectum. The aim of this paper is to present the role and the importance of double contrast barium enema (DCBE) in the diagnostic process. Case report. A 77 years old male was admitted because of suspected inflammation in the area of total endoprosthesis of the left hip, inserted ten years before. Listeria monocytogenes was isolated from the aspirate and the patient treated with antibiotics. Twenty years ago the patient underwent nephrectomy because of hypernephroma of left kidney. At the time of admission he had sideropenic anaemia and he was febrile. Conclusion. The patient underwent many diagnostic procedures: ultrasound (US), computed tomography (CT), double contrast barium enema, which showed a tumour in the cecum, small bowel follow-through and scintigraphy. The diagnosis of primary non-Hodgkin lymphoma was established by histology after biopsy at colonoscopy. (author)

  17. White-matter abnormalities in unirradiated patients cured of primary central nervous system lymphoma

    International Nuclear Information System (INIS)

    Kim, L.; Hochberg, F.H.; Shaeffer, P.

    2000-01-01

    On MRI, primary brain tumors are commonly seen as contrast-enhancing masses surrounded by areas of abnormal signal on T2-weighted images. Following successful treatment tumors may no longer show contrast enhancement. The residual abnormalities are assumed to be represent ''edema'' and infiltrating tumor cells. We report nine patients with primary lymphoma of the central nervous system who had complete responses to intravenous methotrexate, but did not receive intrathecal chemotherapy or cranial irradiation. After complete resolution of contrast-enhancing lesions, persistent abnormalities on T2-weighted images in the region of prior tumor were initially assumed to reflect residual viable tumor. As they remained unchanged for years, however, this may not hold true in the cases in which primary central nervous system lymphoma responds to chemotherapy alone. (orig.)

  18. Primary NK/T cell lymphoma nasal type of the stomach with skin involvement: a case report

    Directory of Open Access Journals (Sweden)

    Sebastian Kobold

    2009-12-01

    Full Text Available Since nasal NK/T cell lymphoma and NK/T cell lymphoma nasal type are rare diseases, gastric involvement has seldom been seen. We report a unique case of a patient with a primary NK/T cell lymphoma nasal type of the stomach with skin involvement. The patient had no history of malignant diseases and was diagnosed with hematemesis and intense bleeding from his gastric primary site. Shortly after this event, exanthemic skin lesions appeared with concordant histology to the primary site. Despite chemotherapy, the patient died one month after the first symptomatic appearance of disease.

  19. Magnetic resonance features of primary central nervous system lymphoma in the immunocompetent patient: a pictorial essay

    International Nuclear Information System (INIS)

    Yap, Kelvin K.; Sutherland, Tom; Liew, Elain; Tartaglia, Con J.; Trost, Nick; Pang, Mei

    2012-01-01

    Primary central nervous system lymphoma (PCNSL) is an uncommon but important variant of non-Hodgkin lymphoma and represents up to 6% of all primary central nervous system (CNS) malignancies. Recognition of this entity by radiologist on MRI may avoid unnecessary neurosurgical resection and redirect to biopsy. The pretreatment MRI of patients with biopsy proven PCNSL from the last 5 years at our institution was reviewed. Selected examples were used to construct a pictorial essay to illustrate some of the typical and atypical MR features of PCNSL. MRI of other CNS conditions with imaging similarities to PCNSL was included to demonstrate possible mimics. The typical features of PCNSL lymphoma are intra-axial homogenous single or multiple contrast enhancing lesions, with marked surrounding oedema and restricted diffusion, usually contacting a cerebrospinal fluid (CSF) surface. Necrosis, peripheral enhancement, haemorrhage or calcification are unusual and other diagnoses should be considered if any of these features are present. Potential mimics include high grade glioma, infarcts, metastatic disease, demyelination, abscess and secondary lymphoma. Careful assessment of the MR features and correlation with the clinical findings should enable the radiologists to raise the possibility of PCNSL and minimise the risk of unnecessary resection.

  20. A Case Report of Primary B–Cell Lymphoma of the Urinary Bladder

    Directory of Open Access Journals (Sweden)

    M. Abbasi

    2004-07-01

    Full Text Available Primary malignant lymphoma of the urinary bladder is very rare. Less than 100 cases have been reported. The best treatment approach for this disease remained unknown.In this article we reported a 41-year-old-female who was admitted to Sina hospital with the chief complaint of macrohematuria that was followed by dysuria , frequency , noturia and urgency. Other examinations were normal and there was no organomegaly and lymphadenopathy.In ultrasonography the thickening of trigone zone of the urinary bladder was reported. The patient underwent a transurethral biopsy of the bladder that revealed malignant lymphoma , intermediate grade , diffuse mixed small and large cell type ( B-cell lymphoma. The reports of computed tomography scan of the thorax , abdomen and pelvis and bonemarrow biopsy were normal and results of metastatic work up were negative.Primary lymphoma of the urinary bladder was diagnosed and a combination of systemic chemotherapy and relatively low dose irradiation were done for the patient. The patient is in complete remission with this kind of treatment now.

  1. Transcriptome patterns from primary cutaneous Leishmania braziliensis infections associate with eventual development of mucosal disease in humans.

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    Ana Claudia Maretti-Mira

    Full Text Available INTRODUCTION: Localized Cutaneous Leishmaniasis (LCL and Mucosal Leishmaniasis (ML are two extreme clinical forms of American Tegumentary Leishmaniasis that usually begin as solitary primary cutaneous lesions. Host and parasite factors that influence the progression of LCL to ML are not completely understood. In this manuscript, we compare the gene expression profiles of primary cutaneous lesions from patients who eventually developed ML to those that did not. METHODS: Using RNA-seq, we analyzed both the human and Leishmania transcriptomes in primary cutaneous lesions. RESULTS: Limited number of reads mapping to Leishmania transcripts were obtained. For human transcripts, compared to ML patients, lesions from LCL patients displayed a general multi-polarization of the adaptive immune response and showed up-regulation of genes involved in chemoattraction of innate immune cells and in antigen presentation. We also identified a potential transcriptional signature in the primary lesions that may predict long-term disease outcome. CONCLUSIONS: We were able to simultaneously sequence both human and Leishmania mRNA transcripts in primary cutaneous leishmaniasis lesions. Our results suggest an intrinsic difference in the immune capacity of LCL and ML patients. The findings correlate the complete cure of L. braziliensis infection with a controlled inflammatory response and a balanced activation of innate and adaptive immunity.

  2. Low-dose (10-Gy) total skin electron beam therapy for cutaneous T-cell lymphoma: an open clinical study and pooled data analysis.

    Science.gov (United States)

    Kamstrup, Maria R; Gniadecki, Robert; Iversen, Lars; Skov, Lone; Petersen, Peter Meidahl; Loft, Annika; Specht, Lena

    2015-05-01

    Cutaneous T-cell lymphomas (CTCLs) are dominated by mycosis fungoides (MF) and Sézary syndrome (SS), and durable disease control is a therapeutic challenge. Standard total skin electron beam therapy (TSEBT) is an effective skin-directed therapy, but the possibility of retreatments is limited to 2 to 3 courses in a lifetime due to skin toxicity. This study aimed to determine the clinical effect of low-dose TSEBT in patients with MF and SS. In an open clinical study, 21 patients with MF/SS stages IB to IV were treated with low-dose TSEBT over dose of 10 Gy in 10 fractions. Data from 10 of these patients were published previously but were included in the current pooled data analysis. Outcome measures were response rate, duration of response, and toxicity. The overall response rate was 95% with a complete cutaneous response or a very good partial response rate (dose (10-Gy) TSEBT offers a high overall response rate and is relatively safe. With this approach, reirradiation at times of relapse or progression is likely to be less toxic than standard dose TSEBT. It remains to be established whether adjuvant and combination treatments can prolong the beneficial effects of low-dose TSEBT. Copyright © 2015 Elsevier Inc. All rights reserved.

  3. Low-Dose Total Skin Electron Beam Therapy as a Debulking Agent for Cutaneous T-Cell Lymphoma: An open-label prospective phase II study

    DEFF Research Database (Denmark)

    Kamstrup, M R; Lindahl, Lise Maria; Gniadecki, R

    2012-01-01

    Background: Total skin electron beam therapy (TSEBT) is a powerful treatment for cutaneous T-cell lymphomas (CTCL). Based on the occurrence of relapses with low radiation doses, doses of 30-36 Gy are commonly used but most patients still eventually relapse and repeat treatment courses are limited...... due to the cumulative toxicity. Complete response rates are about 60-90% for T2-4 stages with a 5-year relapse-free survival of 10-25% for stages IB-III. Objectives: To evaluate prospectively the efficacy of low-dose TSEBT (10 Gy) in terms of complete cutaneous response rate, overall response rate...... and response duration in CTCL. Methods: Ten patients with stage IB-IV mycosis fungoides (MF) were treated in an open-label manner with 4 fractions of 1 Gy/week TSEB to a total skin dose of 10 Gy. Treatment responses were assessed at 1 and 3 months after treatment and subsequently at least every 6 months...

  4. Low-Dose (10-Gy) Total Skin Electron Beam Therapy for Cutaneous T-Cell Lymphoma: An Open Clinical Study and Pooled Data Analysis

    Energy Technology Data Exchange (ETDEWEB)

    Kamstrup, Maria R., E-mail: mkam0004@bbh.regionh.dk [Department of Dermatology, Bispebjerg Hospital, University of Copenhagen, Copenhagen (Denmark); Gniadecki, Robert [Department of Dermatology, Bispebjerg Hospital, University of Copenhagen, Copenhagen (Denmark); Iversen, Lars [Department of Dermatology, Aarhus University Hospital, Aarhus (Denmark); Skov, Lone [Department of Dermatology, Gentofte Hospital, University of Copenhagen, Copenhagen (Denmark); Petersen, Peter Meidahl [Department of Oncology and Hematology, Rigshospitalet, University of Copenhagen, Copenhagen (Denmark); Loft, Annika [Department of Clinical Physiology, Nuclear Medicine and PET, Rigshospitalet, University of Copenhagen, Copenhagen (Denmark); Specht, Lena [Department of Oncology and Hematology, Rigshospitalet, University of Copenhagen, Copenhagen (Denmark)

    2015-05-01

    Purpose: Cutaneous T-cell lymphomas (CTCLs) are dominated by mycosis fungoides (MF) and Sézary syndrome (SS), and durable disease control is a therapeutic challenge. Standard total skin electron beam therapy (TSEBT) is an effective skin-directed therapy, but the possibility of retreatments is limited to 2 to 3 courses in a lifetime due to skin toxicity. This study aimed to determine the clinical effect of low-dose TSEBT in patients with MF and SS. Methods and Materials: In an open clinical study, 21 patients with MF/SS stages IB to IV were treated with low-dose TSEBT over <2.5 weeks, receiving a total dose of 10 Gy in 10 fractions. Data from 10 of these patients were published previously but were included in the current pooled data analysis. Outcome measures were response rate, duration of response, and toxicity. Results: The overall response rate was 95% with a complete cutaneous response or a very good partial response rate (<1% skin involvement with patches or plaques) documented in 57% of the patients. Median duration of overall cutaneous response was 174 days (5.8 months; range: 60-675 days). TSEBT-related acute adverse events (grade 1 or 2) were observed in 60% of patients. Conclusions: Low-dose (10-Gy) TSEBT offers a high overall response rate and is relatively safe. With this approach, reirradiation at times of relapse or progression is likely to be less toxic than standard dose TSEBT. It remains to be established whether adjuvant and combination treatments can prolong the beneficial effects of low-dose TSEBT.

  5. Potential role of enzastaurin in the treatment of patients with relapsed or refractory advanced cutaneous T-cell lymphomas: a review

    Directory of Open Access Journals (Sweden)

    Katz DA

    2012-06-01

    Full Text Available Deborah A Katz, Janet MD Plate, Sunita Nathan, Lydia UshaDivision of Hematology and Oncology, Rush University Medical Center, Chicago, IL, USAAbstract: Cutaneous T-cell lymphomas (CTCLs are rare extranodal non-Hodgkin lymphomas characterized by neoplastic T-lymphocyte accumulation in the skin. The two most common types of CTCLs are mycosis fungoides and the leukemic variant, Sézary syndrome. Prognosis of CTCLs depends on the stage, with a poor prognosis in advanced-stage disease. A number of agents have recently been developed for the treatment of CTCLs: chemotherapeutic agents such as pralatrexate, interferon-alpha, retinoids such as bexarotene, monoclonal antibodies such as alemtuzumab, and histone deacetylase inhibitors such as vorinostat and romidepsin. Nevertheless, there is no cure for CTCLs except for allogeneic stem cell transplant. A promising new drug is enzastaurin. Enzastaurin is a novel serine/threonine kinase inhibitor that binds to protein kinase C-β (PKC-β and inhibits the phosphoinositide-3 kinase (PI3K/AKT/phosphatase and tensin homolog (PTEN signaling pathway. Enzastaurin induces apoptosis and inhibits angiogenesis; it was also shown to suppress growth of CTCL cell lines in vitro. Given its low toxicity, enzastaurin has been tested against both solid tumors and hematologic malignancies. This article is focused on the potential role of enzastaurin in the treatment of CTCLs. A phase II multicenter trial evaluated enzastaurin monotherapy in patients with CTCLs. However, the results from this study were disappointing, demonstrating that enzastaurin had only modest clinical activity. Hence, enzastaurin is not currently developed for treating CTCLs. Potential strategies to improve enzastaurin efficacy against CTCLs are discussed: validation of enzastaurin targets such as PKC-β expression in CTCL lesions and or/blood; measurement of serum vascular endothelial growth factor levels; dose optimization; combining enzastaurin with

  6. Review of epidemiology, clinical presentation, diagnosis, and treatment of common primary psychiatric causes of cutaneous disease.

    Science.gov (United States)

    Krooks, J A; Weatherall, A G; Holland, P J

    2018-06-01

    Approximately half of all patients presenting to dermatologists exhibit signs and symptoms of psychiatric conditions that are either primary or secondary to cutaneous disease. Because patients typically resist psychiatric consult, dermatologists often are on the front line in evaluating and treating these patients. Accordingly, distinguishing the specific underlying or resulting psychiatric condition is essential for effective treatment. The etiology, epidemiology, clinical presentation, diagnosis, and first-line treatment of specific primary psychiatric causes of dermatologic conditions, including delusional infestation, Morgellons syndrome, olfactory reference syndrome, body dysmorphic disorder, excoriation disorder, trichotillomania, and dermatitis artefacta are discussed here, followed by a discussion of the recommended treatment approach with an overview of the different first-line therapies discussed in this review, specifically cognitive behavioral therapy, atypical antipsychotics, selective serotonin reuptake inhibitors, and tricyclic antidepressants. Included is a guide for dermatologists to use while prescribing these medications.

  7. Primary mucosa-associated lymphoid tissue (MALT) lymphoma of thyroid gland arising from coexisting Hashimoto's thyroiditis: a case report

    International Nuclear Information System (INIS)

    Lee, Sang Kwon; Kwon, Sun Young; Kim, Young Hwan; Choi, Jin Soo; Sohn, Chul Ho; Lee, Hee Jung; Woo, Seong Ku; Suh, Soo Ji

    2006-01-01

    We report herein on a case of primary mucosa-associated lymphoid tissue (MALT) lymphoma of the thyroid gland in a 57-year-old woman with coexisting Hashimoto's thyroiditis, and we include its characteristic imaging, histopathologic and immunohistochemical findings

  8. Primary Non-Hodgkin's lymphoma of the tongue: A rare presentation

    Directory of Open Access Journals (Sweden)

    Lavanya Karanam

    2016-01-01

    Full Text Available The head and neck is the second most common region for extranodal lymphomas. The most common site is the Waldeyer's ring, and involvement of the base of tongue is extremely rare. We present a rare case of a young female with primary non-Hodgkin's lymphoma (NHL of the base of tongue. A 23-year-old female presented with a history of foreign body sensation in her throat for a month. Oral examination revealed a lobulated smooth mass at the base of tongue. Contrast-enhanced computed tomography neck shows polypoidal homogeneously enhancing soft tissue lesion in the base of tongue extending till the lateral pharyngeal wall. The biopsy of the lesion was reported as NHL. Hodgkin's lymphoma should be kept in the differential diagnosis of swelling arising from the base of tongue. We report a rare and varied presentation of extranodal lymphoma. A careful clinical evaluation supported by histopathological and radiologic investigations will help in identifying the disease at an early stage, resulting in a better prognosis.

  9. Diagnostic value of kinetic analysis using dynamic FDG PET in immunocompetent patients with primary CNS lymphoma

    International Nuclear Information System (INIS)

    Nishiyama, Yoshihiro; Yamamoto, Yuka; Monden, Toshihide; Sasakawa, Yasuhiro; Satoh, Katashi; Ohkawa, Motoomi; Kawai, Nobuyuki

    2007-01-01

    The purpose of this study was to investigate the accumulation of FDG in immunocompetent patients with primary central nervous system (CNS) lymphoma using qualitative and quantitative PET images and to compare baseline with follow-up PET after therapy. Twelve immunocompetent patients with CNS lymphoma were examined. Dynamic emission data were acquired for 60 min immediately following injection of FDG. In seven patients, repeated PET studies were performed after treatment. Applying a three-compartment five-parameter model, K 1 , k 2 , k 3 , k 4 , vascular fraction (V B ) and cerebral metabolic rate of glucose (CMR Glc ) were obtained. We evaluated the FDG uptake visually using qualitative and parametric images and quantitatively using parametric images. A total of 12 lesions were identified in ten patients with newly diagnosed CNS lymphoma. On visual analysis, ten lesions showed an increase on qualitative images, eight showed an increase on K 1 images, 12 showed an increase on k 3 images and ten showed an increase on CMR Glc images. On quantitative analysis, k 2 , k 3 and CMR Glc values of the lesion were significantly different from those of the normal grey matter (p 3 and CMR Glc images. The K 1 , k 2 , k 3 and CMR Glc values after treatment were significantly different from those obtained before treatment (p 3 , using dynamic FDG PET might be helpful for diagnosis of CNS lymphoma and for monitoring therapeutic assessment. (orig.)

  10. MR findings of primary central nervous system lymphoma in a child

    International Nuclear Information System (INIS)

    Ciftci, E.; Erden, I.; Akyar, S.

    1998-01-01

    MR imaging of a rare case of primary central nervous system lymphoma in a 12-year-old boy with headache and seizure is presented. MR imaging showed a mass lesion located in the corpus callosum. Diagnosis was made by stereotactic biopsy. The patient was treated with radiotherapy. Early control MR imaging showed a decrease in tumor size but after 12 months an increase in tumor size was detected. (orig.)

  11. Adult T-Cell Leukemia/Lymphoma

    Science.gov (United States)

    ... Non-Hodgkin Lymphoma Peripheral T-Cell Lymphoma Primary Central Nervous System Lymphoma T-Cell Lymphoma Transformed Mycosis Fungoides Waldenstrom Macroglobulinemia Young Adult Lymphoma Overview Treatment Options Relapsed/Refractory Long-term ...

  12. Bilateral Primary Adrenal Lymphoma in a 59- year-old Female

    Directory of Open Access Journals (Sweden)

    Alireza Ahmadi

    2018-02-01

    Full Text Available Occasionally, lymphoma involves the adrenal glands; however, primary adrenal lymphoma (PAL is rare and only few cases have been reported. We report a case of a 59-year-old female with primary adrenal diffuse large B-cell lymphoma (DLBCL manifested by weakness, fatigue, anorexia, and hyper pigmentation of skin. The patient initially responded to intravenous hydrocortisone in large doses by total remission of the symptoms. An abdominal computed tomography scan showed a hypodense mass in the right subdiaphragmatic space, which was suggestive of an adrenal gland tumor with adjacent liver involvement. Additionally, a smaller lesion with similar characteristics was found in the left adrenal gland. The results obtained from adrenal gland needle biopsy confirmed the diagnosis of DLBCL. Moreover, primary laboratory findings demonstrated hyponatremia, hyperkalemia, fasting blood sugar level of 153 mg/dl, and alkaline phosphatase level of 663 U/L. The mentioned symptoms and laboratory findings were indicative of adrenal insufficiency. After about 2 months, her level of consciousness decreased and urinary and fecal incontinence occurred. Therefore, brain involvement was suspected, and magnetic resonance imaging of the brain showed heterogeneous enhancement (24.8 mm in the posterior aspect of the left temporal lobe together with patchy foci of enhancement in around the ventricular areas of the brain that showed metastatic lesions of PAL. This case should remind clinicians that PAL may be a cause of adrenal incidentaloma, especially when the patient presents with the symptoms of adrenal insufficiency.

  13. Thin and thick primary cutaneous melanomas reveal distinct patterns of somatic copy number alterations.

    Science.gov (United States)

    Montagnani, Valentina; Benelli, Matteo; Apollo, Alessandro; Pescucci, Chiara; Licastro, Danilo; Urso, Carmelo; Gerlini, Gianni; Borgognoni, Lorenzo; Luzzatto, Lucio; Stecca, Barbara

    2016-05-24

    Cutaneous melanoma is one of the most aggressive type of skin tumor. Early stage melanoma can be often cured by surgery; therefore current management guidelines dictate a different approach for thin (thick (>4mm) melanomas. We have carried out whole-exome sequencing in 5 thin and 5 thick fresh-frozen primary cutaneous melanomas. Unsupervised hierarchical clustering analysis of somatic copy number alterations (SCNAs) identified two groups corresponding to thin and thick melanomas. The most striking difference between them was the much greater abundance of SCNAs in thick melanomas, whereas mutation frequency did not significantly change between the two groups. We found novel mutations and focal SCNAs in genes that are embryonic regulators of axon guidance, predominantly in thick melanomas. Analysis of publicly available microarray datasets provided further support for a potential role of Ephrin receptors in melanoma progression. In addition, we have identified a set of SCNAs, including amplification of BRAF and ofthe epigenetic modifier EZH2, that are specific for the group of thick melanomas that developed metastasis during the follow-up. Our data suggest that mutations occur early during melanoma development, whereas SCNAs might be involved in melanoma progression.

  14. J chain and myocyte enhancer factor 2B are useful in differentiating classical Hodgkin lymphoma from nodular lymphocyte predominant Hodgkin lymphoma and primary mediastinal large B-cell lymphoma.

    Science.gov (United States)

    Moore, Erika M; Swerdlow, Steven H; Gibson, Sarah E

    2017-10-01

    Although most classical Hodgkin lymphomas (CHLs) are easily distinguished from nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) and primary mediastinal large B-cell lymphoma (PMBL), cases with significant CD20 expression cause diagnostic confusion. Although the absence of OCT-2 and BOB.1 are useful in these circumstances, a variable proportion of CHLs are positive for these antigens. We investigated the utility of J chain and myocyte enhancer factor 2B (MEF2B) in the diagnosis of CHL; NLPHL; PMBL; T-cell/histiocyte-rich large B-cell lymphoma (TCRLBL); and B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and CHL, compared with OCT-2 and BOB.1. J chain and MEF2B highlighted lymphocyte predominant (LP) cells in 20/20 (100%) NLPHLs and were negative in 43/43 (100%) CHLs. Fourteen of 15 (93%) PMBLs and 4/4 (100%) TCRLBLs were MEF2B positive, whereas 67% of PMBLs and 50% of TCRLBLs were J chain positive. Three of 3 B-cell lymphomas, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and CHL, were negative for J chain and MEF2B. J chain and MEF2B were 100% sensitive and specific for NLPHL versus CHL. MEF2B was 100% sensitive and 98% specific for PMBL versus CHL. Whereas loss of OCT-2 and/or BOB.1 expression had a sensitivity of only 86% and specificity of 100% for CHL versus NLPHL, PMBL, and TCRLBL, lack of both J chain and MEF2B expression was 100% sensitive and 97% specific. J chain and MEF2B are highly sensitive and specific markers of NLPHL versus CHL; are particularly useful in highlighting LP cells; and, with rare exception, are of greater utility than OCT-2 and BOB.1 in differentiating CHL from NLPHL and other large B-cell lymphomas. Copyright © 2017 Elsevier Inc. All rights reserved.

  15. Role of 18F-FDG PET/CT in primary brain lymphoma.

    Science.gov (United States)

    de-Bonilla-Damiá, Á; Fernández-López, R; Capote-Huelva, F J; de la Cruz-Vicente, F; Egea-Guerrero, J J; Borrego-Dorado, I

    To study the usefulness of 18 F-FDG PET/CT in the initial evaluation and in the response assessment in primary brain lymphoma. A retrospective analysis was carried out on 18 patients diagnosed with primary brain lymphoma, a histological subtype of diffuse large B-cell lymphoma, on whom an initial 18 F-FDG PET/CT and MRI was performed, with 7 of the cases being analysed after the completion of treatment in order to assess response and clinical follow up. Initial 18 F-FDG PET/CT showed 26 hypermetabolic foci, whereas 46 lesions were detected by MRI. The average SUV maximum of the lesions was 17.56 with T/N 3.55. The concordance of both tests for identifying the same number of lesions was moderate, obtaining a kappa index of 0.395 (P<.001). In the evaluation of treatment, MRI identified 16 lesions compared to 7 pathological accumulations observed by 18 F-FDG PET/CT. The concordance of both tests to assess type of response to treatment was moderate (kappa index 0.41) (P=.04). In both the initial evaluation and the assessment of the response to treatment, PET/CT led to a change strategy in 22% of patients who had lesions outside the cerebral parenchyma. MRI appears to be the method of choice for detecting brain disease in patients with primary brain lymphoma, whereas 18 F-FDG PET/CT seems to play a relevant role in the assessment of extra-cerebral disease. Copyright © 2017 Elsevier España, S.L.U. y SEMNIM. All rights reserved.

  16. Operative and conservative management of primary gastric lymphoma: interim results of a German multicenter study

    International Nuclear Information System (INIS)

    Willich, Normann A.; Reinartz, Gabriele; Horst, Ekkehard J.; Delker, Georg; Reers, Berthold; Hiddemann, Wolfgang; Tiemann, Markus; Parwaresch, Reza; Grothaus-Pinke, Bernward; Kocik, Juergen; Koch, Peter

    2000-01-01

    Purpose/Objective: Biology and appropriate management of gastrointestinal (GI) lymphomas are matters of an ongoing controversial debate. To evaluate histological features, sites of involvement and management of primary GI-lymphomas, a prospective multicentric study was initiated in 10/1992. Aim of study was the further standardization of operative and conservative treatment modalities. Materials and Methods: Study started 10/1992 and was closed 11/1996. A total of 381 evaluable patients had been accrued then. Standardized diagnostic workup included endoscopic and radiological evaluation of the complete GI-tract as well as a central histological review. Diagnosis was established after Lewin, stage classification was made after Musshoff, and histological classification was made after Isaacson. Treatment decision concerning operative or conservative management was due to the initially acting physician. Patients with resection of low grade lymphoma received total abdominal irradiation 30 Gy + 10 Gy boost to incompletely resected areas. After resection of high grade lymphoma CHOP chemotherapy (4 cycles for stage IE, 6 cycles for higher stages) after McKelvy was followed by total abdominal irradiation 30 Gy for stage IE respectively involved field irradiation 30 Gy for higher stages with 10 Gy boost to incompletely resected areas. Primary conservative treatment consisted of six cycles COP chemotherapy after Bagley for low grade lymphomas stage >IE and total abdominal irradiation 30 Gy + 10 Gy boost to involved areas for all stages. Patients with high grade lymphomas received 4 x CHOP followed by total abdominal irradiation 30 Gy + 10 Gy boost to involved areas or 6 x CHOP plus involved field radiation therapy with 40 Gy. 257 patients are considered for analysis due to exclusion criteria of the study, 190 of them were suffered from gastric lymphoma. Their median observation time is 29 months, maximum observation time is 68 months. Results: Sites of involvement were

  17. Fusion Protein Cytokine Therapy After Rituximab in Treating Patients With B-Cell Non-Hodgkin Lymphoma

    Science.gov (United States)

    2015-06-03

    Anaplastic Large Cell Lymphoma; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Marginal Zone Lymphoma; Small Intestine Lymphoma; Splenic Marginal Zone Lymphoma; Testicular Lymphoma; Waldenstrom Macroglobulinemia

  18. Lack of T-cell receptor-induced signaling is crucial for CD95 ligand up-regulation and protects cutaneous T-cell lymphoma cells from activation-induced cell death.

    Science.gov (United States)

    Klemke, Claus-Detlev; Brenner, Dirk; Weiss, Eva-Maria; Schmidt, Marc; Leverkus, Martin; Gülow, Karsten; Krammer, Peter H

    2009-05-15

    Restimulation of previously activated T cells via the T-cell receptor (TCR) leads to activation-induced cell death (AICD), which is, at least in part, dependent on the death receptor CD95 (APO-1, FAS) and its natural ligand (CD95L). Here, we characterize cutaneous T-cell lymphoma (CTCL) cells (CTCL tumor cell lines and primary CTCL tumor cells from CTCL patients) as AICD resistant. We show that CTCL cells have elevated levels of the CD95-inhibitory protein cFLIP. However, cFLIP is not responsible for CTCL AICD resistance. Instead, our data suggest that reduced TCR-proximal signaling in CTCL cells is responsible for the observed AICD resistance. CTCL cells exhibit no PLC-gamma1 activity, resulting in an impaired Ca(2+)release and reduced generation of reactive oxygen species upon TCR stimulation. Ca(2+) and ROS production are crucial for up-regulation of CD95L and reconstitution of both signals resulted in AICD sensitivity of CTCL cells. In accordance with these data, CTCL tumor cells from patients with Sézary syndrome do not up-regulate CD95L upon TCR-stimulation and are therefore resistant to AICD. These results show a novel mechanism of AICD resistance in CTCL that could have future therapeutic implications to overcome apoptosis resistance in CTCL patients.

  19. ONC201 selectively induces apoptosis in cutaneous T-cell lymphoma cells via activating pro-apoptotic integrated stress response and inactivating JAK/STAT and NF-κB pathways.

    Science.gov (United States)

    Ni, Xiao; Zhang, Xiang; Hu, Cheng-Hui; Langridge, Timothy; Tarapore, Rohinton S; Allen, Joshua E; Oster, Wolfgang; Duvic, Madeleine

    2017-09-22

    Cutaneous T-cell lymphomas (CTCLs) are extremely symptomatic and still incurable, and more effective and less toxic therapies are urgently needed. ONC201, an imipridone compound, has shown efficacy in pre-clinical studies in multiple advanced cancers. This study was to evaluate the anti-tumor activity of ONC201 on CTCL cells. The effect of ONC201 on the cell growth and apoptosis were evaluated in CTCL cell lines (n=8) and primary CD4 + malignant T cells isolated from CTCL patients (n=5). ONC201 showed a time-dependent cell growth inhibition in all treated cell lines with a concentration range of 1.25-10.0 μM. ONC201 also induced apoptosis in tested cells with a narrow concentration range of 2.5-10.0 μM, evidenced by increased Annexin V + cells, accompanied by accumulated sub-G1 portions. ONC201 only induced apoptosis in CD4 + malignant T cells, not in normal CD4 + T cells. The activating transcription factor 4 (ATF4), a hallmark of integrated stress response, was upregulated in response to ONC201 whereas Akt was downregulated. In addition, molecules in JAK/STAT and NF-κB pathways, as well as IL-32β, were downregulated following ONC201 treatment. Thus, ONC201 exerts a potent and selective anti-tumor effect on CTCL cells. Its efficacy may involve activating integrated stress response through ATF4 and inactivating JAK/STAT and NF-κB pathways.

  20. Isolated primary central nervous system lymphoma arising from the optic chiasm.

    Science.gov (United States)

    Vassal, F; Pommier, B; Boutet, C; Forest, F; Campolmi, N; Nuti, C

    2014-12-01

    A 58-year-old previously healthy woman rapidly developed progressive bilateral visual loss. Magnetic resonance imaging revealed a bulging appearance of the optic chiasm, with homogeneous enhancement after gadolinium administration, which suggested an optic glioma or inflammatory disease. In the absence of (para)clinical clues for a specific diagnosis despite extensive investigation, a biopsy of one optic nerve was performed, resulting in a diagnosis of non-Hodgkin B-cell lymphoma. There was no evidence of any other ocular or systemic involvement, therefore the conclusion was that this immunocompetent patient had a primary central nervous system lymphoma isolated in the anterior visual pathway. Treatment included two cycles of polychemotherapy (rituximab, methotrexate, carmustine, etoposide, methylprednisolone), followed by autologous peripheral blood stem cell transplantation and rituximab plus cytarabine consolidation therapy. Subsequently, the patient exhibited significant improvement in vision, and was still disease-free at the 1-year follow-up examination. The aim of the present paper was to provide well-documented clinical, radiological, and intraoperative features of isolated primary malignant lymphoma arising from the anterior visual pathway. A better recognition of this rare pathological entity is necessary for clinicians who may encounter similar presentations, as prompt management is crucial for both a visual and vital prognosis. Copyright © 2014 Elsevier Masson SAS. All rights reserved.

  1. Primary testicular diffuse large B-cell lymphoma: A case report

    Directory of Open Access Journals (Sweden)

    Muhammad Sadiq

    2017-12-01

    Full Text Available Primary testicular diffuse large-B cell lymphoma (DLBCL is an uncommon and aggressive disease with predominant manifestation in the older age. Herein, we report a case of 47-year-old male patient who presented with three months history of left testis swelling. The patient underwent unilateral (left radical orchiectomy. Histopathological examination revealed extensive involvement and replacement of testicular parenchyma by a tumor composed of large discohesive sheets of cells with pleomorphic, hyperchromatic nuclei and prominent nucleoli. Immunohistochemical (IHC staining showed reactivity for LCA & Pan B (CD20 and negativity for OCT 3/4, SALL4 and Inhibin. Moreover, Pan T (CD3 highlighted reactive T-cells. These features rendered the diagnosis of DLBCL of testis. The hybrid 2-[fluorine-18] fluoro-2-deoxy-d-glucose (FDG positron emission tomography/computed tomography (PET/CT demonstrated two para-aortic FDG avid lymph nodes on the left side at the level of L2 vertebra. Presently, the patient has been planned for doxorubicin-based chemotherapy (i.e., cyclophosphamide, doxorubicin, vincristine and prednisone; CHOP along with intrathecal Methroxate (MTX, which would presumably improve the prognosis. Our study would expand the pool of this uncommon tumor towards its better understanding. Keywords: Primary testicular lymphoma, Diffuse large-B cell lymphoma, Orchiectomy, Doxorubicin-based chemotherapy

  2. A case of primary mucosa-associated lymphoid tissue lymphoma of the vagina.

    Science.gov (United States)

    Yoshinaga, Kousuke; Akahira, Jun-Ichi; Niikura, Hitoshi; Ito, Kiyoshi; Moriya, Takuya; Murakami, Takashi; Kameoka, Jun-Ichi; Ichinohasama, Ryo; Okamura, Kunihiro; Yaegashi, Nobuo

    2004-09-01

    We report the first case of primary mucosa-associated lymphoid tissue (MALT) lymphoma of the vagina, the diagnosis of which is supported by genetic and immunophenotypic studies. A 65-year-old, para 2 woman presented to our hospital in July 1997 with a history of prolonged vaginal discharge. Although cytologic examination suggested possible malignancy, a biopsy of the vaginal wall was diagnosed as chronic inflammation. In June 2000, she underwent gynecologic examination because of anuria. Excisional biopsy revealed subepithelial infiltration of atypical lymphoid cells that stained for CD20, CD79a, and BCL-2; stained weakly for IgM; and did not stain for CD3, CD5, CD7, CD10, CD56, CD23, and IgD, suggesting marginal zone B-cell lineage. Monoclonality was detected by Southern blot analysis, and this patient was finally diagnosed as having primary MALT lymphoma of the vagina. She received 3 cycles of chemotherapy (THP-COP) and concurrent radiation to the whole pelvis. The patient is alive and well 40 months after treatment. Because the vagina is one of the mucosa-associated tissues, MALT lymphoma, though rare, must be included in the differential diagnosis of the vaginal neoplasms.

  3. The possible role of maintenance treatment for primary central nervous system lymphoma.

    Science.gov (United States)

    Bairey, Osnat; Siegal, Tali

    2018-03-11

    Primary central nervous system lymphoma (PCNSL) is a rare and aggressive brain tumor. The prognosis is poor, with high rates of relapse and disease progression after treatment. In addition, PCNSL affects a largely older population, so that a significant proportion of patients are ineligible for intensive therapies and high-dose chemotherapy. The elderly patients are also susceptible to the accelerated and detrimental cognitive side effects of whole-brain irradiation which is an alternative consolidation to high-dose chemotherapy. Maintenance therapy has been shown to be a promising strategy to prolong remission time in other hematopoietic malignancies. Herein, we discuss the place of maintenance treatment in PCNSL in view of perspective obtained from hematological malignancies and non-Hodgkin's lymphoma. Copyright © 2018 Elsevier Ltd. All rights reserved.

  4. Primary cutaneous nocardiosis caused by Nocardia brasiliensis following a wasp sting.

    Science.gov (United States)

    Chu, L; Xu, X; Ran, Y

    2017-04-10

    We report a case of an 87-year-old woman who presented with painful erythema of her right forearm 10 days after she had been stung by a wasp on her right hand. The lesion had rapidly deteriorated during the week before presentation, and treatment with antibiotics and glucocorticoids did not improve the condition. After careful evaluation, we performed cultures from the lesion aspiration, and morphological and genetic analysis of bacteria cultures confirmed a bacterial infection with Nocardia brasiliensis. The patient recovered after 3 weeks. Primary cutaneous nocardiosis due to Nocardia spp. is relatively uncommon in clinics, but it was the distance of the lesions from the affected area of the wasp sting that has made this an even rarer case and of interest to report. © 2017 British Association of Dermatologists.

  5. Are the cutaneous manifestations in patients with primary antiphospholipid syndrome a marker for predicting lung manifestations?

    Science.gov (United States)

    Kontic, Milica; Stojanovich, Ljudmila; Mijailović-Ivković, Milena; Velinović, Mladen; Srnka, Jasminka; Zdravkovic, Marija

    2018-01-01

    The aim of this study was to investigate association between pulmonary and skin manifestations in a large group of patients with primary antiphospholipid syndrome (PAPS) as well as their connection with antiphospholipid antibodies. Our prospective study comprises of 390 patients with primary APS. Antiphospholipid antibody (aPL) analysis included detection of aCL (IgG/IgM), ß2GPI (IgG/IgM) and LA. Distinct pulmonary and skin associations were determined, as well as their associations with aPL. In PAPS patients the presence of LA was more common in PTE (p=0.005) and in pulmonary microthrombosis (p=0.003). We revealed statistical significance considering the presence of aCL IgM and pulmonary microthrombosis (p=0.05). Skin ulcerations correlated with positive titres aCL IgM and ß2 GPI IgM (p=0.03 and 0.04, respectively), while pseudovasculitis correlated with positive titres ß2 GPI IgM (p=0.02). PAPS patients were more more likely to develop pulmonary thromboembolisam if they had livedo reticularis (p=0.005), skin ulcerations (p=0.007), pseudovasculitic lesions (p=0.01), superficial cutaneous necrosis (p=0.005), and digital gangrene (p=0.02). Patients were also more prone to pulmonary microthrombosis if they already had livedo reticularis (p=0.03), skin ulcerations (p=0.007), pseudovasculitic lesions (p=0.05), superficial cutaneous necrosis (p=0.006), and digital gangrene (p=0.02). There is strong link between some pulmonary and skin manifestations in PAPS patients, suggesting complexity and evolutionary nature of APS. The presence of skin manifestations may be a high risk factor for several types of serious pulmonary manifestations in PAPS. Certain aPL types are associated with distinct pulmonary and skin manifestation, suggesting their predictive role.

  6. Adenoviral gene delivery to primary human cutaneous cells and burn wounds.

    Science.gov (United States)

    Hirsch, Tobias; von Peter, Sebastian; Dubin, Grzegorz; Mittler, Dominik; Jacobsen, Frank; Lehnhardt, Markus; Eriksson, Elof; Steinau, Hans-Ulrich; Steinstraesser, Lars

    2006-01-01

    The adenoviral transfer of therapeutic genes into epidermal and dermal cells is an interesting approach to treat skin diseases and to promote wound healing. The aim of this study was to assess the in vitro and in vivo transfection efficacy in skin and burn wounds after adenoviral gene delivery. Primary keratinocytes (HKC), fibroblasts (HFB), and HaCaT cells were transfected using different concentrations of an adenoviral construct (eGFP). Transfection efficiency and cytotoxicity was determined up to 30 days. Expression was quantified by FACS analysis and fluorimeter. Cytotoxicity was measured using the trypan blue exclusion method. 45 male Sprague Dawley rats received 2x10(8) pfu of Ad5-CMV-LacZ or carrier control intradermally into either superficial partial thickness scald burn or unburned skin. Animals were euthanized after 48 h, 7 or 14 days posttreatment. Transgene expression was assessed using immunohistochemistry and bioluminescent assays. The highest transfection rate was observed 48 h posttransfection: 79% for HKC, 70% for HFB, and 48% for HaCaT. The eGFP expression was detectable in all groups over 30 days (P>0.05). Cytotoxic effects of the adenoviral vector were observed for HFB after 10 days and HaCaT after 30 days. Reporter gene expression in vivo was significantly higher in burned skin compared with unburned skin (P=0,004). Gene expression decreases from 2 to 7 days with no significant expression after 14 days. This study demonstrates that effective adenoviral-mediated gene transfer of epidermal primary cells and cell-lines is feasible. Ex vivo gene transfer in epithelial cells might have promise for the use in severely burned patients who receive autologous keratinocyte sheets. Transient cutaneous gene delivery in burn wounds using adenoviral vectors causes significant concentrations in the wound tissue for at least 1 week. Based on these findings, we hypothesize that transient cutaneous adenoviral gene delivery of wound healing promoting factors has

  7. Primary tonsillar lymphomas according to the new World Health Organization classification: to report 87 cases and literature review and analysis.

    Science.gov (United States)

    Mohammadianpanah, Mohammad; Daneshbod, Yahya; Ramzi, Mani; Hamidizadeh, Nasrin; Dehghani, Seyed Javad; Bidouei, Farzad; Khademi, Bijan; Ahmadloo, Niloofar; Ansari, Mansour; Omidvari, Shapour; Mosalaei, Ahmad; Dehghani, Mehdi

    2010-10-01

    The present study aimed to define the natural history, World Health Organization (WHO) classification, prognostic factors, and treatment outcome of 87 patients with primary lymphoma of the palatine tonsil and literature review and analysis. Between 1990 and March 2008, 87 consecutive patients diagnosed with primary lymphoid malignancy of the palatine tonsil. All pathologic specimens were reviewed and reclassified according to the recent WHO classification. To investigate the association of tonsillar lymphomas with Epstein-Barr virus (EBV), in situ hybridization was performed for 24 tonsillar lymphomas (23 diffuse large B-cell lymphoma (DLBC) and one classic Hodgkin's disease) and ten normal tonsils as control group. In literature review, we found 26 major related series including 1,602 patients with primary tonsillar lymphoma. The median age of our patients was 52 years (range 11-86 years). There were 39 women and 48 men with a median follow-up of 67 months for living patients. The vast majority (95%) of patients had B-cell phenotype. DLBC was the most frequent histology. In situ hybridization revealed none of 23 DLBC to be positive for EBV. The 5-year disease-free and overall survival rates were 78.9% and 86%, respectively. In the literature review and by analyzing the data collection from 26 major reported series, the median age was 55 years and male/female ratio was 1.3:1. Intermediate grade tumors consisted of 72% of all tonsillar lymphomas and B-cell lymphomas constituted 82% of all cell immunophenotypes. The 5-year disease-free and overall survival rates were 61% and 67%, respectively. The vast majority of tonsillar lymphomas are of B-cell origin and with intermediate to high-grade histology. These neoplasms tend to present in early stage disease and to have favorable outcome. WHO classification predicts more accurately treatment outcome of patients with tonsillar lymphoma. The association of DLBC in the palatine tonsil with EBV infection is infrequent.

  8. Primary diffuse large B cell lymphoma arising from a leiomyoma of the uterine corpus.

    Science.gov (United States)

    Zhao, Lianhua; Ma, Qiang; Wang, Qiushi; Zeng, Ying; Luo, Qingya; Xiao, Hualiang

    2016-01-20

    Primary diffuse large B cell lymphoma (DLBCL) of the uterus is rare, and primary DLBCL arising from a uterine leiomyoma (collision tumor) has not been reported in the literature. We describe the clinical, histological, immunohistochemical, and molecular features of primary DLBCL arising from a leiomyoma in the uterine corpus. A 73-year-old female patient had a uterine mass for 23 years. An ultrasound scan revealed marked enlargement of the uterus, measuring 18.2 × 13 × 16.3 cm, with a 17.6 × 10.9 × 11.6 cm hypoechoic mass in the uterine corpus. The tumors consisted of medium- to large-sized cells exhibiting a diffuse pattern of growth with a well-circumscribed leiomyoma. The neoplastic cells strongly expressed CD79α, CD20 and PAX5. Molecular analyses indicated clonal B-cell receptor gene rearrangement. To the best of our knowledge, no previous cases of primary DLBCL arising from a leiomyoma have been reported. It is necessary to differentiate a diagnosis of primary DLBCL arising from a leiomyoma from that of leiomyoma with florid reactive lymphocytic infiltration (lymphoma-like lesion). Careful analysis of clinical, histological, immunophenotypic, and genetic features is required to establish the correct diagnosis.

  9. Genetically Modified Peripheral Blood Stem Cell Transplant in Treating Patients With HIV-Associated Non-Hodgkin or Hodgkin Lymphoma

    Science.gov (United States)

    2015-05-06

    Adult Nasal Type Extranodal NK/T-cell Lymphoma; AIDS-related Diffuse Large Cell Lymphoma; AIDS-related Diffuse Mixed Cell Lymphoma; AIDS-related Diffuse Small Cleaved Cell Lymphoma; AIDS-related Immunoblastic Large Cell Lymphoma; AIDS-related Lymphoblastic Lymphoma; AIDS-related Peripheral/Systemic Lymphoma; AIDS-related Small Noncleaved Cell Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Hepatosplenic T-cell Lymphoma; HIV-associated Hodgkin Lymphoma; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Noncutaneous Extranodal Lymphoma; Peripheral T-cell Lymphoma; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides/Sezary Syndrome; Recurrent Small Lymphocytic Lymphoma; Refractory Hairy Cell Leukemia; Small Intestine Lymphoma; Splenic Marginal Zone Lymphoma; Stage I AIDS-related Lymphoma; Stage II AIDS-related Lymphoma; Stage III AIDS-related Lymphoma; Stage IV AIDS-related Lymphoma; T-cell Large Granular Lymphocyte Leukemia; Testicular Lymphoma; Waldenström Macroglobulinemia

  10. Radiation therapy for primary breast lymphoma in male gynecomastia: a rare case report and review of the literature.

    Science.gov (United States)

    Ishibashi, Naoya; Hata, Masaharu; Mochizuki, Takao; Ogawa, Kogi; Sugiura, Hiroaki; Takekawa, Yoshinori; Maebayashi, Toshiya; Aizawa, Takuya; Sakaguchi, Masakuni; Abe, Osamu

    2016-10-01

    Primary breast lymphoma is a rare type of non-Hodgkin lymphoma and usually affects women, although a few cases have been reported in men. Chemotherapy and radiation therapy, or a combination of both, are frequently administered for treatment of primary breast lymphoma, as local control by surgical resection is poor. No standard therapy has been established, and the optimal radiation dose and irradiation field for male patients are unknown. The present report describes a 75-year-old man with bilateral cirrhosis-induced gynecomastia who was diagnosed with primary breast lymphoma; specifically, diffuse large B-cell lymphoma. Because of his hepatic dysfunction, he was treated with radiation therapy alone. Radiation therapy was followed by eight cycles of rituximab monotherapy. Clinical response was good, with no signs of relapse. Clinicians may benefit from knowledge regarding effective treatment of primary breast lymphoma in male patients, which has been rarely reported owing to the low incidence of this condition. The outcome in the present case may help to establish effective treatment guidelines in similar cases.

  11. A case of primary diffuse large B-cell non-Hodgkin's lymphoma misdiagnosed as chronic periapical periodontitis.

    Science.gov (United States)

    Jessri, M; AbdulMajeed, A A; Matias, M A; Farah, C S

    2013-06-01

    Lymphoma is a malignant neoplasm of component cells of the lymphoid system which is very rare in the jaws. Here we report a case of primary diffuse large B-cell lymphoma located in the periapical region of a mandibular molar which was misdiagnosed as chronic periapical periodontitis. The present case was diagnosed at an early stage and effectively managed by chemotherapy. Although lymphoma of the mandible is rare, it must be considered in the differential diagnosis of radiolucent lesions in this region. Lack of knowledge of this rare presentation may lead to delays in diagnosis and poor prognosis. © 2013 Australian Dental Association.

  12. 17-DMAG in Treating Patients With Metastatic or Unresectable Solid Tumors or Lymphomas

    Science.gov (United States)

    2013-01-24

    Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides/Sezary Syndrome; Recurrent Small Lymphocytic Lymphoma; Splenic Marginal Zone Lymphoma; Stage III Adult Burkitt Lymphoma; Stage III Adult Diffuse Large Cell Lymphoma; Stage III Adult Diffuse Mixed Cell Lymphoma; Stage III Adult Diffuse Small Cleaved Cell Lymphoma; Stage III Adult Hodgkin Lymphoma; Stage III Adult T-cell Leukemia/Lymphoma; Stage III Cutaneous T-cell Non-Hodgkin Lymphoma; Stage III Grade 1 Follicular Lymphoma; Stage III Grade 2 Follicular Lymphoma; Stage III Grade 3 Follicular Lymphoma; Stage III Mantle Cell Lymphoma; Stage III Marginal Zone Lymphoma; Stage III Mycosis Fungoides/Sezary Syndrome; Stage III Small Lymphocytic Lymphoma; Stage IV Adult Burkitt Lymphoma; Stage IV Adult Diffuse Large Cell Lymphoma; Stage IV Adult Diffuse Mixed Cell Lymphoma; Stage IV Adult Diffuse Small Cleaved Cell Lymphoma; Stage IV Adult Hodgkin Lymphoma; Stage IV Adult T-cell Leukemia/Lymphoma; Stage IV Cutaneous T-cell Non-Hodgkin Lymphoma; Stage IV Grade 1 Follicular Lymphoma; Stage IV Grade 2 Follicular Lymphoma; Stage IV Grade 3 Follicular Lymphoma; Stage IV Mantle Cell Lymphoma; Stage IV Marginal Zone Lymphoma; Stage IV Mycosis Fungoides/Sezary Syndrome; Stage IV Small Lymphocytic Lymphoma; Unspecified Adult Solid Tumor, Protocol Specific; Waldenstr

  13. F18 FDG positron emission tomography revelation of primary testicular lymphoma with concurrent multiple extra nodal involvement

    International Nuclear Information System (INIS)

    Vamsy, Mohana; Dattatreya, P.S.; Parakh, Megha; Dayal, Monal; Prabhakar Rao, V.V.S.

    2013-01-01

    Primary testicular lymphoma (PTL) a relatively rare disease of non-Hodgkin's lymphomas occurring with a lesser incidence of 1-2% has a propensity to occur at later ages above 50 years. PTL spreads to extra nodal sites due to deficiency of extra cellular adhesion molecules. We present detection of multiple sites of extra nodal involvement of PTL by F-18 positron emission tomography/computed tomography study aiding early detection of the dissemination thus aiding in staging and management. (author)

  14. Primary non-Hodgkin's lymphoma of the common bile duct: A case report and literature review

    Directory of Open Access Journals (Sweden)

    Ali Zakaria

    2017-01-01

    Full Text Available Hepatobiliary involvement by malignant lymphoma is usually a secondary manifestation of systemic disease, whereas primary non-Hodgkin's lymphoma of the extrahepatic biliary ducts is an extremely rare entity. We describe the case of a 57-year-old man who presented with an acute onset of obstructive jaundice and severe itching. Abdominal ultrasonography and computed tomography revealed intrahepatic and common hepatic ducts dilatation. Magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography showed a mid-common bile duct stricture. The patient was presumed to have cholangiocarcinoma of the common bile duct, and an en bloc resection of the tumor with Roux-en-Y hepaticojejunostomy and porta-hepatis lymph nodes dissection was performed. Histopathology and immunohistochemistry revealed a large B cell non-Hodgkin's lymphoma. The patient received six cycles of combination chemotherapy using cyclophosphamide, vincristine, prednisone, and rituximab (CVP-R protocol, and after a 5-year follow-up he is still in complete remission. We also reviewed the cases published from 1982 to 2012, highlighting the challenges in reaching a correct preoperative diagnosis and the treatment modalities used in each case.

  15. Retrospective study of 48 cases of primary central nervous system lymphoma

    Directory of Open Access Journals (Sweden)

    Lucas Alessandro

    2017-02-01

    Full Text Available Primary central nervous system lymphoma (PCNSL is an infrequent form of non-Hodgkin lymphoma restricted to the CNS. More than 90% are type B and mainly affect patients aged 50-70 years. Immunodeficiency is the most important risk factor. The aim of our study was to evaluate the immune status, clinical presentation and findings in complementary studies of PCNSL patients. A retrospective analysis of 48 cases treated in our center between January 1992 and May 2015 was performed. Median age at diagnosis was 61 years (range 25-84; with male predominance (2.1:1. Forty one cases (85% were immunocompetent patients. Brain MRI findings showed parenchymal involvement in 45 cases (94%, 43% with frontal lobe and 35% basal ganglia, 4% had meningeal involvement and 2% had ophthalmic involvement at diagnosis. Fifty-five percent had restricted signal on diffusion weighted imaging and contrast enhancement was found in 89%. Pyramidal syndrome was the main initial clinical manifestation (56%. There were abnormal findings in 62% of CSF samples, but in only 11.1% positive cytology results were detected. The most frequent type was diffuse large B-cell lymphoma (83%, being B-cell type the most common form between them (96%. In our series PCNSL was more frequent in immunocompetent elderly male subjects. At initial evaluation, clinical manifestations and MRI findings were variable. The initial suspicion of this entity would allow an early diagnosis, avoiding empirical treatments that may confuse or delay diagnosis

  16. Systematic analysis of a xenograft mice model for KSHV+ primary effusion lymphoma (PEL.

    Directory of Open Access Journals (Sweden)

    Lu Dai

    Full Text Available Kaposi's sarcoma-associated herpesvirus is the causative agent of primary effusion lymphoma (PEL, which arises preferentially in the setting of infection with human immunodeficiency virus (HIV. Even with standard cytotoxic chemotherapy, PEL continues to cause high mortality rates, requiring the development of novel therapeutic strategies. PEL xenograft models employing immunodeficient mice have been used to study the in vivo effects of a variety of therapeutic approaches. However, it remains unclear whether these xenograft models entirely reflect clinical presentations of KSHV(+ PEL, especially given the recent description of extracavitary solid tumor variants arising in patients. In addition, effusion and solid tumor cells propagated in vivo exhibit unique biology, differing from one another or from their parental cell lines propagated through in vitro culture. Therefore, we used a KSHV(+ PEL/BCBL-1 xenograft model involving non-obese diabetic/severe-combined immunodeficient (NOD/SCID mice, and compared characteristics of effusion and solid tumors with their parent cell culture-derived counterparts. Our results indicate that although this xenograft model can be used for study of effusion and solid lymphoma observed in patients, tumor cells in vivo display unique features to those passed in vitro, including viral lytic gene expression profile, rate of solid tumor development, the host proteins and the complex of tumor microenvironment. These items should be carefully considered when the xenograft model is used for testing novel therapeutic strategies against KSHV-related lymphoma.

  17. The relationship between MDM2 expression and tumor thickness and invasion in primary cutaneous malignant melanoma

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    Parvin Rajabi

    2012-01-01

    Full Text Available Background: Malignant melanoma is the most invasive cutaneous tumor which is associated with an incredibly high mortality rate. The most reliable histological factors associated with melanoma prognosis are tumor thickness- measured by the Breslow index- and invasion depth- measured by Clark level. Murine double minute 2 (MDM2 gene inhibits p53-dependent apoptosis. An increase in MDM2 expression has been found in many tumors. This study aimed to investigate MDM2 expression and its correlation with tumor thickness and invasion level in malignant melanoma. Materials and Methods: This study evaluated paraffin blocks from 43 randomly selected patients with primary cutaneous melanoma who referred to the main university pathology center in Isfahan, Iran. MDM2 expression rate was assessed via immunohistochemical techniques and hematoxylin and eosin staining to determine tumor thickness and invasion level. Correlations between MDM2 expression and tumor thickness and invasion were analyzed using Spearman′s correlation coefficient in SPSS 17 . Results: The mean age of patients was 61.2 ± 15 years. Men and women constituted 55.8% and 44.2% of the participants, respectively. The rate of MDM2 positivity was 28.9%. MDM2 expression was directly associated with tumor thickness (r = 0.425; p = 0.002 and weakly with invasion level (r = 0.343; p = 0.01. Conclusions: Despite the low MDM2 expression rate observed in this study, direct relationships between MDM2 positivity and tumor thickness and invasion level were identified. MDM2 expression can thus be suggested as a potential new predictive prognostic factor.

  18. MicroRNA Expression in Early Mycosis Fungoides Is Distinctly Different from Atopic Dermatitis and Advanced Cutaneous T-Cell Lymphoma

    DEFF Research Database (Denmark)

    Ralfkiaer, Ulrik; Lindahl, Lise Maria; Litman, Thomas

    2014-01-01

    Mycosis fungoides (MF) is the most common variant of cutaneous T-cell lymphoma (CTCL). MF is characterized by chronic inflammation dominated by cluster of differentiation 4-positive (CD4(+)) T-cells and T helper 2 cytokines, and as the malignant T-cell clone is initially elusive, early diagnosis...... is often impossible. MF usually takes an indolent course, but for unknown reasons may turn into an aggressive disease with a poor prognosis. Herein, we used a global quantitative real-time polymerase chain reaction platform to study microRNA (miR) expression in patients with early MF (n=13), more advanced...... CTCL (n=42), and atopic dermatitis (AD, n=20). Thirty-eight miRs were differentially expressed (≥2-fold) in early MF vs. AD and 36 in early MF vs. more advanced disease. miRs that distinguish early MF from AD included both up-regulated (miR-155, miR-146a, 146b-5p, miR-342-3p, let-7i*) and down...

  19. Use of antibodies against the variable regions of the T-cell receptor alpha/beta heterodimer for the study of cutaneous T-cell lymphomas.

    Science.gov (United States)

    Ralfkiaer, E; Wollf-Sneedorff, A; Vejlsgaard, G L

    1991-11-01

    Recent studies have suggested that antibodies against the variable (V) regions of the T-cell antigen receptor (TCR) may be used as markers for clonality and malignancy in T-cell infiltrates. We have investigated this by examining biopsy samples from 45 patients with cutaneous T-cell lymphomas (CTCL) for reactivity with seven antibodies against different V-gene families on the TCR alpha/beta heterodimer, i.e. ICI (V beta 5a), W112 (V beta 5b), OT145 (V beta 6a), 16G8 (V beta 8a), S511 (V beta 12a), F1 (V alpha 2a) and LC4 (alpha beta Va). Serial biopsies were available in 13 patients and a total of 62 samples were studied. The neoplastic cells in five cases were positive for either V beta 5 (one case), V beta 6 (one case), V beta 8 (two cases) or V beta 12 (one case). In the remaining 40 cases, no staining was seen of the neoplastic cells. These findings indicate that while antibodies against the TCR V-regions may be used as clonotypic markers for certain T-cell neoplasms, there is as yet not a sufficient number of anti-TCR V-region antibodies available for the routine diagnosis of these conditions.

  20. Primary B cell lymphoma of the tongue base: a case report

    Science.gov (United States)

    Bechir, Achour; Asma, Achour; Haifa, Regaieg; Nesrine, Abdessayed; Yosra, Ben Youssef; Badreddine, Sriha; Abderrahim, Khelif

    2016-01-01

    Primary non-Hodgkin’s lymphoma’s of the tongue is very rare and accounts for 1% of all malignant tumor of the oral cavity. Clinical features are non-specific ulcerative lesions that do not heal. In the literature, the majority of cases are diffuse large B cell type however, T cell phenotype also may occur. We describe a 77 years old man, who presented with an ulcerative mass in the left margin of the tongue the diagnosis diffuse large B cell lymphoma was confirmed. The patient is actually on treatment R-mini CEOP and has favorable evolution. PMID:28292136

  1. Psoriasis and risk of malignant lymphoma

    DEFF Research Database (Denmark)

    Kamstrup, M R; Skov, L; Zachariae, C

    2018-01-01

    In patients with psoriasis, the risk of lymphoma has been a subject of controversy and data from larger studies are limited1-4 . We therefore investigated the 5-year risk of new-onset Hodgkin lymphoma (HL), non-Hodgkin lymphoma (NHL) (excluding cutaneous T-cell lymphoma [CTCL]), and CTCL...

  2. Cutaneous Lymphoma International Consortium Study of Outcome in Advanced Stages of Mycosis Fungoides and Sézary Syndrome

    DEFF Research Database (Denmark)

    Scarisbrick, Julia J; Prince, H Miles; Vermeer, Maarten H

    2015-01-01

    PURPOSE: Advanced-stage mycosis fungoides (MF; stage IIB to IV) and Sézary syndrome (SS) are aggressive lymphomas with a median survival of 1 to 5 years. Clinical management is stage based; however, there is wide range of outcome within stages. Published prognostic studies in MF/SS have been single......, proliferation index, large-cell transformation, WBC/lymphocyte count, serum lactate dehydrogenase, and identical T-cell clone in blood and skin. Data were collected at specialist centers on patients diagnosed with advanced-stage MF/SS from 2007. Each parameter recorded at diagnosis was tested against overall...... survival (OS). RESULTS: Staging data on 1,275 patients with advanced MF/SS from 29 international sites were included for survival analysis. The median OS was 63 months, with 2- and 5-year survival rates of 77% and 52%, respectively. The median OS for patients with stage IIB disease was 68 months...

  3. Stage IE Primary Bone Lymphoma:Limb Salvage for Local Recurrence

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    Khodamorad Jamshidi

    2015-01-01

    Full Text Available Background:   Primary bone lymphoma or non-Hodgkin lymphoma of bone is a rare disease. There are only a few case series of stage IE of this condition in medical literature. The aim of this study is to determine the rate of survival   for stage IE after combined modality treatment, the rate of local recurrence, and the results of limb salvage in cases   of local recurrence.     Methods:   We collected data from 61 patients with histologically confirmed PBL treated at the Musculoskeletal   Oncology Department of our hospital from 2000 to 2010. Retrospective evaluation included demographics, symptoms, tumor locations, outcomes of surgical treatment for local recurrence and survival rates. Results:   All patients received Combined Modality Therapy. Overall,five year survival was 89% and five year disease free survival rate was 78%. Local recurrence occurred in 6 patients during follow up period, which was treated surgically     by wide excision and reconstruction. The mean follow-up for the local recurrence group was 36(24-54 months and mortality rate in this group was 17%. Conclusions:   Combined Modality Therapy for stage IE primary bone lymphomaresults in good survival rate. In case   of local recurrence, wide excision and reconstruction improves the outcomes.

  4. Bilateral primary adrenal lymphoma with central nervous system involvement: a case report

    International Nuclear Information System (INIS)

    Nacif, Marcelo Souto; Heizer, Marcia; Nahime, Carolina Benvegnu; Santos, Alair Augusto Sarmet Moreira Damas dos

    2005-01-01

    The authors review the main findings of primary adrenal lymphoma on abdominal and brain computed tomography (CT) scans and brain magnetic resonance imaging (MRI), and report a rare case of a 72-year-old male patient with deterioration in mental status progressing from recent memory loss to disorientation and ultimately dementia in a period of two months. The patient was investigated using imaging methods including brain (without intravenous administration of iodinated contrast media), chest and abdomen (pre and post intravenous administration of iodinated contrast media) CT scans and brain MRI (pre and post intravenous administration of gadolinium). Brain CT scan showed slightly hyperdense nodules adjacent to the lateral ventricles. No gross abnormalities were seen on the chest CT scan. CT of the abdomen showed solid nodules on both adrenals. Brain MRI showed diffuse nodular enhancement of the ependyma of the fourth ventricle, third ventricle, frontal and temporal horns, atria and body of the lateral ventricles. Definitive diagnosis was made by immunohistochemical study of the biopsed adrenal. After a comprehensive review of the literature the authors concluded that CT and MRI are essential methods for the detection and accurate evaluation of primary adrenal lymphomas, particularly when there is involvement of the central nervous system. MRI is more sensitive in the detection of extracerebral lesions in the epidural and subdural spaces, especially after intravenous administration of gadolinium. (author)

  5. Primary lymphoma of brain: results of management of a modern cohort with radiation therapy

    International Nuclear Information System (INIS)

    Laperriere, Normand J.; Cerezo, Laura; Milosevic, Michael F.; Wong, C. Shun; Patterson, Bruce; Panzarella, Tony

    1997-01-01

    Purpose: To assess the outcome and prognostic factors for patients with primary lymphoma of brain managed with radiation therapy between 1979 and 1988. Methods and materials: A retrospective review was undertaken of 49 patients referred to Princess Margaret Hospital. There were 25 males and 24 females. Median age was 60 years, with a range of 17-80 years. Tumors were located supratentorially in 35, infratentorially in 10, and both in 4 patients. Single masses were demonstrated on CT brain in 36, and multiple lesions in 13 patients. Cranial irradiation was given in 48, and 11 patients received chemotherapy. All patients in this series were immunocompetent. Results: Over a follow-up range of 3-11 years of surviving patients, with a median of 6 years, (40(49)) patients have died. Overall median survival was 1.4 years (17 months) and 5-year actuarial survival was 26%. Statistical analysis revealed the following significant factors: Karnofsky performance status (KPS), age, and distribution pattern of disease on presenting CT brain. Five-year actuarial survival for patients with a KPS > 60 or 60, 5-year actuarial survival was 42% and 9%, respectively (P = 0.03); for patients with solitary or multiple lesions, 5-year actuarial survival was 30% and 15%, respectively (P = 0.04). Conclusions: We conclude that Karnofsky performance status, age, and distribution pattern on pretreatment CT of brain are significant prognostic factors in primary lymphoma of brain, and that new approaches need to be developed for these patients

  6. Radiographic findings in 37 cases of primary CNS lymphoma in immunocompetent patients

    International Nuclear Information System (INIS)

    Coulon, A.; Lafitte, F.; Martin-Duverneuil, N.; Chiras, J.; Hoang-Xuan, K.; Mokhtari, K.; Blustajn, J.

    2002-01-01

    Because of the increasing incidence of primary central nervous system lymphoma (PCNSL), it is essential to recognize this disease in order to start appropriate treatment. We present the characteristic CT and MRI features of this tumour. The findings of 32 CT and 31 MR of 37 immunocompetent patients with biopsy-proved PCNSL are reviewed. The main features are presented and analysed, and are discussed in comparison with proven literature data. Primary central nervous system lymphoma presents as supratentorial solitary lesions in approximately 80% of the patients and multiple lesions in 20%. In contrast to classical data, the lesions are located in deep structures only in one-third of the cases, and involve posterior fossa in 10% of cases. Most of the lesions are hyperdense or isodense (92%) on CT, hypointense or isointense on T1-weighted images, and only about 40% are hyperintense on T2-weighted images. Nearly all the lesions enhance, except after corticosteroid administration. They produce mild oedema and mass effect. Meningeal or ventricular enhancement are rare but suggestive. Calcification, haemorrhage or necrosis are scarce. Although PCNSL in immunocompetent patients have a variable CT and MR appearance, the imaging data often suggest the diagnosis. (orig.)

  7. Radiographic findings in 37 cases of primary CNS lymphoma in immunocompetent patients

    Energy Technology Data Exchange (ETDEWEB)

    Coulon, A.; Lafitte, F.; Martin-Duverneuil, N.; Chiras, J. [Department of Neuroradiology, Hopital de la Salpetriere, Paris (France); Hoang-Xuan, K. [Department of Neurology, Hopital de la Salpetriere, Paris (France); Mokhtari, K. [Department of Anatomopathology, Hopital de la Salpetriere, Paris (France); Blustajn, J. [Department of Radiology, Fondation Rothschild, Paris (France)

    2002-02-01

    Because of the increasing incidence of primary central nervous system lymphoma (PCNSL), it is essential to recognize this disease in order to start appropriate treatment. We present the characteristic CT and MRI features of this tumour. The findings of 32 CT and 31 MR of 37 immunocompetent patients with biopsy-proved PCNSL are reviewed. The main features are presented and analysed, and are discussed in comparison with proven literature data. Primary central nervous system lymphoma presents as supratentorial solitary lesions in approximately 80% of the patients and multiple lesions in 20%. In contrast to classical data, the lesions are located in deep structures only in one-third of the cases, and involve posterior fossa in 10% of cases. Most of the lesions are hyperdense or isodense (92%) on CT, hypointense or isointense on T1-weighted images, and only about 40% are hyperintense on T2-weighted images. Nearly all the lesions enhance, except after corticosteroid administration. They produce mild oedema and mass effect. Meningeal or ventricular enhancement are rare but suggestive. Calcification, haemorrhage or necrosis are scarce. Although PCNSL in immunocompetent patients have a variable CT and MR appearance, the imaging data often suggest the diagnosis. (orig.)

  8. MDX-010 in Treating Patients With Recurrent or Refractory Lymphoma

    Science.gov (United States)

    2014-05-22

    Adult Grade III Lymphomatoid Granulomatosis; B-cell Chronic Lymphocytic Leukemia; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Refractory Hairy Cell Leukemia; Small Intestine Lymphoma; Splenic Marginal Zone Lymphoma; Testicular Lymphoma; Waldenström Macroglobulinemia

  9. Lysosomal-associated membrane protein-2 plays an important role in the pathogenesis of primary cutaneous vasculitis.

    Science.gov (United States)

    Takeuchi, Sora; Kimura, Satoko; Soma, Yoshinao; Waki, Masashi; Yamaguchi, Madoka; Nakazawa, Daigo; Tomaru, Utano; Ishizu, Akihiro; Kawakami, Tamihiro

    2013-09-01

    Recent research suggests that lysosomal-associated membrane protein-2 (LAMP-2) could be one of the target antigens in the pathogenesis of vasculitides. We established a transgenic rat model, env-pX rats, with various vasculitides including cutaneous vasculitis. Human primary cutaneous vasculitis includes cutaneous polyarteritis nodosa (CPN) and Henoch-Schönlein purpura (HSP). We measured serum anti-LAMP-2 antibody levels in morbid env-pX rats and injected anti-LAMP-2 antibody into premorbid env-pX rats. We further measured serum anti-LAMP-2 antibody levels in patients with CPN and HSP. Cutaneous vasculitis was observed in ∼30% of 6-month-old morbid env-pX rats. In contrast, these findings were rare in premorbid env-pX rats under 3 months old. We also examined 85 patients with CPN and 36 adult patients with HSP. Serum anti-LAMP-2 antibody levels were determined using ELISA. Premorbid env-pX rats under 3 months old were given an i.v. injection of anti-LAMP-2 antibody at day 0 and day 7. At day 14, these rats underwent histopathological and direct immunofluorescence examination. Cell surface LAMP-2 expression of rat neutrophils was examined by flow cytometry. Serum anti-LAMP-2 antibody levels were significantly higher in morbid env-pX rats than in wild-type normal rats. In addition, the levels in the cutaneous vasculitis group of morbid env-pX rats were significantly higher than the no cutaneous vasculitis group. Intravenous anti-LAMP-2 antibody injection into premorbid env-pX rats under 3 months old induced infiltration of neutrophils into cutaneous small vessels. Anti-LAMP-2 antibody-binding neutrophils were detected there. LAMP-2 expression on the cell surface of neutrophils in premorbid env-pX rats under PMA stimulation was higher compared with controls. Serum anti-LAMP-2 antibody levels in CPN and HSP were significantly higher than those of healthy controls. These data support a positive relationship between anti-LAMP-2 antibody and cutaneous vasculitis.

  10. Clinical Significance of "Double-hit" and "Double-protein" expression in Primary Gastric B-cell Lymphomas.

    Science.gov (United States)

    He, Miaoxia; Chen, Keting; Li, Suhong; Zhang, Shimin; Zheng, Jianming; Hu, Xiaoxia; Gao, Lei; Chen, Jie; Song, Xianmin; Zhang, Weiping; Wang, Jianmin; Yang, Jianmin

    2016-01-01

    Primary gastric B-cell lymphoma is the second most common malignancy of the stomach. There are many controversial issues about its diagnosis, treatment and clinical management. "Double-hit" and "double-protein" involving gene rearrangement and protein expression of c-Myc and bcl2/bcl6 are the most used terms to describe DLBCL poor prognostic factors in recent years. However, very little is known about the role of these prognostic factors in primary gastric B-cell lymphomas. This study aims to obtain a molecular pathology prognostic model of gastric B-cell lymphoma for clinical stratified management by evaluating how the "double-hit" and "double-protein" in tumor cells as well as microenvironmental reaction of tumor stromal tissue affect clinical outcome in primary gastric B-cell lymphomas. Data and tissues of 188 cases diagnosed with gastric B-cell lymphomas were used in this study. Tumor tissue microarray (TMA) of formalin fixed and paraffin embedded (FFPE) tissues was constructed for fluorescence in situ hybridization (FISH) and immunohistochemistry (IHC) analysis with a serial of biomarkers containing MYC, BCL2, BCL6, CD31, SPARC, CD10, MUM1 and Ki-67. Modeled period analysis was used to estimate 3-year and 5-year overall survival (OS) and disease-free survival (DFS) distributions. There was no definite "double-hit" case though the gene rearrangement of c-Myc (5.9%), bcl2 (0.1%) and bcl6 (7.4%) was found in gastric B-cell lymphomas. The gene amplification or copy gains of c-Myc (10.1%), bcl-2 (17.0%) and bcl-6 (0.9%) were present in these lymphomas. There were 12 cases of the lymphomas with the "double-protein" expression of MYC and BCL2/BCL6. All patients with "double-protein" gastric B-cell lymphomas had poor outcome compared with those without. More importantly, "MYC-BCL2-BCL6" negative group of gastric B-cell lymphoma patients had favorable clinical outcome regardless clinical stage, pathological types and therapeutic modalities. And the similar better

  11. A Novel Missense Mutation in Oncostatin M Receptor Beta Causing Primary Localized Cutaneous Amyloidosis

    Directory of Open Access Journals (Sweden)

    Marjan Saeedi

    2014-01-01

    Full Text Available Primary localized cutaneous amyloidosis (PLCA is a chronic skin disorder, caused by amyloid material deposition in the upper dermis. Autosomal dominant PLCA has been mapped earlier to pathogenic missense mutations in the OSMR gene, which encodes the oncostatin M receptor ß subunit (OSMRß. OSMRß is interleukin-6 family cytokine receptors and possesses two ligands, oncostatin M and interleukin-31, which both have biologic roles in inflammation and keratinocyte cell proliferation, differentiation, and apoptosis. Here, we identified a new OSMR mutation in a Kurdish family for the first time. Blood samples were taken from all the affected individuals in the family. DNA extraction was performed using salting out technique. Primers were designed for intron flanking individual exons of OSMR gene which were subjected to direct sequencing after PCR amplification for each sample. Sequencing showed a C/T substitution at position 613 in the proband. This mutation results in an L613S (leucine 613 to serine amino acid change. The identified mutation was observed in all affected family members but not in 100 ethnically matched healthy controls. Elucidating the molecular basis of familial PLCA provides new insight into mechanisms of itch in human skin and may lead to new therapeutic targets for pruritus.

  12. CASE REPORT: Primary Cutaneous Nocardiosis of Axillary Region: A Case Report

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    Basavaraj V. Peerapur

    2012-07-01

    Full Text Available Background: Nocardiosis is an uncommon but world-wide infection caused by several species of soil-borne aerobic bacteria belonging to the genus Nocardia. Primary cutaneous nocardiosis (PCN is an uncommon entity. It usually occurs among immunocompetent but occupationally predisposed individuals. Clinically, it can present as acute infection (abscess or cellulitis, mycetoma, or sporotrichoid infection [1]. Here we are reporting a case of PCN presented as mycetoma in axilla which is a rare site. Case History: The patient had extensive lesions in and around the axilla, which could be attributed to the fact that the patient, being an agriculturist, had been exposed to recurrent trauma while carrying firewood and soiled sacks. Single lesion initiated four years ago, progressed to multiple lesions with few healed scars. Despite the treatment in several hospitals, lesions recurred. The present patient was diagnosed as PCN caused by Nocardia brasiliensis and appropriately treated. Conclusion: Nocardia infection should be considered in the differential diagnosis of a supportive and granulomatous dermatitis that presents clinically as multiple discharging sinuses with papules and nodules in and around axilla apart from tuberculosis.

  13. Further insights into the natural history and management of primary cutaneous neuroendocrine (merkel cell) carcinoma

    International Nuclear Information System (INIS)

    Boyle, Frances; Pendlebury, Susan; Bell, David

    1995-01-01

    Purpose: Primary cutaneous neuroendocrine (Merkel cell) carcinoma is a rare neoplasm with aggressive behavior but potential for response to radiotherapy and chemotherapy. Optimal treatment regimens are evolving based on reports of case series and a growing understanding of the natural history. Methods and Materials: A retrospective analysis of 34 cases treated at two Australian Institutions over 13 years is presented, focusing on clinical features and response to therapy. Results: The aggressive nature of this neoplasm is confirmed by the local recurrence rate of 22% following surgical excision, the development of regional node metastases in 76%, and of distant metastases in 70%. Overall median survival was 24 months with 65% of patients succumbing to metastatic disease. An association with B cell malignancies and immunosuppressive therapy is noted, with these patients having a poorer outcome, and one spontaneous remission was observed. Radiation therapy produced responses in 21 of 30 measurable sites (11 complete, 10 partial), and in 11 sites irradiated prophylactically there was only one infield relapse (9%). Responses to chemotherapy were observed in 8 of 20 applications (40%), particularly carboplatin and etoposide given in the setting of regional node disease. Conclusion: In this poor prognosis tumor, further investigation of adjuvant radiotherapy and chemotherapy is warranted, as responsiveness of recurrent disease is confirmed. Immunological factors appear important in the natural history, and their manipulation may offer additional therapeutic options

  14. [Primary peripheral T-cell lymphoma of the penis: a case report and review of the literature].

    Science.gov (United States)

    Shi, Yan-Lin; Yin, Hong-Lin; Zhou, Xiao-Jun; Zhou, Hang-Bo; Lu, Zhen-Feng

    2008-11-01

    To report a case of primary peripheral T-cell lymphoma of the penis. We analyzed the clinicopathological characteristics of the case of primary peripheral T-cell lymphoma using histological, cytochemical and immunohistochemical methods and by review of the literature. The patient was a 65 years old man and presented with a diffuse enlargement of the penis as the initial sign, followed by erosive ulcer in the caput penis and inguinal lymphadenectasis. The tumor was pathohistologically manifested as an epidermal ulcer, with tumorous necrosis around the capillary, infiltrative growth and atypical changes of the neoplastic cells and proliferation of capillaries. Immunohistochemically, the tumor cells were positive for CD43 and CD3, but negative for CD20, CD79a, CD34, CD30, CD56 and CD34. Clinically it responded to the chemotherapy designed for peripheral T-cell lymphoma. Primary peripheral T-cell lymphoma of the penis is an extremely rare malignant tumor, the diagnosis of which relies on histopathological examination, immunohistochemical staining and differentiation between squamous cell carcinoma and other types of lymphoma.

  15. Experience-induced plasticity of cutaneous maps in the primary somatosensory cortex of adult monkeys and rats.

    Science.gov (United States)

    Xerri, C; Coq, J O; Merzenich, M M; Jenkins, W M

    1996-01-01

    In a first study, the representations of skin surfaces of the hand in the primary somatosensory cortex, area 3b, were reconstructed in owl monkeys and squirrel monkeys trained to pick up food pellets from small, shallow wells, a task which required skilled use of the digits. Training sessions included limited manual exercise over a total period of a few hours of practice. From an early clumsy performance in which many retrieval attempts were required for each successful pellet retrieval, the monkeys exhibited a gradual improvement. Typically, the animals used various combinations of digits before developing a successful retrieval strategy. As the behavior came to be stereotyped, monkeys consistently engaged surfaces of the distal phalanges of one or two digits in the palpation and capture of food pellets from the smallest wells. Microelectrode mapping of the hand surfaces revealed that the glabrous skin of the fingertips predominantly involved in the dexterity task was represented over topographically expanded cortical sectors. Furthermore, cutaneous receptive fields which covered the most frequently stimulated digital tip surfaces were less than half as large as were those representing the corresponding surfaces of control digits. In a second series of experiments, Long-Evans rats were assigned to environments promoting differential tactile experience (standard, enriched, and impoverished) for 80 to 115 days from the time of weaning. A fourth group of young adult rat experienced a severe restriction of forepaw exploratory movement for either 7 or 15 days. Cortical maps derived in the primary somatosensory cortex showed that environmental enrichment induced a substantial enlargement of the cutaneous forepaw representation, and improved its spatial resolution (smaller glabrous receptive fields). In contrast, tactile impoverishment resulted in a degradation of the forepaw representation that was characterized by larger cutaneous receptive fields and the emergence of

  16. Primary nodal peripheral T-cell lymphomas: diagnosis and therapeutic considerations

    Directory of Open Access Journals (Sweden)

    Luis Alberto de Pádua Covas Lage

    2015-08-01

    Full Text Available Nodal peripheral T-cell lymphomas are a rare group of neoplasms derived from post-thymic and activated T lymphocytes. A review of scientific articles listed in PubMed, Lilacs, and the Cochrane Library databases was performed using the term "peripheral T-cell lymphomas". According to the World Health Organization classification of hematopoietic tissue tumors, this group of neoplasms consists of peripheral T-cell lymphoma not otherwise specified (PTCL-NOS, angioimmunoblastic T-cell lymphoma (AITL, anaplastic large cell lymphoma-anaplastic lymphoma kinase positive (ALCL-ALK+, and a provisional entity called anaplastic large cell lymphoma-anaplastic lymphoma kinase negative (ALCL-ALK-. Because the treatment and prognoses of these neoplasms involve different principles, it is essential to distinguish each one by its clinical, immunophenotypic, genetic, and molecular features. Except for anaplastic large cell lymphoma-anaplastic lymphoma kinase positive, which has no adverse international prognostic index, the prognosis of nodal peripheral T-cell lymphomas is worse than that of aggressive B-cell lymphomas. Chemotherapy based on anthracyclines provides poor outcomes because these neoplasms frequently have multidrug-resistant phenotypes. Based on this, the current tendency is to use intensified cyclophosphamide, doxorubicin, vincristine, prednisolone (CHOP regimens with the addition of new drugs, and autologous hematopoietic stem cell transplantation. This paper describes the clinical features and diagnostic methods, and proposes a therapeutic algorithm for nodal peripheral T-cell lymphoma patients.

  17. Primary bone lymphoma: A clinicopathological retrospective study of 28 patients in a single institution

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    Mohammad Reza Ghavam Nasiri

    2011-01-01

    Full Text Available Background: Primary bone lymphoma (PBL is a rare disease and distinct clinicopathological entity. The optimal treatment strategy is still unclear. Because of rarity of PBL, we report our institute experience in PBL clinicopathological feature and treatment results. Methods: 28 patients diagnosed with PBL were referred to Omid Hospital, cancer research center (CRC, between March 2001 and February 2009. Immunophenotype studies on 16 out of 28 pathological blocks were performed. We analyzed disease free survival (DFS and overall survival (OS rates. Results: 14 patients with PBL were analyzed retrospectively. 17 patients (60.7% were male and 11 (39.3% were female with a median age of 41 years (range: 11-79. Long bones were the most primarily site of involvement (71%. 26 (93% patients had diffuse large B cell lymphoma and 2 (7% had small lymphoblastic lymphoma. One (3% patient received radiation alone, 18 (66% cases received combined modality (chemotherapy + radiotherapy and 8 (30% received only chemotherapy during their treatment period. The median follow up was 18 months (range: 1-82. Mean DFS was 51 months (range: 37-66. Overall survival (OS was 54 months (range: 40-68. OS was significantly better in the chemoradiotherapy group compared with other two groups (64 versus 27 months, respectively, p=0.014. DFS was also significantly better in combined modality arm compared with other two groups (64 versus 21 months, respectively, p=0.003. Conclusions: In spite of small number of patients reported in this study, combined modality treatment (chemotherapy and radiotherapy was shown to be useful as an effective treatment strategy in PBL.

  18. Sangivamycin induces apoptosis by suppressing Erk signaling in primary effusion lymphoma cells

    Energy Technology Data Exchange (ETDEWEB)

    Wakao, Kazufumi [Department of Biotechnology, Interdisciplinary Graduate School of Medicine and Engineering, University of Yamanashi, Kofu-shi 400-8511 (Japan); Watanabe, Tadashi [Department of Cell Biology, Kyoto Pharmaceutical University, Misasagi-Shichonocho 1, Yamashinaku, Kyoto 607-8412 (Japan); Takadama, Tadatoshi; Ui, Sadaharu [Department of Biotechnology, Interdisciplinary Graduate School of Medicine and Engineering, University of Yamanashi, Kofu-shi 400-8511 (Japan); Shigemi, Zenpei; Kagawa, Hiroki [Department of Cell Biology, Kyoto Pharmaceutical University, Misasagi-Shichonocho 1, Yamashinaku, Kyoto 607-8412 (Japan); Higashi, Chizuka; Ohga, Rie; Taira, Takahiro [Department of Molecular Cell Biology, Faculty of Medicine, University of Yamanashi, Chuoh-shi 409-3898 (Japan); Fujimuro, Masahiro, E-mail: fuji2@mb.kyoto-phu.ac.jp [Department of Cell Biology, Kyoto Pharmaceutical University, Misasagi-Shichonocho 1, Yamashinaku, Kyoto 607-8412 (Japan)

    2014-02-07

    Highlights: • Sangivamycin induces the apoptosis of B cell lymphoma PEL cells. • Sangivamycin suppresses Erk signaling by inhibiting Erk phosphorylation in PEL cells. • The activation of Erk signaling is essential for PEL cell survival. • Sangivamycin induces the apoptosis of PEL cells without production of progeny virus. • Sangivamycin may serve as a novel drug for the treatment of PEL. - Abstract: Sangivamycin, a structural analog of adenosine and antibiotic exhibiting antitumor and antivirus activities, inhibits protein kinase C and the synthesis of both DNA and RNA. Primary effusion lymphoma (PEL) is an aggressive neoplasm caused by Kaposi’s sarcoma-associated herpesvirus (KSHV) in immunosuppressed patients and HIV-infected homosexual males. PEL cells are derived from post-germinal center B cells, and are infected with KSHV. Herein, we asked if sangivamycin might be useful to treat PEL. We found that sangivamycin killed PEL cells, and we explored the underlying mechanism. Sangivamycin treatment drastically decreased the viability of PEL cell lines compared to KSHV-uninfected B lymphoma cell lines. Sangivamycin induced the apoptosis of PEL cells by activating caspase-7 and -9. Further, sangivamycin suppressed the phosphorylation of Erk1/2 and Akt, thus inhibiting activation of the proteins. Inhibitors of Akt and MEK suppressed the proliferation of PEL cells compared to KSHV-uninfected cells. It is known that activation of Erk and Akt signaling inhibits apoptosis and promotes proliferation in PEL cells. Our data therefore suggest that sangivamycin induces apoptosis by inhibiting Erk and Akt signaling in such cells. We next investigated whether sangivamycin, in combination with an HSP90 inhibitor geldanamycin (GA) or valproate (valproic acid), potentiated the cytotoxic effects of the latter drugs on PEL cells. Compared to treatment with GA or valproate alone, the addition of sangivamycin enhanced cytotoxic activity. Our data thus indicate that

  19. Sangivamycin induces apoptosis by suppressing Erk signaling in primary effusion lymphoma cells

    International Nuclear Information System (INIS)

    Wakao, Kazufumi; Watanabe, Tadashi; Takadama, Tadatoshi; Ui, Sadaharu; Shigemi, Zenpei; Kagawa, Hiroki; Higashi, Chizuka; Ohga, Rie; Taira, Takahiro; Fujimuro, Masahiro

    2014-01-01

    Highlights: • Sangivamycin induces the apoptosis of B cell lymphoma PEL cells. • Sangivamycin suppresses Erk signaling by inhibiting Erk phosphorylation in PEL cells. • The activation of Erk signaling is essential for PEL cell survival. • Sangivamycin induces the apoptosis of PEL cells without production of progeny virus. • Sangivamycin may serve as a novel drug for the treatment of PEL. - Abstract: Sangivamycin, a structural analog of adenosine and antibiotic exhibiting antitumor and antivirus activities, inhibits protein kinase C and the synthesis of both DNA and RNA. Primary effusion lymphoma (PEL) is an aggressive neoplasm caused by Kaposi’s sarcoma-associated herpesvirus (KSHV) in immunosuppressed patients and HIV-infected homosexual males. PEL cells are derived from post-germinal center B cells, and are infected with KSHV. Herein, we asked if sangivamycin might be useful to treat PEL. We found that sangivamycin killed PEL cells, and we explored the underlying mechanism. Sangivamycin treatment drastically decreased the viability of PEL cell lines compared to KSHV-uninfected B lymphoma cell lines. Sangivamycin induced the apoptosis of PEL cells by activating caspase-7 and -9. Further, sangivamycin suppressed the phosphorylation of Erk1/2 and Akt, thus inhibiting activation of the proteins. Inhibitors of Akt and MEK suppressed the proliferation of PEL cells compared to KSHV-uninfected cells. It is known that activation of Erk and Akt signaling inhibits apoptosis and promotes proliferation in PEL cells. Our data therefore suggest that sangivamycin induces apoptosis by inhibiting Erk and Akt signaling in such cells. We next investigated whether sangivamycin, in combination with an HSP90 inhibitor geldanamycin (GA) or valproate (valproic acid), potentiated the cytotoxic effects of the latter drugs on PEL cells. Compared to treatment with GA or valproate alone, the addition of sangivamycin enhanced cytotoxic activity. Our data thus indicate that

  20. Male Breast Cancer as a Second Primary Cancer: Increased Risk Following Lymphoma.

    Science.gov (United States)

    Farr, Deborah E; Thomas, Alexandra; Khan, Seema Ahsan; Schroeder, Mary C

    2017-08-01

    Male breast cancer (MBC) as a second primary cancer (SPC) has a known association with prior MBC. However, its association with non-breast index malignancies, relative to population risk, has not been previously reported. Using Surveillance, Epidemiology, and End Results program (9 catchment area) data, we identified MBCs diagnosed from 1973-2012 as their SPC. Information regarding the index malignancy was also obtained. Standardized incidence ratios (SIR) of MBC as SPC were estimated, along with incidence rates and trends. Kaplan-Meier curves were used to estimate survival. Over a 38-year period, 464 MBCs were identified as SPC. The most common index malignancies were breast (SIR 30.86, 95% confidence interval [CI] 21.50-42.92, p  Male breast cancer as a SPC has increased markedly over 4 decades. Men with a history of lymphoma may experience higher-than-expected rates of breast SPC. These observations warrant further research, and suggest possible etiologic connections with disease biology, prior therapy, or genetics. This study reports that men are presenting more frequently to the clinic with breast cancer, both as an initial cancer and as a second cancer following an earlier malignancy. We also report the novel observation that men who survive lymphoma are at increased risk of developing a subsequent breast cancer. Further work is needed to better understand possible treatment or biologic causes of this association. More immediately, these findings suggest the need for heightened vigilance for male breast cancer overall and, in particular, for male lymphoma survivors. © AlphaMed Press 2017.

  1. Primary EBV-positive Hodgkin's lymphoma of the CNS under azathioprine treatment. Case report and review of the literature

    International Nuclear Information System (INIS)

    Henkenberens, Christoph; Christiansen, Hans; Franzke, Anke; Raab, Peter; Oschlies, Ilske; Klapper, Wolfram

    2014-01-01

    Retrospective and prospective cohort studies suggest that central nervous system involvement occurs in approximately 0.5 % of patients with advanced Hodgkin's lymphoma. The isolated primary intracranial manifestation of Hodgkin's lymphoma is an extremely rare finding, with few cases reported in the literature. Little is known about the optimal treatment and prognosis of these tumors. Here, we present a case report with a review of the literature. A 47-year-old Caucasian man with persistent frontal headache and unspecific vertigo for half a month was diagnosed with nodular space-occupying lesions in the cerebellum. His medical history included multiple sclerosis, which was treated for 20 years with the immunosuppressive drug azathioprine. Further staging revealed no additional lesions suspected of being malignant. The patient underwent total tumor resection. Immunohistopathological examination showed Epstein-Barr virus-associated classic Hodgkin's lymphoma. Diagnostic bone marrow punction excluded lymphoma involvement of the bone marrow. The patient had no B symptoms. Consequently, the patient was classified as having stage I E A disease according to the Modified Ann Arbor Classification of Hodgkin Lymphoma and received systemic chemotherapy followed by radiation therapy for the former cerebellar tumor region. He was in complete clinical remission at the last follow-up 9 months after the initial diagnosis. This case report and literature review suggest that multimodal treatment leads to a remarkable clinical outcome in Hodgkin's lymphoma with intracranial involvement. (orig.) [de

  2. SU-F-R-13: Decoding 18F-FDG Uptake Heterogeneity for Primary and Lymphoma Tumors by Using Texture Analysis in PET Images

    Energy Technology Data Exchange (ETDEWEB)

    Ma, C; Yin, Y [Shandong Cancer Hospital and Institute, Jinan, Shandong (China)

    2016-06-15

    Purpose: To explore 18F-FDG uptake heterogeneity of primary tumor and lymphoma tumor by texture features of PET image and quantify the heterogeneity difference between primary tumor and lymphoma tumor. Methods: 18 patients with primary tumor and lymphoma tumor in lung cancer were enrolled. All patients underwent whole-body 18F-FDG PET/CT scans before treatment. Texture features, based on Gray-level Co-occurrence Matrix, second and high order matrices are extracted from code using MATLAB software to quantify 18F-FDG uptake heterogeneity. The relationships of volume between energy, entropy, correlation, homogeneity and contrast were analyzed. Results: For different cases, tumor heterogeneity was not the same. Texture parameters (contrast, entropy, and correlation) of lymphoma were lower than primary tumor. On the contrast, the texture parameters (energy, homogeneity and inverse different moment) of lymphoma were higher than primary tumor. Significantly, correlations were observed between volume and energy (primary, r=−0.194, p=0.441; lymphoma, r=−0.339, p=0.582), homogeneity (primary, r=−0.146, p=0.382; lymphoma, r=−0.193, p=0.44), inverse difference moment (primary, r=−0.14, p=0.374; lymphoma, r=−0.172, p=0.414) and a positive correlation between volume and entropy (primary, r=0.233, p=0.483; lymphoma, r=0.462, p=0.680), contrast (primary, r=0.159, p=0.399; lymphoma, r=0.341, p=0.584), correlation (primary, r=0.027, p=0.165; lymphoma, r=0.046, p=0.215). For the same patient, energy for primary and lymphoma tumor is equal. The volume of lymphoma is smaller than primary tumor, but the homogeneity were higher than primary tumor. Conclusion: This study showed that there were effective heterogeneity differences between primary and lymphoma tumor by FDG-PET image texture analysis.

  3. SU-F-R-13: Decoding 18F-FDG Uptake Heterogeneity for Primary and Lymphoma Tumors by Using Texture Analysis in PET Images

    International Nuclear Information System (INIS)

    Ma, C; Yin, Y

    2016-01-01

    Purpose: To explore 18F-FDG uptake heterogeneity of primary tumor and lymphoma tumor by texture features of PET image and quantify the heterogeneity difference between primary tumor and lymphoma tumor. Methods: 18 patients with primary tumor and lymphoma tumor in lung cancer were enrolled. All patients underwent whole-body 18F-FDG PET/CT scans before treatment. Texture features, based on Gray-level Co-occurrence Matrix, second and high order matrices are extracted from code using MATLAB software to quantify 18F-FDG uptake heterogeneity. The relationships of volume between energy, entropy, correlation, homogeneity and contrast were analyzed. Results: For different cases, tumor heterogeneity was not the same. Texture parameters (contrast, entropy, and correlation) of lymphoma were lower than primary tumor. On the contrast, the texture parameters (energy, homogeneity and inverse different moment) of lymphoma were higher than primary tumor. Significantly, correlations were observed between volume and energy (primary, r=−0.194, p=0.441; lymphoma, r=−0.339, p=0.582), homogeneity (primary, r=−0.146, p=0.382; lymphoma, r=−0.193, p=0.44), inverse difference moment (primary, r=−0.14, p=0.374; lymphoma, r=−0.172, p=0.414) and a positive correlation between volume and entropy (primary, r=0.233, p=0.483; lymphoma, r=0.462, p=0.680), contrast (primary, r=0.159, p=0.399; lymphoma, r=0.341, p=0.584), correlation (primary, r=0.027, p=0.165; lymphoma, r=0.046, p=0.215). For the same patient, energy for primary and lymphoma tumor is equal. The volume of lymphoma is smaller than primary tumor, but the homogeneity were higher than primary tumor. Conclusion: This study showed that there were effective heterogeneity differences between primary and lymphoma tumor by FDG-PET image texture analysis.

  4. Primary Diffuse Large B-Cell Lymphoma Localized to the Lacrimal Sac: A Case Presentation and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Kevin Zarrabi

    2016-01-01

    Full Text Available We report a rare case of diffuse large B-cell lymphoma (DLBCL of the lacrimal sac in a 50-year-old male. The incidence of primary ocular lymphoma is low and it is considered a rare disease. Moreover, reports of ocular DLBCL are uncommon and the disease remains poorly characterized. Our patient presented for management of osteomyelitis and was incidentally found to have a painless swelling and cyst around his right eye. A PET/CT scan revealed hypermetabolic activity within the lacrimal sac and a subsequent excisional biopsy of the mass yielded histopathology consistent with DLBCL. Consequently, the patient underwent treatment with R-CHOP therapy. The patient responded well to chemotherapy with a substantial shrinkage in tumor burden and the disease remained localized. Herein, we present a rare case of primary ocular lymphoma, highlight the importance of early diagnosis, and review current treatment modalities.

  5. The retinoid X receptor agonist, 9-cis UAB30, inhibits cutaneous T-cell lymphoma proliferation through the SKP2-p27kip1 axis.

    Science.gov (United States)

    Chou, Chu-Fang; Hsieh, Yu-Hua; Grubbs, Clinton J; Atigadda, Venkatram R; Mobley, James A; Dummer, Reinhard; Muccio, Donald D; Eto, Isao; Elmets, Craig A; Garvey, W Timothy; Chang, Pi-Ling

    2018-06-01

    Bexarotene (Targretin ® ) is currently the only FDA approved retinoid X receptor (RXR) -selective agonist for the treatment of cutaneous T-cell lymphomas (CTCLs). The main side effects of bexarotene are hypothyroidism and elevation of serum triglycerides (TGs). The novel RXR ligand, 9-cis UAB30 (UAB30) does not elevate serum TGs or induce hypothyroidism in normal subjects. To assess preclinical efficacy and mechanism of action of UAB30 in the treatment of CTCLs and compare its action with bexarotene. With patient-derived CTCL cell lines, we evaluated UAB30 function in regulating growth, apoptosis, cell cycle check points, and cell cycle-related markers. Compared to bexarotene, UAB30 had lower half maximal inhibitory concentration (IC 50 ) values and was more effective in inhibiting the G1 cell cycle checkpoint. Both rexinoids increased the stability of the cell cycle inhibitor, p27kip1 protein, in part, through targeting components involved in the ubiquitination-proteasome system: 1) decreasing SKP2, a F-box protein that binds and targets p27kip1 for degradation by 26S proteasome and 2) suppressing 20S proteasome activity (cell line-dependent) through downregulation of PSMA7, a component of the 20S proteolytic complex in 26S proteasome. UAB30 and bexarotene induce both early cell apoptosis and suppress cell proliferation. Inhibition of the G1 to S cell cycle transition by rexinoids is mediated, in part, through downregulation of SKP2 and/or 20S proteasome activity, leading to increased p27kip1 protein stability. Because UAB30 has minimal effect in elevating serum TGs and inducing hypothyroidism, it is potentially a better alternative to bexarotene for the treatment of CTCLs. Copyright © 2018 Japanese Society for Investigative Dermatology. Published by Elsevier B.V. All rights reserved.

  6. 'We're all carrying a burden that we're not sharing': a qualitative study of the impact of cutaneous T-cell lymphoma on the family.

    Science.gov (United States)

    Selman, L E; Beynon, T; Radcliffe, E; Whittaker, S; Orlowska, D; Child, F; Harding, R

    2015-06-01

    Cutaneous T-cell lymphoma (CTCL) is a rare, progressive cancer that can be life limiting and highly disfiguring. Patients with CTCL experience poor quality of life; however, there is little published about the experiences of their families. To describe the impact of CTCL on family members and how they cope and adjust, to inform support services. Semistructured qualitative interviews were conducted with adult informal caregivers of patients with CTCL recruited via a supraregional CTCL clinic. Interviews explored the history of each patient's illness, the impact of CTCL on the patient and the family, and views about family support. Data were analysed thematically using the Family Adjustment and Adaptation Response model as an interpretative framework. Fourteen caregivers were interviewed (11 spouses, one friend, two daughters; 10 women, four men; all white British; aged 39-85 years). Three key themes emerged: (i) demands of CTCL (the disease, caregiving, financial impact, physical and emotional intimacy); (ii) family capabilities (family support, information, healthcare provider support, other coping strategies); and (iii) adjustment and adaptation (acceptance, changes in patient-caregiver relationship and family dynamics). CTCL was central in many aspects of caregivers' lives, particularly relationships, communication and intimacy. Our findings demonstrate the multiple demands that CTCL places on caregivers, the capabilities and resources they draw upon to cope, and the significant impact of CTCL on the family. To support families and patients, easily accessible services are needed that include the family in the unit of care, provide support and information, and understand the process of family adjustment and adaptation. © 2014 British Association of Dermatologists.

  7. Primary pulmonary lymphoma mimicking a refractory lung abscess: A case report.

    Science.gov (United States)

    Matsumoto, Takeshi; Otsuka, Kojiro; Funayama, Yuki; Imai, Yukihiro; Tomii, Keisuke

    2015-04-01

    The current study presents a case of primary pulmonary lymphoma (PPL) mimicking refractory lung abscess that was diagnosed at autopsy. An 80-year-old male with clinically inapparent aspiration presented with a large cavitated mass and pleural effusion. A lung abscess and empyema was diagnosed, therefore, antibiotics were administered and the pleural effusion was drained. Various examinations, including a biopsy, yielded no specific diagnosis. The lesion was considered inoperable due to the poor general condition of the patient. Subsequently, the mass that had been diagnosed as a refractory lung abscess became enlarged and a repeat biopsy resulted in a diagnosis of diffuse large B-cell lymphoma. The patient succumbed to sudden respiratory failure, and the final diagnosis of PPL was confirmed at autopsy. PPL is a rare disease that accounts for 0.45% of all pulmonary malignant tumors and is difficult to diagnose in inoperable cases. Therefore, patients with PPL who do not undergo surgery can be misdiagnosed and consequently treated inappropriately. PPL should therefore be considered in the differential diagnosis of a refractory lung abscess.

  8. Whole transcriptome sequencing enables discovery and analysis of viruses in archived primary central nervous system lymphomas.

    Directory of Open Access Journals (Sweden)

    Christopher DeBoever

    Full Text Available Primary central nervous system lymphomas (PCNSL have a dramatically increased prevalence among persons living with AIDS and are known to be associated with human Epstein Barr virus (EBV infection. Previous work suggests that in some cases, co-infection with other viruses may be important for PCNSL pathogenesis. Viral transcription in tumor samples can be measured using next generation transcriptome sequencing. We demonstrate the ability of transcriptome sequencing to identify viruses, characterize viral expression, and identify viral variants by sequencing four archived AIDS-related PCNSL tissue samples and analyzing raw sequencing reads. EBV was detected in all four PCNSL samples and cytomegalovirus (CMV, JC polyomavirus (JCV, and HIV were also discovered, consistent with clinical diagnoses. CMV was found to express three long non-coding RNAs recently reported as expressed during active infection. Single nucleotide variants were observed in each of the viruses observed and three indels were found in CMV. No viruses were found in several control tumor types including 32 diffuse large B-cell lymphoma samples. This study demonstrates the ability of next generation transcriptome sequencing to accurately identify viruses, including DNA viruses, in solid human cancer tissue samples.

  9. Primary T cell central nervous system lymphoblastic lymphoma in a child: case report and literature review.

    Science.gov (United States)

    Mazur, Marcus D; Ravindra, Vijay M; Alashari, Mouied; Raetz, Elizabeth; Poppe, Matthew M; Bollo, Robert J

    2015-06-01

    Primary central nervous system lymphoma (PCNSL) of T cell origin is rare in pediatric patients. We report a case of T cell PCNSL in a 12-year-old boy and review the literature to highlight the importance of brain biopsy to definitively establish the diagnosis when PCNSL is suspected. A 12-year-old boy presented with worsening left-sided weakness, nausea, vomiting, headache, blurred vision, and diplopia. Magnetic resonance imaging revealed right parietal gyral thickening with faint meningeal contrast enhancement. No clear diagnosis was identified after serum testing, cerebrospinal fluid analysis, and cerebral angiography. To establish the diagnosis definitively, a right craniotomy and open, frameless stereotactic biopsy were performed, which yielded the diagnosis of lymphoblastic T cell lymphoma. PCNSL of T cell origin in children remains poorly studied, with only 18 detailed cases reported over the last three decades, including this case. Establishing a definitive diagnosis of PCNSL is challenging, and a brain biopsy is often required to obtain enough tissue for pathological analysis. Increasing awareness and identification of children diagnosed with T cell PCNSL is needed to better understand the molecular biology of this disease and develop more standardized treatment regimens.

  10. Primary cervical and uterine corpus lymphoma; a case report and literature review

    Czech Academy of Sciences Publication Activity Database

    Anagnostopoulos, A.; Mouzakiti, N.; Ruthven, S.; Herod, J.; Kotsyfakis, Michalis

    2013-01-01

    Roč. 6, č. 4 (2013), s. 298-306 ISSN 1940-5901 R&D Projects: GA ČR GAP502/12/2409 Institutional support: RVO:60077344 Keywords : Non-Hodgkin's lymphoma * extra-nodal lymphoma * uterine/cervical lymphoma Subject RIV: EC - Immunology Impact factor: 1.422, year: 2013

  11. Primary Diffuse Large B-cell Lymphoma of the Uterus Manifesting as a Leiomyoma: A Unique Presentation with Review of Literature

    Directory of Open Access Journals (Sweden)

    Rajan Dewar

    2013-01-01

    Full Text Available We report a primary diffuse large B-cell lymphoma of uterine corpus in a 70-years old woman who presented with symptoms of increased urinary frequency and sense of bloating. Magnetic Resonance Imaging (MRI findings were suggestive of a degenerating intramural fibroid. Histological examination of tissue samples obtained during hysteroscopy showed diffuse infiltration of fibrous stroma by atypical enlarged mononuclear cells. Immunohistochemical studies were consistent with the diagnosis of diffuse large B-cell lymphoma.Further imaging studies showed no evidence of lymphoma outside the uterus. To our knowledge,this represents the first welldocumented case of primary uterine lymphoma presenting as a leiomyoma on imaging studies.

  12. Composite cutaneous lymphoma (iatrogenic immunodeficiency-associated lymphoproliferative disorder) in a patient with rheumatoid arthritis treated with methotrexate: Staging and evaluation of response to therapy with "1'8F-FDG PET/CT

    International Nuclear Information System (INIS)

    Makis, William; Ciarallo, Anthony; Gonzalez-Verdecia, Milene; Wang, Beatrice; Probst, Stehan

    2017-01-01

    A 67 year old woman with a 10 year history of rheumatoid arthritis (RA) treated with methotrexate and prednisone, presented with a 2 year history of worsening multiple cutaneous plaques of variable appearance. Two distinct skin lesions were biopsied to reveal a composite cutaneous lymphoma, possibly caused by long term methotrexate therapy. An [18F] fluoro-2-deoxy-D-glucose ("1"8F-FDG) positron emission tomography/computed tomography (PET/CT) was performed to stage the malignancy, and was later repeated to evaluate response to chemotherapy, which guided subsequent management. We present the PET/CT imaging findings of this very rare iatrogenic (methotrexate induced) immunodeficiency-associated lymphoproliferative disorder

  13. Composite cutaneous lymphoma (iatrogenic immunodeficiency-associated lymphoproliferative disorder) in a patient with rheumatoid arthritis treated with methotrexate: Staging and evaluation of response to therapy with {sup 1}'8F-FDG PET/CT

    Energy Technology Data Exchange (ETDEWEB)

    Makis, William [Dept. of Diagnostic Imaging, CCI, Diagnostic Imaging, Edmonton (Canada); Ciarallo, Anthony; Gonzalez-Verdecia, Milene [MUHC Glen Site, Montreal (Canada); Wang, Beatrice [MUHC, Dermatology, Westmount (Canada); Probst, Stehan [MUHC Jewish General Hospital, Nuclear Medicine, Montreal (Canada)

    2017-09-15

    A 67 year old woman with a 10 year history of rheumatoid arthritis (RA) treated with methotrexate and prednisone, presented with a 2 year history of worsening multiple cutaneous plaques of variable appearance. Two distinct skin lesions were biopsied to reveal a composite cutaneous lymphoma, possibly caused by long term methotrexate therapy. An [18F] fluoro-2-deoxy-D-glucose ({sup 18}F-FDG) positron emission tomography/computed tomography (PET/CT) was performed to stage the malignancy, and was later repeated to evaluate response to chemotherapy, which guided subsequent management. We present the PET/CT imaging findings of this very rare iatrogenic (methotrexate induced) immunodeficiency-associated lymphoproliferative disorder.

  14. Primary pulmonary lymphoma-role of fluoro-deoxy-glucose positron emission tomography-computed tomography in the initial staging and evaluating response to treatment - case reports and review of literature

    International Nuclear Information System (INIS)

    Agarwal, Krishan Kant; Dhanapathi, Halanaik; Nazar, Aftab Hasan; Kumar, Rakesh

    2016-01-01

    Primary pulmonary lymphoma (PPL) is an uncommon entity of non-Hodgkin lymphoma, which accounts for <1% of all cases of lymphoma. We present two rare cases of PPL of diffuse large B-cell lymphoma, which underwent 18 fluorine fluoro-deoxy-glucose positron emission tomography-computed tomography for initial staging and response evaluation after chemotherapy

  15. Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma (CLL/SLL)

    Science.gov (United States)

    ... Non-Hodgkin Lymphoma Peripheral T-Cell Lymphoma Primary Central Nervous System Lymphoma T-Cell Lymphoma Transformed Mycosis Fungoides Waldenstrom Macroglobulinemia Young Adult Lymphoma Overview Treatment Options Relapsed/Refractory Long-Term ...

  16. MR findings of primary bone lymphoma in a 15-year-old girl: emphasis on diffusion-weighted imaging

    Energy Technology Data Exchange (ETDEWEB)

    Garrett, Kevin M.; Kim, Hee Kyung; Emery, Kathleen H. [Cincinnati Children' s Hospital Medical Center, Department of Radiology, Cincinnati, OH (United States); Stanek, Jerzy [Cincinnati Children' s Hospital Medical Center, Department of Pathology, Cincinnati, OH (United States)

    2011-05-15

    We report a case of primary bone lymphoma (PBL) in a 15-year-old girl assessed by MR imaging with diffusion-weighted imaging (DWI). DWI has been shown to help characterize the cellularity of solid tumors and this case correlates well with previous data. (orig.)

  17. Primary vertebral and spinal epidural non-Hodgkin's lymphoma with spinal cord compression

    International Nuclear Information System (INIS)

    Boukobza, M.; Mazel, C.; Touboul, E.

    1996-01-01

    We examined eight patients with primary spinal epidural non-Hodgkin's lymphoma presenting with spinal cord compression and proven histologically after laminectomy (7 cases) or biopsy (1 case) by MRI. The most common findings were an isointense or low signal relative to the spinal cord on T1-weighted images (T1WI) and high signal on T2-weighted images (T2WI). Spinal cord compression, vertebral bone marrow and paravertebral extension were assessed. Contrast enhancement was intense in seven of the eight cases and homogeneous in all of them. T2WI (performed in 2 cases) may be useful to distinguish metastatic carcinomas and sarcomas. T1WI demonstrated the full extent of the epidural lesion, which was well-delineated in all cases. When the paravertebral extension is not well-defined, a study with contrast medium should be performed. (orig.). With 3 figs., 1 tab

  18. A retrospective analysis of different modalities for treatment of primary orbital non-Hodgkin's lymphomas

    International Nuclear Information System (INIS)

    Esik, Olga; Ikeda, H.; Mukai, K.; Kaneko, A.

    1996-01-01

    We have reviewed 37 patients with primary orbital lymphoma, using the Ann Arbor criteria and the Working Formulation and its modification. Thirty-one patients had stage I disease, four stage II, one stage III and one stage IV. The male to female ratio was 2.7:1. There were 34 low-grade tumours (including 24 mantle zone) and three intermediate-grade. Patients were divided into three groups according to their primary treatment. Group 1: radiotherapy (17 cases); Group 2: surgery alone (13 cases); Group 3: chemotherapy (seven cases). Patients were followed up from 5 months to 24.3 years, with a mean and median of 7.6 and 6.2 years, respectively. The BMDP software package was used for survival estimation (Kaplan-Meier) and determination of prognostic variable (univariate Cox regression). Local relapse-free survival at 10 years was 100% in Group 1, 0% in Group 2 and 42% in Group 3 with a statistically significant difference (p < 0.01) in favour of radiotherapy. Statistically significant good prognostic features were: complete remission (CR) in response to initial treatment, primary radiotherapy and older age. For stage I cases, there was no difference in distant relapse-free survival in the three groups. The overall cause-specific survival for stage I patients at 10 years was 100% for each group and at 20 years was 100, 67 and 0% for Group 1, 2 and 3. The difference between the primary radiotherapy and chemotherapy-treated groups was significant at the p = 0.08 level. Statistically significant prognostic factors were early stage, low-grade histology and primary radiotherapy. In one patient, ptosis and diplopia appeared after surgery. One case of glaucoma required enucleation, one patient suffered severe dry eye syndrome. All patients ((11(11))) in whom the lens received direct radiation developed cataracts of different degrees if follow-up was long enough. Cataract formation was prevented by adequate lens shielding. One patient in CR from a stage I low-grade tumour died

  19. Multifactorial Hypercalcemia and Literature Review on Primary Hyperparathyroidism Associated with Lymphoma

    Directory of Open Access Journals (Sweden)

    Jelena Maletkovic

    2014-01-01

    Full Text Available The most common cause of hypercalcemia in hospitalized patients is malignancy. Primary hyperparathyroidism most commonly causes hypercalcemia in the outpatient setting. These two account for over 90% of all cases of hypercalcemia. Hypercalcemia can be divided into PTH-mediated and PTH-independent variants. Primary hyperparathyroidism, familial hypocalciuric hypercalcemia, familial hyperparathyroidism, and secondary hyperparathyroidism are PTH mediated. The most common PTH-independent type of hypercalcemia is malignancy related. Several mechanisms lead to hypercalcemia in malignancy-direct osteolysis by metastatic disease or, more commonly, production of humoral factors by the primary tumor also known as humoral hypercalcemia of malignancy that accounts for about 80% of malignancy-related hypercalcemia. The majority of HHM is caused by tumor-produced parathyroid hormone-related protein and less frequently production of 1,25-dihydroxyvitamin D or parathyroid hormone by the tumor. We report the rare case of a patient with hypercalcemia and diagnosed primary hyperparathyroidism. The patient had persistent hypercalcemia after surgical removal of parathyroid adenoma with recorded significant decrease in PTH level. After continued investigation it was found that the patient also had elevated 1,25-dihydroxyvitamin D and further studies confirmed a large spleen mass that was later confirmed to be a lymphoma. This is a rare example of two concomitant causes of hypercalcemia requiring therapy.

  20. Randomized Clinical Trial to Assess the Efficacy of Radiotherapy in Primary Mediastinal Large B-Lymphoma

    Energy Technology Data Exchange (ETDEWEB)

    Aviles, Agustin, E-mail: agustin.aviles@imss.gob.mx [Oncology Research Unit, Oncology Hospital, National Medical Center, IMSS, Mexico, D. F. (Mexico); Neri, Natividad [Department of Hematology, Oncology Hospital, National Medical Center, IMSS, Mexico, D. F. (Mexico); Fernandez, Raul [Department of Radiation Therapy, Oncology Hospital, National Medical Center, IMSS, Mexico, D. F. (Mexico); Huerta-Guzman, Judith; Nambo, Maria J. [Department of Hematology, Oncology Hospital, National Medical Center, IMSS, Mexico, D. F. (Mexico)

    2012-07-15

    Purpose: We developed a controlled clinical trial to assess the efficacy and toxicity of adjuvant-involved field radiotherapy (IFRT) in patients with primary mediastinal B-cell lymphoma that achieved complete response after the patients were treated with cyclophosphamide, doxorubicin, vincristine, prednisone, and rituximab (R-CHOP-14). Methods and Materials: Between January 2001 and June 2004, 124 consecutive patients who were in complete remission after dose dense chemotherapy and rituximab administration (R-CHOP14) were randomly assigned to received IFRT (30 Gy). Sixty-three patients received IFR, and 61 patients did not (control group). Results: The study aimed to include 182 patients in each arm but was closed prematurely because in a security analysis (June 2004), progression and early relapse were more frequent in patients that did not received IFRT. Patients were followed until March 2009, at which point actuarial curves at 10 years showed that progression free-survival was 72% in patients who received IFR and 20% in the control group (p < 0.001), overall survival was 72% and 31%, respectively (p < 0.001). Acute toxicity was mild and well tolerated. Discussion: Adjuvant radiotherapy to sites of bulky disease was the only difference to have an improvement in outcome in our patients; the use of rituximab during induction did not improve complete response rates and did affect overall survival; patients who received rituximab but not IFRT had a worse prognosis. Conclusions: The use of IFRT in patients with primary mediastinal B-cell lymphoma who achieved complete response remain as the best treatment available, even in patients that received rituximab during induction.

  1. Randomized Clinical Trial to Assess the Efficacy of Radiotherapy in Primary Mediastinal Large B-Lymphoma

    International Nuclear Information System (INIS)

    Avilés, Agustin; Neri, Natividad; Fernández, Raúl; Huerta-Guzmán, Judith; Nambo, María J.

    2012-01-01

    Purpose: We developed a controlled clinical trial to assess the efficacy and toxicity of adjuvant-involved field radiotherapy (IFRT) in patients with primary mediastinal B-cell lymphoma that achieved complete response after the patients were treated with cyclophosphamide, doxorubicin, vincristine, prednisone, and rituximab (R-CHOP-14). Methods and Materials: Between January 2001 and June 2004, 124 consecutive patients who were in complete remission after dose dense chemotherapy and rituximab administration (R-CHOP14) were randomly assigned to received IFRT (30 Gy). Sixty-three patients received IFR, and 61 patients did not (control group). Results: The study aimed to include 182 patients in each arm but was closed prematurely because in a security analysis (June 2004), progression and early relapse were more frequent in patients that did not received IFRT. Patients were followed until March 2009, at which point actuarial curves at 10 years showed that progression free-survival was 72% in patients who received IFR and 20% in the control group (p < 0.001), overall survival was 72% and 31%, respectively (p < 0.001). Acute toxicity was mild and well tolerated. Discussion: Adjuvant radiotherapy to sites of bulky disease was the only difference to have an improvement in outcome in our patients; the use of rituximab during induction did not improve complete response rates and did affect overall survival; patients who received rituximab but not IFRT had a worse prognosis. Conclusions: The use of IFRT in patients with primary mediastinal B-cell lymphoma who achieved complete response remain as the best treatment available, even in patients that received rituximab during induction.

  2. Burkitts primary thyroid lymphoma coexistence with Hashimoto's thyroiditis; Linfoma Burkitt primario tiroideo. Coexistencia con tiroiditis de Hashimoto

    Energy Technology Data Exchange (ETDEWEB)

    Higuera, A. [Hospital Alto Guadalquivir. Andujar Jaen (Spain); Vicente, J.; Lazaro, J. C. [Hospital Universitario Reina Sofia. Cordoba (Spain)

    2000-07-01

    Th primary thyroid lymphoma is a rare neoplasm, above all in children. We present a case of a child with Burkitt's thyroid lymphoma as the only manifestation of this disease, associated to lymphocytic thyroiditis. Clinically, it initiated as a rapidly growing goiter with compressive symptomatology. The X-ray findings are described: hypoechoic and hypodense multiple nodes that affect the right thyroid lobe and isthmus, with extraglandular extension to the vascular space and to the mediastinum. The differential diagnosis is considered with other more frequent thyroid pathologies in this age group. (Author) 14 refs.

  3. Primary focal T-cell lymphoma of the liver: a case report and review of the literature.

    Science.gov (United States)

    Cerban, Razvan; Gheorghe, Liana; Becheanu, Gabriel; Serban, Valentin; Gheorghe, Cristian

    2012-06-01

    We present the case of a previously healthy 62 year old man who developed primary non-Hodgkin lymphoma of the liver. Biopsy confirmed that it was a diffuse large anaplastic T-cell lymphoma of an extremely rare type. The diagnosis of this type of lesions is suggested by the presence of a hepatic mass without lymphadenopathy, splenomegaly or bone marrow involvement associated with normal tumor markers (carcinoembryonic antigen, alpha-fetoprotein and CA 19-9 levels). Histological examination of tissue is essential to confirm the diagnosis. Treatment options are surgical resection and/or chemotherapy but the rate of response to treatment varies widely. Some patients can achieve prolonged remission.

  4. Preliminary discussion on the value of 18F-FDG PET/CT in the diagnosis and early staging of non-mycosis fungoides/Sézary's syndrome cutaneous malignant lymphomas

    International Nuclear Information System (INIS)

    Dan, Shao; Qiang, Gao; Shu-Xia, Wang; Chang-Hong, Liang

    2015-01-01

    Highlights: • We discussed the value of PET/CT in the diagnosis and early staging of non-MF/SS CML. • We calculated the sensitivity of CT and PET/CT in the diagnosis of primary skin lesions. • We calculated the value of CT and PET/CT in the diagnosis of LNs and other organs. - Abstract: Objective: To discuss the value of 18 F-fluorodeoxyglucose-positron emission tomography ( 18 F-PET/CT) scans in the diagnosis and early staging of non-mycosis fungoides/Sézary's syndrome cutaneous malignant lymphomas (non-MF/SS CML). Materials and methods: A total of 18 cases with non-MF/SS CML, confirmed by pathology or on clinical grounds, were analyzed in this study. The sensitivity of CT and PET/CT scans in the diagnosis of primary skin lesions, as well as the sensitivity, specificity, accuracy, positive predictive value, and negative predictive value of CT and PET/CT scans in the diagnosis of lymph nodes (LNs) and other organs (except skin and LNs) were calculated. Results: The diagnostic sensitivity of CT and PET/CT scans in the diagnosis of primary skin lesions was 82.4% (14/17) and 100% (17/17), respectively. The diagnostic sensitivity, specificity, accuracy, positive predictive value, and negative predictive value of CT and PET/CT scans in the diagnosis of LN lesions were 55.6% (5/9), 88.9% (8/9), 72.2% (13/18), 83.3% (5/6), 66.7% (8/12), and 88.9% (8/9), 100% (9/9), 94.4% (17/18), 100% (8/8), 90.0% (9/10), respectively. The diagnostic value of the CT and PET/CT scans in the diagnosis of involvement of other organs, were 40.4% (2/5), 100% (13/13), 83.3 (15/18), 100% (2/2), 81.3% (13/16) and 80.6% (4/5), 100% (13/13), 94.4% (17/18), 100% (3/3), 92.9% (13/14), respectively. Conclusions: 18 F-FDG PET/CT has high value in the diagnosis and early staging of non-MF/SS CMLs

  5. Diversity of imaging and pathological features at different stages of primary central nervous system lymphoma

    Directory of Open Access Journals (Sweden)

    Jing LIU

    2018-01-01

    Full Text Available Objective The clinical, imaging and pathological manifestations of one patient at different stages of primary central nervous system lymphoma (PCNSL have been analyzed to disclose its pathogenesis. Methods and Results A 29-year-old female patient showed recurrent onsets. On the first onset, the main clinical manifestation was blurred vision and visual field defect. Cranial MRI showed a patchy lesion in the right parietal and occipital lobes without obvious occupying sign. T1WI showed slight low-intensity sign, T2WI and FLAIR showed high-intensity signs. Enhanced scanning showed heterogeneous punctate enhancement. The lesion disappeared gradually after glucocorticoid impact therapy. Headache, vomiting and left limb paralysis were the main clinical manifestations on the second onset, and MRI showed a lesion in the right frontal and parietal lobes with obvious occupying sign and solid enhancement, low-intensity on T1WI, high-intensity on T2WI and FLAIR. The tumor was totally removed under microscope. Histological findings showed large tumor cells, rich cytoplasm, nuclei with various sizes and shapes, conspicuous mitosis, patchy necrosis, and interstitial small vessel hyperplasia. Immunohistochemical staining showed that membrane of tumor cells was positive for CD20, and nuclei were positive for paired box gene 5 (PAX5 and multiple myeloma oncogene 1 (MUM1. In a few tumor cells, membrane was positive for CD10 and CD30, and nuclei were positive for cyclin D1. Besides, tumor cells were negative for CD3, anaplastic lymphoma kinase (ALK and glial fibrillary acidic protein (GFAP. Ki?67 labeling index was about 80%. EBER in situ hybridization (ISH assay showed that mRNA coded by Epstein?Barr (EB virus was negative. The tumor showed angiotropic characteristics, and the walls of involved blood vessels were wrapped and destructed. The final diagnosis was confirmed as diffuse large B cell lymphoma. Conclusions PCNSL has various imaging features, revealing as

  6. Arctigenin induces the apoptosis of primary effusion lymphoma cells under conditions of glucose deprivation.

    Science.gov (United States)

    Baba, Yusuke; Shigemi, Zenpei; Hara, Naoko; Moriguchi, Misato; Ikeda, Marina; Watanabe, Tadashi; Fujimuro, Masahiro

    2018-02-01

    Kaposi's sarcoma-associated herpesvirus (KSHV) is the causative agent of primary effusion lymphoma (PEL) and Kaposi's sarcoma. PEL is a type of non-Hodgkin's B-cell lymphoma, affecting immunosuppressed individuals, such as post-transplant or AIDS patients. However, since PEL is resistant to chemotherapeutic regimens, new effective treatment strategies are required. Arctigenin, a natural lignan compound found in the plant Arctium lappa, has been widely investigated as a potential anticancer agent in the clinical setting. In the present study, we examined the cytotoxic effects of arctigenin by cell viability assay and found that arctigenin markedly inhibited the proliferation of PEL cells compared with KSHV-uninfected B-lymphoma cells under conditions of glucose deprivation. Arctigenin decreased cellular ATP levels, disrupted mitochondrial membrane potential and triggered caspase-9-mediated apoptosis in the glucose-deprived PEL cells. In addition, western blot analysis using phospho-specific antibodies were used to evaluate activity changes in the signaling pathways of interest. As a result, arctigenin suppressed the activation of the extracellular signal-regulated kinase (ERK) and p38 mitogen-activated protein kinase (p38 MAPK) signaling pathways by inhibiting ERK and p38 MAPK phosphorylation in the glucose-deprived PEL cells. We confirmed that an inhibitor of ERK (U0126) or p38 MAPK (SB202190 and SB203580) suppressed the proliferation of the BC3 PEL cells compared with the KSHV-negative DG75 cells. Moreover, RT-PCR and luciferase reporter assay revealed that arctigenin and p38 MAPK inhibition by SB202190 or SB203580 downregulated the transcriptional expression of unfolded protein response (UPR)‑related molecules, including GRP78 and ATF6α under conditions of glucose deprivation. Finally, we confirmed that arctigenin did not affect KSHV replication in PEL cells, suggesting that arctigenin treatment for PEL does not contribute to the risk of de novo KSHV

  7. Proteasome inhibitors induce apoptosis and reduce viral replication in primary effusion lymphoma cells

    Energy Technology Data Exchange (ETDEWEB)

    Saji, Chiaki [Faculty of Pharmaceutical Sciences, Hokkaido University, Kita-ku, Sapporo 060-0812 (Japan); Higashi, Chizuka; Niinaka, Yasufumi [Faculty of Medicine, University of Yamanashi, Chuoh-shi 409-3898 (Japan); Yamada, Koji [Faculty of Pharmaceutical Sciences, Hokkaido University, Kita-ku, Sapporo 060-0812 (Japan); Noguchi, Kohji [Faculty of Pharmacy, Keio University, 1-5-30 Shiba-koen, Minato-ku, Tokyo 105-8512 (Japan); Fujimuro, Masahiro, E-mail: fuji2@mb.kyoto-phu.ac.jp [Department of Cell Biology, Kyoto Pharmaceutical University, Misasagi-Shichonocho 1, Yamashinaku, Kyoto 607-8412 (Japan)

    2011-12-02

    Highlights: Black-Right-Pointing-Pointer Constitutive NF-{kappa}B signaling is essential for the survival and growth of PEL cells. Black-Right-Pointing-Pointer NF-{kappa}B signaling is upregulated by the proteasome-dependent degradation of I{kappa}B{alpha}. Black-Right-Pointing-Pointer Proteasome inhibitors suppress NF-{kappa}B signaling and induce apoptosis in PEL cells through stabilization of I{kappa}B{alpha}. Black-Right-Pointing-Pointer Proteasome inhibitors suppress viral replication in PEL cells during lytic KSHV infection. -- Abstract: Primary effusion lymphoma (PEL) is an aggressive neoplasm caused by Kaposi's sarcoma-associated herpesvirus (KSHV). This study provides evidence that proteasomal activity is required for both survival of PEL cells stably harboring the KSHV genome and viral replication of KSHV. We evaluated the cytotoxic effects of proteasome inhibitors on PEL cells. The proteasome inhibitors MG132, lactacystin, and proteasome inhibitor I dramatically inhibited cell proliferation and induced apoptosis of PEL cells through the accumulation of p21 and p27. Furthermore, proteasome inhibitors induced the stabilization of NF-{kappa}B inhibitory molecule (I{kappa}B{alpha}) and suppressed the transcriptional activity of NF-{kappa}B in PEL cells. The NF-{kappa}B specific inhibitor BAY11-7082 also induced apoptosis in PEL cells. The constitutive activation of NF-{kappa}B signaling is essential for the survival and growth of B cell lymphoma cells, including PEL cells. NF-{kappa}B signaling is upregulated by proteasome-dependent degradation of I{kappa}B{alpha}. The suppression of NF-{kappa}B signaling by proteasome inhibitors may contribute to the induction of apoptosis in PEL cells. In addition, proteasome activity is required for KSHV replication in KSHV latently infected PEL cells. MG132 reduced the production of progeny virus from PEL cells at low concentrations, which do not affect PEL cell growth. These findings suggest that proteasome

  8. MRI patterns in recurrence of primary CNS lymphoma in immunocompetent patients

    International Nuclear Information System (INIS)

    Schulte-Altedorneburg, Gernot; Heuser, Lothar; Pels, Hendrik

    2012-01-01

    Highlights: ► PCNSL are rare but highly malignant brain tumors. ► PCNSL recur in different anatomic sites compared with initial presentation. ► Non-parenchymal contrast enhancement is a frequent finding at initialdiagnosis and at relapse. -- Abstract: Purpose: Primary CNS lymphomas (PCNSL) are highly malignant non-Hodgkin's B-cell lymphoma restricted to the CNS. While MRI features of PCNSL at initial presentation have been comprehensively described, literature on MRI-characteristics at relapse is sparse. The purpose of this study was to investigate anatomic location and contrast enhancement patterns at PCNSL recurrence by cranial MRI. Methods: Sixteen immunocompetent patients (9 men, 7 women, median age 65 years) with histologically proven PCNSL and initial response to a standardized polychemotherapy, but suffering from a relapse were consecutively recorded. Native and contrast-enhanced MRI examinations carried out at initial presentation and at time of relapse were compared. Anatomical site of parenchymal enhancement, frequency and presence of non-parenchymal contrast enhancement (i.e. ventricular, superficial, subependymal) patterns at initial presentation and at relapse were recorded and compared. Results: Local recurrence was found at the site of the initial tumor presentation in four of the 16 cases. Six of 11 patients presenting a unilateral PCNSL at initial presentation had a bilateral involvement at relapse. In two cases, recurrence appeared solely on the contralateral side without involvement of the hemisphere initially affected. At both dates, subependymal enhancement was the most often found non-parenchymal pattern (six at initial presentation, and five at relapse). The number of patients with a ventricular contrast enhancement increased from one at initial presentation to four at relapse. Conclusions: PCNSL tend to recur in different parenchymal anatomic sites as compared with the site of the initial tumor presentation. Contrast-enhancing non

  9. Leukocyte infiltration and tumor cell plasticity are parameters of aggressiveness in primary cutaneous melanoma.

    NARCIS (Netherlands)

    Hillen, F.; Baeten, C.I.M.; Winkel, van de A.; Creytens, D.; Schaft, van der D.W.J.; Winnepenninckx, V.; Griffioen, A.W.

    2008-01-01

    Various clinical and experimental observations detected an immunological host defense in cutaneous melanoma. In order to investigate the prognostic value of leukocyte effector mechanisms, we examined the presence of different subsets of leukocytes in tumor samples of 58 patients diagnosed with

  10. Primary extranodal lymphomas - spectrum of distribution and morphology with immunophenotyping: A 3-year institutional study

    Directory of Open Access Journals (Sweden)

    Chinnam Aparna

    2015-01-01

    Full Text Available Background: Malignant lymphomas arising in extranodal sites are intriguing. The histological types of lymphomas vary from one site to another. This study is undertaken to diagnose and categorize extranodal lymphomas using histochemistry and immunohistochemistry (IHC. Materials and Methods: Formalin processed paraffin blocks and hematoxylin and eosin stained sections were used for routine histology. IHC was done in all cases. Results: We have encountered 31 cases of extra nodal lymphomas over a period of 3 years. The tumors occurred at different sites, including brain, nasopharynx, nose, gastrointestinal tract, thyroid, bone, testis, breast, lung, vagina, and skin. Majority of the cases were B-cell lymphomas, while four cases were T-cell lymphomas. Among the B-cell lymphomas diffuse large B-cell lymphoma was the most common variant. Conclusion: This study reiterates the key role of IHC particularly when the differential diagnosis includes an undifferentiated epithelial malignancy and a high-grade lymphoma in the extranodal sites as the treatment modalities and prognosis are different.

  11. CD3+, CD56+, CD4−, CD8−, CD20−, CD30− Peripheral T-Cell Non-Hodgkin's Lymphoma: A Rare Case Report

    Directory of Open Access Journals (Sweden)

    Ashish Jagati

    2017-01-01

    Full Text Available Cutaneous T-cell lymphoma (CTCL commonly presents as mycosis fungoides or Sezary syndrome, both having CD4 positivity. A subset of CTCL which lacks CD4 surface marker is classified as cutaneous g and d–T-cell lymphoma (CGD-TCL. Because of its rarity and inability to study large number of patients, the impact of immunophenotype on the clinical outcome of primary CTCL in patients is limited. We report a case of primary CGD-TCL in a 71-year-old male because of this rarity and to emphasize its aggressive nature.

  12. Benefit of Consolidative Radiation Therapy for Primary Bone Diffuse Large B-Cell Lymphoma

    Energy Technology Data Exchange (ETDEWEB)

    Tao, Randa; Allen, Pamela K. [Department of Radiation Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas (United States); Rodriguez, Alma [Department of Lymphoma and Myeloma, The University of Texas MD Anderson Cancer Center, Houston, Texas (United States); Shihadeh, Ferial; Pinnix, Chelsea C.; Arzu, Isadora; Reed, Valerie K. [Department of Radiation Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas (United States); Oki, Yasuhiro; Westin, Jason R.; Fayad, Luis E.; Medeiros, L. Jeffrey [Department of Lymphoma and Myeloma, The University of Texas MD Anderson Cancer Center, Houston, Texas (United States); Dabaja, Bouthaina, E-mail: bdabaja@mdanderson.org [Department of Radiation Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas (United States)

    2015-05-01

    Purpose: Outcomes for patients with diffuse large B-cell lymphoma (DLBCL) differ according to the site of presentation. With effective chemotherapy, the need for consolidative radiation therapy (RT) is controversial. We investigated the influence of primary bone presentation and receipt of consolidative RT on progression-free survival (PFS) and overall survival (OS) in patients with DLBCL. Methods and Materials: We identified 102 patients with primary bone DLBCL treated consecutively from 1988 through 2013 and extracted clinical, pathologic, and treatment characteristics from the medical records. Survival outcomes were calculated by the Kaplan-Meier method, with factors affecting survival determined by log-rank tests. Univariate and multivariate analyses were done with a Cox regression model. Results: The median age was 55 years (range, 16-87 years). The most common site of presentation was in the long bones. Sixty-five patients (63%) received R-CHOP–based chemotherapy, and 74 (72%) received rituximab. RT was given to 67 patients (66%), 47 with stage I to II and 20 with stage III to IV disease. The median RT dose was 44 Gy (range, 24.5-50 Gy). At a median follow-up time of 82 months, the 5-year PFS and OS rates were 80% and 82%, respectively. Receipt of RT was associated with improved 5-year PFS (88% RT vs 63% no RT, P=.0069) and OS (91% vs 68%, P=.0064). On multivariate analysis, the addition of RT significantly improved PFS (hazard ratio [HR] = 0.14, P=.014) with a trend toward an OS benefit (HR=0.30, P=.053). No significant difference in PFS or OS was found between patients treated with 30 to 35 Gy versus ≥36 Gy (P=.71 PFS and P=.31 OS). Conclusion: Patients with primary bone lymphoma treated with standard chemotherapy followed by RT can have excellent outcomes. The use of consolidative RT was associated with significant benefits in both PFS and OS.

  13. Benefit of Consolidative Radiation Therapy for Primary Bone Diffuse Large B-Cell Lymphoma

    International Nuclear Information System (INIS)

    Tao, Randa; Allen, Pamela K.; Rodriguez, Alma; Shihadeh, Ferial; Pinnix, Chelsea C.; Arzu, Isadora; Reed, Valerie K.; Oki, Yasuhiro; Westin, Jason R.; Fayad, Luis E.; Medeiros, L. Jeffrey; Dabaja, Bouthaina

    2015-01-01

    Purpose: Outcomes for patients with diffuse large B-cell lymphoma (DLBCL) differ according to the site of presentation. With effective chemotherapy, the need for consolidative radiation therapy (RT) is controversial. We investigated the influence of primary bone presentation and receipt of consolidative RT on progression-free survival (PFS) and overall survival (OS) in patients with DLBCL. Methods and Materials: We identified 102 patients with primary bone DLBCL treated consecutively from 1988 through 2013 and extracted clinical, pathologic, and treatment characteristics from the medical records. Survival outcomes were calculated by the Kaplan-Meier method, with factors affecting survival determined by log-rank tests. Univariate and multivariate analyses were done with a Cox regression model. Results: The median age was 55 years (range, 16-87 years). The most common site of presentation was in the long bones. Sixty-five patients (63%) received R-CHOP–based chemotherapy, and 74 (72%) received rituximab. RT was given to 67 patients (66%), 47 with stage I to II and 20 with stage III to IV disease. The median RT dose was 44 Gy (range, 24.5-50 Gy). At a median follow-up time of 82 months, the 5-year PFS and OS rates were 80% and 82%, respectively. Receipt of RT was associated with improved 5-year PFS (88% RT vs 63% no RT, P=.0069) and OS (91% vs 68%, P=.0064). On multivariate analysis, the addition of RT significantly improved PFS (hazard ratio [HR] = 0.14, P=.014) with a trend toward an OS benefit (HR=0.30, P=.053). No significant difference in PFS or OS was found between patients treated with 30 to 35 Gy versus ≥36 Gy (P=.71 PFS and P=.31 OS). Conclusion: Patients with primary bone lymphoma treated with standard chemotherapy followed by RT can have excellent outcomes. The use of consolidative RT was associated with significant benefits in both PFS and OS

  14. Identification of primary thyroid lymphoma with medical imaging: A case report and review of the literature.

    Science.gov (United States)

    Wang, Jia-Huan; Chen, Liang; Ren, Ke

    2014-12-01

    Primary thyroid lymphoma (PTL) is a rare thyroid malignancy. Clinical diagnosis of PTL may not be easily established based on imaging studies, as the imaging features of PTL are similar to those of lymphocytic thyroiditis and primary thyroid cancer. The present study describes the case of a patient who was confirmed to have PTL by intra-operative pathological diagnosis. On color Doppler ultrasound, the PTL was shown as a significantly enlarged thyroid with reduced gland echoes. Color Doppler flow imaging showed increased blood flow. By computed tomography, the thyroid was revealed to be enlarged with reduced tissue density, particularly in the left lobe and the isthmus. In addition, calcified spots and swollen lymph nodes were evident. The clinical history of the patient was obtained and the imaging results were retrospectively analyzed. The imaging features of PTL were investigated through reviewing the literature. PTL exhibits specific features on medical imaging that aid in distinguishing it from other thyroid diseases. PTL exhibits specific features on medical imaging that aid in distinguishing PTL from other thyroid diseases, which may aid the support for clinical diagnosis and improve the clinical accuracy.

  15. PRIMARY CHOROIDAL MALIGNANT LYMPHOMA:REPORT OF A CASE AND REVIEW OF LITERATURE

    Directory of Open Access Journals (Sweden)

    F. Asadi Amoli

    2006-06-01

    Full Text Available Non-Hodgkin lymphoma (NHL is one of the masquerade syndromes of malignant melanoma that can occur with two main patterns of presentations in the eye: metastatic involvement of uveal tract, and primary involvement of retina. We report ophthalmic, imaging and histopathological findings in the first case diagnosed as primary choroidal NHL without central nervous system or systemic involvement. A 37-year-old woman presented with the complaint of severe visual loss in her right eye. Significant ocular finding included light perception of vision (LP, 2+ APD, 2+ cells in vitreous and intraocular pressure of 46 mmHg. Fundoscopic examination revealed exudative retinal detachment. Ocular echography showed choroidal thickening in addition to retinal detachment. MRI showed semilunar shape lesion in the posterior right globe suggesting choroidal melanoma. Systemic work-up could not reveal any underlying cause. The patient underwent enucleation with clinical suggestion of choroidal melanoma. Result of histological examination showed NHL (diffuse large Bcell type of choroid. Immunohistochemical staining showed negative staining for HMB-45 and CD3, positive staining for LCA, and CD20. Multiple periodic lumbar puncture, bone marrow biopsies and MRI were unremarkable. No recurrence of tumor in systemic work-up was noted during the 36-months follow-up. Primary choroidal NHL is one of the causes of generalized thickening of choroid and should be considered in differential diagnosis of malignant melanoma. It is recommended to perform fine needle biopsy before performing surgery in any patient who has had an atypical malignant melanoma. This is, so far as we know, the first case diagnosed as primary choroidal NHL.

  16. [Clinicopathologic features of primary hepatic marginal zone lymphoma of mucosa-associated lymphoid tissue and hepatic pseudolymphoma].

    Science.gov (United States)

    Liu, C; Li, X; Li, H; Gong, Q X; Li, Y; Wang, Z; Zhang, Z H

    2018-01-08

    Objective: To study the clinicopathological features of primary hepatic extranodal marginal zone lymphoma of mucosa associated lymphoid tissue (MALT lymphoma) and hepatic pseudolymphoma, and to discuss their differential diagnosis, treatment and prognosis. Methods: Three primary hepatic MALT lymphomas and two hepatic pseudolymphomas collected from January 2012 to March 2017 in the First Affiliated Hospital of Nanjing Medical University were evaluated by HE and immunohistochemistry(IHC), in-situ hybridization and immunoglobulin (Ig) gene rearrangement detection, and the relevant literature reviewed. Results: In the three MALT lymphomas, tumor cells infiltrated the portal areas with nodular pattern, and invaded the surrounding normal liver with serpiginous configuration and formation of confluent sheets. A number of bile ducts were entrapped within the lesions, and showed lymphoepithelial lesion. Reactive lymphoid follicles were present and surrounded by tumor cells, consisting of predominantly centrocyte-like cells and monocytoid B cells. There were clusters of epithelioid histiocytes in one case. The tumor cells were positive for CD20, PAX5 and negative for CD5, CD23, CD10, bcl-6, and cyclin D1. In the two hepatic pseudolymphomas, the lesions presented as solitary nodules well-demarcated from the surrounding liver tissue; one case was partially encapsulated with fibrous tissue. Entrapped bile ducts were only found at the edge of the lesions without lymphoepithelial lesion. The lesions comprised of massive lymphoid proliferation consisting predominantly of reactive lymphoid follicles, but not monocytoid B-cells or atypical cells. By IHC, a mixture of B- and T-cell population was identified. A monoclonal rearrangement of the Ig gene was detected in all three MALT lymphomas but not in two pseudolymphomas. Interphase fluorescence in situ hybridiazation test for MALT1 break-apart gene was positive in two cases of MALT lymphomas and EBER was negative in all studied cases

  17. Clinical outcome and prognostic factors of primary gastric mucosa-associated lymphoid tissue lymphoma: a retrospective analysis of 77 cases

    International Nuclear Information System (INIS)

    Wang Shulian; Song Yongwen; Jin Jing; Wang Weihu; Liu Yueping; Liu Xinfan; Yu Zihao; Li Yexiong; Xue Liyan; Lv Ning

    2009-01-01

    Objective: To analyze the clinical results and prognostic factors of patients with early-stage primary gastric mucosa-associated lymphoid tissue (MALT) lymphoma. Methods: Seventy-seven patients with primary gastric MALT lymphoma treated from 1985 to 2006 were retrospectively analyzed. All patients were pathologically confirmed as MALT lymphoma in stage I, II and II E (by modified Blackedge staging system). Thirty-seven patients had stage I disease, 23 stage II and 17 stage II E . Sixty patients underwent surgical resection and 17 received non-surgical treatment. Survival rates were calculated by the Kaplan-Meier analysis with the Logrank test. Results: With a median follow up of 57 months for the surviving patients (ranging from 1 to 198 months for all patients), the 5-year overall survival rate, disease-free survival rate, loco-regional control rate and distant metastasis free survival rate were 74%, 70%, 76% and 87%, respectively. In univariate analysis, clinical stage was significantly associated with overall survival. Patients with stage I or II disease had a better overall survival than those with stage II E (P=0.01). Tumor size and surgical resection were significantly associated with disease-free survival. Patients with primary tumor 8 cm or less in diameter had better disease-free survival than those with primary tumor more than 8 cm in diameter (P =0.03). Patients who underwent complete resection had better disease-free survival than those who underwent incomplete resection or no surgery (P=0.02). Clinical stage, tumor size and surgical resection were significantly associated with loco-regional control. Patients with stage I or II disease had better loco-regional control than those with stage II E (P=0.03). Patients with primary tumor 8 cm or less in diameter had better loco-regional control than those with primary tumor more than 8 cm in diameter (P=0.01). Patients who underwent complete resection had better loco-regional control than those who underwent

  18. Primary B cell Lymphoma of the tongue: a case report | Hmidi | Pan ...

    African Journals Online (AJOL)

    Malignant lymphoma of the oral cavity is rare and of the tongue even rarer. Location of oral lymphomas is more frequent in masticatory mucosa than in movable mucosa; the lingual and buccal mucosa is rarely involved; whereas the gingival vestibule and Waldeyer's ring seem to be the most frequent site of occurrence.

  19. Primary Hepatic Non-Hodgkin’s Lymphoma: An Enigma Beyond the Liver, a Case Report

    Science.gov (United States)

    Laroia, Shalini Thapar; Rastogi, Archana; Panda, Dipanjan; Sarin, Shiv Kumar

    2015-01-01

    We have discussed a unique presentation of primary diffuse large cell B-cell non-Hodgkin (DLBC NHL) hepatic lymphoma involving the porta hepatis and biliary confluence causing obstructive jaundice with contiguous soft tissue involvement of the right lobe of liver extending up to the right renal cortex. This appears to be the only case in literature where primary hepatic lymphoma has shown contiguous localized intra- and extrahepatic tumor infiltration. A 67-year-old gentleman presented with history of significant loss of appetite and weight in 2 months with associated progressive painless cholestatic jaundice. Physical evaluation revealed normal vitals with pallor, deep icterus, scratch marks over the abdomen, generalized muscle wasting, grade II clubbing and a palpable non-tender liver with a globular, firm mass beneath the liver. He had a total serum bilirubin of 15.9 mg/dL and direct bilirubin of 9.24 mg/dL. His liver enzymes were moderately elevated with raised serum creatinine and dyselectrolytemia. Serology for enterohepatic viruses was negative. Contrast-enhanced magnetic resonance imaging (CEMRI) showed poorly enhancing multiple soft tissue masses in both lobes of liver with the largest mass involving, biliary confluence and porta hepatis causing right bile duct and portal vein encasement. The mass occupied the posterior right lobe and extended to the inferior surface of liver with contiguous invasion of the right renal upper pole cortex. The mass was associated with a retracted liver capsule in the involved segments and delayed enhancement, mimicking a cholangiocarcinoma. Tissue biopsy revealed hepatic DLBC type NHL and patient was subsequently treated with a CHOP-R (cyclophosphamide-doxorubicin-vincristine-prednisolone/rituximab) regimen, on which he has shown non-progressive disease at 1-year follow-up. DLBC NHL of the liver is a very rare tumor with propensity for isolated involvement of the liver and minimal extrahepatic spread. This case shows many

  20. Primary Hepatic Non-Hodgkin's Lymphoma: An Enigma Beyond the Liver, a Case Report.

    Science.gov (United States)

    Laroia, Shalini Thapar; Rastogi, Archana; Panda, Dipanjan; Sarin, Shiv Kumar

    2015-04-01

    We have discussed a unique presentation of primary diffuse large cell B-cell non-Hodgkin (DLBC NHL) hepatic lymphoma involving the porta hepatis and biliary confluence causing obstructive jaundice with contiguous soft tissue involvement of the right lobe of liver extending up to the right renal cortex. This appears to be the only case in literature where primary hepatic lymphoma has shown contiguous localized intra- and extrahepatic tumor infiltration. A 67-year-old gentleman presented with history of significant loss of appetite and weight in 2 months with associated progressive painless cholestatic jaundice. Physical evaluation revealed normal vitals with pallor, deep icterus, scratch marks over the abdomen, generalized muscle wasting, grade II clubbing and a palpable non-tender liver with a globular, firm mass beneath the liver. He had a total serum bilirubin of 15.9 mg/dL and direct bilirubin of 9.24 mg/dL. His liver enzymes were moderately elevated with raised serum creatinine and dyselectrolytemia. Serology for enterohepatic viruses was negative. Contrast-enhanced magnetic resonance imaging (CEMRI) showed poorly enhancing multiple soft tissue masses in both lobes of liver with the largest mass involving, biliary confluence and porta hepatis causing right bile duct and portal vein encasement. The mass occupied the posterior right lobe and extended to the inferior surface of liver with contiguous invasion of the right renal upper pole cortex. The mass was associated with a retracted liver capsule in the involved segments and delayed enhancement, mimicking a cholangiocarcinoma. Tissue biopsy revealed hepatic DLBC type NHL and patient was subsequently treated with a CHOP-R (cyclophosphamide-doxorubicin-vincristine-prednisolone/rituximab) regimen, on which he has shown non-progressive disease at 1-year follow-up. DLBC NHL of the liver is a very rare tumor with propensity for isolated involvement of the liver and minimal extrahepatic spread. This case shows many

  1. Hippuristanol Reduces the Viability of Primary Effusion Lymphoma Cells both in Vitro and in Vivo

    Directory of Open Access Journals (Sweden)

    Masachika Senba

    2013-09-01

    Full Text Available Primary effusion lymphoma (PEL caused by Kaposi’s sarcoma-associated herpesvirus (also known as human herpesvirus-8 shows serious lymphomatous effusion in body cavities. PEL is difficult to treat and there is no standard treatment strategy. Hippuristanol is extracted from Okinawan coral Isis hippuris, and inhibits translational initiation by blocking eukaryotic initiation factor 4A, an ATP-dependent RNA helicase, binding to mRNA. Recently, there has been much interest in targeting translation initiation as an anticancer therapy. Here, we show that treatment of PEL cell lines with hippuristanol resulted in cell cycle arrest at G1 phase, and induced caspases activation and apoptosis. Hippuristanol also reduced the expression of cyclin D2, CDK2, CDK4, CDK6 and prosurvival XIAP and Mcl-1 proteins. Activation of activator protein-1, signal transducers and activators of transcription protein 3 and Akt pathways plays a critical role in the survival and growth of PEL cells. Hippuristanol suppressed the activities of these three pathways by inhibiting the expression of JunB, JunD, c-Fos, signal transducers and activators of transcription protein 3 and Akt proteins. In a xenograft mouse model that showed ascites and diffused organ invasion of PEL cells, treatment with hippuristanol significantly inhibited the growth and invasion of PEL cells compared with untreated mice. The results of the in vitro and in vivo experiments underline the potential usefulness of hippuristanol in the treatment of PEL.

  2. Programmed Cell Death Ligand 1 Expression in Primary Central Nervous System Lymphomas: A Clinicopathological Study.

    Science.gov (United States)

    Hayano, Azusa; Komohara, Yoshihiro; Takashima, Yasuo; Takeya, Hiroto; Homma, Jumpei; Fukai, Junya; Iwadate, Yasuo; Kajiwara, Koji; Ishizawa, Shin; Hondoh, Hiroaki; Yamanaka, Ryuya

    2017-10-01

    Programmed cell death ligand 1 (PD-L1)/programmed cell death 1 (PD-1) have been shown to predict response to PD-L1/PD-1-targeted therapy. We analyzed PD-L1 expression in primary central nervous system lymphomas (PCNSLs). PD-L1 protein and mRNA expression were evaluated in 64 PCNSL tissue samples. IFN-γ, IL-10, CD4, and CD8 mRNA expression was also evaluated. PD-L1 protein was detected in tumor cells in 2 (4.1%) cases and in tumor microenvironments in 25 (52%) cases. PD-L1 mRNA positively correlated with IFN-γ (p=0.0024) and CD4 (p=0.0005) mRNA expression. IFN-γ mRNA positively correlated with CD8 mRNA expression (p=0.0001). Furthermore, tumor cell PD-L1 expression correlated positively with overall survival (p=0.0177), whereas microenvironmental PD-L1 expression exhibited an insignificant negative trend with overall survival (p=0.188). PD-L1 was expressed on both tumor and/or tumor-infiltrating immune cells in PCNSL. The biological roles of this marker warrant further investigation. Copyright© 2017, International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.

  3. Magnetic resonance imaging findings in primary lymphoma of the liver: a case report

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    Bilaj Fatmir

    2012-09-01

    Full Text Available Abstract Introduction Primary lymphoma of the liver is an extremely rare finding, with the few such cases reported in the literature to date describing indeterminate imaging findings, being focused more on computed tomography. To the best of our knowledge, there is no prior report describing magnetic resonance imaging scan findings with such a lesion. In the case reported here, magnetic resonance imaging gave us the opportunity to ascertain the correct diagnosis, confirmed by histopathology, thus avoiding unnecessary surgery or other treatments. Although this condition is rare, knowledge of magnetic resonance imaging findings will be invaluable for radiologists and other medical subspecialties that may face such cases in the future in helping to provide adequate management for affected patients. Case presentation A focal lesion was incidentally detected by ultrasound in a 75-year-old asymptomatic Albanian man being treated for benign hypertrophy of prostate. Chest and abdomen computed tomography scans did not reveal any abnormal findings besides a solid focal lesion on the right lobe of the liver and a mild homogenous enlargement of the prostate gland. Subsequently, magnetic resonance imaging of the upper abdomen was performed for better characterization of this lesion. Our patient was free of symptoms and his laboratory test results were normal. Conclusions The magnetic resonance imaging scan results showed some distinctive features that helped us to make the correct diagnosis, and were thus very important in helping us provide the correct treatment for our patient.

  4. Cooperative roles for emmprin and LYVE-1 in the regulation of chemoresistance for primary effusion lymphoma

    Science.gov (United States)

    Qin, Z; Dai, L; Bratoeva, M; Slomiany, MG; Toole, BP; Parsons, C

    2013-01-01

    The Kaposi’s sarcoma-associated herpesvirus is the causative agent of primary effusion lymphoma (PEL), for which cytotoxic chemotherapy represents the standard of care. The high mortality associated with PEL may be explained in part by resistance of these tumors to chemotherapy. The membrane-bound glycoprotein emmprin (CD147) enhances chemoresistance in tumors through effects on transporter expression, trafficking and interactions. Interactions between hyaluronan and hyaluronan receptors on the cell surface also facilitate emmprin-mediated chemoresistance. Whether emmprin or hyaluronan-receptor interactions regulate chemotherapeutic resistance for virus-associated malignancies is unknown. Using human PEL tumor cells, we found that PEL sensitivity to chemotherapy is directly proportional to expression of emmprin, the lymphatic vessel endothelial hyaluronan receptor-1 (LYVE-1) and a drug transporter known as the breast cancer resistance protein/ABCG2 (BCRP), and that emmprin, LYVE-1 and BCRP interact with each other and colocalize on the PEL cell surface. In addition, we found that emmprin induces chemoresistance in PEL cells through upregulation of BCRP expression, and RNA interference targeting of emmprin, LYVE-1 or BCRP enhances PEL cell apoptosis induced by chemotherapy. Finally, disruption of hyaluronan-receptor interactions using small hyaluronan oligosaccharides reduces expression of emmprin and BCRP while sensitizing PEL cells to chemotherapy. Collectively, these data support interdependent roles for emmprin, LYVE-1 and BCRP in chemotherapeutic resistance for PEL. PMID:21660043

  5. Scrotal Irradiation in Primary Testicular Lymphoma: Review of the Literature and In Silico Planning Comparative Study

    International Nuclear Information System (INIS)

    Brouwer, Charlotte L.; Wiesendanger, Esther M.; Hulst, Peter C. van der; Imhoff, Gustaaf W. van; Langendijk, Johannes A.; Beijert, Max

    2013-01-01

    We examined adjuvant irradiation of the scrotum in primary testicular lymphoma (PTL) by means of a literature review in MEDLINE, a telephone survey among Dutch institutes, and an in silico planning comparative study on scrotal irradiation in PTL. We did not find any uniform adjuvant irradiation technique assuring a safe planning target volume (PTV) coverage in published reports, and the definition of the clinical target volume is unclear. Histopathologic studies of PTL show a high invasion rate of the tunica albuginea, the epididymis, and the spermatic cord. In retrospective studies, a prescribed dose of at least 30 Gy involving the scrotum is associated with best survival. The majority of Dutch institutes irradiate the whole scrotum without using a planning computed tomography scan, with a single electron beam and a total dose of 30 Gy. The in silico planning comparative study showed that all evaluated approaches met a D 95% scrotal dose of at least 85% of the prescription dose, without exceeding the dose limits of critical organs. Photon irradiation with 2 oblique beams using wedges resulted in the best PTV coverage, with a mean value of 95% of the prescribed dose, with lowest maximum dose. Adjuvant photon or electron irradiation of the whole scrotum including the contralateral testicle with a minimum dose of 30 Gy is recommended in PTL. Computed tomography-based radiation therapy treatment planning with proper patient positioning and position verification guarantees optimal dose coverage.

  6. Comparison of primary thyroid lymphoma with anaplastic thyroid carcinoma on computed tomographic imaging

    International Nuclear Information System (INIS)

    Ishikawa, Hitoshi; Mitsuhashi, Norio; Niibe, Hideo

    2002-01-01

    Primary non-Hodgkin's lymphoma (LY) and anaplastic carcinoma (AC) of the thyroid gland are rare malignant tumors, and the initial symptoms of these diseases are very similar. The aim of our study was to compare the characteristics of the two diseases using computed tomographic (CT) scans in order to make an accurate differential diagnosis. Ten patients with LY and 10 with AC were analyzed. Differences in the CT findings of the two diseases were evaluated before treatment and statistically tested with either Student's t-test or the chi-square test. In the analysis of characteristics of CT imaging, the existence of calcification and necrosis, and heterogeneous tumor were dominant findings in AC, and there was a statistically significant difference in frequency between the two diseases (p<0.01). Calcification detected in AC was usually multiple and/or gross (mean size: φ8.2 mm). All lymphadenopathies were delineated as having the same homogeneous attenuation as the tumors in the thyroid gland in LY, but were shown as irregular rim enhancement in AC. The CT features of the two diseases are characteristic in terms of calcification, necrosis, and tumor composition. Evaluation by means of CT imaging is useful in distinguishing between LY and AC. (author)

  7. A Case of Mature Natural Killer-Cell Neoplasm Manifesting Multiple Choroidal Lesions: Primary Intraocular Natural Killer-Cell Lymphoma

    Directory of Open Access Journals (Sweden)

    Yoshiaki Tagawa

    2015-11-01

    Full Text Available Purpose: Natural killer (NK cell neoplasm is a rare disease that follows an acute course and has a poor prognosis. It usually emerges from the nose and appears in the ocular tissue as a metastasis. Herein, we describe a case of NK-cell neoplasm in which the eye was considered to be the primary organ. Case: A 50-year-old female displayed bilateral anterior chamber cells, vitreous opacity, bullous retinal detachment, and multiple white choroidal mass lesions. Although malignant lymphoma or metastatic tumor was suspected, various systemic examinations failed to detect any positive results. A vitrectomy was performed OS; however, histocytological analyses from the vitreous sample showed no definite evidence of malignancy, and IL-10 concentration was low. Enlarged choroidal masses were fused together. Three weeks after the first visit, the patient suddenly developed an attack of fever, night sweat, and hepatic dysfunction, and 5 days later, she passed away due to multiple organ failure. Immunohistochemisty and in situ hybridization revealed the presence of atypical cells positive for CD3, CD56, and Epstein-Barr virus-encoded RNAs, resulting in the diagnosis of NK-cell neoplasm. With the characteristic clinical course, we concluded that this neoplasm was a primary intraocular NK-cell lymphoma. Conclusions: This is the first report to describe primary intraocular NK-cell neoplasm. When we encounter atypical choroidal lesions, we should consider the possibility of NK-cell lymphoma, even though it is a rare disease.

  8. CD5-Positive Primary Intraocular B-Cell Lymphoma Arising during Methotrexate and Tumor Necrosis Factor Inhibitor Treatment

    Directory of Open Access Journals (Sweden)

    Kenji Nagata

    2015-09-01

    Full Text Available Purpose: To report a case of CD5+ primary intraocular B-cell lymphoma arising during methotrexate (MTX and tumor necrosis factor (TNF inhibitor treatment in a young patient with rheumatoid arthritis and uveitis. Case Presentation: A 39-year-old woman treated with MTX and a TNF inhibitor for rheumatoid arthritis and uveitis had steroid-resistant vitreous opacity. A vitreous sample was obtained by using diagnostic vitrectomy and was categorized as class V based on cytologic examination. Flow cytometric analysis of the vitreous sample revealed that abnormal cells were CD5+, CD10-, CD19+, CD20+ and immunoglobulin light-chain kappa+, suggesting the diagnosis of CD5+ primary intraocular B-cell lymphoma. Polymerase chain reaction (PCR detected immunoglobulin heavy-chain gene rearrangement. Epstein-Barr virus (EBV DNA was detected in the vitreous sample by using PCR, and immunohistochemistry revealed EBV latent membrane protein-1 expression in the abnormal cells infiltrating the vitreous. Optic nerve invasion was observed on magnetic resonance imaging. Conclusion: Primary intraocular lymphoma (PIOL may develop in patients receiving MTX and TNF inhibitor treatment. EBV infection may play an important role in the pathogenesis of PIOL arising during immunosuppressive therapy.

  9. Linfoma primário de cólon: relato de caso Primary colorectal lymphoma: case report

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    Rafael Luís Luporini

    2010-09-01

    Full Text Available O linfoma colorretal primário é uma doença rara (0.2 a 0.6% de todas as neoplasias colônicas, apresentando pior prognóstico quando comparado com o linfoma gástrico primário ou com o adenocarcinoma do cólon. É uma doença com sintomatologia inespecífica, o que dificulta o diagnóstico precoce. O objetivo deste relato é mostrar um caso de linfoma primário do cólon, revisar critérios diagnósticos e tratamento.The primary colorectal lymphoma is a rare disease (0.2 to 0.6% of all colonic neoplasias, that has a worse prognosis than primary gastric lymphoma or colon adenocarcinoma. The poor signals makes the early diagnosis difficult. The objectives of this report is to describe a case of primary colon lymphoma, revise diagnosis criteria and treatment.

  10. Mucosa-associated lymphoid tissue (MALT) variant of primary rectal lymphoma: a review of the English literature.

    Science.gov (United States)

    Kelley, Scott R

    2017-03-01

    Primary rectal lymphoma (PRL) is the third most common cause of rectal cancer following adenocarcinoma (90-95 %) and carcinoid (5 %). The most common variant of PRL is the mucosa-associated lymphoid tissue (MALT) type. To date, no study has been able to recommend an optimal treatment algorithm for this rare disease. The aim of our study was to review the English literature on primary rectal MALT lymphoma. A review of the English literature was conducted to identify articles describing the MALT variant of PRL. Fifty-one cases were identified. A complete response was achieved in 12 of 19 cases treated with Helicobacter pylori eradication therapy, 5 of 6 with radiation, 2 of 4 cases with chemotherapy, 2 of 4 with endoscopic resection, 6 of 8 cases with surgical resection, and all 8 with combination therapies. Cases failing initial therapies were responsive to various second-line treatments. Two cases spontaneously regressed with observation alone. Complete regression of primary rectal MALT lymphoma was achieved using various therapeutic strategies, although the numbers of different treatment modalities are too small to draw definitive conclusions.

  11. Utility of F-18 FDG PET/CT on the evaluation of primary bone lymphoma.

    Science.gov (United States)

    Wang, Li-Juan; Wu, Hu-Bing; Wang, Meng; Han, Yan-Jiang; Li, Hong-Sheng; Zhou, Wen-Lan; Wang, Quan-Shi

    2015-11-01

    Primary bone lymphoma (PBL) is a rare type of malignant lymphoma. Few data have been reported regarding the utility of F-18 FDG PET/CT in this disease. The aim of this study was to assess the role of F-18 FDG PET/CT in the diagnosis and therapeutic effect evaluation of PBL. A total of 19 consecutive patients with PBL were enrolled. Whole-body PET/CT scan was performed for all patients. The diagnosis of PBL was established by histopathology and immunohistochemistry. F-18 FDG PET/CT was positive in 94.7% (18/19) of patients. Uptake of FDG in lesions was intense with SUVmax of 15.14 ± 11.82. Multiple involved lesions were found in 47.4% (9/19) patients, while 52.6% presented with a single involved lesion. Based on the lesions, PET detected 98.9% (87/88) lesions. Among them, 71.6% (63/88) lesions were found to be located in axial skeleton and 28.4% (25/88) in the extremity skeleton. FDG PET/CT also found the lesions infiltrate to the surrounding soft tissue in 84.2% (16/19) patients. On the syn-modality CT, the bone destruction was noted in 43.2% (38/88) of the lesions, of which 50.0% lesions presented as slight change in bone density and 50.0% as severe change. The diagnostic sensitivity of PET was much higher than that of CT (98.9% vs. 43.2%, P=0.000). PET/CT was performed for evaluation of treatment response in 13 patients. In 12 patients with complete response(CR), PET/CT found the 25 lesions were F-18 FDG fully resoluted after treatment, however, bone destruction was still presented in 72.0% (18/25) lesions. The present study suggests that F-18 FDG PET/CT was a sensitive imaging modality for diagnosis and treatment response evaluation of PBL. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

  12. FDG avidity and PET/CT patterns in primary gastric lymphoma

    International Nuclear Information System (INIS)

    Radan, Lea; Fischer, Doron; Bar-Shalom, Rachel; Israel, Ora; Dann, Eldad J.; Epelbaum, Ron; Haim, Nissim; Gaitini, Diana

    2008-01-01

    The use of 18F-fluoro-deoxyglucose positron emission tomography/computed tomography (FDG-PET/CT) in primary gastric lymphoma (PGL) is challenging due to physiologic FDG activity in the stomach and variability in the degree of uptake in various histologic subtypes. This study assesses FDG avidity and PET/CT patterns in newly diagnosed PGL. Sixty-two PET/CT studies of newly diagnosed PGL were reviewed (24 low-grade mucosa-associated lymphoid tissue [MALT], 38 aggressive non-Hodgkin's lymphoma [AGNHL]). FDG avidity, patterns (focal/diffuse), and intensity (visually vs. the liver and SUVmax) were assessed and compared to 27 controls. Gastric CT abnormalities and extragastric sites were recorded. Gastric FDG uptake was found in 55/62 (89%) PGL (71% MALT vs. 100% AGNHL, p < 0.001) and 63% controls. A diffuse pattern was found in 60% PGL (76% MALT vs. 53% AGNHL, p = NS) and 47% controls. FDG uptake higher than liver was found in 82% PGL (58% MALT vs. 97% AGNHL, p < 0.05) and 63% controls. SUVmax in FDG-avid PGLs was 15.3 ± 11.7 (5.4 ± 2.9 MALT vs. 19.7 ± 11.5 AGNHL, p < 0.001) and 4.6 ± 1.4 in controls. CT abnormalities were found in 79% PGL (thickening, n = 49; ulcerations, n = 22). Extra-gastric FDG-avid sites were seen in none of MALT, but 61% of AGNHL (nodal, n = 18; nodal and extranodal, n 5). FDG avidity was present in 89% of PGLs, including all patients with AGNHL but only 71% of MALT. FDG uptake can be differentiated, in particular in AGNHL-PGL, from physiologic tracer activity by intensity but not by pattern. Extragastric foci on PET and structural CT abnormalities are additional parameters that can improve PET/CT assessment of PGL. Defining FDG avidity and PET/CT patterns in AGNHL and a subgroup of MALT-PGL before treatment may be important for further monitoring therapy response. (orig.)

  13. FDG avidity and PET/CT patterns in primary gastric lymphoma

    Energy Technology Data Exchange (ETDEWEB)

    Radan, Lea [Rambam Health Care Campus, Department of Nuclear Medicine, Haifa (Israel); Fischer, Doron [Rambam Health Care Campus, Department of Diagnostic Imaging, Haifa (Israel); Bar-Shalom, Rachel; Israel, Ora [Technion - Israel Institute of Technology, Department of Nuclear Medicine, Rambam Health Care Campus and R. and B. Rappaport School of Medicine, Haifa (Israel); Dann, Eldad J. [Technion - Israel Institute of Technology, Department of Hematology, Rambam Health Care Campus, and R. and B. Rappaport School of Medicine, Haifa (Israel); Epelbaum, Ron; Haim, Nissim [Technion - Israel Institute of Technology, Department of Oncology, Rambam Health Care Campus, and R. and B. Rappaport School of Medicine, Haifa (Israel); Gaitini, Diana [Rambam Health Care Campus, and R. and B. Rappaport School of Medicine, Technion - Israel Institute of Technology, Department of Diagnostic Imaging, Haifa (Israel)

    2008-08-15

    The use of 18F-fluoro-deoxyglucose positron emission tomography/computed tomography (FDG-PET/CT) in primary gastric lymphoma (PGL) is challenging due to physiologic FDG activity in the stomach and variability in the degree of uptake in various histologic subtypes. This study assesses FDG avidity and PET/CT patterns in newly diagnosed PGL. Sixty-two PET/CT studies of newly diagnosed PGL were reviewed (24 low-grade mucosa-associated lymphoid tissue [MALT], 38 aggressive non-Hodgkin's lymphoma [AGNHL]). FDG avidity, patterns (focal/diffuse), and intensity (visually vs. the liver and SUVmax) were assessed and compared to 27 controls. Gastric CT abnormalities and extragastric sites were recorded. Gastric FDG uptake was found in 55/62 (89%) PGL (71% MALT vs. 100% AGNHL, p < 0.001) and 63% controls. A diffuse pattern was found in 60% PGL (76% MALT vs. 53% AGNHL, p = NS) and 47% controls. FDG uptake higher than liver was found in 82% PGL (58% MALT vs. 97% AGNHL, p < 0.05) and 63% controls. SUVmax in FDG-avid PGLs was 15.3 {+-} 11.7 (5.4 {+-} 2.9 MALT vs. 19.7 {+-} 11.5 AGNHL, p < 0.001) and 4.6 {+-} 1.4 in controls. CT abnormalities were found in 79% PGL (thickening, n = 49; ulcerations, n = 22). Extra-gastric FDG-avid sites were seen in none of MALT, but 61% of AGNHL (nodal, n = 18; nodal and extranodal, n = 5). FDG avidity was present in 89% of PGLs, including all patients with AGNHL but only 71% of MALT. FDG uptake can be differentiated, in particular in AGNHL-PGL, from physiologic tracer activity by intensity but not by pattern. Extragastric foci on PET and structural CT abnormalities are additional parameters that can improve PET/CT assessment of PGL. Defining FDG avidity and PET/CT patterns in AGNHL and a subgroup of MALT-PGL before treatment may be important for further monitoring therapy response. (orig.)

  14. Primary epidural lymphoma without vertebral involvement in a HIV-positive patient

    International Nuclear Information System (INIS)

    Perez, M. O.; Grive, E.; Quiroga, S.; Rovira, A.

    1999-01-01

    Epidural involvement is rarely associated with lymphoma, it being more typical of non-Hodgkin's lymphoma in advanced stages of the disease. The invasion of the epidural space is usually caused by the extension of a paravertebral mass or by the affected vertebrae. However, the epidural space alone can be involved. We present a case of epidural lymphoma in a patient who presented with clinical evidence of spinal cord compression. Magnetic resonance disclosed the existence of an epidural mass compressing and displacing the spinal cord without involving the adjacent vertebra or the associated paravertebral mass. (Author) 9 refs

  15. Subsequent primary malignancies after diffuse large B-cell lymphoma in the modern treatment era.

    Science.gov (United States)

    Tao, Li; Clarke, Christina A; Rosenberg, Aaron S; Advani, Ranjana H; Jonas, Brian A; Flowers, Christopher R; Keegan, Theresa H M

    2017-07-01

    With the addition of rituximab and other treatment advances, survival after diffuse large B-cell lymphoma (DLBCL) has improved, but subsequent primary malignancies (SPMs) have emerged as an important challenge for DLBCL survivorship. We calculated standardized incidence ratios (SIRs) and 95% confidence intervals (CIs) for SPMs among 23 879 patients who survived at least 1 year after a first primary DLBCL diagnosed during 1989-2012, compared to the general population in California. Cumulative incidence (CMI) of SPMs, accounting for the competing risk of death, also was calculated. We found that the incidence of acute myeloid leukaemia (AML) nearly doubled in the post-rituximab era [SIR (95% CI) 4·39 (2·51-7·13) pre- (1989-2000) and 8·70 (6·62-11·22) post-rituximab (2001-2012)]. Subsequent thyroid cancer was rare pre-rituximab, but increased substantially after 2001 [0·66 (0·08-2·37) vs. 2·27(1·44-3·41)]. The 5-year CMI for all SPMs (4·77% pre- vs. 5·41% post-rituximab, P = 0·047), AML (0·15% vs. 0·41%, P = 0·003), thyroid cancer (0·03% vs. 0·15%, P = 0·003) and melanoma (0·25% vs. 0·42%, P = 0·020) were greater in DLBCL patients diagnosed in the post- versus pre-rituximab period. This study provides insight into the changing pattern of SPM occurrence after the introduction of rituximab, which may elucidate the aetiology of SPMs and should guide future cancer surveillance efforts among DLBCL patients. © 2017 John Wiley & Sons Ltd.

  16. Primary cutaneous amyloidosis involving the external ears along with the classical sites

    Directory of Open Access Journals (Sweden)

    Khaitan Binod

    2001-01-01

    Full Text Available A 26- year- old woman had multiple itchy persistent gradually progressive papular lesions on the forearms and shins for 10 and 4 years respectively. She also noticed similar lesions on both the ears for 4 years. There were no systemic symptoms. Cutaneous examination revealed multiple 2-3 mm discrete firm hyperpigmented papules on the extensors of forearms, shins and earlobes. Skin biopsy from all sites demonstrated deposits of amyloid in the papillary dermis. The patient was treated with cyclophosphamide 50 mg daily orally. There was more than 50% improvement in her lesions.

  17. [Primary presentation of non-hodgkin lymphoma. Report of a case].

    Science.gov (United States)

    Mirpuri-Mirpuri, P G; Alvarez-Cordovés, M M; Pérez-Monje, A

    2013-09-01

    Lymphomas are the most common non-epithelial tumors of the head and neck and its incidence has increased in recent decades. Around 10% are extranodal lymphomas, and in more than half of the cases are located in Waldeyer's lymphatic ring. The most common presenting symptoms are odynophagia and dysphagia (68%), and symptoms suggestive of oropharyngeal cancer such as cough, hoarseness, earache, feeling of occupation in the back of the mouth, throat or neck. In non-Hodgkin lymphomas in this location, B symptoms (weight loss, fever and sweating) are rare (5%). The histological subtype of each individual lymphoma affects the evaluation, therapy and prognosis. Copyright © 2012 Sociedad Española de Médicos de Atención Primaria (SEMERGEN). Publicado por Elsevier España. All rights reserved.

  18. Treatment Options for Primary Refractory/Recurrent Hodgkin Lymphoma in Children and Adolescents

    Science.gov (United States)

    ... Reporting & Auditing Grant Transfer Grant Closeout Contracts & Small Business Training Cancer Training at NCI (Intramural) Resources for ... memory. Second cancers (new types of cancer). For female survivors of Hodgkin lymphoma, there is an increased ...

  19. Lymphoma of the Eyelid

    DEFF Research Database (Denmark)

    Svendsen, Frederik Holm; Rasmussen, Peter Kristian; Coupland, Sarah E.

    2017-01-01

    Purpose To document subtype-specific clinical features of lymphoma of the eyelid, and their effect on patient outcome. Design Retrospective observational case series. Methods Patient data were collected from 7 international eye cancer centers from January 1, 1980 through December 31, 2015....... The cases included primary and secondary lymphomas affecting the eyelid. Overall survival, disease-specific survival (DSS), and progression-free survival were the primary endpoints. Results Eighty-six patients were included. Mean age was 63 years and 47 (55%) were male. Non-Hodgkin B-cell lymphomas...... constituted 83% (n = 71) and T-cell lymphomas constituted 17% (n = 15). The most common subtypes were extranodal marginal-zone lymphoma (EMZL) (37% [n = 32]), follicular lymphoma (FL) (23% [n = 20]), diffuse large B-cell lymphoma (DLBCL) (10% [n = 9]), mantle cell lymphoma (MCL) (8% [n = 7]), and mycosis...

  20. Primary Breast Burkitt’s Lymphoma in an HIV-Infected Woman

    Directory of Open Access Journals (Sweden)

    Bangaly Traoré

    2015-01-01

    Full Text Available A 30-year-old HIV positive woman presented with a multifocal mass tumour associated with axillary and lateral-cervical lymphadenopathy in the right breast. Laboratory examination of the biopsy confirmed a case of mammary Burkitt’s lymphoma with a nodular infiltration of the breast. Antiretroviral treatment and chemotherapy were effective to control the tumour. Although Burkitt’s lymphoma rarely involves the breasts, it should be considered during routine breast examination of African woman.

  1. Adult T-cell leukemia/lymphoma treatment in Bahia, Brazil

    Directory of Open Access Journals (Sweden)

    Pedro Dantas Oliveira

    Full Text Available Abstract Background: Adult T-cell leukemia/lymphoma is a peripheral disease associated with human T-cell lymphotropic virus type 1. Treatment is carried out according to clinical type with watchful waiting being recommended for less aggressive types. Aggressive adult T-cell leukemia/lymphoma is generally treated with chemotherapy and/or antivirals. The objective of this study was to correlate the survival of patients diagnosed in Bahia, Brazil, with the therapeutic approaches employed and to evaluate what issues existed in their treatment processes. Methods: Eighty-three adult T-cell leukemia/lymphoma patients (26 smoldering, 23 chronic, 16 acute, 13 lymphoma and five primary cutaneous tumoral with available data were included in this study. Results: Complete response was achieved in seven smoldering patients with symptomatic treatment, in two with chronic disease using antivirals/chemotherapy, in one with acute disease using antivirals and in one lymphoma using the LSG15 regimen [vincristine, cyclophosphamide, doxorubicin, and prednisolone (VCAP; doxorubicin, ranimustine, and prednisolone (AMP; and vindesine, etoposide, carboplatin, and prednisolone (VECP]. Smoldering patients who received symptomatic treatment presented longer survival. Favorable chronic patients treated with antivirals presented longer survival compared to the unfavorable subtype. However, for the acute form, first-line chemotherapy was better, albeit without significance, than antivirals. Only one of the patients with lymphoma and primary cutaneous tumors responded. Conclusions: Watchful waiting associated with phototherapy represents the best option for smoldering adult T-cell leukemia/lymphoma with survival in Bahia being superior to that described in Japan. There was a trend of better results with zidovudine/interferon-alpha in favorable chronic disease. Excellent results were achieved in the lymphoma type treated with the LSG15 protocol. Patients are diagnosed late

  2. Primary Refractory and Relapsed Classical Hodgkin Lymphoma - Significance of Differential CD15 Expression in Hodgkin-Reed-Sternberg Cells

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    Daniel Benharroch, Shai Pilosof, Jacob Gopas, Itai Levi

    2012-01-01

    Full Text Available We recognized a few possible complications of classical Hodgkin lymphoma therapy in a cohort of 209 patients: 8 developed a primary refractory disease (primary progression, 36 showed an early relapse and 21 showed a late relapse. Sialyl-CD15 expression in Hodgkin-Reed-Sternberg cells was significantly more positive in primary refractory Hodgkin lymphoma, which confirms our previously published findings. Bcl-2 showed a significantly lower level of expression in primary refractory disease than in the other follow-up groups. This is in contrast with a previous finding of Bcl-2, associated with a poor prognosis in primary refractory illness. Another category of variables, old age and advanced stages, was significantly different in the various complications but this finding is probably to be expected. We could not demonstrate a difference between the sequels and the control group with regard to several clinical and immunohistochemical markers. Sialyl-CD15 and Bcl-2 expression, in contrast, were confirmed as prognostic factors, mainly of tumor progression into primary refractory disease.

  3. ESHAP chemotherapy is efficient in refractory/relapsed primary central nervous system lymphoma: report of four cases

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    Ungur R

    2015-10-01

    Full Text Available Rodica Ungur,1,2 Adrian Tempescul,3 Christian Berthou,3 Cristina Bagacean,1,2 Doinel Radeanu,1 Adriana Muresan,1 Mihnea Zdrenghea1,21Department of Hematology, Iuliu Hatieganu University of Medicine and Pharmacy Cluj, 2Department of Hematology, Ion Chiricuta Oncology Institute, Cluj-Napoca, Romania; 3Department of Clinical Hematology, Institute of Cancerology and Hematology, Brest Teaching Hospital, Brest, FranceAbstract: Primary central nervous system non-Hodgkin’s lymphoma is a rare presentation, almost always of diffuse large B-cell type. Although there is no consensus regarding therapy for this condition, induction regimens are based on high-dose methotrexate and consolidation whole-brain radiotherapy, or, more preferred recently, blood–brain barrier penetrating drugs such as etoposide, cytarabine, and alkylating agents like temozolomide, ifosfamide, and lomustine. We present here four cases of relapsed/refractory primary central nervous system lymphoma treated with ESHAP (etoposide, solumedrol, high-dose cytarabine, and platinum chemotherapy to complete remission, with the eligible patients proceeding to autologous transplantation. We want to draw attention to this interesting, relatively well tolerated, underused therapeutic option, in a setting where treatment options are scarce and evidence-based recommendations are lacking.Keywords: cerebral lymphoma, PCNSL, refractory, relapsed, platinum

  4. Primary plasmablastic lymphoma of the central nervous system in an immunocompetent man: A case report and review of literature

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    Rama Kumari Badyal

    2014-01-01

    Full Text Available Plasmablastic lymphoma (PBL is an aggressive non-Hodgkin lymphoma classically occurring in individuals infected with human immunodeficiency virus (HIV. It has a predilection for the oral cavity and jaw. However, recent case reports have shown this variety of lymphoma in the stomach, lung, nasal cavity, cervical lymph nodes and jejunum in HIV-negative individuals. In this manuscript we report a case of primary PBL of brain in an HIV-negative heterosexual man, who presented with multiple episodes of seizures and hemiparesis. It proved a diagnostic challenge as initial stereotactic brain biopsy showed only few necrotic fragments and possibility of glioblastoma multiforme was rendered. Later patient underwent craniotomy and subsequent histopathology combined with immunohistochemistry led us in making a correct diagnosis of extramedullary PBL. Extensive systemic work up failed to reveal any disease outside the central nervous system. Only single case of primary PBL of brain in HIV-negative individual has been reported until date. To the best of our knowledge, this is the second report to suggest such an association.

  5. Germline mutations in candidate predisposition genes in individuals with cutaneous melanoma and at least two independent additional primary cancers.

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    Pritchard, Antonia L; Johansson, Peter A; Nathan, Vaishnavi; Howlie, Madeleine; Symmons, Judith; Palmer, Jane M; Hayward, Nicholas K

    2018-01-01

    While a number of autosomal dominant and autosomal recessive cancer syndromes have an associated spectrum of cancers, the prevalence and variety of cancer predisposition mutations in patients with multiple primary cancers have not been extensively investigated. An understanding of the variants predisposing to more than one cancer type could improve patient care, including screening and genetic counselling, as well as advancing the understanding of tumour development. A cohort of 57 patients ascertained due to their cutaneous melanoma (CM) diagnosis and with a history of two or more additional non-cutaneous independent primary cancer types were recruited for this study. Patient blood samples were assessed by whole exome or whole genome sequencing. We focussed on variants in 525 pre-selected genes, including 65 autosomal dominant and 31 autosomal recessive cancer predisposition genes, 116 genes involved in the DNA repair pathway, and 313 commonly somatically mutated in cancer. The same genes were analysed in exome sequence data from 1358 control individuals collected as part of non-cancer studies (UK10K). The identified variants were classified for pathogenicity using online databases, literature and in silico prediction tools. No known pathogenic autosomal dominant or previously described compound heterozygous mutations in autosomal recessive genes were observed in the multiple cancer cohort. Variants typically found somatically in haematological malignancies (in JAK1, JAK2, SF3B1, SRSF2, TET2 and TYK2) were present in lymphocyte DNA of patients with multiple primary cancers, all of whom had a history of haematological malignancy and cutaneous melanoma, as well as colorectal cancer and/or prostate cancer. Other potentially pathogenic variants were discovered in BUB1B, POLE2, ROS1 and DNMT3A. Compared to controls, multiple cancer cases had significantly more likely damaging mutations (nonsense, frameshift ins/del) in tumour suppressor and tyrosine kinase genes and

  6. AIDS-related primary central nervous system lymphoma: a Norwegian national survey 1989–2003

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    Haldorsen, Ingfrid S; Kråkenes, Jostein; Goplen, Anne K; Dunlop, Oona; Mella, Olav; Espeland, Ansgar

    2008-01-01

    Primary central nervous system lymphoma (PCNSL) is a frequent complication in acquired immunodeficiency syndrome (AIDS). The objective of this survey was to investigate incidence, clinical features, radiological findings, histologic diagnosis, treatment and outcome for all patients with histologically verified AIDS-related PCNSL diagnosed in Norway in 1989–2003. We identified the patients by chart review of all cases recorded as PCNSL in The Norwegian Cancer Registry (by law recording all cases of cancer in Norway) and all cases recorded as AIDS-related PCNSL in the autopsy registry at a hospital having 67% autopsy rate and treating 59% of AIDS patients in Norway, from 1989 to 2003. Histologic material and radiological images were reviewed. We used person-time techniques to calculate incidence rates of PCNSL among AIDS patients based on recordings on AIDS at the Norwegian Surveillance System for Communicable Diseases (by law recording all cases of AIDS in Norway). Twenty-nine patients had histologically confirmed, newly diagnosed AIDS-related PCNSL in Norway from 1989–2003. Only 2 patients had this diagnosis established while alive. AIDS patients had 5.5% lifetime risk of PCNSL. Their absolute incidence rate of PCNSL per 100 person-years was 1.7 (95%CI: 1.1–2.4) and decreased during the consecutive 5-year periods from 3.6, to 2.5, and to 0.4 (p < 0.001). Median survival from initial symptom of PCNSL was 2.3 months, but one patient was still alive 4 years after completed radiotherapy. This is the first national survey to confirm decreasing incidence of AIDS-related PCNSL. Despite dismal survival in most patients, the possibility of long term survival should prompt more aggressive diagnostics in suspected PCNSL

  7. Differentially regulated miRNAs as prognostic biomarkers in the blood of primary CNS lymphoma patients.

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    Roth, Patrick; Keller, Andreas; Hoheisel, Jörg D; Codo, Paula; Bauer, Andrea S; Backes, Christina; Leidinger, Petra; Meese, Eckart; Thiel, Eckhard; Korfel, Agnieszka; Weller, Michael

    2015-02-01

    Despite improved therapeutic regimens, primary CNS lymphoma (PCNSL) remains a therapeutic challenge. A prognostic classification of PCNSL patients may represent an important step towards optimised patient-adapted therapy. However, only higher age and low Karnofsky Performance Status (KPS) have repeatedly been reported to be associated with shorter overall survival (OS). Here we characterised microRNA (miRNA) fingerprints in the blood of PCNSL patients with short-term survival (STS) versus long-term survival (LTS) to assess their potential as novel prognostic biomarkers. Blood was collected from patients enrolled in the G-PCNSL-SG1 trial, a phase III study for patients with newly diagnosed PCNSL. miRNAs were extracted from the blood and analysed by next generation sequencing. The STS group comprised 20 patients with a median OS of 3 months and was compared to 20 LTS patients with a median OS of 55 months. The cohorts were balanced for age and KPS. Twelve annotated miRNAs were significantly deregulated between the two groups. Among them, miR-151a-5p and miR-151b exhibited the most prominent differences. Importantly, the combination of several miRNA allowed for a good separation between short- and long-term survivors with maximal Area Under Curve (AUC) above 0.75. Besides the known miRNAs we identified putative novel miRNA candidates with potential regulatory influence of PCNSL. Finally, the differential regulation of the most promising candidate miRNAs was confirmed by real-time polymerase chain reaction (PCR) in a validation cohort consisting of 20 STS and LTS patients. In conclusion, peripheral blood miRNA expression patterns hold promise as a prognostic tool in PCNSL patients. Copyright © 2014 Elsevier Ltd. All rights reserved.

  8. Acquired immunodeficiency syndrome-related primary cerebral lymphoma: response to irradiation

    International Nuclear Information System (INIS)

    Khoo, V.S.; Wilson, P.C.; Sexton, M.J.; Liew, K.H.

    2000-01-01

    Acquired immunodeficiency syndrome-related primary cerebral lymphoma (AIDS-PCL) is uncommon. Fourteen cases of presumed AIDS-PCL between 1986 and 1995 were reviewed retrospectively in order to characterize the natural history, and the response to radiotherapy. The median age was 38 years (range 24-65). The median interval between seropositive diagnosis of HIV and AIDS-PCL was 28 months (range 5-113). The median duration of symptoms was 2 weeks (range 0.2-12). At presentation, the Eastern Cooperative Oncology Group performance status (PS) was PS1 (2/14 patients), PS2 (6/14) and PS3 (6/14). The symptoms and signs were non-specific and depended on the site and extent of cerebral involvement. There was no characteristic pattern of brain imaging in terms of size, number, location or pattern of contrast enhancement of the cerebral lesions. Nine patients received various fractionation-dose schedules (range 8-50 Gy). Complete and partial responses were seen in 2/9 and 3/9 cases, respectively. Clinical stabilization of neurological symptoms was noted in 3/9 cases and disease progression in 1/9. The median survival times (MST) from presentation for irradiated and non-irradiated patients were 9.3 and 2.1 weeks, respectively (range 0.9-43.1). Although patient selection introduced bias, there appears to be a modest improvement in MST for treated patients. The MST with radiotherapy alone remains poor, but radiotherapy may provide palliation. For some selected patients, a prolonged response is possible. Copyright (1999) Blackwell Science Pty Ltd

  9. Histogram analysis of apparent diffusion coefficient maps for differentiating primary CNS lymphomas from tumefactive demyelinating lesions.

    Science.gov (United States)

    Lu, Shan Shan; Kim, Sang Joon; Kim, Namkug; Kim, Ho Sung; Choi, Choong Gon; Lim, Young Min

    2015-04-01

    This study intended to investigate the usefulness of histogram analysis of apparent diffusion coefficient (ADC) maps for discriminating primary CNS lymphomas (PCNSLs), especially atypical PCNSLs, from tumefactive demyelinating lesions (TDLs). Forty-seven patients with PCNSLs and 18 with TDLs were enrolled in our study. Hyperintense lesions seen on T2-weighted images were defined as ROIs after ADC maps were registered to the corresponding T2-weighted image. ADC histograms were calculated from the ROIs containing the entire lesion on every section and on a voxel-by-voxel basis. The ADC histogram parameters were compared among all PCNSLs and TDLs as well as between the subgroup of atypical PCNSLs and TDLs. ROC curves were constructed to evaluate the diagnostic performance of the histogram parameters and to determine the optimum thresholds. The differences between the PCNSLs and TDLs were found in the minimum ADC values (ADCmin) and in the 5th and 10th percentiles (ADC5% and ADC10%) of the cumulative ADC histograms. However, no statistical significance was found in the mean ADC value or in the ADC value concerning the mode, kurtosis, and skewness. The ADCmin, ADC5%, and ADC10% were also lower in atypical PCNSLs than in TDLs. ADCmin was the best indicator for discriminating atypical PCNSLs from TDLs, with a threshold of 556×10(-6) mm2/s (sensitivity, 81.3 %; specificity, 88.9%). Histogram analysis of ADC maps may help to discriminate PCNSLs from TDLs and may be particularly useful in differentiating atypical PCNSLs from TDLs.

  10. MR imaging characteristics in primary lymphoma of bone with emphasis on non-aggressive appearance

    International Nuclear Information System (INIS)

    Heyning, Fenna H.; Kroon, Herman M.J.A.; Hogendoorn, Pancras C.W.; Taminiau, Antonie H.M.; Woude, Henk-Jan van der

    2007-01-01

    To assess the heterogeneity of magnetic resonance (MR) imaging characteristics in primary lymphoma of bone (PLB), in particular the non-aggressive appearance. In a retrospective study, MR imaging features were analyzed in 29 patients with histologically proven PLB. The following parameters were evaluated: tumor size, bone marrow and extension into soft tissues, signal characteristics of bone marrow and soft-tissue components, including enhancement, and involvement of cortical bone (complete disruption, focal destruction, permeative destruction and cortical thickening). PLB presented with extension into the soft tissue in 22 (76%) of 29 patients, was only subtle in three of these 22 patients, and was absent in seven patients. Signal intensity (SI) of the soft-tissue part was most frequently homogeneously isointense with muscle on T1-weighted images (90%) and high on T2-weighted images (91%). Enhancement was predominantly homogeneous and diffuse (82%). In 93% of patients cortical bone appeared abnormal: among those patients complete cortical disruption was seen in 28%, with extension into soft tissues in all but one patient; a permeative pattern of destruction was present in 52% of patients, 66% of these had an associated soft-tissue mass. Two patients with normal-appearing cortical bone had no extension into soft tissues. In two patients focal cortical destruction was noticed; in one patient cortical bone was homogeneously thickened, and in one patient PLB was selectively localized within the cortical bone. SI of the bone marrow tumor component was more frequently heterogeneous (in 54%), compared with the soft-tissue component, being high on T2-weighted images in 89%, intermediate in 7% and low in 4%. Similarly, enhancement was heterogeneous in 59%. The MR imaging appearance of PLB is variable. In 31% of PLB patients, the tumor was intra-osseous, with linear cortical signal abnormalities or even normal-appearing or thickened cortical bone without soft-tissue mass

  11. High soluble CD30, CD25 and IL-6 may identify patients with worse survival in CD30+ cutaneous lymphomas and early mycosis fungoides

    Science.gov (United States)

    Kadin, Marshall E.; Pavlov, Igor; Delgado, Julio C.; Vonderheid, Eric C.

    2011-01-01

    Histopathology alone cannot predict outcome of patients with CD30+ primary cutaneous lymphoproliferative disorders (CD30CLPD) and early mycosis fungoides (MF). To test the hypothesis that serum cytokines/cytokine receptors provide prognostic information in these disorders, we measured soluble CD30 (sCD30), sCD25, and selected cytokines in cell cultures and sera of 116 patients with CD30CLPD and 96 patients with early MF followed up to 20 years. Significant positive correlation was found between sCD30 levels and sCD25, CD40L, IL-6, and IL-8, suggesting CD30+ neoplastic cells secrete these cytokines, but not Th2 cytokines. In vitro studies confirmed sCD30, sCD25, IL-6 and IL-8 are secreted by CD30CLPD-derived cell lines. CD30CLPD patients with above normal sCD30 and sCD25 had worse overall and disease-related survivals, but only sCD30 retained significance in Cox models that included advanced age. High sCD30 also identified patients with worse survival in early MF. Increased IL-6 and IL-8 correlated with poor disease-related survival in CD30CLPD patients, We conclude that: (1) neoplastic cells of some CD30CLPD patients do not resemble Th2 cells, (2) high serum sCD30, sCD25, IL-6, and perhaps IL-8 levels may provide prognostic information useful for patient management. PMID:22071475

  12. Epstein-Barr virus-associated primary central nervous system lymphoma in a child with the acquired immunodeficiency syndrome. A case report and review of the literature.

    Science.gov (United States)

    Rodriguez, M M; Delgado, P I; Petito, C K

    1997-12-01

    A 34-month-old black boy who had contracted acquired immunodeficiency syndrome from his mother presented with fever, vomiting, and cough. He was cachectic, hypertonic, and developmentally delayed. A brain computed tomography scan revealed masses in the left frontal horn, subependymal, and periventricular regions; secondary edema; and hydrocephalus. The differential diagnosis was cerebral lymphoma versus toxoplasmosis. The patient had disseminated Mycobacterium avium-intracellulare infection, lymphoid interstitial pneumonitis, as well as Pseudomonas and Klebsiella pneumonia. He died of respiratory insufficiency 53 days after admission. The autopsy confirmed a primary cerebral B-cell lymphoma, large cell type, which was positive for Epstein-Barr virus, latent phase, by in situ hybridization. Primary central nervous system lymphomas are rare in children, in contrast to adults. To our knowledge, only five well-documented cases of primary cerebral lymphomas in infants and children with acquired immunodeficiency syndrome have been reported previously. The current study shows that these childhood lymphomas are associated with and presumably caused by Epstein-Barr virus and thus have a pathogenesis similar to that of primary central nervous system lymphomas in adults.

  13. Helicobacter pylori-Negative Primary Rectal MALT Lymphoma: Complete Remission after Radiotherapy

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    Takuma Okamura

    2012-05-01

    Full Text Available Rectal mucosa-associated lymphoid tissue (MALT lymphoma is a rare condition. Although the majority of patients undergo surgical resection, a definitive treatment for rectal MALT lymphoma has not yet been established. In the present study, we report the outcome of radiotherapy in 3 patients with rectal MALT lymphoma. Our cohort ranged from 56 to 65 years of age. The male/female ratio was 1:2, and all patients were in stage I (Lugano classification of the disease. Endoscopic findings revealed elevated lesions resembling submucosal tumors in 2 patients, and a sessile elevated lesion with a nodular surface in 1 patient. One of the 3 patients underwent magnifying endoscopy with crystal violet staining that demonstrated a type I pit pattern (Kudo’s classification lesion with a broad intervening area caused by the upthrust of the tumor from the submucosa. All patients tolerated radiotherapy at doses of 30 Gy without major complications and achieved complete remission. Follow-up ranged from 13 to 75 months (mean 51.0 months, revealing no recurrence of MALT lymphoma. As such, we propose radiotherapy to be a safe and effective means for treating rectal MALT lymphoma.

  14. Linfoma primário do sistema nervoso central Primary central nervous system lymphoma

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    Marcelo Bellesso

    2008-02-01

    Full Text Available O linfoma primário do sistema nervoso central (LPSNC é um linfoma extralinfonodal que, ao diagnóstico, encontra-se restrito ao parênquima cerebral, às meninges e/ou cordão espinhal e/ou olhos. Sua incidência triplicou nas últimas três décadas para 0,4 casos por 100.000 habitantes, representando 4% dos tumores do sistema nervoso central (SNC. Embora pacientes infectados pelo HIV tenham 3.600 vezes maior risco para o desenvolvimento do LPSNC, a incidência não aumentou apenas neste grupo de pessoas. Dados sugerem reduções da incidência de LPSNC em pacientes infectados após a introdução de drogas anti-retrovirais. Cerca de 90% dos casos de LPSNC são classificados como linfoma difuso de grandes células B, 10% têm envolvimento ocular e 10% são HIV positivos. A apresentação clínica depende da localização tumoral, prevalecendo os sintomas neurológicos em detrimento aos sistêmicos. Os exames de tomografia computadorizada (TC e ressonância nuclear magnética (RNM são essenciais para o diagnóstico, porém o exame confirmatório deve ser o anatomopatológico. O estadiamento deve ser feito com exames de imagem e biópsia de medula óssea (BMO bilateral. Os principais fatores de mau prognóstico são: performance status do paciente acima de 1, idade superior a 60 anos, DHL elevada, hiperproteinorraquia e acometimento de área cerebral não hemisférica. Alguns fatores de prognóstico biológicos também podem influenciar na sobrevida, a exemplo da expressão de Bcl-6, que confere melhor prognóstico. O tratamento de escolha é a combinação de quimioterapia contendo altas doses de metotrexate e radioterapia (RDT. Devido às altas taxas de neurotoxicidade associada à RDT, seu uso tem ficado mais restrito aos pacientes idosos, e os recidivados ou refratários.Primary Central Nervous System lymphoma (PCNSL is an extranodal non-Hodgkin lymphoma in the brain, leptomeninges, spinal cord or eyes. The incidence of PCNSL increased

  15. Ibrutinib in Treating Relapsed or Refractory B-Cell Non-Hodgkin Lymphoma in Patients With HIV Infection

    Science.gov (United States)

    2015-08-18

    Adult B Acute Lymphoblastic Leukemia; Chronic Lymphocytic Leukemia; Cutaneous B-Cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue; HIV Infection; Intraocular Lymphoma; Multicentric Angiofollicular Lymphoid Hyperplasia; Nodal Marginal Zone Lymphoma; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Immunoblastic Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Small Lymphocytic Lymphoma; Refractory Chronic Lymphocytic Leukemia; Refractory Hairy Cell Leukemia; Refractory Plasma Cell Myeloma; Small Intestinal Lymphoma; Splenic Marginal Zone Lymphoma; Testicular Lymphoma; Waldenstrom Macroglobulinemia

  16. Treatment of primary brain lymphoma without immune deficiency, The importance of chemotherapy before radiotherapy

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    Keihani M

    1999-09-01

    Full Text Available The purpose of this study was to find a more efficacious treatment for patients with primary central nervous system Lymphoma using chemotherapy. The objective was to determine the optimal time for radiotherapy treatment in relation to chemotherapy. Retrospective evaluation in patients with brain lymphoma was conducted from 1992 to 1998. Twenty-three patients were evaluated. Patients were divided into two groups based on the timing of radiotherapy in relation to the chemotherapy. The first group of patients (n=13 initially received radiotherapy followed by chemotherapy. Five of these patients receied classic CHOP (cyclophosphamide, Doxorubicine, Vincistine and Prednisone, six patients received Cis-platin (60 Megs/M2 and Etoposide (120 Megs/M2 and two patients received Cis-platin (60 Megs/M2, Etoposide (120 Megs/M2 and Cytarabine (600 Megs/M2 every 2 to 3 weeks. The second group of patients (Group II, n=10 received the followeing treatment regimen: a course of BCNU 120 Megs/M2 with Ifosfamide 1200 Megs/M2, Mesna and Etoposide 120 Megs/M2 on the first day of treatment (course A. Two weeks later, treatment was continued with a course of Cis-platin 35 Megs/M2 and Cytarabine 600 Megs/M2 (course B. The treatment was continued 14 days later with a course of Mitoxantron 12 Megs/M2, Ifosfamide 1200 Megs/M2 puls Mesna (course C. After the fourth week of chemotherapy, these patients received radiotherapy to the brain (5000 RADS in 4 weeks. During radiotherapy and at the beginning of course chemotherapy, intrathecal therapy with Methorexate 12 Megs/M2 and Cytarabine 60 Megs/M2 was given. Immediately after radiotherapy, the same chemothotrexate 12 Megs/M2 and Cytarabine 60 Megs/M2 was given. Immediately after radiotherapy, the same chemotherapy treatment was repeated to a total of 3 times. After complete clearance of the tumor determined by MRI and absence of tumor cells in the spinal fluid, the chemotherapeutic regimen was repeated one last time. The

  17. Primary Effusion Lymphoma Involving both Pleural and Abdominal Cavities in a Patient with Hepatitis B Virus-related Liver Cirrhosis

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    Pei-Ying Hsieh

    2007-01-01

    Full Text Available Primary effusion lymphoma (PEL is an unusual form of non-Hodgkin's lymphoma, which is characterized by lymphomatous effusion in body cavities, but no associated mass lesions. It is usually associated with an immunodeficient state most often with the human immunodeficiency virus (HIV. We describe a 54-year-old man with HIV-negative PEL, with a history of hepatitis B virus-related liver cirrhosis. Both abdominal and pleural cavities were involved; no solid tumor masses were found and bone marrow investigations were normal. The ascites and pleural effusion contained numerous pleomorphic lymphoid cells. Immunophenotyping was positive for CD138. Chromosome study showed complex cytogenetics. The genomic human herpesvirus-8 was detected in the lymphoma cells. It is postulated that the immuno-suppressed state in this patient may have been caused by cirrhosis. The patient received four cycles of chemotherapy of CHOP and Picibanil (OK-432 intraperitoneal administration. However, no durable remission was achieved. Adefovir failed to halt the progressive liver failure after the development of YMDD mutant related to lamivudine. He died of sepsis and hepatic failure.

  18. Is 18F-FDG PET/CT useful for distinguishing between primary thyroid lymphoma and chronic thyroiditis?

    Science.gov (United States)

    Nakadate, Masashi; Yoshida, Katsuya; Ishii, Akihiro; Koizumi, Masayuki; Tochigi, Naobumi; Suzuki, Yoshio; Ryu, Yoshiharu; Nakagawa, Tassei; Umehara, Isao; Shibuya, Hitoshi

    2013-09-01

    This study aims to investigate the usefulness of (18)F-FDG PET/CT for distinguishing between primary thyroid lymphoma (PTL) and chronic thyroiditis. We retrospectively reviewed the data of 196 patients with diffuse (18)F-FDG uptake of the thyroid gland and enrolled patients who were diagnosed as having PTL or chronic thyroiditis based on the medical records, pathological findings, and laboratory data. The enrolled patients comprised 10 PTL patients (M/F = 4:6) and 51 chronic thyroiditis patients (M/F = 8:43). Images had been acquired on a PET/CT scanner at 100 minutes after intravenous injection of (18)F-FDG. The PTL group consisted of 7 patients with diffuse large B-cell lymphoma (DLBCL) and 3 with mucosa-associated lymphoid tissue (MALT) lymphoma. The maximum standardized uptake value (SUV(max)) was significantly higher in the PTL group than that in the chronic thyroiditis group (25.3 ± 8.0 and 7.4 ± 3.2, P thyroiditis group (46.1 ± 7.0 HU and 62.1 ± 6.9 HU, P thyroiditis. Thus, (18)F-FDG PET/CT may be useful for distinguishing between PTL and chronic thyroiditis.

  19. SU-E-I-100: Heterogeneity Studying for Primary and Lymphoma Tumors by Using Multi-Scale Image Texture Analysis with PET-CT Images

    Energy Technology Data Exchange (ETDEWEB)

    Li, Dengwang [Shandong Normal University, Jinan, Shandong Province (China); Wang, Qinfen [Shandong Normal University, Jinan, Shandong (China); Li, H; Chen, J [Shandong Cancer Hospital and Institute, Jinan, Shandong (China)

    2014-06-01

    Purpose: The purpose of this research is studying tumor heterogeneity of the primary and lymphoma by using multi-scale texture analysis with PET-CT images, where the tumor heterogeneity is expressed by texture features. Methods: Datasets were collected from 12 lung cancer patients, and both of primary and lymphoma tumors were detected with all these patients. All patients underwent whole-body 18F-FDG PET/CT scan before treatment.The regions of interest (ROI) of primary and lymphoma tumor were contoured by experienced clinical doctors. Then the ROI of primary and lymphoma tumor is extracted automatically by using Matlab software. According to the geometry size of contour structure, the images of tumor are decomposed by multi-scale method.Wavelet transform was performed on ROI structures within images by L layers sampling, and then wavelet sub-bands which have the same size of the original image are obtained. The number of sub-bands is 3L+1.The gray level co-occurrence matrix (GLCM) is calculated within different sub-bands, thenenergy, inertia, correlation and gray in-homogeneity were extracted from GLCM.Finally, heterogeneity statistical analysis was studied for primary and lymphoma tumor using the texture features. Results: Energy, inertia, correlation and gray in-homogeneity are calculated with our experiments for heterogeneity statistical analysis.Energy for primary and lymphomatumor is equal with the same patient, while gray in-homogeneity and inertia of primaryare 2.59595±0.00855, 0.6439±0.0007 respectively. Gray in-homogeneity and inertia of lymphoma are 2.60115±0.00635, 0.64435±0.00055 respectively. The experiments showed that the volume of lymphoma is smaller than primary tumor, but thegray in-homogeneity and inertia were higher than primary tumor with the same patient, and the correlation with lymphoma tumors is zero, while the correlation with primary tumor isslightly strong. Conclusion: This studying showed that there were effective heterogeneity

  20. Radiation therapy of primary extranodal Non-Hodgkin's lymphoma of head and neck. Results of a prospective multicenter study

    International Nuclear Information System (INIS)

    Hoederath, A.; Sack, H.; Stuschke, M.; Lampka, E.

    1996-01-01

    Between January 1986 and August 1993, 63 patients with primary extranodal Non-Hodgkin lymphoma of head and neck region, stages IE and IIE were treated with radiotherapy. The histological classification followed the Kiel classification, staging the Ann Arbor classification. Patient characteristics: 33 Male, 30 female; age 18 to 84 years; tumor localisation: Tonsils 26, nasopharynx 7, oropharynx 8, paranasal sinus 11, salivary glands 7, floor of mouth/gingiva 3, larynx 1. Mean follow-up is 74 months. Low-grade lymphoma in stages I and II CS were treated with definitive radiation therapy according to the concepts of epithelial tumors of the same localisation (target volume and technique).The adjuvant dose was 30 Gy and in the tumor volume 40 Gy, 2 Gy daily. 28 patients were registered, 18 in stage I and 10 in stage II. High-grade lymphoma were treated with definitive radiation therapy according to the concepts of epithelial tumors of the same localisation, too. The dose was 40 respectively 50 Gy, followed by 4 course of adjuvant chemotherapy with CHOP. Thirty-five patients were enrolled, of whom only 10 received chemotherapy. The overall 5-year survical rates were for low-grade 67% and for high grade lymphoma 88%. The corresponding relapse-free survival rates were 54/68%, respectively. Only 1 patient failed within the irradiated target volume. Recurrences occurred at sites distant to the irradiated volume in nodal and extranodal regions. Prognosis was influenced by histologic grade. Significant trends were not observed for other potential pretreatment parameters (age, stage, localisation, bulk). (orig./MG) [de

  1. Lenalidomide and Combination Chemotherapy (DA-EPOCH-R) in Treating Patients With MYC-Associated B-Cell Lymphomas

    Science.gov (United States)

    2017-09-28

    Adult Grade III Lymphomatoid Granulomatosis; B-cell Chronic Lymphocytic Leukemia; Contiguous Stage II Adult Diffuse Large Cell Lymphoma; Contiguous Stage II Adult Diffuse Mixed Cell Lymphoma; Contiguous Stage II Adult Diffuse Small Cleaved Cell Lymphoma; Contiguous Stage II Adult Immunoblastic Large Cell Lymphoma; Contiguous Stage II Grade 1 Follicular Lymphoma; Contiguous Stage II Grade 2 Follicular Lymphoma; Contiguous Stage II Grade 3 Follicular Lymphoma; Contiguous Stage II Mantle Cell Lymphoma; Contiguous Stage II Marginal Zone Lymphoma; Contiguous Stage II Small Lymphocytic Lymphoma; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Noncontiguous Stage II Adult Diffuse Large Cell Lymphoma; Noncontiguous Stage II Adult Diffuse Mixed Cell Lymphoma; Noncontiguous Stage II Adult Diffuse Small Cleaved Cell Lymphoma; Noncontiguous Stage II Adult Immunoblastic Large Cell Lymphoma; Noncontiguous Stage II Grade 1 Follicular Lymphoma; Noncontiguous Stage II Grade 2 Follicular Lymphoma; Noncontiguous Stage II Grade 3 Follicular Lymphoma; Noncontiguous Stage II Mantle Cell Lymphoma; Noncontiguous Stage II Marginal Zone Lymphoma; Noncontiguous Stage II Small Lymphocytic Lymphoma; Progressive Hairy Cell Leukemia, Initial Treatment; Small Intestine Lymphoma; Splenic Marginal Zone Lymphoma; Stage 0 Chronic Lymphocytic Leukemia; Stage I Adult Diffuse Large Cell Lymphoma; Stage I Adult Diffuse Mixed Cell Lymphoma; Stage I Adult Diffuse Small Cleaved Cell Lymphoma; Stage I Adult Hodgkin Lymphoma; Stage I Adult Immunoblastic Large Cell Lymphoma; Stage I Chronic Lymphocytic Leukemia; Stage I Grade 1 Follicular Lymphoma; Stage I Grade 2 Follicular Lymphoma; Stage I Grade 3 Follicular Lymphoma; Stage I Mantle Cell Lymphoma; Stage I Marginal Zone Lymphoma; Stage I Small Lymphocytic Lymphoma; Stage II Adult Hodgkin Lymphoma; Stage II Chronic Lymphocytic

  2. Primary endobronchial marginal zone B-cell lymphoma of bronchus-associated lymphoid tissue: CT findings 7 patients

    International Nuclear Information System (INIS)

    Yoon, Ra Gyoung; Kim, Mi Young; Song, Jae Woo; Chae, Eun Jin; Choi, Chang Min; Jang, Se Jin

    2013-01-01

    To investigate CT and 1 8F -fluorodeoxyglucose (1 8F -FDG) positron-emission tomography/CT findings of primary endobronchial marginal zone B-cell lymphoma of the bronchus-associated lymphoid tissue (BALT). From June 2006 through April 2012, seven patients (six female, one male; age range, 21-61 years; mean age, 49 years) were examined who were pathologically diagnosed with the primary endobronchial marginal zone B-cell lymphoma of BALT. We evaluated the locations and characteristics of the lesions on CT and 1 8F -FDG-PET/CT scans. The lesions were classified into the following three patterns: 1) solitary intraluminal nodule; 2) several tiny nodular protrusions; and 3) diffuse wall thickening. A solitary intraluminal nodule was observed in four patients (57.1%), several tiny nodular protrusion in two patients (28.6%), and diffuse wall thickening in one patient (14.3%). The lesions were categorized into 3 major locations: confined to the trachea (n 3), confined to the lobar bronchus (n = 2), and diffuse involvement of the trachea and both main bronchi (n = 2). All lesions demonstrated homogeneous iso-attenuation as compared with muscle on pre- and post-enhancement scans. Secondary findings in the lungs (n = 3; 42.9%) included postobstructive lobar atelectasis (n = 1), air trapping (n = 1), and pneumonia (n = 1). On 1 8F -FDG-PET/CT (n = 5), 4 lesions showed homogeneous uptake with maximum standardized uptake values (mSUV), ranging 2.3-5.7 (mean mSUV: 3.3). One lesion showed little FDG uptake. Primary endobronchial marginal zone B-cell lymphoma of the BALT manifests as three distinct patterns on CT, with the solitary intraluminal nodule presenting as the main pattern. Most lesions demonstrate homogeneous but weak FDG uptake on 1 8F -FDG-PET/CT.

  3. Primary endobronchial marginal zone B-cell lymphoma of bronchus-associated lymphoid tissue: CT findings 7 patients

    Energy Technology Data Exchange (ETDEWEB)

    Yoon, Ra Gyoung; Kim, Mi Young; Song, Jae Woo; Chae, Eun Jin; Choi, Chang Min; Jang, Se Jin [University of Ulsan College of Medicine, Seoul (Korea, Republic of)

    2013-04-15

    To investigate CT and 1{sup 8F}-fluorodeoxyglucose (1{sup 8F}-FDG) positron-emission tomography/CT findings of primary endobronchial marginal zone B-cell lymphoma of the bronchus-associated lymphoid tissue (BALT). From June 2006 through April 2012, seven patients (six female, one male; age range, 21-61 years; mean age, 49 years) were examined who were pathologically diagnosed with the primary endobronchial marginal zone B-cell lymphoma of BALT. We evaluated the locations and characteristics of the lesions on CT and 1{sup 8F}-FDG-PET/CT scans. The lesions were classified into the following three patterns: 1) solitary intraluminal nodule; 2) several tiny nodular protrusions; and 3) diffuse wall thickening. A solitary intraluminal nodule was observed in four patients (57.1%), several tiny nodular protrusion in two patients (28.6%), and diffuse wall thickening in one patient (14.3%). The lesions were categorized into 3 major locations: confined to the trachea (n 3), confined to the lobar bronchus (n = 2), and diffuse involvement of the trachea and both main bronchi (n = 2). All lesions demonstrated homogeneous iso-attenuation as compared with muscle on pre- and post-enhancement scans. Secondary findings in the lungs (n = 3; 42.9%) included postobstructive lobar atelectasis (n = 1), air trapping (n = 1), and pneumonia (n = 1). On 1{sup 8F}-FDG-PET/CT (n = 5), 4 lesions showed homogeneous uptake with maximum standardized uptake values (mSUV), ranging 2.3-5.7 (mean mSUV: 3.3). One lesion showed little FDG uptake. Primary endobronchial marginal zone B-cell lymphoma of the BALT manifests as three distinct patterns on CT, with the solitary intraluminal nodule presenting as the main pattern. Most lesions demonstrate homogeneous but weak FDG uptake on 1{sup 8F}-FDG-PET/CT.

  4. Long-Term Outcomes of 107 Cases of Primary Thyroid Mucosa-Associated Lymphoid Tissue Lymphoma at a Single Medical Institution in Japan.

    Science.gov (United States)

    Watanabe, Natsuko; Narimatsu, Hiroto; Noh, Jaeduk Yoshimura; Iwaku, Kenji; Kunii, Yo; Suzuki, Nami; Ohye, Hidemi; Suzuki, Miho; Matsumoto, Masako; Yoshihara, Ai; Kameyama, Kaori; Kobayashi, Kazuhiko; Kami, Masahiro; Sugino, Kiminori; Ito, Koichi

    2018-02-01

    Thyroid mucosa-associated lymphoid tissue (MALT) lymphoma is a type of extranodal lymphoma with a favorable prognosis. To provide information on long-term outcomes that would facilitate establishment of the optimal management strategy for thyroid lymphoma. Medical records of 107 patients (median age 67 years, 20 males, 87 females) who were diagnosed with localized thyroid MALT lymphoma stage IE or IIE at Ito Hospital were retrospectively reviewed. Overall and event-free survival (EFS). Initial treatments included radiation therapy (RT) alone (n = 58), combined modality therapy (CMT) (n = 48), or chemotherapy alone (n = 1). All 107 patients responded to the treatment, six of whom experienced relapse. Only one patient died of lymphoma. The 5-year overall survival (OS) and EFS rates were 94% [95% confidence interval (CI), 87% to 97%] and 92% (95% CI, 85% to 95%), respectively, and the 10-year OS and EFS rates were 91% (95% CI, 83% to 95%) and 84% (95% CI, 74% to 90%), respectively. Of the 106 patients with information available on adverse events, 71 patients (67%) developed hypothyroidism after primary thyroid lymphoma treatment. The CMT group showed additional chemotherapy-induced adverse reactions in the form of neutropenia, neuropathy, constipation, and pneumonia. The 5-year OS rates of patients treated with CMT and RT were 93% (95% CI, 81% to 98%) and 94% (95% CI, 84% to 98%), respectively. Long-term outcomes of localized thyroid MALT lymphoma are favorable with all initial treatment modalities. Copyright © 2017 Endocrine Society

  5. Breast lymphoma

    African Journals Online (AJOL)

    To fulfil the criteria for primary breast lymphoma, the following characteristics were reqUired: (I) technically adequate specimens; (iI) mammary tissue and lymphomatous infiltrate in close association; (iil) no evidence of concurrent widespread disease; and (iv) no previous. Haematology/Oncology Division, Department of ...

  6. Highly selective fusion and accumulation of hybrid liposomes into primary effusion lymphoma cells along with induction of apoptosis

    Energy Technology Data Exchange (ETDEWEB)

    Towata, Tomomi [Division of Applied Life Science, Graduate School of Engineering, Sojo University, 4-22-1 Ikeda, Kumamoto 860-0082 (Japan); Division of Hematopoiesis, Center for AIDS Research, Kumamoto University, 2-2-1 Honjo, Kumamoto 860-0811 (Japan); Komizu, Yuji [Division of Applied Life Science, Graduate School of Engineering, Sojo University, 4-22-1 Ikeda, Kumamoto 860-0082 (Japan); Suzu, Shinya [Division of Hematopoiesis, Center for AIDS Research, Kumamoto University, 2-2-1 Honjo, Kumamoto 860-0811 (Japan); Ueoka, Ryuichi, E-mail: ueoka@life.sojo-u.ac.jp [Division of Applied Life Science, Graduate School of Engineering, Sojo University, 4-22-1 Ikeda, Kumamoto 860-0082 (Japan); Okada, Seiji, E-mail: okadas@kumamoto-u.ac.jp [Division of Hematopoiesis, Center for AIDS Research, Kumamoto University, 2-2-1 Honjo, Kumamoto 860-0811 (Japan)

    2010-03-12

    Primary effusion lymphoma (PEL) is an aggressive neoplasm caused by human herpes virus-8 infection, and is generally resistant to chemotherapy. Hybrid liposomes, composed of dimyristoylphosphatidylcholine (DMPC) and polyoxyethylene (21) dodecyl ether (C{sub 12}(EO){sub 21}) (HL-21), were rapidly accumulated in the membrane of PEL cells. HL-21 also increased membrane fluidity of PEL cells, and induced caspase-3 activation along with cell death. These results suggest that HL-21 should be an effective and attractive regent for PEL treatment.

  7. Severe sunburn and subsequent risk of primary cutaneous malignant melanoma in scotland.

    Science.gov (United States)

    MacKie, R. M.; Aitchison, T.

    1982-01-01

    A case-control study of occupational and recreational sun exposure, Mediterranean and other sun-exposed holidays, tanning history and history of isolated episodes of severe sunburn has been carried out on 113 patients with cutaneous malignant melanoma and 113 age- and sex-matched controls. Social class and skin type were also considered in the analysis of the data which involved the use of conditional multiple logistic regression. A highly significant increase in the history of severe sunburn was recorded in melanoma patients of both sexes in the 5-year period preceding presentation with their tumour. Higher social class and negative history of recreational sun exposure were also significantly increased in patients by comparison with controls. In the male group severe sunburn, lack of occupational sun exposure and higher social class were significant factors while in the female group only severe sunburn was significantly increased in the melanoma patients. This study thus provides evidence to suggest that short intense episodes of UV exposure resulting in burning may be one of the aetiological factors involved in subsequent development of melanoma. PMID:7150488

  8. Primary Intra-aortic Epstein-Barr Virus-Positive Large B-Cell Lymphoma Presenting as Aortic Mural Thrombosis: An Entity Distinct From Intravascular Large B-Cell Lymphoma.

    Science.gov (United States)

    Nakao, Ryuta; Sakashita, Aki; Omoto, Atsushi; Sato, Osamu; Hino, Yoko; Yanagisawa, Akio; Urata, Yoji

    2017-12-01

    Intravascular selective growth of neoplastic B lymphocytes is a characteristic finding of intravascular large B-cell lymphoma (IVLBCL). However, because neoplastic B cells of IVLBCL grow merely in the lumina of capillaries or small vessels, primary IVLBCL of the great vessels is considered exceptional. To our knowledge, only 2 primary B-cell lymphomas in the lumina of the vena cava have been reported. However, there has been no report of primary B-cell lymphoma with intra-aortic growth. We describe a novel manifestation of primary Epstein-Barr virus-positive large B-cell lymphoma mainly affecting the lumina of the aorta and its major branches in a 76-year-old man. He had a long-term fever that was refractory to antibiotics and aortic mural thrombosis with visceral embolization. Because he had no detectable mass suggesting a malignancy, it was difficult to diagnose while he was alive. He died without anticancer treatment, and the confirmed diagnosis was made at autopsy.

  9. Intussusception as clinical presentation of primary non-Hodgkin lymphoma of the colon in a HIV-patient

    Directory of Open Access Journals (Sweden)

    Marcelo Corti

    Full Text Available Intestinal intussusception rarely occurs in the adult population and accounts only for 1% to 5% of all the causes of intestinal obstruction. This complication is more frequent in the small bowel and can be due to different aetiologies, including inflammatory, infectious or neoplastic diseases. Malignancies account for 50% to 60% of all cases of colon invagination. The gastrointestinal (GI tract is the most common site for extra-nodal non-Hodgkin lymphomas (NHL, representing 5% to 20% of all the cases. However, primary NHL of the GI tract is a very infrequent clinic-pathological entity and accounts only for 1% to 4% of all the neoplasms of the GI tract. Primary NHL of the colon is a rare disease and it comprises only 0.2% to 1.2% of all colonic malignancies. Here we describe a case of an AIDS adult patient who developed an intussusception secondary to a primary large B cell lymphoma of the transverse colon. English and Spanish literature was reviewed.

  10. Gene Mutation Profiles in Primary Diffuse Large B Cell Lymphoma of Central Nervous System: Next Generation Sequencing Analyses

    Science.gov (United States)

    Todorovic Balint, Milena; Jelicic, Jelena; Mihaljevic, Biljana; Kostic, Jelena; Stanic, Bojana; Balint, Bela; Pejanovic, Nadja; Lucic, Bojana; Tosic, Natasa; Marjanovic, Irena; Stojiljkovic, Maja; Karan-Djurasevic, Teodora; Perisic, Ognjen; Rakocevic, Goran; Popovic, Milos; Raicevic, Sava; Bila, Jelena; Antic, Darko; Andjelic, Bosko; Pavlovic, Sonja

    2016-01-01

    The existence of a potential primary central nervous system lymphoma-specific genomic signature that differs from the systemic form of diffuse large B cell lymphoma (DLBCL) has been suggested, but is still controversial. We investigated 19 patients with primary DLBCL of central nervous system (DLBCL CNS) using the TruSeq Amplicon Cancer Panel (TSACP) for 48 cancer-related genes. Next generation sequencing (NGS) analyses have revealed that over 80% of potentially protein-changing mutations were located in eight genes (CTNNB1, PIK3CA, PTEN, ATM, KRAS, PTPN11, TP53 and JAK3), pointing to the potential role of these genes in lymphomagenesis. TP53 was the only gene harboring mutations in all 19 patients. In addition, the presence of mutated TP53 and ATM genes correlated with a higher total number of mutations in other analyzed genes. Furthermore, the presence of mutated ATM correlated with poorer event-free survival (EFS) (p = 0.036). The presence of the mutated SMO gene correlated with earlier disease relapse (p = 0.023), inferior event-free survival (p = 0.011) and overall survival (OS) (p = 0.017), while mutations in the PTEN gene were associated with inferior OS (p = 0.048). Our findings suggest that the TP53 and ATM genes could be involved in the molecular pathophysiology of primary DLBCL CNS, whereas mutations in the PTEN and SMO genes could affect survival regardless of the initial treatment approach. PMID:27164089

  11. Primary central nervous system lymphoma: age and performance status are more important than treatment modality

    International Nuclear Information System (INIS)

    Corry, June; Smith, Jennifer G.; Wirth, Andrew; Quong, George; Liew, Kuen Hoe

    1998-01-01

    Purpose: To assess prognostic factors and treatment modalities of patients with primary central nervous system lymphoma (PCNSL) in terms of response rates, patterns of failure and overall survival. Methods and Materials: Sixty-two patients presenting with PCNSL between 1982 and 1994 at Peter MacCallum Cancer Institute with no evidence of human immunodeficiency virus infection were included in the study. Their median age was 60 years; World Health Organisation (WHO) performance status was ≥2 in 85%. All patients were planned to receive whole brain irradiation; 7 also received spinal irradiation. The median planned dose to the target volume was 50.4 Gy. Twenty patients were planned to receive chemotherapy as well. Patients were followed up to June 20, 1995, giving a median follow-up for 14 surviving patients of 5.4 years, range 0.3 to 10.2 years. Results: The clinical response rate to treatment was 77% [95% confidence interval (CI) 65 to 87%]. The estimated median overall survival was 20.6 months (CI 12.4 to 33.4 months). On univariate analysis male gender, age <60 years, WHO performance status ≤1, treatment to the target volume ≥45 Gy, and treatment with additional chemotherapy, were associated with a significantly better overall survival (p < 0.05). On multivariate analysis only age and performance status remained significant prognostic variables. Relapse involved the central nervous system or cerebrospinal fluid (CSF) in all patients with known sites of relapse except three who had ocular relapse only. There was a low incidence of relapse in the initial brain site (23% of known cases) and a high incidence (50%) of CSF/spinal cord relapse. Of 48 deaths, 15 were related to initial or subsequent treat