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Sample records for sarcoma primary effusion

  1. Azidothymidine Sensitizes Primary Effusion Lymphoma Cells to Kaposi Sarcoma-Associated Herpesvirus-Specific CD4+ T Cell Control and Inhibits vIRF3 Function.

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    Samantha J Williamson

    2016-11-01

    Full Text Available Kaposi sarcoma-associated herpesvirus (KSHV is linked with the development of Kaposi sarcoma and the B lymphocyte disorders primary effusion lymphoma (PEL and multi-centric Castleman disease. T cell immunity limits KSHV infection and disease, however the virus employs multiple mechanisms to inhibit efficient control by these effectors. Thus KSHV-specific CD4+ T cells poorly recognize most PEL cells and even where they can, they are unable to kill them. To make KSHV-infected cells more sensitive to T cell control we treated PEL cells with the thymidine analogue azidothymidine (AZT, which sensitizes PEL lines to Fas-ligand and TRAIL challenge; effector mechanisms which T cells use. PELs co-cultured with KSHV-specific CD4+ T cells in the absence of AZT showed no control of PEL outgrowth. However in the presence of AZT PEL outgrowth was controlled in an MHC-restricted manner. To investigate how AZT sensitizes PELs to immune control we first examined BJAB cells transduced with individual KSHV-latent genes for their ability to resist apoptosis mediated by stimuli delivered through Fas and TRAIL receptors. This showed that in addition to the previously described vFLIP protein, expression of vIRF3 also inhibited apoptosis delivered by these stimuli. Importantly vIRF3 mediated protection from these apoptotic stimuli was inhibited in the presence of AZT as was a second vIRF3 associated phenotype, the downregulation of surface MHC class II. Although both vFLIP and vIRF3 are expressed in PELs, we propose that inhibiting vIRF3 function with AZT may be sufficient to restore T cell control of these tumor cells.

  2. Role of defective Oct-2 and OCA-B expression in immunoglobulin production and Kaposi's sarcoma-associated herpesvirus lytic reactivation in primary effusion lymphoma.

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    Di Bartolo, Daniel L; Hyjek, Elizabeth; Keller, Shannon; Guasparri, Ilaria; Deng, Hongyu; Sun, Ren; Chadburn, Amy; Knowles, Daniel M; Cesarman, Ethel

    2009-05-01

    Primary effusion lymphoma (PEL) is a distinct type of B-cell non-Hodgkin lymphoma characterized by the presence of Kaposi's sarcoma-associated herpesvirus (KSHV/human herpesvirus 8). Despite having a genotype and gene expression signature of highly differentiated B cells, PEL does not usually express surface or cytoplasmic immunoglobulin (Ig). We show the lack of Oct-2 and OCA-B transcription factors to be responsible, at least in part, for this defect in Ig production. Like Ig genes, ORF50, the key regulator of the switch from latency to lytic reactivation, contains an octamer motif within its promoter. We therefore examined the impact of Oct-2 and OCA-B on ORF50 activation. The binding of Oct-1 to the ORF50 promoter has been shown to significantly enhance ORF50 transactivation. We found that Oct-2, on the other hand, inhibited ORF50 expression and consequently lytic reactivation by competing with Oct-1 for the octamer motif in the ORF50 promoter. Our data suggest that Oct-2 downregulation in infected cells would be favorable to KSHV in allowing for efficient viral reactivation.

  3. Primary renal synovial sarcoma

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    Girish D. Bakhshi

    2012-03-01

    Full Text Available Primary Renal Sarcoma is rare tumor comprising only 1% of all renal tumours. Synovial sarcomas are generally deep-seated tumors arising in the proximity of large joints of adolescents and young adults and account for 5-10% of all soft tissue tumours. Primary synovial sarcoma of kidney is rare and has poor prognosis. It can only be diagnosed by immunohistochemistry. It should be considered as a differential in sarcomatoid and spindle cell tumours. We present a case of 33-year-old female, who underwent left sided radical nephrectomy for renal tumour. Histopathology and genetic analysis diagnosed it to be primary renal synovial sarcoma. Patient underwent radiation therapy and 2 years follow up is uneventful. A brief case report with review of literature is presented.

  4. Multimodality management of primary diaphragmatic synovial sarcoma: First report

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    Preyas J Vaidya

    2016-01-01

    Full Text Available Synovial cell sarcoma is an extremely rare tumor of mesenchymal origin. It commonly affects the soft tissues of the extremities but could possibly origin from the head and neck, heart, lung, pleura, mediastinum, esophagus, abdominal wall and the mesentery, and retroperitoneum. Primary synovial sarcoma of pleura, mediastinum, and lung have been reported. Primary synovial sarcoma of the diaphragm has not been reported to the best of our knowledge. We report a case of primary synovial cell sarcoma of the diaphragm presenting as a recurrent pleural effusion and pain in the left hypochondrium managed with multimodality approach.

  5. Ewing’s Sarcoma Presenting as Pleural Effusion

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    Nuzhat Husain

    2011-09-01

    Full Text Available  A 20-year-old female presented to the Pulmonary Medicine Department with complaints of fever, left sided chest pain and progressive dyspnoea of four months duration. Radiological examination revealed a mass lesion with massive pleural effusion and rib erosion. Histopathology showed neoplastic cells with scanty cytoplasm, hyperchromatic nuclei and rosette formation suggestive of Ewing sarcoma. The rarity of this tumor and its unusual presentation prompted this report.

  6. Primary Intradural Extraosseous Ewing's Sarcoma

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    Kim, Seok Won; Shin, Ho

    2009-01-01

    Ewing's sarcoma usually arises from skeletal bone, but rarely may have an extraskeletal origin. However, Ewing's sarcoma that originates around the spinal column, especially, the intradural extramedullary type is extremely rare. We report a rare case of primary intraspinal extraskeletal Ewing's sarcoma.

  7. Primary fibro sarcoma of the heart.

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    Kabashi, Serbeze; Hoxha, Naim; Gashi, Shkelzen; Ahmegjekaj, Ilir; Bejta, Ilir; Sadiku, Muharrem; Ymeri, Halit; Kabashi, Antigona; Bicaj, Xhavit; Mucaj, Sefedin

    2013-01-01

    Primary malignant heart tumors represent rare entities where fibro sarcoma represents about 3% of all. Introducing the patient: A 15 years old patient with cardiac insufficiency (heart failure) symptoms, such as weakness, cyanosis, palpitations and breathing difficulties; enlargement of upper mediastinum and pleural effusion. Through echocardiography a pericardial effusion and intracavitary thrombus in atrium was diagnosed. With computed tomography is diagnosed a tumoral mass in right atrium which is also spread in the right ventricle of the heart. Tumor is completely removed; pat histology result showed primary fibro sarcoma of the heart. At that time no metastasis was found. Conclusion. Primary malignant heart tumors may manifest like cardiac insufficiency or like systemic diseases. Fibrosarcomas are rare and have bad prognosis. On average patients can live around six months after initial symptoms appeared and diagnosis of the tumor was done. In the case of cardiac insufficiency with differential diagnosis we should also think of heart tumors, which could certainly be proved for or eliminated by echocardiography.

  8. Extra osseous primary Ewing's sarcoma.

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    Ali, Syed Asad; Muhammad, Agha Taj; Soomro, Abdul Ghani; Siddiqui, Akmal Jamal

    2010-01-01

    The case of 20 years old boy with an extra osseous Ewing's sarcoma is described. He was initially diagnosed as a case of infiltrative malignant tumour of left suprarenal gland on the basis of preoperative workup but postoperative biopsy of surgically excised specimen confirmed Extra-osseous Ewing's Sarcoma (EES) suprarenal gland with no evidence of malignancy on skeletal scintiscan, bone marrow aspirate and histopathology Suprarenal location of primary EES is unknown and probably has not been reported in literature. We report a unique case of EES.

  9. Primary clear cell sarcoma of bone

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    Choi, J.H.; Gu, M.J.; Kim, M.J.; Bae, Y.K.; Choi, W.H.; Shin, D.S.; Cho, K.H.

    2003-01-01

    Clear cell sarcoma is a rare soft tissue sarcoma of young adults with melanocytic differentiation. It occurs predominantly in the soft tissue of extremities, typically involving tendons and aponeuroses. Primary clear cell sarcoma of bone is extremely rare. We report a case of primary clear cell sarcoma of the right first metatarsal in a 48-year-old woman and provide a literature review of the entity. (orig.)

  10. Markers to differentiate between Kaposi's sarcoma and tuberculous pleural effusions in HIV-positive patients.

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    Coleman, M; Finney, L J; Komrower, D; Chitani, A; Bates, J; Chipungu, G A; Corbett, E; Allain, T J

    2015-02-01

    Kaposi's sarcoma (KS) and tuberculosis (TB) commonly cause pleural effusions in high human immunodeficiency virus (HIV) burden resource-limited countries. Differentiating between them is challenging, as pleural biopsy and TB culture are rarely available. To identify markers to differentiate between TB effusions and KS effusions in HIV-positive patients, and to compare liquid culture and Xpert MTB/RIF in pleural fluid. Fifty HIV-positive patients with pleural effusions recruited in Malawi underwent pleural ultrasound and aspiration. Fluid visual inspection, cell count, bacterial culture, glucose/protein, solid and liquid TB culture and Xpert were performed. The mean age of the patients was 32 years; 30/50 (60%) were male and 29 (58%) had cutaneous/oral KS. Thirteen (26%) pleural fluid samples were liquid culture-positive for TB, while 9/13 (69%) were Xpert-positive. Three (10.3%) KS patients had culture-positive TB effusions; 17 (58.6%) had KS effusions. The relative risk of TB in KS patients increased with limited KS, loculated fluid and low glucose. Eleven (52.3%) non-KS patients had culture-positive TB effusions associated with male sex, straw-coloured fluid and fibrin stranding on ultrasound. KS patients were most likely to have KS effusion, but TB should be considered. Most non-KS patients had TB, supporting the use of World Health Organization guidelines. Xpert identified two thirds of liquid culture-positive results.

  11. Imaging characteristics of primary cranial Ewing sarcoma

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    Li, Wai-Yung; Saunders, Dawn E.; Brock, Penelope

    2005-01-01

    Ewing sarcoma accounts for 10-15% of all childhood malignant bone tumours and is second in prevalence to osteosarcoma. The skull bones are an unusual site of origin of primary Ewing sarcoma in children. Previous reports concentrate on the neurosurgical aspects and relatively good outcome compared to other bone tumours of the calvarium. Reported cases mainly describe the imaging characteristics on CT. To describe the MRI and CT features of primary cranial Ewing sarcoma. The neuroimaging of three cases of primary cranial Ewing sarcoma are reviewed. Our three cases show an extra-axial mass that is high attenuation on CT and low signal on T2-weighted MRI. Haemorrhagic components, dural extension and contrast enhancement are also characteristic features. CT attenuation and magnetic resonance signal characteristics reflect sheets of densely packed cells seen in Ewing sarcoma. (orig.)

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

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    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.)

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

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    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.)

  14. Primary clear cell sarcoma of rib

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    Hersekli, Murat Ali; Ozkoc, Gurkan; Akpinar, Sercan; Ozalay, Metin; Tandogan, Reha N.; Bircan, Sema; Tuncer, Ilhan

    2005-01-01

    Clear cell sarcoma (malignant melanoma of soft tissues) is a very rare soft tissue neoplasm. It generally arises in tendons and aponeuroses. Although metastasis of malignant melanoma to bone is not uncommon, primary clear cell sarcoma of bone is an extremely rare neoplasm. To our knowledge five cases have been reported in the English literature. We present a case of primary clear cell sarcoma of bone in a 28-year-old woman arising in the left ninth rib. We treated the patient with total excision of the mass and postoperative radiotherapy. The patient is alive and well without local recurrence or distant metastasis at 33 months after surgery. (orig.)

  15. Primary pulmonary sarcoma metastasising to the skin during pregnancy.

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    Arikan, Ilker; Barut, Aykut; Harma, Muge; Harma, Mehmet Ibrahim; Erdem, Zuhal; Gezer, Sener

    2012-12-01

    Lung cancer during pregnancy is a rare situation which is being increasingly reported during the past two decades due to a rising trend of cigarette smoking among young women and the tendency to delay pregnancy to a later age in life. We describe the case of a 32-year-old woman with primary pulmonary sarcoma, diagnosed at 31st week of pregnancy. X-ray chest and thoracic magnetic resonance imaging revealed a 9 x 6 cm mass in the left mediastinum, with tracheal shift, and pleural effusion. Biopsy performed during broncoscopy, was reported as mesenchymal tumour. She delivered a baby by Caesarean section at the 32nd week of gestation due to the development of superior vena cava syndrome. A skin biopsy taken 3 weeks later from the nodular lesion at the periumblical region was reported as a tumour metastasis. She received radiotherapy for 10 days, but died in the intensive care unit. Malignancies, even those as uncommon as a pulmonary sarcoma, should be considered in the differential diagnosis of pleural effusion during pregnancy.

  16. Giant primary synovial sarcoma of the anterior mediastinum: A case ...

    African Journals Online (AJOL)

    2015-06-11

    Jun 11, 2015 ... We present a case of primary monophasic synovial sarcoma of the anterior ... Here, we report a case of ... fatigue and anorexia, but no weight loss. ..... Primary intrathoracic synovial sarcoma: A clinicopathologic study of. 40 t (X ...

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

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    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.

  18. Primary extraskeletal Ewing's sarcoma/primitive neuroectodermal tumour of breast

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    Ikhwan, S M; Kenneth, V K T; Seoparjoo, A; Zin, A A M

    2013-01-01

    Primary primitive neuroectodermal tumour (PNET) and extraskeletal Ewing's sarcoma belongs to the Ewing's family of tumours. Primary tumours arising from breast are very rare. There are only a few case reports published on primary extraskeletal Ewing's sarcoma and PNET arising from breast. We present an extremely rare case of an inoperable primary Ewing's sarcoma arising from left breast with contralateral breast, lymphatic and lung metastasis.

  19. Primary extraskeletal Ewing's sarcoma/primitive neuroectodermal tumour of breast.

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    Ikhwan, S M; Kenneth, V K T; Seoparjoo, A; Zin, A A M

    2013-06-21

    Primary primitive neuroectodermal tumour (PNET) and extraskeletal Ewing's sarcoma belongs to the Ewing's family of tumours. Primary tumours arising from breast are very rare. There are only a few case reports published on primary extraskeletal Ewing's sarcoma and PNET arising from breast. We present an extremely rare case of an inoperable primary Ewing's sarcoma arising from left breast with contralateral breast, lymphatic and lung metastasis.

  20. Systematic analysis of a xenograft mice model for KSHV+ primary effusion lymphoma (PEL.

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    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.

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

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    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.

  2. Giant primary synovial sarcoma of the anterior mediastinum: A case ...

    African Journals Online (AJOL)

    Primary synovial sarcoma is a very rare tumor of the mediastinum, which is unreported in the entire subcontinent of West Africa, and presents daunting challenges from diagnosis to management with lack of standard management strategies. We present a case of primary monophasic synovial sarcoma of the anterior ...

  3. Radiological Findings of Primary Retroperitoneal Ewing Sarcoma

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    Ulusan, S.; Koc, Z.; Tuba Canpolat, E.; Colakoglu, T. [Depts. of Radiology, Pathology, and General Surgery, Baskent Univ. Faculty of Medicine, Adana (Turkey)

    2007-09-15

    Ewing sarcomas are most commonly located in bone, while extra skeletal involvement of the retroperitoneum is extremely rare. We describe the radiologic and pathological findings in an adult patient with retroperitoneal extra skeletal Ewing sarcoma. Keywords: Color Doppler ultrasound; computed tomography; extra skeletal Ewing sarcoma; magnetic resonance imaging; ultrasound.

  4. Radiological Findings of Primary Retroperitoneal Ewing Sarcoma

    International Nuclear Information System (INIS)

    Ulusan, S.; Koc, Z.; Tuba Canpolat, E.; Colakoglu, T.

    2007-01-01

    Ewing sarcomas are most commonly located in bone, while extra skeletal involvement of the retroperitoneum is extremely rare. We describe the radiologic and pathological findings in an adult patient with retroperitoneal extra skeletal Ewing sarcoma. Keywords: Color Doppler ultrasound; computed tomography; extra skeletal Ewing sarcoma; magnetic resonance imaging; ultrasound

  5. Primary Kaposi sarcoma of the subcutaneous tissue

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    Dezube Bruce J

    2008-09-01

    Full Text Available Abstract Background Involvement of the subcutis by Kaposi sarcoma (KS occurs primarily when cutaneous KS lesions evolve into deep penetrating nodular tumors. Primary KS of the subcutaneous tissue is an exceptional manifestation of this low-grade vascular neoplasm. Case presentation We present a unique case of acquired immune deficiency syndrome (AIDS-associated KS manifesting primarily in the subcutaneous tissue of the anterior thigh in a 43-year-old male, which occurred without overlying visible skin changes or concomitant KS disease elsewhere. Radiological imaging and tissue biopsy confirmed the diagnosis of KS. Conclusion This is the first documented case of primary subcutaneous KS occurring in the setting of AIDS. The differential diagnosis of an isolated subcutaneous lesion in an human immunodeficiency virus (HIV-infected individual is broad, and requires both imaging and a histopathological diagnosis to guide appropriate therapy.

  6. Primary extra-skeletal Ewing's sarcoma mimicking a disc protrusion.

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    Ruelle, A; Boccardo, M

    1987-07-01

    One of the rarest cases of primary epidural neoplasm is a soft tissue sarcoma histologically similar to Ewing's sarcoma of the bone. In the literature only eleven cases of such an extra-skeletal Ewing's sarcoma have been described. The authors report an additional case presenting as a disc protrusion in a young male. The authors include some diagnostic, prognostic and nosologic remarks about this condition.

  7. Primary Intestinal Lymphangiectasia Manifested as Unusual Edemas and Effusions

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    Wang, Xuefeng; Jin, Hong; Wu, Weilu

    2016-01-01

    Abstract Primary intestinal lymphangiectasia (PIL) is a rare disorder of unknown etiology characterized by diffuse or localized dilation and eventual rupture of the enteric lymphatic vessels in mucosa, submucosa, and/or subserosa. Lymph, rich in all kinds of proteins and lymphocytes, leaks into the gastrointestinal tract via the affected lymphatic vessels causing hypoproteinemia and lymphopenia. The main symptom is variable degrees of pitting edemas of bilateral lower limbs. But edemas of any other parts of body, and mild serous effusions may also occur sometimes. PIL occurs in conjunction with a right hemifacial edema, a right upper limb lymphedema, asymmetric bilateral calves edemas, and a unilateral massive pleural effusion seems never to be reported before. In addition, increased enteric protein loss that may cause severe hypoproteinemia usually get overlooked, and the lymphatic system disorders always put the diagnoses in a dilemma. We described a case of a 17-year-old Chinese girl with a history of gradually progressive swellings of right-sided face, right upper limb, and bilateral calves since 3 to 4 months of age. A right-sided massive pleural effusion, a moderate pericardial effusion, and a mild ascites have been proved unchanged by a series of computerized tomography (CT) scans since 5 years ago. The diagnosis of PIL was finally confirmed by severe hypoproteinemia, endoscopic changes, and histology of jejunum biopsy. Further lymphoscintigraphy and lymphangiography also identified lymph leakage in her bowel and several abnormal lymphatic vessels. A high-protein, low-fat diet supplemented with medium-chain triglycerides (MCT) showed some benefit. This case suggested that PIL was a rare but important etiology of hypoproteinemia, effusions, and edemas. PIL, effusions, and lymphedema can be the features of multisegmental generalized lymphatic dysplasia. In addition, both lymphoscintigraphy and intranodal lymphangiography could be considered when lymphatic

  8. Newly diagnosed primary hypothyroidism applicant with massive pericardial effusion and acute renal failure

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    Ates I

    2016-01-01

    Full Text Available Objective. While non-symptomatic pericardial effusion is seen in primary hypothyroidism, massive pericardial effusion is a very rare finding. In the literature, newly diagnosed primary hypothyroidism cases presenting with massive pericardial effusion or acute renal failure are present, but we did not encounter any case first presenting with combination of two signs. In this case report, primary hypothyroidism case that presenting with massive pericardial effusion and acute renal failure will be discussed.

  9. Primary Adult Renal Ewing's Sarcoma: A Rare Entity

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    Ravindra Mukkunda; Ramachandran Venkitaraman; Khin Thway; Toon Min; Cyril Fisher; Alan Horwich; Ian Judson

    2009-01-01

    Background. Ewing's sarcoma of extraskeletal origin is uncommon and that is of primary renal origin in adults are rare. There is no consensus on the optimal management of Ewing's tumors of renal origin. Methods. A retrospective review of the clinical features, treatment, and outcome of adult patients with primary renal extra-skeletal Ewing's sarcoma who were treated at the Royal Marsden hospital from January 1993–December 2007 is reported. Results. Seven adult patien...

  10. Sarcomas cutâneos primários Primary cutaneous sarcomas

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    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. Hippuristanol Reduces the Viability of Primary Effusion Lymphoma Cells both in Vitro and in Vivo

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    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.

  12. Primary Intestinal Lymphangiectasia (Waldmann's Disease) Presenting with Chylous Effusions in a 15-Year-Old.

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    Surampalli, Vijay; Ramaswamy, Srinath; Surendran, Deepanjali; Bammigatti, Chanaveerappa; Swaminathan, Rathinam Palamalai

    2017-08-01

    Primary Intestinal Lymphangiectasia (PIL) is a rare disease of unknown aetiology which presents in the paediatric age group with anasarca, diarrhoea, hypoproteinaemia, lymphoedema and chylous effusions. Tuberculosis, filariasis, chest trauma, malignancies and haematological disorders usually contribute to most cases of secondary lymphangiectasia and chylous effusions. We hereby describe a case of PIL presenting with chylous effusions which remained undiagnosed for eight years.

  13. Primary Synovial Sarcoma of External Auditory Canal: A Case Report.

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    Devi, Aarani; Jayakumar, Krishnannair L L

    2017-07-20

    Synovial sarcoma is a rare malignant tumor of mesenchymal origin. Primary synovial sarcoma of the ear is extremely rare and to date only two cases have been published in English medical literature. Though the tumor is reported to have an aggressive nature, early diagnosis and treatment may improve the outcome. Here, we report a rare case of synovial sarcoma of the external auditory canal in an 18-year-old male who was managed by chemotherapy and referred for palliation due to tumor progression.

  14. Primary Cystic Pleuropulmonary Synovial Sarcoma Presenting as Recurrent Pneumothorax

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    Eric D. Johnson

    2017-07-01

    Full Text Available Primary pleuropulmonary synovial sarcomas are quite rare, representing 0.1–0.5% of all pulmonary malignancies. We report an entirely cystic monophasic synovial sarcoma in a 25-year-old male who presented with recurrent pneumothorax and no evidence of a mass lesion on imaging. The purpose of this case report is to increase awareness of neoplasms clinically presenting as a pneumothorax with no imagining evidence of a mass-forming lesion and emphasize the significance of fluorescent in situ hybridization testing in nontypical synovial sarcoma cases.

  15. Primary Occipital Ewing’s Sarcoma with Subsequent Spinal Seeding

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    Ali Alqahtani

    2017-01-01

    Full Text Available Ewing’s sarcoma is a primary bone cancer that mainly affects the long bones. This malignancy is particularly common in pediatric patients. Primary cranial involvement accounts for 1% of cases, with occipital involvement considered extremely rare. In this case study, primary occipital Ewing’s sarcoma with a posterior fossa mass and subsequent relapse resulting in spinal seeding is reported. A 3-year-old patient presented with a 1-year history of left-sided headaches, localized over the occipital bone with progressive torticollis. Computed tomography (CT imaging showed a mass in the left posterior fossa compressing the brainstem. The patient then underwent surgical excision followed by adjuvant chemoradiation therapy. Two years later, the patient presented with severe lower back pain and urinary incontinence. Whole-spine magnetic resonance imaging (MRI showed cerebrospinal fluid (CSF seeding from the L5 to the S4 vertebrae. Primary cranial Ewing’s sarcoma is considered in the differential diagnosis of children with extra-axial posterior fossa mass associated with destructive permeative bone lesions. Although primary cranial Ewing’s sarcoma typically has good prognosis, our patient developed metastasis in the lower spine. Therefore, with CNS Ewing’s sarcoma, screening of the entire neural axis should be taken into consideration for early detection of CSF seeding metastasis in order to decrease the associated morbidity and mortality.

  16. Primary Occipital Ewing's Sarcoma with Subsequent Spinal Seeding.

    Science.gov (United States)

    Alqahtani, Ali; Amer, Roaa; Bakhsh, Eman

    2017-01-01

    Ewing's sarcoma is a primary bone cancer that mainly affects the long bones. This malignancy is particularly common in pediatric patients. Primary cranial involvement accounts for 1% of cases, with occipital involvement considered extremely rare. In this case study, primary occipital Ewing's sarcoma with a posterior fossa mass and subsequent relapse resulting in spinal seeding is reported. A 3-year-old patient presented with a 1-year history of left-sided headaches, localized over the occipital bone with progressive torticollis. Computed tomography (CT) imaging showed a mass in the left posterior fossa compressing the brainstem. The patient then underwent surgical excision followed by adjuvant chemoradiation therapy. Two years later, the patient presented with severe lower back pain and urinary incontinence. Whole-spine magnetic resonance imaging (MRI) showed cerebrospinal fluid (CSF) seeding from the L5 to the S4 vertebrae. Primary cranial Ewing's sarcoma is considered in the differential diagnosis of children with extra-axial posterior fossa mass associated with destructive permeative bone lesions. Although primary cranial Ewing's sarcoma typically has good prognosis, our patient developed metastasis in the lower spine. Therefore, with CNS Ewing's sarcoma, screening of the entire neural axis should be taken into consideration for early detection of CSF seeding metastasis in order to decrease the associated morbidity and mortality.

  17. Primary Renal Synovial Sarcoma: A Rare Case Report

    Directory of Open Access Journals (Sweden)

    Taha Numan Yıkılmaz

    2016-12-01

    Full Text Available Synovial sarcoma (SS is mainly derived from soft tissues. Primary renal SS is a very rare malignancy with around 60 cases reported in the literature. We report a renal mass which was undistinguishable from urothelial carcinoma clinically and pathologically but diagnosed as a primary renal SS at the definitive pathological diagnosis.

  18. Primary clear cell sarcoma of bone: a unique site of origin

    International Nuclear Information System (INIS)

    Gelczer, R.K.; Wenger, D.E.; Wold, L.E.

    1999-01-01

    Clear cell sarcoma is a rare soft tissue neoplasm, accounting for less than 1% of soft tissue sarcomas. We are presenting a case of a clear cell sarcoma of bone which, to our knowledge, is the only report of a primary clear cell sarcoma of bone. (orig.)

  19. Primary Adult Renal Ewing's Sarcoma: A Rare Entity

    Science.gov (United States)

    Mukkunda, Ravindra; Venkitaraman, Ramachandran; Thway, Khin; Min, Toon; Fisher, Cyril; Horwich, Alan; Judson, Ian

    2009-01-01

    Background. Ewing's sarcoma of extraskeletal origin is uncommon and that is of primary renal origin in adults are rare. There is no consensus on the optimal management of Ewing's tumors of renal origin. Methods. A retrospective review of the clinical features, treatment, and outcome of adult patients with primary renal extra-skeletal Ewing's sarcoma who were treated at the Royal Marsden hospital from January 1993–December 2007 is reported. Results. Seven adult patients with primary renal Ewing's sarcoma were identified. All four patients with nonmetastatic disease had radical nephrectomy and received adjuvant chemotherapy +/− radiotherapy. Two developed metastatic disease while on adjuvant chemotherapy, and one patient relapsed after 55 months. The three patients with metastatic disease at presentation did not have nephrectomy and were treated with chemotherapy. All three patients had disease progression with a dismal outcome. Only one patient in the whole group is alive and disease free. The median overall survival was 62.8 months, and the median disease-free survival in patients with nonmetastatic disease after combined modality treatment was 30.3 months. Conclusion. Primary adult renal Ewing's sarcoma is an aggressive tumor with a propensity for early metastasis. Radical nephrectomy with adjuvant combination chemotherapy produced the best results but the outlook remained poor with only one patient experiencing long disease-free survival. PMID:19478963

  20. Primary Adult Renal Ewing's Sarcoma: A Rare Entity

    Directory of Open Access Journals (Sweden)

    Ravindra Mukkunda

    2009-01-01

    Full Text Available Background. Ewing's sarcoma of extraskeletal origin is uncommon and that is of primary renal origin in adults are rare. There is no consensus on the optimal management of Ewing's tumors of renal origin. Methods. A retrospective review of the clinical features, treatment, and outcome of adult patients with primary renal extra-skeletal Ewing's sarcoma who were treated at the Royal Marsden hospital from January 1993–December 2007 is reported. Results. Seven adult patients with primary renal Ewing's sarcoma were identified. All four patients with nonmetastatic disease had radical nephrectomy and received adjuvant chemotherapy +/− radiotherapy. Two developed metastatic disease while on adjuvant chemotherapy, and one patient relapsed after 55 months. The three patients with metastatic disease at presentation did not have nephrectomy and were treated with chemotherapy. All three patients had disease progression with a dismal outcome. Only one patient in the whole group is alive and disease free. The median overall survival was 62.8 months, and the median disease-free survival in patients with nonmetastatic disease after combined modality treatment was 30.3 months. Conclusion. Primary adult renal Ewing's sarcoma is an aggressive tumor with a propensity for early metastasis. Radical nephrectomy with adjuvant combination chemotherapy produced the best results but the outlook remained poor with only one patient experiencing long disease-free survival.

  1. Primary Ewing's Sarcoma of the temporal bone in an infant.

    Science.gov (United States)

    Goudarzipour, Kourosh; Shamsian, Shahin; Alavi, Samin; Nourbakhsh, Kazem; Aghakhani, Roxana; Eydian, Zahra; Arzanian, Mohammad Taghi

    2015-04-01

    Introduction : Ewing's sarcoma is the second most common primary malignant tumor of bone found in children after Osteosarcoma. It accounts for 4-9% of primary malignant bone tumors and it affects bones of the skull or face in only 1-4% of cases. Hence it rarely affects the head and neck. Subject and Method : In this case report, we describe a case of primary Ewing's sarcoma occurring in the temporal bone. The tumor was surgically excised, and the patient underwent chemotherapy for ten months. Results : Neither recurrence nor distant metastasis was noted in these 10 months after surgery but about 18 months after surgery our patient was expired. Conclusion : Although the prognosis of Ewing's sarcoma is generally poor because of early metastasis to the lungs and to other bones, a review of the article suggested that Ewing's sarcoma occurring in the skull can often be successfully managed by intensive therapy with radical excision and chemotherapy. This result was supported by the case reported here.

  2. 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

  3. Cytogenetically confirmed primary Ewing's sarcoma of the pancreas.

    Science.gov (United States)

    Golhar, Ankush; Ray, Samrat; Haugk, Beate; Singhvi, Suresh Kumar

    2017-05-04

    Ewing's sarcoma is a highly aggressive malignant tumour most commonly affecting long bones in children and adolescents. It is part of the Ewing's sarcoma family of tumours (ESFTs) that also include peripheral primitive neuroectodermal tumour and Askin's tumours. ESFTs share common cytogenetic aberrations, antigenic profiles and proto-oncogene expression with an overall similar clinical course. In 99% of ESFTs, genetic translocation with molecular fusion involves the EWSR1 gene on 22q12. Approximately 30% of ESFTs are extraosseous, most commonly occurring in the soft tissues of extremities, pelvis, retroperitoneum and chest wall. Primary presentation in solid organs is very rare but has been described in multiple sites including the pancreas. Accurate diagnosis of a Ewing's sarcoma in a solid organ is critical in facilitating correct treatment. We report the case of a 17-year-old girl with cytogenetically confirmed primary pancreatic Ewing's sarcoma and provide a brief review of the published literature. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  4. Experiences with surgically treated primary or secondary hepatic sarcoma.

    Science.gov (United States)

    Fahrner, René; Dennler, Sandra G C; Dondorf, Felix; Ardelt, Michael; Rauchfuss, Falk; Settmacher, Utz

    2017-06-01

    Liver resection in hepatic sarcoma is rare, but other alternative treatment options are scarce. Surgery offers the only aggressive approach to achieve a tumour-free state. The aim of this investigation was to evaluate the outcome and survival of these patients at a single hepato-biliary university hospital. Between January 2004 and July 2013, 896 anatomical liver resections were performed. Eleven liver resections (1.2%) were performed due to primary hepatic sarcoma or hepatic sarcoma metastases. The demographic and clinical parameters were collected from the institutional patients' records. In eight patients (83%), liver resection was performed due to hepatic sarcoma metastases. The surgical procedures were as follows: two patients (18%) had segmentectomy, six patients (55%) had hemihepatectomy or extended hemihepatectomy and three patients (27%) had multivisceral resections. In nine patients (82%), the resection margins were tumour free. In 55% (n = 6) of the patients, the maximal tumour diameter was greater than 10 cm. The postoperative morbidity was low with a Clavien-Dindo score of 2 (range 0-5). One patient died on postoperative day 2 after multivisceral resection. During the follow-up of 932 days (range 2-2.220 days) the 1-, 2- and 3-year survival rates were 91, 63 and 45%, respectively. Tumour recurrence was detected in seven patients (63%). Liver resections in patients with primary or secondary hepatic sarcoma are rare. The main goal in these patients is to achieve complete tumour resection because chemotherapy offers no suitable alternative, but the long-term survival rates are limited because of high a recurrence rate even after aggressive surgical approaches.

  5. Primary synovial sarcoma of the abdominal wall: A case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Alsaif H Saif

    2008-01-01

    Full Text Available Synovial sarcoma is a malignant mesenchymal neoplasm which commonly occurs in the extremities of adults, in close association with joint capsules, tendon sheaths, bursae and fascial structures. Only a few cases of synovial sarcoma occurring in the abdominal wall have been reported. A case of a primary synovial sarcoma arising from the anterior abdominal wall fascial aponeurosis is presented.

  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. [Clinical, pathological and imaging features of primary pelvic Ewing's sarcoma].

    Science.gov (United States)

    Liu, J; Chen, Y; Ling, X L; Gong, Y; Ding, J P; Zhang, Z K; Wang, Y J

    2016-07-19

    To explore the clinical, pathological and imaging features of Ewing's sarcoma in pelvis and to improve knowledge and diagnosis of the disease. A retrospective analysis of the clinical, pathological and imaging data of pathologically confirmed 13 cases of Ewing's sarcoma in pelvis was carried out between May 2008 and March 2016 in the Affiliated Hospital of Hangzhou Normal University, the Third Hospital of Hebei Medical University and the Second Hospital of Hebei Medical University. The median age 13 cases of pelvic primary Ewing's sarcoma was 17 years old.The X-ray and CT imagings showed osteolytic and mixed bone destruction, CT showed mixed type in 10 cases, 8 cases of bone tumors as a flocculent, 10 cases of bone expansion failure, 10 cases of periosteal reaction, the layered 5 cases, radial in 5 cases.Thirteen cases showed soft tissue mass, soft tissue mass was equal or slightly lower density.Four cases showed heterogeneous contrast enhancement.The lesions showed low signal in T1WI and mixed high signal in T2WI of magnetic resonance imaging(MRI). The boundary of the lesions were obscure, and 5 cases had patchy necrosis area, and 9 cases had incomplete false capsule, surrounding soft tissue was violated.Four cases showed heterogeneous contrast enhancement after MRI enhancement scan. The age of onset of Ewing's sarcoma of the pelvis is more concentrated in about 15 years.The imaging feaures are mixed bone destruction and more bone is swelling and permeability damage, soft tissue mass is larger, bone tumor is cloudy or acicular, periosteal reaction in a layered and radial, most cases show that the false envelope is not complete.Combined with clinical and imaging examination, the diagnosis of the disease can be made.

  8. 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

  9. 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

  10. Breast Carcinoma Cells in Primary Tumors and Effusions Have Different Gene Array Profiles

    Directory of Open Access Journals (Sweden)

    Sophya Konstantinovsky

    2010-01-01

    Full Text Available The detection of breast carcinoma cells in effusions is associated with rapidly fatal outcome, but these cells are poorly characterized at the molecular level. This study compared the gene array signatures of breast carcinoma cells in primary carcinomas and effusions. The genetic signature of 10 primary tumors and 10 effusions was analyzed using the Array-Ready Oligo set for the Human Genome platform. Results for selected genes were validated using PCR, Western blotting, and immunohistochemistry. Array analysis identified 255 significantly downregulated and 96 upregulated genes in the effusion samples. The majority of differentially expressed genes were part of pathways involved in focal adhesion, extracellular matrix-cell interaction, and the regulation of the actin cytoskeleton. Genes that were upregulated in effusions included KRT8, BCAR1, CLDN4, VIL2, while DCN, CLDN19, ITGA7, and ITGA5 were downregulated at this anatomic site. PCR, Western blotting, and immunohistochemistry confirmed the array findings for BCAR1, CLDN4, VIL2, and DCN. Our data show that breast carcinoma cells in primary carcinomas and effusions have different gene expression signatures, and differentially express a large number of molecules related to adhesion, motility, and metastasis. These differences may have a critical role in designing therapy and in prognostication for patients with metastatic disease localized to the serosal cavities.

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

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    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.

  12. Primary Pulmonary Ewing’s Sarcoma: Rare Cause of Superior Vena Cava Syndrome in Children

    OpenAIRE

    Mehra, Shibani; Atwal, Swapndeep Singh; Garga, Umesh Chandra

    2014-01-01

    Ewing’s sarcoma is a common malignant bone tumour presenting in children and young adults. Rarely extra- skeletal soft tissues and visceral organs can also be the site of origin of Ewing’s sarcoma. Primary pulmonary Ewing’s sarcoma is an extremely rare malignancy which occurs in the paediatric population. We report an unusual case of primary pulmonary Ewing’s sarcoma in a nine year old girl who presented with features of superior vena cava syndrome in the emergency department. The diagnosis w...

  13. Primary Pulmonary Ewing's Sarcoma: Rare Cause of Superior Vena Cava Syndrome in Children.

    Science.gov (United States)

    Mehra, Shibani; Atwal, Swapndeep Singh; Garga, Umesh Chandra

    2014-08-01

    Ewing's sarcoma is a common malignant bone tumour presenting in children and young adults. Rarely extra- skeletal soft tissues and visceral organs can also be the site of origin of Ewing's sarcoma. Primary pulmonary Ewing's sarcoma is an extremely rare malignancy which occurs in the paediatric population. We report an unusual case of primary pulmonary Ewing's sarcoma in a nine year old girl who presented with features of superior vena cava syndrome in the emergency department. The diagnosis was confirmed pathologically both by light microscopy and immunohistochemistry. The patient was put on chemotherapy and surgery was planned but the patient expired within three days of starting chemotherapy.

  14. Primary pleomorphic sarcoma of the ovary with rhabdomyosarcomatous differentiation

    Directory of Open Access Journals (Sweden)

    Mukherjee Sumana

    2009-04-01

    Full Text Available A 49-year-old patient presented with a right ovarian mass, which, on microscopy, showed to consist of haphazardly oriented large pleomorphic cells with abundant cytoplasm. Periodic acid Schiff stain was positive but negative with diastase digestion. Immunohistochemical staining with Desmin showed intense cytoplasmic positivity in almost all the cells. Cytokeratin, epithelial membrane antigen, smooth muscle actin, HMB-45, S-100 and neurone-specific enolase were negative. Immunohistochemical staining with Myogenin showed intense nuclear positivity. There was no other primary tumor on extensive search. A diagnosis of primary sarcoma of the ovary with rhabdomyosarcomatous differentiation was made. The incidence of similar tumors of the ovary are low and therefore little data are available on this uniformly lethal tumor. Thus, such cases need to be reported to pool experience so that the tumor can be diagnosed early.

  15. Rapidly progressive course of primary renal synovial sarcoma: Case report

    Directory of Open Access Journals (Sweden)

    Marković-Lipkovski Jasmina

    2013-01-01

    Full Text Available Introduction. Primary kidney sarcoma, especially synovial sarcoma (SS, is a very rare neoplasm. Pre-operative signs and symptoms are very similar to renal cell carcinoma, therefore, the proper diagnosis is very difficult and usually made after nephrectomy. This is a case report of primary renal SS. Case Outline. A 38-year-old man presented with a history of fever and hematuria, and right flank pain 3 weeks ago. Abdominal computerized tomography revealed a heterogeneous well-marginated soft tissue mass arising in the lower part of the right kidney. Right nephrectomy was performed. A cystic tumor of 120x85 mm in size with soft solid growth, and with the extensive areas of hemorrhage and necrosis was seen on gross examination. Histopathology revealed a neoplasm composed of solid monomorphic sheets of spindle cells. Immunohistochemistry showed tumor cells strongly positive for BCL2, CD99, CD56 and vimentin, and focally positive for epithelial membrane antigen (EMA. The histological diagnosis of primary renal SS was based on morphology and immunohistochemistry. FISH analysis and RT-PCR was carried out on formalin-fixed paraffin-embedded tissue sections. The molecular analysis demonstrated translocation of SYT gene on chromosome 18 and SSX2 gene on chromosome X. The findings were consistent with diagnosis of SS. Conclusion. Our case shows that histopathological diagnosis of primary kidney SS, although difficult, is possible to be made on the basis of morphological and immunohistochemical analysis. However, this diagnosis should be corroborated by molecular techniques confirming SYT-SSX translocation on chromosome 18 and chromosome X. Here we present visceral monophasic SS with aggressive clinical course and poor outcome. [Projekat Ministarstva nauke Republike Srbije, br. OI 175047

  16. Impact of Insurance Coverage on Outcomes in Primary Breast Sarcoma

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    Julie L. Koenig

    2018-01-01

    Full Text Available Private insurance is associated with better outcomes in multiple common cancers. We hypothesized that insurance status would significantly impact outcomes in primary breast sarcoma (PBS due to the additional challenges of diagnosing and coordinating specialized care for a rare cancer. Using the National Cancer Database, we identified adult females diagnosed with PBS between 2004 and 2013. The influence of insurance status on overall survival (OS was evaluated using the Kaplan–Meier estimator with log-rank tests and Cox proportional hazard models. Among a cohort of 607 patients, 67 (11.0% had Medicaid, 217 (35.7% had Medicare, and 323 (53.2% had private insurance. Compared to privately insured patients, Medicaid patients were more likely to present with larger tumors and have their first surgical procedure further after diagnosis. Treatment was similar between patients with comparable disease stage. In multivariate analysis, Medicaid (hazard ratio (HR, 2.47; 95% confidence interval (CI, 1.62–3.77; p<0.001 and Medicare (HR, 1.68; 95% CI, 1.10–2.57; p=0.017 were independently associated with worse OS. Medicaid insurance coverage negatively impacted survival compared to private insurance more in breast sarcoma than in breast carcinoma (interaction p<0.001. In conclusion, patients with Medicaid insurance present with later stage disease and have worse overall survival than privately insured patients with PBS. Worse outcomes for Medicaid patients are exacerbated in this rare cancer.

  17. Primary Ewing's sarcoma of the vertebral column

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    Ilaslan, Hakan; Sundaram, Murali [Department of Radiology, Mayo Clinic, Ch2-290 200 First Street, SW, Rochester, 55905, MN (United States); Unni, K.Krishnan [Department of Laboratory Medicine and Pathology, Mayo Clinic, 200 First Street, SW, 55905, Rochester, MN (United States); Dekutoski, Mark B. [Department of Orthopedic Surgery, Mayo Clinic, 200 First Street, SW, 55905, Rochester, MN (United States)

    2004-09-01

    To determine the demographics, imaging findings, clinical symptoms, and prognosis of primary vertebral Ewing's sarcoma (PVES). A retrospective review of medical records and radiological studies of patients diagnosed with PVES from 1936 through 2001 in our institution and Department of Pathology consultation files was undertaken. Metastatic and soft tissue Ewing's sarcoma cases were excluded. From a total of 1,277 cases of Ewing's sarcoma, 125 (9.8%) had a primary vertebral origin. There were 48 females and 76 males. Patient ages ranged from 4 to 54 (mean 19.3, standard deviation 10.7, median 16) years. Vertebral column distribution was four cervical (3.2%), 13 thoracic (10.5%), 31 lumbar (25%), and 67 sacrum (53.2%). More than one vertebral segment was involved in ten cases (8%). Satisfactory imaging studies were available in 51 patients: 49 radiographs, 27 computerized tomography (CT), and 23 magnetic resonance imaging (MRI) studies. The majority of tumors were lytic (93%). Three cases were mixed lytic and sclerotic (6%) and one sclerotic. In the nonsacral spine, the majority of lesions (12/20) involved the posterior elements with extension into the vertebral body. Five cases were centered in the vertebral body with extension into the posterior elements. Two cases were limited to the posterior elements, and one case solely involved the vertebral body. Ala was the most frequently affected site in the sacrum (18/26). Spinal canal invasion was frequent (91%). Detailed clinical information was available in 53 patients. Duration of symptoms ranged from 1 to 30 (mean 7) months. Local pain was the first symptom and seen in all cases. Neurological deficits were present in 21 (40%) cases. All patients received radiation in various dosages; 70% additionally received chemotherapy. Twenty-five patients had surgery, and two patients received bone marrow transplantation. Forty-five patients had follow-up; the five-year disease-free survival probability is 0

  18. Bone scintigraphy in Ewing's sarcoma during and after treatment - prognostic information from the primary tumor site

    International Nuclear Information System (INIS)

    Piers, D.A.; Veenhoven, R.H.; Kamps, W.A.; Woldring, M.G.

    1988-01-01

    A bone scan can be negative in Ewing's sarcoma. The bone scan during and after treatment can give prognostic information on the primary tumor site: A persisting hot spot strongly suggests the presence of local malignancy, while a hot spot becomming negative points to local cure of Ewing's sarcoma. (orig.)

  19. 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

  20. Pericardial Effusion with Cardiac Tamponade as a Form of Presentation of Primary Hypothyroidism

    Directory of Open Access Journals (Sweden)

    Rachid Acir

    2002-01-01

    Full Text Available The authors describe a case of pericardial effusion accompanied by cardiac tamponade caused by primary hypothyroidism. Diagnosis was made by exclusion, because other causes of cardiac tamponade are more frequent. Emergency treatment of cardiac tamponade is pericardiocentesis (with possible pericardial window, and, after stabilization, performance of hormonal reposition therapy with L-thyroxin.

  1. Chylous pleural effusion associated with primary lymphedema and lymphangioma-like malformations

    NARCIS (Netherlands)

    Bresser, P.; Kromhout, J. G.; Reekers, J. A.; Verhage, T. L.

    1993-01-01

    We describe a patient with a chylous pleural effusion associated with primary lymphedema of his right leg and abdominal wall. On evaluation a generalized, severe hypoplasia of the lymphatic system turned out to be associated with hyperplastic, lymphangioma-like malformations

  2. Primary Sjögren's syndrome accompanied by pleural effusion: a case report and literature review.

    Science.gov (United States)

    Ma, Dedong; Lu, Hongxiu; Qu, Yiqing; Wang, Shanshan; Ying, Yangyang; Xiao, Wei

    2015-01-01

    Sjögren's syndrome (SS) is a systemic autoimmune disease characterized by the infiltration of lymphocytes in exocrine glands, specifically the salivary and lacrimal glands, resulting in the typical symptoms of xerophthalmia and xerostomia. SS may be accompanied by pleural effusion when the lung is involved, but this occurrence has been reported in only 10 cases in the literature. We report the case of a 42 year-old woman with severe bilateral pleural effusion for eight years. Primary Sjögren's Syndrome was finally diagnosed based on the presence of xerophthalmia and xerostomia, biopsy of the minor salivary glands, and positive anti-SS-A antibody in the serum and pleural effusion. Biopsy of the parietal pleura through video-assisted thoracoscopy revealed infiltration of lymphocytes. The patient had a long history of pleural effusion without clear etiology. Malignant disease was first suspected because of abnormal density lesion on the left lung and malignant cells found on cytology, but PET-CT revealed no malignant lesion. Examinations did not support infection, malignant tumor, pulmonary sarcoidosis, or other connective tissue diseases. This data could be useful for the future study of pleural effusion in SS.

  3. CT diagnosis of pleural dissemination without pleural effusion in primary lung cancer

    International Nuclear Information System (INIS)

    Murayama, Sadayuki; Murakami, Junji; Yoshimitsu, Kengo; Torii, Yoshikuni; Masuda, Kouji; Ishida, Teruyoshi.

    1996-01-01

    We retrospectively reviewed the CT scans of 25 primary lung cancers with disseminated pleural nodules or minimal malignant pleural effusion that were not recognized preparatively. Special attention was devoted to abutting interlobar fissures, thick major fissures, and disseminated nodules on the chest wall, the diaphragm, and in the interlobar fissures. Among 10 primary tumors abutting interlobar fissures, nine (90%) had at least one of these findings. Among 15 primary lung tumors which did not abut interlobar fissures, four (27%) had at least one of these findings. We conclude that CT is a useful modality for detecting the pleural dissemination of primary lung cancers when primary lung cancers abut interlobar fissures even if no pleural effusion is detectable on CT. (author)

  4. Optimal management of primary retroperitoneal sarcoma: an update.

    Science.gov (United States)

    Miah, Aisha B; Hannay, Jonathan; Benson, Charlotte; Thway, Khin; Messiou, Christina; Hayes, Andrew J; Strauss, Dirk C

    2014-05-01

    Soft tissue sarcomas are a group of heterogeneous neoplasms with more than 50 histological subtypes exhibiting major differences in terms of pathogenesis, genetic alterations and clinical behavior. Sarcomas represent approximately 1% of malignancies with retroperitoneal sarcomas representing 10-15% of all soft tissue sarcomas. Surgery is currently the only modality which offers the chance of cure. Surgery for retroperitoneal sarcomas presents specific challenges due their location in a complex space surrounded by vital structures and visceral organs often prohibiting resection with wide margins. Furthermore, even after complete resection local recurrence is common and the leading cause of death. In this article the authors describe the initial investigations, prognostic factors and optimal surgical management. The evidence and current research as regards the role of multimodality treatment is reviewed and discussed.

  5. Unusual Presentation of a Primary Ewing's Sarcoma of the Spine with Paraplegia: A Case Report.

    Science.gov (United States)

    Kannan, Karthik Kailash; Sundarapandian, Rajkumar Jayachandran; Surulivel, Vignesh Jayabalan

    2015-03-01

    Ewing's sarcoma is a primary malignancy of the bone affecting individuals in the second decade of life. Primary sarcomas of the spine are rare and the occurrence of Primary Ewing's sarcoma in the spine is very rare. Ewing's sarcoma occurring in the spine is divided into two types, Ewing's sarcoma of sacral spine which are very aggressive with poor prognosis and Ewing's sarcoma of the non sacral spine which is an extremely rare occurrence. Patient may present with neurological deficit when the tumour extends into the spinal canal causing spinal cord compression. Magnetic resonance imaging (MRI) is very sensitive in diagnosing the tumour and defining the extent of the tumour. Here we report an 18-year-old boy who presented with back pain and complete paraplegia of two months duration. The MRI gave a differential diagnosis of infective pathology due to the fluid collection in the paraspinal region, followed by primary malignancy as the second diagnosis. Patient underwent posterior spinal decompression and stabilization, and intaoperatively there was significant collection of pus whose culture showed no growth. The histopathology and immunohistochemistry studies confirmed the diagnosis of Ewing's sarcoma and patient was started on combination chemotherapy and radiotherapy.

  6. Primary Intestinal Lymphangiectasia Manifested as Unusual Edemas and Effusions: A Case Report.

    Science.gov (United States)

    Wang, Xuefeng; Jin, Hong; Wu, Weilu

    2016-03-01

    Primary intestinal lymphangiectasia (PIL) is a rare disorder of unknown etiology characterized by diffuse or localized dilation and eventual rupture of the enteric lymphatic vessels in mucosa, submucosa, and/or subserosa. Lymph, rich in all kinds of proteins and lymphocytes, leaks into the gastrointestinal tract via the affected lymphatic vessels causing hypoproteinemia and lymphopenia. The main symptom is variable degrees of pitting edemas of bilateral lower limbs. But edemas of any other parts of body, and mild serous effusions may also occur sometimes. PIL occurs in conjunction with a right hemifacial edema, a right upper limb lymphedema, asymmetric bilateral calves edemas, and a unilateral massive pleural effusion seems never to be reported before. In addition, increased enteric protein loss that may cause severe hypoproteinemia usually get overlooked, and the lymphatic system disorders always put the diagnoses in a dilemma.We described a case of a 17-year-old Chinese girl with a history of gradually progressive swellings of right-sided face, right upper limb, and bilateral calves since 3 to 4 months of age. A right-sided massive pleural effusion, a moderate pericardial effusion, and a mild ascites have been proved unchanged by a series of computerized tomography (CT) scans since 5 years ago. The diagnosis of PIL was finally confirmed by severe hypoproteinemia, endoscopic changes, and histology of jejunum biopsy. Further lymphoscintigraphy and lymphangiography also identified lymph leakage in her bowel and several abnormal lymphatic vessels. A high-protein, low-fat diet supplemented with medium-chain triglycerides (MCT) showed some benefit.This case suggested that PIL was a rare but important etiology of hypoproteinemia, effusions, and edemas. PIL, effusions, and lymphedema can be the features of multisegmental generalized lymphatic dysplasia. In addition, both lymphoscintigraphy and intranodal lymphangiography could be considered when lymphatic system

  7. Oxidative stress and antioxidant status in primary bone and soft tissue sarcoma

    International Nuclear Information System (INIS)

    Nathan, Fatima M; Singh, Vivek A; Dhanoa, Amreeta; Palanisamy, Uma D

    2011-01-01

    Oxidative stress is characterised by an increased level of reactive oxygen species (ROS) that disrupts the intracellular reduction-oxidation (redox) balance and has been implicated in various diseases including cancer. Malignant tumors of connective tissue or sarcomas account for approximately 1% of all cancer diagnoses in adults and around 15% of paediatric malignancies per annum. There exists no information on the alterations of oxidant/antioxidant status of sarcoma patients in literature. This study was aimed to determine the levels of oxidative stress and antioxidant defence in patients with primary bone and soft tissue sarcoma and to investigate if there exists any significant differences in these levels between both the sarcomas. The study cohort consisted of 94 subjects; 20 soft tissue sarcoma, 27 primary bone sarcoma and 47 healthy controls. Malondialdehyde (MDA) and protein carbonyls were determined to assess their oxidative stress levels while antioxidant status was evaluated using catalase (CAT), superoxide dismutase (SOD), thiols and trolox equivalent antioxidant capacity (TEAC). Sarcoma patients showed significant increase in plasma and urinary MDA and serum protein carbonyl levels (p < 0.05) while significant decreases were noted in TEAC, thiols, CAT and SOD levels (p < 0.05). No significant difference in oxidative damage was noted between both the sarcomas (p > 0.05). In conclusion, an increase in oxidative stress and decrease in antioxidant status is observed in both primary bone and soft tissue sarcomas with a similar extent of damage. This study offers the basis for further work on whether the manipulation of redox balance in patients with sarcoma represents a useful approach in the design of future therapies for bone disease

  8. Infection or Glioma? The False Dilemma of Primary Central Nervous System Histiocytic Sarcoma.

    Science.gov (United States)

    Clifton, William; Akinduro, Oluwaseun Oluwadara; Lopez-Chiriboga, Sebastian; Whitaker, Dale Alan; Reimer, Ronald

    2017-10-01

    Primary central nervous system (CNS) histiocytic sarcoma is an extremely rare lymphoproliferative disorder that affects the CNS and behaves aggressively. Only 27 cases of primary CNS histiocytic sarcoma have been reported. The paucity of literature on this entity has made diagnosis and treatment difficult both for the surgeon and the pathologist. In this case of primary CNS histiocytic sarcoma, a middle-aged woman presented from an outside institution with a supposed cerebellar abscess. Intraoperative frozen pathology was initially interpreted as high-grade glioma; however, final pathology demonstrated histiocytic sarcoma. This report makes a significant contribution to the literature on this rare malignant disease by outlining a similar presentation among several cases and providing a thorough overview of existing criteria for diagnosis and management. Copyright © 2017 Elsevier Inc. All rights reserved.

  9. Primary Ewing's sarcoma of the skull: radical resection and immediate cranioplasty after chemotherapy. A technical note.

    Science.gov (United States)

    Castle, Maria; Rivero, Mónica; Marquez, Javier

    2013-02-01

    The current standard treatment of Ewing's sarcoma is chemotherapy followed by surgery, making an immediate cranial reconstruction in a one-step surgical procedure possible. We describe the technique used to repair a cranial defect after the resection of a primary Ewing's sarcoma of the skull in a one-step surgical procedure. Bone repair with a custom-made cranioplasty immediately after resection of a primary Ewing's sarcoma of the skull avoids deformities and late complications associated with reconstructive surgery after radiotherapy and not interfere with radiotherapy and neither with follow-up. A one-step surgical procedure after chemotherapy for primary Ewing's sarcoma of the skull could be safer, less aggressive and more radical; avoiding deformities and late complications.

  10. Pericardial Effusion due to Primary Malignant Pericardial Mesothelioma: A Common Finding but an Uncommon Cause

    Directory of Open Access Journals (Sweden)

    Valery Istomin

    2016-01-01

    Full Text Available This case report describes a 37-year-old female who was admitted to our Emergency Department because of shortness of breath. On physical examination, she had dyspnea and tachycardia and blood pressure was 80/50 mmHg with a pulsus paradoxus of 22 mmHg. Neck veins were distended, heart sounds were distant, and dullness was found on both lung bases. Her chest X-ray revealed bilateral pleural effusion and cardiomegaly. On both computed tomography and echocardiography the heart was of normal size and a large pericardial effusion was noted. The echocardiogram showed signs of impending tamponade, so the patient underwent an emergent pericardiocentesis. No infectious etiology was found and she was assumed to have viral pericarditis and was treated accordingly. However, when the pericardial effusion recurred and empirical therapy for tuberculosis failed, a pericardial window was performed. A typical staining pattern for mesothelioma was found on her pericardial biopsy specimen. Since no other mesodermal tissue was affected, a diagnosis of primary malignant pericardial mesothelioma was made. Chemotherapy was not effective and she passed away a year after the diagnosis was made. This case highlights the difficulties in diagnosing this uncommon disease in patients that present with the common finding of pericardial effusion.

  11. [Clinical analysis of prenatal diagnosis and intervention for primary pleural effusion of 13 cases].

    Science.gov (United States)

    Wang, X Q; Li, W J; Yan, R L; Xiang, J W; Liu, M Y

    2018-02-25

    Objective: To optimize the clinical managements of primary fetal hydrothorax (PFHT) fetus by comparing the perinatal survival rate of different prenatal treatments. Methods: Totally 13 fetuses diagnosed with PFHT from July 2009 to December 2015 in the First Affiliated Hospital of Jinan University were collected and received prenatal expectant treatment, thoracocentesis (TC), and thoraco-amniotic shunting (TAS), respectively. The perinatal survival rate was compared among the three treatments. Results: Among 13 fetuses of PFHT, pleural effusion was absorbed or remained stable in 2(2/13) cases, and progressed in 11(11/13) cases. Six cases received expectant treatment (2 cases had termination of pregnancy due to progressing effusion, 2 cases had term delivery, and 2 cases had intrauterine death); the perinatal survival rate was 2/6. Six cases received TC (2 cases had term delivery, 2 cases had preterm delivery, and 2 cases had termination of pregnancy due to progressing effusion), the perinatal survival rate was 4/6. One case received TC+TAS (term delivery), the perinatal survival rate was 1/1. The overall perinatal survival rate of prenatal intrauterine intervention was 5/7. Conclusions: The clinical process of PFHT is changeable, and the pleural effusion will progress with gestational age. Intrauterine interventions could improve the perinatal survival rate.

  12. Sarcoma pleomórfico primário do pulmão Primary pleomorphic sarcoma of the lung

    Directory of Open Access Journals (Sweden)

    Marcus da Matta Abreu

    2008-07-01

    Full Text Available A classificação recente da Organização Mundial da Saúde (OMS para os tumores pulmonares unificou o heterogéneo grupo do carcinoma de não pequenas células (CPNPC, que engloba os sarcomas e os tumores com componentes sarcomatosos, sob a denominação “carcinoma com elementos pleomórficos, sarcomatóides ou sarcomatosos”. Este grupo inclui diferentes entidades, como o carcinoma pleomórfico (CP, o carcinoma de células fusiformes (CCF, carcinoma de células gigantes (CCG, os carcinossarcomas (CS e o blastoma pulmonar (BP. De uma forma geral, estes tumores são raros e representam 0,1% a 0,4% de todas as neoplasias pulmonares. Ocorrem mais comummente em homens fumadores, com alta carga tabágica, e acomete-os em torno dos 60 anos e comummente tem evolução clínica muito agressiva. Os autores relatam o caso de um doente portador de sarcoma pleomórfico primário pulmonar e revêem a literatura sobre o assunto.The World Health Organization (WHO classification of lung cancers ranks the heterogeneous nonsmall cell lung cancer (N-SCLC group, encompassing sarcoma or sarcoma-containing tumours under one heading: “carcinomas with pleomorphic, sarcomatoid or sarcomatous elements”. This group contains entities such as pleomorphic carcinoma (PC, spindle cell carcinoma (SCC, giant cell carcinoma (GCC, carcinosarcoma (CS and pulmonary blastoma (PB. These tumors are rare overall, making up approx. 0.1-0.4% of all lung malignancies. They are more commonly found in males who are heavy smokers, diagnosed at the age of 60 on average and follow an aggressive clinical course. The authors describe the case of a male patient with primary pleomorphic lung sarcoma and also include a review of the literature.

  13. Combination of arsenic and interferon-α inhibits expression of KSHV latent transcripts and synergistically improves survival of mice with primary effusion lymphomas.

    Directory of Open Access Journals (Sweden)

    Hiba El Hajj

    Full Text Available BACKGROUND: Kaposi sarcoma-associated herpesvirus (KSHV is the etiologic agent of primary effusion lymphomas (PEL. PEL cell lines infected with KSHV, but negative for Epstein-Barr virus have a tumorigenic potential in non-obese diabetic/severe combined immunodeficient mice and result in efficient engraftment and formation of malignant ascites with notable abdominal distension, consistent with the clinical manifestations of PEL in humans. METHODOLOGY/PRINCIPAL FINDINGS: Using this preclinical mouse model, we demonstrate that the combination of arsenic trioxide and interferon-alpha (IFN inhibits proliferation, induces apoptosis and downregulates the latent viral transcripts LANA-1, v-FLIP and v-Cyc in PEL cells derived from malignant ascites. Furthermore, this combination decreases the peritoneal volume and synergistically increases survival of PEL mice. CONCLUSION/SIGNIFICANCE: These results provide a promising rationale for the therapeutic use of arsenic/IFN in PEL patients.

  14. Combination of arsenic and interferon-α inhibits expression of KSHV latent transcripts and synergistically improves survival of mice with primary effusion lymphomas.

    Science.gov (United States)

    El Hajj, Hiba; Ali, Jihane; Ghantous, Akram; Hodroj, Dana; Daher, Ahmad; Zibara, Kazem; Journo, Chloé; Otrock, Zaher; Zaatari, Ghazi; Mahieux, Renaud; El Sabban, Marwan; Bazarbachi, Ali; Abou Merhi, Raghida

    2013-01-01

    Kaposi sarcoma-associated herpesvirus (KSHV) is the etiologic agent of primary effusion lymphomas (PEL). PEL cell lines infected with KSHV, but negative for Epstein-Barr virus have a tumorigenic potential in non-obese diabetic/severe combined immunodeficient mice and result in efficient engraftment and formation of malignant ascites with notable abdominal distension, consistent with the clinical manifestations of PEL in humans. Using this preclinical mouse model, we demonstrate that the combination of arsenic trioxide and interferon-alpha (IFN) inhibits proliferation, induces apoptosis and downregulates the latent viral transcripts LANA-1, v-FLIP and v-Cyc in PEL cells derived from malignant ascites. Furthermore, this combination decreases the peritoneal volume and synergistically increases survival of PEL mice. These results provide a promising rationale for the therapeutic use of arsenic/IFN in PEL patients.

  15. Primary intimal sarcoma of the left atrium presenting with constitutional symptoms

    Science.gov (United States)

    Ferreira, António; Felgueiras, Paula; Silva, Augusta; Ribeiro, Carlos; Guerra, Diana; de Melo, Daniel Pereira; Manuel Lopes, José

    2017-01-01

    Abstract Intimal (spindle-cell) sarcomas are exceptionally rare and are highly aggressive cardiac tumors. The authors describe a case of a 43-year-old female, presenting with a 3-month history of constitutional symptoms with fever, night sweats, anorexia and weight loss, associated with productive cough and pleural effusion that was admitted with clinical suspicion of pulmonary tuberculosis. The patient developed sudden acute heart failure symptoms during hospitalization, leading to mechanical ventilation. Computed tomography scan with contrast showed a cardiac tumor filling the left atrium causing compression of pulmonary veins. Surgical resection was performed and histologic examination revealed an intimal sarcoma. Although commenced on adjuvant chemotherapy, local tumor recurrence occurred with pericardium invasion. The patient died within 4 months of initial diagnosis. This report aims to describe an unusual presentation of this rare disease entity, and to discuss its highly aggressive clinical course. PMID:28694971

  16. Primary soft tissue Ewing's sarcoma of the maxillary sinus in elderly patients: presentation, management and prognosis.

    Science.gov (United States)

    Dutta, M; Ghatak, S; Biswas, G; Sen, A

    2014-06-01

    Nonosseous or soft tissue Ewing's sarcoma is a rare form of Ewing's sarcoma/primitive neuroectodermal tumour that seldom affects the head and neck region. Involvement of the nose and paranasal sinuses is extremely uncommon, with only eight of such patients being reported to date, mostly affecting adolescents and young adults. To our knowledge, this study is the first comprehensive report of primary soft tissue Ewing's sarcoma involving the paranasal sinuses in an elderly patient who successfully completed treatment. We herein discuss the pathogenesis, management and factors affecting the prognosis of this rare group of tumours involving the nose and paranasal sinuses, in relation to the available literature.

  17. Primary unclassified sarcoma of the parotid gland: a case of diagnostic and therapeutic challenge.

    Science.gov (United States)

    Saravakos, Panagiotis; Hartwein, Joerg; Fayyazi, Afshin

    2017-05-01

    Malignant salivary gland sarcomas represent a clinically and histologically diagnostic challenge. Primary unclassified sarcomas of the parotid gland consist a rare salivary gland tumor. We report an unusual case of such a tumor, which occurred in the right parotid gland of a 54-year-old male and presented as an asymptomatic painless mass. The pathologoanatomical examination revealed a rhabdoid large-cell unclassified sarcoma. The patient was treated with superficial parotidectomy and adjuvant radiotherapy. No recurrence was noted in a 10-year follow-up period. Due to the rare occurrence of primary unclassified sarcomas, there is no evidence-based treatment of choice. An optimal approach is best planned in a multidisciplinary setting, taking into consideration the resectability of the tumor, individual patient characteristics, presence of local or distant metastatic activity, local infiltrative behavior and tumor stage. A close follow-up of the patient is strongly recommended.

  18. Primary undifferentiated sarcoma of the meninges: A case report and comprehensive review of the literature.

    Science.gov (United States)

    Wapshott, Taylor; Schammel, Christine M G; Schammel, David P; Rezeanu, Luminita; Lynn, Michael

    2018-05-21

    Sarcomas make up 1% of all cases of adult cancer, with 5-10% of those classified as undifferentiated pleomorphic sarcomas (UPS/PUS) and 0.1-4.3% primary intracranial sarcomas. Intracranial undifferentiated sarcoma is characterized by an earlier age of onset and generally poorer prognosis compared to extracranial undifferentiated sarcomas. Current therapies involve surgical excision with wide margins and radiotherapy, with minimal data available regarding the efficacy of chemotherapy. A 79-year-old man with a history of remote superficial bladder cancer presented with a large frontal scalp lesion. A biopsy was initially attempted by a dermatologist in the outpatient setting, but a follow-up CT scan revealed a skull-eroding, enhancing soft tissue lesion. Neurosurgical treatment revealed an undifferentiated sarcoma. The patient underwent adjuvant radiation therapy of 59.4 Gy fractionated over 45 days following surgery. Follow-up brain MRIs at 1-, 6-, 9-, 12-, 15-, 21-, and 27 months after surgery have not shown any indications of local recurrence or tumor metastasis. Despite the high propensity that undifferentiated sarcomas have for recurrence and metastasis and the patient's advanced age, this patient remains uniquely disease-free. We provide a description of an unusual case and comprehensive literature review of UPS to clarify the hallmarks of the disease, identify the difficulties in diagnosis, and provide a summary of therapies employed in the literature with their corresponding patient outcomes. Copyright © 2018 Elsevier Ltd. All rights reserved.

  19. Primary Intracranial Sarcoma Presenting as Chronic Subdural Fluid Collections in a Child.

    Science.gov (United States)

    Glenn, Chad A; Fung, Kar-Ming; Tullos, Hurtis J; McNall-Knapp, Rene Y; Gunda, Divya; Mapstone, Timothy B

    2016-02-01

    Chronic subdural hematoma in the pediatric population often results from trauma. Asymptomatic and benign-appearing subdural collections are generally managed conservatively without operative intervention. Primary intracranial sarcomas are uncommon entities. Diagnosis of sarcoma can be difficult because these lesions often manifest as apparent hematoma. Presented is the case of a primary intracranial mucoid spindle cell sarcoma that arose in a child with a history of benign-appearing bilateral subdural fluid collections in the setting of nonaccidental trauma. The patient was initially managed conservatively because her neurological examination result was normal and her subdural collections decreased in size on repeated imaging. The collections did not resolve completely. Years later, she exhibited weakness, seizure, and an increase in the size of her subdural fluid collection. Subdural drainage was attempted without significant effect. Cytologic assessment of fluid was negative for malignant cells. Magnetic resonance imaging revealed multiple enhancing masses along the subdural collection. The patient eventually underwent craniotomy in which a diagnosis of sarcoma was obtained. Pathological and radiographic findings as well as oncological management are reviewed. The authors also review the natural history and treatment of primary intracranial sarcoma in the pediatric population. Early contrasted magnetic resonance imaging should be obtained in patients with subdural fluid collections that appear asymmetric or do not resolve in the expected time course, despite having a normal neurologic examination result. Negative cytologic assessment does not exclude sarcoma diagnosis. Copyright © 2016 Elsevier Inc. All rights reserved.

  20. Primary Ewing's Sarcoma of the Temporal Bone: A Rare Case Report and Literature Review.

    Science.gov (United States)

    Gupta, Divya; Gulati, Achal; Purnima

    2017-09-01

    Ewing's sarcoma is a malignant, round cell tumor arising from the bones and primarily affecting children and adolescent, accounting for 3 % of all childhood malignancies. Although the long bones and the trunk are typically affected, rare cases of it involving isolated bones throughout the body have been reported. Involvement of the skull bones is rare, constituting 1-6 % of the total Ewing's sarcoma cases but those affecting the cranial bones are rarer still, constituting only 1 %. We describe an 8 months old infant having Ewing sarcoma, of the petrous and mastoid parts of temporal bone along with the occipital bone, whose clinical presentation mimicked mastoiditis with facial nerve palsy. We discuss the clinical and therapeutic course of an extensive primary Ewing sarcoma of the temporal bone, which was treated without performing surgery and review this entity's literature in detail.

  1. Primary gastric Kaposi's sarcoma presenting first with upper ...

    African Journals Online (AJOL)

    Kaposi's sarcoma (KS) is the most common tumour among human immunodeficiency syndrome (HIV) infected individuals, but its involvement of the gastrointestinal tract was reported long before the acquired immunodeficiency syndrome (AIDS) epidemic. Although most cases of gastrointestinal KS are asymptomatic, ...

  2. Primary extraskeletal Ewing's sarcoma/primitive neuroectodermal tumor of breast

    Directory of Open Access Journals (Sweden)

    Smita Srivastava

    2016-01-01

    Full Text Available Extraskeletal Ewing's sarcoma (EES is a rare soft tissue tumor that is morphologically indistinguishable from skeletal ES. We report a case of a 25-year-old female with recurrent EES/primitive neuroectodermal tumor of right breast with imaging findings on mammogram, ultrasound, magnetic resonance imaging breast, and positron emission tomography–computed tomography.

  3. Primary effusion lymphoma in an elderly patient effectively treated by lenalidomide: case report and review of literature

    International Nuclear Information System (INIS)

    Antar, A; El Hajj, H; Jabbour, M; Khalifeh, I; EL-Merhi, F; Mahfouz, R; Bazarbachi, A

    2014-01-01

    Primary effusion lymphoma (PEL) is a rare aggressive subset of non-Hodgkin B-cell lymphoma. It is caused by Kaposi sarcoma-associated herpesvirus/human herpesvirus type 8 (KSHV/HHV8). It occurs mainly, but not exclusively, in HIV-positive patients. PEL predominantly develops in serous cavities and occasionally in extracavitary regions. PEL carries a very poor prognosis with a median survival time of <6 months. Indeed, currently used treatment modalities such as CHOP chemotherapy are far from achieving complete and sustainable remission. Therefore, there is no clear standard of care established in the treatment of PEL patients, stressing the need for novel-targeted approaches. Here, we have attempted a comprehensive assessment of the treatment of PEL, discussed avant-garde therapies and updated the state of preclinical research with promising clinical applications in the field. These include inhibitors of viral replication, modulators of cell signaling and inflammation, nuclear factor kappa B (NF-κB) and histone deacetylase inhibitors, and recently the combination of arsenic trioxide and interferon-alpha. Some of these targeted therapies have not yet reached clinical studies, although others were used in a few individual case reports with low numbers of patients. We also describe the first case of a 77-year-old, HIV-negative, HHV8-positive patient diagnosed with PEL limited to the pleural and peritoneal cavities. He received lenalidomide 25 mg/day for 21 days every 28 days. Treatment was well tolerated with no side effects. He rapidly improved after 1 month of treatment and progressively achieved complete remission persistent after 18 months of therapy. We believe that this review will bridge an important gap between classical chemotherapy and modern approaches of targeted therapy. Finally, our findings warrant further evaluation of lenalidomide in future prospective clinical studies

  4. Genetic profiling differentiates second primary tumors from metastases in adult metachronous soft tissue sarcoma

    DEFF Research Database (Denmark)

    Fernebro, Josefin; Carneiro, Ana; Rydholm, Anders

    2008-01-01

    Purpose. Patients with soft tissue sarcomas (STS) are at increased risk of second primary malignancies, including a second STS, but distinction between metastases and a second primary STS is difficult. Patients and Methods. Array-based comparative genomic hybridization (aCGH) was applied to 30 mu...

  5. Primary pericranial Ewing's sarcoma on the temporal bone: A case report.

    Science.gov (United States)

    Kawano, Hiroto; Nitta, Naoki; Ishida, Mitsuaki; Fukami, Tadateru; Nozaki, Kazuhiko

    2016-01-01

    Primary Ewing's sarcoma originating in the pericranium is an extremely rare disease entity. A 9-year-old female patient was admitted to our department due to a left temporal subcutaneous mass. The mass was localized under the left temporal muscle and attached to the surface of the temporal bone. Head computed tomography revealed a mass with bony spicule formation on the temporal bone, however, it did not show bone destruction or intracranial invasion. F-18 fluorodeoxyglucose positron emission tomography showed no lesions other than the mass on the temporal bone. Magnetic resonance imaging showed that the mass was located between the temporal bone and the pericranium. The mass was completely resected with the underlying temporal bone and the overlying deep layer of temporal muscle, and was diagnosed as primary Ewing's sarcoma. Because the tumor was located in the subpericranium, we created a new classification, "pericranial Ewing's sarcoma," and diagnosed the present tumor as pericranial Ewing's sarcoma. We herein present an extremely rare case of primary pericranial Ewing's sarcoma that developed on the temporal bone.

  6. Kaposi's Sarcoma-Associated Herpesvirus | Center for Cancer Research

    Science.gov (United States)

    The discovery of KSHV in 1994 was a historical landmark in tumor virology and human cancer research. KSHV's subsequent identification as a cause of Kaposi sarcoma and its association with primary effusion lymphoma and multicentric Castleman disease soon attracted the attention of hundreds of research laboratories and motivated thousands of virologists and oncologists to switch

  7. Effective radiation therapy on two cases of primary Ewing's sarcoma of the rib

    International Nuclear Information System (INIS)

    Monzen, Yoshio; Nakanishi, Kazue; Ajimu, Akira; Ishida, Yutaka; Fujimoto, Toshifumi; Hayashi, Kuniaki; Hombo, Zenichiro; Amamoto, Yuhei.

    1987-01-01

    Two cases of primary Ewing's sarcoma of the rib are reported, in which radiation therapy was quite effective. Case 1 was an 18-year-old female who had had an operation and radiation therapy for Ewing's sarcoma of the left 7th rib. She was referred to our hospital after a recurrent tumor was found. Radiation therapy (tumor dose 46.2 Gy) and chemotherapy were given. The tumor disappeared and there has been no relapse for 1 year and 3 months after the treatment. Case 2 was a 2-year-old-infant. Radiation therapy (tumor dose 74 Gy) was given for primary Ewing's sarcoma of the left 6th rib. The tumor became small and was successfully removed at operation. There has been no relapse or distant metastasis for 8 months following the operation. We emphasize the importance of multidisciplinary treatment in case 1 and the usefulness of preoperative radiotherapy in case 2. (author)

  8. The value of local treatment in patients with primary, disseminated, multifocal Ewing sarcoma (PDMES)

    NARCIS (Netherlands)

    Haeusler, Julia; Ranft, Andreas; Boelling, Tobias; Gosheger, Georg; Braun-Munzinger, Gabriele; Vieth, Volker; Burdach, Stefan; van den Berg, Henk; Juergens, Heribert; Dirksen, Uta

    2010-01-01

    The value of local treatment in patients with primary, disseminated, multifocal Ewing sarcoma (PDMES) was investigated. We analyzed 120 patients registered into the European Ewing Tumor Working Initiative of National Groups (EURO-E.W.I.N.G. 99) trial at the trial center of Muenster from 1998 to

  9. Primary Disseminated Multifocal Ewing Sarcoma: Results of the Euro-EWING 99 Trial

    NARCIS (Netherlands)

    Ladenstein, Ruth; Pötschger, Ulrike; Le Deley, Marie Cécile; Whelan, Jeremy; Paulussen, Michael; Oberlin, Odile; van den Berg, Henk; Dirksen, Uta; Hjorth, Lars; Michon, Jean; Lewis, Ian; Craft, Alan; Jürgens, Heribert

    2010-01-01

    Purpose To improve the poor prognosis of patients with primary disseminated multifocal Ewing sarcomas (PDMES) with a dose-intense treatment concept. Patients and Methods From 1999 to 2005, 281 patients with PDMES were enrolled onto the Euro-EWING 99 R3 study. Median age was 16.2 years (range, 0.4 to

  10. CT findings of primary undifferentiated pleomorphic sarcoma in the small bowel: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Youe Ree; Lee, Young Hwan; Yoon, Kwon Ha; Yun, Ki Jung [Wonkwang University School of Medicine and Hospital, Institute of Wonkwang Medical Science, Iksan (Korea, Republic of)

    2015-11-15

    Undifferentiated pleomorphic sarcoma (UPS), previously known as malignant fibrous histiocytoma, is a soft tissue sarcoma arising from mesenchymal tissue of the body. UPS of the gastrointestinal tract is known to be rare and only a few cases have been reported in the literature. Based on our case and review of the other relevant literature, the CT findings of primary UPS of the small bowel included nodular bowel wall thickening with homogeneous enhancement. It presents as a rapidly growing tumor without bowel obstruction, and it may be accompanied by distant metastasis.

  11. MRI of primary meningeal sarcomas in two children: differential diagnostic considerations

    International Nuclear Information System (INIS)

    Pfluger, T.; Weil, S.; Weis, S.

    1997-01-01

    Meningeal sarcomas are very rare, highly aggressive tumours affecting children more frequently than adults. The clinical course and MRI of meningeal sarcomas in two cases are discussed with special regard to possible misinterpretation. In one case MRI demonstrated a circumscribed mass in contact with the meninges, with central areas of haemorrhage. In the other, a case of primary leptomeningeal sarcomatosis, several MRI examinations over the course of almost a year were unhelpful, despite severe neurological complaints. Then MRI revealed meningeal contrast enhancement all over the brain and spinal canal, together with cerebral infarcts. MRI of meningeal sarcomas has not been discussed in the literature. MRI did not permit specific diagnosis, but enabled visualisation of the extent of the tumour and/or meningeal involvement. Early histological diagnosis is indispensable for adequate treatment. (orig.)

  12. Lung Metastasis of Primary Alveolar Soft-Part Sarcoma Occurring 20 Years after Initial Treatment

    Directory of Open Access Journals (Sweden)

    R. F. Falkenstern-Ge

    2013-01-01

    Full Text Available A 30-year old woman was referred to our center because of suspicion of a primary lung tumor of the right upper lobe. Histological examination of the lung lesion revealed lung metastasis of a previously treated alveolar soft part sarcoma of the musculus vastus medialis of the right femur, which was resected 20 years ago. Alveolar soft-part sarcoma is a rare malignant tumor that occurs most often in the soft tissue of lower limbs. It is a slow-growing malignant soft tissue tumor arising in muscle tissue, usually in young adults. Due to pleural and extensive mediastinal infiltration with bilateral lung metastases, a systemic treatment with chemotherapy doxorubicin and ifosfamide was initiated. Late metastases from previously treated alveolar part sarcoma should be considered in patients with suspicious lung lesions even if surgical treatment was performed a long time ago.

  13. Prognostic factors for survival among patients with primary bone sarcomas of small bones

    Directory of Open Access Journals (Sweden)

    Wang Z

    2018-05-01

    Full Text Available Zhan Wang,1,* Shu Li,2,* Yong Li,1 Nong Lin,1 Xin Huang,1 Meng Liu,1 Weibo Pan,1 Xiaobo Yan,1 Lingling Sun,1 Hengyuan, Li,1 Binghao Li,1 Hao Qu,1 Yan Wu,1 Peng Lin,1 Zhaoming Ye1 1Department of Orthopaedics, The Second Affiliated Hospital of Zhejiang University School of Medicine, Centre for Orthopaedic Research, Orthopedics Research Institute of Zhejiang University, Hangzhou, China; 2Key Laboratory of Cancer Prevention and Intervention, Key Laboratory of Molecular Biology in Medical Sciences, National Ministry of Education, Department of Hematology, The Second Affiliated Hospital, Zhejiang University School of Medicine, Cancer Institute, Hangzhou, China *These authors contributed equally to this work Background: Primary bone sarcomas of the hands or feet are rare lesions and poorly documented. Moreover, the prognostic determinants of bone sarcomas of the hands or feet have not been reported. Materials and methods: The Surveillance, Epidemiology, and End Results (SEER program database was used to screen patients with bone sarcomas of the hands or feet from 1973 to 2013, with attention paid to chondrosarcoma, Ewing sarcoma, and osteosarcoma. The prognostic values of overall survival (OS and cancer-specific survival (CSS were assessed using Cox proportional hazards regression model with univariate and multivariate analyses. The Kaplan–Meier method was used to obtain OS and CSS curves. Results: A total of 457 cases were selected from the SEER database. Chondrosarcoma was the most common form of lesion in hands or feet or both, followed by Ewing sarcoma and osteosarcoma. The 5- and 10-year OS rates of the entire group were 75.7% and 66.1%, respectively. The 5- and 10-year CSS rates were 78.7% and 73.7%, respectively. Multivariate analysis revealed that age under 40 years, localized stage, low grade, surgical treatment, and first primary tumor were associated with improved OS, and decade of diagnosis, stage, grade, and surgery were independent

  14. Histology-based classification predicts pattern of recurrence and improves risk stratification in primary retroperitoneal sarcoma

    Science.gov (United States)

    Tan, Marcus C.B.; Brennan, Murray F.; Kuk, Deborah; Agaram, Narasimhan P.; Antonescu, Cristina; Qin, Li-Xuan; Moraco, Nicole; Crago, Aimee M.; Singer, Samuel

    2015-01-01

    Objective To determine the prognostic significance of histologic type/subtype in a large series of patients with primary resected retroperitoneal sarcoma. Summary Background Data The histologic diversity and rarity of retroperitoneal sarcoma has hampered the ability to predict patient outcome. Methods From a single-institution, prospective database, 675 patients treated surgically for primary, non-metastatic retroperitoneal sarcoma during 1982–2010 were identified and histologic type/subtype was reviewed. Clinicopathologic variables were analyzed for association with disease-specific death (DSD), local recurrence (LR), and distant recurrence (DR). Results Median follow-up for survivors was 7.5 years. The predominant histologies were well-differentiated liposarcoma, dedifferentiated liposarcoma, and leiomyosarcoma. Five-year cumulative incidence of DSD was 31%, and factors independently associated with DSD were R2 resection, resection of ≥3 contiguous organs, and histologic type. Five-year cumulative incidence for LR was 39% and for DR was 24%. R1 resection, age, tumor size, and histologic type were independently associated with LR; size, resection of ≥3 organs, and histologic type were independently associated with DR. Liposarcoma and leiomyosarcoma were associated with late recurrence and DSD (as long as 15 years from diagnosis). For solitary fibrous tumor, local recurrence was uncommon (sarcoma. Histology predicts the pattern and incidence of LR and DR and will aid in more accurate patient counseling and selection of patients for adjuvant therapy trials. PMID:25915910

  15. Primary pleuropulmonary synovial sarcoma with brain metastases in a paediatric patient: an unusual presentation.

    Science.gov (United States)

    Chirmade, Pushpak Chandrakant; Parikh, Sonia; Anand, Asha; Panchal, Harsha; Patel, Apurva; Shah, Sandip

    2017-01-01

    Primary lung neoplasms are rare in children. The most common primary lung malignancies in children are pleuropulmonary blastoma and carcinoid tumour. Synovial sarcoma (SS) accounts for approximately 1% of all childhood malignancies. In absolute terms, the SS of the lungs and pleura are extremely rare and pose a diagnostic difficulty. Soft tissue sarcomas usually have a high potential for metastases, however, metastasis to the brain is rare, even in widely disseminated disease, and it has been described only in 3 case reports previously. Primary pleuropulmonary SS with brain metastases is even rarer. Here we present a case of an 11-year-old boy who presented with respiratory complaints, viz. fever and cough for 20 days. Initial impression was lung abscess, however, on histopathological, immunohistochemical and molecular study, the disorder was diagnosed as synovial sarcoma. After a week from the first consult, the child developed neurological symptoms, viz., an episode of convulsion and gradually worsening power of the lower limb. Computed tomography scan and Magnetic Resonance Spectroscopy was suggestive of brain metastases. Given the rarity of primary lung neoplasms in children, clinical detection remains a challenge. Delayed diagnoses are common as respiratory symptoms may be attributed to inflammatory or infective processes. Primary pleuropulmonary synovial sarcoma is a rare tumour and it is not known to commonly metastasise to the brain. Though rare, primary pleuropulmonary SS should be considered an important differential among peadiatric primary lung neoplasms due to its potential for curability if detected early, and more aggressive metastatic pattern, e.g. brain metastases making early detection imperative.

  16. Imaging Primary Mouse Sarcomas After Radiation Therapy Using Cathepsin-Activatable Fluorescent Imaging Agents

    Energy Technology Data Exchange (ETDEWEB)

    Cuneo, Kyle C. [Department of Radiation Oncology, Duke University School of Medicine, Durham, North Carolina (United States); Mito, Jeffrey K.; Javid, Melodi P. [Department of Pharmacology and Cancer Biology, Duke University School of Medicine, Durham, North Carolina (United States); Ferrer, Jorge M. [Department of Chemistry, Massachusetts Institute of Technology, Cambridge, Massachusetts (United States); Kim, Yongbaek [Department of Clinical Pathology, College of Veterinary Medicine, Seoul National University, Seoul (Korea, Republic of); Lee, W. David [The David H. Koch Institute for Integrative Cancer Research, Massachusetts Institute of Technology, Cambridge, Massachusetts (United States); Bawendi, Moungi G. [Department of Chemistry, Massachusetts Institute of Technology, Cambridge, Massachusetts (United States); Brigman, Brian E. [Department of Orthopedic Surgery, Duke University School of Medicine, Durham, North Carolina (United States); Kirsch, David G., E-mail: david.kirsch@duke.edu [Department of Radiation Oncology, Duke University School of Medicine, Durham, North Carolina (United States); Department of Pharmacology and Cancer Biology, Duke University School of Medicine, Durham, North Carolina (United States)

    2013-05-01

    Purpose: Cathepsin-activated fluorescent probes can detect tumors in mice and in canine patients. We previously showed that these probes can detect microscopic residual sarcoma in the tumor bed of mice during gross total resection. Many patients with soft tissue sarcoma (STS) and other tumors undergo radiation therapy (RT) before surgery. This study assesses the effect of RT on the ability of cathepsin-activated probes to differentiate between normal and cancerous tissue. Methods and Materials: A genetically engineered mouse model of STS was used to generate primary hind limb sarcomas that were treated with hypofractionated RT. Mice were injected intravenously with cathepsin-activated fluorescent probes, and various tissues, including the tumor, were imaged using a hand-held imaging device. Resected tumor and normal muscle samples were harvested to assess cathepsin expression by Western blot. Uptake of activated probe was analyzed by flow cytometry and confocal microscopy. Parallel in vitro studies using mouse sarcoma cells were performed. Results: RT of primary STS in mice and mouse sarcoma cell lines caused no change in probe activation or cathepsin protease expression. Increasing radiation dose resulted in an upward trend in probe activation. Flow cytometry and immunofluorescence showed that a substantial proportion of probe-labeled cells were CD11b-positive tumor-associated immune cells. Conclusions: In this primary murine model of STS, RT did not affect the ability of cathepsin-activated probes to differentiate between tumor and normal muscle. Cathepsin-activated probes labeled tumor cells and tumor-associated macrophages. Our results suggest that it would be feasible to include patients who have received preoperative RT in clinical studies evaluating cathepsin-activated imaging probes.

  17. Pyrrolidinium fullerene induces apoptosis by activation of procaspase-9 via suppression of Akt in primary effusion lymphoma

    Energy Technology Data Exchange (ETDEWEB)

    Watanabe, Tadashi [Department of Cell Biology, Kyoto Pharmaceutical University, Misasagi-Shichonocho 1, Yamashinaku, Kyoto 607-8412 (Japan); Nakamura, Shigeo [Department of Chemistry, Nippon Medical School, 1-7-1 Kyonan-cho, Musashino, Tokyo 180-0023 (Japan); Ono, Toshiya; Ui, Sadaharu [Department of Biotechnology, Interdisciplinary Graduate School of Medicine and Engineering, University of Yamanashi, Kofu 400-8511 (Japan); Yagi, Syota; Kagawa, Hiroki [Department of Cell Biology, Kyoto Pharmaceutical University, Misasagi-Shichonocho 1, Yamashinaku, Kyoto 607-8412 (Japan); Watanabe, Hisami [Center of Molecular Biosciences, Tropical Biosphere Research Center, University of the Ryukyus, 1 Senbaru, Nishihara-cho, Okinawa 903-0213 (Japan); Ohe, Tomoyuki; Mashino, Tadahiko [Department of Pharmaceutical Sciences, Faculty of Pharmacy, Keio University, 1-5-30 Shibakoen, 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)

    2014-08-15

    Highlights: • Seven fullerenes were evaluated in terms of their cytotoxic effects on B-lymphomas. • Pyrrolidinium fullerene induced apoptosis of KSHV-infected B-lymphoma PEL cells. • The activation of Akt is essential for PEL cell survival. • Pyrrolidinium fullerene activated caspase-9 by inactivating Akt in PEL cells. • Pyrrolidinium fullerene have potential as novel drugs for the treatment of PEL. - Abstract: Primary effusion lymphoma (PEL) is a subtype of non-Hodgkin’s B-cell lymphoma and is an aggressive neoplasm caused by Kaposi’s sarcoma-associated herpesvirus (KSHV) in immunosuppressed patients. In general, PEL cells are derived from post-germinal center B-cells and are infected with KSHV. To evaluate potential novel anti-tumor compounds against KSHV-associated PEL, seven water-soluble fullerene derivatives were evaluated as potential drug candidates for the treatment of PEL. Herein, we discovered a pyrrolidinium fullerene derivative, 1,1,1′,1′-tetramethyl [60]fullerenodipyrrolidinium diiodide, which induced apoptosis of PEL cells via a novel mechanism, the caspase-9 activation by suppressing the caspase-9 phosphorylation, causing caspase-9 inactivation. Pyrrolidinium fullerene treatment reduced significantly the viability of PEL cells compared with KSHV-uninfected lymphoma cells, and induced the apoptosis of PEL cells by activating caspase-9 via procaspase-9 cleavage. Pyrrolidinium fullerene additionally reduced the Ser473 phosphorylation of Akt and Ser196 of procaspase-9. Ser473-phosphorylated Akt (i.e., activated Akt) phosphorylates Ser196 in procaspase-9, causing inactivation of procaspase-9. We also demonstrated that Akt inhibitors suppressed the proliferation of PEL cells compared with KSHV-uninfected cells. Our data therefore suggest that Akt activation is essential for cell survival in PEL and a pyrrolidinium fullerene derivative induced apoptosis by activating caspase-9 via suppression of Akt in PEL cells. In addition, we evaluated

  18. Pyrrolidinium fullerene induces apoptosis by activation of procaspase-9 via suppression of Akt in primary effusion lymphoma

    International Nuclear Information System (INIS)

    Watanabe, Tadashi; Nakamura, Shigeo; Ono, Toshiya; Ui, Sadaharu; Yagi, Syota; Kagawa, Hiroki; Watanabe, Hisami; Ohe, Tomoyuki; Mashino, Tadahiko; Fujimuro, Masahiro

    2014-01-01

    Highlights: • Seven fullerenes were evaluated in terms of their cytotoxic effects on B-lymphomas. • Pyrrolidinium fullerene induced apoptosis of KSHV-infected B-lymphoma PEL cells. • The activation of Akt is essential for PEL cell survival. • Pyrrolidinium fullerene activated caspase-9 by inactivating Akt in PEL cells. • Pyrrolidinium fullerene have potential as novel drugs for the treatment of PEL. - Abstract: Primary effusion lymphoma (PEL) is a subtype of non-Hodgkin’s B-cell lymphoma and is an aggressive neoplasm caused by Kaposi’s sarcoma-associated herpesvirus (KSHV) in immunosuppressed patients. In general, PEL cells are derived from post-germinal center B-cells and are infected with KSHV. To evaluate potential novel anti-tumor compounds against KSHV-associated PEL, seven water-soluble fullerene derivatives were evaluated as potential drug candidates for the treatment of PEL. Herein, we discovered a pyrrolidinium fullerene derivative, 1,1,1′,1′-tetramethyl [60]fullerenodipyrrolidinium diiodide, which induced apoptosis of PEL cells via a novel mechanism, the caspase-9 activation by suppressing the caspase-9 phosphorylation, causing caspase-9 inactivation. Pyrrolidinium fullerene treatment reduced significantly the viability of PEL cells compared with KSHV-uninfected lymphoma cells, and induced the apoptosis of PEL cells by activating caspase-9 via procaspase-9 cleavage. Pyrrolidinium fullerene additionally reduced the Ser473 phosphorylation of Akt and Ser196 of procaspase-9. Ser473-phosphorylated Akt (i.e., activated Akt) phosphorylates Ser196 in procaspase-9, causing inactivation of procaspase-9. We also demonstrated that Akt inhibitors suppressed the proliferation of PEL cells compared with KSHV-uninfected cells. Our data therefore suggest that Akt activation is essential for cell survival in PEL and a pyrrolidinium fullerene derivative induced apoptosis by activating caspase-9 via suppression of Akt in PEL cells. In addition, we evaluated

  19. Sarcoma de Kaposi primário do pênis Primary Kaposi's sarcoma of the penis

    Directory of Open Access Journals (Sweden)

    Maira Mitsue Mukai

    2009-10-01

    Full Text Available Sarcoma de Kaposi é um tumor vascular que afeta a parede dos vasos linfáticos. Possui quatro formas: clássica, endêmica, iatrogênica e associada ao HIV. É uma doença sistêmica, maligna, multifatorial e de curso variável. A apresentação inicial no pênis é rara, e mais observada em pacientes HIV positivos. Em pacientes HIV negativos, os casos que ocorrem nesta região, apresentam-se com pápulas, nódulos, placas e lesões verruciformes, assintomáticas. Para o tratamento da forma clássica, dispõem-se de excisão cirúrgica, crioterapia, eletrocirurgia, laser e radioterapia. Neste trabalho, é relatado um caso raro de um paciente com a forma clássica, em região peniana tratado com sucesso com radioterapia.Kaposi's sarcoma is a vascular tumor involving the wall of lymphatic vessels. There are four types: classic, endemic, iatrogenic and HIV-associated. It is a systemic, malignant and multifactorial disease and has a variable course. The primary presentation on the penis is uncommon and is mainly observed in HIV-positive patients. In HIV-negative individuals, asymptomatic papules, nodules, plaques and verrucous lesions are found. The treatment for the classic form involves surgery, cryotherapy, electrosurgery, laser and radiation therapy. The authors present a rare case of a patient with the classic form on the penis, who was successfully treated by radiation therapy.

  20. A computational profiling of changes in gene expression and transcription factors induced by vFLIP K13 in primary effusion lymphoma.

    Directory of Open Access Journals (Sweden)

    Vasu Punj

    Full Text Available Infection with Kaposi's sarcoma associated herpesvirus (KSHV has been linked to the development of primary effusion lymphoma (PEL, a rare lymphoproliferative disorder that is characterized by loss of expression of most B cell markers and effusions in the body cavities. This unique clinical presentation of PEL has been attributed to their distinctive plasmablastic gene expression profile that shows overexpression of genes involved in inflammation, adhesion and invasion. KSHV-encoded latent protein vFLIP K13 has been previously shown to promote the survival and proliferation of PEL cells. In this study, we employed gene array analysis to characterize the effect of K13 on global gene expression in PEL-derived BCBL1 cells, which express negligible K13 endogenously. We demonstrate that K13 upregulates the expression of a number of NF-κB responsive genes involved in cytokine signaling, cell death, adhesion, inflammation and immune response, including two NF-κB subunits involved in the alternate NF-κB pathway, RELB and NFKB2. In contrast, CD19, a B cell marker, was one of the genes downregulated by K13. A comparison with K13-induced genes in human vascular endothelial cells revealed that although there was a considerable overlap among the genes induced by K13 in the two cell types, chemokines genes were preferentially induced in HUVEC with few exceptions, such as RANTES/CCL5, which was induced in both cell types. Functional studies confirmed that K13 activated the RANTES/CCL5 promoter through the NF-κB pathway. Taken collectively, our results suggest that K13 may contribute to the unique gene expression profile, immunophenotype and clinical presentation that are characteristics of KSHV-associated PEL.

  1. Primary Ewing Sarcoma/Primitive Neuroectodermal Tumor of the Stomach

    Directory of Open Access Journals (Sweden)

    Safi Khuri

    2016-11-01

    Full Text Available Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET is a tumor of small round cells arising in skeletal tissues. These tumors rarely arise in the stomach. We present a 31-year-old healthy female patient who was admitted to our surgical ward due to upper gastrointestinal hemorrhage. Upper endoscopy revealed a large ulcerated bleeding mass originating from the lesser curvature. Biopsy revealed tumor cell immunoreactivity positive for CD99, vimentin, and Ki67 (an index of proliferation. These findings were compatible with gastric ES/PNET. The fluorescence in situ hybridization analysis result for the EWSR1 gene rearrangement (11: 22 translocation was positive. The patient refused neoadjuvant treatment and thus underwent an operation during which a mass at the lesser curvature of the stomach was found. The mass was adhering to the pancreatic tail and to the mesentery of the transverse and descending colon. Total gastrectomy, distal pancreatectomy, splenectomy, and left adrenalectomy were done. The patient refused adjuvant treatment. She is free of disease 3 years after surgery.

  2. Unusual Presentation of a Primary Ewing’s Sarcoma of the Spine with Paraplegia: A Case Report

    Science.gov (United States)

    Sundarapandian, Rajkumar Jayachandran; Surulivel, Vignesh Jayabalan

    2015-01-01

    Ewing’s sarcoma is a primary malignancy of the bone affecting individuals in the second decade of life. Primary sarcomas of the spine are rare and the occurrence of Primary Ewing’s sarcoma in the spine is very rare. Ewing’s sarcoma occurring in the spine is divided into two types, Ewing’s sarcoma of sacral spine which are very aggressive with poor prognosis and Ewing’s sarcoma of the non sacral spine which is an extremely rare occurrence. Patient may present with neurological deficit when the tumour extends into the spinal canal causing spinal cord compression. Magnetic resonance imaging (MRI) is very sensitive in diagnosing the tumour and defining the extent of the tumour. Here we report an 18-year-old boy who presented with back pain and complete paraplegia of two months duration. The MRI gave a differential diagnosis of infective pathology due to the fluid collection in the paraspinal region, followed by primary malignancy as the second diagnosis. Patient underwent posterior spinal decompression and stabilization, and intaoperatively there was significant collection of pus whose culture showed no growth. The histopathology and immunohistochemistry studies confirmed the diagnosis of Ewing’s sarcoma and patient was started on combination chemotherapy and radiotherapy. PMID:25954672

  3. Solitary extra-skeletal sinonasal metastasis from a primary skeletal Ewing's sarcoma.

    Science.gov (United States)

    Hayes, S M; Jani, T N; Rahman, S M; Jogai, S; Harries, P G; Salib, R J

    2011-08-01

    Ewing's sarcoma is a rare, malignant tumour predominantly affecting young adolescent males. We describe a unique case of an isolated extra-skeletal metastasis from a skeletal Ewing's sarcoma primary, arising in the right sinonasal cavity of a young man who presented with severe epistaxis and periorbital cellulitis. Histologically, the lesion comprised closely packed, slightly diffuse, atypical cells with round, hyperchromatic nuclei, scant cytoplasm and occasional mitotic figures, arranged in a sheet-like pattern. Immunohistochemical analysis showed positive staining only for cluster of differentiation 99 glycoprotein. Fluorescent in situ hybridisation identified the Ewing's sarcoma gene, confirming the diagnosis. Complete surgical resection was achieved via a minimally invasive endoscopic transnasal approach; post-operative radiotherapy. Ten months post-operatively, there were no endoscopic or radiological signs of disease. Metastatic Ewing's sarcoma within the head and neck is incredibly rare and can pose significant diagnostic and therapeutic challenges. An awareness of different clinical presentations and distinct histopathological features is important to enable early diagnosis. This case illustrates one potential management strategy, and reinforces the evolving role of endoscopic transnasal approaches in managing sinonasal cavity and anterior skull base tumours.

  4. No evidence of cholesteatoma in untreated otitis media with effusion in children with primary ciliary dyskinesia.

    Science.gov (United States)

    Ghedia, Reshma; Ahmed, Jahangir; Navaratnam, Annakan; Harcourt, Jonny

    2018-02-01

    Primary Ciliary Dyskinesia (PCD) describes a group of inherited disorders that result in abnormal ciliary motion leading to mucous stasis. Clinical features include almost universally otitis media with effusion (OME), particularly in infants. PCD patients provide us with a cohort of patients with OME that is not treated with ventilatory tube (VT) insertion as these have been shown to result in frequent complications including chronic otorrhoea, early extrusion and persistent perforation without significant improvement to hearing in the long term. This cohort was used to investigate whether children with PCD and OME not treated with VT were predisposed to cholesteatoma formation in the setting of a paediatric quaternary referral centre. A retrospective chart review was performed of all the children attending a multi-disciplinary PCD clinic at a national quaternary referral centre with a diagnosis of OME. We reviewed otoscopic findings, and audiometry and tympanometry results. We assessed the children in four groups: Watchful waiting, hearing aids, VT, and VT and hearing aids. One-hundred-and-one of 107 patients included in the study had a diagnosis of otitis media with effusion. No child with OME and PCD was diagnosed with a cholesteatoma during the follow up period. The only children who had insertion of a ventilatory tube were those who had the procedure prior to the formal diagnosis of PCD. We found a significant complication rate in the children with VT insertion. Hearing improved over time. The prevalence of retraction pockets in untreated OME was 1.72% (3 out of 174 ears). In children with PCD, OME is an almost universal finding in younger children, but not in adolescents. The study supports the current preference to avoid VT insertion in children with PCD as it confers a significantly higher rate of complications. No cases of cholesteatoma were found in this cohort of PCD children with OME managed without VTs. Crown Copyright © 2017. Published by Elsevier B

  5. Primary Ewing’s sarcoma of the spine: case report Sarcoma de Ewing primário da coluna vertebral: relato de caso

    Directory of Open Access Journals (Sweden)

    Leandro I. Dini

    2006-09-01

    Full Text Available Primary malignant sarcomas of the spine are extremely rare. Because of biological heterogeneity, these tumors have variable sensitivity to radiation and chemotherapy. Adequate local control through complete tumor removal is an important therapeutic goal. However, aggressive resection of tumors in the spinal column must be coupled with restoration of spinal column stability and minimization of neural deficits. The balance of these factors makes treatment of primary sarcomas of the spine challenging, and dictates an individual approach to treatment. We report on a 18 years old man with primary Ewing's sarcoma of the nonsacral spine. The clinical picture and imaging characteristics were analyzed as well as the management modalities and outcome.Sarcomas malignos primários da coluna são extremamente raros. Devido sua heterogeneidade biológica, estes tumores apresentam sensibilidade variada à radioterapia e à quimioterapia.O controle local adequado através da ressecção tumoral completa é um objetivo terapêutico importante. Contudo, a ressecção completa dos tumores da coluna vertebral deve ser realizada com a restauração da estabilidade espinhal e minimização do déficit neurológico. O equilíbrio entre estes fatores torna o tratamento dos sarcomas da coluna desafiador, exigindo-se uma abordagem individualizada para o tratamento de cada paciente. Relatamos um caso de sarcoma de Ewing da coluna não-sacral em um homem de 18 anos. O quadro clínico e características de imagem foram analisados, bem como a modalidades de manejo e o prognóstico.

  6. Up-regulation of tumor suppressor genes by exogenous dhC16-Cer contributes to its anti-cancer activity in primary effusion lymphoma.

    Science.gov (United States)

    Cao, Yueyu; Qiao, Jing; Lin, Zhen; Zabaleta, Jovanny; Dai, Lu; Qin, Zhiqiang

    2017-02-28

    Primary effusion lymphoma (PEL) is a rare and highly aggressive B-cell malignancy with Kaposi's sarcoma-associated herpesvirus (KSHV) infection, while lack of effective therapies. Our recent data indicated that targeting the sphingolipid metabolism by either sphingosine kinase inhibitor or exogenous ceramide species induces PEL cell apoptosis and suppresses tumor progression in vivo. However, the underlying mechanisms for these exogenous ceramides "killing" PEL cells remain largely unknown. Based on the microarray analysis, we found that exogenous dhC16-Cer treatment affected the expression of many cellular genes with important functions within PEL cells such as regulation of cell cycle, cell survival/proliferation, and apoptosis/anti-apoptosis. Interestingly, we found that a subset of tumor suppressor genes (TSGs) was up-regulated from dhC16-Cer treated PEL cells. One of these elevated TSGs, Thrombospondin-1 (THBS1) was required for dhC16-Cer induced PEL cell cycle arrest. Moreover, dhC16-Cer up-regulation of THBS1 was through the suppression of multiple KSHV microRNAs expression. Our data demonstrate that exogenous ceramides display anti-cancer activities for PEL through regulation of both host and oncogenic virus factors.

  7. Primary lung sarcoma treated with stereotactic ablative radiotherapy: a case report

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    Yeo SG

    2017-07-01

    Full Text Available Seung-Gu Yeo Department of Radiation Oncology, Soonchunhyang University College of Medicine, Soonchunhyang University Hospital, Cheonan, Republic of Korea Abstract: Primary lung sarcoma (PLS is an extremely rare, very aggressive malignancy. Surgical removal is considered the treatment of choice, and patients who have been given conventional radiotherapy have had inferior outcomes. This study is the first describing a case of PLS treated with stereotactic ablative radiotherapy (SABR, which precisely targets a small tumor with a markedly higher biologically effective dose than conventional radiotherapy. The patient was an 82-year-old man who was diagnosed with primary lung leiomyosarcoma based on radiology, pathology, and immunohistochemical examinations. The PLS was located in the right lower lobe and measured 2.5 cm. No regional nodal or distant organ metastasis was observed. He was inoperable medically. The SABR was performed using volumetric modulated arc therapy and a dose of 56 Gy in four fractions. Follow-up computed tomography 2 months after SABR revealed a complete tumor response. The toxicity was limited to mild respiratory symptoms. The patient is alive and has had no evidence of disease for 2 years. This study suggests that SABR can be a safe and effective treatment option for PLS. Keywords: primary lung sarcoma, leiomyosarcoma, stereotactic ablative radiotherapy, stereotactic body radiotherapy, radiation therapy, sarcoma 

  8. Primary Pulmonary Synovial Sarcoma: A Case with Unique and Impressive Computed Tomography Findings

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    Jaspreet S Kambo

    2015-01-01

    Full Text Available Primary pulmonary synovial sarcoma (PPSS is a rare malignancy. Its etiology, imaging features and optimal treatment are not well understood. Pulmonary pseudoaneurysms and lymphadenopathy are rare complications of synovial sarcomas. A 40-year-old woman with mild hemoptysis and thoracic back pain underwent a computed tomography scan that revealed multiple pulmonary lesions, paraesophageal lymphadenopathy and incidental bilateral pulmonary emboli. A diagnosis of PPSS was made through the identification of an SS18 translocation by fluorescence in situ hybridization. She was started on adriamycin, ifosfamide and mesna chemotherapy. Over the subsequent two months, she developed three pulmonary artery pseudoaneurysms, ultimately requiring endovascular coiling. Seven months after starting treatment, the patient was asymptomatic. The lesions and lymphadenopathy decreased in size. The present case highlights complications of a rare malignancy and demonstrates positive response to ifosfamide-based chemotherapy in the setting of PPSS.

  9. Primary Ewing's Sarcoma/Primitive Neuroectodermal Tumor of Kidney with Caval Involvement in a Pregnant Woman.

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    Ding, Yinghui; Huang, Zhenlin; Ding, Yafei; Jia, Zhankui; Gu, Chaohui; Xue, Rui; Yang, Jinjian

    2016-01-01

    In this article, we report the case of a woman in whom was found an abdominal mass during pregnancy and who underwent nephrectomy and extraction of the emboli after delivery. The kidney had a volume of 15 × 10 × 8 cm and pathological diagnosis was primary Ewing's sarcoma. The patient was treated with conventional chemotherapy for 1 year after surgery, at which time multiple metastases were found. From this case, we surmise that hormonal changes that occur during pregnancy may accelerate the growth of Ewing's sarcoma of the kidney, suggesting that renal tumors in pregnant women demand serious attention and that anti-cancer treatment should begin as soon as possible. © 2016 S. Karger AG, Basel.

  10. Multimodality Imaging Approach towards Primary Aortic Sarcomas Arising after Endovascular Abdominal Aortic Aneurysm Repair: Case Series Report

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    Kamran, Mudassar, E-mail: kamranm@mir.wustl.edu; Fowler, Kathryn J., E-mail: fowlerk@mir.wustl.edu; Mellnick, Vincent M., E-mail: mellnickv@mir.wustl.edu [Washington University School of Medicine, Mallinckrodt Institute of Radiology (United States); Sicard, Gregorio A., E-mail: sicard@wudosis.wustl.edu [Washington University School of Medicine, Department of Surgery (United States); Narra, Vamsi R., E-mail: narrav@mir.wustl.edu [Washington University School of Medicine, Mallinckrodt Institute of Radiology (United States)

    2016-06-15

    Primary aortic neoplasms are rare. Aortic sarcoma arising after endovascular aneurysm repair (EVAR) is a scarce subset of primary aortic malignancies, reports of which are infrequent in the published literature. The diagnosis of aortic sarcoma is challenging due to its non-specific clinical presentation, and the prognosis is poor due to delayed diagnosis, rapid proliferation, and propensity for metastasis. Post-EVAR, aortic sarcomas may mimic other more common aortic processes on surveillance imaging. Radiologists are rarely knowledgeable about this rare entity for which multimodality imaging and awareness are invaluable in early diagnosis. A series of three pathologically confirmed cases are presented to display the multimodality imaging features and clinical presentations of aortic sarcoma arising after EVAR.

  11. Otitis media with effusion (OME in primary care: follow-up protocol

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    José Ignacio BENITO-OREJAS

    2016-07-01

    Full Text Available Introduction and objective: With the intention of improving the quality of care of children with otitis media with effusion (OME, the pediatricians of primary care (PAP have been provided procedures diagnostic and training media, establishing a rule of consensus action. In this paper we present the Protocol developed for the monitoring of OME in children. Method: A team of pediatricians and otolaryngologists from the same Healthcare Area, who have worked to achieve this consensus. Through the published guides and review of the literature on OME, we find his best adaptation to our environment. Results: We expose the necessary steps for the diagnosis, follow-up, referral and documentation of OME in children. Discussion: A correct therapeutic intervention for OME in children involves documenting patient characteristics and clinical findings of the pneumatic otoscopy and tympanometry, perform periodic and vigilant monitoring of potential disruptions, know and apply the reasons for referral to specialized care and maintaining good communication between pediatricians and ENT. Conclusions: The agreement between pediatricians and ENT has helped establish a joint guide of action in children for OME, which together with the improvement of diagnosis, will result in a greater degree of efficiency in the childhood care of OME.

  12. Primary Synovial Sarcoma of Kidney: A Rare Differential Diagnosis of Renomegaly

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    Gaurang Modi

    2014-01-01

    Full Text Available Synovial sarcomas (SS are classified as subgroup of soft tissue sarcomas affecting mainly extremities of young adults. Primary SS of kidney are very rare tumours with poor prognosis. Though they have characteristic histology and immunohistochemistry (IHC due to rarity of incidence it is difficult to diagnose them. Sometimes chromosomal rearrangement studies are required to confirm the diagnosis. We are presenting a case of 41-year-old male who was referred to our cancer centre for evaluation of left renal mass. CT scan of abdomen revealed a large left renal mass encasing the aorta. Biopsy of renal mass revealed poorly differentiated sarcoma and IHC was positive for vimentin, CD99, and BCL2 and negative for AE1, epithelial membrane antigen, and leukocyte common antigen. The patient was clinically inoperable as renal mass was encasing the aorta. So he was subsequently offered palliative chemotherapy in form of ifosfamide and adriamycin. CT abdomen shows partial response after 3 cycles of chemotherapy according to RECIST criteria.

  13. Age dependency of primary tumor sites and metastases in patients with Ewing sarcoma.

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    Worch, Jennifer; Ranft, Andreas; DuBois, Steven G; Paulussen, Michael; Juergens, Heribert; Dirksen, Uta

    2018-06-01

    The median age of patients with Ewing sarcoma (EwS) at diagnosis is around 14-15 years. Older age is associated with a worse outcome. The correlation of age at diagnosis on sites of disease has not been fully described. The goal of this study was to evaluate the differences in sites of primary tumor and metastatic tumor involvement according to age groups. EwS data from the Gesellschaft für Pädiatrische Onkologie und Hämatology (GPOH) database of the Cooperative Ewing Sarcoma Study (CESS) 81/86 and the European Intergroup Cooperative Ewing's Sarcoma Study EICESS 92 and the EUROpean Ewing tumor Working Initiative of National Groups-99-Protocol (EURO-E.W.I.N.G.-99) study were analyzed. Patient and tumor characteristics were evaluated statistically using chi square tests. The study population included 2,635 patients with bone EwS. Sites of primary and metastatic tumors differed according to the age groups of young children (0-9 years), early adolescence (10-14 years), late adolescence (15-19 years), young adults (20-24 years), and adults (more than 24 years). Young children demonstrated the most striking differences in site of disease with a lower proportion of pelvic primary and axial tumors. They presented less often with metastatic disease at diagnosis. Site of primary and metastatic tumor involvement in EwS differs according to patient age. The biological and developmental etiology for these differences requires further investigations. © 2018 Wiley Periodicals, Inc.

  14. A Case of Low-Grade Primary Cardiac Lymphoma with Pericardial Effusion Diagnosed by Combined 18F-Fluorodeoxyglucose Positron Emission Tomography and Computed Tomography (FDG-PET/CT) Imaging and Effusion Cytology.

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    Fukunaga, Hisanori; Tatewaki, Yasuko; Mutoh, Tatsushi; Shimomura, Hideo; Yamamoto, Shuzo; Terao, Chiaki; Totsune, Tomoko; Nakagawa, Manabu; Taki, Yasuyuki

    2018-03-14

    BACKGROUND Primary cardiac lymphoma is rare and can be an aggressive disease, depending on the grade. A case is reported of low-grade primary cardiac lymphoma associated with a pericardial effusion. 18F-fluorodeoxyglucose positron emission tomography and computed tomography (FDG-PET/CT) imaging was useful in the diagnosis and in evaluating the disease activity in this case. CASE REPORT A 72-year-old Japanese woman visited a general practitioner, complaining of dyspnea associated with cardiac tamponade. Pericardiocentesis was performed, and Group V malignant cells were identified by cytology, suspicious for malignant lymphoma. Whole-body FDG-PET/CT scans showed no pleural effusion or lymph node metastasis supporting the diagnosis of primary cardiac lymphoma diagnosed on pericardial effusion. The laboratory investigations showed that levels of serum soluble interleukin-2 (IL-2) receptor (sIL-2R), a diagnostic and prognostic marker for malignant lymphoma, were not elevated (258 U/ml). A six-month follow-up FDG-PET/CT scan showed an increased volume of the pericardial effusion and mild but abnormal uptake diffusely in the pericardial space, and the sIL-2R was slightly elevated (860 U/ml). No abnormal FDG accumulation outside the retained pericardial effusion was noted, which was compatible with a clinical picture of low-grade primary cardiac lymphoma, and in a period of watchful waiting during the first two years later, the sIL-2R had reduced to 195 U/ml. CONCLUSIONS This is a rare case of low-grade primary cardiac lymphoma detected in a pericardial effusion, and highlights the utility of the FDG-PET/CT scan as a valuable diagnostic and follow-up modality.

  15. Hemostatic findings of pleural fluid in dogs and the association between pleural effusions and primary hyperfibrino(geno)lysis: A cohort study of 99 dogs.

    Science.gov (United States)

    Zoia, Andrea; Drigo, Michele; Piek, Christine J; Simioni, Paolo; Caldin, Marco

    2018-01-01

    The primary objective of this study was to determine if activation of coagulation and fibrinolysis occurs in canine pleural effusions. Thirty-three dogs with pleural effusions of different origin were studied. Pleural effusion fibrinogen concentrations were significantly lower, while pleural fibrin-fibrinogen degradation products (FDPs) and D-dimer concentrations were significantly higher than those in plasma (P dogs with pleural effusion, and whether the presence of a concurrent inflammatory process may have activated the hemostatic cascade, with its intrinsically linked secondary hyperfibrinolysis, masking the concurrent PHF. The previously 33 selected dogs with pleural effusion (group 1) were compared to two control groups of 33 healthy (group 2) and 33 sick dogs without pleural effusion (group 3). Serum fibrinogen, FDPs, D-dimer, C-reactive protein (CRP), fibrinogen/CRP ratio, and frequency of PHF were determined. Fibrinogen, FDPs, D-dimer and CRP concentrations in group 1 were significantly increased compared to group 2 (P dogs with pleural effusion compared to healthy dogs. Nevertheless, the decrease in the fibrinogen/CRP ratio in group 1 compared to group 3, considering the higher FDPs and similar D-dimer concentrations, would suggest that PHF is also more frequent in dogs with pleural effusion compared to sick control dogs, and that this phenomenon is hidden due to concurrent secondary hyperfibrinolysis.

  16. Primary histiocytic sarcoma of the uterine cervix: an extremely rare entity

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    Binesh Fariba

    2017-03-01

    Full Text Available Objective. Histiocytic sarcoma (HS is an extraordinary rare tumor and it has an offensive clinical course. HS of the uterine cervix is a far uncommon tumor with just a few cases described so far. Here we presented a case of primary HS of the uterine cervix in a 62-year-old female initially misdiagnosed as large cell non-keratinizing squamous cell carcinoma. To the best of our knowledge, this is the first reported case of HS of the uterine cervix from Iran.

  17. Effect of nasal balloon autoinflation in children with otitis media with effusion in primary care: an open randomized controlled trial

    Science.gov (United States)

    Williamson, Ian; Vennik, Jane; Harnden, Anthony; Voysey, Merryn; Perera, Rafael; Kelly, Sadie; Yao, Guiqing; Raftery, James; Mant, David; Little, Paul

    2015-01-01

    Background: Otitis media with effusion is a common problem that lacks an evidence-based nonsurgical treatment option. We assessed the clinical effectiveness of treatment with a nasal balloon device in a primary care setting. Methods: We conducted an open, pragmatic randomized controlled trial set in 43 family practices in the United Kingdom. Children aged 4–11 years with a recent history of ear symptoms and otitis media with effusion in 1 or both ears, confirmed by tympanometry, were allocated to receive either autoinflation 3 times daily for 1–3 months plus usual care or usual care alone. Clearance of middle-ear fluid at 1 and 3 months was assessed by experts masked to allocation. Results: Of 320 children enrolled, those receiving autoinflation were more likely than controls to have normal tympanograms at 1 month (47.3% [62/131] v. 35.6% [47/132]; adjusted relative risk [RR] 1.36, 95% confidence interval [CI] 0.99 to 1.88) and at 3 months (49.6% [62/125] v. 38.3% [46/120]; adjusted RR 1.37, 95% CI 1.03 to 1.83; number needed to treat = 9). Autoinflation produced greater improvements in ear-related quality of life (adjusted between-group difference in change from baseline in OMQ-14 [an ear-related measure of quality of life] score −0.42, 95% CI −0.63 to −0.22). Compliance was 89% at 1 month and 80% at 3 months. Adverse events were mild, infrequent and comparable between groups. Interpretation: Autoinflation in children aged 4–11 years with otitis media with effusion is feasible in primary care and effective both in clearing effusions and improving symptoms and ear-related child and parent quality of life. Trial registration: ISRCTN, No. 55208702. PMID:26216608

  18. Primary intimal sarcoma of the pulmonary artery 1 case report and retrospective analysis of 20 cases published in China

    International Nuclear Information System (INIS)

    Guo Lingchuan; Chen Yanbin

    2012-01-01

    Objective: To explore the clinical and histopathological features of primary, intimal sarcoma of the pulmonary artery. Methods: One case of primary intimal sarcoma of the pulmonary artery diagnosed in our hospital and 20 cases published openly in China were analysed retrospectively. Results: The clinical presentation of primary intimal sarcoma of the pulmonary artery was progressive dyspnea, cough, chest pain, syncope, bloody phlegm, fever and fatigue. From the radiological finding, its signs were of pulmonary, artery dilation, reduced pulmonary vasculature and cardiomegaly, soft tissue mass near the right ventricle outflow tract. Histological examination of intimal sarcoma of the pulmonary artery revealed a poorly differentiated mesenchymal tumor of fibroblastic or myofibroblastic differentiation, consisting of mildly atypical spindle cells with atypia, mitosis, and nuclear polymorphism. Immunohistochemical analysis showed positive staining with antibodies against vimentin, alpha-smooth muscle actin, while negative for desmin. Conclusion: Primary, intimal sarcoma of the pulmonary artery has atypical clinical and radiological manifestation with poor prognosis, its definite diagnosis depends on histopathology. (authors)

  19. A RARE CASE OF ATYPICAL PRIMARY EWING’S SARCOMA OF OCCIPITAL BONE

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    K. Srihari

    2016-07-01

    Full Text Available BACKGROUND Ewing’s Sarcoma is an aggressive malignant neoplasm most frequently manifesting in the second decade of life and accounting for 4% of childhood and adolescent malignancies. These tumours were first described by James Ewing in 1921 as tumours that arise from bone. These osseous lesions have since become infamous for their highly aggressive course with 20% to 30% of patients having evidence of metastasis at the time of diagnosis and an estimated 10-year survival rate of 50%. Metastases to the CNS have most recently been estimated to occur in less than 5% of cases and are usually due to direct extension of an osseous lesion into the extradural space or more rarely through haematogenous spread. CASE REPORT In this article, we report a case of 17-year-old boy who presented to the radiology department with complaints of recurrent episodes of headache and vomiting for the past 3 days. On radiological investigation, there was a large well-defined, lobulated, extra-axial mass lesion measuring 3.6 X 5.7 X 5.9 cm noted in the supratentorial left occipital region which was fairly enhancing after contrast administration. The mass was causing permeative type of destruction of the left occipital bone and extending into extracranial soft tissue. Final diagnosis was done by biopsy and histopathology which showed “Atypical Ewing’s Sarcoma” of the left occipital bone. Considering its unusual site and soft tissue extension, we report this case of Primary Atypical Ewing’s sarcoma of occipital bone. CONCLUSION Primary cranial Ewing's sarcoma is to be considered in the differential diagnosis in children with a tumour involving the skull with destruction of the bone and presence of extra-axial soft tissue swelling. CT is the excellent modality for demonstration of bone destruction while MRI depicts soft tissue extension and metastasis if any.

  20. Primary spinal epidural extraosseous Ewing's sarcoma: Report of five cases and literature review

    International Nuclear Information System (INIS)

    Mukhopadhyay, P.; Gairola, M.; Julka, P.K.; Rath, G.K.; Sharma, M.C.; Thulkar, S.

    2001-01-01

    Ewing's sarcoma is the most common malignant bone tumour occurring in children and adolescents and exists in two different clinico pathological entities: osseous Ewing's sarcoma (OES) and extraosseous Ewing's sarcoma (EES). Five cases of primary epidural EES are described, which presented with non-specific symptoms leading to a long diagnostic delay. The median age at diagnosis was 22 years (range 13-36 years). The median diagnostic delay was 3 months. All patients had one or more neurological deficits. All underwent surgical exploration with a laminectomy and partial resection followed by adjuvant radiotherapy to a dose of 46-50 Gy and chemotherapy with VAC (vincristine, adriamycin and cyclophosphamide) alternating with ICE (ifosphamide, cisplatin and etoposide) for at least six cycles. The mean follow-up period is 21.2 months (range 11-32 months). Four of the five patients achieved a complete remission and are disease free at the time of writing this report. Two patients have a residual neurological deficit - both having presented with long history of neurological deficit. Primary spinal epidural EES should be suspected whenever young patients present with back pain and/or radicular pain, have abnormal neurology and an extradural mass is demonstrated on MRI. Surgical excision followed by adjuvant radiotherapy (50 Gy) and combination chemotherapy (VAC alternating with ICE) achieved local and systemic control in these patients. A greater number of patients and longer follow up are required to evolve a generally accepted treatment policy for this aggressive but potentially curable malignancy. Copyright (2001) Blackwell Science Pty Ltd

  1. 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.

  2. Kaposi sarcoma herpesvirus pathogenesis

    Science.gov (United States)

    Koch, Sandra; Schulz, Thomas F.

    2017-01-01

    Kaposi sarcoma herpesvirus (KSHV), taxonomical name human gammaherpesvirus 8, is a phylogenetically old human virus that co-evolved with human populations, but is now only common (seroprevalence greater than 10%) in sub-Saharan Africa, around the Mediterranean Sea, parts of South America and in a few ethnic communities. KSHV causes three human malignancies, Kaposi sarcoma, primary effusion lymphoma, and many cases of the plasmablastic form of multicentric Castleman's disease (MCD) as well as occasional cases of plasmablastic lymphoma arising from MCD; it has also been linked to rare cases of bone marrow failure and hepatitis. As it has colonized humans physiologically for many thousand years, cofactors are needed to allow it to unfold its pathogenic potential. In most cases, these include immune defects of genetic, iatrogenic or infectious origin, and inflammation appears to play an important role in disease development. Our much improved understanding of its life cycle and its role in pathogenesis should now allow us to develop new therapeutic strategies directed against key viral proteins or intracellular pathways that are crucial for virus replication or persistence. Likewise, its limited (for a herpesvirus) distribution and transmission should offer an opportunity for the development and use of a vaccine to prevent transmission. This article is part of the themed issue ‘Human oncogenic viruses’. PMID:28893942

  3. Primary Ewing’s Sarcoma of the Spine in a Two-Year-Old Boy

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    Ali J. Electricwala

    2016-01-01

    Full Text Available Ewing’s Sarcoma (ES is a highly malignant bone tumour. It may involve any part of the skeleton but the most frequent parts are the ilium and diaphysis of femur and tibia (Alfeeli et al., 2005; Zhu et al., 2012. Primary ES of the spine is extremely rare (Yan et al., 2011. It accounts for only 3.5 to 14.9 percent of all primary bone sarcomas. The age of presentation ranges from 12 to 24 years (median 21 years (Ferguson, 1999; Sharafuddin et al., 1992; Klimo Jr. et al., 2009. We report an unusual case of primary ES of the spine in a two-year-old boy, who presented to us with paraparesis and features of cauda equina syndrome. MRI scan showed a tumour mass arising from the pedicle of L4 vertebra invading the spinal canal. Tc-99 bone scan showed increased tracer uptake in L4 vertebra and normal tracer uptake elsewhere in the skeleton. After reaching the diagnosis of a space occupying lesion invading the lumber spinal canal, we performed a decompressive laminectomy and a biopsy was sent which confirmed the diagnosis of ES. Immunohistochemistry showed tumour cells staining positive for CD-99 (specific stain for ES. Gene testing showed an EWS-FLI 1 chimera. Surgery was followed by good improvement in motor signs. The child was then referred to a specialized oncotherapy centre for further treatment, radiation, and chemotherapy. To the best of our knowledge, we are the first to report primary ES of the spine at the age of two years.

  4. 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.

  5. Primary renal undifferentiated sarcoma as an infiltrative mass in a 12 year old boy

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    Kim, Yong Hee; Kim, Myung Joon; Lee, Mi Jung [Dept. of Radiology and Research Institute of Radiological Science, Severance Children' s Hospital, Yonsei University College of Medicine, Seoul (Korea, Republic of); Kim, Se Hwa [Dept. of Pathology, Severance Hospital, Yonsei University College of Medicine, Seoul (Korea, Republic of)

    2015-09-15

    Undifferentiated sarcomas are rare tumors not classified into any sarcoma subtype. Due to their rarity, imaging findings of undifferentiated sarcomas are poorly characterized. The purpose of this report was to present imaging findings of a pathologically confirmed undifferentiated sarcoma originated from the left kidney of a 12-year-old boy. The mass was infiltrative involving the renal pelvis. It mimicked massive hilar lymphadenopathy with a preserved renal contour visible by both ultrasonography and CT. Renal vein thrombosis was also observed. Although undifferentiated sarcomas are rare, they should be considered in differential diagnosis of infiltrative renal masses with renal pelvis invasion in children.

  6. Primary renal undifferentiated sarcoma as an infiltrative mass in a 12 year old boy

    International Nuclear Information System (INIS)

    Kim, Yong Hee; Kim, Myung Joon; Lee, Mi Jung; Kim, Se Hwa

    2015-01-01

    Undifferentiated sarcomas are rare tumors not classified into any sarcoma subtype. Due to their rarity, imaging findings of undifferentiated sarcomas are poorly characterized. The purpose of this report was to present imaging findings of a pathologically confirmed undifferentiated sarcoma originated from the left kidney of a 12-year-old boy. The mass was infiltrative involving the renal pelvis. It mimicked massive hilar lymphadenopathy with a preserved renal contour visible by both ultrasonography and CT. Renal vein thrombosis was also observed. Although undifferentiated sarcomas are rare, they should be considered in differential diagnosis of infiltrative renal masses with renal pelvis invasion in children

  7. Primary Ewing's sarcoma of the sinonasal tract in adults: A challenging disease.

    Science.gov (United States)

    Lombardi, Davide; Mattavelli, Davide; Redaelli De Zinis, Luca O; Accorona, Remo; Morassi, Maria L; Facchetti, Fabio; Ferrari, Vittorio; Farina, Davide; Bertulli, Rossella; Nicolai, Piero

    2017-03-01

    Sinonasal localization of Ewing's sarcoma in adults is an exceedingly rare event. The clinical records of 5 patients with primary sinonasal Ewing's sarcoma treated from 1992 to 2012 were retrospectively analyzed. All pathologic slides were reviewed by 2 experienced pathologists. All patients underwent multimodality treatments. Median age was 36 years (range, 25-52 years). At referral, 2 patients had the original diagnosis changed by review of the histologic slides. Tumors were classified as T4aN0M0 (4 patients) and T2N0M0 (1 patient). Median follow-up was 110 months (range, 70-139 months). Only 1 patient, who started treatment elsewhere based on an incorrect histologic diagnosis, experienced multiple recurrences and eventually died of widespread metastasis. Correct pathologic diagnosis can have a crucial impact on treatment planning and outcome. Multimodality therapy is the key for long-term successful results. Because of the rarity of the tumor, referral to highly experienced care centers is strongly recommended. © 2016 Wiley Periodicals, Inc. Head Neck 39: E45-E50, 2017. © 2016 Wiley Periodicals, Inc.

  8. Pleural effusion

    Science.gov (United States)

    Complications of pleural effusion may include: Lung damage Infection that turns into an abscess, called an empyema Air in the chest cavity ( pneumothorax ) after drainage of the effusion Pleural thickening (scarring of the lining of the ...

  9. Subdural effusion

    Science.gov (United States)

    ... page: //medlineplus.gov/ency/article/001422.htm Subdural effusion To use the sharing features on this page, please enable JavaScript. A subdural effusion is a collection of cerebrospinal fluid (CSF) trapped ...

  10. Hemostatic findings of pleural fluid in dogs and the association between pleural effusions and primary hyperfibrino(geno)lysis: A cohort study of 99 dogs

    Science.gov (United States)

    Drigo, Michele; Piek, Christine J.; Simioni, Paolo; Caldin, Marco

    2018-01-01

    The primary objective of this study was to determine if activation of coagulation and fibrinolysis occurs in canine pleural effusions. Thirty-three dogs with pleural effusions of different origin were studied. Pleural effusion fibrinogen concentrations were significantly lower, while pleural fibrin-fibrinogen degradation products (FDPs) and D-dimer concentrations were significantly higher than those in plasma (P pleural fluids, there is evidence of coagulation activation and fibrinolysis. The secondary aims of the current study were to determine if primary hyperfibrinolysis ([PHF] i.e., elevated plasma FDPs with a normal D-dimer concentrations), occurs in dogs with pleural effusion, and whether the presence of a concurrent inflammatory process may have activated the hemostatic cascade, with its intrinsically linked secondary hyperfibrinolysis, masking the concurrent PHF. The previously 33 selected dogs with pleural effusion (group 1) were compared to two control groups of 33 healthy (group 2) and 33 sick dogs without pleural effusion (group 3). Serum fibrinogen, FDPs, D-dimer, C-reactive protein (CRP), fibrinogen/CRP ratio, and frequency of PHF were determined. Fibrinogen, FDPs, D-dimer and CRP concentrations in group 1 were significantly increased compared to group 2 (P pleural effusion compared to healthy dogs. Nevertheless, the decrease in the fibrinogen/CRP ratio in group 1 compared to group 3, considering the higher FDPs and similar D-dimer concentrations, would suggest that PHF is also more frequent in dogs with pleural effusion compared to sick control dogs, and that this phenomenon is hidden due to concurrent secondary hyperfibrinolysis. PMID:29462172

  11. Primary breast sarcoma: A retrospective study over 35 years from a single institution

    DEFF Research Database (Denmark)

    Holm, Maibritt Provstgaard; Aggerholm-Pedersen, Ninna; Mele, Marco

    2016-01-01

    AIM: The aim of this study was to contribute to the collected knowledge of prognostic factors in primary breast sarcomas (PBS) to the benefit of possible future prospective studies and therapeutic guidelines. METHOD: All patients with pathologically verified PBS in the period of 1979-2014 were......, size, histological classification, prior radiation and grade. Prognostic factors were determined by the use of Cox proportional hazard ratio. RESULTS: In total 42 patients were identified. Surgical resection was the main method of treatment. Nineteen (45%) patients were initially selected...... in those with superficial tumors was observed as well as an increased incidence in radiation-induced angiosarcoma (AS) of the breast, however, prognosis was no different from non-radiation-induced AS. CONCLUSION: Prognostic factors in PBS patients were size and grade with a trend towards better survival...

  12. CT differential diagnosis of primary pelvic osteosarcoma, chondrosarcoma and Ewing's sarcoma

    International Nuclear Information System (INIS)

    Heller, M.; Heyer, D.; Spielmann, R.P.; Buecheler, E.

    1990-01-01

    The value of CT for the differential diagnosis of primary malignant tumours in the pelvis was investigated in the case of three types of tumour: Osteosarcomas, chondrosarcomas and Ewing's sarcomas. A total of 78 CT examinations in 29 patients was used. The results show that CT, using suitable techniques (high resolution etc.) constitutes a valuable diagnostic method for differentiating these bone tumours. This applies not only for the localisation of the tumour and for defining its extent, but also for showing the morphology of intra- and extra-osseous soft tissue components and their patterns of calcification. It is possible to recognise patterns of growth and of tissue destruction that are typical of individual tumours. (orig.) [de

  13. Primary Mediastinal Synovial Sarcoma Presenting as Superior Vena Cava Syndrome: A Rare Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Irappa Madabhavi

    2015-01-01

    Full Text Available Primary mediastinal sarcomas are aggressive tumors with a very rare incidence. This report describes the case of a 35-year-old male patient who presented with acute symptoms of dyspnoea, facial puffiness, voice-hoarseness, and engorged neck veins. With the clinical picture consistent with the superior vena cava (SVC syndrome, the patient was investigated with computed tomography of the chest. This revealed a large soft tissue density mass lesion compressing the SVC along with other critical superior mediastinal structures. Histopathological evaluation of the mass revealed features consistent with a soft tissue sarcoma and positive staining was observed for vimentin and S-100. Cytogenetic analysis by fluorescent in situ hybridisation (FISH demonstrated the t(X:18 translocation. Thus diagnosis was established as primary mediastinal synovial sarcoma. Patient was treated with three cycles of neoadjuvant chemotherapy, to which there was a partial response as per the RECIST criteria. Surgical excision of the mediastinal mass was performed, and further postoperative treatment with adjuvant chemoradiotherapy was provided. Patient currently is free of disease. This is to the best of our knowledge the first report in the world literature of a successfully treated case of “primary mediastinal sarcomas presenting as SVC syndrome.” Patient is under regular surveillance at our clinic and remains free of recurrence one year after treatment completion.

  14. Carbon ion radiotherapy for localized primary sarcoma of the extremities: Results of a phase I/II trial

    International Nuclear Information System (INIS)

    Sugahara, Shinji; Kamada, Tadashi; Imai, Reiko; Tsuji, Hiroshi; Kameda, Noriaki; Okada, Tohru; Tsujii, Hirohiko; Tatezaki, Shinichirou

    2012-01-01

    Purpose: To determine the effectiveness of carbon ion radiotherapy (CIRT) for localized primary sarcomas of the extremities in a prospective study. Patients and materials: From April 2000 to May 2010, 17 (male/female: 12/5) patients with localized primary sarcoma of the extremities received CIRT. The median age was 53 years (range: 14–87 years). Nine patients had primary diseases and eight had recurrent diseases. Of the 17 patients, eight refused amputation, and the remaining nine refused surgical resection. Tumors were located in the upper limbs in four patients and lower limbs in 13. Histological diagnosis was osteosarcoma in three patients, liposarcoma in two, synovial sarcoma in two, rhabdomyosarcoma in two, pleomorphic sarcoma in two, and miscellaneous in six. The CIRT dose to the limb was 52.8 GyE for one patient, 64 GyE for three, 70.4 GyE for 13 in 16 fixed fractions over 4 weeks. Records were reviewed and outcomes including radiologic response, local control (progression-free), and survival were analyzed. Results: The median follow-up was 37 months (range: 11–97 months). Radiological response rate was 65% (PR in 11, SD in 5, and PD in 1). The local control rate at 5 years was 76%. The overall survival rate at 5 years was 56%. Of the 17 patients, 10 survived without disease progression. Four patients had local recurrences, one was salvaged by repeated CIRT and the other three died due to systemic diseases. Distant failure was observed in six patients. One patient suffered from femoral fracture (grade 3) and received surgical fixation 27 months after CIRT. No other severe reactions (grade 3) were observed. Conclusions: CIRT is suggested to be an effective and safe treatment for patients who refuse surgery for localized primary sarcomas of the extremities.

  15. Imaging findings of primary breast sarcoma: Results of a first multicenter study

    Energy Technology Data Exchange (ETDEWEB)

    Wienbeck, Susanne, E-mail: susanne.wienbeck@med.uni-goettingen.de [University of Göttingen, Institute of Diagnostic and Interventional Radiology, Robert- Koch-Str. 40, 37075 Göttingen (Germany); Meyer, Hans Jonas [University Hospital Halle, Department of Radiology, Ernst-Grube-Str. 40, 06120 Halle (Germany); Herzog, Aimee [University of Jena, Institute of Diagnostic and Interventional Radiology, Erlanger Allee 101, 07747 Jena (Germany); Nemat, Sogand [University of Saarland, Clinic for Diagnostic and Interventional Radiology, Kirrberger Str. 100, 66424 Homburg (Germany); Teifke, Andrea [University of Mainz, Department of Diagnostic and Interventional Radiology, Langenbeckstr. 1, 55131 Mainz (Germany); Heindel, Walter [University of Münster, Institute of Clinical Radiology, Albert-Schweitzer-Str. 33, 48149 Münster (Germany); Schäfer, Fritz [University of Kiel, Institute of Radiology and Neuroradiology, Arnold-Heller-Str. 3, 24105 Kiel (Germany); Kinner, Sonja [University of Essen, Institute of Diagnostic and Interventional Radiology and Neuroradiology, Hufelandstr. 55, 45147 Essen (Germany); Müller-Schimpfle, Markus [Clinic Frankfurt Höchst, Department of Radiology, Gotenstr. 6, 65929 Frankfurt am Main (Germany); Surov, Alexey [University Hospital Halle, Department of Radiology, Ernst-Grube-Str. 40, 06120 Halle (Germany); University of Leipzig, Department of Diagnostic and Interventional Radiology, Liebigstr. 20, 04103 Leipzig (Germany)

    2017-03-15

    Purpose: To investigate imaging findings in patients with primary breast sarcoma (PBS). Materials and methods: A retrospective search in the databases of 10 radiological departments in Germany from 2000 to 2011 was performed. Only histologically proven cases of PBS were included into the study. Mammography was available in 31 patients (33 lesions), ultrasound images in 24 patients (24 lesions), and for 10 patients (14 lesions) magnetic resonance imaging (MRI) of the breast was performed. The breast findings were classified according to the American College of Radiology Breast Imaging Reporting and Data Systems (BI-RADS) 5th edition categories. Collected data were evaluated by means of descriptive statistics. Results: Forty-two female patients (mean age 62.0 years, range, 30–86 years) were included in the study. Clinically, all women had painless lumps. Irregular (53.3% [16/30]) or oval (30.0% [9/30]) mass with indistinct (73.3% [22/30]) or microlobulated (10% [3/30]) margins were common findings on mammograms. Ultrasound revealed typically an irregular (79.2% [19/24]), hypoechoic (62.5% [15/24]) mass, with indistinct margins (79.2% [19/24]), and posterior acoustic shadowing (79.2% [19/24]). MRI showed irregular masses (81.8% [9/11]) with irregular or spiculated margins, and a rapid initial signal increase with a delayed washout in kinetic analysis. Conclusion: Overall, PBS has no pathognomonic imaging features and can mimic those of invasive mammary carcinoma. Breast sarcoma should be taken into the differential diagnosis of breast findings described above.

  16. Primary Ewing's sarcoma-primitive neuroectodermal tumor of the uterus: a case report and literature review.

    Science.gov (United States)

    Park, Jeong-Yeol; Lee, Sun; Kang, Hyoung Jin; Kim, Hy-Sook; Park, Sang-Yoon

    2007-08-01

    Primary Ewing's sarcoma-primitive neuroectodermal tumor (ES-PNET) of the uterus is an extremely rare malignancy. A 30-year-old Korean woman presented with abnormal uterine bleeding with uterine enlargement. A computed tomography (CT) scan and magnetic resonance imaging (MRI) of the abdomen and pelvis showed a huge uterine mass measuring 18 x 20 x 21 cm, metastasis to both pelvic and para-aortic lymph nodes, and omental infiltration. The pathology report of the uterine mass described a uniformly hypercellular tumor, which was arranged in diffuse solid sheets of uniform, small, rounded, and sometimes spindle-shaped cells, with scanty cytoplasm. Immunohistochemically, the mass tested positive for vimentin, CD99, and chromogranin. The patient received several courses of combination chemotherapy and radiotherapy but died from tumor progression 16 months after the initial diagnosis. This is a rare case of primary uterine ES-PNET in a woman of reproductive age. A review of the literature indicates that primary uterine ES-PNET requires early diagnosis and multimodality treatment including surgery, chemotherapy, and radiotherapy. The behavior of this tumor is potentially aggressive.

  17. High incidence of Kaposi sarcoma-associated herpesvirus infection in HIV-related solid immunoblastic/plasmablastic diffuse large B-cell lymphoma

    NARCIS (Netherlands)

    Deloose, S. T. P.; Smit, L. A.; Pals, F. T.; Kersten, M.-J.; van Noesel, C. J. M.; Pals, S. T.

    2005-01-01

    Kaposi sarcoma-associated herpesvirus ( KSHV) is known to be associated with two distinct lymphoproliferative disorders: primary effusion lymphoma (PEL) and multicentric Castleman disease (MCD)/MCD-associated plasmablastic lymphoma. We here report a high incidence of KSHV infection in solid

  18. Primary ewing sarcoma of the anterior mandible localized to the midline

    NARCIS (Netherlands)

    Ko, E.; Brouns, E.R.E.A.; Korones, D.N.; Pochal, W.F.; Philipone, E.M.; Zegarelli, D.J.; Yoon, A.J.

    2013-01-01

    Ewing sarcoma is a malignant, small, round blue-cell tumor of the bone that is usually located in the long bones and the pelvis. Fewer than 3% of all Ewing sarcomas originate in the head and neck region and these are mostly located in the posterior mandible. We report the case of a 17-year-old girl

  19. Primary effusion lymphoma associated with Human Herpes Virus-8 and Epstein Barr virus in an HIV-infected woman from Kampala, Uganda: a case report

    Directory of Open Access Journals (Sweden)

    Osuwat Lawrence O

    2011-02-01

    Full Text Available Abstract Introduction Primary effusion lymphoma is a recently recognized entity of AIDS related non-Hodgkin lymphomas. Despite Africa being greatly affected by the HIV/AIDS pandemic, an extensive MEDLINE/PubMed search failed to find any report of primary effusion lymphoma in sub-Saharan Africa. To our knowledge this is the first report of primary effusion lymphoma in sub-Saharan Africa. We report the clinical, cytomorphologic and immunohistochemical findings of a patient with primary effusion lymphoma. Case presentation A 70-year-old newly diagnosed HIV-positive Ugandan African woman presented with a three-month history of cough, fever, weight loss and drenching night sweats. Three weeks prior to admission she developed right sided chest pain and difficulty in breathing. On examination she had bilateral pleural effusions. Haematoxylin and eosin stained cytologic sections of the formalin-fixed paraffin-embedded cell block made from the pleural fluid were processed in the Department of Pathology, Makerere University, College of Health Sciences, Kampala, Uganda. Immunohistochemistry was done at the Institute of Haematology and Oncology "L and A Seragnoli", Bologna University School of Medicine, Bologna, Italy, using alkaline phosphatase anti-alkaline phosphatase method. In situ hybridization was used for detection of Epstein-Barr virus. The tumor cells were CD45+, CD30+, CD38+, HHV-8 LANA-1+; but were negative for CD3-, CD20-, CD19-, and CD79a- and EBV RNA+ on in situ hybridization. CD138 and Ki-67 were not evaluable. Our patient tested HIV positive and her CD4 cell count was 127/μL. Conclusions A definitive diagnosis of primary effusion lymphoma rests on finding a proliferation of large immunoblastic, plasmacytoid and anaplastic cells; HHV-8 in the tumor cells, an immunophenotype that is CD45+, pan B-cell marker negative and lymphocyte activated marker positive. It is essential for clinicians and pathologists to have a high index of suspicion of

  20. Primary retroperitoneal soft tissue sarcoma: Imaging appearances, pitfalls and diagnostic algorithm.

    Science.gov (United States)

    Messiou, C; Moskovic, E; Vanel, D; Morosi, C; Benchimol, R; Strauss, D; Miah, A; Douis, H; van Houdt, W; Bonvalot, S

    2017-07-01

    Although retroperitoneal sarcomas are rare tumours, they can be encountered by a wide variety of clinicians as they can be incidental findings on imaging or present with non specific symptoms and signs. Surgical resection can offer hope of cure and patient outcomes are improved when patients are managed in high-volume specialist centers. Failure to recognize retroperitoneal sarcomas on imaging can lead to inappropriate management in inexperienced centers. Therefore it is critical that a diagnosis of retroperitoneal sarcoma should be considered in the differential diagnosis of a retroperitoneal mass with prompt referral to a soft tissue sarcoma unit. In particular, the most common retroperitoneal sarcoma subtypes, liposarcoma and leiomyosarcoma, have characteristic imaging appearances which are discussed. This review therefore aims to set the context and guide clinicians through a diagnostic pathway for retroperitoneal masses in adults which arise extrinsic to the solid abdominal viscera. Copyright © 2016 The Authors. Published by Elsevier Ltd.. All rights reserved.

  1. Prevalence of and risk factors for otitis media with effusion in primary school children: case control study in Erzurum, Turkey.

    Science.gov (United States)

    Kucur, Cüneyt; Şimşek, Eda; Kuduban, Ozan; Özbay, İsa

    2015-01-01

    A total of 1,021 children attending 2 primary schools in districts in Erzurum were enrolled in a study evaluating the prevalence of otitis media with effusion (OME) and its relationship with various risk factors. The prevalence of OME in this study was 6.8% (69/1021). The difference in OME prevalence between age groups (9 years) was statistically significant (p0.05), consanguineous marriage (p>0.05) and history of hearing loss in the parents (p>0.05) were not statistically significant. Parents need to be informed about the symptoms of and risk factors for OME to avoid delayed diagnosis, which can lead to permanent hearing loss.

  2. Primary Vaginal Extraosseous Ewing Sarcoma/Primitive Neuroectodermal Tumor with Cranial Metastasis

    Directory of Open Access Journals (Sweden)

    Chi-Man Yip

    2009-06-01

    Full Text Available Extraosseous Ewing sarcoma is now regarded as a member of the Ewing sarcoma/primitive neuroectodermal tumor (PNET family. It typically involves the soft tissues of the chest wall, pelvis, paravertebral region, abdominal wall, retroperitoneal region and extremities of children, adolescents and young adults, but it seldom occurs in the female genital tract. We report an extremely rare case of retrospective diagnosis of vaginal extraosseous Ewing sarcoma/PNET which metastasized to the right frontoparietal scalp, skull, and dura. Surgical resection, followed by adjuvant radiotherapy and chemotherapy resulted in a favourable clinical outcome. Both the vaginal and head tumors had similar light microscopic features supporting the diagnosis.

  3. The efficacy of postoperative radiotherapy in localized primary soft tissue sarcoma treated with conservative surgery

    International Nuclear Information System (INIS)

    Zhao, Ru-Ping; Yu, Xiao-Li; Zhang, Zhen; Jia, Li-Juan; Feng, Yan; Yang, Zhao-Zhi; Chen, Xing-Xing; Wang, Jian; Ma, Sheng-Lin; Guo, Xiao-Mao

    2016-01-01

    To evaluate the efficacy of postoperative radiotherapy (RT) on local failure-free survival (LFFS), distant metastasis-free survival (DMFS) and overall survival (OS) in patients with localized primary soft tissue sarcoma (STS) and to identify prognostic factors. Between January 2000 and July 2010, 220 consecutive patients with localized primary STS, who received conservative surgery with or without postoperative RT, were enrolled in the study. Survival curves were constructed by the Kaplan-Meier method and log-rank test was used to assess statistical significance. Multivariate analysis was applied to identify the prognostic factors. After a median follow-up of 68 months (range, 5–127 months), the 5-year LFFS, DMFS and OS were 70.0, 78.2 and 71.2 %, respectively. Tumor size, histological subtypes, margin status and postoperative RT were independent predictors for OS. Postoperative RT was associated with a significant reduced local recurrence risk versus surgery alone (hazard ratio [HR] = 0.408, 95 % confidence interval [CI] 0.235–0.707, P = 0.001), with 5-year LFFS of 81.1 and 63.6 %, respectively (log-rank, P = 0.004). The log-rank test showed that postoperative RT had a tendency of improving OS compared with surgery alone, with 5-year OS of 74.8 and 65.0 %, respectively (P = 0.089). Multivariate analysis demonstrated that postoperative RT significantly reduced mortality rate compared with surgery alone (HR = 0.512, 95 % CI 0.296–0.886, p = 0.017), especially in patients with liposarcoma (p = 0.034). Postoperative radiotherapy reduce both local recurrence and STS mortality in patients with localized primary STS. The efficacy of RT on survival warrants further prospective study. The online version of this article (doi:10.1186/s13014-016-0605-y) contains supplementary material, which is available to authorized users

  4. Primary Ewing’s Sarcoma of the Kidney in a 73-Year-Old Man

    Directory of Open Access Journals (Sweden)

    T. B. Wedde

    2011-01-01

    Full Text Available Objective. Ewing’s sarcoma of the kidney is rare and is usually found in young adults. We present here a single case study of Ewing's sarcoma found in an elderly man. Material and methods. A 73-year-old man underwent routine surgery for hydrocoele of the testis. He developed urinary obstruction symptoms, and radiological examinations revealed a tumour in the right kidney. Results. Microscopical, immunohistochemical, and molecular pathological analysis of the tumour was consistent with Ewing's sarcoma. FISH showed rearrangement of chromosomes 22q12 (EWSR1. The patient subsequently underwent nephrectomy followed by 6 adjuvant chemotherapy cycles. Follow-up after 7 months shows no recurrence. Conclusion. This case report presents not only the rare finding of Ewing's sarcoma in the kidney, but also the occurrence of this tumour entity in an elderly patient. Treatment options for the different types of renal tumours are vastly different and the need for a correct diagnosis is, therefore, vital.

  5. Synovial sarcoma of primary bone origin: a rare case in a rare site with atypical features

    International Nuclear Information System (INIS)

    Jung, Seung Chai; Choi, Jung-Ah; Lee, Joon Woo; Kang, Heung Sik; Chung, Jin-Haeng; Oh, Joo Han

    2007-01-01

    Synovial sarcoma of bone origin is extremely rare and difficult to diagnose. We present a case in which the lesion arose in the cortex of the distal tibia. It showed heterogeneous intermediate signal intensity on T1-weighted images and heterogeneous intermediate to low signal intensity on T2-weighted images with heterogeneous contrast enhancement on MRI. The lesion was confirmed as synovial sarcoma using a combination of histological and molecular genetic studies. (orig.)

  6. Hemostatic findings of pleural fluid in dogs and the association between pleural effusions and primary hyperfibrino(genolysis: A cohort study of 99 dogs.

    Directory of Open Access Journals (Sweden)

    Andrea Zoia

    Full Text Available The primary objective of this study was to determine if activation of coagulation and fibrinolysis occurs in canine pleural effusions. Thirty-three dogs with pleural effusions of different origin were studied. Pleural effusion fibrinogen concentrations were significantly lower, while pleural fibrin-fibrinogen degradation products (FDPs and D-dimer concentrations were significantly higher than those in plasma (P < 0.001 for all comparisons. These results show that, in canine pleural fluids, there is evidence of coagulation activation and fibrinolysis. The secondary aims of the current study were to determine if primary hyperfibrinolysis ([PHF] i.e., elevated plasma FDPs with a normal D-dimer concentrations, occurs in dogs with pleural effusion, and whether the presence of a concurrent inflammatory process may have activated the hemostatic cascade, with its intrinsically linked secondary hyperfibrinolysis, masking the concurrent PHF. The previously 33 selected dogs with pleural effusion (group 1 were compared to two control groups of 33 healthy (group 2 and 33 sick dogs without pleural effusion (group 3. Serum fibrinogen, FDPs, D-dimer, C-reactive protein (CRP, fibrinogen/CRP ratio, and frequency of PHF were determined. Fibrinogen, FDPs, D-dimer and CRP concentrations in group 1 were significantly increased compared to group 2 (P < 0.001 for all comparisons. FDPs and CRP concentrations in group 1 were also significantly increased compared to group 3 (P = 0.001 and P < 0.001, respectively. The fibrinogen/CRP ratio was significantly decreased in group 1 compared to groups 2 and 3 (P < 0.001 for both comparison. The frequency of PHF was significantly higher in group 1 compared to groups 2 (P = 0.004, but not compared to group 3. These results support the hypothesis that PHF occurs significantly more often in dogs with pleural effusion compared to healthy dogs. Nevertheless, the decrease in the fibrinogen/CRP ratio in group 1 compared to group 3

  7. 18F-fluorodeoxyglucose positron emission tomography and magnetic resonance imaging findings of primary intracranial histiocytic sarcoma in a dog

    International Nuclear Information System (INIS)

    Kang, B.T.; Park, C.; Yoo, J.H.; Gu, S.H.; Jang, D.P.; Kim, Y.B.; Woo, E.J.; Kim, D.Y.; Cho, Z.H.; Park, H.M.

    2009-01-01

    A 10-year-old, neutered male, Maltese dog presented with a three week history of intention tremor, right hind limb rigidity, poor coordination, and occasional circling to the left. On magnetic resonance imaging (MRI) of the brain, a mass was identified in the right occipital lobe and cerebellum. Three weeks after the initial MRI scan, we performed an sup(18)F-fluorodeoxyglucose positron emission tomography (FDG-PET) of the brain. The FDG-PET demonstrated areas of hypermetabolism in the right occipital lobe, cerebellum, pons, and medulla oblongata. When the standardized uptake value was calculated, the hypermetabolic lesion was higher than the gray matter values. The anatomical location of the hypermetabolic lesion was more precisely identified by the PET-MRI fusion images. The dog was definitively diagnosed as a primary histiocytic sarcoma of the brain. This is the first report of PET findings of an intracranial histiocytic sarcoma in a dog

  8. Recent advances in understanding Kaposi’s sarcoma-associated herpesvirus [version 1; referees: 2 approved

    Directory of Open Access Journals (Sweden)

    Nathan J. Dissinger

    2016-04-01

    Full Text Available Kaposi’s sarcoma (KS-associated herpesvirus (KSHV is an oncogenic human herpesvirus. KSHV is associated with three cancers in the human population: KS, primary effusion lymphoma (PEL, and multicentric Castleman’s disease (MCD. KS is the leading cause of cancer in HIV-infected individuals. In this review, we discuss the most recent discoveries behind the mechanisms of KSHV latency maintenance and lytic replication. We also review current therapies for KSHV-associated cancers.

  9. Ewing sarcoma

    International Nuclear Information System (INIS)

    Hamanoue, Satoshi; Makimoto, Atsushi

    2007-01-01

    Ewing sarcoma is the second most frequent primary bone cancer affecting children or young adults. Advances in molecular biology have revealed common chromosomal translocations such as EWS-FLI1 among Ewing sarcoma and related diseases such as primitive neuroectodermal tumor (PNET), so these are considered as Ewing sarcoma family tumor (ESFT). Although fewer than 10% of patients with ESFT survived before establishment of modern multiagent chemotherapy, the multimodal therapeutic regimens including combination chemotherapy, radiotherapy, and surgery can cure 60% of patients with localized disease, due to the collaborative research in European-American or the international trials. The standard chemotherapy for localized ESFT now comprises vincristine, actinomycin D, cyclophosphamide and doxorubicin (VACD) in Europe or vincristine, doxorubicin, cyclophosphamide, ifosfamide and etoposide (VDC-IE) in North America. Meanwhile, those with metastatic disease have a much worse outcome with an approximately 10-30% 5-year event-free survival rate. New American-European collaborative trials such as EURO-E.W.I.N.G. 99 are in progress for further improvement of the cure rate in localized and metastatic ESFT. In Japan, Japan Ewing Sarcoma Study Group (JESS) phase II clinical trial for localized ESFT, and some clinical trials including new drugs are ongoing and waiting for results. (author)

  10. Management of malignant pleural effusions.

    LENUS (Irish Health Repository)

    Uzbeck, Mateen H

    2010-06-01

    Malignant pleural effusions are a common clinical problem in patients with primary thoracic malignancy and metastatic malignancy to the thorax. Symptoms can be debilitating and can impair tolerance of anticancer therapy. This article presents a comprehensive review of pharmaceutical and nonpharmaceutical approaches to the management of malignant pleural effusion, and a novel algorithm for management based on patients\\' performance status.

  11. Targeted therapy for sarcomas

    Directory of Open Access Journals (Sweden)

    Forscher C

    2014-03-01

    Full Text Available Charles Forscher,1 Monica Mita,2 Robert Figlin3 1Sarcoma Program, Samuel Oschin Comprehensive Cancer Institute, Cedars-Sinai Medical Center, Los Angeles, CA, USA; 2Experimental Therapeutics Program, Samuel Oschin Comprehensive Cancer Institute, Cedars-Sinai Medical Center, Los Angeles, CA, USA; 3Academic Development Program, Samuel Oschin Comprehensive Cancer Institute, and Division of Hematology/Oncology, Cedars-Sinai Medical Center, Los Angeles, CA, USA Abstract: Sarcomas are tumors of mesenchymal origin that make up approximately 1% of human cancers. They may arise as primary tumors in either bone or soft tissue, with approximately 11,280 soft tissue tumors and 2,650 bone tumors diagnosed each year in the United States. There are at least 50 different subtypes of soft tissue sarcoma, with new ones described with ever-increasing frequency. One way to look at sarcomas is to divide them into categories on the basis of their genetic make-up. One group of sarcomas has an identifiable, relatively simple genetic signature, such as the X:18 translocation seen in synovial sarcoma or the 11:22 translocation seen in Ewing's sarcoma. These specific abnormalities often lead to the presence of fusion proteins, such as EWS-FLI1 in Ewing's sarcoma, which are helpful as diagnostic tools and may become therapeutic targets in the future. Another group of sarcomas is characterized by complex genetic abnormalities as seen in leiomyosarcoma, osteosarcoma, and undifferentiated sarcoma. It is important to keep these distinctions in mind when contemplating the development of targeted agents for sarcomas. Different abnormalities in sarcoma could be divided by tumor subtype or by the molecular or pathway abnormality. However, some existing drugs or drugs in development may interfere with or alter more than one of the presented pathways. Keywords: sarcoma, targeted agents, tyrosine kinase inhibitors, mTor inhibition

  12. Outcome of primary soft tissue sarcoma of the knee and elbow

    International Nuclear Information System (INIS)

    Alektiar, Kaled M.; McKee, Andrea B.; Jacobs, Jordan M.; McKee, Brady J.; Healey, John H.; Brennan, Murray F.

    2002-01-01

    Purpose: Concern is frequently raised regarding the tolerance of irradiation over a joint space. The purpose of this study was to determine the outcome in terms of relapse and potential complications in patients with knee and elbow soft tissue sarcoma treated with limb-sparing surgery with or without adjuvant radiotherapy (RT). Methods and Materials: A review of our prospective database between June 1982 and December 1999 identified 86 adult patients with primary soft tissue sarcoma arising from the knee (n=65; 76%) or elbow (n=21; 24%) treated with limb-sparing surgery. Tumors had high-grade histologic features in 72% and were >5 cm in 48% of patients. Adjuvant RT was given to 46 (54%) of 86 patients. The type of RT was postoperative external beam RT in 63% and brachytherapy in 37%. Of the 46 patients who received RT, 85% (n=39) had deep, 78% (n=36) high-grade, and 54% (n=25) >5-cm tumors. Complications were assessed in terms of wound complications requiring repeated surgery, bone fracture, nerve damage, and joint stiffness. Results: With a median follow-up of 48 months (range 4-175), the 5-year actuarial rate of local control, distant control, and overall survival was 75% (95% confidence interval [CI] 64-85%), 82% (95% CI 73-91%), and 81% (95% CI 71-91%), respectively. The 5-year local control rate for patients who received RT was 80% vs. 71% for those who did not (p=0.3). The type of RT did not significantly influence the local control rate. Patients treated with external beam RT had a 5-year local control rate of 84% compared with 73% for those treated with brachytherapy (p=0.4). On multivariate analysis, tumor size >5 cm retained its significance as an independent predictor of poor local control (p=0.04; relative risk 3; 95% CI 1-6). In addition, high-grade histologic features emerged as an independent predictor of local recurrence (p=0.02; relative risk 4; 95% CI 1-20). No statistically significant difference was found between the RT and no-RT group in terms

  13. Breast sarcomas. Literature review

    Directory of Open Access Journals (Sweden)

    D. A. Ryabchikov

    2014-01-01

    Full Text Available The article presents an overview of the literature about breast sarcomas (nonepithelial malignances. Primary sarcomas are extremely rare, with less than 1 % of all malignant tumors of the breast. Breast carcinomas cause an increased interest of the scientists due to their unique clinical and pathological features and unpredictable prognosis.

  14. Chemokines in Ewing sarcoma

    NARCIS (Netherlands)

    Sand, L.G.L.

    2016-01-01

    Ewing sarcoma is an aggressive primary malignant bone tumor with high degree of tumor vascularization and is the second most common sarcoma of bone in children and young adults. Patients with disseminated disease at diagnosis or early relapse have a poor prognosis. To identify novel therapies and

  15. A rare cause of pleural effusion: ruptured primary pleural hydatid cyst.

    Science.gov (United States)

    Erkoç, Mustafa Fatih; Öztoprak, Bilge; Alkan, Sevil; Okur, Aylin

    2014-03-06

    Hydatidosis is an endemic parasitic disease in Mediterranean countries, often caused by the dog tapeworm Echinococcus granulosus. The disease predominantly affects the liver (60-70%) and lungs (30%), and the surgical management is considered as the gold standard for treatment. Besides anaphylactic reactions, the most frequent complication of the hydatid disease is rupture into neighbouring structures, often affecting the bronchi, gastrointestinal tract and peritoneal/pleural cavities, according to its location. Primary pleural hydatidosis is an extremely rare entity and we present a ruptured pleural hydatid cyst with unusual location.

  16. 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

  17. Imaging features of primary Sarcomas of the great vessels in CT, MRI and PET/CT: a single-center experience

    International Nuclear Information System (INIS)

    Falck, Christian von; Meyer, Bernhard; Fegbeutel, Christine; Länger, Florian; Bengel, Frank; Wacker, Frank; Rodt, Thomas

    2013-01-01

    To investigate the imaging features of primary sarcomas of the great vessels in CT, MRI and 18 F-FDG PET/CT. Thirteen patients with a primary sarcoma of the great vessels were retrospectively evaluated. All available images studies including F-18 FDG PET(/CT) (n = 4), MDCT (n = 12) and MRI (n = 6) were evaluated and indicative image features of this rare tumor entity were identified. The median interval between the first imaging study and the final diagnosis was 11 weeks (0–12 weeks). The most frequently observed imaging findings suggestive of malignant disease in patients with sarcomas of the pulmonary arteries were a large filling defect with vascular distension, unilaterality and a lack of improvement despite effective anticoagulation. In patients with aortic sarcomas we most frequently observed a pedunculated appearance and an atypical location of the filling defect. The F-18 FDG PET(/CT) examinations demonstrated an unequivocal hypermetabolism of the lesion in all cases (4/4). MRI proved lesion vascularization in 5/6 cases. Intravascular unilateral or atypically located filling defects of the great vessels with vascular distension, a pedunculated shape and lack of improvement despite effective anticoagulation are suspicious for primary sarcoma on MDCT or MRI. MR perfusion techniques can add information on the nature of the lesion but the findings may be subtle and equivocal. F-18 FDG PET/CT may have a potential role in these patients and may be considered as part of the imaging workup

  18. Primary Ewing’s sarcoma of the kidney: A case report

    Directory of Open Access Journals (Sweden)

    Faris Alasmari

    Full Text Available This is a 15-year-old female who presented with sudden onset left flank pain associated with nausea and vomiting and a history of weight loss. Radiological investigation revealed a large non-obstructive tumor involving the lower pole of the left kidney which was primarily thought to be a renal cell carcinoma. She underwent left open radical nephrectomy with adrenalectomy. Histopathology of the resected specimen showed features of Ewing’s sarcoma of the kidney which was confirmed by cytogenetic analysis. This is a rare disease especially in the pediatric group and in reporting such a rare case we hope it helps in identifying a potential course of the disease and its response to the involved treatment. Keywords: Case report, Ewing’s sarcoma, Kidney, Tumor, Nephrectomy

  19. Kaposi’s Sarcoma-associated herpesvirus microRNAs

    Directory of Open Access Journals (Sweden)

    Eva eGottwein

    2012-05-01

    Full Text Available Kaposi’s Sarcoma-associated herpesvirus (KSHV is a human pathogenic -herpesvirus strongly associated with the development of Kaposi’s Sarcoma and B cell proliferative disorders, including primary effusion lymphoma (PEL. The identification and functional investigation of non-coding RNAs expressed by KSHV is a topic with rapidly emerging importance. KSHV miRNAs derived from 12 stem-loops located in the major latency locus have been the focus of particular attention. Recent studies describing the transcriptome-wide identification of mRNA targets of the KSHV miRNAs suggest that these miRNAs have evolved a highly complex network of interactions with the cellular and viral transcriptomes. Relatively few KSHV miRNA targets, however, have been characterized at a functional level. Here, our current understanding of KSHV miRNA expression, targets and function will be reviewed.

  20. Alveolar soft part sarcoma: the new primary intracranial malignancy : A case report and review of the literature.

    Science.gov (United States)

    Kumar, Aditaya; Alrohmain, B; Taylor, W; Bhattathiri, P

    2017-07-26

    The purpose of this paper is to serve as a reference to aid in the management of this poorly understood intracranial malignancy. The authors report their experience treating the eighth ostensible case of a primary intracranial alveolar soft part sarcoma (ASPS). A 21-year-old man presented to hospital after collapsing. He gave a 1-year history of headache, a 2-month history of reduced visual acuity and on examination had left facial paraesthesia with left-sided incoordination. MRI of the brain revealed a large left posterior fossa mass. The patient underwent resection of the tumour with good recovery in function. Immunohistochemical analysis of the tumour specimen confirmed an ASPS, and multimodal imaging in search of an extra-cranial disease primary was negative. A review of the literature yielded only seven other cases of primary intracranial ASPS. A variety of diagnostic imaging modalities were employed in search of a disease primary, as were various combinations of surgical resection, chemotherapy and radiotherapy as treatment. Half of the cases documented delayed disease recurrence. The authors discuss the following: the unique radiological and immunohistological characteristics of this disease including the potential for its misdiagnosis; the investigations required to diagnose a primary intracranial ASPS; the efficacy of current medical and surgical treatment options and the factors that will aid in prognostication. This is the first review of this new primary intracranial malignancy. From our analysis, we offer a joint radiological and immunohistochemical algorithm for the diagnosis of primary intracranial ASPS and specific operative considerations prior to resection.

  1. Postirradiation sarcoma in retinoblastoma. Induction or predisposition

    International Nuclear Information System (INIS)

    Schwarz, M.B.; Burgess, L.P.; Fee, W.E. Jr.; Donaldson, S.S.

    1988-01-01

    An alarmingly high rate of postirradiation sarcomas following treatment for retinoblastoma has been described in the literature. We present four new cases and report 57 others from the English literature. Osteogenic sarcoma was the predominant histologic type (58%), followed by fibrosarcoma (21%) and various other sarcomas (21%). The average latency period between irradiation and development of the second primary (sarcoma) was 12.4 years. Irrespective of irradiation, a genetic linkage between retinoblastoma and osteogenic sarcoma on the 13q14 chromosome is recognized. Through a pleiotropic effect of this same chromosome, a predisposition for other sarcomas may exist as well. Finally, a strong role for radiation induction is proposed for all of these postirradiation sarcomas. This is based on the increased number of sarcomas arising in the field of prior irradiation (sites uncharacteristic of spontaneously occurring primary sarcomas) and the prolonged latency periods.13 references

  2. Ewing sarcoma

    Science.gov (United States)

    Bone cancer - Ewing sarcoma; Ewing family of tumors; Primitive neuroectodermal tumors (PNET); Bone neoplasm - Ewing sarcoma ... to the lungs and other bones. At the time of diagnosis, spread is seen in about one- ...

  3. Parapneumonic pleural effusion

    Science.gov (United States)

    Pleural effusion - pneumonia ... Pneumonia, most commonly from bacteria, causes parapneumonic pleural effusion. ... Call your provider if you have symptoms of pleural effusion. Call your provider or go to the emergency ...

  4. Kaposi's Sarcoma

    Science.gov (United States)

    ... Name: Category: Share: Yes No, Keep Private Kaposi’s Sarcoma Share | Kaposi’s sarcoma (KS) is a vascular neoplasm of the skin ... symptoms of HIV infection. This type of Kaposi's sarcoma progresses slowly, with new lesions appearing every few ...

  5. Limb salvage with microvascular free fibula following primary bone sarcoma resection

    Directory of Open Access Journals (Sweden)

    Sahasrabudhe Parag

    2016-01-01

    Full Text Available Background: Extremity sarcomas are challenging to manage. Total eradication of tumour has to be balanced with restoration of limb function to prevent mortality and morbidity. Disease-free survival with maximum limb function is the ultimate goal in these patients. Materials and Methods: We present a series of ten cases of extremity malignancies, where limb salvage was attempted with microvascular free fibula for limb reconstruction from the period of 2008 to 2015. Results: Of the ten cases in the study, there were two females and eight males. There were nine patients with lower limb malignancies and one patient with upper limb malignancy. There were four patients with Ewing's sarcoma of femur, five patients with osteosarcoma of femur and one patient with chondrosarcoma of the humerus. The follow-up period ranged from 1.2 to 6.2 years with mean follow-up of 3.1 years. There were two deaths during follow-up, both were due to distant metastasis. The assessment of the function was done on the basis of Musculoskeletal Tumour Society functional score. Maximum score was 30 and minimum score was 24, the average score being 26. Of the eight surviving patients, three patients had full weightbearing, four patients had partial weightbearing at end of 2 years and one patient of upper limb reconstruction had complete upper limb function. None of the patients had to undergo limb amputation. Conclusion: Limb salvage with vascularised fibula graft offers good functional outcome along with good disease-free survival rates.

  6. Pleural effusion

    International Nuclear Information System (INIS)

    Jimenez Q, Andres; Camacho D, Fidel

    2009-01-01

    The pleural effusion is defined as the abnormal accumulation of liquid in the pleural space that produces to itself for increase in the production or decrease of the drainage, common reasons in clinics disorders. Inside the reasons of the increase of the production we can enunciate an increase of the capillary pleural permeability, decrease of the oncotic pressure capillary and increase of the hydrostatic capillary pressure, there are less clear the reasons of the decrease of the drainage but are outlined the alteration of the lymphatic pleural contractibility , infiltration of vessels and lymphatic nodules for neoplasia diseases and alterations to pleural level that they should prevent that the this liquid touch the lymphatic pores. The objective of this review is the analysis of the physiological bases of the pleura and the production of the pleural liquid, the physiological aspects of the pleural effusion, the approach of the diagnose and the medical and surgical managing of the same one for the different reasons that produce it.

  7. Primary intracranial soft tissue sarcomas in children, adolescents, and young adults: single institution experience and review of the literature.

    Science.gov (United States)

    Maher, Ossama M; Khatua, Soumen; Mukherjee, Devashis; Olar, Adriana; Lazar, Alexander; Luthra, Raja; Liu, Diane; Wu, Jimin; Ketonen, Leena; Zaky, Wafik

    2016-03-01

    There is a paucity of literature reporting the outcome of intracranial sarcomas (IS) in children, adolescents, and young adults (CAYA). A multimodal therapeutic approach is commonly used, with no well-established treatment consensus. We conducted a retrospective review of CAYA with IS, treated at our institution, to determine their clinical findings, treatments, and outcomes. Immunohistochemistry (PDGFRA and EGFR) and DNA sequencing were performed on 5 tumor samples. A literature review of IS was also conducted. We reviewed 13 patients (median age, 7 years) with a primary diagnosis of IS between 1990 and 2015. Diagnoses included unclassified sarcoma (n = 9), chondrosarcoma (n = 2), and rhabdomyosarcoma (n = 2). Five patients underwent upfront gross total resection (GTR) of the tumor. The 5-drug regimen (vincristine, doxorubicin, cyclophosphamide, etoposide, and ifosfamide) was the most common treatment used. Nine patients died due to progression or recurrence (n = 8) or secondary malignancy (n = 1). The median follow-up period of the 4 surviving patients was 1.69 years (range 1.44-5.17 years). The 5-year progression-free survival and overall survival rates were 21 and 44 %, respectively. BRAF, TP53, KRAS, KIT, ERBB2, MET, RET, ATM, and EGFR mutations were detected in 4 of the 5 tissue samples. All 5 samples were immunopositive for PDGFRA, and only 2 were positive for EGFR. IS remain a therapeutic challenge due to high progression and recurrence rates. Collaborative multi-institutional studies are warranted to delineate a treatment consensus and investigate tumor biology to improve the disease outcome.

  8. Bone sarcomas

    International Nuclear Information System (INIS)

    Mudry, P.

    2008-01-01

    Bone sarcomas are malignancies with peak incidence in adolescents and young adults. The most frequent are osteosarcoma and Ewing sarcoma/PNET, in an older adults are seen chondrosarcomas, other ones are rare. In general, biology of sarcomas is closely related to pediatric malignancies with fast growth, local aggressiveness, tendency to early hematogenic dissemination and chemo sensitivity. Diagnostics and treatment of bone sarcomas should be done in well experienced centres due to low incidence and broad issue of this topic. An interdisciplinary approach and staff education is essential in due care of patients with bone sarcoma. If these criteria are achieved, the cure rate is contemporary at 65 - 70 %, while some subpopulation of patients has chance for cure up to 90 %. Osteosarcoma and Ewing sarcoma/PNET are discussed below as types of most frequent bone sarcoma. (author)

  9. Primary synovial sarcoma of the thyroid gland: case report and review of the literature.

    Science.gov (United States)

    Boudin, Laurys; Fakhry, Nicolas; Chetaille, Bruno; Perrot, Delphine; Nguyen, Anh Tuan; Daidj, Nassima; Guiramand, Jérôme; Sarran, Anthony; Moureau-Zabotto, Laurence; Bertucci, François

    2014-01-01

    Synovial sarcoma (SVS) of the thyroid gland is exceedingly rare. We report the case of a 55-year-old man with a rapidly growing 7-cm neck mass. Because of suspicion of anaplastic thyroid carcinoma, a total thyroidectomy was planned, without preoperative cytology. During surgery, the tumor ruptured, leading to fragmented and incomplete resection. The morphological and immunohistochemical aspects suggested thyroid SVS, which was confirmed by fluorescent in situ hybridization (SYT gene rearrangement). The patient experienced immediate local relapse in close contact with large vessels and the thyroid cartilage and was referred to our institution. Doxorubicin-ifosfamide chemotherapy led to a minor response that authorized secondary conservative surgery. Because of microscopically incomplete resection, adjuvant radiotherapy was chosen and is ongoing 10 months after initial surgery. The prognosis of thyroid SVS is associated with a high risk for local and metastatic relapses. Pretreatment diagnosis is fundamental and may benefit from molecular analysis. Margin-free monobloc surgical excision is the best chance for cure, but adjuvant chemotherapy and radiotherapy deserve to be discussed.

  10. Primary Synovial Sarcoma of the Thyroid Gland: Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Laurys Boudin

    2014-01-01

    Full Text Available Synovial sarcoma (SVS of the thyroid gland is exceedingly rare. We report the case of a 55-year-old man with a rapidly growing 7-cm neck mass. Because of suspicion of anaplastic thyroid carcinoma, a total thyroidectomy was planned, without preoperative cytology. During surgery, the tumor ruptured, leading to fragmented and incomplete resection. The morphological and immunohistochemical aspects suggested thyroid SVS, which was confirmed by fluorescent in situ hybridization (SYT gene rearrangement. The patient experienced immediate local relapse in close contact with large vessels and the thyroid cartilage and was referred to our institution. Doxorubicin-ifosfamide chemotherapy led to a minor response that authorized secondary conservative surgery. Because of microscopically incomplete resection, adjuvant radiotherapy was chosen and is ongoing 10 months after initial surgery. The prognosis of thyroid SVS is associated with a high risk for local and metastatic relapses. Pretreatment diagnosis is fundamental and may benefit from molecular analysis. Margin-free monobloc surgical excision is the best chance for cure, but adjuvant chemotherapy and radiotherapy deserve to be discussed.

  11. Primary histiocytic sarcoma arising in the head and neck with predominant spindle cell component

    Directory of Open Access Journals (Sweden)

    Zhao XF

    2007-02-01

    Full Text Available Abstract This is the first case report of Histiocytic Sarcoma (HS with predominant spindle cell component occurring in the head and neck region of a 41-year-old man. The tumor was composed of sheets of large round to oval cells with pleomorphic vesicular nuclei, prominent nucleoli and abundant eosinophilic cytoplasm. Multinucleated forms, numerous mitoses, and tumor necrosis were also noted. Sheets, fascicles, and whorls of spindle cells with spindled to ovoid vesicular nuclei, small to medium-sized distinct nucleoli, and eosinophilic cytoplasm were frequently observed. Immunohistochemical staining in the tumor cells was positive for CD163, CD68, lysozyme, CD45, and NSE. Focal expression of CD4 and S-100 was also noted. Electron microscopy demonstrated an abundance of lysosomes in the cytoplasm of tumor cells. Chromosome study revealed a 57–80 hyperdiploid [7]/46, XY [13] karyotype, including 3 to 4 copies of various chromosomes. The immunohistochemical and ultrastructural findings confirmed the diagnosis of HS.

  12. Primary Ewing’s Sarcoma of the Sinonasal Tract: A Case Report

    Directory of Open Access Journals (Sweden)

    Tomoharu Suzuki

    2017-01-01

    Full Text Available A 23-year-old male presented with a 3-month history of left purulent rhinorrhea, progressive nasal obstruction, and intermittent epistaxis. A fiberoptic examination revealed a large vascular polypoid mass completely filling the left nasal cavity. CT and MRI scans showed a large hypervascular mass involving the left nasal airway, maxillary antrum, and the anterior ethmoid cells. There was no bony erosion or contiguous spread, and the remaining sinuses, orbit, and cranial fossa were uninvolved. The patient underwent complete removal of the mass via an external lateral rhinotomy approach. The soft mass was large and vascular. A microscopic analysis revealed an undifferentiated tumor consisting of a solid sheet of small, round blue cells. Mitotic figures were also present. Immunohistochemically, the tumor cells were strongly positive for CD99. Molecular studies using a PCR confirmed the chromosomal translocation of FLI1 (exon 6. These findings were considered diagnostic for Ewing’s sarcoma. Postoperatively, the patient was treated with combined chemotherapy and radiotherapy. Adjuvant chemotherapy consisting of vincristine, doxorubicin, and cyclophosphamide alternating with ifosfamide and etoposide (total: 7 cycles was commenced. He also received radiation therapy for local control (total dose: 50.4 Gy. The patient is currently alive without any evidence of recurrence or metastasis.

  13. DNA Methylation and Gene Expression Profiling of Ewing Sarcoma Primary Tumors Reveal Genes That Are Potential Targets of Epigenetic Inactivation

    Directory of Open Access Journals (Sweden)

    Nikul Patel

    2012-01-01

    Full Text Available The role of aberrant DNA methylation in Ewing sarcoma is not completely understood. The methylation status of 503 genes in 52 formalin-fixed paraffin-embedded EWS tumors and 3 EWS cell lines was compared to human mesenchymal stem cell primary cultures (hMSCs using bead chip methylation analysis. Relative expression of methylated genes was assessed in 5-Aza-2-deoxycytidine-(5-AZA-treated EWS cell lines and in a cohort of primary EWS samples and hMSCs by gene expression and quantitative RT-PCR. 129 genes demonstrated statistically significant hypermethylation in EWS tumors compared to hMSCs. Thirty-six genes were profoundly methylated in EWS and unmethylated in hMSCs. 5-AZA treatment of EWS cell lines resulted in upregulation of expression of hundreds of genes including 162 that were increased by at least 2-fold. The expression of 19 of 36 candidate hypermethylated genes was increased following 5-AZA. Analysis of gene expression from an independent cohort of tumors confirmed decreased expression of six of nineteen hypermethylated genes (AXL, COL1A1, CYP1B1, LYN, SERPINE1, and VCAN. Comparing gene expression and DNA methylation analyses proved to be an effective way to identify genes epigenetically regulated in EWS. Further investigation is ongoing to elucidate the role of these epigenetic alterations in EWS pathogenesis.

  14. A Unique Case of Primary Ewing’s Sarcoma of the Cervical Spine in a 53-Year-Old Male: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Marshall T. Holland

    2015-01-01

    Full Text Available Extraskeletal Ewing’s sarcoma (EES is a rare presentation, representing only 15% of all primary Ewing’s sarcoma cases. Even more uncommon is EES presenting as a primary focus in the spinal canal. These rapidly growing tumors often present with focal neurological symptoms of myelopathy or radiculopathy. There are no classic characteristic imaging findings and thus the physician must keep a high index of clinical suspicion. Diagnosis can only be definitively made by histopathological studies. In this report, we discuss a primary cervical spine EES in a 53-year-old man who presented with a two-month history of left upper extremity pain and acute onset of weakness. Imaging revealed a cervical spinal canal mass. After undergoing cervical decompression, histopathological examination confirmed a diagnosis of Ewing’s sarcoma. A literature search revealed fewer than 25 reported cases of primary cervical spine EES published in the past 15 years and only one report demonstrating this pathology in a patient older than 30 years of age age=38. Given the low incidence of this pathology presenting in this age group and the lack of treatment guidelines, each patient’s plan should be considered on a case-by-case basis until further studies are performed to determine optimal evidence based treatment.

  15. Front-line window therapy with cisplatin in patients with primary disseminated Ewing sarcoma: A study by the Associazione Italiana di Ematologia ed Oncologia Pediatrica and Italian Sarcoma Group.

    Science.gov (United States)

    Luksch, Roberto; Grignani, Giovanni; D'Angelo, Paolo; Prete, Arcangelo; Puma, Nadia; Podda, Marta; Casanova, Michela; Ferrari, Andrea; Morosi, Carlo; Fagioli, Franca; Aglietta, Massimo; Ferrari, Stefano; Picci, Piero; Massimino, Maura

    2017-12-01

    The aim was to assess the activity of cisplatin (CDDP) in Ewing sarcoma (ES). The study consisted of front-line window therapy with CDDP 120 mg/sqm every 3 weeks for two courses in children and young adults with primary disseminated ES. Response was assessed using the Response Evaluation Criteria in Solid Tumours criteria, and Simon's two-stage design was applied. Twelve consecutive patients were enrolled in stage 1. Only one objective response was observed. Since the target response rate was not achieved, accrual was stopped and CDDP as a single agent in ES was judged unworthy of further assessment. © 2017 Wiley Periodicals, Inc.

  16. Kaposi's sarcoma

    International Nuclear Information System (INIS)

    Kirova, Y.M.; Belembaogo, E.; Frikha, H.; Yu, S.J.; Le Bourgeois, J.P.

    1997-01-01

    Moriz Kaposi was the first who, in 1872, described five patients presenting with 'sarcoma idiopathicum multiple hemorrhagicum'. In 1912 Sternberg termed this disease Kaposi's sarcoma. Since then various forms of this rare disease have been observed. In 1914 Hallenberg described the first cases of African or endemic Kaposi's sarcoma. In the 1960's the first reports discussing Kaposi's sarcoma following organ transplantation and immunosuppressive therapy were published. After 1981, the epidemic form associated with the acquired immunodeficiency syndrome (AIDS) was described. All these forms, their history, treatment methods and the role of radiation therapy in the management of this rare malignancy are discussed, and the literature is reviewed. (authors)

  17. Imaging Ewing's sarcoma

    International Nuclear Information System (INIS)

    Henk, C.B.; Grampp, S.; Kainberger, F.; Breitenseher, M.; Imhof, H.; Mostbeck, G.H.

    1998-01-01

    Ewing's sarcoma is a highly malignant neoplasm of the bone whose origin is still uncertain. A strong relationship exists between Ewing's sarcoma and tumors of neural origin (Ewing family of tumors). Ewing's sarcoma must be distinguished from other round-cell tumors like lymphoma and neuroblastoma and also must be differentiated from osteogenic sarcomas. On plain radiographs, Ewing's sarcoma appears as a lytic or mixed lytic-sclerotic, rarely as predominantly sclerotic lesion with margins Lodwick grade III. It is located primarily in the diaphyseal and metadiaphyseal regions of the long bones of the lower extremities. A large soft tissue tumor is usually present. Magnetic resonance imaging is the imaging modality of choice to evaluate the extent of the primary lesion, to monitor the response to neoadjuvant chemotherapy and to follow up non-resected Ewing's sarcomas. Bone scintigraphy is necessary to detect skeletal metastasis, and 201 thallium scanning has been shown to be sensitive in the monitoring of treatment response. Today, computed tomography is not longer used to image the tumor site; however, spiral CT of the lungs plays a central role as a staging and follow-up tool. (orig.) [de

  18. Ewing's Sarcoma Localized in the Mandible: A Case Report

    OpenAIRE

    Akbayram, S; Başaranoglu, M; Kaya, A; Açıkgöz, M; Üstyol, L; Taşkın, GA; Dogan, M

    2015-01-01

    Ewing's sarcoma is one of the most aggressive primary bone tumours. Ewing's sarcoma arising from the bones of the head and neck region is extremely rare; only 4–9% of all Ewing's sarcoma originate in this region. We report a case of Ewing's sarcoma localized in the mandible because of its unusual presentation.

  19. Dual-Energy Micro-Computed Tomography Imaging of Radiation-Induced Vascular Changes in Primary Mouse Sarcomas

    International Nuclear Information System (INIS)

    Moding, Everett J.; Clark, Darin P.; Qi, Yi; Li, Yifan; Ma, Yan; Ghaghada, Ketan; Johnson, G. Allan; Kirsch, David G.; Badea, Cristian T.

    2013-01-01

    Purpose: To evaluate the effects of radiation therapy on primary tumor vasculature using dual-energy (DE) micro-computed tomography (micro-CT). Methods and Materials: Primary sarcomas were generated with mutant Kras and p53. Unirradiated tumors were compared with tumors irradiated with 20 Gy. A liposomal-iodinated contrast agent was administered 1 day after treatment, and mice were imaged immediately after injection (day 1) and 3 days later (day 4) with DE micro-CT. CT-derived tumor sizes were used to assess tumor growth. After DE decomposition, iodine maps were used to assess tumor fractional blood volume (FBV) at day 1 and tumor vascular permeability at day 4. For comparison, tumor vascularity and vascular permeability were also evaluated histologically by use of CD31 immunofluorescence and fluorescently-labeled dextrans. Results: Radiation treatment significantly decreased tumor growth from day 1 to day 4 (P 2 =0.53) and dextran accumulation (R 2 =0.63) on day 4, respectively. Despite no change in MVD measured by histology, tumor FBV significantly increased after irradiation as measured by DE micro-CT (0.070 vs 0.091, P<.05). Both dextran and liposomal-iodine accumulation in tumors increased significantly after irradiation, with dextran fractional area increasing 5.2-fold and liposomal-iodine concentration increasing 4.0-fold. Conclusions: DE micro-CT is an effective tool for noninvasive assessment of vascular changes in primary tumors. Tumor blood volume and vascular permeability increased after a single therapeutic dose of radiation treatment

  20. Dual-Energy Micro-Computed Tomography Imaging of Radiation-Induced Vascular Changes in Primary Mouse Sarcomas

    Energy Technology Data Exchange (ETDEWEB)

    Moding, Everett J. [Department of Pharmacology and Cancer Biology, Duke University Medical Center, Durham, North Carolina (United States); Clark, Darin P.; Qi, Yi [Center for In Vivo Microscopy, Department of Radiology, Duke University Medical Center, Durham, North Carolina (United States); Li, Yifan; Ma, Yan [Department of Radiation Oncology, Duke University Medical Center, Durham, North Carolina (United States); Ghaghada, Ketan [The Edward B. Singleton Department of Pediatric Radiology, Texas Children' s Hospital, Houston, Texas (United States); Johnson, G. Allan [Center for In Vivo Microscopy, Department of Radiology, Duke University Medical Center, Durham, North Carolina (United States); Kirsch, David G. [Department of Pharmacology and Cancer Biology, Duke University Medical Center, Durham, North Carolina (United States); Department of Radiation Oncology, Duke University Medical Center, Durham, North Carolina (United States); Badea, Cristian T., E-mail: cristian.badea@duke.edu [Center for In Vivo Microscopy, Department of Radiology, Duke University Medical Center, Durham, North Carolina (United States)

    2013-04-01

    Purpose: To evaluate the effects of radiation therapy on primary tumor vasculature using dual-energy (DE) micro-computed tomography (micro-CT). Methods and Materials: Primary sarcomas were generated with mutant Kras and p53. Unirradiated tumors were compared with tumors irradiated with 20 Gy. A liposomal-iodinated contrast agent was administered 1 day after treatment, and mice were imaged immediately after injection (day 1) and 3 days later (day 4) with DE micro-CT. CT-derived tumor sizes were used to assess tumor growth. After DE decomposition, iodine maps were used to assess tumor fractional blood volume (FBV) at day 1 and tumor vascular permeability at day 4. For comparison, tumor vascularity and vascular permeability were also evaluated histologically by use of CD31 immunofluorescence and fluorescently-labeled dextrans. Results: Radiation treatment significantly decreased tumor growth from day 1 to day 4 (P<.05). There was a positive correlation between CT measurement of tumor FBV on day 1 and extravasated iodine on day 4 with microvascular density (MVD) on day 4 (R{sup 2}=0.53) and dextran accumulation (R{sup 2}=0.63) on day 4, respectively. Despite no change in MVD measured by histology, tumor FBV significantly increased after irradiation as measured by DE micro-CT (0.070 vs 0.091, P<.05). Both dextran and liposomal-iodine accumulation in tumors increased significantly after irradiation, with dextran fractional area increasing 5.2-fold and liposomal-iodine concentration increasing 4.0-fold. Conclusions: DE micro-CT is an effective tool for noninvasive assessment of vascular changes in primary tumors. Tumor blood volume and vascular permeability increased after a single therapeutic dose of radiation treatment.

  1. Upregulation of NKX2.2, a target of EWSR1/FLI1 fusion transcript, in primary renal Ewing sarcoma

    Directory of Open Access Journals (Sweden)

    Yoshinari Yamamoto

    2015-01-01

    Full Text Available Renal Ewing sarcoma (ES is a rare malignant tumor characterized by fusion of the EWSR1 gene with a member of the ETS family of oncogenes, arising at a specific chromosomal translocation. Diagnosis of ES can be problematic, especially from cytological or small bioptical specimens because the differential diagnoses comprising a diverse group of small round blue cell tumors (SRBCTs. We report a case of primary renal ES in a young male, which had a t(11;22 (q24;q12 chromosome translocation encoding a type2 EWSR1/FLI1 fusion transcript. The tumor cells showed diffuse cytoplasmic immunoreactivity for CD99 and diffuse nuclear immunoreactivity for NKX2.2, an important oncogenic transcriptional target of EWSR1/FLI1, not only in the histological, but also in the cytological specimens. From the results of this case, we speculate that NKX2.2, in combination with CD99, may be a useful immunocytochemical marker to distinguish renal ES from other SRBCTs of kidney.

  2. Synovial sarcoma

    Directory of Open Access Journals (Sweden)

    Sucari S.C. Vlok

    2014-12-01

    Full Text Available Synovial sarcoma is a malignant, predominantly juxta-articular, soft-tissue tumour representing approximately 10% of all soft-tissue sarcomas. Frequently initially incorrectly diagnosed as a benign lesion, it should be considered as a diagnosis when a young adult patient presents with a calcified juxta-articular soft-tissue mass of insidious onset.

  3. Kaposi sarcoma

    Science.gov (United States)

    ... please enable JavaScript. Kaposi sarcoma is a cancerous tumor of the connective tissue, and is often associated with HIV/AIDS . Causes Before the HIV/AIDS epidemic, Kaposi sarcoma was seen mainly in older Italian ... this group, the tumors developed slowly. In people with HIV/AIDS, the ...

  4. Epidemic Kaposi Sarcoma

    Science.gov (United States)

    ... Sarcoma Treatment Childhood Vascular Tumors Treatment Research Kaposi Sarcoma Treatment (PDQ®)–Patient Version General Information About Kaposi Sarcoma Go to Health Professional Version Key Points Kaposi ...

  5. Classic Kaposi Sarcoma

    Science.gov (United States)

    ... Sarcoma Treatment Childhood Vascular Tumors Treatment Research Kaposi Sarcoma Treatment (PDQ®)–Patient Version General Information About Kaposi Sarcoma Go to Health Professional Version Key Points Kaposi ...

  6. Kaposi's Sarcoma-Associated Herpesvirus-Related Solid Lymphoma Involving the Heart and Brain

    Directory of Open Access Journals (Sweden)

    Jason R. Andrews

    2011-01-01

    Full Text Available Since its discovery in 1994, Kaposi's sarcoma-associated herpesvirus (KSHV has been associated with lymphoproliferative disorders, particularly in patients infected with human immunodeficiency virus (HIV. The disorders most strongly linked to KSHV are multicentric Castleman's Disease (MCD, primary effusion lymphoma, and diffuse large B-cell lymphomas. We report an unusual case of KSHV-associated lymphoma in an HIV-infected patient manifesting with myocardial and central nervous system involvement. We discuss this case in the context of increasing array of KSHV-associated lymphomas. In the HIV-infected patient with a mass lesion, a history of cutaneous Kaposi's sarcoma and prolonged immunosuppression should alert clinicians as to the possibility of KSHV-associated lymphoproliferative disorders, in order to establish a timely diagnosis.

  7. Ewing's Sarcoma: An Analysis of miRNA Expression Profiles and Target Genes in Paraffin-Embedded Primary Tumor Tissue.

    Science.gov (United States)

    Parafioriti, Antonina; Bason, Caterina; Armiraglio, Elisabetta; Calciano, Lucia; Daolio, Primo Andrea; Berardocco, Martina; Di Bernardo, Andrea; Colosimo, Alessia; Luksch, Roberto; Berardi, Anna C

    2016-04-30

    The molecular mechanism responsible for Ewing's Sarcoma (ES) remains largely unknown. MicroRNAs (miRNAs), a class of small non-coding RNAs able to regulate gene expression, are deregulated in tumors and may serve as a tool for diagnosis and prediction. However, the status of miRNAs in ES has not yet been thoroughly investigated. This study compared global miRNAs expression in paraffin-embedded tumor tissue samples from 20 ES patients, affected by primary untreated tumors, with miRNAs expressed in normal human mesenchymal stromal cells (MSCs) by microarray analysis. A miRTarBase database was used to identify the predicted target genes for differentially expressed miRNAs. The miRNAs microarray analysis revealed distinct patterns of miRNAs expression between ES samples and normal MSCs. 58 of the 954 analyzed miRNAs were significantly differentially expressed in ES samples compared to MSCs. Moreover, the qRT-PCR analysis carried out on three selected miRNAs showed that miR-181b, miR-1915 and miR-1275 were significantly aberrantly regulated, confirming the microarray results. Bio-database analysis identified BCL-2 as a bona fide target gene of the miR-21, miR-181a, miR-181b, miR-29a, miR-29b, miR-497, miR-195, miR-let-7a, miR-34a and miR-1915. Using paraffin-embedded tissues from ES patients, this study has identified several potential target miRNAs and one gene that might be considered a novel critical biomarker for ES pathogenesis.

  8. Primary Renal Sarcomas in the Intergroup Rhabdomyosarcoma Study Group (IRSG) Experience, 1972–2005: A Report from the Children’s Oncology Group

    Science.gov (United States)

    Raney, Beverly; Anderson, James; Arndt, Carola; Crist, Willam; Maurer, Harold; Qualman, Stephen; Wharam, Moody; Meyer, William

    2009-01-01

    Purpose To describe clinical and pathologic characteristics and outcome of patients with renal sarcomas. Patients/Methods The IRSG database includes newly diagnosed patients Anaplasia was present in six (60%) of the tumors. Patients’ ages ranged from 2.6–17.8 years. Tumor diameters ranged from 7–15 cm (median, 12 cm). At diagnosis, seven patients had localized disease: four underwent complete removal of tumor (Group I), two had microscopic residual (Group II), and one had gross residual tumor (Group III). Three patients had distant metastases (Group IV) in lungs and bone. Nine patients received vincristine, actinomycin D and cyclophosphamide (VAC). Two Group I patients received no radiation therapy (XRT); others received XRT to the primary tumor and to some metastatic sites. Nine patients achieved complete disappearance of tumor, six due to the initial operation. Tumors recurred in lung (N=2) or brain (N=1) in Group IV patients; each died within 16 months. The Group III patient died of Aspergillus pneumonia. The six Group I and II patients survive, continuously disease-free, at 2.7 to 17.3 years (median, 4.7 years). Conclusions Patients with renal sarcomas often present with large tumors, many of them containing anaplastic features. Removing all gross disease at diagnosis, if feasible, is a critical component of treatment to curing patients with renal sarcoma. PMID:18523987

  9. Radiological findings of pulmonary Kaposi's sarcoma. Manifestaciones radiologicas del sarcoma de Kaposi pulmonar

    Energy Technology Data Exchange (ETDEWEB)

    Rosello, J A; Hernandez, S; Arranz, M; Jareo, J; Ancoechea, J

    1994-01-01

    Kaposi's sarcoma (KS) is the most common neoplasm in AIDS patients. The incidence of pulmonary involvement is approximately 20%. The radiological findings are reported in plain chest x-ray and computed tomography (CT) in 15 patients diagnosed as having pulmonary Kaposi's sarcoma, in whom concomitant pulmonary infection was ruled out. The most common radiological pattern was that of bilateral perihilar interstitial involvement (86%), while poorly defined multiple nodules seldom presented (13%). In 40% of cases, the pulmonary parenchymal lesion was accompanied by pleural effusion. This sign is useful in the differential diagnosis involving opportunistic P. carinii pneumonia, a very common process in these patients which rarely presents with pleural effusion. The chest CT finding that was most characteristic of pulmonary Kaposi's sarcoma was bilateral perihilar peribronchovascular enlargement. (Author)

  10. Radiological findings of pulmonary Kaposi's sarcoma. Manifestaciones radiologicas del sarcoma de Kaposi pulmonar

    Energy Technology Data Exchange (ETDEWEB)

    Rosello, J.A.; Hernandez, S.; Arranz, M.; Jareo, J.; Ancoechea, J.

    1994-01-01

    Kaposi's sarcoma (KS) is the most common neoplasm in AIDS patients. The incidence of pulmonary involvement is approximately 20%. The radiological findings are reported in plain chest x-ray and computed tomography (CT) in 15 patients diagnosed as having pulmonary Kaposi's sarcoma, in whom concomitant pulmonary infection was ruled out. The most common radiological pattern was that of bilateral perihilar interstitial involvement (86%), while poorly defined multiple nodules seldom presented (13%). In 40% of cases, the pulmonary parenchymal lesion was accompanied by pleural effusion. This sign is useful in the differential diagnosis involving opportunistic P. carinii pneumonia, a very common process in these patients which rarely presents with pleural effusion. The chest CT finding that was most characteristic of pulmonary Kaposi's sarcoma was bilateral perihilar peribronchovascular enlargement. (Author)

  11. Effusion under the microscope.

    LENUS (Irish Health Repository)

    Chong, S G

    2012-03-01

    Pseudochylothorax is a very rare form of pleural effusion. It is also called chyliform or cholesterol pleural effusion. It is usually a unilateral process and approximately one-third of patients are asymptomatic at presentation. We report a case of a 60 year old man with a background of rheumatoid arthritis who presented with progressive dyspnea. Chest X-ray revealed a new left pleural effusion and a small persistent right pleural effusion. He presented 5 years prior due to recurrent pleural effusion and no diagnosis was made. Repeat thoracentesis yielded 350 milliliters of thick, milky, tan-colored fluid.

  12. Uterine sarcoma

    Science.gov (United States)

    ... Livingstone; 2014:chap 88. Crum CP, Laury AR, Hirsch MS, Quick CM, Peters WA. Undifferentiated uterine sarcoma. ... Crum CP, Quick CM, Laury AR, Peters WA, Hirsch MS, eds. Gynecologic and Obstetric Pathology . Philadelphia, PA: ...

  13. Primary pulmonary sarcoma in a rhesus monkey after inhalation of plutonium dioxide

    International Nuclear Information System (INIS)

    Hahn, F.F.; Brooks, A.L.; Mewhinney, J.A.

    1987-01-01

    A pulmonary fibrosarcoma of bronchial origin was discovered in a Rhesus monkey that died of pulmonary fibrosis 9 years after inhalation of plutonium-239 dioxide and with a radiation dose to lung of 1400 rad (14 Gy). It grew around the major bronchus of the right cardiac lung lobe and extended into the bronchial lumen and into surrounding pulmonary parenchyma. It also readily invaded muscular pulmonary arteries, resulting in infarction and scarring in the right cardiac lobe. Despite this aggressive growth, the tumor did not metastasize. The primary cause of death was severe pulmonary fibrosis involving the alveolar septa and and perivascular and peribronchial interstitium. Bullous or pericitrical emphysema was prominent. The initial lung burden of plutonium in this monkey was 270 nCi (10 kBq) which is equivalent to approximately 500 times the maximum permissible lung burden for man on a radioactivity per unit body weight basis. The time-dose relationship for survival is consistent with that of dogs and baboons that inhaled plutonium dioxide and died with lung tumors

  14. Primary spindle cell sarcoma of the prostate and 18 F-fluorodeoxyglucose-positron-emission tomography/computed tomography findings

    Directory of Open Access Journals (Sweden)

    Hakan Öztürk

    2015-01-01

    Conclusion: Spindle sarcomas of the prostate have quite aggressive nature and they have high potential to metastase. Average life expectancy is <1 year and the prognosis is poor. CTx and radiation therapy can′t yield curative effects due to poor differentiation.

  15. Epigallocatechin-3-Gallate Suppresses Human Herpesvirus 8 Replication and Induces ROS Leading to Apoptosis and Autophagy in Primary Effusion Lymphoma Cells

    Directory of Open Access Journals (Sweden)

    Ching-Yi Tsai

    2017-12-01

    Full Text Available Epigallocatechin-3-gallate (EGCG, the major constituent of green tea, has been shown to induce cell death in cancer cells. Primary effusion lymphoma (PEL is an aggressive neoplasm caused by human herpesvirus 8 (HHV8. In this study, we examined the role of EGCG on PEL cells in cell death and HHV8 replication. We performed trypan blue exclusion assay to assess the cell viability of PEL cells, flow cytometry analysis to examine the cell cycle distribution and reactive oxygen species (ROS generation, caspase-3 activity to assay apoptosis, acridine orange staining to determine autophagy, and immunoblotting to detect the protein levels involved in apoptosis and autophagy as well as mitogen activated protein kinases (MAPKs activation upon EGCG treatment. The expression of the HHV8 lytic gene was determined by luciferase reporter assay and reverse transcription-PCR, and viral progeny production was determined by PCR. Results revealed that EGCG induced cell death and ROS generation in PEL cells in a dose-dependent manner. N-acetylcysteine (NAC inhibited the EGCG-induced ROS and rescued the cell from EGCG-induced cell death. Even though EGCG induced ROS generation in PEL cells, it reduced the production of progeny virus from PEL cells without causing HHV8 reactivation. These results suggest that EGCG may represent a novel strategy for the treatment of HHV8 infection and HHV8-associated lymphomas.

  16. Recurrent Uveal Effusion after Laser Iridotomy

    Directory of Open Access Journals (Sweden)

    Hiroshi Sakai

    2017-01-01

    Full Text Available A 59-year-old woman was seen by an ophthalmologist for blurred vision, ocular pain, headache, and nausea. She was diagnosed with acute primary angle closure (APAC and successfully treated with medications. Using ultrasound biomicroscopy (UBM, engorged episcleral vein was observed and small uveal effusion was diagnosed after laser peripheral iridotomy (LPI. The uveal effusion disappeared and was again diagnosed by UBM together with anterior segment inflammation with ocular pain. Iritis caused by LPI after APAC might be a cause of uveal effusion in this specific case.

  17. Primary effect of chemotherapy on the transcription profile of AIDS-related Kaposi's sarcoma

    International Nuclear Information System (INIS)

    Kuyl, Antoinette C van der; Burg, Remco van den; Zorgdrager, Fokla; Dekker, John T; Maas, Jolanda; Noesel, Carel JM van; Goudsmit, Jaap; Cornelissen, Marion

    2002-01-01

    Drugs & used in anticancer chemotherapy have severe effects upon the cellular transcription and replication machinery. From in vitro studies it has become clear that these drugs can affect specific genes, as well as have an effect upon the total transcriptome. Total mRNA from two skin lesions from a single AIDS-KS patient was analyzed with the SAGE (Serial Analysis of Gene Expression) technique to assess changes in the transcriptome induced by chemotherapy. SAGE libraries were constructed from material obtained 24 (KS-24) and 48 (KS-48) hrs after combination therapy with bleomycin, doxorubicin and vincristine. KS-24 and KS-48 were compared to SAGE libraries of untreated AIDS-KS, and to libraries generated from normal skin and from isolated CD4+ T-cells, using the programs USAGE and HTM. SAGE libraries were also compared with the SAGEmap database. In order to assess the primary response of AIDS-related Kaposi's sarcoma (AIDS-KS) to chemotherapy in vivo, we analyzed the transcriptome of AIDS-KS skin lesions from a HIV-1 seropositive patient at two time points after therapy. The mRNA profile was found to have changed dramatically within 24 hours after drug treatment. There was an almost complete absence of transcripts highly expressed in AIDS-KS, probably due to a transcription block. Analysis of KS-24 suggested that mRNA pool used in its construction originated from poly(A) binding protein (PABP) mRNP complexes, which are probably located in nuclear structures known as interchromatin granule clusters (IGCs). IGCs are known to fuse after transcription inhibition, probably affecting poly(A)+RNA distribution. Forty-eight hours after chemotherapy, mRNA isolated from the lesion was largely derived from infiltrating lymphocytes, confirming the transcriptional block in the AIDS-KS tissue. These in vivo findings indicate that the effect of anti-cancer drugs is likely to be more global than up- or downregulation of specific genes, at least in this single patient with

  18. Bilateral parietal extradural metastatic ewing's sarcoma simulating acute epidural hematoma

    International Nuclear Information System (INIS)

    Aslam, E.; Imran, M.; Faridi, N.M.

    2006-01-01

    Sarcomas usually metastasize to lugs. The following case report describes an unusual metastasis of Ewing's sarcoma to extradural parietal region bilaterally. The primary was found at lower end of ulna. (author)

  19. Alveolar Soft Part Sarcoma of Urinary Bladder Occurring as a Second Primary Malignancy: A Case Report and Literature Review

    Directory of Open Access Journals (Sweden)

    Manint Usawachintachit

    2016-01-01

    Full Text Available We report a case of bladder alveolar soft part sarcoma in an 18-year-old Thai male patient who had been treated with testicular radiation and systemic chemotherapy for acute lymphoblastic leukemia with testicular relapse. He presented with recurrent dysuria and gross hematuria. Cystoscopy revealed a 2-centimeter irregular sessile mass at the bladder base adjacent to left ureteral orifice. Transurethral resection of the tumor was performed. The histopathological diagnosis was alveolar soft part sarcoma. Chest and abdominal computed tomography showed no evidence of metastasis. He was treated with partial cystectomy and left ureteral reimplantation with negative surgical margin. No evidence of recurrence was found during a 28-month follow-up period with surveillance cystoscopy and computed tomography of the chest and abdomen.

  20. Evaluation of Risk Factors for Vertebral Compression Fracture after Carbon-Ion Radiotherapy for Primary Spinal and Paraspinal Sarcoma

    Directory of Open Access Journals (Sweden)

    Yoshihiro Matsumoto

    2017-01-01

    Full Text Available Background and Purpose. Carbon-ion radiotherapy (C-ion RT was effective therapy for inoperable spinal and paraspinal sarcomas. However, a significant adverse event following radiotherapies is vertebral compression fractures (VCFs. In this study, we investigated the incidence of and risk factors for post-C-ion RT VCFs in patients with spinal or paraspinal sarcomas. Material and Methods. Thirty consecutive patients with spinal or paraspinal sarcomas treated with C-ion RT were retrospectively reviewed. Various clinical parameters and the Spinal Instability Neoplastic Score (SINS were used to evaluate the risk factors for post-C-ion RT VCFs. Results. The overall incidence of VCFs was 23% (median time: 7 months. Patients with VCFs showed a markedly higher SINS score (median value, 9 points than those without VCF (5 points. The area under the receiver operating characteristic curve for the SINS score was 0.88, and the optimum SINS cut-off score was 8 points. The cumulative incidence of VCFs at 1 year was 9% for patients with a SINS score under 8 points, versus 80% for those with a SINS score of 8 points or higher (p<0.0001. Conclusions. In patients with a SINS score of 8 points or higher, referral to a spine surgeon for stabilization and multidisciplinary discussion is appropriate.

  1. Ewing's sarcoma mimicking a meningioma in radiological findings: a case report

    International Nuclear Information System (INIS)

    Kwon, Hee Jin; Choi, Sun Seob

    2007-01-01

    Ewing's sarcoma is an uncommon primary bone tumor. Primary Ewing's sarcoma of the cranium is extremely rare and constitutes only 1% of all Ewing's sarcoma cases. Usually, primary Ewing's sarcoma of the carnium manifests as an expansile osteolytic malignant bone tumor with or without intracranial extension. We report here the radiological findings of a case of Ewing's sarcoma mimicking a meningioma in an 18-year-old man

  2. Biochemical comparison of osteoarthritic knees with and without effusion

    Science.gov (United States)

    2011-01-01

    Background Several symptom-relieving interventions have been shown to be efficacious among osteoarthritis (OA) patients with knee effusion; however, not every symptomatic knee OA patient has clinical effusion. Results may be over-generalized since it is unclear if effused knees represent a unique pathological condition or subset compared to knees without effusion. The primary purpose of this study was to determine if biochemical differences existed between OA knees with and without effusion. Methods The present cross-sectional study consisted of 22 volunteers (11 with knee effusion, 11 without knee effusion) with confirmed late-stage radiographic knee OA (Kellgren-Lawrence score ≥ 3). Synovial fluid samples were collected and analyzed using a custom multiplex enzyme-linked immunosorbent assay to determine eight specific biomarker concentrations (e.g., catabolic, anabolic). Results Matrix metalloproteinase (MMP)-3, tissue inhibitor of MMPs (TIMP)-1, TIMP-2, and interleukin-10 were significantly higher in the knees with effusion than in the knees without effusion. Conclusions The biochemical differences that existed between knees with and without effusion provide support that OA subsets may exist, characterized by distinct biochemical characteristics and clinical findings (e.g., effusion). PMID:22122951

  3. Triptolide inhibits transcription of hTERT through down-regulation of transcription factor specificity protein 1 in primary effusion lymphoma cells

    Energy Technology Data Exchange (ETDEWEB)

    Long, Cong; Wang, Jingchao [Department of Pathogen Biology, School of Basic Medical Sciences, Wuhan University, Wuhan, 430071 (China); Guo, Wei [Department of Pathology and Physiology, School of Basic Medical Sciences, Wuhan University, Wuhan, 430071 (China); Wang, Huan; Wang, Chao; Liu, Yu [Department of Pathogen Biology, School of Basic Medical Sciences, Wuhan University, Wuhan, 430071 (China); Sun, Xiaoping, E-mail: xsun6@whu.edu.cn [Department of Pathogen Biology, School of Basic Medical Sciences, Wuhan University, Wuhan, 430071 (China); State Key Laboratory of Virology, Wuhan University, Wuhan, 430072 (China)

    2016-01-01

    Primary effusion lymphoma (PEL) is a rare and aggressive non-Hodgkin's lymphoma. Human telomerase reverse transcriptase (hTERT), a key component responsible for the regulation of telomerase activity, plays important roles in cellular immortalization and cancer development. Triptolide purified from Tripterygium extracts displays a broad-spectrum bioactivity profile, including immunosuppressive, anti-inflammatory, and anti-tumor. In this study, it is investigated whether triptolide reduces hTERT expression and suppresses its activity in PEL cells. The mRNA and protein levels of hTERT were examined by real time-PCR and Western blotting, respectively. The activity of hTERT promoter was determined by Dual luciferase reporter assay. Our results demonstrated that triptolide decreased expression of hTERT at both mRNA and protein levels. Further gene sequence analysis indicated that the activity of hTERT promoter was suppressed by triptolide. Triptolide also reduced the half-time of hTERT. Additionally, triptolide inhibited the expression of transcription factor specificity protein 1(Sp1) in PEL cells. Furthermore, knock-down of Sp1 by using specific shRNAs resulted in down-regulation of hTERT transcription and protein expression levels. Inhibition of Sp1 by specific shRNAs enhanced triptolide-induced cell growth inhibition and apoptosis. Collectively, our results demonstrate that the inhibitory effect of triptolide on hTERT transcription is possibly mediated by inhibition of transcription factor Sp1 in PEL cells. - Highlights: • Triptolide reduces expression of hTERT by decreasing its transcription level. • Triptolide reduces promoter activity and stability of hTERT. • Triptolide down-regulates expression of Sp1. • Special Sp1 shRNAs inhibit transcription and protein expression of hTERT. • Triptolide and Sp1 shRNA2 induce cell proliferation inhibition and apoptosis.

  4. Granulocytic sarcoma.

    Science.gov (United States)

    Hutchison, R E; Kurec, A S; Davey, F R

    1990-12-01

    Granulocytic sarcoma is a variant presentation of acute myeloblastic leukemia, occurring in extramedullary locations. It is uncommon, but it may occur at any site and at any age, which necessitates its inclusion in the differential diagnosis of all undifferentiated tumors. Histology, touch-imprint cytology, cytochemistry, immunocytochemistry, electron microscopy, and molecular studies all contribute to the diagnosis.

  5. Osteogenic sarcoma

    International Nuclear Information System (INIS)

    Morales A, Nilson; Lozano B, Mauricio; Mejia P, Fernando

    1990-01-01

    Osteogenic sarcoma is one of the two main bone neoplasms in our population. It is very important for the radiologist to be familiar with its clinical and radio graphical features. This article is a literature review of its most important aspects, with some comments on our observations at the National Cancer Institute

  6. Differentiation of subdural effusions

    International Nuclear Information System (INIS)

    Wetterling, T.; Rama, B.

    1989-01-01

    Although X-ray computerized tomography facilitates the diagnosis of intracranial disorders, differentiation of the lesions like extracerebral effusions is often unsatisfactory. Epidural and acute subdural haematoma shown as hyperdensity in CT requires an emergency neurosurgical operation, so that differentiation of these hyperdense effusions may not be required. But the discrimination of the effusions shown as hypodensity in CT (chronic subdural haematoma, subdural hygroma, subdural empyema as well as arachnoid cysts) is urgent because of the different treatment of these effusions. The clinical differentiation is hampered by unspecific neurologic symptoms and the lack of adequate laboratory tests. Some aspects facilitating the diagnostic decision are presented. Recent magnetic resonance (MR) studies promise further progress in differentiating between subdural effusions. (orig.) [de

  7. Chylothorax associated with non-endemic Kaposi's sarcoma ...

    African Journals Online (AJOL)

    Chylothorax is a rare cause of pleural effusion, seen in approximately 2% of cases. In HIV-positive patients with Kaposi's sarcoma (KS), the development of chylothorax presents as a diagnostic challenge with an aggressive course and poor, often lethal outcome. In this clinical scenario, the aetiology of chylothorax may ...

  8. Stages of Ewing Sarcoma

    Science.gov (United States)

    ... adults. Ewing sarcoma has also been called peripheral primitive neuroectodermal tumor, Askin tumor (Ewing sarcoma of the ... Ewing sarcoma are usually done at the same time. The following tests and procedures may be used ...

  9. A Unique Case of Malignant Pleuropericardial Effusion: HHV-8-Unrelated PEL-Like Lymphoma—A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Farhan Mohammad

    2014-01-01

    Full Text Available Primary effusion lymphoma (PEL or body cavity lymphoma is a rare type of extra nodal lymphoma of B-cell origin that presents as lymphomatous effusion(s without any nodal enlargement or tumor masses. It belongs to the group of AIDS related non-Hodgkin’s lymphomas. First described in 1996 in HIV infected individuals who were coinfected with Kaposi’s sarcoma-associated herpesvirus (KSHV or HHV-8 virus, it was included as a separate entity in WHO classification of tumors of hematopoietic and lymphoid tissue in the year 2001. The definition included association with HHV-8 virus as a mandatory diagnostic criterion. However, cases were later reported where PEL-like disease process was diagnosed in HHV-8 negative patients. This was eventually recognized as a rare but distinct entity termed as “HHV-8-unrelated PEL-like lymphoma”. Herein, we are reporting a case of an elderly patient who presented with a large pleuropericardial effusion and was eventually diagnosed with this entity. Till date, only around 50 cases of HHV-8-unrelated PEL-like lymphoma have been reported and our case being EBV, HIV, and Hepatitis C negative makes it very unique and rare occurrence. We are also presenting a review of relevant literature focused mainly on comparing outcomes in patients treated with and without chemotherapy.

  10. Extrauterine Low-Grade Endometrial Stromal Sarcoma

    Directory of Open Access Journals (Sweden)

    Yu-Ju Chen

    2005-12-01

    Conclusions: Low-grade endometrial stromal sarcoma typically has an indolent clinical course and favorable prognosis. Surgical resection is the primary therapeutic approach, and adjuvant therapy with radiotherapy, chemotherapy, or progesterone therapy should be considered for the management of residual or recurrent low-grade endometrial stromal sarcomas.

  11. [Update on soft tissue sarcomas].

    Science.gov (United States)

    Bui, Binh Nguyen; Tabrizi, Reza; Dagada, Corinne; Trufflandier, Nathalie; St ckle, Eberhard; Coindre, Jean-Michel

    2002-01-01

    Important refinements have taken place in the diagnosis of soft tissue sarcoma with extensive use of immuno-histochemistry. New entities have been described, while malignant histiocytofibroma, the most diagnosed sarcoma type during the last two decades, has been dismembered. As for prognosis, the new UICC classification is effectively more discriminating in the definition of prognostic groups; but the usefullness of new biological or genetic markers remains to be assessed. Several breakthrough have taken place in the last years in the treatment of soft tissue sarcoma. Isolated limb perfusion with TNF, hyperthermia and melphalan have proven its efficacy, and is now an alternative to preoperative chemotherapy and/or radiotherapy for limb sparing treatment of the primary tumor site or to amputation. For systemic treatments, novel cytostatic drugs have been shown to be active in sarcomas, including ecteinascidine (ET743) and Glivec (STI571). This last drug has been shown to be remarkably active in c-kit+ stromal sarcoma of the gastro-intestinal tract. It can hopefully regarded as an example for targeted therapies, which may come with a better understanding of the molecular mechanisms triggered by the fundamental, specific genetic alterations shown in sarcoma.

  12. Large Right Pleural Effusion

    Directory of Open Access Journals (Sweden)

    Robert Rowe

    2016-09-01

    Full Text Available History of present illness: An 83-year-old male with a distant history of tuberculosis status post treatment and resection approximately fifty years prior presented with two days of worsening shortness of breath. He denied any chest pain, and reported his shortness of breath was worse with exertion and lying flat. Significant findings: Chest x-ray and bedside ultrasound revealed a large right pleural effusion, estimated to be greater than two and a half liters in size. Discussion: The incidence of pleural effusion is estimated to be at least 1.5 million cases annually in the United States.1 Erect posteroanterior and lateral chest radiography remains the mainstay for diagnosis of a pleural effusion; on upright chest radiography small effusions (>400cc will blunt the costophrenic angles, and as the size of an effusion grows it will begin to obscure the hemidiphragm.1 Large effusions will cause mediastinal shift away from the affected side (seen in effusions >1000cc.1 Lateral decubitus chest radiography can detect effusions greater than 50cc.1 Ultrasonography can help differentiate large pulmonary masses from effusions and can be instrumental in guiding thoracentesis.1 The patient above was comfortable at rest and was admitted for a non-emergent thoracentesis. The pulmonology team removed 2500cc of fluid, and unfortunately the patient subsequently developed re-expansion pulmonary edema and pneumothorax ex-vacuo. It is generally recommended that no more than 1500cc be removed to minimize the risk of re-expansion pulmonary edema.2

  13. Sarcoma Immunotherapy

    International Nuclear Information System (INIS)

    Gouw, Launce G.; Jones, Kevin B.; Sharma, Sunil; Randall, R. Lor

    2011-01-01

    Much of our knowledge regarding cancer immunotherapy has been derived from sarcoma models. However, translation of preclinical findings to bedside success has been limited in this disease, though several intriguing clinical studies hint at the potential efficacy of this treatment modality. The rarity and heterogeneity of tumors of mesenchymal origin continues to be a challenge from a therapeutic standpoint. Nonetheless, sarcomas remain attractive targets for immunotherapy, as they can be characterized by specific epitopes, either from their mesenchymal origins or specific alterations in gene products. To date, standard vaccine trials have proven disappointing, likely due to mechanisms by which tumors equilibrate with and ultimately escape immune surveillance. More sophisticated approaches will likely require multimodal techniques, both by enhancing immunity, but also geared towards overcoming innate mechanisms of immunosuppression that favor tumorigenesis

  14. Sarcoma Immunotherapy

    Energy Technology Data Exchange (ETDEWEB)

    Gouw, Launce G., E-mail: launce.gouw@hsc.utah.edu [Departments of Oncology, Huntsman Cancer Institute at the University of Utah, 2000 Circle of Hope, Salt Lake City, UT 84112 (United States); Jones, Kevin B. [Departments of Orthopaedic Surgery, Huntsman Cancer Institute at the University of Utah, 2000 Circle of Hope, Salt Lake City, UT 84112 (United States); Sharma, Sunil [Departments of Oncology, Huntsman Cancer Institute at the University of Utah, 2000 Circle of Hope, Salt Lake City, UT 84112 (United States); Randall, R. Lor [Departments of Orthopaedic Surgery, Huntsman Cancer Institute at the University of Utah, 2000 Circle of Hope, Salt Lake City, UT 84112 (United States)

    2011-11-10

    Much of our knowledge regarding cancer immunotherapy has been derived from sarcoma models. However, translation of preclinical findings to bedside success has been limited in this disease, though several intriguing clinical studies hint at the potential efficacy of this treatment modality. The rarity and heterogeneity of tumors of mesenchymal origin continues to be a challenge from a therapeutic standpoint. Nonetheless, sarcomas remain attractive targets for immunotherapy, as they can be characterized by specific epitopes, either from their mesenchymal origins or specific alterations in gene products. To date, standard vaccine trials have proven disappointing, likely due to mechanisms by which tumors equilibrate with and ultimately escape immune surveillance. More sophisticated approaches will likely require multimodal techniques, both by enhancing immunity, but also geared towards overcoming innate mechanisms of immunosuppression that favor tumorigenesis.

  15. Kaposi's sarcoma-associated herpesvirus latency-associated nuclear antigen prolongs the life span of primary human umbilical vein endothelial cells.

    Science.gov (United States)

    Watanabe, Takahiro; Sugaya, Makoto; Atkins, April M; Aquilino, Elisabeth A; Yang, Aparche; Borris, Debra L; Brady, John; Blauvelt, Andrew

    2003-06-01

    Tumor spindle cells in all clinical types of Kaposi's sarcoma (KS) are infected with Kaposi's sarcoma-associated herpesvirus (KSHV). Although KSHV contains more than 80 genes, only a few are expressed in tumor spindle cells, including latency-associated nuclear antigen (LANA) and k-cyclin (kCYC). To assess the oncogenic potential of LANA and kCYC, primary human umbilical vein endothelial cells (HUVEC) and murine NIH 3T3 cells were stably transduced by using recombinant retroviruses expressing these genes or the known viral oncogene simian virus 40 large T antigen (LTAg). Interestingly, LANA-transduced HUVEC proliferated faster and demonstrated a greatly prolonged life span (mean +/- standard deviation, 38.3 +/- 11.0 passages) than untransduced cells and vector-transduced cells (<20 passages). By contrast, kCYC-transduced HUVEC did not proliferate faster or live longer than control cells. LANA- and kCYC-transduced HUVEC, but not LTAg-transduced HUVEC, retained the ability to form normal vessel-like structures in an in vitro model of angiogenesis. In cellular assays of transformation, LANA- and kCYC-transduced NIH 3T3 cells demonstrated minimal or no anchorage-independent growth in soft agar and no tumorigenicity when injected into nude mice, unlike LTAg-transduced NIH 3T3 cells. Lastly, gene expression profiling revealed down-regulation, or silencing, of a number of genes within LANA-transduced HUVEC. Taken together, these results suggest that KSHV LANA is capable of inducing prolonged life span, but not transformation, in primary human cells. These findings may explain why LANA-expressing spindle cells proliferate within KS tumors, yet most often do not demonstrate biologic characteristics of transformation or true malignant conversion.

  16. Optimization of a widefield structured illumination microscope for non-destructive assessment and quantification of nuclear features in tumor margins of a primary mouse model of sarcoma.

    Directory of Open Access Journals (Sweden)

    Henry L Fu

    Full Text Available Cancer is associated with specific cellular morphological changes, such as increased nuclear size and crowding from rapidly proliferating cells. In situ tissue imaging using fluorescent stains may be useful for intraoperative detection of residual cancer in surgical tumor margins. We developed a widefield fluorescence structured illumination microscope (SIM system with a single-shot FOV of 2.1 × 1.6 mm (3.4 mm(2 and sub-cellular resolution (4.4 µm. The objectives of this work were to measure the relationship between illumination pattern frequency and optical sectioning strength and signal-to-noise ratio in turbid (i.e. thick samples for selection of the optimum frequency, and to determine feasibility for detecting residual cancer on tumor resection margins, using a genetically engineered primary mouse model of sarcoma. The SIM system was tested in tissue mimicking solid phantoms with various scattering levels to determine impact of both turbidity and illumination frequency on two SIM metrics, optical section thickness and modulation depth. To demonstrate preclinical feasibility, ex vivo 50 µm frozen sections and fresh intact thick tissue samples excised from a primary mouse model of sarcoma were stained with acridine orange, which stains cell nuclei, skeletal muscle, and collagenous stroma. The cell nuclei were segmented using a high-pass filter algorithm, which allowed quantification of nuclear density. The results showed that the optimal illumination frequency was 31.7 µm(-1 used in conjunction with a 4 × 0.1 NA objective (v=0.165. This yielded an optical section thickness of 128 µm and an 8.9 × contrast enhancement over uniform illumination. We successfully demonstrated the ability to resolve cell nuclei in situ achieved via SIM, which allowed segmentation of nuclei from heterogeneous tissues in the presence of considerable background fluorescence. Specifically, we demonstrate that optical sectioning of fresh intact thick tissues

  17. Primary Ewing's Sarcoma of Frontoparietal Bone with Major Soft Tissue Extension: An Unusual Presentation and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Anshu Gupta

    2012-01-01

    Full Text Available An 11-year-old girl presented with progressively increasing swelling in scalp of 8-month duration with no neurological deficit. Local examination showed a hard swelling that seemed to be arising from frontal bone. General and systemic examination was normal. MRI revealed a well-defined lytic lesion in left frontoparietal bone with a subgaleal component. The patient was operated upon and excision of tumor with reconstruction of skull was done. Histopathological examination showed a monomorphic small round cell tumor of bone infiltrating into the subcutaneous tissue. Immunohistochemical stain showed diffuse immunopositivity for MIC-2 in tumor cells, thus final diagnosis of Ewing’s sarcoma was made. The patient was kept for follow up for 3 months and had no symptoms.

  18. Delays in the management of retroperitoneal sarcomas

    DEFF Research Database (Denmark)

    Seinen, Jojanneke; Almquist, Martin; Styring, Emelie

    2010-01-01

    Retroperitoneal sarcomas are rare and treatment should optimally be centralized. Despite successful centralization with 90% of the patients referred prior to surgery, delays occur, which led us to assess lead times in a population-based series. Method. Patients diagnosed with retroperitoneal...... sarcoma in the southern Sweden health care region 2003-2009 were eligible for the study. Data on referrals and diagnostic investigations were collected from clinical files from primary health care, local hospitals, and from the sarcoma centre. Lead times were divided into patient delays and health care...... at the general practitioner, 36 days at local hospitals, and 55 days at the sarcoma centre. Conclusion. Centralization per se is not sufficient for optimized and efficient management. Our findings suggest that delays can be minimized by direct referral of patients from primary health care to sarcoma centers...

  19. General Information about Kaposi Sarcoma

    Science.gov (United States)

    ... Sarcoma Treatment Childhood Vascular Tumors Treatment Research Kaposi Sarcoma Treatment (PDQ®)–Patient Version General Information About Kaposi Sarcoma Go to Health Professional Version Key Points Kaposi ...

  20. A rare case of primary clear cell sarcoma of the pubic bone resembling small round cell tumor: an unusual morphological variant

    International Nuclear Information System (INIS)

    Nakayama, Shoko; Tsuji, Motomu; Hanafusa, Toshiaki; Yokote, Taiji; Iwaki, Kazuki; Akioka, Toshikazu; Miyoshi, Takuji; Hirata, Yuji; Takayama, Ayami; Nishiwaki, Uta; Masuda, Yuki

    2012-01-01

    Clear cell sarcoma (CCS) and malignant melanoma share overlapping immunohistochemistry with regard to the melanocytic markers HMB45, S100, and Melan-A. However, the translocation t(12; 22)(q13; q12) is specific to CCS. Therefore, although these neoplasms are closely related, they are now considered to be distinct entities. However, the translocation is apparently detectable only in 50%–70% of CCS cases. Therefore, the absence of a detectable EWS/AFT1 rearrangement may occasionally lead to erroneous exclusion of a translocation-negative CCS. Therefore, histological assessment is essential for the correct diagnosis of CCS. Primary CCS of the bone is exceedingly rare. Only a few cases of primary CCS arising in the ulna, metatarsals, ribs, radius, sacrum, and humerus have been reported, and primary CCS arising in the pubic bone has not been reported till date. We present the case of an 81-year-old man with primary CCS of the pubic bone. Histological examination of the pubic bone revealed monomorphic small-sized cells arranged predominantly as a diffuse sheet with round, hyperchromatic nuclei and inconspicuous nucleoli. The cells had scant cytoplasm, and the biopsy findings indicated small round cell tumor (SRCT). Immunohistochemical staining revealed the tumor cells to be positive for HMB45, S100, and Melan-A but negative for cytokeratin (AE1/AE3) and epithelial membrane antigen. To the best of our knowledge, this is the first case report of primary CCS of the pubic bone resembling SRCT. This ambiguous appearance underscores the difficulties encountered during the histological diagnosis of this rare variant of CCS. Awareness of primary CCS of the bone is clinically important for accurate diagnosis and management when the tumor is located in unusual locations such as the pubic bone and when the translocation t(12; 22)(q13; q12) is absent

  1. Postradiation sarcomas: importance of surgery

    International Nuclear Information System (INIS)

    Lagrange, J.L.; Ramaioli, A.; Chateau, M.C.; Pignol, J.P.; Marchal, C.; Resbeut, M.; Richaud, P.; Rambert, P.; Tortechaux, J.; Seng, S.H.; La Fontan, B. de; Reme-Saumon, M.; Roullet, B.; Bof, J.; Coindre, J.M.

    1997-01-01

    Purpose: To evaluate the role of surgery in the treatment of Post-radiation sarcomas Materials. Post-radiation sarcomas is a rare entity and large series have rarely been reported. In order to improve knowledge about this entity the Radiotherapist group of the French Cancer Centres (FNCLCC) decided to collect retrospectively the cases treated in their institutions. In order to be sure of the histology, all the cases were reviewed by a panel of pathologists of the FNCLCC Pathologist group. A total of 129 cases of sarcomas, and 108 were reviewed; analysis of 8 is in progress, and no material was obtained in the other 11 cases. The diagnosis of sarcomas was confirmed in 80 cases. All patients (60 F, 20 M) have received radiation therapy (median dose 50 Gy; 9-110 Gy) for the treatment of the primary tumor. At this time the age was 44 years (6-83 y). Diagnoses included: breast C. 42%, Lymphomas 11.5%, gynaecological C. 10% benign lesions 5% miscellaneous. Sarcomas developed after a mean interval of 12 years (3-64 y), in bone in 30% of the cases and in soft tissue in 70%. The majority of lesions (90%) developed in the irradiated field (dose received was between 50 Gy and 60 Gy). Histologically there were 29% Malignant HistiocytofibroSarcomas, 19% OsteoSarcomas, 15% FibroSarcomas, 9% LipoSarcomas, 6% LeiomyoSarcomas, miscellaneous sarcomas 22%. Treatment included: Surgery 28 cases, Surgery+Chemotherapy 17 cases, Chemotherapy only 16 cases, Radiation therapy only 1 case, surgery + Radiation therapy 5 cases, Radiation therapy +chemotherapy 6 cases, Surgery + Radiation therapy + Chemotherapy 7 cases, no treatment 5 cases. Results. The outcome is known for all but 3 patients. 51 patients have died (44 of their sarcoma, 4 of the primary tumour, 2 of other cause and 1 iatrogenic). Median survival is 23 months (95% confidence interval 16-29 mo) but 9 patients survived 5 yr or more. Median survival was 43 mo for patients treated by surgery (28p), 6 mo for chemotherapy group (16 p

  2. The Epidemiology of Sarcoma

    Directory of Open Access Journals (Sweden)

    Burningham Zachary

    2012-10-01

    Full Text Available Abstract Sarcomas account for over 20% of all pediatric solid malignant cancers and less than 1% of all adult solid malignant cancers. The vast majority of diagnosed sarcomas will be soft tissue sarcomas, while malignant bone tumors make up just over 10% of sarcomas. The risks for sarcoma are not well-understood. We evaluated the existing literature on the epidemiology and etiology of sarcoma. Risks for sarcoma development can be divided into environmental exposures, genetic susceptibility, and an interaction between the two. HIV-positive individuals are at an increased risk for Kaposi’s sarcoma, even though HHV8 is the causative virus. Radiation exposure from radiotherapy has been strongly associated with secondary sarcoma development in certain cancer patients. In fact, the risk of malignant bone tumors increases as the cumulative dose of radiation to the bone increases (p for trend

  3. Diagnostic Tools of Pleural Effusion

    OpenAIRE

    Na, Moon Jun

    2014-01-01

    Pleural effusion is not a rare disease in Korea. The diagnosis of pleural effusion is very difficult, even though the patients often complain of typical symptoms indicating of pleural diseases. Pleural effusion is characterized by the pleural cavity filled with transudative or exudative pleural fluids, and it is developed by various etiologies. The presence of pleural effusion can be confirmed by radiological studies including simple chest radiography, ultrasonography, or computed tomography....

  4. Reconstruction of the proximal humerus with a composite of extracorporeally irradiated bone and endoprosthesis following excision of high grade primary bone sarcomas.

    Science.gov (United States)

    Moran, Matthew; Stalley, Paul D

    2009-10-01

    Functional reconstruction of the shoulder joint following excision of a malignant proximal humeral tumour is a difficult proposition. Eleven patients with primary osteosarcoma or Ewing's sarcoma underwent reconstruction with a composite of extra-corporeally irradiated autograft with the addition of a long stemmed hemiarthroplasty. At a mean follow-up of 5.8 years two patients had died from disseminated disease and one patient had undergone amputation for local recurrence. The eight patients with a surviving limb were examined clinically and radiographically. The mean Toronto Extremity Salvage Score was 74 and Musculo-Skeletal Tumour Society score 66. Rotation was well preserved but abduction (mean 32 degrees ) and flexion (40 degrees ) were poor. There was a high rate of secondary surgery, with five out of eleven patients requiring re-operation for complications of reconstruction surgery. Radiographic estimate of graft remaining at follow up was 71%. There were no infections, revisions or radiographic failures. Whilst the reconstructions were durable in the medium term, the functional outcome was no better than with other reported reconstructive methods. The composite technique was especially useful in subtotal humeral resections, allowing preservation of the elbow joint even with very distal osteotomy. Bone stock is restored, which may be useful for future revision surgery in this young group of patients.

  5. Primary Ewing's sarcoma/primitive neuroectodermal tumor of the ileum: case report of a 16-year-old Chinese female and literature review.

    Science.gov (United States)

    Li, Teng; Zhang, Fang; Cao, Yarui; Ning, Shoubin; Bi, Yongmin; Xue, Weicheng; Ren, Li

    2017-05-04

    Ewing's sarcoma (ES) and primitive neuroectodermal tumors (PNET) are closely related tumors. Although soft tissue ES/PNET are common in clinical practice, they are rare in the small intestine. Because of the absence of characteristic clinical symptoms, they are easily misdiagnosed as other benign or malignant diseases. Here, we present the case of a 16-year-old female who complained of anemia and interval hematochezia. Her serum test results showed only a slight elevation of CA-125 and a low level of hemoglobin. Computer tomography and magnetic resonance imaging revealed a cystic and solid mass in the lower abdominal quadrant and pelvic region, which prompted suspicion of a malignant gastrointestinal stromal tumor of the small intestine. After resection, the tumor's histology and immunohistochemistry (positive for CD99, vimentin and synaptophysin) results suggested ES/PNET. Fluorescent in situ hybridization tests proved the breakpoint rearrangement of the EWSR1 gene in chr 22.Ultrastructural analysis revealed neurosecretory and glycogen granules in the tumor cell cytoplasm. Together, these data supported the diagnosis of a rare case of localized ES/PNET in the small intestine without adjuvant chemo- or radiotherapy. To our knowledge, this is the first report from China of a primary small bowel ES/PNET in the English-language literature. In addition, on the basis of findings from previous publications and the current case, the optimal treatment for localized gastrointestinal ES/PNET is discussed.

  6. Soft Tissue Sarcoma

    Science.gov (United States)

    ... muscles, tendons, fat, and blood vessels. Soft tissue sarcoma is a cancer of these soft tissues. There ... have certain genetic diseases. Doctors diagnose soft tissue sarcomas with a biopsy. Treatments include surgery to remove ...

  7. Delays in the management of retroperitoneal sarcomas

    DEFF Research Database (Denmark)

    Seinen, Jojanneke; Almquist, Martin; Styring, Emelie

    2010-01-01

    Retroperitoneal sarcomas are rare and treatment should optimally be centralized. Despite successful centralization with 90% of the patients referred prior to surgery, delays occur, which led us to assess lead times in a population-based series. Method. Patients diagnosed with retroperitoneal...... sarcoma in the southern Sweden health care region 2003-2009 were eligible for the study. Data on referrals and diagnostic investigations were collected from clinical files from primary health care, local hospitals, and from the sarcoma centre. Lead times were divided into patient delays and health care...... delays caused by primary health care, local hospitals, or procedures at the sarcoma centre. Results. Complete data were available from 33 patients and demonstrated a median patient delay of 23¿days (0-17¿months) and median health care delay of 94¿days (1-40¿months) with delays of median 15¿days...

  8. Ewing's sarcoma, a rare but dangerous tumor

    Directory of Open Access Journals (Sweden)

    Theophilus Maksha Dabkana

    2015-01-01

    Full Text Available Ewing's sarcoma or Ewing tumor is a rare primary bone tumor that affects mainly children and adolescents. It belongs to a group of cancers known collectively as Ewing sarcoma family tumors or Ewing family tumors. By the time, the patients present and diagnosis is made, the disease is usually advanced. We reviewed the case files of two patients managed in our hospital within one (2013. Fine-needle aspirations for cytology (FNAC and tissue biopsy were used for diagnosis in the two patients we had. The two patients, both males aged 20 years and 38 years presented late and FNAC and tissue biopsy revealed Ewing's sarcoma. They were referred for radio- and chemotherapy. However, due to poor socioeconomic status, they died of their primary disease. Unless diagnosed early, and in the absence of a multidisciplinary approach, Ewing's sarcoma is a fatal disease.

  9. Therapy of Ewing's sarcoma

    International Nuclear Information System (INIS)

    Dunst, J.; Sauer, R.

    1993-01-01

    Therapy of Ewing's sarcoma requires a qualified clinical, radiological, and pathohistological diagnosis and, in particular, an optimal therapy by an experienced team of oncological specialists. Important prognostic factors are the presence of hematogenous metastases at diagnosis, the initial tumor volume, the response to chemotherapy, and adequate local therapy. Presently, cure rates of more than 60% can be achieved for localized Ewing's sarcoma by combination of local therapy and chemotherapy. The four-drug combination VACA (vincristin, actinomycin D, cyclophosphamide, adriamycin) can be considered as cytostatic gold standard. More aggressive regimens (VAIA, EVAIA, autologous bone marrow transplant) may be beneficial in subgroups and are under investigation. Concerning local therapy adequate radiotherapy plays a major role and achieves the same survival rates as radical surgery, comparable patient selection provided. Several factors have impact on radiotherapeutic results, especially total dose (45 Gy large volume, 55 Gy to the primary tumor), target volume (safety margin at least 2 cm according to the pretreatment volume, at least 5 cm in proximal and distal extension of long bones), timing of radiotherapy (as early as possible) and quality of treatment. Radiotherapy as sole local treatment is indicated in inoperable lesions (spine, sacrum, skull) and in small, good-responding tumors. High-risk patients should receive combined radiotherapeutic-surgical treatment, preferably as pre-operative irradiation. The value of hyperfractionation is not yet proven despite theoretical advantages. (orig.) [de

  10. Tuberculous Pleural Effusion

    Science.gov (United States)

    Cohen, Leah A.; Light, Richard W.

    2015-01-01

    When a patient presents with new pleural effusion, the diagnosis of tuberculous (TB) pleuritis should be considered. The patient is at risk for developing pulmonary or extrapulmonary TB if the diagnosis is not made. Between 3% and 25% of patients with TB will have TB pleuritis. The incidence of TB pleuritis is higher in patients who are human immunodeficiency virus (HIV)-positive. Pleural fluid is an exudate that usually has a predominance of lymphocytes. The easiest way to diagnose TB pleuritis in a patient with lymphocytic pleural effusion is to demonstrate a pleural fluid adenosine deaminase level above 40 IU/L. The treatment for TB pleuritis is the same as that for pulmonary TB. Tuberculous empyema is a rare occurrence, and the treatment is difficult. PMID:29404070

  11. Uterus effusion after irradiation for carcinoma of the cervix

    International Nuclear Information System (INIS)

    Ma Shaokang; Gao Juzhen; Wu Lingying

    2007-01-01

    Objective: To analyse the clinical characteristics of patients with uterus effusion after irradiation for carcinoma of the cervix. Methods: 151 patients with uterus effusion after irradiation for carcinoma of the cervix were retrospectively analyzed. Results: All these 151 patients who had been treated by radiotherapy had FIGO stage IIB or IIIB lesions including 32 adenocarcinomas. The most common symptom was irregular vaginal discharge or drainage (65 patients), followed by irregular vaginal bleeding (32 patients), and abdominal or pelvic mass (27 patients). Twenty-seven patients were entirely asymptomatic. The positive rate of uterus effusion found by B-ultrasound scan was 100%. When uterus effusion was found, 87 patients (57.6%) had malignant tumor, including 62 with residual or recurrent cervical carcinoma and 25 with secondary primary malignant uterus tumor. Sixty-four patients had uterus effusion alone including 8 with pyometra. Of these 64 patients, 17 underwent abdominal hysterectomy with bilateral salpingo-oophorectomy (BSOH), the others were treated with drainage till the fluid disappeared. Of the 62 patients with residual or recurrent cervical carcinoma, 19 underwent BSOH and 43 were treated by palliative irradiation or chemotherapy. Twenty-five patients who had secondary primary malignant uterus tumor were treated by BSOH. The 5-year survival rate of patients with uterus effusion alone, with residual tumor (with recurrent tumor), or with secondary primary malignancy were 54.8%, 0%, 28.7% and 47.5%, respectively. The prognosis of the patients with uterus effusion alone was better than those with secondary primary malignant uterus tumor. Patients with residual or recurrent cervical cancer had the worst prognosis. Conclusions: Patients with uterus effusion after irradiation for carcinoma of the cervix often have tumor including secondary primary, malignant uterus tumor. The prognosis is different, which depends on if the patient has tumor or not. (authors)

  12. Postradiation sarcoma involving the spine

    International Nuclear Information System (INIS)

    Sundaresan, N.; Huvos, A.G.; Krol, G.; Hughes, J.E.; Cahan, W.G.

    1986-01-01

    Postradiation sarcomas arising many years after treatment of cancer are long term sequelae of therapy. We describe the clinical features, radiographic findings, and results of treatment in 13 patients with such sarcomas encountered over a 6-year period. Of these patients, 9 had bone sarcomas and the remaining 4 had paraspinal tumors arising from adjacent soft tissue and nerve. The primary cancer for which radiation was given included Hodgkin's disease (4 patients), breast cancer (2 patients), cervix cancer (2 patients), and a variety of others (5 patients). The latent interval to the occurrence of the second neoplasm varied from 6 to 30 years (median, 10 years) after treatment of the original tumor. Despite aggressive treatment, the overall prognosis was poor. The median survival was 8 months, with only 3 surviving more than 2 years. Although rare, postradiation sarcoma should be considered in the differential diagnosis of patients presenting with late onset of spinal pain or neurological symptoms after clinical remission of an original cancer

  13. Palliative Treatment of Malignant Pleural Effusion

    Directory of Open Access Journals (Sweden)

    Chenyang Liu

    2015-01-01

    Full Text Available Malignant pleural effusion (MPE is a common clinical problem caused by cancers. Pleural effusion can be the first sign of cancer in more than 25% of patients. Lung cancer and breast cancer are the most common cancers that metastasize to the pleura in men and women, respectively. Other cancers, including, but not limited to, lymphomas, ovarian cancer, stomach cancer, and several unknown primary cancers can also lead to MPE. Dyspnea and chest pain are the most common symptoms of MPE along with other symptoms such as a cough, weight loss, anorexia, fatigue, and weakness. Aggravation of these symptoms is closely related to the rate of accumulation of pleural effusion. Treatment options to MPE are determined by the type and extent of the underlying malignancy. The major goals of the treatment are to relieve symptoms, restore functions, improve the quality of life, and minimize the duration of hospital stay and costs. Although some patients can be treated with systemic therapies, most of these treatments are temporary, and MPE would recur soon. Hence, further palliative treatments to effectively control pleural effusions and relieve symptoms are necessary. This review addresses the pathophysiology of MPE and the treatment options for patients with MPE.

  14. Ewing’s Sarcoma: An Analysis of miRNA Expression Profiles and Target Genes in Paraffin-Embedded Primary Tumor Tissue

    Directory of Open Access Journals (Sweden)

    Antonina Parafioriti

    2016-04-01

    Full Text Available The molecular mechanism responsible for Ewing’s Sarcoma (ES remains largely unknown. MicroRNAs (miRNAs, a class of small non-coding RNAs able to regulate gene expression, are deregulated in tumors and may serve as a tool for diagnosis and prediction. However, the status of miRNAs in ES has not yet been thoroughly investigated. This study compared global miRNAs expression in paraffin-embedded tumor tissue samples from 20 ES patients, affected by primary untreated tumors, with miRNAs expressed in normal human mesenchymal stromal cells (MSCs by microarray analysis. A miRTarBase database was used to identify the predicted target genes for differentially expressed miRNAs. The miRNAs microarray analysis revealed distinct patterns of miRNAs expression between ES samples and normal MSCs. 58 of the 954 analyzed miRNAs were significantly differentially expressed in ES samples compared to MSCs. Moreover, the qRT-PCR analysis carried out on three selected miRNAs showed that miR-181b, miR-1915 and miR-1275 were significantly aberrantly regulated, confirming the microarray results. Bio-database analysis identified BCL-2 as a bona fide target gene of the miR-21, miR-181a, miR-181b, miR-29a, miR-29b, miR-497, miR-195, miR-let-7a, miR-34a and miR-1915. Using paraffin-embedded tissues from ES patients, this study has identified several potential target miRNAs and one gene that might be considered a novel critical biomarker for ES pathogenesis.

  15. Diagnostic Tools of Pleural Effusion

    Science.gov (United States)

    2014-01-01

    Pleural effusion is not a rare disease in Korea. The diagnosis of pleural effusion is very difficult, even though the patients often complain of typical symptoms indicating of pleural diseases. Pleural effusion is characterized by the pleural cavity filled with transudative or exudative pleural fluids, and it is developed by various etiologies. The presence of pleural effusion can be confirmed by radiological studies including simple chest radiography, ultrasonography, or computed tomography. Identifying the causes of pleural effusions by pleural fluid analysis is essential for proper treatments. This review article provides information on the diagnostic approaches of pleural effusions and further suggested ways to confirm their various etiologies, by using the most recent journals for references. PMID:24920946

  16. Methimazole associated eosinophilic pleural effusion: a case report.

    Science.gov (United States)

    Gaspar-da-Costa, Pedro; Duarte Silva, Filipa; Henriques, Júlia; do Vale, Sónia; Braz, Sandra; Meneses Santos, João; M M Victorino, Rui

    2017-03-21

    Adverse reactions associated to anti-thyroid drugs include fever, rash, arthralgia, agranulocytosis and hepatitis that are thought to be hypersensitivity reactions. Five cases of pleural effusion associated to thionamides have also been reported, two with propylthiouracil and three with carbimazole. We report here a case of a 75-year-old man admitted because of unilateral pleural effusion. The patient had a recent diagnosis of hyperthyroidism and 6 days after starting methimazole complained of pleuritic chest pain. He had elevated C-reactive protein and erythrocyte sedimentation rate and normal white blood cell count and liver enzymes. Chest radiography showed a moderate right pleural effusion and the ultrasound revealed a loculated effusion that was shown to be an eosinophilic exudate. The temporal relationship between methimazole intake and the development of pleural effusion combined with the extensive exclusion of alternative causes, namely infectious, neoplastic and primary auto-immune diseases, led to the diagnosis of hypersensitivity reaction to methimazole. The thionamide was stopped and corticosteroid was started with complete resolution of the pleural effusion in 3 months. Awareness of this rare adverse reaction of anti-thyroid drugs is important and methimazole can be added to the list of possible etiologies of drug-induced eosinophilic pleural effusion.

  17. General Information about Ewing Sarcoma

    Science.gov (United States)

    ... adults. Ewing sarcoma has also been called peripheral primitive neuroectodermal tumor, Askin tumor (Ewing sarcoma of the ... Ewing sarcoma are usually done at the same time. The following tests and procedures may be used ...

  18. Treatment Option Overview (Ewing Sarcoma)

    Science.gov (United States)

    ... Ewing Sarcoma Treatment Osteosarcoma Treatment Research Ewing Sarcoma Treatment (PDQ®)–Patient Version General Information About Ewing Sarcoma ... started or in another part of the body. Treatment Option Overview Key Points There are different types ...

  19. The role of imaging in the evaluation of extraskeletal Ewing's sarcoma

    African Journals Online (AJOL)

    Primary bone tumours account for 5% of all adolescent and child cancers. When Ewing's sarcoma arises in soft tissue rather than bone, it is referred to as extraskeletal Ewing's sarcoma (ESS). Computed tomography (CT) scanning and magnetic resonance imaging (MRI) features of Ewing's sarcoma are non-specific, and a ...

  20. A Case of Massive Pleural Effusion: Pleurodesis by Bleomycin.

    Science.gov (United States)

    Hasan, R; Khan, O S; Aftabuddin, M; Razzaque, A M; Chowdhury, G A

    2016-04-01

    Malignant pleural effusion is a common complication of primary and metastatic pleural malignancies. Pleurodesis for the management of malignant pleural effusion is intended to achieve symphysis between parietal and visceral pleura, and to prevent relapse of pleural effusion. Many chemical agents are tried to induce inflammation and damage of the pleural mesothelial layer to achieve this symphysis. Hemorrhagic pleural effusion, especially in the right hemithorax commonly occurs as presentation of primary and metastatic pleural malignancies. This case reports massive right-sided hemorrhagic pleural effusion as the sole manifestation of primary lung cancer in a 45 year old man. Patient attended our department of thoracic surgery complaining of cough, shortness of breath and right sided chest pain. A chest X-ray and chest computer tomography (CT) radiograph shows right sided massive pleural effusion. Right sided tube thoracotomy done. Pleural fluid study was done. Fluid for cytopathology was positive for malignant cell. Computed tomography guided fine needle aspiration cytology from right lung lesion was also done. Diagnosis was as small cell carcinoma. Pleural effusion resolved after 9(th) post operative day of chest tube insertion. Bleomycin pleurodesis was done. Day after pleurodesis intra thoracic tube was removed and patient was discharged from hospital on 10(th) Post operative day with an advice to attend the oncology department for further treatment. The protocol of tube thoracostomy and chemical pleurodesis was almost always successful in giving symptomatic relief of respiratory distress for a considerable period of time. However, chemical pleurodesis is not possible in all cases of malignant pleural effusion because it has got potential complication including death.

  1. Pleural effusion in the dog and cat

    International Nuclear Information System (INIS)

    Forrester, S.D.

    1990-01-01

    The most common causes of pleural effusion and their management are discussed under the headings: pleural anatomy; pleural fluid movement; mechanisms of pleural effusion; diagnosis; patterns of pleural effusion

  2. The Danish Sarcoma Database

    DEFF Research Database (Denmark)

    Jørgensen, Peter Holmberg; Lausten, Gunnar Schwarz; Pedersen, Alma B

    2016-01-01

    AIM: The aim of the database is to gather information about sarcomas treated in Denmark in order to continuously monitor and improve the quality of sarcoma treatment in a local, a national, and an international perspective. STUDY POPULATION: Patients in Denmark diagnosed with a sarcoma, both...... skeletal and ekstraskeletal, are to be registered since 2009. MAIN VARIABLES: The database contains information about appearance of symptoms; date of receiving referral to a sarcoma center; date of first visit; whether surgery has been performed elsewhere before referral, diagnosis, and treatment; tumor...... of Diseases - tenth edition codes and TNM Classification of Malignant Tumours, and date of death (after yearly coupling to the Danish Civil Registration System). Data quality and completeness are currently secured. CONCLUSION: The Danish Sarcoma Database is population based and includes sarcomas occurring...

  3. Adherence to treatment guidelines for primary sarcomas affects patient survival: a side study of the European CONnective TIssue CAncer NETwork (CONTICANET).

    Science.gov (United States)

    Rossi, C R; Vecchiato, A; Mastrangelo, G; Montesco, M C; Russano, F; Mocellin, S; Pasquali, S; Scarzello, G; Basso, U; Frasson, A; Pilati, P; Nitti, D; Lurkin, A; Ray-Coquard, I

    2013-06-01

    The impact of adherence to clinical practice guidelines (CPGs) for loco-regional treatment (i.e. surgery and radiotherapy) and chemotherapy on local disease control and survival in sarcoma patients was investigated in a European study conducted in an Italian region (Veneto). The completeness of the adherence to the Italian CPGs for sarcomas treatment was assessed by comparing the patient's charts and the CPGs. Propensity score-adjusted multivariate survival analysis was used to assess the impact of CPGs adherence on patient clinical outcomes. A total of 151 patients were included. Adherence to CPGs for loco-regional therapy and chemotherapy was observed in 106 out of 147 (70.2%) and 129 out of 139 (85.4%) patients, respectively. Non-adherence to CPGs for loco-regional treatment was independently associated with AJCC stage III disease [odds ratio (OR) 1.77, P = 0.011] and tumor-positive excision margin (OR 3.55, P = 0.003). Patients not treated according to the CPGs were at a higher risk of local recurrence [hazard ratio (HR) 5.4, P < 0.001] and had a shorter sarcoma-specific survival (HR 4.05, P < 0.001), independently of tumor stage. Incomplete adherence to CPGs for loco-regional treatment of sarcomas was associated with worse prognosis in patients with non-metastatic tumors.

  4. Complex treatment of localized bone marrow sarcoma in children

    International Nuclear Information System (INIS)

    Kolygin, B.A.; Punanov, Yu.A.; Malinin, A.P.; Safonova, S.A.

    1997-01-01

    The retrospective analysis included the results of the treatment of 67 children suffering from localized sarcomas of bone (Ewing's sarcoma, lymphosarcoma). The advantage was demonstrated in patients, received combination of chemotherapy and radiotherapy on the involved bone. The resection of the primary tumor in combination with radio-chemotherapy improves the 10-year survival

  5. Pleural Effusion in Multiple Myeloma.

    Science.gov (United States)

    Wang, Zhuo; Xia, Guoguang; Lan, Ling; Liu, Fayong; Wang, Yanxun; Liu, Baoyue; Ding, Yi; Dai, Li; Zhang, Yunjian

    2016-01-01

    Pleural effusion is rarely observed in patients with multiple myeloma (MM). Myeloma cell infiltration or invasion to the pleura is very rare. This study aimed to investigate the clinical characteristics of pleural effusion in patients with MM. We retrospectively reviewed the medical records of patients diagnosed with pleural effusion, MM, and pleural effusion with MM between 2004 and 2014 at Beijing Jishuitan Hospital. The present study included patients with pleural effusion who underwent cytological, bacteriological, biochemical and other testing. The cytopathology of abnormal pleural effusion cells was not diagnostic, thus flow cytometry was performed. MM was defined using the diagnosis standard of NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) 2014 for MM. This study included 3,480 pleural effusion patients and 319 MM patients. There were 34 patients with both MM and pleural effusion (17 men and 17 women). The average age was 63 years (range, 48-84 years). Pleural effusion with MM was caused by congestive heart disease, chronic renal failure, hypoalbuminemia, pulmonary infarctions, cirrhosis, pulmonary arterial hypertension, parapneumonic effusion, tuberculous pleural effusion, and myelomatous pleural effusion (MPE). The diagnosis of MPE was confirmed by the detection of myeloma cells in the pleural fluid using flow cytometric analyses. There were only 2 MPE cases in our study. The first MPE case was a woman. The first clinical manifestation was pleural effusion, and the diagnosis was non-secretory MM, DSS stage IIIA (Durie-Salmon staging system); ISS stage I (the International Staging System). The second MPE case was a man who was diagnosed with MM IgA-κ, DSS stage IIIA; ISS stage II. The detection rate of MPE was very low. MPE tended to present with yellow exudates and the lack of physical and chemical characteristics. Furthermore, patients with MPE exhibited many yellow nodules on the pleura. These nodules were lobulated and had abundant

  6. Adult prostate sarcoma: the M. D. Anderson Cancer Center Experience.

    Science.gov (United States)

    Sexton, W J; Lance, R E; Reyes, A O; Pisters, P W; Tu, S M; Pisters, L L

    2001-08-01

    Sarcoma of prostate origin is rare. Historically, long-term survival rates for adult patients with prostate sarcoma are poor. We analyzed the experience of 1 institution with prostate sarcoma during the last 3 decades. The records of 21 patients with prostate sarcoma were reviewed to identify symptoms at presentation, diagnostic procedures, presence and development of metastases, staging evaluation, histological subtype, grade and size of the primary tumor, and treatment sequence, including surgery, and preoperative and postoperative therapies. Several clinicopathological variables were assessed for prognostic importance. Most patients presented with urinary obstruction. The diagnosis of prostate sarcoma was usually established with ultrasound guided biopsy or transurethral resection. Histological subtypes were leiomyosarcoma in 12, rhabdomyosarcoma in 4, malignant fibrous histiocytoma in 1 and unclassified sarcoma in 4 patients. At last followup, 8 patients had no evidence of disease after a median of 81.5 months (range 10 to 197). The remaining 13 patients died of sarcoma (median survival 18 months, range 3 to 94). The 1, 3 and 5-year actuarial survival rates for all 21 patients were 81%, 43% and 38%, respectively. Factors predictive of long-term survival were negative surgical margins (p = 0.0005) and absence of metastatic disease at presentation (p = 0.0004). Tumor size and grade, and the histological subtype of prostate sarcoma had no significant influence on actuarial survival. The long-term disease specific survival rate for adults with prostate sarcoma is poor. Early diagnosis and complete surgical resection offer patients the best chance for cure.

  7. Uma nova abordagem para as endopróteses parciais de joelho em sarcomas primários ósseos A new approach to partial knee endoprosthesis in primary bone sarcomas

    Directory of Open Access Journals (Sweden)

    Valter Penna

    2009-02-01

    Full Text Available OBJETIVO: As endopróteses parciais de joelho para as ressecções em sarcomas ósseos demonstram serem boa solução para o tratamento de pacientes com imaturidade esquelética. O objetivo deste estudo é avaliar o escore funcional, as vantagens, as desvantagens e indicações para esta técnica cirúrgica em quatorze pacientes em um protocolo brasileiro de osteossarcoma e sarcoma de Ewing. MÉTODOS: Análise retrospectiva realizada para identificar a evolução funcional e as possíveis complicações do procedimento. 14 pacientes com idade entre 10 e 22 anos avaliados funcionalmente pelos critérios de Enneking/ISOLS (International Society of Limb Salvage, sendo todos operados na mesma Instituição e pelo mesmo cirurgião. Foram utilizadas endopróteses parciais das extremidades distal do fêmur e proximal da tíbia com reconstrução ligamentar. ReSULTADOS: A análise do escore funcional de Enneking/ISOLS demonstrou 78,6 % de excelentes resultados e 21,4% de bons. Dos 14 pacientes, todos portadores de tumores primitivos ósseos em protocolo de quimioterapia, nove não apresentaram nenhum tipo de complicação e cinco indivíduos evoluíram com complicações relacionadas ao procedimento, sendo que houve relação estatística positiva entre os maus resultados e a presença de complicações (p=0,027. CONCLUSÃO: As endopróteses parciais de joelhos são menos prejudiciais ao estoque ósseo de pacientes com esqueleto imaturo. As críticas sobre os maus resultados funcionais estão sendo suplantadas pelas novas técnicas de reconstrução, corretos protocolos de reabilitação, qualidade e tecnologia dos implantes, e o aumento da curva de aprendizado. Essa opção de tratamento per-mite a preservação do estoque ósseo e a possibilidade de revisão da artroplastia não convencional de modo menos agressivo.OBJECTIVE: Partial knee endoprosthesis to bone sarcomas resections seems to be a good solution to treat this immature skeletal patients

  8. Soft tissue sarcoma - diagnosis and treatment

    International Nuclear Information System (INIS)

    Ruka, W.; Rutkowski, P.; Krzakowski, M.

    2009-01-01

    Significant progress in the treatment of soft tissue sarcoma (STS), both primary tumor and local recurrences/metastatic disease, has been achieved in recent years. Surgery is essential modality, but the use of combined treatment (standard combination of surgery with adjuvant radiotherapy, chemotherapy in selected cases and perioperative rehabilitation) in highly-experienced centers increased possibility of cure and limitations of extent of local surgery. Current combined therapy together with the use of reconstructive methods allows for limb-sparing surgery in majority of soft tissue sarcoma patients (amputation in 10% of cases as compared to approximately 50% in the 1960 - 70s). The slow, but constant, increase of rate of soft tissue sarcoma patients with long-term survival has been observed. Contemporary 5-year overall survival rate in patients with extremity soft tissue sarcomas is 55 -78%. In case of diagnosis of metastatic disease the prognosis is still poor (survival of approximately 1 year). Good results of local therapy may be expected only after planned (e.g., after preoperative biopsy - tru - cut or incisional) radical surgical excision of primary tumor with pathologically negative margins (R0 resection). Following appropriate diagnostic check-up, adjuvant radiotherapy is necessary in the majority of patients treated with radical surgery need, as well as long-term rehabilitation and follow-up examinations in treating center are needed for at least 5 years. The progress is due to the introduction of targeted therapy acting on molecular or genetic cellular disturbances detected during studies on etiopathogenetic mechanisms of sarcoma subtypes. In view of rarity of sarcomas and necessity of multidisciplinary therapy, the crucial issue is that management of these tumors should be hold in experienced oncological sarcoma centers. (authors)

  9. [Sarcoid pleural effusion].

    Science.gov (United States)

    Rodríguez-Núñez, Nuria; Rábade, Carlos; Valdés, Luis

    2014-12-09

    Pleural effusion (PE) is a very uncommon manifestation of sarcoidosis. It is equally observed in men and women, can appear at any age and in all radiologic stages, though it is more common in stages i and ii. Effusions have usually a mild or medium size and mainly involve the right side. Various mechanisms can be implicated. PE will be a serous exudate if there is an increase in the capillary permeability due to direct involvement of the pleural membrane, a chylothorax if mediastinum lymph nodes compress the thoracic duct and/or the lymphatic drainage from the pleural cavity, an hemothorax if granuloma compress or invade pleural small vessels or capillaries, and even a transudate if there is compression of the inferior vena cava, atelectasis due to complete bronchial obstruction or when the resolution of the PE is incomplete with chronic thickening of visceral pleura (trapped lung). It manifests biochemically as a pauci-cellular exudate with a predominance of lymphocytes, though there can be a preponderance of eosinophils or neutrophils. Protein concentrations are usually proportionately higher than lactate dehidrogenase, adenosine deaminase is normally low and it is possible to find increased levels of CA-125 in women. The tuberculin test is negative and pleural or lung biopsies yield the diagnosis by confirming the presence of non-caseating granulomata. These PE can have a favorable self-limited outcome, even though in most cases treatment with corticosteroids is needed, while surgery is required in a few cases. Copyright © 2013 Elsevier España, S.L.U. All rights reserved.

  10. [Primitive cutaneous Ewing's sarcoma: a diagnostic and therapeutic dilemma].

    Science.gov (United States)

    Delaplace, M; Mélard, P; Perrinaud, A; Goré, C; Vergier, B; Machet, L

    2011-05-01

    Ewing's sarcoma (or peripheral neuroectodermal tumour) is generally found in bone tissue, and a primary dermal site is extremely rare. We report a case of primary cutaneous Ewing's sarcoma in a 21-year-old woman. A 21-year-old woman presented with a scapular lesion that had been slowly developing for one year. The 1-cm lesion was removed and histological examination showed proliferation of small round cells in the dermis. Immunostaining revealed cytoplasmic membrane expression of CD99 and a negative immunoprofile for other small round-cell tumors. Ewing's sarcoma fusion gene transcripts were detected using fluorescence in situ hybridization (FISH). A staging examination revealed no other abnormalities. It was decided to treat the lesion as for osseous Ewing's sarcoma with wide resection followed by systemic adjuvant chemotherapy. Cutaneous Ewing's sarcoma raises concerns about diagnosis and treatment. Owing to the non-specificity of its clinical presentation, histology and immunoprofile, diagnosis of superficial Ewing's sarcoma is difficult and numerous differential diagnoses must be considered. When dealing with a surface tumour, the diagnosis of cutaneous Ewing's sarcoma must be considered. CD99 immunostaining and molecular testing for evidence of EWSR1 rearrangement are useful investigations to confirm the diagnosis. Furthermore, modalities of treatment must be carefully discussed. Cutaneous Ewing's sarcoma is currently treated in the same way as osseous Ewing's sarcoma (wide surgical excision, adjuvant radiotherapy when surgical margins are unsatisfactory, systemic adjuvant chemotherapy, and, in some cases, bone marrow transplant). However, some studies show a more favourable prognosis for cutaneous Ewing's sarcoma than for osseous Ewing's sarcoma. We may thus ask whether such aggressive multimodal treatment is needed. Copyright © 2010 Elsevier Masson SAS. All rights reserved.

  11. Uterine sarcoma - current perspectives.

    Science.gov (United States)

    Benson, Charlotte; Miah, Aisha B

    2017-01-01

    Uterine sarcomas comprise a group of rare tumors with differing tumor biology, natural history and response to treatment. Diagnosis is often made following surgery for presumed benign disease. Currently, preoperative imaging does not reliably distinguish between benign leiomyomas and other malignant pathology. Uterine leiomyosarcoma is the most common sarcoma, but other subtypes include endometrial stromal sarcoma (low grade and high grade), undifferentiated uterine sarcoma and adenosarcoma. Clinical trials have shown no definite survival benefit of adjuvant radiotherapy or chemotherapy and have been hampered by the rarity and heterogeneity of these disease types. There is a role of adjuvant treatment in carefully selected cases following multidisciplinary discussion at sarcoma reference centers. In patients with metastatic disease, systemic chemotherapy can then be considered. There is activity of a number of agents, including doxorubicin, trabectedin, gemcitabine-based chemotherapy, eribulin and pazopanib. Patients should be considered for clinical trial entry where possible. Close international collaboration is important to allow progress in this group of diseases.

  12. Ewing's Sarcoma of Bone Tumor Cells Produce MCSF that Stimulates Monocyte Proliferation in a Novel Mouse Model of Ewing's Sarcoma of Bone

    OpenAIRE

    Margulies, BS; DeBoyace, SD; Damron, TA; Allen, MJ

    2015-01-01

    Ewing's sarcoma of bone is a primary childhood malignancy of bone that is treated with X-radiation therapy in combination with surgical excision and chemotherapy. To better study Ewing's sarcoma of bone we developed a novel model of primary Ewing's sarcoma of bone and then treated animals with X-radiation therapy. We identified that uncontrolled tumor resulted in lytic bone destruction while X-radiation therapy decreased lytic bone destruction and increased limb-length asymmetry, a common, cr...

  13. [The diagnostic value of medical thoracoscopy for unexplained pleural effusion].

    Science.gov (United States)

    Jiang, Shu-juan; Mu, Xiao-yan; Zhang, Song; Su, Li-li; Ma, Wei-xia

    2013-05-01

    To explore the endoscopic features of patients with unexplained pleural effusion, and to evaluate the diagnostic value of medical thoracoscopy. A retrospective analysis of 2380 patients with unexplained pleural effusion (1320 males and 1060 females; age 15-94 years) in Shandong Provincial Hospital from 1992 to 2011 were performed .The diagnosis was confirmed by medical thoracoscopy. The endoscopic findings of malignant pleural effusion mostly showed nodules of varying sizes. The nodules could be grape-like, cauliflower-like, fused into masses, or diffused small nodules . The appearance of cancerous nodules was more diversified compared to tuberculous nodules. Tuberculous pleurisy was manifested as diffuse pleural congestion and miliary changes, multiple small gray-white nodules, fibrin deposition and adhesion in the pleural cavity, pleural thickening and loculation . The pathological diagnosis was as follows: pleural metastases in 899 (37.8%), primary pleural mesothelioma in 439 (18.4%), tuberculous pleurisy in 514 (21.6%), non-specific inflammation in 226 (9.5%), empyema in 190 (8.0%), hepatic pleural effusion in 36 (1.5%) and pleural effusion of unknown causes in 76 (3.2%) cases. The diagnostic positive rate of medical thoracoscopy was 96.8%. No serious complications were observed. Medical thoracoscopy is a relatively safe procedure and has an important application value in the diagnosis of unexplained pleural effusion.

  14. External-beam radiation therapy combined with limb-sparing surgery in elderly patients (>70 years) with primary soft tissue sarcomas of the extremities. A retrospective analysis

    Energy Technology Data Exchange (ETDEWEB)

    Andrae, Claudia; Rauch, Josefine; Belka, Claus [University Hospital of Munich (LMU), Department of Radiation Oncology, Munich (Germany); Klein, Alexander; Duerr, Hans Roland [University Hospital of Munich (LMU), Department of Orthopedics, Munich (Germany); Lindner, Lars Hartwin [University Hospital of Munich (LMU), Deparment of Internal Medicine, Munich (Germany); Knoesel, Thomas [University Hospital of Munich (LMU), Institute of Pathology, Munich (Germany); Angele, Martin [University Hospital of Munich (LMU), Department of Surgery, Munich (Germany); Baur-Melnyk, Andrea [University Hospital of Munich (LMU), Department of Radiology, Munich (Germany); Roeder, Falk [University Hospital of Munich (LMU), Department of Radiation Oncology, Munich (Germany); German Cancer Research Center (DKFZ), CCU Molecular Radiation Oncology, Heidelberg (Germany)

    2017-08-15

    To report our experience with EBRT combined with limb-sparing surgery in elderly patients (>70 years) with primary extremity soft tissue sarcomas (STS). Retrospectively analyzed were 35 patients (m:f 18:17, median 78 years) who all presented in primary situation without nodal/distant metastases (Charlson score 0/1 in 18 patients; ≥2 in 17 patients). Median tumor size was 10 cm, mainly located in lower limb (83%). Stage at presentation (UICC7th) was Ib:3%, 2a:20%, 2b:20%, and 3:57%. Most lesions were high grade (97%), predominantly leiomyosarcoma (26%) and undifferentiated pleomorphic/malignant fibrous histiocytoma (23%). Limb-sparing surgery was preceded (median 50 Gy) or followed (median 66 Gy) by EBRT. Median follow-up was 37 months (range 1-128 months). Margins were free in 26 patients (74%) and microscopically positive in 9 (26%). Actuarial 3- and 5-year local control rates were 88 and 81% (4 local recurrences). Corresponding rates for distant control, disease-specific survival, and overall survival were 57/52%, 76/60%, and 72/41%. The 30-day mortality was 0%. Severe postoperative complications were scored in 8 patients (23%). Severe acute radiation-related toxicity was observed in 2 patients (6%). Patients with Charlson score ≥2 had a significantly increased risk for severe postoperative complications and acute radiation-related side effects. Severe late toxicities were found in 7 patients (20%), including fractures in 3 (8.6%). Final limb preservation rate was 97%. Combination of EBRT and limb-sparing surgery is feasible in elderly patients with acceptable toxicities and encouraging but slightly inferior outcome compared to younger patients. Comorbidity correlated with postoperative complications and acute toxicities. Late fracture risk seems slightly increased. (orig.) [German] Erfahrungsbericht zur perkutanen Radiotherapie (EBRT) kombiniert mit extremitaetenerhaltender Operation bei Patienten >70 Jahre mit Weichteilsarkom der Extremitaet. Retrospektiv

  15. Immunosuppressive Therapy-Related Kaposi Sarcoma

    Science.gov (United States)

    ... Sarcoma Treatment Childhood Vascular Tumors Treatment Research Kaposi Sarcoma Treatment (PDQ®)–Patient Version General Information About Kaposi Sarcoma Go to Health Professional Version Key Points Kaposi ...

  16. Fibromyxoid sarcoma of the pancreas

    Directory of Open Access Journals (Sweden)

    Čolović Radoje

    2008-01-01

    Full Text Available Introduction Fibromyxoid sarcoma is a rare mesenchymal neoplasm, usually appearing in the soft tissue of the extremities, less frequently in the groin, trunk, neck, and upper extremities. Within the abdomen, the tumour is usually localised within the retroperitoneum. Case OutlineWe present a 56-year-old woman in whom, during the routinely performed investigation for atacks of choking with lots of bronchial secretion, and arterial hypertension, an ultrasonographer found a tumour within the head of the pancreas 6×6 cm in diameter. At operation, a dark pink, lobulated soft tumour, surrounded by a tiny capsule, clearly different from the completely normal pancreatic tissue of the posterior side of the head of the pancreas, was easily and ideally excised.The postoperative recovery was stormy. She developed postoperative pancreatitis, temporary biliary and duodenal fistula, which all settled by conservative treatment. The histology of the 80 g weighing tumour showed a circumscribed fibromyxoid sarcoma of low malignancy. Immunohistochemistry showed diffuse vimentin and CD34 strong positivity, as well as focal anti-SMA and anti-EMA immunopositivity. Six months after surgery, she died with signs of cerebrovascular insult, asthmatic status, and recurrent suppurative abdominal fistula, probably related to the previous pancreatitis. Ultrasonography showed a possible liver secondary. The exact cause of death was not confirmed as the autopsy was refused by the family. Conclusion Primary sarcomas of the pancreas are very rare, but should be considered in differential diagnosis of pancreatic neoplasms. To the best of our knowledge, there has been no previously described fibromyxoid sarcoma of the pancreas. .

  17. A very complicated pleural effusion

    Directory of Open Access Journals (Sweden)

    J.P. Gilbert*

    2013-12-01

    Discussion: Re-expansion pulmonary oedema is a recognised complication of large pleural effusion drainage. The mechanism remains unclear, although reduced left ventricular function, in this case from a possible pericardial effusion, may be a precipitant. To prevent this phenomenon the British Thoracic Society recommends draining a maximum of 1.5 litres of fluid. This case was further complicated by a pneumothorax; again a recognised complication, especially if there is underlying poor compliance of the lung parenchyma. Re-expansion pulmonary oedema has an incidence of <1% and pneumothorax <5%. Their occurrence has not previously been reported simultaneously. Large pleural effusions are commonly encountered in clinical practice in South Africa. The existence of multiple co-morbidities including tuberculosis, HIV and impaired cardiac function may complicate their management. This case highlights the need for close monitoring and controlled drainage of pleural effusions in emergency practice.

  18. The Danish Sarcoma Database

    Directory of Open Access Journals (Sweden)

    Jorgensen PH

    2016-10-01

    Full Text Available Peter Holmberg Jørgensen,1 Gunnar Schwarz Lausten,2 Alma B Pedersen3 1Tumor Section, Department of Orthopedic Surgery, Aarhus University Hospital, Aarhus, 2Tumor Section, Department of Orthopedic Surgery, Rigshospitalet, Copenhagen, 3Department of Clinical Epidemiology, Aarhus University Hospital, Aarhus, Denmark Aim: The aim of the database is to gather information about sarcomas treated in Denmark in order to continuously monitor and improve the quality of sarcoma treatment in a local, a national, and an international perspective. Study population: Patients in Denmark diagnosed with a sarcoma, both skeletal and ekstraskeletal, are to be registered since 2009. Main variables: The database contains information about appearance of symptoms; date of receiving referral to a sarcoma center; date of first visit; whether surgery has been performed elsewhere before referral, diagnosis, and treatment; tumor characteristics such as location, size, malignancy grade, and growth pattern; details on treatment (kind of surgery, amount of radiation therapy, type and duration of chemotherapy; complications of treatment; local recurrence and metastases; and comorbidity. In addition, several quality indicators are registered in order to measure the quality of care provided by the hospitals and make comparisons between hospitals and with international standards. Descriptive data: Demographic patient-specific data such as age, sex, region of living, comorbidity, World Health Organization's International Classification of Diseases – tenth edition codes and TNM Classification of Malignant Tumours, and date of death (after yearly coupling to the Danish Civil Registration System. Data quality and completeness are currently secured. Conclusion: The Danish Sarcoma Database is population based and includes sarcomas occurring in Denmark since 2009. It is a valuable tool for monitoring sarcoma incidence and quality of treatment and its improvement, postoperative

  19. Soft tissue sarcoma - Compliance with guidelines

    NARCIS (Netherlands)

    Nijhuis, PHA; Schaapveld, M; Otter, R; Hoekstra, HJ

    2001-01-01

    BACKGROUND. Because soft tissue sarcomas (STS) are rare, guidelines for the diagnosis and treatment of patients with STS were developed. Because the diagnostic management is essential for definitive treatment, adherence to these guidelines is important. METHODS. Primary STS registered by the

  20. Intracranial Dural Metastasis of Ewing's Sarcoma: a Case Report

    International Nuclear Information System (INIS)

    Kim, Eung Yeop; Lee, Seung Koo; Kim, Dong Joon; Kim, Jin Na; Lee, Kyu Sung; Jung, Woo Hee; Kim, Dong Ik

    2008-01-01

    Ewing's sarcoma is a malignant bone tumor that can occur anywhere in the body, but it is most commonly observed in the long bones of the arms and legs, the pelvis and in the chest. The predominant sites of metastasis include the lung (38%), bone (including the spine; 31%), and the bone marrow (11%). Metastasis of Ewing's sarcoma to the central nervous system (CNS) is relatively rare, and most of the previous reports have demonstrated involvement of the bony calvarium or brain parenchyma. We describe here the imaging findings of dural metastasis of Ewing's sarcoma, and these imaging findings have not been previously reported on in the medical literature. In conclusion, dural metastasis of Ewing's sarcoma is very rare and its imaging characteristics are similar to those of a primary tumor, which mimic the findings of a schwannoma or meningioma. Despite its rarity, secondary Ewing's sarcoma may be included in the differential diagnosis of extra-axial dural masses

  1. Kaposi's sarcoma-associated herpesvirus-encoded LANA associates with glucocorticoid receptor and enhances its transcriptional activities

    International Nuclear Information System (INIS)

    Togi, Sumihito; Nakasuji, Misa; Muromoto, Ryuta; Ikeda, Osamu; Okabe, Kanako; Kitai, Yuichi; Kon, Shigeyuki; Oritani, Kenji; Matsuda, Tadashi

    2015-01-01

    Kaposi's sarcoma-associated herpesvirus (KSHV)-encoded latency-associated nuclear antigen (LANA), which interacts with cellular proteins, plays a central role in modification of viral and/or cellular gene expression. Here, we show that LANA associates with glucocorticoid receptor (GR), and that LANA enhances the transcriptional activity of GR. Co-immunoprecipitation revealed a physical interaction between LANA and GR in transiently transfected 293T and HeLa cells. In human B-lymphoma cells, LANA overexpression enhanced GR activity and cell growth suppression following glucocorticoid stimulation. Furthermore, confocal microscopy showed that activated GR was bound to LANA and accumulated in the nucleus, leading to an increase in binding of activated GR to the glucocorticoid response element of target genes. Taken together, KSHV-derived LANA acts as a transcriptional co-activator of GR. Our results might suggest a careful use of glucocorticoids in the treatment of patients with KSHV-related malignancies such as Kaposi's sarcoma, primary effusion lymphoma, and multicentric Castleman disease. - Highlights: • KSHV-LANA enhances the transcriptional activity of GR in 293T and HeLa cells. • KSHV-LANA physically associates with GR. • KSHV-LANA enhances GR activation and cell growth suppression in human B-lymphocytes. • KSHV-LANA influences the nuclear retention and DNA binding activity of GR

  2. Kaposi's sarcoma-associated herpesvirus-encoded LANA associates with glucocorticoid receptor and enhances its transcriptional activities

    Energy Technology Data Exchange (ETDEWEB)

    Togi, Sumihito; Nakasuji, Misa; Muromoto, Ryuta; Ikeda, Osamu; Okabe, Kanako; Kitai, Yuichi; Kon, Shigeyuki [Department of Immunology, Graduate School of Pharmaceutical Sciences Hokkaido University, Sapporo 060-0812 (Japan); Oritani, Kenji [Department of Hematology and Oncology, Graduate School of Medicine, Osaka University, 2-2 Yamada-oka, Suita, Osaka 565-0871 (Japan); Matsuda, Tadashi, E-mail: tmatsuda@pharm.hokudai.ac.jp [Department of Immunology, Graduate School of Pharmaceutical Sciences Hokkaido University, Sapporo 060-0812 (Japan)

    2015-07-31

    Kaposi's sarcoma-associated herpesvirus (KSHV)-encoded latency-associated nuclear antigen (LANA), which interacts with cellular proteins, plays a central role in modification of viral and/or cellular gene expression. Here, we show that LANA associates with glucocorticoid receptor (GR), and that LANA enhances the transcriptional activity of GR. Co-immunoprecipitation revealed a physical interaction between LANA and GR in transiently transfected 293T and HeLa cells. In human B-lymphoma cells, LANA overexpression enhanced GR activity and cell growth suppression following glucocorticoid stimulation. Furthermore, confocal microscopy showed that activated GR was bound to LANA and accumulated in the nucleus, leading to an increase in binding of activated GR to the glucocorticoid response element of target genes. Taken together, KSHV-derived LANA acts as a transcriptional co-activator of GR. Our results might suggest a careful use of glucocorticoids in the treatment of patients with KSHV-related malignancies such as Kaposi's sarcoma, primary effusion lymphoma, and multicentric Castleman disease. - Highlights: • KSHV-LANA enhances the transcriptional activity of GR in 293T and HeLa cells. • KSHV-LANA physically associates with GR. • KSHV-LANA enhances GR activation and cell growth suppression in human B-lymphocytes. • KSHV-LANA influences the nuclear retention and DNA binding activity of GR.

  3. Clinical management of soft tissue sarcomas

    International Nuclear Information System (INIS)

    Pinedo, H.M.; Verweij, J.

    1986-01-01

    This book is concerned with the clinical management of soft tissue sarcomas. Topics covered include: Radiotherapy; Pathology of soft tissue sarcomas; Surgical treatment of soft tissue sarcomas; and Chemotherapy in advanced soft tissue sarcomas

  4. Ewing`s Sarcoma

    Directory of Open Access Journals (Sweden)

    Agnieszka Budny

    2017-06-01

    Full Text Available Ewing's sarcoma is a small round-cell tumor typically arising in the bones, rarely in soft tissues, of children and adolescents. Clinical presentation is usually dominated by local bone pain and a mass. Magnetic resonance best defines the extent of the lesion. Patients diagnosed with Ewing's sarcoma within  last years show a improving  survival rate . Rehabilitation seems to be a crucial part of multimodal therapy.

  5. The role of liquid-based cytology and ancillary techniques in pleural and pericardic effusions: an institutional experience.

    Science.gov (United States)

    Rossi, Esther Diana; Bizzarro, Tommaso; Schmitt, Fernando; Longatto-Filho, Adhemar

    2015-04-01

    Fine-needle aspiration cytology (FNAC) of serous membrane effusions may fulfil a challenging role in the diagnostic analysis of both primary and metastatic disease. From this perspective, liquid-based cytology (LBC) represents a feasible and reliable method for empowering the performance of ancillary techniques (ie, immunocytochemistry and molecular testing) with high diagnostic accuracy. In total, 3171 LBC pleural and pericardic effusions were appraised between January 2000 and December 2013. They were classified as negative for malignancy (NM), suspicious for malignancy (SM), or positive for malignancy (PM). The cytologic diagnoses included 2721 NM effusions (2505 pleural and 216 pericardic), 104 SM effusions (93 pleural and 11 pericardic), and 346 PM effusions (321 pleural and 25 pericardic). The malignant pleural series included 76 unknown malignancies (36 SM and 40 PM effusions), 174 metastatic lesions (85 SM and 89 PM effusions), 14 lymphomas (3 SM and 11 PM effusions), 16 mesotheliomas (5 SM and 11 SM effusions), and 3 myelomas (all SM effusions). The malignant pericardic category included 20 unknown malignancies (5 SM and 15 PM effusions), 15 metastatic lesions (1 SM and 14 PM effusions), and 1 lymphoma (1 PM effusion). There were 411 conclusive immunocytochemical analyses and 47 molecular analyses, and the authors documented 88% sensitivity, 100% specificity, 98% diagnostic accuracy, 98% negative predictive value, and 100% positive predictive value for FNAC. FNAC represents a primary diagnostic tool for effusions and a reliable approach with which to determine the correct follow-up. Furthermore, LBC is useful for ancillary techniques, such as immunocytochemistry and molecular analysis, with feasible diagnostic and predictive utility. © 2015 American Cancer Society.

  6. Considerations for the long term treatment of pediatric sarcoma survivors

    Directory of Open Access Journals (Sweden)

    Kurt R Weiss

    2018-01-01

    Full Text Available Sarcomas are primary malignancies of the connective tissues. They are exceedingly rare in adults, but much more common in children. The historically recent advent of cytotoxic chemotherapy for pediatric sarcomas has revolutionized the treatment of these diseases and dramatically improved their prognoses. There is thus a population of pediatric sarcoma survivors that are “coming of age” as adults. However, this progress is not without consequences. Due to aggressive treatment protocols that include various combinations of surgery, chemotherapy, and radiation therapy, pediatric sarcoma survivors are at risk of myriad physical, medical, and psychological difficulties as they enter adulthood. These include but are not limited to physical disabilities, chemotherapy-induced cardiac issues, second malignancies, and anxiety. These patients pose unique challenges to their adult primary care physicians. One possible solution to these challenges is multidisciplinary sarcoma survivorship clinics. By paying greater attention to the unique issues of pediatric sarcoma survivors, involved physicians can maximize the physical and emotional health of pediatric sarcoma survivors.

  7. Treatment Option Overview (Kaposi Sarcoma)

    Science.gov (United States)

    ... Treatment Childhood Vascular Tumors Treatment Research Kaposi Sarcoma Treatment (PDQ®)–Patient Version General Information About Kaposi Sarcoma ... Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery ) and treatment ...

  8. Pleuropericardial effusion associated with minoxidil administration

    Science.gov (United States)

    Webb, D. B.; Whale, R. J.

    1982-01-01

    A patient on minoxidil developed pericardial and pleural effusions with a high protein content. This finding is not compatible with the view that such effusions in patients taking minoxidil are transudates. PMID:7111124

  9. [Pleural effusion: diagnosis and management].

    Science.gov (United States)

    Pastré, J; Roussel, S; Israël Biet, D; Sanchez, O

    2015-04-01

    Pleural effusion management is a common clinical situation associated with numerous pulmonary, pleural or extra-pulmonary diseases. A systematic approach is needed to enable a rapid diagnosis and an appropriate treatment. Pleural fluid analysis is the first step to perform which allows a presumptive diagnosis in most cases. Otherwise, further analysis of the pleural fluid or thoracic imaging or pleural biopsy may be necessary. This review aims at highlighting the important elements of the work-up required by a pleural effusion. Copyright © 2014 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.

  10. Synovial sarcoma with radiological appearances of primitive neuroectodermal tumour/Ewing sarcoma: differentiation by molecular genetic studies

    International Nuclear Information System (INIS)

    O'Donnell, P.; Diss, T.C.; Whelan, J.; Flanagan, A.M.

    2006-01-01

    Synovial sarcoma (SS) arises in soft tissues but may invade adjacent bone. We describe a case of SS presenting as aggressive lysis of the proximal ulna, the imaging of which suggested a primary bone lesion. Needle biopsy showed a 'small round blue cell tumour', and a primitive neuroectodermal tumour (PNET)/Ewing sarcoma was suggested on the basis of the imaging appearances. The definitive diagnosis of synovial sarcoma was made following molecular genetic studies, which demonstrated a fusion product incorporating the genes SYT and SSX1. The importance of correct diagnosis to guide appropriate management, and, therefore, the necessity for molecular genetic studies, is discussed. (orig.)

  11. A rare case of synovial sarcoma of the prostate | Dhabalia | African ...

    African Journals Online (AJOL)

    Prostatic synovial sarcomas are exceedingly rare. To our knowledge, only six primary cases have been reported so far. We herein describe a primary synovial sarcoma of the prostate seen in a 25- year-old male patient, the youngest patient seen with this disease to date. He was referred to our department with the diagnosis ...

  12. Management of Septated Malignant Pleural Effusions

    OpenAIRE

    Banka, Radhika; Terrington, Dayle; Mishra, Eleanor K.

    2018-01-01

    Purpose of Review: We review recent studies of patients with septated malignant pleural effusions, to understand what the clinical implications for patients are and what evidence-based methods should be used to manage these effusions. Recent Findings: Fibrinolytics improve effusion size assessed radiologically in patients with a chest drain inserted for septated malignant pleural effusions but this does not translate into an improvement in breathlessness relief or pleurodesis success. Fibrino...

  13. Postoperative pleural effusion following upper abdominal surgery

    DEFF Research Database (Denmark)

    Nielsen, P H; Jepsen, S B; Olsen, A D

    1989-01-01

    amylases, sex, smoking habits, or weight. There was no correlation between the localization of the pleural effusions and that of the abdominal incisions. There was a positive correlation between atelectasis and pleural effusion, but no evidence of a causal relationship. Pleural effusions might be related...

  14. Osteogenic sarcoma of the skull. A clinicopathologic study of 19 patients

    International Nuclear Information System (INIS)

    Huvos, A.G.; Sundaresan, N.; Bretsky, S.S.; Butler, A.

    1985-01-01

    The authors studied 19 patients with well documented osteogenic sarcomas arising in the skull, which represent 1.6% of all osteogenic sarcomas registered during a 60-year period (1921-1981). Ten sarcomas were primary, de novo tumors. Nine others developed secondary osteogenic sarcomas; among these, six arose as a complication of Paget's disease, two followed irradiation, and one was associated with pre-existent fibrous dysplasia. The sarcomas arose in equal proportion in both sexes with the men being much older (mean age, 44 years) as compared to the women (mean age, 31 years). Patients with de novo osteogenic sarcomas were considerably younger than those with secondary lesions. Osteoblastic osteogenic sarcoma was by far the most common histologic variant in both the primary and the Paget's sarcomas. None of the patients with Paget's sarcoma lived longer than 1 year; the median survival here was 4 months. Patients with de novo osteogenic sarcomas fared much better and there are four long-term survivors (longer than 3 years) who are currently disease-free

  15. Lymphoproliferative disorder in pleural effusion in a subject with past asbestos exposure

    Directory of Open Access Journals (Sweden)

    Naofumi Hara

    2015-01-01

    Full Text Available Primary effusion lymphoma (PEL is a subtype of non-Hodgkin lymphoma that presents as serous effusions without detectable masses or organomegaly. Here we report a case of PEL-like lymphoma in a patient with past asbestos exposure. A 65-year-old man was referred to our hospital due to dyspnea upon exertion. He had been exposed to asbestos for three years in the construction industry. Chest X-ray and CT images demonstrated left pleural effusion. Cytological analysis of the pleural effusion revealed large atypical lymphocytes with distinct nuclear bodies and high nucleus-to-cytoplasm ratio. Immunohistochemical analyses showed that the cells were CD20+, CD3−, CD5−, and CD10−. These findings led to a diagnosis of diffuse large B-cell lymphoma. PEL or PEL-like lymphoma should be considered a potential cause of pleural effusion in subjects with past asbestos exposure.

  16. The epigenetic landscape of latent Kaposi sarcoma-associated herpesvirus genomes.

    Directory of Open Access Journals (Sweden)

    Thomas Günther

    Full Text Available Herpesvirus latency is generally thought to be governed by epigenetic modifications, but the dynamics of viral chromatin at early timepoints of latent infection are poorly understood. Here, we report a comprehensive spatial and temporal analysis of DNA methylation and histone modifications during latent infection with Kaposi Sarcoma-associated herpesvirus (KSHV, the etiologic agent of Kaposi Sarcoma and primary effusion lymphoma (PEL. By use of high resolution tiling microarrays in conjunction with immunoprecipitation of methylated DNA (MeDIP or modified histones (chromatin IP, ChIP, our study revealed highly distinct landscapes of epigenetic modifications associated with latent KSHV infection in several tumor-derived cell lines as well as de novo infected endothelial cells. We find that KSHV genomes are subject to profound methylation at CpG dinucleotides, leading to the establishment of characteristic global DNA methylation patterns. However, such patterns evolve slowly and thus are unlikely to control early latency. In contrast, we observed that latency-specific histone modification patterns were rapidly established upon a de novo infection. Our analysis furthermore demonstrates that such patterns are not characterized by the absence of activating histone modifications, as H3K9/K14-ac and H3K4-me3 marks were prominently detected at several loci, including the promoter of the lytic cycle transactivator Rta. While these regions were furthermore largely devoid of the constitutive heterochromatin marker H3K9-me3, we observed rapid and widespread deposition of H3K27-me3 across latent KSHV genomes, a bivalent modification which is able to repress transcription in spite of the simultaneous presence of activating marks. Our findings suggest that the modification patterns identified here induce a poised state of repression during viral latency, which can be rapidly reversed once the lytic cycle is induced.

  17. Management of malignant pleural effusion

    NARCIS (Netherlands)

    Boshuizen, R.C.

    2017-01-01

    The first part of this thesis focuses on IPCs (indwelling pleural catheters) in malignant pleural effusion (MPE) management. In an invited review, the (dis)advantages and prejudices of IPCs are described (Chapter1.1). Since costs and reimbursement issues are the main reasons in the Netherlands to

  18. Primary renal osteosarcoma: A case report

    African Journals Online (AJOL)

    C. Ahomadégbé

    Primitive renal osteosarcoma is a rare sarcoma of the kidney with only 27 cases reported in the literature. Its histogenesis .... low-grade extraskeletal osteosarcoma. Skeletal ... et al. Primary Ewing's sarcoma/primitive neuroectodermal tumor of.

  19. Matrix metalloproteinase 1: role in sarcoma biology.

    Directory of Open Access Journals (Sweden)

    Muhammad Umar Jawad

    2010-12-01

    Full Text Available In carcinomas stromal cells participate in cancer progression by producing proteases such as MMPs. The expression MMP1 is a prognostic factor in human chondrosarcoma, however the role in tumor progression is unknown. Laser capture microdissection and In Situ hybridization were used to determine cellular origin of MMP1 in human sarcomas. A xenogenic model of tumor progression was then used and mice were divided in two groups: each harboring either the control or a stably MMP1 silenced cell line. Animals were sacrificed; the neovascularization, primary tumor volumes, and metastatic burden were assessed. LCM and RNA-ISH analysis revealed MMP1 expression was predominantly localized to the tumor cells in all samples of sarcoma (p = 0.05. The percentage lung metastatic volume at 5 weeks (p = 0.08 and number of spontaneous deaths secondary to systemic tumor burden were lower in MMP1 silenced cell bearing mice. Interestingly, this group also demonstrated a larger primary tumor size (p<0.04 and increased angiogenesis (p<0.01. These findings were found to be consistent when experiment was repeated using a second independent MMP1 silencing sequence. Prior clinical trials employing MMP1 inhibitors failed because of a poor understanding of the role of MMPs in tumor progression. The current findings indicating tumor cell production of MMP1 by sarcoma cells is novel and highlights the fundamental differences in MMP biology between carcinomas and sarcomas. The results also emphasize the complex roles of MMP in tumor progression of sarcomas. Not only does metastasis seem to be affected by MMP1 silencing, but also local tumor growth and angiogenesis are affected inversely.

  20. Massive pericardial effusion associated with hypothyroidism.

    Science.gov (United States)

    Ionescu, Simona Daniela; Tănase, Daniela Maria; Ouatu, Anca; Ambăruş, V; Dosa, Anca; Arsenescu-Georgescu, Cătălina

    2014-01-01

    The diagnosis of hypothyroidism is difficult because hypothyroidism in adults and especially the elderly, classic, has an insidious onset with a range of nonspecific symptoms which may delay diagnosis for months or even years. Old age seems to represent trigger factor for autoimmune diseases, including hypothyroidism. Clinical features in hypothyroidism, such as weight gain, fatigue, cold intolerance, constipation, dry skin, edema and muscle weakness, and decreased osteo-tendinous reflexes are usually subtle and can be overlooked. Thyroid dysfunction may be associated with a negative impact on the cardiovascular system. Pericardial, pleural and peritoneal effusions are common findings in hypothyroidism. This case report represents a typical primary hypothyroidism (autoimmune) and shows the clinical features of this disease. Basically we talked about a severe myxedema with the involvement of internal organs in an elderly woman and the euthyroidism restoration, under thyroid replacement therapy, was correlated with the clinical improvement and cardiovascular and neurological status, with radiographic remission and regression to extinction of pericardial effusion at repeated echocardiographic evaluations.

  1. Primary Angiosarcoma of the Spleen: An Oncological Enigma

    Directory of Open Access Journals (Sweden)

    Myoteri Despoina

    2014-01-01

    Full Text Available Introduction. Primary splenic angiosarcoma is an extremely unusual neoplasm originating from sinusoidal vascular endothelium. Surgical extirpation is the mainstay of treatment of this highly malignant disease. Case Presentation. An 82-year-old woman was admitted with left pleural effusion and a palpable left upper quadrant abdominal mass, secondary to splenomegaly by two large splenic tumors. Classic open splenectomy was performed and angiosarcoma of the spleen was the final histopathological diagnosis, which was primary since no other disease site was revealed. Discussion. The incidence of the disease is 0.14–0.23 cases per million, with slight male predominance. Etiology is not established and clinical presentation may confuse even experienced physicians. Imaging modalities cannot differentiate the lesion from other vascular splenic neoplasms and the correct diagnosis is mainly set after histopathological examination of the resected spleen. As with other sarcomas, surgery is the only curative approach, while chemo- and radiotherapy have poor results. Prognosis remains dismal.

  2. Physiology of breathlessness associated with pleural effusions

    Science.gov (United States)

    Thomas, Rajesh; Jenkins, Susan; Eastwood, Peter R.; Lee, Y.C. Gary; Singh, Bhajan

    2015-01-01

    Purpose of review Pleural effusions have a major impact on the cardiorespiratory system. This article reviews the pathophysiological effects of pleural effusions and pleural drainage, their relationship with breathlessness, and highlights key knowledge gaps. Recent findings The basis for breathlessness in pleural effusions and relief following thoracentesis is not well understood. Many existing studies on the pathophysiology of breathlessness in pleural effusions are limited by small sample sizes, heterogeneous design and a lack of direct measurements of respiratory muscle function. Gas exchange worsens with pleural effusions and improves after thoracentesis. Improvements in ventilatory capacity and lung volumes following pleural drainage are small, and correlate poorly with the volume of fluid drained and the severity of breathlessness. Rather than lung compression, expansion of the chest wall, including displacement of the diaphragm, appears to be the principle mechanism by which the effusion is accommodated. Deflation of the thoracic cage and restoration of diaphragmatic function after thoracentesis may improve diaphragm effectiveness and efficiency, and this may be an important mechanism by which breathlessness improves. Effusions do not usually lead to major hemodynamic changes, but large effusions may cause cardiac tamponade and ventricular diastolic collapse. Patients with effusions can have impaired exercise capacity and poor sleep quality and efficiency. Summary Pleural effusions are associated with abnormalities in gas exchange, respiratory mechanics, respiratory muscle function and hemodynamics, but the association between these abnormalities and breathlessness remains unclear. Prospective studies should aim to identify the key mechanisms of effusion-related breathlessness and predictors of improvement following pleural drainage. PMID:25978627

  3. Subdural effusions in children under two years

    International Nuclear Information System (INIS)

    Rothenberger, A.; Brandl, H.

    1980-01-01

    We investigated 161 children under 24 months of age by CT of the skull and reviewed the anamnestic and clinical history. 87 children showed subdural effusions, and 74 did not. There were 33 patients with other pathological findings in CT, and 41 had normal scans. Age and sex distribution as well as localization of the subdural effusions were consistent with the literature. The 87 children with subdural effusions represented 1,7% in a sample of about 5.000 CT scans. CT was the most reliable method for diagnosis of subdural effusions, compared to other techniques. There was a preponderance of small subdural effusions from 1 to 7 mm thickness (51%). Other CT abnormalities accompanying subdural effusions were found. Most frequently the interhemispheric sulcus was dilated and an internal hydrocephalus was present. Also in our group there were 7 anamnestical and 6 clinical symptoms highly diagnostic of subdural effusions. (orig.) [de

  4. Unusual Presentation of Postradiation Sarcoma With Subsequent Intestinal Metastasis Revealed by 18F-FDG PET/CT.

    Science.gov (United States)

    Lee, Tse-Hao; Chang, Cheng-Pei; Wu, Yuan-Hung; Huang, Wen-Sheng

    2017-10-01

    Radiation-related sarcoma is a rare but serious complication for patients after receiving radiation therapy initially for their primary malignancy. However, it usually takes several years and needs enough in-field radiation dose to develop this sarcoma. We describe a 60-year-old man with right axillary sarcoma revealed by whole-body F-FDG PET/CT 1 year after chemoradiotherapy, which presented ultrashort latency period and did not match the consensus definition of radiation-related sarcoma. Besides, metastatic sarcoma to small intestine was accidentally noted in the F-FDG PET/CT image.

  5. Sarcoma as a second malignancy after treatment for breast cancer

    International Nuclear Information System (INIS)

    Yap, Johnny; Chuba, Paul J.; Thomas, Ron; Aref, Amr; Lucas, David; Severson, Richard K.; Hamre, Merlin

    2002-01-01

    Background: Second malignant neoplasms may be a consequence of radiotherapy for the treatment of breast cancer. Prior studies evaluating sarcomas as second malignant neoplasms in breast cancer patients have been limited by the numbers of patients and relatively low incidence of sarcoma. Using data from the Surveillance, Epidemiology and End Results registries, we evaluated the influence of radiation therapy on the development of subsequent sarcomas in cases with primary breast cancer. Methods: Cases with primary invasive breast cancer (n=274,572) were identified in the Surveillance, Epidemiology and End Results Cancer Incidence Public-Use Database (1973-1997). The database was then queried to determine the cases developing subsequent sarcomas (n=263). Eighty-seven of these cases received radiation therapy, and 176 had no radiation therapy. The cumulative incidence of developing secondary sarcoma and the survival post developing secondary sarcoma were determined by the Kaplan-Meier method. Results: The occurrence of sarcoma was low, regardless of whether cases received or did not receive radiation therapy: 3.2 per 1,000 (SE [standard error] = 0.4) and 2.3 per 1,000 (SE=0.2) cumulative incidence at 15 years post diagnosis, respectively (p=0.001). Of the sarcomas occurring within the field of radiation, angiosarcoma accounted for 56.8%, compared to only 5.7% of angiosarcomas occurring in cases not receiving radiotherapy. The cumulative incidence of angiosarcoma at 15 years post diagnosis was 0.9 per 1,000 for cases receiving radiation (SE=0.2) and 0.1 per 1,000 for cases not receiving radiation (SE<0.1). Overall survival was poor for cases of sarcoma after breast cancer (27-35% at 5 years), but not significantly different between patients receiving or not receiving radiation therapy for their primary breast cancer. Conclusions: Radiotherapy in the treatment of breast cancer is associated with an increased risk of subsequent sarcoma, but the magnitude of this risk is

  6. Leukosis/Sarcoma Group

    Science.gov (United States)

    The leukosis/sarcoma (L/S) group of diseases designates a variety of transmissible benign and malignant neoplasms of chickens caused by members that belong to the family Retroviridae. Because the expansion of the literature on this disease, it is no longer feasible to cite all relevant publications ...

  7. Extremity perfusion for sarcoma

    NARCIS (Netherlands)

    Hoekstra, Harald Joan

    2008-01-01

    For more than 50 years, the technique of extremity perfusion has been explored in the limb salvage treatment of local, recurrent, and multifocal sarcomas. The "discovery" of tumor necrosis factor-or. in combination with melphalan was a real breakthrough in the treatment of primarily irresectable

  8. Synovial sarcoma mechanisms

    DEFF Research Database (Denmark)

    Svejstrup, Jesper Q

    2013-01-01

    Human synovial sarcoma is caused by a chromosome translocation, which fuses DNA encoding SSX to that encoding the SS18 protein. Kadoch and Crabtree now show that the resulting cellular transformation stems from disruption of the normal architecture and function of the human SWI/SNF (BAF) complex....

  9. An Unusual Location of Extraosseous Ewing's Sarcoma

    Directory of Open Access Journals (Sweden)

    Lisanne Geens

    2013-05-01

    Full Text Available Ewing's sarcoma (ES is the second most common malignant bone tumor in children and young adults. ES also occurs as a primary soft tissue neoplasm without involvement of bone. We report the second case of extraosseous (EO ES emerging from the omentum and a review of the relevant literature. EO ES should be included in the differential diagnosis of soft tissue neoplasms in the abdomen.

  10. Immunotherapy of childhood Sarcomas

    Directory of Open Access Journals (Sweden)

    Stephen S Roberts

    2015-08-01

    Full Text Available Pediatric sarcomas are a heterogeneous group of malignant tumors of bone and soft tissue origin. Although more than 100 different histologic subtypes have been described, the majority of pediatric cases belong to the Ewing’s family of tumors, rhabdomyosarcoma and osteosarcoma. Most patients that present with localized stage are curable with surgery and/or chemotherapy; however, those with metastatic disease at diagnosis or those who experience a relapse continue to have a very poor prognosis. New therapies for these patients are urgently needed. Immunotherapy is an established treatment modality for both liquid and solid tumors, and in pediatrics, most notably for neuroblastoma and osteosarcoma. In the past, immunomodulatory agents such as interferon, interleukin-2, and Liposomal-muramyl  tripeptide phosphatidyl-ethanolamine (L-MTP have been tried, with some activity seen in subsets of patients; additionally, various cancer vaccines have been studied with possible benefit. Monoclonal antibody therapies against tumor antigens such as disialoganglioside GD2 or immune checkpoint targets such as CTLA4 and PD-1 are being actively explored in pediatric sarcomas. Building on the success of adoptive T cell therapy for EBV-related lymphoma, strategies to redirect T cells using chimeric antigen receptors and bispecific antibodies are rapidly evolving with potential for the treatment of sarcomas. This review will focus on recent preclinical and clinical developments in targeted agents for pediatric sarcomas with emphasis on the immunobiology of immune checkpoints, immunoediting, tumor microenvironment, antibody engineering, cell engineering, and tumor vaccines. The future integration of antibody based and cell based therapies into an overall treatment strategy of sarcoma will be discussed.

  11. Systematic identification of cellular signals reactivating Kaposi sarcoma-associated herpesvirus.

    Directory of Open Access Journals (Sweden)

    Fuqu Yu

    2007-03-01

    Full Text Available The herpesvirus life cycle has two distinct phases: latency and lytic replication. The balance between these two phases is critical for viral pathogenesis. It is believed that cellular signals regulate the switch from latency to lytic replication. To systematically evaluate the cellular signals regulating this reactivation process in Kaposi sarcoma-associated herpesvirus, the effects of 26,000 full-length cDNA expression constructs on viral reactivation were individually assessed in primary effusion lymphoma-derived cells that harbor the latent virus. A group of diverse cellular signaling proteins were identified and validated in their effect of inducing viral lytic gene expression from the latent viral genome. The results suggest that multiple cellular signaling pathways can reactivate the virus in a genetically homogeneous cell population. Further analysis revealed that the Raf/MEK/ERK/Ets-1 pathway mediates Ras-induced reactivation. The same pathway also mediates spontaneous reactivation, which sets the first example to our knowledge of a specific cellular pathway being studied in the spontaneous reactivation process. Our study provides a functional genomic approach to systematically identify the cellular signals regulating the herpesvirus life cycle, thus facilitating better understanding of a fundamental issue in virology and identifying novel therapeutic targets.

  12. Massive pericardial effusion and rhabdomyolysis secondary to untreated severe hypothyroidism: the first report.

    Science.gov (United States)

    Zare-Khormizi, M R; Rahmanian, M; Pourrajab, F; Akbarnia, S

    2014-10-01

    Hypothyroidism is an endocrine disease with various clinical manifestations. It is a rare cause for rhabdomyolysis and massive pericardial effusion. We describe a case of severe hypothyroidism secondary to autoimmune hashimoto thyroiditis with massive pericardial effusion and rhabdomyolysis. Improvement of mentioned complications after hypothyroidism treatment and rule out of other possible causes are supportive clues that hypothyroidism is the main cause of patient's rare presentation. With the best of our knowledge, it is the first report of rhabdomyolysis and massive pericardial effusion coincidence in a patient of adult population with primary uncontrolled hypothyroidism for years.

  13. Parapneumonic effusions in children: five years’ experience

    Directory of Open Access Journals (Sweden)

    Atilla Cifci

    2017-12-01

    Full Text Available Aim: Most severe complication of respiratory tract infections that causing morbidity and mortality in children is parapneumonic effusion(PPE. PPE is a pleural exudate that is related with primary pneumonia. The early and appropriate antibiotic treatment is very important in follow-up of patients who are diagnosed as parapneumonic effusion and also the timing of interventional and surgical treatment is important to decrease morbidity and mortalitiy in whom clinical and laboratory findings are not cured enough. Materials and Methods: In this study, the clinical and laboratory findings of parapneumonic effusion one hundred patients applied to one center in five years time are discussed. Results: The mean age of patients were 52 months(1.5-156, 52 were male(52%.The 71% of patients were smaller than five years old. The mean duration of hospitalization of patients were 19.6 days(1-45 days. Most frequent spymptom in application was fever, most frequent sign were tachycardia and retractions, most frequent laboratory anormality was high white blood count. The most frequent microorganism in pleural fluid culture was S.aureus. Conclusion: The determination of pathogens causing parapneumonic effusions in our country is very important for starting most suitable treatment early and to decrease morbidity and mortality. [J Contemp Med 2017; 7(4.000: 340-347

  14. [Pleural lymphatics and effusions].

    Science.gov (United States)

    Mordant, P; Arame, A; Legras, A; Le Pimpec Barthes, F; Riquet, M

    2013-06-01

    The pleural lymphatic system has a great absorption capacity. Its most known function is fluid resorption. The pleura which cover the lungs (visceral pleura), the mediastinum, diaphragm and thoracic wall (parietal pleura) are formed by a mesothelial cell layer (mesothelium). This permeable layer is in direct contact with the vascular endothelium. The mesothelium is based over a connective tissue (interstitium) containing the blood and lymphatic vessels. The primary lymphatic vessels drain interstitium but are also in direct contact with pleural space by the stoma or openings, situated in the lower parts of parietal pleura, i.e: diaphragm, over lower ribs and mediastinum but not existing in the adjacent visceral pleura. In addition, a part of interstitial pulmonary fluid entered in the pleural cavity by passing the visceral pleura would be absorbed by these openings. The resorption process is active and directly related to the function of smooth muscles of lymphatic vessels. Besides resorption, we must emphasize that this "pumping" activity is permanent and the origin of negative pressure (the pleural void) in pleural cavity, a unique property. The other resorbed elements are molecules, bacterial and cellular debris, cells, red blood and cancer cells. Copyright © 2013. Published by Elsevier Masson SAS.

  15. Ewing's sarcoma of bone tumor cells produces MCSF that stimulates monocyte proliferation in a novel mouse model of Ewing's sarcoma of bone.

    Science.gov (United States)

    Margulies, B S; DeBoyace, S D; Damron, T A; Allen, M J

    2015-10-01

    Ewing's sarcoma of bone is a primary childhood malignancy of bone that is treated with X-radiation therapy in combination with surgical excision and chemotherapy. To better study Ewing's sarcoma of bone we developed a novel model of primary Ewing's sarcoma of bone and then treated animals with X-radiation therapy. We identified that uncontrolled tumor resulted in lytic bone destruction while X-radiation therapy decreased lytic bone destruction and increased limb-length asymmetry, a common, crippling complication of X-radiation therapy. Osteoclasts were indentified adjacent to the tumor, however, we were unable to detect RANK-ligand in the Ewing's tumor cells in vitro, which lead us to investigate alternate mechanisms for osteoclast formation. Ewing's sarcoma tumor cells and archival Ewing's sarcoma of bone tumor biopsy samples were shown to express MCSF, which could promote osteoclast formation. Increased monocyte numbers were detected in peripheral blood and spleen in animals with untreated Ewing's sarcoma tumor while monocyte number in animals treated with x-radiation had normal numbers of monocytes. Our data suggest that our Ewing's sarcoma of bone model will be useful in the study Ewing's sarcoma tumor progression in parallel with the effects of chemotherapy and X-radiation therapy. Copyright © 2015 Elsevier Inc. All rights reserved.

  16. Ewing's Sarcoma of Bone Tumor Cells Produce MCSF that Stimulates Monocyte Proliferation in a Novel Mouse Model of Ewing's Sarcoma of Bone

    Science.gov (United States)

    Margulies, BS; DeBoyace, SD; Damron, TA; Allen, MJ

    2015-01-01

    Ewing's sarcoma of bone is a primary childhood malignancy of bone that is treated with X-radiation therapy in combination with surgical excision and chemotherapy. To better study Ewing's sarcoma of bone we developed a novel model of primary Ewing's sarcoma of bone and then treated animals with X-radiation therapy. We identified that uncontrolled tumor resulted in lytic bone destruction while X-radiation therapy decreased lytic bone destruction and increased limb-length asymmetry, a common, crippling complication of X-radiation therapy. Osteoclasts were indentified adjacent to the tumor, however, we were unable to detect RANK-ligand in the Ewing's tumor cells in vitro, which lead us to investigate alternate mechanisms for osteoclast formation. Ewing's sarcoma tumor cells and archival Ewing's sarcoma of bone tumor biopsy samples were shown to express MCSF, which could promote osteoclast formation. Increased monocyte numbers were detected in peripheral blood and spleen in animals with untreated Ewing's sarcoma tumor while monocyte number in animals treated with x-radiation had normal numbers of monocytes. Our data suggest that our Ewing's sarcoma of bone model will be useful in the study Ewing's sarcoma tumor progression in parallel with the effects of chemotherapy and X-radiation therapy. PMID:26051470

  17. MR imaging of pleural and peritoneal effusion

    International Nuclear Information System (INIS)

    Shiono, Takahiro; Yoshikawa, Kohki; Takenaka, Eiichi; Hisamatsu, Katsuji

    1993-01-01

    The purpose of this study was to assess the efficacy of MR imaging in predicting the quality of ascites and pleural effusion. MR examinations of 20 patients with different benign or malignant diseases accompanied with ascites or pleural effusion were retrospectively studied. Results were compared with histopathological findings. On T 1 - and T 2 -weighted images, the MR intensity of the effusion depended mainly on the concentration of protein, whereas on gradient echo images, it depended mainly on the concentration of blood. MR examination was useful in non-invasively differentiating exudative or hemorrhagic effusion from serous effusion. Knowledge or inference of the quality of effusion can direct MR interpretation and may improve diagnostic accuracy. (author)

  18. Pericardial Effusion and Pericardiocentesis: Role of Echocardiography

    Science.gov (United States)

    2012-01-01

    Pericardial effusion can develop from any pericardial disease, including pericarditis and several systemic disorders, such as malignancies, pulmonary tuberculosis, chronic renal failure, thyroid diseases, and autoimmune diseases. The causes of large pericardial effusion requiring invasive pericardiocentesis may vary according to the time, country, and hospital. Transthoracic echocardiography is the most important tool for diagnosis, grading, the pericardiocentesis procedure, and follow up of pericardial effusion. Cardiac tamponade is a kind of cardiogenic shock and medical emergency. Clinicians should understand the tamponade physiology, especially because it can develop without large pericardial effusion. In addition, clinicians should correlate the echocardiographic findings of tamponade, such as right ventricular collapse, right atrial collapse, and respiratory variation of mitral and tricuspid flow, with clinical signs of clinical tamponade, such as hypotension or pulsus paradoxus. Percutaneous pericardiocentesis has been the most useful procedure in many cases of large pericardial effusion, cardiac tamponade, or pericardial effusion of unknown etiology. The procedure should be performed with the guidance of echocardiography. PMID:23236323

  19. Roentgenologic examination in Kaposi's sarcoma

    International Nuclear Information System (INIS)

    Kossovoj, A.L.

    1990-01-01

    Review of roentgenologic investigations into Kaposi's sarcoma is presented. It is shown that Kaposi's sarcoma is a disease injuring skin, osteal system, lungs and mediastinum, gastroeuteric tract and lymphatic nodes. Roentgenologic changes of soft tissues of limbs, osteal system, chest and gastroenteric tract organs are described. Manifestations of a tumor of any localization are quite different which makes it more difficult to perform roentgenologic diagnosis. An increase of Kaposi's sarcoma occurrence in patients suffering from aids as the disease increases is indicated

  20. Uterine sarcoma – current perspectives

    Directory of Open Access Journals (Sweden)

    Benson C

    2017-08-01

    Full Text Available Charlotte Benson,1 Aisha B Miah1,2 1Sarcoma Unit, Royal Marsden Hospital, 2Department of Radiotherapy and Imaging, The Institute of Cancer Research, London, UK Abstract: Uterine sarcomas comprise a group of rare tumors with differing tumor biology, natural history and response to treatment. Diagnosis is often made following surgery for presumed benign disease. Currently, preoperative imaging does not reliably distinguish between benign leiomyomas and other malignant pathology. Uterine leiomyosarcoma is the most common sarcoma, but other subtypes include endometrial stromal sarcoma (low grade and high grade, undifferentiated uterine sarcoma and adenosarcoma. Clinical trials have shown no definite survival benefit of adjuvant radiotherapy or chemotherapy and have been hampered by the rarity and heterogeneity of these disease types. There is a role of adjuvant treatment in carefully selected cases following multidisciplinary discussion at sarcoma reference centers. In patients with metastatic disease, systemic chemotherapy can then be considered. There is activity of a number of agents, including doxorubicin, trabectedin, gemcitabine-based chemotherapy, eribulin and pazopanib. Patients should be considered for clinical trial entry where possible. Close international collaboration is important to allow progress in this group of diseases. Keywords: sarcoma, leiomyosarcoma, endometrial stromal sarcoma, undifferentiated uterine sarcoma, leiomyoma

  1. Targeted therapies for bone sarcomas

    International Nuclear Information System (INIS)

    Mudry, P.

    2011-01-01

    Therapy success in bone sarcoma is significantly better compared to history cohorts with 60 - 70 % overall survival to date. Unfortunately, there is yet no shift and movement in better survival of patients with relapsed and refractory bone sarcomas during last twenty years. This article reviews targeted therapeutics for bone sarcomas which are under investigation and which could give chance to patients suffering from relapsed and chemo resistant bone sarcomas. Majority of the targeted drugs are given as part of phase 1 or 2 studies. (author)

  2. Lymphocytic Pleural Effusion in Acute Melioidosis

    Directory of Open Access Journals (Sweden)

    Kuo-Mou Chung

    2007-10-01

    Full Text Available An endemic outbreak of melioidosis developed in southern Taiwan following a flood caused by a typhoon in July 2005. A total of 27 patients were diagnosed with the acute and indigenous form of pulmonary melioidosis. Parapneumonic pleural effusions were noted on chest X-rays in six patients. Thoracentesis was done in three patients and all revealed lymphocyte predominance in differential cell count. Burkholderia pseudomallei was isolated in the pleural effusion in one of them. All three patients survived after antibiotic treatment. Lymphocytic pleural effusion is generally seen in tuberculosis or malignancy. However, our findings suggest that melioidosis should be considered in the differential diagnosis of lymphocytic pleural effusion.

  3. Morel-Lavallee effusions in the thigh

    International Nuclear Information System (INIS)

    Parra, J.A.; Fernandez, M.A.; Encinas, B.; Rico, M.

    1997-01-01

    Objective. To assess the radiological features of Morel-Lavallee effusion in the thigh. Design and patients. A restrospective study of clinical records and radiological studies was carried out on three patients with Morel-Lavallee effusion. Results and conclusions. Ultrasound and computed tomography (CT) were able to locate the effusion in the three patients. CT demonstrated a capsule around the lesion. A fluid-fluid level was visible with both techniques. Ultrasound and CT are good methods for assessing patients with Morel-Lavallee effusion of the thigh. CT can be used to distinguish patients requiring surgical treatment from those who could be treated by conservative measures. (orig.). With 4 figs

  4. Microfilaria in pleural effusion: An unusual association

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    Rehena Sarkar

    2016-01-01

    Full Text Available Lymphatic filariasis is a major public health problem in tropical countries and India is endemic for it. However, lymphatic filariasis presenting as pleural effusion is an unusual manifestation and finding microfilaria in pleural effusion without any lung pathology is rare. We report a case of pleural effusion without any underlying lung pathology and normal blood picture. Clinical cure occurred after treatment with diethyl-carbamazepine. Filariasis should be kept in view while considering the differential diagnosis of idiopathic pleural effusion, especially in endemic countries.

  5. Ultrasonographic Observations of the Pleural Effusion

    International Nuclear Information System (INIS)

    Lee, Dong Hoo; Park, Sung Soo; Lee, Chung Hee

    1982-01-01

    Five cases of patients with pleural effusion were evaluated by the grey-scale ultrasonography. Ultrasonography of pleural effusion in each case was represented as fluid accumulation within the pleural cavity with anechoic crescent moon shape or saddle appearance marginated by diaphragm. Ptosis of the liver with demonstrable right diaphragm was assessment in the severe right pleural effusion. it is emphasized that the practical advantages of the ultrasonographic approach were notable both in establishing diagnosis and in treatment of pleural effusion,with special regarding of noninvasiveness particularly in the women of pregnancy, of staging in the patient with malignant lymphoma, and of safety in a subsequent thoracentesis under the ultrasonographic guidance

  6. Ultrasonographic Observations of the Pleural Effusion

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Dong Hoo; Park, Sung Soo; Lee, Chung Hee [Hanyang University School of Medicine, Seoul (Korea, Republic of)

    1982-12-15

    Five cases of patients with pleural effusion were evaluated by the grey-scale ultrasonography. Ultrasonography of pleural effusion in each case was represented as fluid accumulation within the pleural cavity with anechoic crescent moon shape or saddle appearance marginated by diaphragm. Ptosis of the liver with demonstrable right diaphragm was assessment in the severe right pleural effusion. it is emphasized that the practical advantages of the ultrasonographic approach were notable both in establishing diagnosis and in treatment of pleural effusion,with special regarding of noninvasiveness particularly in the women of pregnancy, of staging in the patient with malignant lymphoma, and of safety in a subsequent thoracentesis under the ultrasonographic guidance

  7. Prostatic sarcoma after treatment of rectal cancer

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    Hill Andrew G

    2007-07-01

    Full Text Available Abstract Background The relationship between radiation exposure for treatment of cancer and occurrence of a second primary cancer at the irradiated site is well known. This phenomenon is however rare in prostate. Case presentation A 75-year-old farmer was treated for rectal cancer with preoperative 45 Gy of radiotherapy and abdominoperineal resection. Four years later he developed symptoms of bladder outlet obstruction and acute urinary retention. He underwent a transurethral resection of the prostate. Histological examination of the removed prostate tissue and immunohistochemistry revealed it to be a poorly differentiated sarcoma. Conclusion We believe this to be the first reported case of radiation-induced sarcoma following radiotherapy treatment for rectal cancer. Since radiotherapy plays a pivotal role in the contemporary treatment of rectal adenocarcinoma, it is relevant to be aware of the potential long-term carcinogenic complications of radiotherapy of the pelvis.

  8. Evaluation of therapeutic results in Ewing's sarcoma

    International Nuclear Information System (INIS)

    Johnson, R.E.; Pomeroy, T.C.

    1975-01-01

    The philosophy pervading the treatment approach to Ewing's sarcoma was to have therapy encompass all foci of disease, including sites of occult or potential involvement in addition to obvious clinical manifestations. The experience with integrated methods of treatment in 66 consecutive patients at the National Cancer Institute is reviewed. A median survival of 18 months (44 percent 2 year survival rate) for patients with recognizable metastases on admission bears impressive witness to the value of adjuvant therapy in Ewing's sarcoma. Even more encouraging, an uncorrected 5 year survival rate of 53 percent (42 percent continuously free of disease) for patients given ''pyrophylactic'', adjuvant therapy indicates the potential for permanent control of disease in a significant fraction of cases with clinically localized primary tumors. (U.S.)

  9. Deep-seated sarcomas of the penis

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    Alberto A. Antunes

    2005-06-01

    Full Text Available Mesenchymal neoplasias represent 5% of tumors affecting the penis. Due to the rarity of such tumors, there is no agreement concerning the best method for staging and managing these patients. Sarcomas of the penis can be classified as deep-seated if they derive from the structures forming the spongy body and the cavernous bodies. Superficial lesions are usually low-grade and show a small tendency towards distant metastasis. In contrast, deep-seated lesions usually show behavior that is more aggressive and have poorer prognosis. The authors report 3 cases of deep-seated primary sarcomas of the penis and review the literature on this rare and aggressive neoplasia.

  10. Oncological outcomes of patients with Ewing's sarcoma: is there a difference between skeletal and extra-skeletal Ewing's sarcoma?

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    Pradhan, A; Grimer, R J; Spooner, D; Peake, D; Carter, S R; Tillman, R M; Abudu, A; Jeys, L

    2011-04-01

    The aim of this study was to identify whether there was any difference in patient, tumour, treatment or outcome characteristics between patients with skeletal or extra-skeletal Ewing's sarcoma. We identified 300 patients with new primary Ewing's sarcoma diagnosed between 1980 and 2005 from the centres' local database. There were 253 (84%) with skeletal and 47 (16%) with extra-skeletal Ewing's sarcomas. Although patients with skeletal Ewing's were younger (mean age 16.8 years) than those with extra-skeletal Ewing's sarcoma (mean age 27.5 years), there was little difference between the groups in terms of tumour stage or treatment. Nearly all the patients were treated with chemotherapy and most had surgery. There was no difference in the overall survival of patients with skeletal (64%) and extra-skeletal Ewing's sarcoma (61%) (p = 0.85), and this was also the case when both groups were split by whether they had metastases or not. This large series has shown that the oncological outcomes of Ewing's sarcoma are related to tumour characteristics and patient age, and not determined by whether they arise in bone or soft tissue.

  11. Parapneumonic pleural effusion: early versus late thoracoscopy

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    Rodrigo Romualdo Pereira

    2017-07-01

    Full Text Available ABSTRACT Objective: To evaluate the best time to perform thoracoscopy for the treatment of complicated parapneumonic pleural effusion in the fibrinopurulent phase in patients ≤ 14 years of age, regarding the postoperative evolution and occurrence of complications. Methods: This was a retrospective comparative study involving patients with parapneumonic pleural effusion presenting with septations or loculations on chest ultrasound who underwent thoracoscopy between January of 2000 and January of 2013. The patients were divided into two groups: early thoracoscopy (ET, performed by day 5 of hospitalization; and late thoracoscopy (LT, performed after day 5 of hospitalization. Results: We included 60 patients, 30 in each group. The mean age was 3.4 years; 28 patients (46.7% were male; and 47 (78.3% underwent primary thoracoscopy (no previous simple drainage. The two groups were similar regarding gender, age, weight, and type of thoracoscopy (p > 0.05 for all. There was a significant difference between the ET and the LT groups regarding the length of the hospital stay (14.5 days vs. 21.7 days; p < 0.001. There were also significant differences between the groups regarding the duration of fever in days; the total number of days from admission to the initiation of drainage; and the total number of days with the drain in place. Eight patients (13.6% had at least one post-thoracoscopy complication, there being no difference between the groups. There were no deaths. Conclusions: Performing ET by day 5 of hospitalization was associated with shorter hospital stays, shorter duration of drainage, and shorter duration of fever, although not with a higher frequency of complications, requiring ICU admission, or requiring blood transfusion.

  12. Multifocal osteogenic sarcoma in Paget's disease

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    Vuillemin-Bodaghi, V.; Parlier-Cuau, C.; Laredo, J.D.; Cywiner-Golenzer, C.; Quillard, A.; Kaplan, G.

    2000-01-01

    The most serious complication of Paget's disease is sarcomatous degeneration of pagetic bone. Multifocal sarcomatous degeneration occurs mainly in polyostotic Paget's disease. Multifocal Paget's sarcoma is uncommon and can arise in any site. We report two cases of synchronous multifocal sarcomatous degeneration. The two patients were elderly women (aged 77 and 86 years, respectively) who developed sarcomatous lesions concomitantly, in the first case report in left ilium, left tibia, and first lumbar vertebra and in the second case report in the skull, right ilium, and sacrum. Whether these cases are due to the simultaneous development of several primaries or to metastases from a single primary remains unclear. (orig.)

  13. External-beam radiation therapy combined with limb-sparing surgery in elderly patients (>70 years) with primary soft tissue sarcomas of the extremities : A retrospective analysis.

    Science.gov (United States)

    Andrä, Claudia; Klein, Alexander; Dürr, Hans Roland; Rauch, Josefine; Lindner, Lars Hartwin; Knoesel, Thomas; Angele, Martin; Baur-Melnyk, Andrea; Belka, Claus; Roeder, Falk

    2017-08-01

    To report our experience with EBRT combined with limb-sparing surgery in elderly patients (>70 years) with primary extremity soft tissue sarcomas (STS). Retrospectively analyzed were 35 patients (m:f 18:17, median 78 years) who all presented in primary situation without nodal/distant metastases (Charlson score 0/1 in 18 patients; ≥2 in 17 patients). Median tumor size was 10 cm, mainly located in lower limb (83%). Stage at presentation (UICC7th) was Ib:3%, 2a:20%, 2b:20%, and 3:57%. Most lesions were high grade (97%), predominantly leiomyosarcoma (26%) and undifferentiated pleomorphic/malignant fibrous histiocytoma (23%). Limb-sparing surgery was preceded (median 50 Gy) or followed (median 66 Gy) by EBRT. Median follow-up was 37 months (range 1-128 months). Margins were free in 26 patients (74%) and microscopically positive in 9 (26%). Actuarial 3‑ and 5‑year local control rates were 88 and 81% (4 local recurrences). Corresponding rates for distant control, disease-specific survival, and overall survival were 57/52%, 76/60%, and 72/41%. The 30-day mortality was 0%. Severe postoperative complications were scored in 8 patients (23%). Severe acute radiation-related toxicity was observed in 2 patients (6%). Patients with Charlson score ≥2 had a significantly increased risk for severe postoperative complications and acute radiation-related side effects. Severe late toxicities were found in 7 patients (20%), including fractures in 3 (8.6%). Final limb preservation rate was 97%. Combination of EBRT and limb-sparing surgery is feasible in elderly patients with acceptable toxicities and encouraging but slightly inferior outcome compared to younger patients. Comorbidity correlated with postoperative complications and acute toxicities. Late fracture risk seems slightly increased.

  14. Synovial sarcoma of the kidney in a young patient with a review of the literature

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    Mahmoud Abbas

    2014-06-01

    Full Text Available Synovial sarcoma (SS is a soft tissue, generally deep seated neoplasms that occurs generally in the proximity of large joints. We report of a case of a 33-year-old man who was diagnosed with primary SS of the kidney which is an extremely rare tumor that accounts for less than 2% of malignant renal tumors. Contemporary management of renal synovial sarcoma includes surgical resection and ifosfamide-based chemotherapy and they remain the mainstay of therapy of synovial sarcoma, which is often applied, combined as part of an aggressive treatment approach. Fewer than 50 patients have been described in the English literature. Physicians should be aware of the possibility of malignancy in cystic renal masses and raise the suspicion of synovial sarcoma, especially when patients with renal masses are young adults. Along with the case report a literature review on primary synovial sarcomas of the kidney is provided with focus on the renal tumors’ differential diagnosis.

  15. Efficacy of Wnt-1 monoclonal antibody in sarcoma cells

    International Nuclear Information System (INIS)

    Mikami, Iwao; Koizumi, Kiyoshi; Jablons, David M; You, Liang; He, Biao; Xu, Zhidong; Batra, Sonny; Lee, Amie Y; Mazieres, Julien; Reguart, Noemi; Uematsu, Kazutsugu

    2005-01-01

    Sarcomas are one of the most refractory diseases among malignant tumors. More effective therapies based on an increased understanding of the molecular biology of sarcomas are needed as current forms of therapy remain inadequate. Recently, it has been reported that Wnt-1/β-catenin signaling inhibits apoptosis in several cancers. In this study, we investigated the efficacy of a monoclonal anti-Wnt-1 antibody in sarcoma cells. We treated cell lines A-204, SJSA-1, and fresh primary cultures of lung metastasis of sarcoma with a monoclonal anti-Wnt-1 antibody. Wnt-1 siRNA treatment was carried out in A-204. We assessed cell death using Crystal Violet staining. Apoptosis induction was estimated by flow cytometry analysis (Annexin V and PI staining). Cell signaling changes were determined by western blotting analysis. We detected Wnt-1 expression in all tissue samples and cell lines. Significant apoptosis induction was found in monoclonal anti-Wnt-1 antibody treated cells compared to control monoclonal antibody treated cells (p < 0.02). Similarly, we observed increased apoptosis in Wnt-1 siRNA treated cells. Blockade of Wnt-1 signaling in both experiments was confirmed by analyzing intracellular levels of Dishevelled-3 and of cytosolic β-catenin. Furthermore, the monoclonal anti-Wnt-1 antibody also induced cell death in fresh primary cultures of metastatic sarcoma in which Wnt-1 signaling was active. Our results indicate that Wnt-1 blockade by either monoclonal antibody or siRNA induces cell death in sarcoma cells. These data suggest that Wnt-1 may be a novel therapeutic target for the treatment of a subset of sarcoma cells in which Wnt-1/β-catenin signaling is active

  16. Pembrolizumab in advanced soft-tissue sarcoma and bone sarcoma (SARC028): a multicentre, two-cohort, single-arm, open-label, phase 2 trial.

    Science.gov (United States)

    Tawbi, Hussein A; Burgess, Melissa; Bolejack, Vanessa; Van Tine, Brian A; Schuetze, Scott M; Hu, James; D'Angelo, Sandra; Attia, Steven; Riedel, Richard F; Priebat, Dennis A; Movva, Sujana; Davis, Lara E; Okuno, Scott H; Reed, Damon R; Crowley, John; Butterfield, Lisa H; Salazar, Ruth; Rodriguez-Canales, Jaime; Lazar, Alexander J; Wistuba, Ignacio I; Baker, Laurence H; Maki, Robert G; Reinke, Denise; Patel, Shreyaskumar

    2017-11-01

    Patients with advanced sarcomas have a poor prognosis and few treatment options that improve overall survival. Chemotherapy and targeted therapies offer short-lived disease control. We assessed pembrolizumab, an anti-PD-1 antibody, for safety and activity in patients with advanced soft-tissue sarcoma or bone sarcoma. In this two-cohort, single-arm, open-label, phase 2 study, we enrolled patients with soft-tissue sarcoma or bone sarcoma from 12 academic centres in the USA that were members of the Sarcoma Alliance for Research through Collaboration (SARC). Patients with soft-tissue sarcoma had to be aged 18 years or older to enrol; patients with bone sarcoma could enrol if they were aged 12 years or older. Patients had histological evidence of metastatic or surgically unresectable locally advanced sarcoma, had received up to three previous lines of systemic anticancer therapy, had at least one measurable lesion according to the Response Evaluation Criteria In Solid Tumors version 1.1, and had at least one lesion accessible for biopsy. All patients were treated with 200 mg intravenous pembrolizumab every 3 weeks. The primary endpoint was investigator-assessed objective response. Patients who received at least one dose of pembrolizumab were included in the safety analysis and patients who progressed or reached at least one scan assessment were included in the activity analysis. Accrual is ongoing in some disease cohorts. This trial is registered with ClinicalTrials.gov, number NCT02301039. Between March 13, 2015, and Feb 18, 2016, we enrolled 86 patients, 84 of whom received pembrolizumab (42 in each disease cohort) and 80 of whom were evaluable for response (40 in each disease cohort). Median follow-up was 17·8 months (IQR 12·3-19·3). Seven (18%) of 40 patients with soft-tissue sarcoma had an objective response, including four (40%) of ten patients with undifferentiated pleomorphic sarcoma, two (20%) of ten patients with liposarcoma, and one (10%) of ten patients

  17. Disseminated Kaposi sarcoma with epithelioid morphology in an HIV/AIDS patient: A previously unreported variant.

    Science.gov (United States)

    Basra, Pukhraz; Paramo, Juan; Alexis, John

    2018-04-16

    Kaposi sarcoma is an oligoclonal HHV-8-driven vascular proliferation that was first described by a Viennese dermatologist Dr Moritz Kaposi. The disease has been seen in different clinical-epidemiological settings with a wide morphologic spectrum. We report a 52-year-old Caucasian man with HIV/AIDS and Kaposi sarcoma who presented with dyspnea and pleural effusion. He reported numerous tender subcutaneous nodules developing over the past few months on his chest, back and abdomen. An excisional biopsy of one of the nodules was performed. Touch preps revealed malignant cells in clusters. Microscopically, the neoplasm appeared undifferentiated with an epithelioid morphology, and involved the dermis and subcutaneous fat. Despite the medical history, Kaposi sarcoma was not considered foremost in the differential diagnosis. The malignant cells were positive for vimentin and negative for S100 protein, keratin AE1/3, CK7, CK20, napsin A, TTF-1 and synaptophysin. Additional stains revealed positivity for HHV-8, CD31 and D2-40, supporting the diagnosis of Kaposi sarcoma. Kaposi sarcoma has been well described with many variants that may cause diagnostic difficulty. An epithelioid variant has not been reported and consequently, may cause misinterpretation of an otherwise well-known entity that may become life threatening if appropriate treatment is not initiated in a timely manner. © 2018 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  18. Vascular endothelial growth factor in diagnosis of pleural effusion

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    Nasr H. Khalil

    2017-01-01

    Conclusion: VEGF pleural fluid level could differentiate between malignant and non malignant effusion, while could not differentiate between tuberculous and nontuberculous, or between parapneumonic and nonparapneumonic exudative effusions.

  19. CT findings of posterior pararenal effusion

    International Nuclear Information System (INIS)

    Koh, Byung Hee; Cho, On Koo; Kim, Sonn Yong

    1990-01-01

    The posterior pararenal space(PPS) is a potential space between the posterior renal fascia and the transversalis fascia. We reviewed 12 cases of posterior pararenal effusion. The causes of the effusion were retroperitoneal hemorrhage due to trauma(7 cases) or rupture of renal tumor(1 case), pancreatitis(2 cases), urinoma (1 case) and amebic colitis of the cecum(1 case). The CT findings of the effusion were semilunar fluid density in the dependant portion of the PPS(9/12), partial obliteration of the PPS extending to the flank stripe(2/12), and total obliteration of the PPS extending to the anterior abdominal wall(1/12). The effusion in the ipsilateral perirenal space and thickening of the ipsilateral posterior renal fascia were combined in all cases. The effusion in the ipsilateral anterior pararenal space was associated in 11 cases of 12. The effusion in the PPS is not uncommon findings of retroperitoneal hemorrhage or effusion, but usually related with perirenal or anterior pararenal effusion with no clinical significance

  20. Sarcoma risk after radiation exposure

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    Berrington de Gonzalez Amy

    2012-10-01

    Full Text Available Abstract Sarcomas were one of the first solid cancers to be linked to ionizing radiation exposure. We reviewed the current evidence on this relationship, focusing particularly on the studies that had individual estimates of radiation doses. There is clear evidence of an increased risk of both bone and soft tissue sarcomas after high-dose fractionated radiation exposure (10 + Gy in childhood, and the risk increases approximately linearly in dose, at least up to 40 Gy. There are few studies available of sarcoma after radiotherapy in adulthood for cancer, but data from cancer registries and studies of treatment for benign conditions confirm that the risk of sarcoma is also increased in this age-group after fractionated high-dose exposure. New findings from the long-term follow-up of the Japanese atomic bomb survivors suggest, for the first time, that sarcomas can be induced by acute lower-doses of radiation (

  1. Immunophenotypic analysis of the Kaposi sarcoma herpesvirus (KSHV; HHV-8)-infected B cells in HIV+ multicentric Castleman disease (MCD).

    Science.gov (United States)

    Chadburn, A; Hyjek, E M; Tam, W; Liu, Y; Rengifo, T; Cesarman, E; Knowles, D M

    2008-11-01

    Kaposi sarcoma herpesvirus (KSHV) is aetiologically related to Kaposi sarcoma, classical and extracavitary primary effusion lymphoma (PEL; EC-PEL) and multicentric Castleman disease (MCD), entities preferentially occurring in HIV-infected individuals. Characterization of HIV-associated PELs/EC-PELs suggests that the KSHV-infected malignant cells originate from a pre-terminal stage of B-cell differentiation. However, only limited phenotypic studies have been performed on HIV+ MCD, including for PR domain containing 1 with zinc finger domain/B lymphocyte-induced maturation protein 1 (PRDM1/BLIMP1), a key regulator of terminal B-cell differentiation. The aim was to characterize KSHV-infected cells in 17 cases of HIV+ MCD. Double immunohistochemistry and immunohistochemistry-in situ hybridization were used to characterize the KSHV-infected cells in MCD; the results were compared with the phenotypic profiles of 39 PELs/EC-PELs and seven PEL cell lines. Whereas the immunophenotype of KSHV-infected cells in MCD and malignant KSHV+ PEL cells was similar (PAX5, Bcl-6-; PRDM1/BLIMP1, IRF4/MUM1+; Ki67+), the MCD KSHV-infected cells differed, as they expressed OCT2, cytoplasmic lambda immunoglobulin; variably expressed CD27; lacked CD138; and were Epstein-Barr virus negative. Although both PEL and MCD originate from KSHV-infected pre-terminally differentiated B cells, these findings, with previously reported genetic studies, indicate HIV+ MCD may arise from extrafollicular B cells, whereas PELs may originate from cells that have traversed the germinal centre.

  2. Multiple skeletal muscle metastases revealing a cardiac intimal sarcoma

    Energy Technology Data Exchange (ETDEWEB)

    Crombe, Amandine [Institut Bergonie, Department of Radiology, Bordeaux (France); Lintingre, Pierre-Francois; Dallaudiere, Benjamin [Clinique du Sport de Bordeaux-Merignac, Department of Musculoskeletal Radiology, Merignac (France); Le Loarer, Francois [Institut Bergonie, Department of Pathology, Bordeaux (France); Lachatre, Denis [Dupuytren University Hospital, Department of Radiology, Limoges (France)

    2018-01-15

    We report the case of a 59-year-old female with progressive bilateral painful swelling of the thighs. MRI revealed multiple intramuscular necrotic masses with similar morphologic patterns. Whole-body CT and 18-FDG PET-CT scans demonstrated additional hypermetabolic muscular masses and a lobulated lesion within the left atrial cavity. As biopsy of a muscular mass was compatible with a poorly differentiated sarcoma with MDM2 oncogene amplification, two diagnoses were discussed: a dedifferentiated liposarcoma with muscle and heart metastases or a primary cardiac sarcoma, mainly a cardiac intimal sarcoma, with muscular metastases, which was finally confirmed by array-comparative genomic hybridization (aCGH) in a sarcoma reference center. This case emphasizes the potential for intimal sarcoma to disseminate in skeletal muscle prior to any other organ and the need for a genomic approach in addition to classical radiopathologic analyses to distinguish primary from secondary locations facing simultaneous tumors of the heart and skeletal muscles with MDM2 amplification. (orig.)

  3. Clinical experience with intravenous radiosensitizers in unresectable sarcomas

    International Nuclear Information System (INIS)

    Kinsella, T.J.; Glatstein, E.

    1987-01-01

    Traditionally, adult bone and soft tissue sarcomas have been considered to be ''radioresistant.'' Because of this philosophy, patients who present with locally advanced, unresectable sarcomas often are treated in a palliative fashion, usually with low-dose radiotherapy. Over the last 6 years, 29 patients with unresectable primary or metastatic sarcomas were treated using a combination of intravenous chemical radiosensitizers and high-dose irradiation. Twenty-two of 29 patients achieved clinical local control, with six patients having a complete clinical response. The time to tumor response is often several months or longer, which is in contrast to other tumor histologies (carcinomas, lymphomas), where tumor response usually occurs over several weeks. Several large tumors have shown only a minimal tumor response, yet were found to be sterilized in posttreatment biopsy or autopsy examination. Of 15 patients with primary sarcomas without metastases, 11 patients (73%) remain free of local tumor progression from 12 to 83 months. Adult high-grade sarcomas can be controlled with high-dose radiotherapy and intravenous radiosensitizers, although the precise role of these agents is unclear

  4. Diagnosis and treatment of Ewing's sarcoma

    International Nuclear Information System (INIS)

    Iwamoto, Yukihide

    2007-01-01

    Ewing's sarcoma is a small round-cell tumor typically arising in the bones, rarely in soft tissues, of children and adolescents. Ewing's sarcoma has retained the most unfavorable prognosis of all primary musculoskeletal tumors. Prior to the use of multi-drug chemotherapy, long-term survival was less than 10%. The development of multi-disciplinary therapy with chemotherapy, irradiation, and surgery has increased current long-term survival rates in most clinical centers to greater than 50%. In addition, the preferred method of tumor resection has changed; limb salvage has nearly replaced amputation of the affected limb. Limb salvage procedures can be performed in place of amputation without compromising patient survival rates. Recent studies have revealed that the pathognomonic translocations involving the EWS gene on chromosome 22 and an ETS-type gene, which is most commonly the Fli1 gene on chromosome 11, are implicated in more than 95% of Ewing's sarcomas, primitive neuroectodermal tumors and Askin's tumors. Therefore, these lesions have become regarded as a single entity, dubbed the Ewing's family of tumors. Reverse transcription polymerase chain reaction (RT-PCR) to detect EWS-ETS gene arrangements is widely used to confirm the diagnosis of Ewing's family of tumors. Experimental results suggest that inhibition of the signaling pathway downstream of the EWS-ETS gene may lead to the development of molecularly targeted therapy in the future. (author)

  5. Role of Bronchoscopy in Malignant Pleural effusion

    Directory of Open Access Journals (Sweden)

    Gomathi. R. G.

    2016-04-01

    Full Text Available The aim of this study was to assess the role of Bronchoscopy in plural effusion in cancer condition. Pleural effusion is one of the commonest problems with which patients present to the hospital. Around a million patients worldwide develop pleural effusion each year. This is a Prospective and Observational Study. All patients diagnosed to have pleural effusion by xray, clinical examination and ultrasound examination of pleura if needed will undergo informed. All 32 patients underwent bronchoscopy procedure, 30 patients had endobronchial mass and biopsy was done which was positive for malignancy and 2 patients had bronchial wash cytology positive for malignancy We conclude that bronchoscopy has a definite role in the etiological diagnosis of pleural effusion.

  6. Diagnostic Study of Tumor Characteristics in Patients With Ewing's Sarcoma

    Science.gov (United States)

    2013-06-20

    Localized Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor

  7. Carbon Ion Radiotherapy for Unresectable Retroperitoneal Sarcomas

    International Nuclear Information System (INIS)

    Serizawa, Itsuko; Kagei, Kenji; Kamada, Tadashi; Imai, Reiko; Sugahara, Shinji; Okada, Tohru; Tsuji, Hiroshi; Ito, Hisao; Tsujii, Hirohiko

    2009-01-01

    Purpose: To evaluate the applicability of carbon ion radiotherapy (CIRT) for unresectable retroperitoneal sarcomas with regard to normal tissue morbidity and local tumor control. Methods and Materials: From May 1997 to February 2006, 24 patients (17 male and 7 female) with unresectable retroperitoneal sarcoma received CIRT. Age ranged from 16 to 77 years (median, 48.6 years). Of the patients, 16 had primary disease and 8 recurrent disease. Histologic diagnoses were as follows: malignant fibrous histiocytoma in 6, liposarcoma in 3, malignant peripheral nerve sheath tumor in 3, Ewing/primitive neuroectodermal tumor (PNET) in 2, and miscellaneous in 10 patients. The histologic grades were as follows: Grade 3 in 15, Grade 2-3 in 2, Grade 2 in 3, and unknown in 4. Clinical target volumes ranged between 57 cm 3 and 1,194 cm 3 (median 525 cm 3 ). The delivered carbon ion dose ranged from 52.8 to 73.6 GyE in 16 fixed fractions over 4 weeks. Results: The median follow-up was 36 months (range, 6-143 months). The overall survival rates at 2 and 5 years were 75% and 50%, respectively. The local control rates at 2 and 5 years were 77% and 69%. No complications of the gastrointestinal tract were encountered. No other toxicity greater than Grade 2 was observed. Conclusions: Use of CIRT is suggested to be effective and safe for retroperitoneal sarcomas. The results obtained with CIRT were a good overall survival rate and local control, notwithstanding the fact that most patients were not eligible for surgical resection and had high-grade sarcomas.

  8. Pleural effusion: diagnosis, treatment, and management

    Science.gov (United States)

    Karkhanis, Vinaya S; Joshi, Jyotsna M

    2012-01-01

    A pleural effusion is an excessive accumulation of fluid in the pleural space. It can pose a diagnostic dilemma to the treating physician because it may be related to disorders of the lung or pleura, or to a systemic disorder. Patients most commonly present with dyspnea, initially on exertion, predominantly dry cough, and pleuritic chest pain. To treat pleural effusion appropriately, it is important to determine its etiology. However, the etiology of pleural effusion remains unclear in nearly 20% of cases. Thoracocentesis should be performed for new and unexplained pleural effusions. Laboratory testing helps to distinguish pleural fluid transudate from an exudate. The diagnostic evaluation of pleural effusion includes chemical and microbiological studies, as well as cytological analysis, which can provide further information about the etiology of the disease process. Immunohistochemistry provides increased diagnostic accuracy. Transudative effusions are usually managed by treating the underlying medical disorder. However, a large, refractory pleural effusion, whether a transudate or exudate, must be drained to provide symptomatic relief. Management of exudative effusion depends on the underlying etiology of the effusion. Malignant effusions are usually drained to palliate symptoms and may require pleurodesis to prevent recurrence. Pleural biopsy is recommended for evaluation and exclusion of various etiologies, such as tuberculosis or malignant disease. Percutaneous closed pleural biopsy is easiest to perform, the least expensive, with minimal complications, and should be used routinely. Empyemas need to be treated with appropriate antibiotics and intercostal drainage. Surgery may be needed in selected cases where drainage procedure fails to produce improvement or to restore lung function and for closure of bronchopleural fistula. PMID:27147861

  9. Diagnosis and management of pericardial effusion

    Science.gov (United States)

    Sagristà-Sauleda, Jaume; Mercé, Axel Sarrias; Soler-Soler, Jordi

    2011-01-01

    Pericardial effusion is a common finding in everyday clinical practice. The first challenge to the clinician is to try to establish an etiologic diagnosis. Sometimes, the pericardial effusion can be easily related to a known underlying disease, such as acute myocardial infarction, cardiac surgery, end-stage renal disease or widespread metastatic neoplasm. When no obvious cause is apparent, some clinical findings can be useful to establish a diagnosis of probability. The presence of acute inflammatory signs (chest pain, fever, pericardial friction rub) is predictive for acute idiopathic pericarditis irrespective of the size of the effusion or the presence or absence of tamponade. Severe effusion with absence of inflammatory signs and absence of tamponade is predictive for chronic idiopathic pericardial effusion, and tamponade without inflammatory signs for neoplastic pericardial effusion. Epidemiologic considerations are very important, as in developed countries acute idiopathic pericarditis and idiopathic pericardial effusion are the most common etiologies, but in some underdeveloped geographic areas tuberculous pericarditis is the leading cause of pericardial effusion. The second point is the evaluation of the hemodynamic compromise caused by pericardial fluid. Cardiac tamponade is not an “all or none” phenomenon, but a syndrome with a continuum of severity ranging from an asymptomatic elevation of intrapericardial pressure detectable only through hemodynamic methods to a clinical tamponade recognized by the presence of dyspnea, tachycardia, jugular venous distension, pulsus paradoxus and in the more severe cases arterial hypotension and shock. In the middle, echocardiographic tamponade is recognized by the presence of cardiac chamber collapses and characteristic alterations in respiratory variations of mitral and tricuspid flow. Medical treatment of pericardial effusion is mainly dictated by the presence of inflammatory signs and by the underlying disease if

  10. The result analysis of 18F-FDG imaging in suspected malignant pleural effusion or atelectasis on CT scanning

    International Nuclear Information System (INIS)

    Wang Huoqiang; Wu Jiyong; Pan Huizhong; Liu Jinjun; Zhao Xianguo

    2004-01-01

    Objective: To determine the ability of 18 F-fluorodeoxyglucose (FDG) dual-head tomography with coincidence (DHTC) imaging in detecting lung cancer in patients with suspected malignant pleural effusion or malignant atelectasis on CT scanning and to differentiate benign and malignant pleural effusions in patients with lung cancer. Methods: One hundred and ten patients with suspected malignant pleural effusion (n=84) or atelectasis (n=26) but without primary lesions in the lungs on CT scanning underwent 18 F-FDG DHTC. Results: Thirty-eight of 110 patients were proven with lung cancer. Among the 38 lung cancer patients, 30 of them had pleural effusion and 8 of them had atelectasis. Seventy-two of 110 patients were proven with benign lung diseases. The sensitivity, specificity and accuracy of 18 F-FDG DHTC for detecting lung cancer in patients with suspected malignant pleural effusion or atelectasis were 97%, 78% and 85%, respectively. In 30 patients with lung cancer plus pleural effusion, 18 F-FDG DHTC correctly detected the presence of malignant pleural effusion and malignant pleural metastatic involvement in 18 of 21 patients and excluded malignant pleural effusion or pleural metastatic involvement in 8 of 9 patients (sensitivity, specificity and accuracy of 86%, 8/9 and 87%, respectively). Conclusion: 18 F-FDG DHTC imaging is a highly accurate and reliable noninvasive test for detecting primary malignant lesions in lung in patients with pleural effusion or atelectasis findings on CT scanning and is useful to differentiate malignant from benign pleural effusion in patients with lung cancer. (authors)

  11. Alveolar Soft Part Sarcoma.

    Science.gov (United States)

    Jaber, Omar I; Kirby, Patricia A

    2015-11-01

    Alveolar soft part sarcoma is a rare neoplasm usually arising in the soft tissues of the lower limbs in adults and in the head and neck region in children. It presents primarily as a slowly growing mass or as metastatic disease. It is characterized by a specific chromosomal alteration, der(17)t(X:17)(p11:q25), resulting in fusion of the transcription factor E3 (TFE3) with alveolar soft part sarcoma critical region 1 (ASPSCR1) at 17q25. This translocation is diagnostically useful because the tumor nuclei are positive for TFE3 by immunohistochemistry. Real-time polymerase chain reaction to detect the ASPSCR1-TFE3 fusion transcript on paraffin-embedded tissue blocks has been shown to be more sensitive and specific than detection of TFE3 by immunohistochemical stain. Cathepsin K is a relatively recent immunohistochemical stain that can aid in the diagnosis. The recent discovery of the role of the ASPSCR1-TFE3 fusion protein in the MET proto-oncogene signaling pathway promoting angiogenesis and cell proliferation offers a promising targeted molecular therapy.

  12. Study on osteogenic sarcoma

    International Nuclear Information System (INIS)

    Park, Eung Chun; Kim, Young Il; Choi, Won Jae; Kim, Young Jin

    1993-01-01

    The author observed a case of osteogenic sarcoma in a 11-year-old female with complaint of painful swelling on face in right side. The observed results were as follows: 1. Large hematoma was observed, and patient complained painfull swelling on c/c site. 2. Predisposing factor of osteogenic sarcoma was not clear, but patient had history of extraction before patient visiting infirmary of our dental collage. 3. Serologic findings were not specific, and serum aldaline level was normal. 4. Radiographic findings were as follows: a. Diffuse faint radiopacit in the lesion. b. Bony destruction and increased radiopacity in right antrum. c. Displacement of multiple teeth on involved area(i. e no 12, 15, 55, 16, 17, 18) d. Increased periodontal space in single tooth(no 13) e. Destruction of bony crypt on involved teeth(no 13, 14, 15, 17, 18) f. Loss of lamina dura of three teeth in involved area(no 11, 12, 16) 5. Computed tomographic findings were as follows: a. Large calcific and heterogenous component mass in the Rt. maxillary sinus, and this mass extending to Rt. maxilla, alveolar bone, ethmoid sinus. b. Soft tissue bulging in to Rt. side nasal cavity and oral cavity. c. Bone destruction of maxillary sinus wall and Rt. alveolar bone.

  13. Study on osteogenic sarcoma

    Energy Technology Data Exchange (ETDEWEB)

    Park, Eung Chun; Kim, Young Il; Choi, Won Jae; Kim, Young Jin [Dept. of Dentomaxillofacial Radiology, College of Dentistry, Chosun University, Kwangju (Korea, Republic of)

    1993-02-15

    The author observed a case of osteogenic sarcoma in a 11-year-old female with complaint of painful swelling on face in right side. The observed results were as follows: 1. Large hematoma was observed, and patient complained painfull swelling on c/c site. 2. Predisposing factor of osteogenic sarcoma was not clear, but patient had history of extraction before patient visiting infirmary of our dental collage. 3. Serologic findings were not specific, and serum aldaline level was normal. 4. Radiographic findings were as follows: a. Diffuse faint radiopacit in the lesion. b. Bony destruction and increased radiopacity in right antrum. c. Displacement of multiple teeth on involved area (i. e no 12, 15, 55, 16, 17, 18) d. Increased periodontal space in single tooth (no 13) e. Destruction of bony crypt on involved teeth (no 13, 14, 15, 17, 18) f. Loss of lamina dura of three teeth in involved area (no 11, 12, 16) 5. Computed tomographic findings were as follows: a. Large calcific and heterogenous component mass in the Rt. maxillary sinus, and this mass extending to Rt. maxilla, alveolar bone, ethmoid sinus. b. Soft tissue bulging in to Rt. side nasal cavity and oral cavity. c. Bone destruction of maxillary sinus wall and Rt. alveolar bone.

  14. Mast cells mediate malignant pleural effusion formation.

    Science.gov (United States)

    Giannou, Anastasios D; Marazioti, Antonia; Spella, Magda; Kanellakis, Nikolaos I; Apostolopoulou, Hara; Psallidas, Ioannis; Prijovich, Zeljko M; Vreka, Malamati; Zazara, Dimitra E; Lilis, Ioannis; Papaleonidopoulos, Vassilios; Kairi, Chrysoula A; Patmanidi, Alexandra L; Giopanou, Ioanna; Spiropoulou, Nikolitsa; Harokopos, Vaggelis; Aidinis, Vassilis; Spyratos, Dionisios; Teliousi, Stamatia; Papadaki, Helen; Taraviras, Stavros; Snyder, Linda A; Eickelberg, Oliver; Kardamakis, Dimitrios; Iwakura, Yoichiro; Feyerabend, Thorsten B; Rodewald, Hans-Reimer; Kalomenidis, Ioannis; Blackwell, Timothy S; Agalioti, Theodora; Stathopoulos, Georgios T

    2015-06-01

    Mast cells (MCs) have been identified in various tumors; however, the role of these cells in tumorigenesis remains controversial. Here, we quantified MCs in human and murine malignant pleural effusions (MPEs) and evaluated the fate and function of these cells in MPE development. Evaluation of murine MPE-competent lung and colon adenocarcinomas revealed that these tumors actively attract and subsequently degranulate MCs in the pleural space by elaborating CCL2 and osteopontin. MCs were required for effusion development, as MPEs did not form in mice lacking MCs, and pleural infusion of MCs with MPE-incompetent cells promoted MPE formation. Once homed to the pleural space, MCs released tryptase AB1 and IL-1β, which in turn induced pleural vasculature leakiness and triggered NF-κB activation in pleural tumor cells, thereby fostering pleural fluid accumulation and tumor growth. Evaluation of human effusions revealed that MCs are elevated in MPEs compared with benign effusions. Moreover, MC abundance correlated with MPE formation in a human cancer cell-induced effusion model. Treatment of mice with the c-KIT inhibitor imatinib mesylate limited effusion precipitation by mouse and human adenocarcinoma cells. Together, the results of this study indicate that MCs are required for MPE formation and suggest that MC-dependent effusion formation is therapeutically addressable.

  15. Cancer incidence after retinoblastoma - Radiation dose and sarcoma risk

    NARCIS (Netherlands)

    Wong, FL; Boice, JD; Abramson, DH; Tarone, RE; Kleinerman, RA; Stovall, M; Goldman, MB; Seddon, JM; Tarbell, N; Fraumeni, JF; Li, FP

    1997-01-01

    Context.-There is a substantial risk of a second cancer for persons with hereditary retinoblastoma, which is enhanced by radiotherapy. Objective.-To examine long-term risk of new primary cancers in survivors of childhood retinoblastoma and quantify the role of radiotherapy in sarcoma development.

  16. Human immunodeficiency virus negative Kaposi sarcoma and lymphoproliferative disorders

    NARCIS (Netherlands)

    Fossati, S; Boneschi, [No Value; Ferrucci, S; Brambilla, L

    1999-01-01

    BACKGROUND. The concomitant occurrence of more than one primary neoplasm in the same individual has led researchers to seek possible common etiopathogenetic factors. Kaposi sarcoma (KS) is a multicentric neoplasm of vascular origin and perhaps viral etiology. Four forms of KS are known: classic or

  17. Testicular myeloid sarcoma: case report.

    Science.gov (United States)

    Zago, Luzia Beatriz Ribeiro; Ladeia, Antônio Alexandre Lisbôa; Etchebehere, Renata Margarida; de Oliveira, Leonardo Rodrigues

    2013-01-01

    Myeloid sarcomas are extramedullary solid tumors composed of immature granulocytic precursor cells. In association with acute myeloid leukemia and other myeloproliferative disorders, they may arise concurrently with compromised bone marrow related to acute myeloid leukemia, as a relapsed presentation, or occur as the first manifestation. The testicles are considered to be an uncommon site for myeloid sarcomas. No therapeutic strategy has been defined as best but may include chemotherapy, radiotherapy and/or hematopoietic stem cell transplantation. This study reports the evolution of a patient with testicular myeloid sarcoma as the first manifestation of acute myeloid leukemia. The patient initially refused medical treatment and died five months after the clinical condition started.

  18. The microbiome of otitis media with effusion.

    Science.gov (United States)

    Chan, Chun Ling; Wabnitz, David; Bardy, Jake Jervis; Bassiouni, Ahmed; Wormald, Peter-John; Vreugde, Sarah; Psaltis, Alkis James

    2016-12-01

    The adenoid pad has been considered a reservoir for bacteria in the pathogenesis of otitis media with effusion. This study aimed to characterize the middle ear microbiota in children with otitis media with effusion and establish whether a correlation exists between the middle ear and adenoid microbiota. Prospective, controlled study. Middle ear aspirates adenoid pad swabs were collected from 23 children undergoing ventilation tube insertion. Adenoid swabs from patients without ear disease were controls. Samples were analyzed using 16S rRNA sequencing on the Illumina MiSeq platform. Thirty-five middle ear samples were collected. The middle ear effusion microbiota was dominated by Alloiococcus otitidis (23% mean relative abundance), Haemophilus (22%), Moraxella (5%), and Streptococcus (5%). Alloiococcus shared an inverse correlation with Haemophilus (P = .049) and was found in greater relative abundance in unilateral effusion (P = .004). The microbiota of bilateral effusions from the same patient were similar (P effusion microbiota were found to be dissimilar to that of the adenoid (P = .01), whereas the adenoid microbiota of otitis media with effusion and control patients were similar (P > .05) (permutational multivariate analysis of the variance). Dissimilarities between the local microbiota of the adenoid and the middle ear question the theory that the adenoid pad is a significant reservoir to the middle ear in children with otitis media with effusion. A otitidis had the greatest cumulative relative abundance, particularly in unilateral effusions, and shares an inverse correlation with the relative abundance of Haemophilus. NA Laryngoscope, 126:2844-2851, 2016. © 2016 The American Laryngological, Rhinological and Otological Society, Inc.

  19. Intracardiac Low-grade Sarcoma Following Treatment for Ewing Sarcoma.

    Science.gov (United States)

    Ortiz, Michael V; Magnan, Heather; Slotkin, Emily K; Ambati, Srikanth R; Chou, Alexander J; Wexler, Leonard H; Meyers, Paul A; Walsh, Michael F; Heaton, Todd; Girardi, Leonard N; Wolden, Suzanne L; Price, Anita P; Kennedy, Jennifer A; Zehir, Ahmet; Hameed, Meera; Berger, Michael F; Kentsis, Alex; Shukla, Neerav

    2017-11-01

    A 16-year-old male was diagnosed with Ewing sarcoma of the ribcage with pulmonary metastases. Six months after completion of scheduled therapy, he was found to have a new intracardiac mass, presumed recurrent Ewing sarcoma. EWSR1 fusion was not detected by droplet digital polymerase chain reaction from blood plasma. After no improvement with salvage chemotherapy, he underwent surgical resection that identified a low-grade spindle cell sarcoma. Despite the near-synchronous presentation of 2 unrelated sarcomas, extensive genomic analyses did not reveal any unifying somatic or germline mutations nor any apparent cancer predisposition. This case also highlights the potential role of utilizing plasma cell-free DNA for diagnosing tumors in locations where biopsy confers high morbidity.

  20. Knudsen effusion mass spectrometry. Chapter 20

    International Nuclear Information System (INIS)

    Sai Baba, M.

    1997-01-01

    The Knudsen effusion mass spectrometric method for the determination of vapour pressures and thermodynamic properties is described. The aim of the article is to give a general introduction to the method rather than to give a critical review of the technique. The latest developments in this area of research are reviewed by the peers in the field during the triennial international mass spectrometric conferences. The Knudsen effusion mass spectrometric method is being applied for thermodynamic measurements. In recent times, laser vaporisation mass spectrometric methods have emerged as a source of determination of vapour pressures at very high temperatures and beyond the pressure regime far exceeding Knudsen effusion range

  1. Effusion plate using additive manufacturing methods

    Science.gov (United States)

    Johnson, Thomas Edward; Keener, Christopher Paul; Ostebee, Heath Michael; Wegerif, Daniel Gerritt

    2016-04-12

    Additive manufacturing techniques may be utilized to construct effusion plates. Such additive manufacturing techniques may include defining a configuration for an effusion plate having one or more internal cooling channels. The manufacturing techniques may further include depositing a powder into a chamber, applying an energy source to the deposited powder, and consolidating the powder into a cross-sectional shape corresponding to the defined configuration. Such methods may be implemented to construct an effusion plate having one or more channels with a curved cross-sectional geometry.

  2. Pericardial effusion in pulmonary arterial hypertension

    Science.gov (United States)

    2013-01-01

    Abstract Pulmonary arterial hypertension (PAH) is a serious condition that can lead to right heart failure and death. Pericardial effusion in PAH is associated with significant morbidity and mortality, and its pathogenesis is complex and poorly understood. There are few data on the prevalence of pericardial effusion in PAH, and more importantly, the management of pericardial effusion is controversial. Current literature abounds with case reports, case series, and retrospective studies that have limited value for assessing this association. Hence, we summarize the available evidence on this ominous association and identify areas for future research. PMID:24618534

  3. Radiation therapy in retroperitoneal sarcoma management.

    Science.gov (United States)

    Haas, Rick L; Baldini, Elizabeth H; Chung, Peter W; van Coevorden, Frits; DeLaney, Thomas F

    2018-01-01

    Surgery is potentially curative for primary non-metastatic retroperitoneal soft tissue sarcomas (RPS), although patients remain at risk for local recurrence. To reduce this risk, the addition of radiotherapy to radical surgery may be considered. Nevertheless, level I evidence to support radiotherapy is currently lacking. The results from the EORTC-STBSG 62092-22092 studying this question are awaited. This manuscript addresses issues to consider when radiation-oncologists engage in a multidisciplinary treatment approach for RPS patients, including radiotherapy. © 2017 Wiley Periodicals, Inc.

  4. Cystic synovial sarcomas: imaging features with clinical and histopathologic correlation

    Energy Technology Data Exchange (ETDEWEB)

    Nakanishi, Hirofumi; Araki, Nobuhito [Department of Orthopedic Surgery, Osaka Medical Center for Cancer and Cardiovascular Diseases, 1-3-3, Nakamichi, Higashinari-Ku, 537-8511, Osaka (Japan); Sawai, Yuka [Department of Radiology, Osaka Medical Center for Cancer and Cardiovascular Diseases, Osaka (Japan); Kudawara, Ikuo [Department of Orthopedic Surgery, Osaka National Hospital, Osaka (Japan); Mano, Masayuki; Ishiguro, Shingo [Department of Pathology, Osaka Medical Center for Cancer and Cardiovascular Diseases, Osaka (Japan); Ueda, Takafumi; Yoshikawa, Hideki [Department of Orthopedic Surgery, Osaka University Graduate School of Medicine, Suita, Osaka (Japan)

    2003-12-01

    To characterize the radiological and clinicopathologic features of cystic synovial sarcoma. Seven patients with primary cystic synovial sarcoma were evaluated. Computed tomography (CT) and magnetic resonance (MR) imaging were undertaken at the first presentation. The diagnosis of synovial sarcoma was made on the basis of histological examinations followed by molecular analysis. Radiological and clinicopathologic findings were reviewed. CT showed well-defined soft tissue mass without cortical bone erosion and invasion. Calcification was seen at the periphery of the mass in three cases. T2-weighted MR images showed multilocular inhomogeneous intensity mass in all cases, five of which showed fluid-fluid levels. On gross appearance, old and/or fresh hematomas were detected in six cases. In the one remaining case, microscopic hemorrhage in the cystic lumen was proven. Four cases had poorly differentiated areas. In five cases prominent hemangiopericytomatous vasculature was observed. Histologic grade was intermediate in one tumor and high in six. One case had a history of misdiagnosis for tarsal tunnel syndrome, one for lymphadenopathy, two for sciatica and two for hematoma. All cystic synovial sarcomas demonstrated multilocularity with well-circumscribed walls and internal septae. Synovial sarcoma should be taken into consideration in patients with deeply situated multicystic mass with triple signal intensity on T2-weighted MR imaging. (orig.)

  5. Cystic synovial sarcomas: imaging features with clinical and histopathologic correlation

    International Nuclear Information System (INIS)

    Nakanishi, Hirofumi; Araki, Nobuhito; Sawai, Yuka; Kudawara, Ikuo; Mano, Masayuki; Ishiguro, Shingo; Ueda, Takafumi; Yoshikawa, Hideki

    2003-01-01

    To characterize the radiological and clinicopathologic features of cystic synovial sarcoma. Seven patients with primary cystic synovial sarcoma were evaluated. Computed tomography (CT) and magnetic resonance (MR) imaging were undertaken at the first presentation. The diagnosis of synovial sarcoma was made on the basis of histological examinations followed by molecular analysis. Radiological and clinicopathologic findings were reviewed. CT showed well-defined soft tissue mass without cortical bone erosion and invasion. Calcification was seen at the periphery of the mass in three cases. T2-weighted MR images showed multilocular inhomogeneous intensity mass in all cases, five of which showed fluid-fluid levels. On gross appearance, old and/or fresh hematomas were detected in six cases. In the one remaining case, microscopic hemorrhage in the cystic lumen was proven. Four cases had poorly differentiated areas. In five cases prominent hemangiopericytomatous vasculature was observed. Histologic grade was intermediate in one tumor and high in six. One case had a history of misdiagnosis for tarsal tunnel syndrome, one for lymphadenopathy, two for sciatica and two for hematoma. All cystic synovial sarcomas demonstrated multilocularity with well-circumscribed walls and internal septae. Synovial sarcoma should be taken into consideration in patients with deeply situated multicystic mass with triple signal intensity on T2-weighted MR imaging. (orig.)

  6. [Ewing sarcoma located in the mandible: A case report].

    Science.gov (United States)

    Hernandez, M; Droz, D; Mansuy, L; Simon, E; Chastagner, P

    2015-06-01

    Ewing sarcoma is the second most common primary malignant bone cancer in children and adolescents. Clinical presentation is usually dominated by local pain and a palpable mass. These symptoms justify imaging investigations: the first one, when an osseous lesion is suspected, is usually a conventional radiograph in two planes. Ewing sarcoma appears as a poorly defined osteolytic lesion that may frequently be associated with cortical erosion or laminar periosteal response ("onion skin"). However, this aspect is not pathognomonic and the definitive diagnosis is made by biopsy. Absence of pain or an unusual localization can lead to misdiagnosis. We report the case of a 7-year-old boy with Ewing sarcoma located in the mandible with a clinical picture including progressive mandibular swelling but no pain. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

  7. Clinical Importance of Angiogenic Cytokines, Fibrinolytic Activity and Effusion Size in Parapneumonic Effusions

    Science.gov (United States)

    Chung, Chi-Li; Hsiao, Shih-Hsin; Hsiao, George; Sheu, Joen-Rong; Chen, Wei-Lin; Chang, Shi-Chuan

    2013-01-01

    Objective To investigate the relationship among angiogenic cytokines, fibrinolytic activity and effusion size in parapneumonic effusion (PPE) and their clinical importance. Methods From January 2008 through December 2010, 26 uncomplicated (UPPE) and 38 complicated (CPPE) PPE were studied. Based on chest ultrasonography, there were non-loculated in 30, uni-loculated in 12, and multi-loculated effusions in 22 patients. The effusion size radiological scores, and effusion vascular endothelial growth factor (VEGF), interleukin (IL)-8, plasminogen activator inhibitor type-1 (PAI-1) and tissue type plasminogen activator (tPA) were measured on admission. Treatment outcome and pleural fibrosis, defined as radiological residual pleural thickening (RPT), were assessed at 6-month follow-up. Results The effusion size and effusion VEGF, IL-8 and PAI-1/tPA ratio were significantly higher in CPPE than in UPPE, and significantly higher in multi-loculated PPE than in non-locualted and uni-loculated PPE, respectively. VEGF (cutoff value 1975 pg/ml) and IL-8 (cutoff value 1937 pg/ml) seemed best to discriminate between UPPE and CPPE. VEGF, IL-8 and effusion size correlated positively with PAI-1/tPA ratio in both UPPE and CPPE. Moreover, the level of VEGF, but not IL-8, correlated positively with effusion size in all patients (r = 0.79, peffusion were prone to have medical treatment failure (n = 10; VEGF, odds ratio 1.01, p = 0.02; effusion size, odds ratio 1.26, p = 0.01). Additionally, ten patients with RPT had larger effusion size and higher levels of VEGF and PAI-1/tPA ratio than did those without. Conclusions In PPE, VEGF and IL-8 levels are valuable to identify CPPE, and higher VEGF level or larger effusion is associated with decreased fibrinolytic activity, development of pleural loculation and fibrosis, and higher risk of medical treatment failure. PMID:23308155

  8. Drugs Approved for Kaposi Sarcoma

    Science.gov (United States)

    This page lists cancer drugs approved by the Food and Drug Administration (FDA) for Kaposi sarcoma. The list includes generic names and brand names. The drug names link to NCI's Cancer Drug Information summaries.

  9. Imaging of soft tissue sarcomas

    International Nuclear Information System (INIS)

    Vanel, D.; Le Treut, A.

    1988-01-01

    Modern imaging of soft tissue sarcomas now includes ultrasounds, CT and MRI. These new techniques allow a better evaluation of initial local extension, of the response to treatment and are able to detect local recurrences early [fr

  10. Ewing's sarcoma of the patella.

    Science.gov (United States)

    Gorelik, Natalia; Dickson, Brendan C; Wunder, Jay S; Bleakney, Robert

    2013-05-01

    Ewing's sarcoma is a relatively rare malignancy, occurring mainly between 4 and 25 years of age. It usually arises from the pelvis, followed by the femur, tibia, and remainder of both the long bones of the extremities and flat bones of the axial skeleton. To the best of our knowledge, Ewing's sarcoma of the patella has never been reported previously. Patellar tumors occur infrequently and represent an uncommon etiology of anterior knee pain. We describe the rare case of a 41-year-old man who presented with a 3-4 month history of escalating right anterior knee pain and swelling. Imaging demonstrated an aggressive patellar tumor with an adjacent soft tissue mass. The diagnosis of Ewing's sarcoma was confirmed by pathology. Physicians should be aware of atypical locations for Ewing's sarcoma and, conversely, of rare tumors arising in the patella and accounting for anterior knee pain. Early recognition of such malignancies allows prompt initiation of treatment, hence improving prognosis.

  11. Surgical versus non-surgical approach in primary desmoid-type fibromatosis patients: A nationwide prospective cohort from the French Sarcoma Group.

    Science.gov (United States)

    Penel, Nicolas; Le Cesne, Axel; Bonvalot, Sylvie; Giraud, Antoine; Bompas, Emmanuelle; Rios, Maria; Salas, Sébastien; Isambert, Nicolas; Boudou-Rouquette, Pascaline; Honore, Charles; Italiano, Antoine; Ray-Coquard, Isabelle; Piperno-Neumann, Sophie; Gouin, François; Bertucci, François; Ryckewaert, Thomas; Kurtz, Jean-Emmanuel; Ducimetiere, Françoise; Coindre, Jean-Michel; Blay, Jean-Yves

    2017-09-01

    The outcome of desmoid-type fibromatosis (DTF) is unpredictable. Currently, a wait-and-see approach tends to replace large en bloc resection as the first therapeutic approach. Nevertheless, there are no validated factors to guide the treatment choice. We conducted a prospective study of 771 confirmed cases of DTF. We analysed event-free survival (EFS) based on the occurrence of relapse after surgery, progressive disease during the wait-and-see approach, or change in therapeutic strategy. Identification of prognostic factors was performed using classical methods (log-rank test and Cox model). Overall, the 2-year EFS was 56%; this value did not differ between patients undergoing an operation and those managed by the wait-and-see approach (53% versus 58%, p = 0.415). In univariate analysis, two prognostic factors significantly influenced the outcome: the nature of diagnostic sampling (p = 0.466) and primary location (p = 0.0001). The 2-year EFS was only 32% after open biopsy. The 2-year EFS was 66% for favourable locations (abdominal wall, intra-abdominal, breast, digestive viscera and lower limb) and 41% for unfavourable locations. Among patients with favourable locations, the 2-year EFS was similar in patients treated by both surgery (70%) and the wait-and-see approach (63%; p = 0.413). Among patients with unfavourable locations, the 2-year EFS was significantly enhanced in patients initially managed with the wait-and-see approach (52%) compared with those who underwent initial surgery (25%; p = 0.001). The location of DTF is a major prognostic factor for EFS. If these findings are confirmed by independent analysis, personalised management of DTF must consider this easily obtained parameter. Copyright © 2017 Elsevier Ltd. All rights reserved.

  12. Treatment options in otitis media with effusion.

    Science.gov (United States)

    Upadhya, Ila; Datar, J

    2014-01-01

    Secretary Otitis media with effusion (OME) is the accumulation of mucus in the middle ear and sometimes in the mastoid air cell system. The main etiological factor is alteration in mucociliary system of middle ear secondary to ET malfunction which may be primary or secondary. OME is the cause of concern due to its occurance in paediatric age group, highest at 2 years of age, presenting as impairment of hearing leading to delayed speech and language development, poor academic performance and behavioral problems. In spite of this there are no confirmed guidelines of treatment to overcome. Many treatment options are available medical as well as surgical. Prospective study conducted to evaluate various treatment options revealed that auto inflation of ET is the main stay of treatment. If the ET malfunction is due to any reasons like adenoids, deviated nasal septum, hypertrophied turbinates or any other cause surgical intervention of the same gives 100% results. Medical management gives good results but recurrence is equally common.

  13. A PRACTICAL METHOD FOR QUANTIFICATION OF PLEURAL EFFUSION BY USG

    OpenAIRE

    Swish Kumar; Dinesh Kumar; Suganita; Singh; Vijay Shankar; Rajeev; Ajay; Anjali

    2016-01-01

    OBJECTIVE The aim of this study is to find a correlation between pleural separation and amount of aspirated effusion. METHODS Total 20 adult patients with 25 effusions were taken into the study with chest x-ray showing homogeneous opacity in either one or both of the lung field, which was confirmed on USG. Only uncomplicated pleural effusion were taken into study. Effusion with septations or encysted effusion or pyothorax were excluded from the study. RESULTS...

  14. Promiscuous partnerships in Ewing's sarcoma.

    Science.gov (United States)

    Sankar, Savita; Lessnick, Stephen L

    2011-07-01

    Ewing's sarcoma is a highly aggressive bone and soft tissue tumor of children and young adults. At the molecular genetic level Ewing's sarcoma is characterized by a balanced reciprocal translocation, t(11;22)(q24;q12), which encodes an oncogenic fusion protein and transcription factor EWS/FLI. This tumor-specific chimeric fusion retains the amino terminus of EWS, a member of the TET (TLS/EWS/TAF15) family of RNA-binding proteins, and the carboxy terminus of FLI, a member of the ETS family of transcription factors. In addition to EWS/FLI, variant translocation fusions belonging to the TET/ETS family have been identified in Ewing's sarcoma. These studies solidified the importance of TET/ETS fusions in the pathogenesis of Ewing's sarcoma and have since been used as diagnostic markers for the disease. EWS fusions with non-ETS transcription factor family members have been described in sarcomas that are clearly distinct from Ewing's sarcoma. However, in recent years there have been reports of rare fusions in "Ewing's-like tumors" that harbor the amino-terminus of EWS fused to the carboxy-terminal DNA or chromatin-interacting domains contributed by non-ETS proteins. This review aims to summarize the growing list of fusion oncogenes that characterize Ewing's sarcoma and Ewing's-like tumors and highlights important questions that need to be answered to further support the existing concept that Ewing's sarcoma is strictly a "TET/ETS" fusion-driven malignancy. Understanding the molecular mechanisms of action of the various different fusion oncogenes will provide better insights into the biology underlying this rare but important solid tumor. Copyright © 2011 Elsevier Inc. All rights reserved.

  15. Knee effusion after total knee replacement.

    OpenAIRE

    Cameron, H. U.

    1993-01-01

    The various causes of effusions in artificial knees can be divided into four groups: implant related, technique related, interface problems, and infection. Diagnosis can be made from the patient's history and a clinical examination. Treatment is usually surgical revision.

  16. Proclus and Mulla Sadra on First Effusion

    Directory of Open Access Journals (Sweden)

    Saeed Rahimiyan

    2014-01-01

    Full Text Available Mulla Sadra Mulla Sadra's philosophical system is built upon the belief in unique origin of world to which all diversities can be reduced. Then First Effusion debate can be taken on in this system. According to the principle which reads "from the One nothing is effused but One", contingent beings could not have been emanated from the Necessary Being in a horizontal fashion due to their diversity. Rather they have been effused from the Necessary in a vertical fashion and through a hierarchy of causes and effects. The first stage of the hierarchy is being represented by the First Effusion. Pre-Sadraeian theosophers regarded the First Effusion the first intellect in vertical chain of intellects. Mulla Sadra have two theories of the First Effusion. In some of his books he introduces the first intellect as the first entity effused from the Necessary keeping his pace with his own predecessors. But in some other works like Asfar he describes the Ever-unfolding existence as the First Effusion and takes it to be his final position. Having replaced existential gradation with causation and also individual unity of existence with existential gradation, Mulla Sadra asserts that the First Effusion could only be the Ever-unfolding Existence which is a manifestation of Divine Essence not an independent existence. It is indeed nothing but the Divine Essence although in the form of its first manifestation. Thus the Ever-unfolding Existence has three distinguished attributes which make it qualified to stand in direct relationship with Divine Essence, Existential (and not conceptual universality and comprehensiveness which enables this existence to include the other manifestions. Lack of particular limitation and determination; this is why it can reveal itself to every being in its own peculiar existential terms. Having unity while being imbued in diversity and being diversed while having unity (due to its true but shadowy unity (Rahimian, 1383: 187. First

  17. Radiosensitivity of soft tissue sarcomas

    International Nuclear Information System (INIS)

    Hirano, Toru; Iwasaki, Katsuro; Suzuki, Ryohei; Monzen, Yoshio; Hombo, Zenichiro

    1989-01-01

    The correlation between the effectiveness of radiation therapy and the histology of soft tissue sarcomas was investigated. Of 31 cases with a soft tissue sarcoma of an extremity treated by conservative surgery and postoperative radiation of 3,000-6,000 cGy, local recurrence occurred in 12; 5 out of 7 synovial sarcomas, 4 of 9 MFH, one of 8 liposarcomas, none of 4 rhabdomyosarcomas and 2 of 3 others. As for the histological subtyping, the 31 soft tissue sarcomas were divided into spindle cell, pleomorphic cell, myxoid and round cell type, and recurrence rates were 75%, 33.3%, 16.7% and 0%, respectively. From the remarkable difference in recurrent rate, it was suggested that round cell and myxoid type of soft tissue sarcomas showed a high radiosensitivity compared to the spindle cell type with low sensitivity. Clarifying the degree of radiosensitivity is helpful in deciding on the management of limb salvage in soft tissue sarcomas of an extremity. (author)

  18. Dose-Volume Histogram Parameters and Clinical Factors Associated With Pleural Effusion After Chemoradiotherapy in Esophageal Cancer Patients

    International Nuclear Information System (INIS)

    Shirai, Katsuyuki; Tamaki, Yoshio; Kitamoto, Yoshizumi; Murata, Kazutoshi; Satoh, Yumi; Higuchi, Keiko; Nonaka, Tetsuo; Ishikawa, Hitoshi; Katoh, Hiroyuki; Takahashi, Takeo; Nakano, Takashi

    2011-01-01

    Purpose: To investigate the dose-volume histogram parameters and clinical factors as predictors of pleural effusion in esophageal cancer patients treated with concurrent chemoradiotherapy (CRT). Methods and Materials: Forty-three esophageal cancer patients treated with definitive CRT from January 2001 to March 2007 were reviewed retrospectively on the basis of the following criteria: pathologically confirmed esophageal cancer, available computed tomography scan for treatment planning, 6-month follow-up after CRT, and radiation dose ≥50 Gy. Exclusion criteria were lung metastasis, malignant pleural effusion, and surgery. Mean heart dose, mean total lung dose, and percentages of heart or total lung volume receiving ≥10-60 Gy (Heart-V 10 to V 60 and Lung-V 10 to V 60 , respectively) were analyzed in relation to pleural effusion. Results: The median follow-up time was 26.9 months (range, 6.7-70.2) after CRT. Of the 43 patients, 15 (35%) developed pleural effusion. By univariate analysis, mean heart dose, Heart-V 10 to V 60 , and Lung-V 50 to V 60 were significantly associated with pleural effusion. Poor performance status, primary tumor of the distal esophagus, and age ≥65 years were significantly related with pleural effusion. Multivariate analysis identified Heart-V 50 as the strongest predictive factor for pleural effusion (p = 0.01). Patients with Heart-V 50 50 50 ≥40% had 6%, 44%, and 64% of pleural effusion, respectively (p 50 is a useful parameter for assessing the risk of pleural effusion and should be reduced to avoid pleural effusion.

  19. An undiagnosed pleural effusion with surprising consequences

    Directory of Open Access Journals (Sweden)

    A.G. Casalini

    2017-01-01

    With this case report, we would like to underline the importance of making a correct diagnosis of any pleural effusion as soon as possible by at least a thoracocentesis. If untreated, tuberculosis may easily disseminate to other organs. Some considerations and suggestions for antibiotic treatment of pleural effusion will also be given, since many antibiotics have some anti-tuberculosis effect and may delay the diagnosis of this infectious disease.

  20. Massive pleural effusion in a young woman

    OpenAIRE

    Ghiringhelli, Paolo; Cattaneo, Roberto; Tiso, Angelo; Cesaro, Claudia

    2012-01-01

    Pleural effusion is a clinical manifestation shared by several underlying pathologies. The differential diagnosis is based on the clinical history, the physical examination, the analysis of the pleural fluid, and the laboratory data (mainly blood tests). There are cases, such as the patient described, where TC is not enough, and unusual imaging techniques are required for the study of pleural effusion, i.e. magnetic resonance cholangiography, cholangiopancreatography (MRCP) and endoscopic ret...

  1. Routes to Diagnosis for Suspected Sarcoma: The Impact of Symptoms and Clinical Findings on the Diagnostic Process

    Directory of Open Access Journals (Sweden)

    Heidi Buvarp Dyrop

    2016-01-01

    Full Text Available Background and Objectives. Sarcoma patients often experience delay before diagnosis. We examined the association between presenting symptoms/signs and time intervals for suspected sarcoma patients. Methods. 545 consecutive patients suspected for sarcoma referred over a one-year period were included. Median time intervals in routes to diagnosis were collected from medical records and questionnaires. Results. 102 patients (18.7% had a sarcoma; 68 (12.5% had other malignancies. Median interval for the patient (time from first symptom to first doctor visit, primary care, local hospital, sarcoma center, diagnostic, and total interval for sarcoma patients were 77, 17, 29, 17, 65, and 176 days, respectively. Sarcoma patients visited more hospital departments and had longer median primary care (+10 days and diagnostic intervals (+19 days than patients with benign conditions. Median primary care (−19 days and sarcoma center (−4 days intervals were shorter for patients with a lump versus no lump. Median patient (+40 days, primary care (+12 days, diagnostic (+17 days, and total intervals (+78 days were longer for patients presenting with pain versus no pain. GP suspicion of malignancy shortened local hospital (−20 days and total intervals (−104 days. Conclusions. The main part of delay could be attributed to the patient and local hospitals. Length of time intervals was associated with presenting symptoms/signs and GP suspicion.

  2. Chylous pericardial effusion after pulmonary lobectomy.

    Science.gov (United States)

    Yang, Weixiong; Luo, Canqiao; Liu, Zhenguo; Cheng, Chao

    2017-07-01

    Chylous pericardial effusion is a rarely reported complication of lung cancer surgery. Here, we report a case of an elderly man who suffered chylous pericardial effusion after radical right upper lung resection for cancer. The massive chylous effusion first occurred in the pericardium, drained to the right chest after the drainage of the hydropericardium and subsequently moved back to the pericardium again. Lymphoscintigraphy examination indicated that a chylous fistula was present in the plane of the tracheal carina. After failure to control the chylous effusion with conservative medical treatment, the patient underwent video-assisted thoracic surgery through the left chest for thoracic duct ligation and pericardial fenestration. The patient was ultimately discharged without recurrence of the effusion after surgical treatment. This case report discusses the possible mechanism of chylopericardium after lung cancer surgery and suggests some strategies to prevent postoperative chylous pericardial effusion. © The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

  3. Reversible tricuspid valve stenosis due to a metastatic dissemination of a noncardiac sarcoma.

    Science.gov (United States)

    Uribe-Etxebarria, Naia; Voces, Roberto; Rodriguez, Miguel Angel; Llorente, Alberto; Perez, Pedro; Aramendi, Jose I

    2005-07-01

    Malignant disease is present in the pericardium of 1.5% to 20.6% of patients dying of malignant diseases as was examined postmortem. We present a case of a 57-year-old man with a history of Hodgkin's disease and a sarcoma of gluteus who presented with tachypnea, generalized weakness, and anasarca for 7 days. The echocardiogram revealed the presence of a significant pericardial thickening and localized pericardial effusion resulting from a tricuspid stenosis. A right anterior thoracotomy was performed, and a pericardiectomy (4 x 4 cm) was done. The histologic examination of the pericardium revealed the presence of a metastatic dissemination from a sarcoma. The cause for the clinical presentation and the treatment of malignant pericardial disease are discussed.

  4. A case of Meigs' syndrome with preceding pericardial effusion in advance of pleural effusion.

    Science.gov (United States)

    Okuda, Kenichi; Noguchi, Satoshi; Narumoto, Osamu; Ikemura, Masako; Yamauchi, Yasuhiro; Tanaka, Goh; Takai, Daiya; Fukayama, Masashi; Nagase, Takahide

    2016-05-10

    Meigs' syndrome is defined as the presence of a benign ovarian tumor with pleural effusion and ascites that resolve after removal of the tumor. The pathogenesis of the production of ascites and pleural effusion in this syndrome remains unknown. Aside from pleural effusion and ascites, pericardial effusion is rarely observed in Meigs' syndrome. Here, we report the first case of Meigs' syndrome with preceding pericardial effusion in advance of pleural effusion. An 84-year-old Japanese non-smoking woman with a history of lung cancer, treated by surgery, was admitted due to gradual worsening of dyspnea that had occurred over the previous month. She had asymptomatic and unchanging pericardial effusion and a pelvic mass, which had been detected 3 and 11 years previously, respectively. The patient was radiologically followed-up without the need for treatment. Two months before admission, the patient underwent a right upper lobectomy for localized lung adenocarcinoma and intraoperative pericardial fenestration confirmed that the pericardial effusion was not malignant. However, she began to experience dyspnea on exertion leading to admission. A chest, abdomen, and pelvis computed tomography scan confirmed the presence of right-sided pleural and pericardial effusion and ascites with a left ovarian mass. Repeated thoracentesis produced cultures that were negative for any microorganism and no malignant cells were detected in the pleural effusions. Pleural fluid accumulation persisted despite a tube thoracostomy for pleural effusion drainage. With a suspicion of Meigs' syndrome, the patient underwent surgical resection of the ovarian mass and histopathological examination of the resected mass showed ovarian fibroma. Pleural and pericardial effusion as well as ascites resolved after tumor resection, confirming a diagnosis of Meigs' syndrome. This clinical course suggests a strong association between pericardial effusion and ovarian fibroma, as well as pleural and peritoneal

  5. Post-radiation sarcomas: A review of the clinical and imaging features in 63 cases

    International Nuclear Information System (INIS)

    Sheppard, Declan G.; Libshitz, Hermann I.

    2001-01-01

    AIMS: The development of sarcomas is a recognized complication of radiation therapy. We set out to retrospectively review the clinical and therapeutic demographics, as well as the cross-sectional imaging findings in patients with post-radiation sarcomas. MATERIALS AND METHODS: Sixty-three patients with post-radiation sarcomas were identified at a single institution. Computed tomography and/or magnetic resonance imaging was available for all patients. The medical records were reviewed for the primary diagnoses, the radiation history, and the latency period to the development of the sarcoma. RESULTS: There were 43 women and 20 men with a mean age of 52.8 years. The mean radiation dose delivered was 50.1 Gy, with a mean latency period for the development of the sarcoma of 15.5 years. The most common primary diagnoses were breast cancer, lymphoma and head and neck cancer. The most common sarcoma histopathologies were osteosarcoma and malignant fibrous histiocytoma. The most common imaging findings were a soft tissue mass and bone destruction. CONCLUSIONS: Post-radiation sarcomas, while uncommon, are not rare. The imaging findings are not pathognomonic, but an appreciation of the expected latency period may help to suggest the diagnosis. Sheppard, D.G. and Libshitz, H.I. (2001)

  6. Postirradiation soft tissue sarcoma occurring in breast cancer patients: report of seven cases and results of combination chemotherapy

    International Nuclear Information System (INIS)

    Kuten, A.; Sapir, D.; Cohen, Y.; Haim, N.; Borovik, R.; Robinson, E.

    1985-01-01

    Seven cases of soft tissue sarcoma developing after primary or postoperative radiotherapy for breast carcinoma are reported. The sarcomas occurred within the irradiated volume, after a latent period of 4-26 years. These cases conform well to established criteria for the diagnosis of radiation-induced sarcoma. Chemotherapy, consisting of the four-drug combination CYVADIC (cyclophosphamide, vincristine, adriamycin, DTIC) was employed in six of the seven patients. Only two of them achieved partial remission, lasting only 2 and 3 months, respectively. The effectiveness of adriamycin-containing chemotherapy regimens in soft tissue sarcomas as well as the remote hazard of radiation-related sarcoma in primary or postoperative breast irradiation are discussed

  7. Synovial sarcoma of the abdominal wall

    International Nuclear Information System (INIS)

    Matushita, J.P.K.; Matushita, J.S.

    1989-01-01

    A case report of synovial sarcoma arising in the abdominal wall is presented. A brief review of the clinical and radiological features of synovial sarcoma is made. Pre-operative diagnosis of an abdominal wall synovial sarcoma is virtually impossible, but should be considered when a soft tissue swelling is found to show amorphous stippled calcification X-ray. (author) [pt

  8. Dexamethasone for Parapneumonic Pleural Effusion: A Randomized, Double-Blind, Clinical Trial.

    Science.gov (United States)

    Tagarro, Alfredo; Otheo, Enrique; Baquero-Artigao, Fernando; Navarro, María-Luisa; Velasco, Rosa; Ruiz, Marta; Penín, María; Moreno, David; Rojo, Pablo; Madero, Rosario

    2017-06-01

    To assess whether dexamethasone (DXM) decreases the time to recovery in patients with parapneumonic pleural effusion. This was a multicenter, randomized, double blind, parallel-group, placebo-controlled clinical trial of 60 children, ranging in age from 1 month to 14 years, with community-acquired pneumonia (CAP) and pleural effusion. Patients received either intravenous DXM (0.25?mg/kg/dose) or placebo every 6 hours over a period of 48 hours, along with antibiotics. The primary endpoint was the time to recovery in hours, defined objectively. We also evaluated complications and adverse events. Among the 60 randomized patients (mean age, 4.7 years; 58% female), 57 (95%) completed the study. Compared with placebo recipients, the patients receiving DXM had a shorter time to recovery, after adjustment by severity group and stratification by center (hazard ratio, 1.95; 95% CI, 1.10-3.45; P?=?.021). The median time to recovery for patients receiving DXM was 68 hours (2.8 days) shorter than patients receiving placebo (109 hours vs 177 hours; P?=?.037). In exploratory subgroup analysis, the median time to recovery for patients with simple effusion receiving DXM was 76 hours (3.1 days) shorter than for patients with simple effusion receiving placebo (P?=?.017). The median time to recovery for patients with complicated effusion receiving DXM was 14 hours (0.5 days) shorter than for patients with complicated effusion receiving placebo (P?=?.66). The difference in the effect of DXM in the 2 severity groups was not statistically significant (P?=?.138 for interaction). There were no significant differences in complications or adverse events attributable to the study drugs, except for hyperglycemia. In this trial, DXM seemed to be a safe and effective adjunctive therapy for parapneumonic pleural effusion. ClinicalTrials.gov: NCT01261546. Copyright © 2017 Elsevier Inc. All rights reserved.

  9. Uterine sarcoma – current perspectives

    Science.gov (United States)

    Benson, Charlotte; Miah, Aisha B

    2017-01-01

    Uterine sarcomas comprise a group of rare tumors with differing tumor biology, natural history and response to treatment. Diagnosis is often made following surgery for presumed benign disease. Currently, preoperative imaging does not reliably distinguish between benign leiomyomas and other malignant pathology. Uterine leiomyosarcoma is the most common sarcoma, but other subtypes include endometrial stromal sarcoma (low grade and high grade), undifferentiated uterine sarcoma and adenosarcoma. Clinical trials have shown no definite survival benefit of adjuvant radiotherapy or chemotherapy and have been hampered by the rarity and heterogeneity of these disease types. There is a role of adjuvant treatment in carefully selected cases following multidisciplinary discussion at sarcoma reference centers. In patients with metastatic disease, systemic chemotherapy can then be considered. There is activity of a number of agents, including doxorubicin, trabectedin, gemcitabine-based chemotherapy, eribulin and pazopanib. Patients should be considered for clinical trial entry where possible. Close international collaboration is important to allow progress in this group of diseases. PMID:28919822

  10. Dural metastasis of Ewing's sarcoma.

    Science.gov (United States)

    Ben Nsir, Atef; Boughamoura, Mohamed; Maatouk, Mezri; Kilani, Mohamed; Hattab, Nejib

    2013-01-01

    Metastatic Ewing's sarcoma to the central nervous system is an uncommon condition and debate concerning the true origin of its metastases is still up to date. To the best of our knowledge, only two cases of dural metastatic Ewing's sarcoma have been published in the English medical literature. We present an additional case in a 24-year-old female and discuss the pathogenesis of these unusual tumors with review of the relevant literature concerning their treatment and outcome. A 24-year-old female with previous history of pelvis Ewing's sarcoma and recently discovered lung metastases, presented with moderate headache for the past 2 weeks and weakness in her left leg for the past 2 days. Computed tomography scan and magnetic resonance imaging revealed an extra-axial right frontoparietal mass invading the superior sagittal sinus but with clear delineation with brain parenchyma. Imaging features were suggestive of a meningioma as no abnormalities in the skull abutting to the tumor were noted. The patient underwent surgical removal of her tumor. Near total resection was achieved and histological examination showed evidence of metastatic Ewing's sarcoma. Postoperative adjuvant radiation and chemotherapy were administered. The patient improved well postoperatively with full recovery of her motor weakness. She is symptom free with no signs of progression, at most recent follow-up, 8 months after surgery. Despite its rarity, metastatic Ewing's sarcoma must be considered in the differential diagnosis of extra-axial dural masses particularly meningiomas.

  11. [Extra-skeletal Ewing's sarcoma. A case report with detailed review of the literature].

    Science.gov (United States)

    Auge, B; Pusel, J

    1990-01-01

    Extra-skeletal Ewing's Sarcoma (EES): a clinico-pathological entity described in 1975 by L. Angervall and F.M. Enzinger. This article reports a new case in which the primary was localized in the right forearm and metastasized to the left lung fourteen years later. A detailed review of the literature emphasized similarities and differences between osseous and extra-osseous Ewing's Sarcoma.

  12. Bone Tumor Environment as a Potential Therapeutic Target in Ewing Sarcoma

    OpenAIRE

    Redini, Fran?oise; Heymann, Dominique

    2015-01-01

    Ewing sarcoma is the second most common pediatric bone tumor, with three cases per million worldwide. In clinical terms, Ewing sarcoma is an aggressive, rapidly fatal malignancy that mainly develops not only in osseous sites (85%) but also in extra-skeletal soft tissue. It spreads naturally to the lungs, bones, and bone marrow with poor prognosis in the two latter cases. Bone lesions from primary or secondary (metastases) tumors are characterized by extensive bone remodeling, more often due t...

  13. Radio-induced sarcomas in survivors of Ewing sarcoma

    International Nuclear Information System (INIS)

    Boriani, S.; Sudanese, A.; Toni, A.; Monesi, M.; Ciaroni, D.; Mancini, A.; Frezza, G.; Barbieri, E.; Picci, P.; Bacci, G.

    1988-01-01

    Of 255 cases of Ewing's sarcoma recorded at the Bone Tumor Center of the Rizzoli Orthopaedic Institute, 78 patients (irradiated and with a follow-up of longer than3 years) were considered ''at risk'' for the development of a second radio-induced sarcoma (RIS). Three of the 78 patients developed an RIS in the irradiated field. Theoretical and statistical analyses were carried out considering different modalities of local treatment. Statistically, the only significant factor was related to the irradiation dose. Surgical resection seems to prevent RIS

  14. Clinical importance of angiogenic cytokines, fibrinolytic activity and effusion size in parapneumonic effusions.

    Directory of Open Access Journals (Sweden)

    Chi-Li Chung

    Full Text Available OBJECTIVE: To investigate the relationship among angiogenic cytokines, fibrinolytic activity and effusion size in parapneumonic effusion (PPE and their clinical importance. METHODS: From January 2008 through December 2010, 26 uncomplicated (UPPE and 38 complicated (CPPE PPE were studied. Based on chest ultrasonography, there were non-loculated in 30, uni-loculated in 12, and multi-loculated effusions in 22 patients. The effusion size radiological scores, and effusion vascular endothelial growth factor (VEGF, interleukin (IL-8, plasminogen activator inhibitor type-1 (PAI-1 and tissue type plasminogen activator (tPA were measured on admission. Treatment outcome and pleural fibrosis, defined as radiological residual pleural thickening (RPT, were assessed at 6-month follow-up. RESULTS: The effusion size and effusion VEGF, IL-8 and PAI-1/tPA ratio were significantly higher in CPPE than in UPPE, and significantly higher in multi-loculated PPE than in non-locualted and uni-loculated PPE, respectively. VEGF (cutoff value 1975 pg/ml and IL-8 (cutoff value 1937 pg/ml seemed best to discriminate between UPPE and CPPE. VEGF, IL-8 and effusion size correlated positively with PAI-1/tPA ratio in both UPPE and CPPE. Moreover, the level of VEGF, but not IL-8, correlated positively with effusion size in all patients (r = 0.79, p<0.001 and in UPPE (r = 0.64, p<0.001 and CPPE (r = 0.71, p<0.001 groups. The patients with higher VEGF or greater effusion were prone to have medical treatment failure (n = 10; VEGF, odds ratio 1.01, p = 0.02; effusion size, odds ratio 1.26, p = 0.01. Additionally, ten patients with RPT had larger effusion size and higher levels of VEGF and PAI-1/tPA ratio than did those without. CONCLUSIONS: In PPE, VEGF and IL-8 levels are valuable to identify CPPE, and higher VEGF level or larger effusion is associated with decreased fibrinolytic activity, development of pleural loculation and fibrosis, and higher risk of medical treatment failure.

  15. Epidemiology and therapies for metastatic sarcoma

    Directory of Open Access Journals (Sweden)

    Amankwah EK

    2013-05-01

    Full Text Available Ernest K Amankwah,1 Anthony P Conley,2 Damon R Reed2 1Department of Cancer Epidemiology, H Lee Moffitt Cancer Center and Research Institute, Tampa, FL, USA; 2Sarcoma Department, H Lee Moffitt Cancer Center and Research Institute, Tampa, FL, USA Abstract: Sarcomas are cancers arising from the mesenchymal layer that affect children, adolescents, young adults, and adults. Although most sarcomas are localized, many display a remarkable predilection for metastasis to the lungs, liver, bones, subcutaneous tissue, and lymph nodes. Additionally, many sarcoma patients presenting initially with localized disease may relapse at metastatic sites. While localized sarcomas can often be cured through surgery and often radiation, controversies exist over optimal management of patients with metastatic sarcoma. Combinations of chemotherapy are the most effective in many settings, and many promising new agents are under active investigation or are being explored in preclinical models. Metastatic sarcomas are excellent candidates for novel approaches with additional agents as they have demonstrated chemosensitivity and affect a portion of the population that is motivated toward curative therapy. In this paper, we provide an overview on the common sarcomas of childhood (rhabdomyosarcoma, adolescence, and young adults (osteosarcoma, Ewing sarcoma, synovial sarcoma, and malignant peripheral nerve sheath tumor and older adults (leiomyosarcoma, liposarcoma, and undifferentiated high grade sarcoma in terms of the epidemiology, current therapy, promising therapeutic directions and outcome with a focus on metastatic disease. Potential advances in terms of promising therapy and biologic insights may lead to more effective and safer therapies; however, more clinical trials and research are needed for patients with metastatic sarcoma. Keywords: chemotherapy, pediatric sarcoma, rhabdomyosarcoma, osteosarcoma, Ewing sarcoma, synovial sarcoma

  16. Myofibroblastic sarcoma of the base of tongue. Case report and review of the literature

    International Nuclear Information System (INIS)

    Takacsi-Nagy, Zoltan; Fodor, Janos; Murakoezy, Gyoergyi; Pogany, Peter; Orosz, Zsolt

    2009-01-01

    Background: Mesenchymal malignancies with myofibroblastic differentiation exhibit a spectrum from low-grade myofibroblastic sarcoma mimicking fibromatosis to pleomorphic high-grade sarcoma. Low-grade myofibroblastic sarcoma shows a wide anatomic distribution with a predilection for the head-and-neck region; however, intermediate- and high-grade myofibroblastic sarcomas in this localization are exceptional. Case Report: A 56-year-old woman with intermediate-grade myofibroblastic sarcoma of the base of tongue is presented. She was treated with surgical excision, but computed tomography proved local residual tumor. Reexcision and chemotherapy were refused by the patient. Irradiation was given to a total dose of 66 Gy. Result: 50 months after completion of radiotherapy, the patient is in good health without any evidence of disease. According to the review of the literature, base of tongue as the primary site of myofibroblastic sarcoma has not been published so far. Conclusion: Similarly to the low-grade form, intermediate- and high-grade myofibroblastic sarcomas may also occur in the head-and-neck region. In case of incomplete excision, radiotherapy may be an effective treatment. (orig.)

  17. Protocol of the Australasian Malignant Pleural Effusion (AMPLE) trial: a multicentre randomised study comparing indwelling pleural catheter versus talc pleurodesis

    Science.gov (United States)

    Fysh, Edward T H; Thomas, Rajesh; Read, Catherine A; Lam, Ben C H; Yap, Elaine; Horwood, Fiona C; Lee, Pyng; Piccolo, Francesco; Shrestha, Ranjan; Garske, Luke A; Lam, David C L; Rosenstengel, Andrew; Bint, Michael; Murray, Kevin; Smith, Nicola A; Lee, Y C Gary

    2014-01-01

    Introduction Malignant pleural effusion can complicate most cancers. It causes breathlessness and requires hospitalisation for invasive pleural drainages. Malignant effusions often herald advanced cancers and limited prognosis. Minimising time spent in hospital is of high priority to patients and their families. Various treatment strategies exist for the management of malignant effusions, though there is no consensus governing the best choice. Talc pleurodesis is the conventional management but requires hospitalisation (and substantial healthcare resources), can cause significant side effects, and has a suboptimal success rate. Indwelling pleural catheters (IPCs) allow ambulatory fluid drainage without hospitalisation, and are increasingly employed for management of malignant effusions. Previous studies have only investigated the length of hospital care immediately related to IPC insertion. Whether IPC management reduces time spent in hospital in the patients’ remaining lifespan is unknown. A strategy of malignant effusion management that reduces hospital admission days will allow patients to spend more time outside hospital, reduce costs and save healthcare resources. Methods and analysis The Australasian Malignant Pleural Effusion (AMPLE) trial is a multicentred, randomised trial designed to compare IPC with talc pleurodesis for the management of malignant pleural effusion. This study will randomise 146 adults with malignant pleural effusions (1:1) to IPC management or talc slurry pleurodesis. The primary end point is the total number of days spent in hospital (for any admissions) from treatment procedure to death or end of study follow-up. Secondary end points include hospital days specific to pleural effusion management, adverse events, self-reported symptom and quality-of-life scores. Ethics and dissemination The Sir Charles Gairdner Group Human Research Ethics Committee has approved the study as have the ethics boards of all the participating hospitals. The

  18. Massive pleural effusion in a young woman

    Directory of Open Access Journals (Sweden)

    Paolo Ghiringhelli

    2012-12-01

    Full Text Available Pleural effusion is a clinical manifestation shared by several underlying pathologies. The differential diagnosis is based on the clinical history, the physical examination, the analysis of the pleural fluid, and the laboratory data (mainly blood tests. There are cases, such as the patient described, where TC is not enough, and unusual imaging techniques are required for the study of pleural effusion, i.e. magnetic resonance cholangiography, cholangiopancreatography (MRCP and endoscopic retrograde cholangiopancreatography (ERCP.This case analyses a 42-year-old female patient who arrived with progressive dyspnoea, chest pain, cough, a history of alcohol abuse, and a recent episode of acute pancreatitis. The physical examination revealed signs of right-sided pleural effusion. These features, together with laboratory data, made it possible to pose the diagnosis of pancreaticopleural fistula, to treat it, and to obtain a complete healing in a two-month period.

  19. Investigating a Case of Recurrent Pleural Effusion

    Directory of Open Access Journals (Sweden)

    Patrícia Rodrigues

    2011-01-01

    Full Text Available We describe the case of a patient with long-standing Parkinson's disease and recurrent bilateral pleural effusions. The pleural fluid was an exudate, rich in normal lymphocytes, and the echocardiogram, chest computerized axial tomography, and immunological, microbiological and cytological studies were negative. The patient had been taking bromocriptine, which can be related to chronic pleural effusions. Using Pubmed, we found about 40 cases of pleuropulmonary changes or constrictive pericarditis that were related to bromocriptine. We decided to suspend this drug, with resolution of the pleural effusion and respiratory complaints for more than a year now. We discuss possible underlining mechanisms for this and emphasize the importance of collecting the past medical history and medication and of considering possible iatrogenic effects.

  20. Radiotherapy in complex management of Ewing sarcoma

    International Nuclear Information System (INIS)

    Ruseva, Tz.; Marinova, L.; Hristozova, I.; Sokolov, T.

    1996-01-01

    Over the period 1981-1995, 32 patients with non-metastatic Ewing sarcoma, aged 2 to 22 years, underwent treatment and follow-up study. A locally advanced tumor is found in 85% of patients. Complex radio- and chemotherapy is carried out in all patients, and additional radical surgery - in eight. 60 Co-radiotherapy with gradual radiation field reduction is applied, applying a local dose of 60 - 64 Gy in the tumor area. Chemotherapy is performed according to the VACA protocol. Local tumor control is achieved in all patients with primary tumor located in long bones, and only in 1 of 5 patients with pelvic localization. The overall survival established is as follows: 1 year survival - 100 %, 2 years - 50 %, 3 years - 40 % and 5 years - 34 %. Local recurrence is recorded in 2 cases (6.2%). The distant metastases observed involve the lungs (13), bones (4), lung and bones (2) and soft tissues (3). The Ewing sarcoma is sensible to chemo- and radiotherapy, but the conventional methods do not improve survival. Based on the analysis of the clinical results, emphasis is laid on the necessity of complex treatment with a special reference to alternative therapeutic approaches. 13 refs., 4 figs., 2 tabs

  1. Epidemiology and therapies for metastatic sarcoma

    Science.gov (United States)

    Amankwah, Ernest K; Conley, Anthony P; Reed, Damon R

    2013-01-01

    Sarcomas are cancers arising from the mesenchymal layer that affect children, adolescents, young adults, and adults. Although most sarcomas are localized, many display a remarkable predilection for metastasis to the lungs, liver, bones, subcutaneous tissue, and lymph nodes. Additionally, many sarcoma patients presenting initially with localized disease may relapse at metastatic sites. While localized sarcomas can often be cured through surgery and often radiation, controversies exist over optimal management of patients with metastatic sarcoma. Combinations of chemotherapy are the most effective in many settings, and many promising new agents are under active investigation or are being explored in preclinical models. Metastatic sarcomas are excellent candidates for novel approaches with additional agents as they have demonstrated chemosensitivity and affect a portion of the population that is motivated toward curative therapy. In this paper, we provide an overview on the common sarcomas of childhood (rhabdomyosarcoma), adolescence, and young adults (osteosarcoma, Ewing sarcoma, synovial sarcoma, and malignant peripheral nerve sheath tumor) and older adults (leiomyosarcoma, liposarcoma, and undifferentiated high grade sarcoma) in terms of the epidemiology, current therapy, promising therapeutic directions and outcome with a focus on metastatic disease. Potential advances in terms of promising therapy and biologic insights may lead to more effective and safer therapies; however, more clinical trials and research are needed for patients with metastatic sarcoma. PMID:23700373

  2. Allergic rhinitis is associated with otitis media with effusion

    DEFF Research Database (Denmark)

    Kreiner-Møller, E; Chawes, B L K; Thomasen, Per Caye

    2012-01-01

    Childhood otitis media with effusion is a common disease and a link to allergic diseases has been suggested.......Childhood otitis media with effusion is a common disease and a link to allergic diseases has been suggested....

  3. Synovial sarcoma mimicking benign peripheral nerve sheath tumor

    Energy Technology Data Exchange (ETDEWEB)

    Larque, Ana B.; Nielsen, G.P.; Chebib, Ivan [Massachusetts General Hospital and Harvard Medical School, Department of Pathology, Boston, MA (United States); Bredella, Miriam A. [Massachusetts General Hospital and Harvard Medical School, Department of Radiology, Boston, MA (United States)

    2017-11-15

    To assess the radiographic and clinicopathologic features of synovial sarcoma of the nerve that were clinically or radiologically interpreted as benign peripheral nerve sheath tumor. Five patients with synovial sarcoma arising from the peripheral nerve and interpreted clinically and radiologically as peripheral nerve sheath tumors were identified. Clinicopathologic and imaging features were evaluated. There were three females and two males, ranging in age from 28 to 50 (mean 35.8) years. Most patients (4/5) complained of a mass, discomfort or pain. MR images demonstrated a heterogeneous, enhancing, soft tissue mass contiguous with the neurovascular bundle. On histologic examination, most tumors were monophasic synovial sarcoma (4/5). At the time of surgery, all tumors were noted to arise along or within a peripheral nerve. All patients were alive with no evidence of disease with median follow-up of 44 (range 32-237) months. For comparison, approximately 775 benign peripheral nerve sheath tumors of the extremities were identified during the same time period. Primary synovial sarcoma of the nerve can mimic peripheral nerve sheath tumors clinically and on imaging and should be included in the differential diagnosis for tumors arising from peripheral nerves. (orig.)

  4. Survival of patients with Ewing's sarcoma in Yazd-Iran.

    Science.gov (United States)

    Akhavan, Ali; Binesh, Fariba; Shamshiri, Hadi; Ghanadi, Fazllolah

    2014-01-01

    The Ewing's sarcoma family is a group of small round cell tumors which accounts for 10-15% of all primary bone neoplasms. The aim of this study was to evaluate the survival of Ewing's sarcoma patients in our province and to determine of influencing factors. All patients with documented Ewing's sarcoma/ primitive neuroectodermal tumor(PNET) family pathology were enrolled in this study during a period of eight years. For all of them local and systemic therapy were carried out. Overall and event free survival and prognostic factors were evaluated. Thirty two patients were enrolled in the study. The median age was 17.5 years. Twenty (65.2%) were male and 9 (28.1%) were aged 14 years or less. Mean disease free survival was 26.8 (95%CI; 13.8-39.9) months and five year disease free survival was 26%. Mean overall survival was 38.7 months (95%CI; 25.9-50.6) and median overall survival was 24 months. Five year overall survival was 25%. From the variables evaluated , only presence of metastatic disease at presentation (p value=0. 028) and complete response (p value =0. 006) had significant relations to overall survival. Survival of Ewing's sarcoma in our province is disappointing. It seems to be mostly due to less effective treatment. Administration of adequate chemotherapy dosage, resection of tumor with negative margins and precise assessment of irradiation volume may prove helpful.

  5. Isolated posterior uveal effusion: expanding the spectrum of the uveal effusion syndrome

    Directory of Open Access Journals (Sweden)

    Pautler SE

    2014-12-01

    Full Text Available Scott E Pautler,1 David J Browning2 1Department of Ophthalmology, University of South Florida, Morsani College of Medicine, Tampa, FL, USA; 2Charlotte Ear Eye Nose and Throat Associates, Charlotte, NC, USA Abstract: Uveal effusion syndrome usually causes peripheral chorioretinal detachment, but posterior effusion may present as isolated macular edema with serous macular detachment in the setting of hyperopia and a thickened posterior choroid. Carbonic anhydrase inhibitors may be effective to treat this condition. Keywords: uveal effusion, serous, macular detachment, macular edema

  6. Pleural effusion in 11:14 translocation q1 multiple myeloma in the setting of proteasome inhibitor presents therapeutic complexity.

    Science.gov (United States)

    Ghannam, Malik; Bryan, Maria; Kuross, Erik; Berry, Brent

    2018-01-01

    Primary malignant pleural effusion has been reported in about 134 cases of multiple myeloma (MM). Associated pleural effusions in cases of MM portend a poor prognosis and identifying them is highly relevant. Reported is the case of a man diagnosed with MM who developed primary myelomatous pleural effusion in the setting of multiple relapses and subsequent mortality within 2 months of the pleural effusion diagnosis. A 61-year-old African American man was diagnosed with MM in 2011. He received induction therapy of lenalidomide and dexamethasone and an autologous stem cell transplant in 2012. Over the next 5 years, the patient went through alternating periods of remission and relapse that were treated with two rounds of thoracic spine radiation therapy and chemotherapeutic agents. In September 2017, the patient presented with worsening dyspnea and was found to have pleural effusion. Fluid analysis showed plasma cell dyscrasia. Fluid drainage was performed, then the patient was discharged after 1 week which was followed by rapid re-accumulation of fluid and rehospitalization about 10 days after discharge. The patient passed away a few weeks after the second admission. Pleural effusion carries a differential diagnosis which may include malignancy but is commonly thought to be less specific to multiple myeloma but should still remain in the differential diagnosis. To our knowledge, this is the first case of myelomatous pleural effusion (MPE) that was reported after multiple relapses of MM. MPE is a very rare complication of MM, and its presence is a strong indicator of imminent mortality and need for comfort care in case of multiple relapses. End-stage pleural effusion in MM in the setting of proteasome inhibitor adds more therapeutic and diagnostic challenges.

  7. Limb-sparing management with surgical resection, external-beam and intraoperative electron-beam radiation therapy boost for patients with primary soft tissue sarcoma of the extremity. A multicentric pooled analysis of long-term outcomes

    Energy Technology Data Exchange (ETDEWEB)

    Calvo, Felipe A. [Hospital General Universitario Gregorio Maranon, Department of Oncology, Madrid (Spain); Complutense University, School of Medicine, Madrid (Spain); Sole, Claudio V. [Hospital General Universitario Gregorio Maranon, Department of Oncology, Madrid (Spain); Complutense University, School of Medicine, Madrid (Spain); Instituto de Radiomedicina, Service of Radiation Oncology, Santiago (Chile); Polo, Alfredo; Montero, Angel [Hospital Universitario Ramon y Cajal, Service of Radiation Oncology, Madrid (Spain); Cambeiro, Mauricio; Martinez-Monge, Rafael [Clinica Universidad de Navarra, Service of Radiation Oncology, Pamplona (Spain); Alvarez, Ana [Hospital General Universitario Gregorio Maranon, Service of Radiation Oncology, Madrid (Spain); Cuervo, Miguel [Hospital General Universitario Gregorio Maranon, Service of Orthopedics and Traumatology, Madrid (Spain); Julian, Mikel San [Clinica Universidad de Navarra, Service of Orthopedics and Traumatology, Pamplona (Spain)

    2014-10-15

    A joint analysis of data from three contributing centres within the intraoperative electron-beam radiation therapy (IOERT) Spanish program was performed to investigate the main contributions of IORT to the multidisciplinary treatment of high-risk extremity soft tissue sarcoma (STS). Patients with an histologic diagnosis of primary extremity STS, with absence of distant metastases, undergoing limb-sparing surgery with radical intent, external beam radiotherapy (median dose 45 Gy) and IOERT (median dose 12.5 Gy) were considered eligible for participation in this study. From 1986-2012, a total of 159 patients were analysed in the study from three Spanish institutions. With a median follow-up time of 53 months (range 4-316 years), 5-year local control (LC) was 82 %. The 5-year IOERT in-field control, disease-free survival (DFS) and overall survival (OS) were 86, 62 and 72 %, respectively. On multivariate analysis, only microscopically involved margin (R1) resection status retained significance in relation to LC (HR 5.20, p < 0.001). With regard to IOERT in-field control, incomplete resection (HR 4.88, p = 0.001) and higher IOERT dose (≥ 12.5 Gy; HR 0.32, p = 0.02) retained a significant association in multivariate analysis. From this joint analysis emerges the fact that an IOERT dose ≥ 12.5 Gy increases the rate of IOERT in-field control, but DFS remains modest, given the high risk of distant metastases. Intensified local treatment needs to be tested in the context of more efficient concurrent, neo- and adjuvant systemic therapy. (orig.) [German] Um den therapeutischen Beitrag einer intraoperativen Bestrahlung mit Elektronen (IOERT) als Teil eines multidisziplinaeren Behandlungskonzepts von Weichteilsarkomen (STS) im Extremitaetenbereich mit hohem Risikoprofil evaluieren zu koennen, wurde anhand des spanischen IOERT-Programms eine gepoolte Datenanalyse von drei teilnehmenden Zentren vorgenommen. Eingeschlossen in diese Studie wurden Patienten mit histologisch

  8. Magnetic resonance imaging features of extremity sarcomas of uncertain differentiation

    International Nuclear Information System (INIS)

    Stacy, G.S.; Nair, L.

    2007-01-01

    The purpose of this review is to illustrate the pertinent clinical and imaging features of extremity sarcomas of uncertain differentiation, including synovial sarcoma, epithelioid sarcoma, clear-cell sarcoma, and alveolar soft part sarcoma. These tumours should be considered in the differential diagnosis when a soft-tissue mass is encountered in the extremity of an adolescent or young adult

  9. Evaluation of CT findings for diagnosis of pleural effusions

    International Nuclear Information System (INIS)

    Arenas-Jimenez, J.; Alonso-Charterina, S.; Fernandez-Latorre, F.; Gil-Sanchez, S.; Sanchez-Paya, J.; Lloret-Llorens, M.

    2000-01-01

    Computed tomography studies are usually used to assess patients with pleural effusions, and radiologists should be aware of the significance of different CT findings for the diagnosis of the effusion. The purpose of this study was to evaluate CT findings for etiological diagnosis of pleural effusions. Contrast-enhanced CT of the chest of 211 patients with pleural effusion of definite diagnosis were evaluated. The CT images were evaluated for the presence and extent of pleural effusion, thickening or nodules, extrapleural fat and other changes in the mediastinum or lung. The CT scans were read by two independent observers and correlation between them was evaluated. Comparison of CT findings between benign and malignant effusions, between exudates and transudates, and between empyemas and the other parapneumonic effusions were carried out. Kappa values for most CT findings were >0.85. Loculation, pleural thickening, pleural nodules, and extrapleural fat of increased density were only present in exudative effusions. Multiple pleural nodules and nodular pleural thickening were the only pleural findings limited to malignant pleural effusions. The signs were also more frequently seen in empyemas than in other parapneumonic effusions. Computed tomography findings can help to distinguish between transudates and exudates. Although there is some overlap between benign and malignant pleural effusions, pleural nodules and nodular pleural thickening were present almost exclusively in the latter. Although differences between CT findings of empyemas and the other parapneumonic effusions exist, there is no finding which can definitely differentiate between them. (orig.)

  10. Clinical value of FDG dual-head tomography with coincidence imaging in suspected malignant pleural effusion or atelectasis

    International Nuclear Information System (INIS)

    Wang Huoqiang; Wu Jiyang; Pan Huizhong; Liu Jinjun; Shen Yi; Zhao Xianguo

    2004-01-01

    Objective: To determine the ability of 18F-fluorodeoxyglucose (FDG) dual-head tomography with coincidence (DHTC) imaging in detecting lung cancer in patients with suspected malignant pleural effusion or malignant atelectasis on CT scanning. On the other hand, used FDG DHTC to differentiate benign and malignant pleural effusions in patients with lung cancer. Methods: Over a 4-year period, 110 patients with abnormal findings on CT scanning for presence of pleural effusion (n=84) and/or pleural thickening or mediastinal lymph nodes enlargement, or presence of atelectasis (n-26). All patients were suspected malignant pleural effusion or atelectasis caused by lung cancer. But all of them could not found primary lesions in the lungs on CT scanning. FDG DHTC was underwent for all the patients. Imagings were visually analyzed by two experienced observers. They were considered positive if the FDG uptake was increased relative to that in the adjacent lung tissue, and the uptake appeared localized pattern. They were considered negative if the uptake was the same as or less than the adjacent lung tissue, and/or the uptake appeared diffused pattern. Results of FDG DHTC were correlated with pathologic diagnosis for lung cancer. In these patients with proven lung cancer and a suspected malignant pleural effusion, FDG DHTC revealed positive findings (malignant pleural effusion) if pleural activity was greater than background mediastinal activity; or FDG DHTC revealed negative findings (benign pleural effusion) if pleural activity was the same or less than background mediastinal activity. Results of FDG DHTC were compared to pleural cytology, histologic findings of pleural biopsy, or clinical follow-up for presence or absence of malignant pleural effusion. Results: 38 of 110 patients were proven with lung cancer. Among the 38 lung cancer patients, 30 of them had a pleural effusion and 8 of them had a atelectasis. 72 of 110 patients were proven with lung benign diseases (50 with

  11. Undifferentiated pleomorphic sarcoma with osteoclast-like giant cells of the female breast

    Directory of Open Access Journals (Sweden)

    Balbi Giancarlo

    2013-01-01

    Full Text Available Abstract The authors describe a case of undifferentiated pleomorphic sarcoma of the breast occurring in a 50-year-old woman who presented with a palpable mass in her right breast. She first noticed the mass one month previously. Core needle biopsy showed connective tissue including epithelioid and spindle cells. The patient underwent total mastectomy without axillary lymph node dissection. Based on examination of the excised tumor, the initial pathologic diagnosis was atypical spindle-shaped and ovoid cells with uncertain malignant potential. Histological findings with immunomarkers led to the final diagnosis of undifferentiated pleomorphic sarcoma. This case highlights a rare and interesting variant of primary breast sarcoma and the important role of immunohistochemistry in defining histological type and differential diagnosis. Hence, undifferentiated pleomorphic sarcoma has been a diagnosis of exclusion performed through sampling and critical use of ancillary diagnostic techniques.

  12. The role of imaging in the evaluation of extraskeletal Ewing’s sarcoma

    Directory of Open Access Journals (Sweden)

    Pieter Barnardt

    2013-03-01

    Full Text Available Primary bone tumours account for 5% of all adolescent and child cancers. When Ewing’s sarcoma arises in soft tissue rather than bone, it is referred to as extraskeletal Ewing’s sarcoma (ESS. Computed tomography (CT scanning and magnetic resonance imaging (MRI features of Ewing’s sarcoma are non-specific, and a radiological differential diagnosis should be considered. Ewing’s sarcoma is confirmed by features on histological analysis. In young people who present with soft-tissue tumours, ESS should be considered. In the management of patients with tumours, imaging techniques are useful for biopsy guidance, evaluating the possibility of resection, and tumour response to treatment.

  13. Extraskeletal Ewing's Sarcoma: insight into a ten years follow-up.

    Science.gov (United States)

    Zitelli, A; Manfredelli, S; Brunotti, G; Marcantonio, M; Pontone, S; Angelici, A

    2013-01-01

    Extraskeletal Ewing's sarcoma is a rare malignant soft tissue tumor, classified within the Ewing's Sarcoma Family Tumors. While the classical Ewing's Sarcoma affects mainly the bone during youth, the Extraskeletal histotype differs for age incidence, primary location and prognosis. Peak incidence and typical location are during adolescence and in the extremities respectively. We report a 30 year old woman case with a positive outcome after ten years from first diagnosis of Extraskeletal Ewing's sarcoma. Treatment was achieved through surgical resection plus adjuvant chemoradiotherapy derived from EW93 and IRS III trials. Conclusion. Our report represents an unusual case due to age of presentation, neoplasm location and long survival reached. In last decades several trials results demonstrated that long survival could be achieved by combined surgery and adjuvant multi-drug treatment.

  14. Anterior mediastinal synovial sarcoma: A case report and literature review

    Directory of Open Access Journals (Sweden)

    Wen-xiang YUE

    2015-01-01

    Full Text Available Objective To study the clinical manifestations, pathologic features, diagnosis, treatment and prognosis of primary synovial sarcoma in the anterior mediastinum. Methods A case of primary synovial sarcoma in the anterior mediastinum was reported. Clinical features, imaging manifestations, pathology features and therapeutic effect were analysed and the relevant literature was reviewed. Results A 48-year-male patient was admitted with complaint of right chest pain for 4 days. Chest computerized tomography revealed a large mass located at the right anterior mediastinum, and it was primarily diagnosed as invasive thymoma. Pathological examination by CT-guided percutaneous needle biopsy manifested that, under microscope, the tumor cells were short and spindle in shape forming a nest structure, suggested it was a thymoma. The patient then underwent resection of thymoma with removal of fat and connective tissue in the anterior mediastinum. During the operation the size of the tumor was 15cm×15cm×10cm, being located at the anterior mediastinum, and it tended to bleed. The diagnosis of primary monophasic synovial sarcoma in the mediastinum was confirmed by postoperative/pathology examination. Immunohistochemistry staining showed that the tumor cells were positive for the markers Bcl-2 and EMA, but negative for the markers CK (pan and S100. The patient suffered from local recurrence with metastases to lung 4 months after surgery. The patient received 2 chemotherapeutic courses with ifosfamide, epirubicin and cisplatin. He died 6 months after surgery. Conclusion Primary synovial sarcoma in the anterior mediastinum is an extremely rare and highly malignant tumor with poor prognosis. The diagnosis depends on the pathological features, immunohistochemistry and RT-PCR. Radical resection combined with comprehensive treatment may improve the survival rate. DOI: 10.11855/j.issn.0577-7402.2014.12.12

  15. Pleural effusions in the postpartum period

    International Nuclear Information System (INIS)

    Stark, P.; Pollack, M.S.

    1986-01-01

    We report on the high frequency of pleural effusions in the immediate postpartum period. Forty-four out of 45 women who examined within 24-48 h after delivery showed evidence of pleural fluid. Under these circumstances, this pleural abnormality should not be considered an indicator of serious cardiopulmonary disease. (orig.) [de

  16. Recurrent Pericardial Effusion Associated with Hypothyroidism in ...

    African Journals Online (AJOL)

    Background: The complex of Down Syndromehypothyroidism-pericardial effusion is largely unreported in sub-Sahara. Objective: To present and highlight an unusual manifestation of hypothyroidism. Methods: A 16-year-old girl with confirmed Down Syndrome presented with complaints of generalised body swelling of eight ...

  17. Diaphragmatic Hernia Masquerading as Pleural Effusion | Nalladaru ...

    African Journals Online (AJOL)

    Rupture of the diaphragm is almost always due to major trauma. We present here an unusual and rare case of late presentation of diaphragmatic hernia after an innocuous injury. The patient was initially misdiagnosed as a left pleural effusion on the basis of chest X.ray and ultrasound findings. Finally, the diagnosis was ...

  18. Diaphragmatic Hernia Masquerading as Pleural Effusion

    African Journals Online (AJOL)

    As many as 30% of diaphragmatic hernias present late.[1] We present an unusual case report of a late presentation of isolated diaphragmatic injury, after apparently minor blunt trauma, which was initially misdiagnosed as pleural effusion. CASE REPORT. A 40-year-old man slipped and fell against the edge of his bath tub.

  19. A large pericardial effusion and bilateral pleural effusions as the initial manifestations of Familial Mediterranean Fever

    OpenAIRE

    Schembri, Emma Louise; Mifsud, Simon; Cassar Demarco, Daniela; Coleiro, Bernard; Mallia, Carmel

    2015-01-01

    Familial Mediterranean Fever (FMF) is a condition characterized by recurrent febrile poly-serositis. Typical presentations of the disease include episodes of fever, abdominal pain and joint pains. Chest pain is a less common presentation. We report a case of FMF which presented with a large pericardial effusion and bilateral pleural effusions in a lady who had no positive family history and negative genetic testing.

  20. Functional signatures of radio-induction in sarcomas developing in the radiation field after radiotherapy

    International Nuclear Information System (INIS)

    Hadj-Hamou, N.S.

    2010-01-01

    Radiotherapy plays an important role in the treatment of cancers. However, exposure to ionizing radiation is a well-known risk factor for secondary cancer development. Currently, rigorous defined scientific criteria are lacking to establish if an individual tumor has a radiation-induced or a sporadic origin. The main aim of my thesis program was to identify a transcriptome signature of the ionizing radiation effects in radiation-induced cancers. The series of cancers used in this study is composed of sarcomas developing in the irradiation field of patients treated by radiotherapy for a primary cancer. Strict selection criteria (histology different from the primary cancer, latency longer than 5 years) were used to establish with a high probability the sarcomas-radiation induced origin. Their transcriptomes were compared with those from patients without irradiation history. A method of classification adapted to small series was used for the study of all the 60 collected sarcomas (34 radiation-induced and 26 sporadic). A learning set composed of 24 sarcomas from known aetiology allowed us to determine a signature of 135 genes discriminating the sarcomas according to their aetiology. The signature classified 86% of the remaining sarcomas as a function of their aetiology with an accuracy of 97%. The analysis of the genes-function shows that the radiation-induced sarcomas suffered the effects of a chronic oxidative stress mainly generated by mitochondrial dysfunctions. This study shows, for the first time, that it is possible to diagnose, at the case by case level, radiation-induced sarcomas on a rigorous scientific basis. (author)

  1. Bilateral clear cell sarcoma of the kidney

    International Nuclear Information System (INIS)

    Zekri, W.; Yehia, D.; Alfaar, A.S.; Elshafie, M.M.; Younes, A.A.; Zaghloul, M.S.; El-Kinaai, N.; Taha, H.; Refaat, A.; Zekri, W.; Elshafie, M.M.; Zaghloul, M.S.; Taha, H.; Refaat, A.; Younes, A.A.; Alfaar, A.S.; Yehia, D.

    2015-01-01

    Clear cell sarcoma of the kidney (CCSK) accounts for 2-5% of all pediatric renal malignancies, and is known for its propensity to metastasize to bone and other sites. We are reporting two cases with bilateral CCSK that were diagnosed at our institution. One patient initially presented with bilateral renal masses, as well as pulmonary, hepatic and bone metastasis; while other present only with bilateral masses with no evident distant metastasis. Both patients received aggressive neo-adjuvant chemotherapy to decrease tumor size. One patient completed his designated treatment and initially showed complete remission (CR); eventually suffering from relapse. The other patient’s tumor progressed during the course of chemotherapy. Both cases manifested brain dissemination at the time of relapse or progression. This emphasizes the importance of staging stratification in CCSK. This also illustrates CCSK’s ability to metastasize to bone and other sites including the brain (a primary relapse site in our cases)

  2. Prevalence and Impact on Weaning of Pleural Effusion at the Time of Liberation from Mechanical Ventilation: A Multicenter Prospective Observational Study.

    Science.gov (United States)

    Dres, Martin; Roux, Damien; Pham, Tài; Beurton, Alexandra; Ricard, Jean-Damien; Fartoukh, Muriel; Demoule, Alexandre

    2017-06-01

    Pleural effusion is frequent in intensive care unit patients, but its impact on the outcome of weaning remains unknown. In a prospective study performed in three intensive care units, pleural ultrasound was performed at the first spontaneous breathing trial to detect and quantify pleural effusion (small, moderate, and large). Weaning failure was defined by a failed spontaneous breathing trial and/or extubation requiring any form of ventilatory support within 48 h. The primary endpoint was the prevalence of pleural effusion according to weaning outcome. Pleural effusion was detected in 51 of 136 (37%) patients and was quantified as moderate to large in 18 (13%) patients. As compared to patients with no or small pleural effusion, their counterparts were more likely to have chronic renal failure (39 vs. 7%; P = 0.01), shock as the primary reason for admission (44 vs. 19%; P = 0.02), and a greater weight gain (+4 [0 to 7] kg vs. 0 [-1 to 5] kg; P = 0.02). The prevalence of pleural effusion was similar in weaning success and weaning failure patients (odds ratio, 1.23; 95% CI, 0.61 to 2.49; P = 0.56), as was the prevalence of moderate to large pleural effusion (odds ratio, 0.89; 95% CI, 0.33 to 2.41; P = 1.00). Duration of mechanical ventilation and intensive care unit length of stay were similar between patients with no or small pleural effusion and those with moderate to large pleural effusion. Significant pleural effusion was observed in 13% of patients at the time of liberation from mechanical ventilation and was not associated with an alteration of weaning outcome. (ANESTHESIOLOGY 2017; 126:1107-15).

  3. Understanding the biology of bone sarcoma from early initiating events through late events in metastasis and disease progression.

    Directory of Open Access Journals (Sweden)

    Limin eZhu

    2013-09-01

    Full Text Available The two most common primary bone malignancies, osteosarcoma and Ewing sarcoma, are both aggressive, highly metastatic cancers that most often strike teens, though both can be found in younger children and adults. Despite distinct origins and pathogenesis, both diseases share several mechanisms of progression and metastasis, including neovascularization, invasion, anoikis resistance, chemoresistance and evasion of the immune response. Some of these processes are well-studies in more common carcinoma models, and the observation from adult diseases may be readily applied to pediatric bone sarcomas. Neovascularization, which includes angiogenesis and vasculogenesis, is a clear example of a process that is likely to be similar between carcinomas and sarcomas, since the responding cells are the same in each case. Chemoresistance mechanisms also may be similar between other cancers and the bone sarcomas. Since osteosarcoma and Ewing sarcoma are mesenchymal in origin, the process of epithelial-to-mesenchymal transformation is largely absent in bone sarcomas, necessitating different approaches to study progression and metastasis in these diseases. One process that is less well-studied in bone sarcomas is dormancy, which allows micrometastatic disease to remain viable but not growing in distant sites – typically the lungs – for months or years before renewing growth to become overt metastatic disease. By understanding the basic biology of these processes, novel therapeutic strategies may be developed that could improve survival in children with osteosarcoma or Ewing sarcoma.

  4. Evaluation of pleural and pericardial effusions by magnetic resonance imaging

    Energy Technology Data Exchange (ETDEWEB)

    Tscholakoff, D.; Sechtem, U.; De Geer, G.; Schmidt, H.; Higgins, C.B.

    1987-08-01

    MR examinations of 36 patients with pleural and/or pericardial effusions were retrospectively evaluated. The purpose of this study was to determine of MR imaging is capable of differentiating between pleural and pericardial effusions of different compositions using standard electrocardiogram (ECG)-gated and nongated spin echo pulse sequences. Additional data was obtained from experimental pleural effusions in 10 dogs. The results of this study indicate that old haemorhages into the pleural or pericardial space can be differentiated from other pleural or pericardial effusions. However, further differentiation between transudates, exudates and sanguinous effusions is not possible on MR images acquired with standard spin echo pulse sequences. (orig./MG)

  5. Pathogenesis of pleural effusion in carcinoma of the breast

    International Nuclear Information System (INIS)

    Weichselbaum, R.; Marck, A.; Hellman, S.

    1977-01-01

    Three hundred and fifty-two patients with carcinoma of the breast treated postoperatively with radiotherapy were analyzed for laterality and incidence of pleural effusion. A statistically significant increase in ipsilateral pleural effusions was noted in the entire group (p less than 0.0001). Thirty-three percent of the patients with chest wall recurrences developed ipsilateral effusions while only 4.5 percent of those who did not develop chest wall recurrences suffered ipsilateral effusions (p less than 0.001). The role of the chest wall lymphatics in the development of malignant pleural effusions is discussed

  6. Utility of semi-rigid thoracoscopy in undiagnosed exudative pleural effusion.

    Science.gov (United States)

    Nattusamy, Loganathan; Madan, Karan; Mohan, Anant; Hadda, Vijay; Jain, Deepali; Madan, Neha Kawatra; Arava, Sudheer; Khilnani, Gopi C; Guleria, Randeep

    2015-01-01

    Semi-rigid thoracoscopy is a safe and efficacious procedure in patients with undiagnosed pleural effusion. Literature on its utility from developing countries is limited. We herein describe our initial experience on the utility of semi-rigid thoracoscopy from a tertiary care teaching and referral center in north India. We also perform a systematic review of studies reporting the utility of semi-rigid thoracoscopy from India. The primary objective was to evaluate the diagnostic utility of semi-rigid thoracoscopy in patients with undiagnosed exudative pleural effusion. Semi-rigid thoracoscopy was performed under local anesthesia and conscious sedation in the bronchoscopy suite. A total of 48 patients underwent semi-rigid thoracoscopy between August 2012 and December 2013 for undiagnosed pleural effusion. Mean age was 50.9 ± 14.1 years (range: 17-78 years). Pre-procedure clinico-radiological diagnoses were malignant pleural effusion [36 patients (75%)], tuberculosis (TB) [10 (20.83%) patients], and empyema [2 patients (4.17%)]. Patients with empyema underwent the procedure for pleural biopsy, optimal placement of intercostal tube and adhesiolysis. Thoracoscopic pleural biopsy diagnosed pleural malignancy in 30 (62.5%) patients and TB in 2 (4.17%) patients. Fourteen (29.17%) patients were diagnosed with non-specific pleuritis and normal pleura was diagnosed on a pleural biopsy in 2 (4.17%) patients. Overall, a definitive diagnosis of either pleural malignancy or TB was obtained in 32 (66.7%) patients. Combined overall sensitivity, specificity, positive predictive value and negative predictive value of thoracoscopic pleural biopsy for malignant pleural effusion were 96.77%, 100%, 100% and 66.67%, respectively. There was no procedure-related mortality. On performing a systematic review of literature, four studies on semi-rigid thoracoscopy from India were identified. Semi-rigid thoracoscopy is a safe and efficacious procedure in patients with undiagnosed exudative

  7. [Sarcoidosis related pleural effusion: 6 case reports and literatures review].

    Science.gov (United States)

    Wang, Feng; Tong, Zhaohui; Wang, Zhen; Wang, Xiaojuan; Xu, Lili

    2015-02-01

    To summarize the clinical features and the diagnosis-treatment points of sarcoidosis related pleural effusion. Six typical sarcoidosis related pleural effusion cases with pathological evidence were reviewed, and the clinical data of these cases were retrospectively analyzed and the related literatures were reviewed. The literature review was carried out respectively with "sarcoidosis", "pleural disease" and "pleural effusion" as the keywords in CNKI and PubMed database by January 2014. Six cases, including 1 male and 5 females, with sarcoidosis related pleural effusions were reported. 3 cases had bilateral effusions, 2 cases had left effusion and 1 case had right effusion. The pleural effusion routine test had a low specificity, which demonstrated that the fluid was exudate and consisted with large number of lymphocytes. 3 of these cases were diagnosed by medical thoracoscopy. Medical thoracoscopy revealed that pleural involvement was variable with multiple nodulespresent in some cases and subtle change in others. A total of 28 literatures and 92 cases with pleural involvement in sarcoidosis were retrieved from CNKI and PubMed database (time range: 2004.1-2014.1), including 59 cases of pleural effusion, 29 cases of pleural thickening, 3 cases of pneumothorax and 1 case of nodules in pleura. Pleural involvement in sarcoidosis was often misdiagnosed or mistreated as tuberculous pleurisy because the routine tests regarding pleural effusion usually had a low specificity. Medical thoracoscopy could provide clinicians with important clues to assist differentiation of the cause for non-conclusive pleural effusion in this situation.

  8. Profiling pleural effusion cells by a diffraction imaging method

    Science.gov (United States)

    Al-Qaysi, Safaa; Hong, Heng; Wen, Yuhua; Lu, Jun Q.; Feng, Yuanming; Hu, Xin-Hua

    2018-02-01

    Assay of cells in pleural effusion (PE) is an important means of disease diagnosis. Conventional cytology of effusion samples, however, has low sensitivity and depends heavily on the expertise of cytopathologists. We applied a polarization diffraction imaging flow cytometry method on effusion cells to investigate their features. Diffraction imaging of the PE cell samples has been performed on 6000 to 12000 cells for each effusion cell sample of three patients. After prescreening to remove images by cellular debris and aggregated non-cellular particles, the image textures were extracted with a gray level co-occurrence matrix (GLCM) algorithm. The distribution of the imaged cells in the GLCM parameters space was analyzed by a Gaussian Mixture Model (GMM) to determine the number of clusters among the effusion cells. These results yield insight on textural features of diffraction images and related cellular morphology in effusion samples and can be used toward the development of a label-free method for effusion cells assay.

  9. Procholecystokinin as marker of human Ewing sarcomas

    DEFF Research Database (Denmark)

    Reubi, Jean Claude; Koefoed, Pernille; Hansen, Thomas von O

    2004-01-01

    PURPOSE: Ewing sarcoma is a rapidly growing mesenchymal tumor in young adults. Although it was shown previously to express the cholecystokinin (CCK) gene, it is unknown whether CCK gene expression is detectable at protein level in Ewing sarcoma tumor cell lines, in tumor tissue, and in plasma from...... Ewing sarcoma patients, and, if so, whether CCK peptides might play a role as tumor markers. EXPERIMENTAL DESIGN: CCK gene expression was evaluated with in situ hybridization or reverse transcription-PCR in tumor tissue. CCK precursors and bioactive CCK were measured with specific RIAs in tumor tissue......, in cell culture medium, and in plasma of Ewing sarcoma patients before and after chemotherapy as well as after tumor recurrence. RESULTS: CCK mRNA was identified in 12 Ewing sarcoma biopsies sampled in two series and in four Ewing sarcoma cell lines but not in unrelated neoplasia. Immunoreactive pro...

  10. Management strategies of sarcomas of the ankle and foot

    International Nuclear Information System (INIS)

    Douglas, R.M.; Mankin, H.J.; Jennings, L.C.; Gebhardt, M.C.; Harmon, D.; Ancukiewicz, M.; Suit, H.D.; Spiro, Ira J.

    1997-01-01

    Purpose: To evaluate the management and outcomes of 40 patients treated at the Massachusetts General Hospital between 1974 and September 1996 for soft tissue sarcomas of the foot or ankle. Materials and Methods: Seventy seven percent of patients had AJCC stage II or III disease. Primary disease was treated in 28 patients while recurrent disease was addressed in 12. The mean tumor size was 4.6 cm with a median tumor size of 4.0 cm, (range 1 to 15 cm). Seventeen patients were treated with surgery followed by radiation therapy for primary or recurrent disease. Preoperative radiation therapy was employed in 15 patients, 5 of whom received additional postoperative radiation therapy for close or positive margins. Six patients received radiation treatment alone. Amputations were performed in 5 patients. Two amputations were performed due to inadequate surgical margins obtained at the time of resection. Three amputations were performed due to local failure. The median preoperative radiation dose was 48 Gy. The median total dose was 59 Gy (range 22 Gy to 70 Gy). Chemotherapy was incorporated in the therapy of 6 patients. Results: After a mean follow-up of 4.9 years, 28 (70%) patients remain without evidence of disease. Four patients have died of disease. Five patients have died of intercurrent disease or another cancer. Three patients remain alive with disease. There were 5 isolated local failures, 4 distant failures and 1 combined local and distant failure. Of the 5 patients with isolated local failures, 3 were treated with radiation alone. The most common site of distant failure was the lung. Actuarial local control rates are 80% at 5 and 10 years in this population. Actuarial overall survival at 5 and 10 years are 83% and 69% respectively. Wound healing difficulties occurred in 13 patients. Seven patients suffered a wound dehiscence. Five patients required therapy for wound dehiscence and infection and one patient was treated for wound infection only. In six patients

  11. Posterior mediastinal biphasic synovial sarcoma in a 12 year-old boy: A case report and review of literature

    Directory of Open Access Journals (Sweden)

    Pal Madhumay

    2010-01-01

    Full Text Available We report a case of biphasic synovial sarcoma of the mediastinum, a very rare tumor, in a 12-year-old boy with left-sided chest pain of 3 years duration at presentation. Chest X-ray showed left-sided opacity with loss of cardiac silhouette and the mediastinum deviated to the opposite side. Computed tomography (CT of thorax showed left-sided posterior mediastinal mass with left-sided pleural effusion and pleural thickening. CT guided fine needle aspiration cytology (FNAC from the mass reported it as spindle cell variant of adenocarcinoma. Ultrasonography (USG of the whole abdomen revealed no abnormality. The mediastinal tumor was resected by left thoracotomy and histopathological report confirmed it to be a biphasic synovial sarcoma with capsule invasion at places.

  12. Intraneural synovial sarcoma of the median nerve

    Directory of Open Access Journals (Sweden)

    Rahul Kasukurthi

    2010-06-01

    Full Text Available Synovial sarcomas are soft-tissue malignancies with a poor prognosis and propensity for distant metastases. Although originally believed to arise from the synovium, these tumors have been found to occur anywhere in the body. We report a rare case of synovial sarcoma arising from the median nerve. To our knowledge, this is the twelfth reported case of intraneural synovial sarcoma, and only the fourth arising from the median nerve. Because the diagnosis may not be apparent until after pathological examination of the surgical speci­men, synovial sarcoma should be kept in mind when dealing with what may seem like a benign nerve tumor.

  13. Adrenal Ewing's Sarcoma in an Elderly Man.

    Science.gov (United States)

    Toda, Kazuyoshi; Ishii, Sumiyasu; Yasuoka, Hidetoshi; Nishioka, Masaki; Kobayashi, Takayuki; Horiguchi, Kazuhiko; Tomaru, Takuya; Ozawa, Atsushi; Shibusawa, Nobuyuki; Satoh, Tetsurou; Koshi, Hiromi; Segawa, Atsuki; Shimizu, Shin-Ichi; Oyama, Tetsunari; Yamada, Masanobu

    2018-02-15

    Ewing's sarcoma usually arises in the bones of children and adolescents. We herein report a 74-year-old man with Ewing's sarcoma in the adrenal gland. The diagnosis was confirmed by a genetic test, pathological studies, and several imaging studies. He already had multiple liver metastases when he was transferred to our hospital and died on the 37th day. The diagnosis was further confirmed by autopsy studies. Adrenal Ewing's sarcoma is very rare, and our patient was older than other reported cases. Ewing's sarcoma should be considered even in elderly patients with adrenal tumors.

  14. Double Feature: Carcinoma and Sarcoma Present in a Single Breast Tumor

    Directory of Open Access Journals (Sweden)

    Catherine M. Stefaniuk

    2012-01-01

    Full Text Available Introduction. Primary breast sarcomas (PBSs are rare nonepithelial breast tumors compromised of mesenchymal mammary tissue. Although its rare nature has made the best mode of PBS treatment difficult to determine, it seems better to treat it more like a sarcoma creating clear negative margins verses breast carcinoma utilizing lumpectomy, partial mastectomy, and total mastectomy. Case. A 47-year-old obese Caucasian postmenopausal female G2P2 presents with a breast lump demonstrating a histological sample with a biphasic pattern consistent with both ductal carcinoma containing typical malignant epithelial cells and sarcomatous differentiation of carcinosarcoma. Conclusion. Carcinosarcoma is a rare breast malignancy. Sarcomas of the breast tend to be negative for estrogen receptor and lack known risk factors. Current recommended treatment is to treat breast sarcomas like other soft tissue sarcomas by performing wide local excision instead of partial mastectomy. Antiestrogens and other chemotherapeutic agents typically used in breast epithelial malignancies are not recommended since these sarcomas tend to be negative with these receptors.

  15. Hypoxia-Inducible Factors: Mediators of Cancer Progression; Prognostic and Therapeutic Targets in Soft Tissue Sarcomas

    International Nuclear Information System (INIS)

    Sadri, Navid; Zhang, Paul J.

    2013-01-01

    Soft-tissue sarcomas remain aggressive tumors that result in death in greater than a third of patients due to either loco-regional recurrence or distant metastasis. Surgical resection remains the main choice of treatment for soft tissue sarcomas with pre- and/or post-operational radiation and neoadjuvant chemotherapy employed in more advanced stage disease. However, in recent decades, there has been little progress in the average five-year survival for the majority of patients with high-grade soft tissue sarcomas, highlighting the need for improved targeted therapeutic agents. Clinical and preclinical studies demonstrate that tumor hypoxia and up-regulation of hypoxia-inducible factors (HIFs) is associated with decreased survival, increased metastasis, and resistance to therapy in soft tissue sarcomas. HIF-mediated gene expression regulates many critical aspects of tumor biology, including cell survival, metabolic programming, angiogenesis, metastasis, and therapy resistance. In this review, we discuss HIFs and HIF-mediated genes as potential prognostic markers and therapeutic targets in sarcomas. Many pharmacological agents targeting hypoxia-related pathways are in development that may hold therapeutic potential for treating both primary and metastatic sarcomas that demonstrate increased HIF expression

  16. Osteogenic sarcoma of the prostate

    Energy Technology Data Exchange (ETDEWEB)

    Nishiyama, Tsutomu; Terunuma, Masahiro [Koseiren Nagaoka Chuo General Hospital, Niigata (Japan); Ikarashi, Toshihiko; Ishizaki, Satoshi

    2001-04-01

    A 76-year-old man was treated with bilateral orchiectomy, estramustine phosphate and pelvic irradiation for prostate cancer. Osteogenic sarcoma of the prostate developed 18 months after the treatment. Postmortem examination revealed that the tumor was 8 cm in diameter and had infiltrated into the bladder and rectal walls and had resulted in peritoneal dissemination. There was no distant metastasis. Macroscopically, the tumor was ashen, firm and relatively homogenous and diffusely spread. Histologically, it was composed of spindle and pleomorphic cells, which were making osteoid with calcification. There was no ordinary tubular formation as shown in adenocarcinoma of the prostate. No positive immunostaining for prostate-specific antigen, epithelial membrane antigen and cytokeratin (AE-1, AE-3) were confirmed. Positive immunostaining for nonepithelial marker vimentin was confirmed. The ultimate diagnosis was osteogenic sarcoma of the prostate. (author)

  17. Irradiation for conjunctival granulocytic sarcoma

    International Nuclear Information System (INIS)

    Fleckenstein, K.; Geinitz, H.; Grosu, A.; Molls, M.; Goetze, K.; Werner, M.

    2003-01-01

    Case History and Findings: A 73-year-old woman with a history of myeloproliferative syndrome (MPS) presented with bilateral chemosis, redness and burning of the eyes. The ocular motility was severely impaired. Ophthalmological examination revealed markedly distended conjunctivas on both sides. Biopsy disclosed conjunctival granulocytic sarcoma as an initial symptom of acute myelogenous leukemia (AML). Diagnosis was confirmed by peripheral blood smear and bone marrow aspiration. Treatment and Outcome: The orbital tumor disappeared completely after local external beam irradiation with a total dose of 30 Gy and no further orbital recurrence occurred. With chemotherapy following irradiation transient hematological remission was achieved. 5 months after diagnosis the patient died of respiratory failure following atypical pneumonia as a consequence of her underlying disorder. Conclusion: Detection of orbital granulocytic sarcoma, even in the absence of typical leukemic symptoms is of practical importance, because treatment with irradiation can lead to stabilization or improvement in the patient's vision. (orig.)

  18. Recursive partitioning analysis (RPA) classification predicts survival in patients with brain metastases from sarcoma.

    Science.gov (United States)

    Grossman, Rachel; Ram, Zvi

    2014-12-01

    Sarcoma rarely metastasizes to the brain, and there are no specific treatment guidelines for these tumors. The recursive partitioning analysis (RPA) classification is a well-established prognostic scale used in many malignancies. In this study we assessed the clinical characteristics of metastatic sarcoma to the brain and the validity of the RPA classification system in a subset of 21 patients who underwent surgical resection of metastatic sarcoma to the brain We retrospectively analyzed the medical, radiological, surgical, pathological, and follow-up clinical records of 21 patients who were operated for metastatic sarcoma to the brain between 1996 and 2012. Gliosarcomas, sarcomas of the head and neck with local extension into the brain, and metastatic sarcomas to the spine were excluded from this reported series. The patients' mean age was 49.6 ± 14.2 years (range, 25-75 years) at the time of diagnosis. Sixteen patients had a known history of systemic sarcoma, mostly in the extremities, and had previously received systemic chemotherapy and radiation therapy for their primary tumor. The mean maximal tumor diameter in the brain was 4.9 ± 1.7 cm (range 1.7-7.2 cm). The group's median preoperative Karnofsky Performance Scale was 80, with 14 patients presenting with Karnofsky Performance Scale of 70 or greater. The median overall survival was 7 months (range 0.2-204 months). The median survival time stratified by the Radiation Therapy Oncology Group RPA classes were 31, 7, and 2 months for RPA class I, II, and III, respectively (P = 0.0001). This analysis is the first to support the prognostic utility of the Radiation Therapy Oncology Group RPA classification for sarcoma brain metastases and may be used as a treatment guideline tool in this rare disease. Copyright © 2014 Elsevier Inc. All rights reserved.

  19. Left atrial spindle cell sarcoma – Case report

    Directory of Open Access Journals (Sweden)

    Nihar Mehta

    2012-07-01

    Full Text Available Primary spindle cell sarcoma of the left atrium is an extremely rare tumour. Surgical excision is the mainstay of treatment since it responds poorly to chemotherapy or radiotherapy. In spite of all the treatment, the prognosis remains poor due to inadvertent delay in diagnosis, few therapeutic options and propensity to metastasize. We present a 47-year-old male who underwent a surgical excision of a left atrial mass in February 2010. It was proved to be a high-grade spindle cell sarcoma on histopathology. He presented again in October 2010 with recurrence of the tumour for which he was re-operated. However, the tumour recurred again within one month, to which the patient succumbed.

  20. Soft-tissue sarcomas. Current aspects of diagnosis and therapy

    International Nuclear Information System (INIS)

    Hohenberger, P.

    1996-01-01

    The decisive factor for promising therapy of soft-tissue sarcomas is primary therapy provided in an experienced tumour unit. This centre must offer the entire spectrum of surgery (vascular, reconstruction and orthopaedic surgery) leading into an interdisciplinary treatment regimen. Initially, MRI would appear to be sufficient for diagnosis. On the other hand, the presence of an experienced pathologist conversant with all means of cytogenetic tumour classification is essential. For interdisciplinary therapy, a radiotherapist with hyperthermia equipment, nuclear medicine specialists and medical oncologists are indispensable. All personnel must be familiar with the special problems associated with sarcomas. The current trend is indeed toward a select number of centres where such skills are focused. (orig.) [de

  1. Lipo sarcoma in small intestine

    International Nuclear Information System (INIS)

    Rodriguez Iglesias, J.; Pineyro Gutierrez, A.; Taroco Medeiros, L.; Fein Kolodny, C.; Navarrete Pedocchi, H.

    1987-01-01

    A case is presented by primitive liposarcoma in small intestine , an extensive bibliographical review foreigner and national in this case. It detach the exceptional of the intestinal topography of the liposarcomas; and making stress in the relative value of the computerized tomography and ultrasonography in the diagnose of the small intestine tumors . As well as in the sarcomas of another topography, chemo and radiotherapy associated to the exeresis surgery, it can be of benefit [es

  2. Synovial sarcoma of the foot

    International Nuclear Information System (INIS)

    Beus, J.; Kreitner, K.F.; Rompe, J.D.; Riehle, H.M.

    1996-01-01

    The case of a 29 year-old female patient who had experienced pain in the right midfoot for 5 years which was diagnosed as a degenerative or rheumatic change and treated by physiotherapy and medication. By means of magnetic resonance imaging we identified a soft-tissue tumor of the midfoot. Histology provided the findings of a monophasic fibrous synovial sarcoma. The case history is reported together with a presentation of the disease and its radiological diagnosis. (orig.) [de

  3. Polycystic liver disease with right pleural effusion

    Science.gov (United States)

    Anggreini, A. Y.; Dairi, L. B.

    2018-03-01

    Polycystic liver disease (PCLD) is a condition in which multiple cysts form in the hepatic parenchyma. The polycystic liver disease is also an autosomal dominant disorder (ADPLD) caused by a mutation in a gene that encodes a protein hepatocystin. PCLD has a prevalence count of 1:200,000 people in the people of America. PCLD occurs ± 24% of patients in the third decade of age to 80% by the sixth decade. Women tend to get larger cysts and more and correlated with the number of pregnancies. The following case report of a woman, 51-years-old who was treated at Haji Adam Malik hospital Medan with a diagnosis of polycystic liver disease with right pleural effusion. Some literature has reported complications of the polycystic liver disease but rarely reported with pleural effusion presentation. The patient had already undergone a puncture of pleural fluid and after three weeks of treatment condition of the patient improved and permitted to be outgoing patient.

  4. Definitive Brachytherapy for Kaposi's Sarcoma

    International Nuclear Information System (INIS)

    Williams, A.; Ezzell, G.; Zalupski, M.; Fontanesi, J.

    1996-01-01

    Purpose: To assess the efficacy and possible complications in patients diagnosed with Kaposi's sarcoma and treated with definitive brachytherapy. Methods and Materials: Between January, 1995 and December, 1995, four patients with Kaposi's sarcoma (KS) were treated with brachytherapy. Three patients, all with positive HIV status were treated using Iridium 192 (Ir-192) sources via a high-dose rate remote afterloader. One patient with endemic KS was treated using the application of catheters loaded with Californium 252. Eight sites were treated and included scalp, feet, nose, penis, hand, neck, and back. Dose rate for Ir-192 was 330cGy/fx to a total dose of 990cGy. The Californium was delivered as 100nGy/b.i.d. to a total dose of 900nGy. Follow-up as ranged from 2-6 months. Results: All four patients remain alive. Seven of eight sites have had complete clinical response and each patient has reported durable pain relief that has not subsided through last follow-up of 1/96. Two of eight sites, both treated with surface mold technique with Californium 252 developed moist desquamation. The remaining six sites did not demonstrate significant toxicity. Conclusion: Brachytherapy can offer Kaposi's sarcoma patients results that are equivalent to external beam radiation therapy, with minimal complications, a shorter treatment time and potential cost effectiveness

  5. Extraskeletal presentation of Ewing's Sarcoma.

    Science.gov (United States)

    Mangual, Danny; Bisbal-Matos, Luis A; Jiménez-Lee, Ricardo; Vélez, Román; Noy, Miguel

    2018-03-01

    The case of a 27-year-old Hispanic female who presented with an occipito-parietal tumor after suffering trauma to the area. A physical examination revealed no tenderness to palpation and with evidence of healing ulcerations. The biopsy was consistent with a synovial sarcoma. A wide excision of the mass (15cm x 14cm x 6cm) followed by a pericranial flap was performed. A follow-up CT showed recurrence involving the parietal sagittal sinus. After a second biopsy the mass was determined to be a small-cell sarcoma, consistent with Ewing's sarcoma. Chemotherapy included 8 cycles of doxorubicin, vincristine, and cyclophosphamide, with alternating cycles of etoposide and ifosfamide. A year later, a second wide excision of the mass was performed, followed by bilaminate skin substitute and skin graft placement for reconstruction of the soft-tissue defect. After chemotherapy, a follow-up PET scan showed no signs of re-uptake in any soft tissue or skeletal structures. After 2 years, the patient remains in complete remission.

  6. Effusion in magnetic resonance imaging of the temporomandibular joint

    Energy Technology Data Exchange (ETDEWEB)

    Nah, Kyung Soo [Pusan National University College of Medicine, Busan (Korea, Republic of)

    2003-03-15

    The purpose of this study was to investigate the distribution and frequency of temporomandibular joint (TMJ) effusion in magnetic resonance (MR) images of patients with disc displacements. On T2 weighted MR images of 148 TMJs taken from 74 patients presenting with TMJ pain and dysfunction, we assessed the cases showing TMJ effusion, defined as an amount of fluid that exceeded the maximum amount seen in a control group of asymptomatic volunteers. The amount of TMJ fluid was graded as: I (none or minimal), II (moderate), III (marked), and IV (extensive), according to a standard set by a reference. Disc displacement categories were also recorded. Of the 148 TMJs examined in this study, 52 joints (35.1%) presented with joint effusion, 24 (16.2%) showing bilateral joint effusion. 38 joints showed upper joint space effusion, and 3 showed lower joint space effusion, and 11 showed both upper and lower joint space effusion. 96 joints (64.9%) had grade I joint fluid, 27 (18.2%) grade II, 15 (10.1%) grade III, and 10 (6.8%) grade IV. 80.0% of the joints presenting with grade IV effusion showed disc displacement without reduction. Joint effusion was found not only in upper, but also in lower joint spaces. The higher the effusion grade, the greater the frequency of disc displacement without reduction.

  7. Bicavitary effusion secondary to liver lobe torsion in a dog

    Directory of Open Access Journals (Sweden)

    Khan Z

    2016-04-01

    Full Text Available Zaheda Khan,1 Kathryn Gates,2 Stephen A Simpson,31Emergency and Critical Care, Animal Specialty and Emergency Center, Los Angeles, CA, 2Emergency and Critical Care, Advanced Critical Care, Emergency and Specialty Services, Culver City, CA 3Emergency and Critical Care, Southern California Veterinary Specialty Hospital, Irvine, CA, USA Abstract: We described the diagnosis and successful treatment of pleural and peritoneal effusion secondary to liver lobe torsion in a dog. A 12-year-old female spayed Borzoi dog was referred for heart failure. Emergency room thoracic and abdominal ultrasound showed a large volume of pleural effusion with mild peritoneal effusion and an abdominal mass. Pleural fluid analysis classified the effusion as exudative. A complete ultrasound revealed mild peritoneal effusion and decreased blood flow to the right liver lobe. Other causes of bicavitary effusion were ruled out based on blood work, ultrasound, echocardiogram, and computed tomography. The patient was taken to surgery and diagnosed with caudate liver lobe torsion and had a liver lobectomy. At the 2-week postoperative recheck, the patient was doing well and there was complete resolution of the pleural effusion. Liver lobe torsion is a rare occurrence in dogs and can be difficult to diagnose. Clinical signs are nonspecific for liver lobe torsion and patients may present in respiratory distress with significant pleural fluid accumulation. When assessing patients with pleural and peritoneal effusion, liver lobe torsion should be considered as a differential diagnosis.Keywords: pleural effusion, peritoneal effusion, hepatic torsion

  8. Distribution of pleural effusion associated with ascites on abdominal CT

    Energy Technology Data Exchange (ETDEWEB)

    Bae, In Young; Park, Chan Sup; Yeon, Jae Woo; Jeon, Yong Sun; Choi, Sung Kyu; Chung, Won Kyun [Inha Univ. Hospital, Songnam (Korea, Republic of)

    1997-04-01

    To determine through an analysis of the location of pleural effusion associated with ascites, as seen on abdominal CT scan, differences in the distribution of pleural effusion according to the etiology and distribution of ascites. We retrospectively evaluated 77 consecutive patients in whom abdominal CT scan revealed pleural effusion associated with ascites. Patients with history of surgery or trauma and those with clinically and radiologically diagnosed lung or pleural diseases were excluded. We compared the location of pleural effusion with the etiology and distribution of ascites. Forty-two patients were suffering from hepatobiliary diseases, mainly right dominant pleural effusion (26/42, 62%). Fourteen had intraperitoneal carcinomatosis with no significant difference between the frequency of right dominant (5/14, 36%) and of left dominant (6/14, 43%) pleural effusion. Eleven patients had pancreatic diseases, with mainly left dominant pleural effusion (6/11, 55%). Patients with right dominant ascites usually had right dominant pleural effusion (22/24, 92%) and those with left dominant ascites had left dominant pleural effusion (9/10, 90%). Ascites-associated pleural effusion correlated with the anatomical location of the etiology of ascites; its laterality was, in addition, usually the same as that of ascites.

  9. Distribution side of pleural effusion in heart failure

    International Nuclear Information System (INIS)

    Park, Cheol Kyu; Park, Young Ha; Jung, Se Young; Park, Seog Hee; Bahk, Yong Whee

    1988-01-01

    The interrelationship between the etiology of pleural effusion and the side of its occurrence has been a subject of clinical interest for many years. It has often been stated that pleural effusion caused by congestive heart failure tends to occur on the right side. However, some authors contended that such a side proclivity is not reliable. In the present study, the authors investigated the laterally of effusion caused by various cardiovascular diseases complicated by heart failure. We reviewed the chest X-ray films and clinical records of 68 patients with proven pleural effusion resulted from heart failure. 1. There were 34 men and 34 women with the age ranging from 10 to 84 years with the mean of 59. 2. The diagnoses were rheumatic heart disease (2 patients), coronary heart disease (10 patients), hypertensive heart disease (8 patients), mitral valvular disease (18 patients), aortic valvular disease (3 patients), and miscellaneous (27 patients). 3. 34 patients had unilateral pleural effusion, 25 and 9, right and left, respectively. Of the 34 patients with bilateral effusion, 13 patients had predominantly right-sided effusion, 5 patients had predominantly left-sided effusion and 16 patients had evenly distributed bilateral effusion. Thus, 55.8% of effusion was right-sided

  10. MR diagnosis of temporomandibular joint. A study of joint effusion

    International Nuclear Information System (INIS)

    Kaneda, Takashi; Yamashiro, Mitsuaki; Ozawa, Kaoru; Suzuki, Hiromi; Okada, Hiroyuki; Yamamoto, Hirotsugu

    1998-01-01

    The purposes of this study were to evaluate the relationship between correlation of MR joint effusion of the temporomandibular joint and disk position, to evaluate the relationship between joint effusion and aging, and to assess the frequency of MR joint effusion of bilateral temporomandibular joints. The temporomandibular joints of 192 patients with clinical symptoms of temporomandibular joint disorders were imaged bilaterally using high field, surface-coil MR imaging. Oblique sagittal and coronal proton density-weighted and T2-weighted images were obtained. Imaging findings of joint effusion were correlated with disk position, aging, and bilateral temporomandibular joints. MR showed effusion in 4% of the joints with normal superior disk position, 36% of the joints with disk displacement with reduction, and 45% of the joints with disk displacement without reduction. There were significant differences in the incidence of joint effusion between normal disk position and anterior disk displacement with or without reduction. Younger patients less than 40 years were significant higher the incidence of joint effusion than those of older patients. A significant association was seen between joint effusion and aging. MR showed effusion in 17% of the unilateral temporomandibular joint, 24% of the bilateral temporomandibular joints. There was no significant difference between unilateral and bilateral case. These results indicated that joint effusion using MR imaging was associated with varied temporomandibular joint pathologic states. (author)

  11. Effusion in magnetic resonance imaging of the temporomandibular joint

    International Nuclear Information System (INIS)

    Nah, Kyung Soo

    2003-01-01

    The purpose of this study was to investigate the distribution and frequency of temporomandibular joint (TMJ) effusion in magnetic resonance (MR) images of patients with disc displacements. On T2 weighted MR images of 148 TMJs taken from 74 patients presenting with TMJ pain and dysfunction, we assessed the cases showing TMJ effusion, defined as an amount of fluid that exceeded the maximum amount seen in a control group of asymptomatic volunteers. The amount of TMJ fluid was graded as: I (none or minimal), II (moderate), III (marked), and IV (extensive), according to a standard set by a reference. Disc displacement categories were also recorded. Of the 148 TMJs examined in this study, 52 joints (35.1%) presented with joint effusion, 24 (16.2%) showing bilateral joint effusion. 38 joints showed upper joint space effusion, and 3 showed lower joint space effusion, and 11 showed both upper and lower joint space effusion. 96 joints (64.9%) had grade I joint fluid, 27 (18.2%) grade II, 15 (10.1%) grade III, and 10 (6.8%) grade IV. 80.0% of the joints presenting with grade IV effusion showed disc displacement without reduction. Joint effusion was found not only in upper, but also in lower joint spaces. The higher the effusion grade, the greater the frequency of disc displacement without reduction.

  12. Mortality Among Patients with Pleural Effusion Undergoing Thoracentesis

    Science.gov (United States)

    DeBiasi, Erin M.; Pisani, Margaret A.; Murphy, Terrence E.; Araujo, Katy; Kookoolis, Anna; Argento, A Christine; Puchalski, Jonathan

    2016-01-01

    Background Of the 1.5 million people diagnosed with pleural effusion annually in the U.S., approximately 178,000 undergo thoracentesis. While it is known that malignant pleural effusion portends a poor prognosis, mortality of patients with nonmalignant effusions has not been well studied. Methods This prospective cohort study evaluated 308 patients undergoing thoracentesis. Chart review was performed to obtain baseline characteristics. The etiology of the effusions was determined using standardized criteria. Mortality was determined at 30-days and 1-year. Results 247 unilateral and 61 bilateral thoracenteses were performed. Malignant effusion had the highest 30-day (37%) and 1 year (77%) mortality. There was substantial patient 30-day and 1-year mortality with effusions due to multiple benign etiologies (29% and 55%), CHF (22% and 53%), and renal failure (14% and 57%). Patients with bilateral pleural effusion, relative to unilateral, were associated with higher risk of death at 30 days and 1 year (17% versus 47%; HR 2.58 CI [1.44–4.63] and 36% versus 69%; HR 2.32 CI [1.55–3.48]). Conclusions Patients undergoing thoracentesis for pleural effusion have high short and long-term mortality. Patients with malignant effusion had the highest mortality followed by multiple benign etiologies, CHF and renal failure. Bilateral pleural effusion is distinctly associated with high mortality. PMID:25837039

  13. Distribution of pleural effusion associated with ascites on abdominal CT

    International Nuclear Information System (INIS)

    Bae, In Young; Park, Chan Sup; Yeon, Jae Woo; Jeon, Yong Sun; Choi, Sung Kyu; Chung, Won Kyun

    1997-01-01

    To determine through an analysis of the location of pleural effusion associated with ascites, as seen on abdominal CT scan, differences in the distribution of pleural effusion according to the etiology and distribution of ascites. We retrospectively evaluated 77 consecutive patients in whom abdominal CT scan revealed pleural effusion associated with ascites. Patients with history of surgery or trauma and those with clinically and radiologically diagnosed lung or pleural diseases were excluded. We compared the location of pleural effusion with the etiology and distribution of ascites. Forty-two patients were suffering from hepatobiliary diseases, mainly right dominant pleural effusion (26/42, 62%). Fourteen had intraperitoneal carcinomatosis with no significant difference between the frequency of right dominant (5/14, 36%) and of left dominant (6/14, 43%) pleural effusion. Eleven patients had pancreatic diseases, with mainly left dominant pleural effusion (6/11, 55%). Patients with right dominant ascites usually had right dominant pleural effusion (22/24, 92%) and those with left dominant ascites had left dominant pleural effusion (9/10, 90%). Ascites-associated pleural effusion correlated with the anatomical location of the etiology of ascites; its laterality was, in addition, usually the same as that of ascites

  14. Incidence and significance of pleural effusion after hepatoma surgery

    International Nuclear Information System (INIS)

    Song, Jae Uoo; Im, Jung Gi; Ahn, Joong Mo; Kim, Seung Cheol; Kim, Sam Soo; Kim, Seung Hoon; Yeon, Kyung Mo

    1994-01-01

    We performed this study to evaluate the clinical significance and temporal changes of pleural effusion developed after the resection of hepatoma. We reviewed retrospectively follow-up chest radiographs of 97 patients who had undergone operation for hepatoma and had no radiologically demonstrable postoperative complications. The duration of pleural effusion was classified into five groups and the amount of pleural effusion at one week after operation was graded into four groups. Statistical significance of the relationship between the duration, amount of pleural effusion and five factors, which are location and size of tumor, age of the patients, methods of operation, and preoperative liver function, was studied respectively. Pleural effusion was developed in 63.9% (62/97) and the mean duration was 2.5 weeks. In 92% (52/56), pleural effusion disappeared spontaneously within four weeks. Patients who had hepatoma in upper portion of the right lobe developed more frequent pleural effusion which persisted longer, and was larger in amount at one week after operation(p<0.05). There were no statistically significant differences between pleural effusion and the other four factors. Pleural effusion following hepatoma surgery should not be regarded as a sign of post-operative complication, as it invariably disappears spontaneously within four weeks. Development of pleural effusion is considered to be caused by local irritation and disturbance of lymphatic flow at the diaphragm

  15. MASSIVE PLEURAL EFFUSION: A CASE REPORT

    Directory of Open Access Journals (Sweden)

    Putu Bayu Dian Tresna Dewi

    2013-03-01

    Full Text Available Pleural effusion is abnormal fluid accumulation within pleural cavity between the parietal pleura and visceralis pleura, either transudation or exudates. A 47 year-old female presented with dyspneu, cough, and decreased of appetite. She had history of right lung tumor. Physical examination revealed asymmetric chest movement where right part of lung was lagged during breathing, vocal fremitus on the right chest was decreased, dullness at the right chest, decreased vesicular sound in the right chest, enlargement of supraclavicular and colli dextra lymph nodes, and hepatomegali. Complete blood count showed leukocytosis. Clinical chemistry analysis showed hipoalbumin and decreased liver function. Blood gas analysis showed hypoxemia. Pleural fluid analysis showed an exudates, murky red liquid color filled with erythrocytes, number of cells. Cytological examination showed existence of a non-small cell carcinoma tends adeno type. From chest X-ray showed massive right pleural effusion. Based on history, physical examination and investigations, she was diagnosed with massive pleural effusion et causa suspected malignancy. She had underwent pleural fluid evacuation and treated with analgesics and antibiotics.

  16. Clinical Investigation of Benign Asbestos Pleural Effusion

    Directory of Open Access Journals (Sweden)

    Nobukazu Fujimoto

    2015-01-01

    Full Text Available There is no detailed information about benign asbestos pleural effusion (BAPE. The aim of the study was to clarify the clinical features of BAPE. The criteria of enrolled patients were as follows: (1 history of asbestos exposure; (2 presence of pleural effusion determined by chest X-ray, CT, and thoracentesis; and (3 the absence of other causes of effusion. Clinical information was retrospectively analysed and the radiological images were reviewed. There were 110 BAPE patients between 1991 and 2012. All were males and the median age at diagnosis was 74 years. The median duration of asbestos exposure and period of latency for disease onset of BAPE were 31 and 48 years, respectively. Mean values of hyaluronic acid, adenosine deaminase, and carcinoembryonic antigen in the pleural fluid were 39,840 ng/mL, 23.9 IU/L, and 1.8 ng/mL, respectively. Pleural plaques were detected in 98 cases (89.1%. Asbestosis was present in 6 (5.5% cases, rounded atelectasis was detected in 41 (37.3% cases, and diffuse pleural thickening (DPT was detected in 30 (27.3% cases. One case developed lung cancer (LC before and after BAPE. None of the cases developed malignant pleural mesothelioma (MPM during the follow-up.

  17. Does surgery or radiation provide the best overall survival in Ewing's sarcoma? A review of the National Cancer Data Base.

    Science.gov (United States)

    Miller, Benjamin J; Gao, Yubo; Duchman, Kyle R

    2017-09-01

    There is continuing debate regarding the ideal modality for local control of the primary tumor for patients with Ewing's sarcoma. The primary aim of this study is to investigate the impact of the method of local control on overall survival in patients with Ewing's sarcoma. The National Cancer Data Base was used to identify patients Ewing's sarcoma of bone. A Kaplan-Meier survival analysis was performed at 2, 5, and 10 years. Factors with a level of significance of P 8 cm, and male sex while controlling for tumor site. Surgery alone was consistently the method of local control that resulted in the highest overall survival. Surgery alone resulted in the best overall survival for patients with Ewing's sarcoma of bone. The results of this investigation provide support to the approach of surgical resection with negative margins when possible. © 2017 Wiley Periodicals, Inc.

  18. Risk Factors Associated With Secondary Sarcomas in Childhood Cancer Survivors: A Report From the Childhood Cancer Survivor Study

    International Nuclear Information System (INIS)

    Henderson, Tara O.; Rajaraman, Preetha; Stovall, Marilyn; Constine, Louis S.; Olive, Aliza; Smith, Susan A.; Mertens, Ann; Meadows, Anna; Neglia, Joseph P.; Hammond, Sue; Whitton, John; Inskip, Peter D.; Robison, Leslie L.; Diller, Lisa

    2012-01-01

    Purpose: Childhood cancer survivors have an increased risk of secondary sarcomas. To better identify those at risk, the relationship between therapeutic dose of chemotherapy and radiation and secondary sarcoma should be quantified. Methods and Materials: We conducted a nested case-control study of secondary sarcomas (105 cases, 422 matched controls) in a cohort of 14,372 childhood cancer survivors. Radiation dose at the second malignant neoplasm (SMN) site and use of chemotherapy were estimated from detailed review of medical records. Odds ratios (ORs) and 95% confidence intervals were estimated by conditional logistic regression. Excess odds ratio (EOR) was modeled as a function of radiation dose, chemotherapy, and host factors. Results: Sarcomas occurred a median of 11.8 years (range, 5.3–31.3 years) from original diagnosis. Any exposure to radiation was associated with increased risk of secondary sarcoma (OR = 4.1, 95% CI = 1.8–9.5). A dose–response relation was observed, with elevated risks at doses between 10 and 29.9 Gy (OR = 15.6, 95% CI = 4.5–53.9), 30–49.9 Gy (OR = 16.0, 95% CI 3.8–67.8) and >50 Gy (OR = 114.1, 95% CI 13.5–964.8). Anthracycline exposure was associated with sarcoma risk (OR = 3.5, 95% CI = 1.6–7.7) adjusting for radiation dose, other chemotherapy, and primary cancer. Adjusting for treatment, survivors with a first diagnosis of Hodgkin lymphoma (OR = 10.7, 95% CI = 3.1–37.4) or primary sarcoma (OR = 8.4, 95% CI = 3.2–22.3) were more likely to develop a sarcoma. Conclusions: Of the risk factors evaluated, radiation exposure was the most important for secondary sarcoma development in childhood cancer survivors; anthracycline chemotherapy exposure was also associated with increased risk.

  19. Testicular granulocytic sarcoma without systemic leukemia

    NARCIS (Netherlands)

    Lagerveld, B. W.; Wauters, C. A. P.; Karthaus, H. F. M.

    2005-01-01

    This case report describes a unilateral testicular granulocytic sarcoma or chloroma. Because of the relatively immature nature of the tumor cells, the histological diagnosis can be difficult. Granulocytic sarcomas are well known in patients with systemic leukemia and can sometimes precede a systemic

  20. Orbital Epithelioid Sarcoma: A Case Report

    NARCIS (Netherlands)

    Jurdy, Lama L.; Blank, Leo E.; Bras, Johannes; Saeed, Peerooz

    2016-01-01

    Epithelioid sarcoma is a rare but often aggressive malignancy of soft tissue that usually occurs in young adults as a superficial lesion in the distal upper limbs. To date, there are only 4 case reports of epithelioid sarcoma primarily occurring in the orbit. Two of these patients were treated with

  1. Iatrogenic colorectal Kaposi sarcoma complicating a refractory ...

    African Journals Online (AJOL)

    Kaposi sarcoma is a mesenchymal tumor associated to a human herpes virus-8. It often occurs in human immunodeficiency virus-positive subjects. Colorectal localization is rare. We report the case of a colorectal Kaposi sarcoma complicating a refractory ulcerative colitis treated with surgery after the failure of ...

  2. Soft Tissue Sarcomas In Children And Adolescents

    International Nuclear Information System (INIS)

    Bajciova, V.

    2008-01-01

    Soft tissue sarcomas are rare tumors, they may occur at any age. It is heterogenous group of different histology types, different biology and different clinical behavior. Different treatment strategy is used for children and adults. Adolescents with sarcomas could be managed by both pediatric and medical oncologists. Decision regarding location of treatment should be based on the best patient interest. (author)

  3. Viral oncogene-induced DNA damage response is activated in Kaposi sarcoma tumorigenesis.

    Directory of Open Access Journals (Sweden)

    Sonja Koopal

    2007-09-01

    Full Text Available Kaposi sarcoma is a tumor consisting of Kaposi sarcoma herpesvirus (KSHV-infected tumor cells that express endothelial cell (EC markers and viral genes like v-cyclin, vFLIP, and LANA. Despite a strong link between KSHV infection and certain neoplasms, de novo virus infection of human primary cells does not readily lead to cellular transformation. We have studied the consequences of expression of v-cyclin in primary and immortalized human dermal microvascular ECs. We show that v-cyclin, which is a homolog of cellular D-type cyclins, induces replicative stress in ECs, which leads to senescence and activation of the DNA damage response. We find that antiproliferative checkpoints are activated upon KSHV infection of ECs, and in early-stage but not late-stage lesions of clinical Kaposi sarcoma specimens. These are some of the first results suggesting that DNA damage checkpoint response also functions as an anticancer barrier in virally induced cancers.

  4. Proteomic study of benign and malignant pleural effusion.

    Science.gov (United States)

    Li, Hongqing; Tang, Zhonghao; Zhu, Huili; Ge, Haiyan; Cui, Shilei; Jiang, Weiping

    2016-06-01

    Lung adenocarcinoma can easily cause malignant pleural effusion which was difficult to discriminate from benign pleural effusion. Now there was no biomarker with high sensitivity and specificity for the malignant pleural effusion. This study used proteomics technology to acquire and analyze the protein profiles of the benign and malignant pleural effusion, to seek useful protein biomarkers with diagnostic value and to establish the diagnostic model. We chose the weak cationic-exchanger magnetic bead (WCX-MB) to purify peptides in the pleural effusion, used matrix-assisted laser desorption/ionization time-of-flight mass spectrometry (MALDI-TOF-MS) to obtain peptide expression profiles from the benign and malignant pleural effusion samples, established and validated the diagnostic model through a genetic algorithm (GA) and finally identified the most promising protein biomarker. A GA diagnostic model was established with spectra of 3930.9 and 2942.8 m/z in the training set including 25 malignant pleural effusion and 26 benign pleural effusion samples, yielding both 100 % sensitivity and 100 % specificity. The accuracy of diagnostic prediction was validated in the independent testing set with 58 malignant pleural effusion and 34 benign pleural effusion samples. Blind evaluation was as follows: the sensitivity was 89.6 %, specificity 88.2 %, PPV 92.8 %, NPV 83.3 % and accuracy 89.1 % in the independent testing set. The most promising peptide biomarker was identified successfully: Isoform 1 of caspase recruitment domain-containing protein 9 (CARD9), with 3930.9 m/z, was decreased in the malignant pleural effusion. This model is suitable to discriminate benign and malignant pleural effusion and CARD9 can be used as a new peptide biomarker.

  5. Characteristics of Patients with Tuberculous Pleural Effusion in Rural Nepal

    OpenAIRE

    M S Paudel; Anjana Kafle; Bishal Khatri Chhetri; Sahadev Prasad Dhungana; Anuj Poudel; Shamsuddhin .

    2013-01-01

      Introduction: Tuberculosis (TB) is a major cause of mortality and morbidity in developing countries. Tubercular Pleural effusion is the second most common form of extra pulmonary tuberculosis (EPTB), superseded in Prevalence only by lymph node tuberculosis. Pleural effusion occurs in approximately 5% of patients with TB. The purpose of this study was to assess the demographic characteristics of patients presenting with pleural effusion in rural Nepal.   Methods: A retrospe...

  6. VEGF Correlates with Inflammation and Fibrosis in Tuberculous Pleural Effusion

    OpenAIRE

    Bien, Mauo-Ying; Wu, Ming-Ping; Chen, Wei-Lin; Chung, Chi-Li

    2015-01-01

    Objective. To investigate the relationship among angiogenic cytokines, inflammatory markers, and fibrinolytic activity in tuberculous pleural effusion (TBPE) and their clinical importance. Methods. Forty-two patients diagnosed with TBPE were studied. Based on chest ultrasonography, there were 26 loculated and 16 nonloculated TBPE patients. The effusion size radiological scores and effusion vascular endothelial growth factor (VEGF), interleukin- (IL-) 8, plasminogen activator inhibitor type-1 ...

  7. Penile epithelioid sarcoma: MR imaging findings

    Energy Technology Data Exchange (ETDEWEB)

    Sirikci, A.; Bayram, M.; Demirci, M. [Department of Radiology, Faculty of Medicine, Gaziantep University, Kolejtepe, Gaziantep (Turkey); Bakir, K. [Department of Pathology, Faculty of Medicine, Gaziantep University, Kolejtepe, Gaziantep (Turkey); Sarica, K. [Department of Urology, Faculty of Medicine, Gaziantep University, Kolejtepe, Gaziantep (Turkey)

    1999-10-01

    Magnetic resonance imaging findings of a 38-year-old man with epithelioid sarcoma of the penis is presented. It started as a firm, painless and slowly growing nodule at the base of his penis 6 months previously which caused pain radiating to the testis during coitus. It has been well known that sarcomas may well mimic reactive processes. Initial presentation of epithelioid sarcoma may provoke considerable diagnostic difficulty, and its differentiation from benign lesions, such as Peyronie`s disease and chronic inflammation, may be a clinical problem. In our present report the MR findings are compared with those of the epithelioid sarcomas of various locations reported in the literature and differential diagnosis of the entity is discussed. To our knowledge, this is the first report regarding the MR findings of the epithelioid sarcoma of penis. (orig.) With 3 figs., 16 refs.

  8. Angiography of histopathologic variants of synovial sarcoma

    International Nuclear Information System (INIS)

    Lois, J.F.; Fischer, H.J.; Mirra, J.M.; Gomes, A.S.; California Univ., Los Angeles

    1986-01-01

    Synovial sarcomas are rare soft tissue tumors which histopathologically can be divided into monophasic, biphasic and mixed variants. As part of a protocol for intra-arterial chemotherapy 12 patients with biopsy proven synovial sarcoma underwent angiography. The angiograms on these patients were reviewed to determine whether synovial sarcomas and their variants demonstrated a characteristic angiographic appearance. Synovial sarcomas appeared angiographically as soft tissue masses which showed a fine network of tumor vessels with an inhomogeneous capillary blush. Their degree of vascularity varied according to their histopathology. Monophasic synovial sarcomas demonstrated in general a higher degree of neovascularity than the biphasic form. This finding was also suggested by histopathologic analysis of the vessels in the tumor. Although angiography did not show a distinctive vascular pattern it may be useful to evaluate tumor size and vascularity. (orig.)

  9. Activation of PI3K/AKT and ERK MAPK signal pathways is required for the induction of lytic cycle replication of Kaposi's Sarcoma-associated herpesvirus by herpes simplex virus type 1

    Directory of Open Access Journals (Sweden)

    Lv Zhigang

    2011-10-01

    Full Text Available Abstract Background Kaposi's sarcoma-associated herpesvirus (KSHV is causally linked to several acquired immunodeficiency syndrome-related malignancies, including Kaposi's sarcoma (KS, primary effusion lymphoma (PEL and a subset of multicentric Castleman's disease. Regulation of viral lytic replication is critical to the initiation and progression of KS. Recently, we reported that herpes simplex virus type 1 (HSV-1 was an important cofactor that activated lytic cycle replication of KSHV. Here, we further investigated the possible signal pathways involved in HSV-1-induced reactivation of KSHV. Results By transfecting a series of dominant negative mutants and protein expressing constructs and using pharmacologic inhibitors, we found that either Janus kinase 1 (JAK1/signal transducer and activator of transcription 3 (STAT3 or JAK1/STAT6 signaling failed to regulate HSV-1-induced KSHV replication. However, HSV-1 infection of BCBL-1 cells activated phosphatidylinositol 3-kinase (PI3K/protein kinase B (PKB, also called AKT pathway and inactivated phosphatase and tensin homologue deleted on chromosome ten (PTEN and glycogen synthase kinase-3β (GSK-3β. PTEN/PI3K/AKT/GSK-3β pathway was found to be involved in HSV-1-induced KSHV reactivation. Additionally, extracellular signal-regulated protein kinase (ERK mitogen-activated protein kinase (MAPK pathway also partially contributed to HSV-1-induced KSHV replication. Conclusions HSV-1 infection stimulated PI3K/AKT and ERK MAPK signaling pathways that in turn contributed to KSHV reactivation, which provided further insights into the molecular mechanism controlling KSHV lytic replication, particularly in the context of HSV-1 and KSHV co-infection.

  10. Radionuclide Peritoneal Scintigraphy in Patients with Ascites and Pleural Effusion

    International Nuclear Information System (INIS)

    Lee, Jae Tae; Lee, Kyu Bo; Whang, Kee Suk; Kim, Gwang Won; Chung, Byung Chun; Cho, Dong Kyu; Chung, Joon Mo

    1990-01-01

    Simultaneous presence of ascites and pleural effusion has been documented in patients with cirrhosis of the liver, renal disease, Meigs' syndrome and in patients undergoing peritoneal dialysis. Mechanisms proposed in the formation of pleural effusion in most of the above diseases are lymphatic drainage and diaphragmatic defect. But sometimes, hepatic hydrothoraxes in the absence of clinical ascites and pleural effusion secondary to pulmonary or cardiac disease are noted. It is not always possible to differentiate between pleural effusion caused by transdiaphragmatic migration of ascites and by other causes based solely on biochemical analysis. Authors performed radionuclide scintigraphy after intraperitoneal administration of 99m Tc-labeled colloid in 23 patients with both ascites and pleural effusion in order to discriminate causative mechanisms responsible for pleural effusion. Scintigraphy demonstrated the transdiaphtagmatic flow of fluid from the peritoneum to pleural cavities in 13 patients correctly. In contrast, in 5 patients with pleural effusion secondary to pulmonary, pleural and cardiac diseases, radiotracers fail to traverse the diaphragm and localize in the pleural space. Ascites draining to mediastinal lymph nodes and blocked passage of lymphatic drainage were also clarified, additionally. Conclusively, radionuclide peritoneal scintigraphy is an accurate, rapid and easy diagnostic tool in patients with both ascites and pleural effusion. It enables the causes of pleural effusion to be elucidated, as well as providing valuable information required when determining the appropriate therapy.

  11. Presumed hydrochlorothiazide-associated immunologic-hypersensitivity-induced pericardial effusion

    Directory of Open Access Journals (Sweden)

    Michael J Chaskes

    2013-08-01

    Full Text Available A 50-year-old Caucasian female presented for a second opinion regarding a newly diagnosed pericardial effusion. Seven months previously, hydrochlorothiazide was introduced into her pharmacologic regimen to aid in the management of her hypertension. A routine echocardiogram indicated a large pericardial effusion with signs of early cardiac tamponade. The patient subsequently underwent successful pericardiocentesis with complete drainage of the pericardial effusion. The effusion was empirically attributed to a viral etiology. Repeat echocardiograms showed recurrence of the pericardial effusion. Prior to undergoing a second pericardiocentesis with pericardial biopsy, as her physicians recommended, the patient sought a second opinion. While obtaining the patient’s history, an allergy to sulfa was elicited. The possibility that the pericardial effusion may be secondary to an immunologic-hypersensitivity reaction was considered. It was recommended the patient discontinue the use of hydrochlorothiazide. Nine days following discontinuation of hydrochlorothiazide and without any other intervention, an echocardiogram was reported to show the size of the pericardial effusion had subsided substantially. Nine weeks following discontinuation, almost complete resolution of the pericardial effusion was reported. It is hypothesized that when treated with hydrochlorothiazide, the patient had an immune response leading to the pericardial effusion.

  12. Radiographic features of pleural effusions in pulmonary embolism

    International Nuclear Information System (INIS)

    Bynum, L.J.; Wilson, J.E.

    1978-01-01

    A prospective analysis of 155 patients with pulmonary embolism was undertaken to describe the radiographic characteristics of associated pleural effusions and related abnormalities. Approximately one half of these patients had pleural effusions. Patients with other potential causes of effusion, such as heart failure, pneumonia, or cancer, were eliminated from further analysis. In the remaining 62 patients, radiographic evidence of pulmonary infarction accompanied pleural effusions in one half of the cases. One third of patients with parenchymal consolidation had no evidence of effusion. Atelectasis and other nonspecific radiographic abnormalities occurred in less than one fifth of the cases. Typically, pleural effusions were small and unilateral, appeared soon after symptoms of thromboembolism began, and tended to reach their maximal size very early in the course of the disorder. Pulmonary infarction was associated with larger effusions that cleared more slowly and were more often bloody in appearance on thoracentesis. Chest pain occurred in all but one patient and was a valuable diagnostic clue. Pain and pleural effusions were always ipsilateral and almost always unilateral, but neither correlated well with the presence or time course of infarction. Effusions that were delayed in onset or that enlarged late in the course were associated with recurrent pulmonary embolism or superinfection. These radiographic features may be helpful in the diagnosis and management of pulmonary embolism

  13. Predictors of Pericardial Effusion in Patients Undergoing Pulmonary Artery Banding.

    Science.gov (United States)

    Noma, Mio; Matsubara, Muneaki; Tokunaga, Chiho; Nakajima, Tomomi; Mathis, Bryan James; Sakamoto, Hiroaki; Hiramatsu, Yuji

    2018-03-01

    Although pulmonary artery banding (PAB) is a common palliative procedure for pediatric heart malformation, there are concerns of pressure overload and concomitant immune reactions in the right ventricle causing postsurgical complications such as pericardial effusion. At this time, no clear guidelines as to potential risk factors or procedural contraindications have been widely disseminated. Therefore, a study was undertaken to examine wide-ranging factors to find potential biomarkers for postsurgical pericardial effusion formation risk. A retrospective study was conducted on all cardiac surgeries performed over an eight-year period, and the main inclusion criterion was pericardial effusion development after PAB that required surgical drainage. Nine cases were then analyzed against a control group of 45 cases with respect to body measurements, concomitant surgeries, genetic screens, laboratory tests results, and cardiac function parameters. Trisomy 21 was strongly associated with the development of severe pericardial effusion after PAB, and postoperative serum albumin levels in patients with trisomy 21 were associated with pericardial effusion development. Other parameters showed no significant correlation with pericardial effusion development. Our data indicate a strong association between trisomy 21 and pericardial effusion requiring drainage after PAB, which is in line with translational research findings. Pressure overload from PAB may play a role in the formation of severe pericardial effusion that is exacerbated by cardiac structural defects commonly associated with trisomy 21. Surgical teams should therefore use caution and plan to implement drainage in PAB cases, and postoperative serum albumin may serve as a useful biomarker for pericardial effusion formation.

  14. VEGF Correlates with Inflammation and Fibrosis in Tuberculous Pleural Effusion

    Science.gov (United States)

    Bien, Mauo-Ying; Wu, Ming-Ping; Chen, Wei-Lin; Chung, Chi-Li

    2015-01-01

    Objective. To investigate the relationship among angiogenic cytokines, inflammatory markers, and fibrinolytic activity in tuberculous pleural effusion (TBPE) and their clinical importance. Methods. Forty-two patients diagnosed with TBPE were studied. Based on chest ultrasonography, there were 26 loculated and 16 nonloculated TBPE patients. The effusion size radiological scores and effusion vascular endothelial growth factor (VEGF), interleukin- (IL-) 8, plasminogen activator inhibitor type-1 (PAI-1), and tissue type plasminogen activator (tPA) were measured. Treatment outcome and pleural fibrosis, defined as radiological residual pleural thickening (RPT), were assessed at 6-month follow-up. Results. The effusion size and effusion lactate dehydrogenase (LDH), VEGF, IL-8, PAI-1, and PAI-1/tPA ratio were significantly higher, while effusion glucose, pH value, and tPA were significantly lower, in loculated than in nonloculated TBPE. VEGF and IL-8 correlated positively with LDH and PAI-1/tPA ratio and negatively with tPA in both loculated and nonloculated TBPE. Patients with higher VEGF or greater effusion size were prone to develop RPT (n = 14; VEGF, odds ratio 1.28, P = 0.01; effusion size, odds ratio 1.01, P = 0.02), and VEGF was an independent predictor of RPT in TBPE (receiver operating characteristic curve AUC = 0.985, P Effusion VEGF correlates with pleural inflammation and fibrosis and may be targeted for adjunct therapy for TBPE. PMID:25884029

  15. Effusion method at age 69: current state of the art

    International Nuclear Information System (INIS)

    Cater, E.D.

    1979-01-01

    Historical notes are presented tracing the development of the effusion methods along with some fundamental equations to be used later. Theoretical and experimental limitations of the method, and of each variant of the effusion method are cited. How these problems have been dealt with and where our understanding of them now lies are discussed, and estimates of the accuracy with which derived results can be ascribed are given. Finally, some newer techniques are mentioned which give some of the same results that have traditionally been obtained by effusion studies, try to place them in perspective, and perhaps suggest some future directions for effusion studies

  16. Pleural effusion segmentation in thin-slice CT

    Science.gov (United States)

    Donohue, Rory; Shearer, Andrew; Bruzzi, John; Khosa, Huma

    2009-02-01

    A pleural effusion is excess fluid that collects in the pleural cavity, the fluid-filled space that surrounds the lungs. Surplus amounts of such fluid can impair breathing by limiting the expansion of the lungs during inhalation. Measuring the fluid volume is indicative of the effectiveness of any treatment but, due to the similarity to surround regions, fragments of collapsed lung present and topological changes; accurate quantification of the effusion volume is a difficult imaging problem. A novel code is presented which performs conditional region growth to accurately segment the effusion shape across a dataset. We demonstrate the applicability of our technique in the segmentation of pleural effusion and pulmonary masses.

  17. Validation of equations for pleural effusion volume estimation by ultrasonography.

    Science.gov (United States)

    Hassan, Maged; Rizk, Rana; Essam, Hatem; Abouelnour, Ahmed

    2017-12-01

    To validate the accuracy of previously published equations that estimate pleural effusion volume using ultrasonography. Only equations using simple measurements were tested. Three measurements were taken at the posterior axillary line for each case with effusion: lateral height of effusion ( H ), distance between collapsed lung and chest wall ( C ) and distance between lung and diaphragm ( D ). Cases whose effusion was aspirated to dryness were included and drained volume was recorded. Intra-class correlation coefficient (ICC) was used to determine the predictive accuracy of five equations against the actual volume of aspirated effusion. 46 cases with effusion were included. The most accurate equation in predicting effusion volume was ( H  +  D ) × 70 (ICC 0.83). The simplest and yet accurate equation was H  × 100 (ICC 0.79). Pleural effusion height measured by ultrasonography gives a reasonable estimate of effusion volume. Incorporating distance between lung base and diaphragm into estimation improves accuracy from 79% with the first method to 83% with the latter.

  18. VEGF Correlates with Inflammation and Fibrosis in Tuberculous Pleural Effusion

    Directory of Open Access Journals (Sweden)

    Mauo-Ying Bien

    2015-01-01

    Full Text Available Objective. To investigate the relationship among angiogenic cytokines, inflammatory markers, and fibrinolytic activity in tuberculous pleural effusion (TBPE and their clinical importance. Methods. Forty-two patients diagnosed with TBPE were studied. Based on chest ultrasonography, there were 26 loculated and 16 nonloculated TBPE patients. The effusion size radiological scores and effusion vascular endothelial growth factor (VEGF, interleukin- (IL- 8, plasminogen activator inhibitor type-1 (PAI-1, and tissue type plasminogen activator (tPA were measured. Treatment outcome and pleural fibrosis, defined as radiological residual pleural thickening (RPT, were assessed at 6-month follow-up. Results. The effusion size and effusion lactate dehydrogenase (LDH, VEGF, IL-8, PAI-1, and PAI-1/tPA ratio were significantly higher, while effusion glucose, pH value, and tPA were significantly lower, in loculated than in nonloculated TBPE. VEGF and IL-8 correlated positively with LDH and PAI-1/tPA ratio and negatively with tPA in both loculated and nonloculated TBPE. Patients with higher VEGF or greater effusion size were prone to develop RPT (n=14; VEGF, odds ratio 1.28, P=0.01; effusion size, odds ratio 1.01, P=0.02, and VEGF was an independent predictor of RPT in TBPE (receiver operating characteristic curve AUC=0.985, P<0.001. Conclusions. Effusion VEGF correlates with pleural inflammation and fibrosis and may be targeted for adjunct therapy for TBPE.

  19. Ether à go-go potassium channel expression in soft tissue sarcoma patients

    Directory of Open Access Journals (Sweden)

    Stühmer Walter

    2006-10-01

    Full Text Available Abstract Background The expression of the human Eag1 potassium channel (Kv10.1 is normally restricted to the adult brain, but it has been detected in both tumour cell lines and primary tumours. Our purpose was to determine the frequency of expression of Eag1 in soft tissue sarcoma and its potential clinical implications. Results We used specific monoclonal antibodies to determine the expression levels of Eag1 in soft tissue sarcomas from 210 patients by immunohistochemistry. Eag1 was expressed in 71% of all tumours, with frequencies ranging from 56% (liposarcoma to 82% (rhabdomyosarcoma. We detected differences in expression levels depending on the histological type, but no association was seen between expression of this protein and sex, age, grade or tumour size. Four cell lines derived from relevant sarcoma histological types (fibrosarcoma and rhabdomyosarcoma were tested for Eag1 expression by real-time RT-PCR. We found all four lines to be positive for Eag1. In these cell lines, blockage of Eag1 by RNA interference led to a decrease in proliferation. Conclusion Eag1 is aberrantly expressed in over 70% sarcomas. In sarcoma cell lines, inhibition of Eag1 expression and/or function leads to reduced proliferation. The high frequency of expression of Eag1 in primary tumours and the restriction of normal expression of the channel to the brain, suggests the application of this protein for diagnostic or therapeutic purposes.

  20. The modified Glasgow prognostic score in patients undergoing surgery for bone and soft tissue sarcoma.

    Science.gov (United States)

    Morhij, Rossel; Mahendra, Ashish; Jane, Mike; McMillan, Donald C

    2017-05-01

    The prognostic significance of markers of the systemic inflammatory response in patients with soft tissue and bone sarcomas remains unclear. Therefore, the present study aimed to compare the prognostic value of markers of the systemic inflammatory response in patients undergoing surgery for primary soft tissue and bone sarcoma. Patients who underwent resection of primary soft tissue/bone sarcoma between 2008 and 2012 and had pre-operative measurements of the systemic inflammatory response [C-reactive protein, albumin, white cell, neutrophil, lymphocyte and platelet counts, and the combination of C-reactive protein and albumin (mGPS)] were included in the study (n = 111). The majority of the patients were ≤50 years old (84%), were female (63%), had soft tissue sarcoma (62%), and had tumours >10 cm (52%), mostly of high grade (85%). The median follow-up of survivors was 50 months (range 34-78); 24 (21%) developed local recurrence, 35 (31%) developed distant metastases and 30 (30%) died of their cancer. On univariate analysis, tumour size (P sarcoma. Copyright © 2017 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.

  1. A Trp53fl/flPtenfl/fl mouse model of undifferentiated pleomorphic sarcoma mediated by adeno-Cre injection and in vivo bioluminescence imaging.

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    Marisa R Buchakjian

    Full Text Available Genetic mouse models of soft tissue sarcoma provide critical insights into disease pathophysiology, which are oftentimes unable to be extracted from human tumor samples or xenograft models. In this study we describe a mouse model of soft tissue sarcoma mediated by adenoviral-Cre recombinase injection into Trp53fl/fl/Ptenfl/fl lox-stop-lox luciferase mice. Injection of adenovirus expressing Cre recombinase, either subcutaneously or intramuscularly in two experimental groups, results in viral infection and gene recombination with 100% penetrance within the first 24 hours following injection. Luciferase expression measured by real-time bioluminescence imaging increases over time, with an initial robust increase following viral injection, followed by a steady rise over the next several weeks as primary tumors develop and grow. Intramuscular injections were more commonly associated with evidence of systemic viral distribution than subcutaneous injections. All mice developed soft tissue sarcomas at the primary injection site, with histological examination identifying 93% of tumors as invasive pleomorphic sarcomas based on microscopic morphology and immunohistochemical expression of sarcoma markers. A lymphocytic infiltrate was present in 64% of the sarcomas in this immunocompetent model and 71% of tumors expressed PD-L1. This is the first report of a viral-Cre mediated Trp53/Pten mouse model of undifferentiated pleomorphic sarcoma. The bioluminescence imaging feature, along with high penetrance of the model and its immunological characteristics, makes it suited for pre-clinical studies of soft tissue sarcoma.

  2. Sarcoma pleomórfico primário do pulmão — 11 anos de sobrevivência

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    M.J. Gonçalves

    2011-01-01

    Full Text Available Resumo: Os sarcomas primários do pulmão são neoplasias raras e potencialmente agressivas, que devem ser distinguidos de outros tumores pulmonares malignos e cujo comportamento biológico não está completamente esclarecido.Os autores apresentam um caso clínico de sarcoma pleomórfico indiferenciado do pulmão, que foi diagnosticado a um homem de 66 anos. Na altura do diagnóstico foi realizada desobstrução brônquica por laser e radioterapia, tendo sido posteriormente observada uma sobrevivência superior a 11 anos. Abstract: Primary sarcomas of the lung are uncommon, potentially aggressive neoplasms that need to be distinguished from other malignant pulmonary tumors and whose biological behavior is as yet not fully understood.Primary undifferentiated pleomorphic sarcoma was diagnosed in the right lung of a 66 year-old man, who has survived over 11 years after treatment with endobronchial tumor debulking by laser therapy and radiotherapy. Palavras-chave: Neoplasias pulmonares, Sarcoma pulmonar, Sarcoma pleomórfico indiferenciado pulmonar, Sobrevivência prolongada, Keywords: Lung neoplasm, Pulmonary sarcoma, Undifferentiated pleomorphic pulmonary sarcoma, Long survivors

  3. Chest tube drainage of transudative pleural effusions hastens liberation from mechanical ventilation.

    Science.gov (United States)

    Kupfer, Yizhak; Seneviratne, Chanaka; Chawla, Kabu; Ramachandran, Kavan; Tessler, Sidney

    2011-03-01

    Pleural effusions occur frequently in patients requiring mechanical ventilatory support. Treatment of the precipitating cause and resolution of the pleural effusion may take considerable time. We retrospectively studied the effect of chest tube drainage of transudative pleural effusions on the liberation of patients from mechanical ventilatory support. Patients in the medical ICU (MICU) at Maimonides Medical Center between January 1, 2009, and October 31, 2009, requiring mechanical ventilatory support with a transudative pleural effusion, were studied retrospectively. They were divided into two groups: standard care and standard care plus chest tube drainage. Chest tubes were placed under ultrasound guidance by trained intensivists. Duration of mechanical ventilatory support was the primary end point. Secondary end points included measures of oxygenation, amount of fluid drained, and complications associated with the chest tube. A total of 168 patients were studied; 88 were treated with standard care and 80 underwent chest tube drainage. Total duration of mechanical ventilatory support was significantly shorter for patients who had chest tube drainage: 3.8±0.5 days vs 6.5±1.1 days for the standard group (P=.03). No differences in oxygenation were noted between the two groups. The average amount of fluid drained was 1,220 mL. No significant complications were caused by chest tube drainage. Chest tube drainage of transudative pleural effusions resulted in more rapid liberation from mechanical ventilatory support. It is a very safe procedure when performed under ultrasound guidance by experienced personnel. ClinicalTrials.gov; Identifier: NCT0114285; URL: www.clinicaltrials.gov.

  4. Treatment of Sarcoma Lung Metastases with Stereotactic Body Radiotherapy

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    Adam D. Lindsay

    2018-01-01

    Full Text Available Background. The most common site of sarcoma metastasis is the lung. Surgical resection of pulmonary metastases and chemotherapy are treatment options that have been employed, but many patients are poor candidates for these treatments for multiple host or tumor-related reasons. In this group of patients, radiation might provide a less morbid treatment alternative. We sought to evaluate the efficacy of radiotherapy in the treatment of metastatic sarcoma to the lung. Methods. Stereotactic body radiotherapy (SBRT was used to treat 117 pulmonary metastases in 44 patients. Patients were followed with serial computed tomography imaging of the chest. The primary endpoint was failure of control of a pulmonary lesion as measured by continued growth. Radiation-associated complications were recorded. Results. The majority of patients (84% received a total dose of 50 Gy per metastatic nodule utilizing an image-guided SBRT technique. The median interval follow-up was 14.2 months (range 1.6–98.6 months. Overall survival was 82% at two years and 50% at five years. Of 117 metastatic nodules treated, six nodules showed failure of treatment (95% control rate. Twenty patients (27% developed new metastatic lesions and underwent further SBRT. The side effects of SBRT included transient radiation pneumonitis n=6, cough n=2, rib fracture n=1, chronic pain n=1, dermatitis n=1, and dyspnea n=1. Conclusion. Stereotactic body radiotherapy is an effective and safe treatment for the ablation of pulmonary metastasis from sarcoma. Further work is needed to evaluate the optimal role of SBRT relative to surgery or chemotherapy for treatment of metastatic sarcoma.

  5. BONE TUMOR ENVIRONMENT AS POTENTIAL THERAPEUTIC TARGET IN EWING SARCOMA

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    Françoise eREDINI

    2015-12-01

    Full Text Available Ewing sarcoma is the second most common pediatric bone tumor, with three cases per million worldwide. In clinical terms, ES is an aggressive, rapidly fatal malignancy that mainly develops in osseous sites (85%, but also in extraskeletal soft tissue. It spreads naturally to the lungs, bones and bone marrow with poor prognosis in the two latter cases. Bone lesions from primary or secondary (metastases tumors are characterized by extensive bone remodeling, more often due to osteolysis. Osteoclast activation and subsequent bone resorption is responsible for the clinical features of bone tumors including pain, vertebral collapse and spinal cord compression. Based on the vicious cycle concept of tumor cells and bone resorbing cells, drugs which target osteoclasts may be promising agents as adjuvant setting for treating bone tumors, including Ewing sarcoma. There is also increasing evidence that cellular and molecular protagonists present in the bone microenvironment play a part in establishing a favorable niche for tumor initiation and progression. The purpose of this review is to discuss the potential therapeutic value of drugs targeting the bone tumor microenvironment in Ewing Sarcoma. The first part of the review will focus on targeting the bone resorbing function of osteoclasts by means of bisphosphonates (BPs or drugs blocking the pro-resorbing cytokine Receptor Activator of NF-kappa B Ligand (RANKL. Second, the role of this peculiar hypoxic microenvironment will be discussed in the context of resistance to chemotherapy, escape from the immune system, or neo-angiogenesis. Therapeutic interventions based on these specificities could be then proposed in the context of Ewing sarcoma.

  6. Epithelioid sarcoma: clinical, MR imaging and pathologic findings

    International Nuclear Information System (INIS)

    Hanna, S.L.; Kaste, S.; Jenkins, J.J.; Hewan-Lowe, K.; Spence, J.V.; Gupta, M.; Monson, D.; Fletcher, B.D.

    2002-01-01

    Objective. To report and describe the MR imaging features of eight new cases of this rare soft tissue sarcoma and correlate them with the clinical and histologic findings.Design and patients. Retrospective analysis was carried out for the MR imaging characteristics and histologic findings of eight patients with pathologically proven epithelioid sarcoma and the literature was reviewed. Findings were correlated in each case with the patient's clinical presentation and eventual outcome.Results. The patients, whose primary tumors ranged from 2.5 cm to 19 cm in maximum dimension, were 1 to 90 years of age. Tumors involved the extremities (n=5), the scalp (n=2) and the paraspinal muscles (n=1). Five tumors presented as well-defined, frequently painful, deeply situated masses and three as subcutaneous nodules or cutaneous ulcers with no palpable mass. Four patients had associated regional lymphadenopathy and one had distant metastases at diagnosis. MR imaging showed tumor infiltration of adjacent tissues in seven patients. Signal characteristics reflected varying degrees of cellularity, and the presence of necrosis, hemorrhage, fibrosis, hyalinization and inflammation. Bone marrow involvement was demonstrated in one patient. Clinical outcomes were generally poor.Conclusions. Epithelioid sarcoma is an aggressive soft tissue sarcoma with a varied clinical presentation, growth pattern, MR signal characteristics and histologic picture. The tumor favors the distal extremities and is commonly infiltrative and accompanied by enlarged regional lymph nodes. This neoplasm may present as an intramuscular mass but should also be suspected in patients with ulcerating cutaneous nodules with or without regional lymphadenopathy. (orig.)

  7. Ewing's Sarcoma and Second Malignancies

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    Joshua D. Schiffman

    2011-01-01

    Full Text Available Ewing's sarcoma (ES is a rare tumor that is most common in children and young adults. Late effects of ES therapy include second cancers, a tragic outcome for survivors of such a young age. This paper will explore the frequencies and types of malignancies that occur after ES. Additionally, it will review how second malignancies have changed with the shift in treatment from high-dose radiation to chemotherapy regimens including alkylators and epipodophyllotoxins. The risk of additional cancers in ES survivors will also be compared to survivors of other childhood cancers. Finally, the possible genetic contribution to ES and second malignancies will be discussed.

  8. Osteosarcoma (Osteogenic sarcoma

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    Picci Piero

    2007-01-01

    Full Text Available Abstract Osteosarcoma is a primary malignant tumour of the skeleton characterised by the direct formation of immature bone or osteoid tissue by the tumour cells. The classic osteosarcoma is a rare (0.2% of all malignant tumours highly malignant tumour, with an estimated incidence of 3 cases/million population/year. Osteosarcoma arises predominantly in the long bones and rarely in the soft tissues. The age at presentation ranges from 10 to 25 years of age. Plain radiographs, computed tomography, magnetic resonance imaging, angiography and dynamic bone scintigraphy are used for diagnosis, evaluation the extent of tumour involvement and decision of the type of operation and, if necessary, the type of reconstruction. Years ago, all patients with osteosarcoma were treated by amputation but the cure rate was under 10% and almost all patients died within a year from diagnosis. Today, for localised osteosarcoma at onset (80% of cases treated in specialized bone tumour centres with pre- and postoperative chemotherapy associated with surgery, the percentage of patients cured varies between 60% and 70%. Surgery is conservative (limb salvage in more than 90% of patients. Prognosis is more severe (cure rate about 30% for tumours located in the axial skeleton and in patients with metastasis at onset.

  9. Large pericardial effusion induced by minoxidil.

    Science.gov (United States)

    Çilingiroğlu, Mehmet; Akkuş, Nuri; Sethi, Salil; Modi, Kalgi A

    2012-04-01

    A 53-year-old male admitted with increased shortness of breath. In the physical examination, he had dyspnea, tachycardia and tachypnea. An echocardiogram showed large pericardial effusion (PE) as well as significant pulmonary hypertension. He had been started recently on minoxidil for blood pressure control. PE was reported to occur with minoxidil treatment both in patients undergoing dialysis and those with normal renal function. Pulmonary hypertension has been reported to affect the cardiac tamponade physiology. Because of significant pulmonary hypertension in our patient, a right heart catheterization was also done, which prevented cardiac tamponade. He was treated conservatively without any intervention, and PE resolved spontaneously after discontinuation of minoxidil.

  10. Pericardial effusion following cardiac surgery. A single-center experience.

    Science.gov (United States)

    Nguyen, Hien Sinh; Nguyen, Hung Doan-Thai; Vu, Thang Duc

    2018-01-01

    Background Pericardial effusion is still a common postoperative complication after open heart surgery with cardiopulmonary bypass. Pericardial effusion significantly prolongs the hospital stay and associated costs as well as affecting overall outcomes after open heart surgery in Hanoi Heart Hospital, a tertiary hospital in Vietnam with an annual volume of 1000 patients. This study aimed to investigate the clinical presentation, incidence, and risk factors of postoperative pericardial effusion, which may ensure better prevention of pericardial effusion and improvement in surgical outcomes after open heart surgery. Methods A cross-sectional study was performed on 1127 patients undergoing open heart surgery from January 2015 to December 2015. Results Thirty-six (3.19%) patients developed pericardial effusion. Of these, 16 (44.4%) had cardiac tamponade. Pericardial effusion occurred after valve procedures in 77.8% of cases. Pericardial effusion was detected after discharge in 47.2% of cases at a mean time of 18.1 ± 13.7 days. Univariate logistic regression analysis showed that age > 25 years, body surface area ≥ 1.28 m 2 , preoperative liver dysfunction, New York Heart Association class III/IV, left ventricular end-diastolic diameter z score ≥ 0.55, and postoperative anticoagulant use were associated with postoperative pericardial effusion. Multivariate logistic regression analysis showed that left ventricular end-diastolic diameter z score ≥ 0.55 was an independent risk factor for postoperative pericardial effusion. Conclusions Routine postoperative echocardiography is necessary to detect postoperative pericardial effusion. Increased left ventricular end-diastolic dimension is an independent predictor of postoperative pericardial effusion.

  11. Prognostic impact of pleural effusion in acute pulmonary embolism.

    Science.gov (United States)

    Kiris, Tuncay; Yazıcı, Selçuk; Koc, Ali; Köprülü, Cinar; Ilke Akyildiz, Zehra; Karaca, Mustafa; Nazli, Cem; Dogan, Abdullah

    2017-07-01

    Background Pulmonary embolism (PE) is a common and life-threatening condition associated with considerable morbidity and mortality. Pleural effusion occurs in about one in three cases; however, data on its prognostic value are scarce. Purpose To investigate the association between pleural effusion and both 30-day and long-term mortality in patients with acute PE. Material and Methods We retrospectively evaluated 463 patients diagnosed with acute PE using computed tomography pulmonary angiography (CTPA). Echocardiographic, demographic, and laboratory data were collected. The study population was divided into two groups: patients with and without pleural effusions. Pleural effusion detected on CT was graded as small, moderate, and large according to the amount of effusion. The predictors of 30-day and long-term total mortality were analyzed. Results Pleural effusions were found in 120 patients (25.9%). After the 30-day follow-up, all-cause mortality was higher in acute PE patients with pleural effusions than in those without (23% versus 9%, P pleural effusions had significantly higher incidence of long-term total mortality than those without pleural effusions (55% versus 23%, P pleural effusion was an independent predictor of 30-day and long-term mortality (odds ratio [OR], 2.154; 95% confidence interval [CI], 1.186-3.913; P = 0.012 and OR, 1.591; 95% CI, 1.129-2.243; P = 0.008, respectively). Conclusion Pleural effusion can be independently associated with both 30-day and long-term mortality in patients with acute PE.

  12. Subdural Effusions with Hydrocephalus after Severe Head Injury: Successful Treatment with Ventriculoperitoneal Shunt Placement: Report of 3 Adult Cases

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    N. Tzerakis

    2010-01-01

    Full Text Available Background. Subdural collections of cerebrospinal fluid (CSF with associated hydrocephalus have been described by several different and sometimes inaccurate terms. It has been proposed that a subdural effusion with hydrocephalus (SDEH can be treated effectively with a ventriculoperitoneal shunt (V-P shunt. In this study, we present our experience treating patients with SDEH without directly treating the subdural collection. Methods. We treated three patients with subdural effusions and hydrocephalus as a result of a head injury. All the patients were treated with a V-P shunt despite the fact that there was an extra-axial CSF collection with midline shift. Results. In all of the patients, the subdural effusions subsided and the ventricular dilatation improved in the postoperative period. The final clinical outcome remains difficult to predict and depends not only on the successful CSF diversion but also on the primary and secondary brain insult. Conclusion. Subdural effusions with hydrocephalus can be safely and effectively treated with V-P shunting, without directly treating the subdural effusion which subsides along with the treatment of hydrocephalus. However, it is extremely important to make an accurate diagnosis of an SDEH and differentiate this condition from other subdural collections which require different management.

  13. Predictive Factors and Treatment Outcomes of Tuberculous Pleural Effusion in Patients With Cancer and Pleural Effusion.

    Science.gov (United States)

    Lee, Jaehee; Lee, Yong Dae; Lim, Jae Kwang; Lee, Deok Heon; Yoo, Seung Soo; Lee, Shin Yup; Cha, Seung Ick; Park, Jae Yong; Kim, Chang Ho

    2017-08-01

    Patients with cancer are at an increased risk of tuberculosis. As pleural effusion has great clinical significance in patients with cancer, the differential diagnosis between tuberculous pleural effusion (TPE) and malignant pleural effusion (MPE) is important. However, the predictive factors and treatment outcomes of TPE in patients with cancer have rarely been studied. Confirmed TPE cases identified at cancer diagnosis and during anticancer management from 2008-2015 were retrospectively investigated. Patients in the study included coexisting TPE and cancer (n = 20), MPE (n = 40) and TPE without cancer (n = 40). Control groups were patients with MPE, and patients with TPE without cancer. Clinical, laboratory and pleural fluid characteristics were compared among groups. Treatment outcomes were compared between patients with TPE with and without cancer. In the final analysis, serum C-reactive protein (S-CRP) ≥3.0mg/dL and pleural fluid adenosine deaminase (ADA) ≥40U/L were independent predictors for identifying TPE in patients with cancer having pleural effusion. The combination of S-CRP with pleural fluid ADA using an "or" rule achieved a sensitivity of 100%, whereas both parameters combined in an "and" rule had a specificity of 98%. Treatment outcomes were not different between the TPE groups with and without cancer. S-CRP and pleural fluid ADA levels may be helpful for predicting TPE in patients with cancer with pleural effusion. The combination of these biomarkers provides better information for distinguishing between TPE and MPE in these patients. Treatment outcomes of TPE in patients with cancer are comparable to those in patients without cancer. Copyright © 2017 Southern Society for Clinical Investigation. Published by Elsevier Inc. All rights reserved.

  14. Diagnostic value of p53 and M67 immunostaining for distinguishing benign from malignant serous effusions

    International Nuclear Information System (INIS)

    Hafez, N.H.; Tahoun, N.S.

    2011-01-01

    The differentiation of benign mesothelial cells from malignant tumor cells, primary, or metastatic, in serous effusions based on cytomorphologic features alone can be problematic. Purpose: This study was conducted to evaluate the utility of p53 and ki67 imrminocytochemical markers in differentiating benign from malignant tumor cells in serous effusions. Patients and methods: Archival Papanicolaou-stained smears of 91 pleura and peritoneal effusions were retrieved from Cytology Unit, Pathology Department, NCI, Cairo University between 2008 and 2010. Forty-one cases were positive for malignant cells and 50 cases were benign based on cytomorphologic features. Cases having doubt were excluded from the study. The slides were de stained and subjected to immunocytochemical staining for p53 and ki67. Histologic sections of colonic carcinoma and tonsillar tissue were used as positive control for p53 and ki67, respectively. Smears having > 5% positively stained nuclei for p53 were taken as positive and labeling index 10% of ki67 was considered positive. Frequencies of the individual immunocytochemical stains; p53 and ki67, in benign and malignant effusion as well as the combination of both stains were calculated. Results: p53 immunostaining showed nuclear positivity in 31 out of 41 malignant effusions (75.6%) and in 3 out of 50 benign effusions (6%), p < 0.005. p53 had 75.6% sensitivity, 94% specificity, 91.2% PPV, and 82.5% NPV. ki67 immunostaining was positive in 30 out of 41 malignant effusions (73.2%) and in 17 out of 50 benign effusions (34%), p < 0.05. ki67 had 73.2% sensitivity, 66% specificity, 63.8% PPV, and 75% NPV. Cases were then analyzed for combined immuno profile of p3 and ki67. Among the 24 cases that coexpressed both antigens, 22 cases (91.7%) were malignant. Thirty two out of 34 cases (94.1%) that showed negative results for both antigens were benign. For the cases that showed p53 immunostaining only, 9 out of 10 cases (90%) were malignant. Fifteen out of

  15. Synovial sarcoma: MR evaluation in 23 patients

    International Nuclear Information System (INIS)

    Galant, J.; Marti-Bonmati, L.; Lafuente, J.; Hernandez, L.; Soler, R.; Saez, F.

    1997-01-01

    The synovial sarcoma is one of the most common soft tissue sarcomas. MR is the technique of choice to determine to local extension of malignant soft tissue tumors. To assess the clinical and MR imaging parameters associated with synovial sarcomas that aid in establishing their diagnosis. We review the clinical findings and images of 23 histologically confirmed synovial sarcomas that were studied by MR. Synovial sarcomas usually develop in young adults as soft tissue tumors, preferentially in the deep tissues of an extremity in close proximity to a joint. They are characterized as having a lobulated contour and septa, frequently infiltrating neighboring tissues at some point, and are heterogeneous. The presence of hemorrhage, as well as infiltration of the fascia in subcutaneous tumors, suggests the diagnosis of synovial sarcoma. The development of perilesional edema is not uncommon. Although, logically, the clinical and radiological features of synovial sarcomas can overlap with those of other soft tissue tumors, the findings described here are fairly characteristic of these lesions: thus, when present, they should serve to orient the diagnostic process. (Author) 16 refs

  16. [Demographic Analysis of Patients with Osteosarcoma, Chonddrosarcoma, Ewing's Sarcoma from one Sarcoma Center in Switzerland].

    Science.gov (United States)

    Hodel, Sandro; Seeli, Franziska; Fuchs, Bruno

    2015-06-17

    Retrospective analysis of presentation, diagnosis and outcome of patients with osteosarcoma, chondrosarcoma and Ewing's sarcoma was performed for a single Sarcoma Center in Zurich at the University Hospital Balgrist. 201 patients were included. Overall survival at five and ten years were 74 ± 6%, 69 ± 7% for osteosarcoma (n = 85, since 2000), 85 ± 7%, 80 ± 9% for Ewing's sarcoma (n = 43, since 1990) and 86 ± 5%, 78 ± 9% for chondrosarcoma (n = 73, since 2000). The here presented overall survival rates from a single Sarcoma Center in Switzerland appear to be equivalent to other large international monocenter studies. The presentation and epidemiology of these patients are in accordance with large multicenter epidemiological studies. A nationwide sarcoma database (SwissSARCOS; www.sarcoma.ch) seems indispensable for more detailed analysis and quality management in such rare diseases.

  17. Hypereosinophilia associated with genital sarcomas

    International Nuclear Information System (INIS)

    Terzieff, V.; Alonso, I.; V ázquez, A.

    2004-01-01

    Eosinophils are phagocytic leukocytes, regulators reactions Mast cell mediated hypersensitivity. toxicity and primarily responsible antiparasitic. Predominate in epithelial tissues near the interface surface (skin, digestive tract) .The cytotoxic reaction exerted by deposit cell surface substances from the granules themselves: peroxidases, neurotoxins, and other cationic proteins. Hypereosinophilia is defined as the increase in eosinophils above 1500 / m m3. The most common causes are parasitic infections and reactions allergic. About 60% of tumors may be associated with an elevation eosinophil discrete but marked eosinophilia in these patients is little frequent. Tumors are most associated lung cancer and tumors hematology. There are few reports of this entity associated with uterine sarcomas. Although the pathophysiologic mechanism is unclear, it is assumed that the base is the increased secretion of cytokines eosinofilopoiétics: interleukins (Il) I L-3, Il-5 and G M-CSFR among other possible. Self-morbidity is primarily maintained hypereosinophilic heart, and is derived from the cytotoxic action, with endomyocardial fibrosis and thrombosis. Treatment should be directed at the control of the underlying disease, as good Tumor response was associated with the account and normalizaciónd eosinófiles. Los corticosteroids prednisone, 60 mg / day po) may be effective because antagonize The stimulatory effect of cytokines. In the vast majority of cases, the disease is associated with hypereosinophilia disseminated and poor overall prognosis We present a case of vaginal sarcoma with pulmonary metastases and hypereosinophilia seniors who responded to treatment with chemotherapy

  18. Therapeutic Angiotensin-(1-7) in Treating Patients With Metastatic Sarcoma That Cannot Be Removed By Surgery

    Science.gov (United States)

    2018-02-27

    Bone Cancer; Chondrosarcoma; Clear Cell Sarcoma of the Kidney; Metastatic Osteosarcoma; Ovarian Sarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Osteosarcoma; Recurrent Uterine Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage III Uterine Sarcoma; Stage IV Adult Soft Tissue Sarcoma; Stage IV Uterine Sarcoma

  19. External and internal hemipelvectomy for sarcomas of the pelvic girdle : consequences of limb-salvage treatment

    NARCIS (Netherlands)

    Ham, SJ; Veth, RPH; van Horn, [No Value; Eisma, WH; Hoekstra, HJ; Schraffordt Koops, H.

    1997-01-01

    The outcome of different Limb-saving treatment modalities for pelvic girdle sarcoma is controversial. The oncological and functional results after 11 external and 10 internal hemipelvectomies and the consequences of limb-salvage treatment were studied in 21 consecutive patients with primary bone (19

  20. Antibiotics for otitis media with effusion in children

    NARCIS (Netherlands)

    Venekamp, R.P.; Burton, M.J.; van Dongen, T.M.A.; van der Heijden, G.J.; van Zon, A.; Schilder, A.G.M.

    2016-01-01

    Background Otitis media with effusion (OME) is characterised by an accumulation of fluid in the middle ear behind an intact tympanic membrane, without the symptoms or signs of acute infection. Since most cases of OME will resolve spontaneously, only children with persistent middle ear effusion and

  1. Hydrothorax, hydromediastinum and pericardial effusion: a complication of intravenous alimentation.

    Science.gov (United States)

    Damtew, B; Lewandowski, B

    1984-01-01

    Complications secondary to intravenous alimentation are rare but potentially lethal. Massive bilateral pleural effusions and a pericardial effusion developed in a patient receiving prolonged intravenous alimentation. Severe respiratory distress and renal failure ensued. He recovered with appropriate treatment. Images Fig. 1 Fig. 2 Fig. 3 PMID:6428731

  2. A case of young woman with recurrent right pleural effusion

    OpenAIRE

    Mehta, Asmita A; Gupta, Amit; Venkitakrishnan, Rajesh

    2015-01-01

    Endomterisois is usually found in women of child-bearing age. A case is presented of massive right-sided pleural effusion caused by endometriosis. The final diagnosis was made by thoracoscopic pleural biopsy. Physicians should be aware of this potentially treatable cause of pleural effusion having excluded other possibilities such as malignancy and tuberculosis.

  3. A case of young woman with recurrent right pleural effusion

    Directory of Open Access Journals (Sweden)

    Asmita A Mehta

    2015-01-01

    Full Text Available Endomterisois is usually found in women of child-bearing age. A case is presented of massive right-sided pleural effusion caused by endometriosis. The final diagnosis was made by thoracoscopic pleural biopsy. Physicians should be aware of this potentially treatable cause of pleural effusion having excluded other possibilities such as malignancy and tuberculosis.

  4. A case of young woman with recurrent right pleural effusion.

    Science.gov (United States)

    Mehta, Asmita A; Gupta, Amit; Venkitakrishnan, Rajesh

    2015-01-01

    Endomterisois is usually found in women of child-bearing age. A case is presented of massive right-sided pleural effusion caused by endometriosis. The final diagnosis was made by thoracoscopic pleural biopsy. Physicians should be aware of this potentially treatable cause of pleural effusion having excluded other possibilities such as malignancy and tuberculosis.

  5. Idiopathic pericardial effusion in 2 year old labrador managed with ...

    African Journals Online (AJOL)

    Ultrasound-guided pericardiocentesis was carried out using a 16 gauge over the needle catheter attached to a 3-way stopcock and a 20mls syringe; about 65mls of clear effusate was aspirated. Laboratory analysis of the effusate revealed that it was a transudate. The patient was placed on 3mg/kg furosemide, twice daily for ...

  6. External radiotherapy in the management of malignant pericardial effusion

    Energy Technology Data Exchange (ETDEWEB)

    Fairlamb, D.J. (The Royal Hospital, Wolverhampton (UK))

    1989-05-01

    Malignant pericardial effusions that are not causing tamponade can be effectively treated by external beam irradiation - a readily available non-invasive treatment. In a consecutive series six out of eight patients achieved good palliation of their effusions as a result of this treatment. (author).

  7. Values Range of Tympanometric Gradient in Otitis Media With Effusion.

    Science.gov (United States)

    Duzer, Sertac; Sakallioglu, Oner; Akyigit, Abdulvahap; Polat, Cahit; Cetiner, Hasan; Susaman, Nihat

    2017-05-01

    The aim of this study was to establish how reliable a given tympanogram is in predicting the presence or absence of a middle ear effusion, and to provide new views for the diagnostic information of tympanometry. The use of tympanometric gradient in addition to static admittance is the focus of this study. The authors enrolled 146 female and 129 male patients. The participants were allocated into groups as follow: Group A1 consisted of 50 healthy children. Group A2 consisted of 86 children with otitis media with effusion. Group B1 consisted of 85 healthy adults. Group B2 consisted of 54 adults with otitis media with effusion. All diagnostic otoscopic examination and tympanometry were performed in both ears. The authors analyzed the distribution of tympanograms in patients with otitis media with effusion and healthy controls. When the right and left ear canal volume of either children or adults with otitis media with effusion compared with healthy controls, no statistically significant different was observed (P > 0.05). On the other hand, the statistically significant difference was detected for the values of compliance, pressure and gradient of either children or adults with otitis media with effusion compared with healthy controls (P effusion. The authors think that tympanometric gradient may be useful to detect the otitis media with effusion.

  8. Computed tomography of the adult traumatic subdural effusion

    International Nuclear Information System (INIS)

    Ara, Seiji; Matsuzaki, Takayuki; Yasumura, Shuichi; Nishiya, Mikio; Nakamura, Junichi

    1979-01-01

    The present authors, since the installation of the CT scanner in Sept., 1976, have conducted an investigation of 14 adult subdural effusion cases arising from head injury which were available for a follow-up by CT scanning. These cases were examined in the first 13 months after installation. Under CT scanning, subdural effusion was recognized as a low-density area of the subdural space. Our findings indicate that, in spite of the fact that it is a subdural collection, only a limited shift of the midline structure was seen; further, the shrinking of the lateral ventricles of the effusion side was also limited. Judging from the CT findings, the neurological signs and the clinical course, the subdural effusions of the 14 cases could be classified into 2 types: (I) the minimal neurological deficit type and (II) the contusion-effusion type. Representative cases of the above are given, and, in addition, indications of surgery are also mentioned. We further state that, since the subdural effusion cases come under Type I and are characterized by the minimal neurological deficit, in many cases they are apt to be overlooked: hence, the application of CT scanning with special regard to such cases was stressed. In order to differentiate chronic subdural hematoma from subdural effusion under CT scanning, a comparative CT study was made using 20 cases of chronic subdural hematoma, and the difference in CT findings from those of subdural effusion is reported. (author)

  9. Radiation-related pericardial effusions in patients with Hodgkin's disease

    International Nuclear Information System (INIS)

    Ruckdeschel, J.C.; Chang, P.; Martin, R.G.; Byhardt, R.W.; O'Connell, M.J.; Sutherland, J.C.; Wiernik, P.H.

    1975-01-01

    Pericardial effusions following radiotherapy for Hodgkins Disease have previously been described as infrequent and related to the total dose of radiation received. Analysis of all chest x-rays on 81 patients who received upper-mantle radiotherapy for Hodgkins Disease at the Baltimore Cancer Research Center between 1968 and 1972 disclosed an incidence of pericardial effusions of 30.9% (25 of 81), with 13.6% (11 of 81) requiring limitation of activity (5) or pericardiectomy (6). Clinical presentation of radiation-related pericardial effusions was subtle, with signs and symptoms a late finding if they occurred. Radiotherapy data was reviewed and no difference in total dose (rads) or time-dose relationships (rets) was found between the groups who did or did not develop effusions. Analysis of multiple pre-treatment clinical and pathological characteristics disclosed four parameters that were felt to be related to the development of pericardial effusions; elevated ESR, normal absolute lymphocyte count, initial presence of extensive mediastinal adenopathy and the addition of adjuvant chemotherapy. The presence of increasing combinations of these pretreatment 'risk factors' led to an increasing likelihood of developing a radiation-related pericardial effusion such that six of seven patients with all four 'risk factors' developed a pericardial effusion. Nine of 13 clinically significanteffusions were associated with the addition of adjuvant chemotherapy. Possible pathogenetic mechanisms that include factors other than radiation dosage and the clinical management of radiation-related pericardial effusions are discussed

  10. VEGF correlates with inflammation and fibrosis in tuberculous pleural effusion.

    Science.gov (United States)

    Bien, Mauo-Ying; Wu, Ming-Ping; Chen, Wei-Lin; Chung, Chi-Li

    2015-01-01

    To investigate the relationship among angiogenic cytokines, inflammatory markers, and fibrinolytic activity in tuberculous pleural effusion (TBPE) and their clinical importance. Forty-two patients diagnosed with TBPE were studied. Based on chest ultrasonography, there were 26 loculated and 16 nonloculated TBPE patients. The effusion size radiological scores and effusion vascular endothelial growth factor (VEGF), interleukin- (IL-) 8, plasminogen activator inhibitor type-1 (PAI-1), and tissue type plasminogen activator (tPA) were measured. Treatment outcome and pleural fibrosis, defined as radiological residual pleural thickening (RPT), were assessed at 6-month follow-up. The effusion size and effusion lactate dehydrogenase (LDH), VEGF, IL-8, PAI-1, and PAI-1/tPA ratio were significantly higher, while effusion glucose, pH value, and tPA were significantly lower, in loculated than in nonloculated TBPE. VEGF and IL-8 correlated positively with LDH and PAI-1/tPA ratio and negatively with tPA in both loculated and nonloculated TBPE. Patients with higher VEGF or greater effusion size were prone to develop RPT (n=14; VEGF, odds ratio 1.28, P=0.01; effusion size, odds ratio 1.01, P=0.02), and VEGF was an independent predictor of RPT in TBPE (receiver operating characteristic curve AUC=0.985, PEffusion VEGF correlates with pleural inflammation and fibrosis and may be targeted for adjunct therapy for TBPE.

  11. Leakage of contrast into a postmeningitic subdural effusion

    International Nuclear Information System (INIS)

    Scotti, G.; Harwood-Nash, D.C.

    1980-01-01

    The CT findings in a 6-month-old boy with hemophilus influenzae meningitis, complicated by the occurrence of bilateral subdural effusions, are described. The effusions were hypodense and the contents markedly enhanced (increase of 40 Hounsfield units) after contrast injection. (orig.)

  12. Ewing's Sarcoma of the Adrenal Gland.

    Science.gov (United States)

    Pal, Dilip Kumar; Chandra, Vipin; Ranjan, Kumar Rajiv; Chakrabortty, Debasis; Banerjee, Manju

    2016-01-01

    Ewing's sarcoma (ES) or primitive neuro-ectodermal tumor (PNET) typically occurs in long or flat bones, the chest wall, extra-skeletal soft tissue, and rarely in solid organs. Incidence of adrenal Ewing's sarcoma is very rare. Here we report a case of Ewing's sarcoma of the right adrenal gland in an 8-year-old girl who presented with an abdominal mass. The huge tumor was managed by preoperative neo-adjuvant chemotherapy followed by surgical resection. She died due to metastasis after five months of surgery.

  13. Cell Cycle Deregulation in Ewing's Sarcoma Pathogenesis

    Science.gov (United States)

    Kowalewski, Ashley A.; Randall, R. Lor; Lessnick, Stephen L.

    2011-01-01

    Ewing's sarcoma is a highly aggressive pediatric tumor of bone that usually contains the characteristic chromosomal translocation t(11;22)(q24;q12). This translocation encodes the oncogenic fusion protein EWS/FLI, which acts as an aberrant transcription factor to deregulate target genes necessary for oncogenesis. One key feature of oncogenic transformation is dysregulation of cell cycle control. It is therefore likely that EWS/FLI and other cooperating mutations in Ewing's sarcoma modulate the cell cycle to facilitate tumorigenesis. This paper will summarize current published data associated with deregulation of the cell cycle in Ewing's sarcoma and highlight important questions that remain to be answered. PMID:21052502

  14. Cell Cycle Deregulation in Ewing's Sarcoma Pathogenesis

    Directory of Open Access Journals (Sweden)

    Ashley A. Kowalewski

    2011-01-01

    Full Text Available Ewing's sarcoma is a highly aggressive pediatric tumor of bone that usually contains the characteristic chromosomal translocation t(11;22(q24;q12. This translocation encodes the oncogenic fusion protein EWS/FLI, which acts as an aberrant transcription factor to deregulate target genes necessary for oncogenesis. One key feature of oncogenic transformation is dysregulation of cell cycle control. It is therefore likely that EWS/FLI and other cooperating mutations in Ewing's sarcoma modulate the cell cycle to facilitate tumorigenesis. This paper will summarize current published data associated with deregulation of the cell cycle in Ewing's sarcoma and highlight important questions that remain to be answered.

  15. Infectious pleural effusion status and treatment progress

    Science.gov (United States)

    Yang, Wei; Zhang, Ze-Ming

    2017-01-01

    Pleural cavity infection continuously seriously threatens human health with continuous medical progress. From the perspective of pathophysiology, it can be divided into three stages: exudative stage, fibrin exudation and pus formation stage, and organization stage. Due to the pathogenic bacteria difference of pleural cavity infection and pulmonary infection, it is very important for disease treatment to analyze the bacteria and biochemical characteristics of the infectious pleural effusion. Most prognoses of patients have been relatively good, while for some patients, the complicated parapneumonic effusion or empyema could be evolved. Antibiotic treatment and sufficient drainage are the foundation for this treatment. No evidence can support the routine use of a fibrin agent. However, it has been reported that the plasminogen activator and deoxyribonuclease can be recommended to be applied in the pleural cavity. In case of failure on conservative medical treatment, operative treatment can be applied such as thoracoscopy and pleural decortication. According to the clinical characteristics of these patients, it is a key to research prognosis, as well as early evaluation and stratification, in the future. PMID:29268539

  16. Pregnancy with bilateral tubercular pleural effusion: challenges.

    Science.gov (United States)

    Ahuja, Vanita; Gombar, Satinder; Kumar, Navneet; Goyal, Nitika; Gupta, Kanika

    2014-04-01

    Pulmonary tuberculosis (TB) during pregnancy mimics some of the physiological changes that occur during pregnancy. Diagnosis is challenging, especially when the patient presents with acute respiratory distress. The incidence of pleural effusion in TB is 3-25% and in the majority of patients, is unilateral. We describe the intensive care management of a 27-year-old pregnant woman admitted to our hospital with life threatening respiratory distress and circulatory shock. She continued to have severe metabolic and respiratory acidosis with shock in spite of the resuscitative measures undertaken. At that point, a bedside lung ultrasonography showed bilateral pleural effusion which was followed with therapeutic thoracocentesis of the right side. This resulted in the stabilization of the respiratory mechanics and haemodynamics of the patient. The pleural fluid culture tested positive for acid fast bacilli after 4 weeks in the intensive care unit. Anti-TB therapy was started and she made a rapid recovery with liberation from mechanical ventilation. The early use of bedside lung ultrasonography was instrumental in the successful management of this patient.

  17. [Eosinophilic pleural effusion possibly induced by fibrin sealant].

    Science.gov (United States)

    Kambayashi, Takatoyo; Suzuki, Takashi

    2012-02-01

    A 74-year-old man underwent right upper lobectomy for the lung cancer and bullectomy of right lower lobe. Fibrin sealant was used for sealing the excision line. The increase of the pleural effusion with increasing C-reactive protein( CRP) and eosinophilia was noted at the 17th day after the operation. The pleural effusion was transparent and yellowish colored suggesting transudatory liquid. The eosinophil in the pleural effusion was as high as 14%. The drainage of the pleural effusion was performed for 2 days resulting in disappearing the abnormal accumulation without any additional treatment. The cause of pleural effusion was supposed to be fibrin sealant by a positive result of the drug lymphocyte stimulation test.

  18. Intraarticular volume and clearance in human synovial effusions

    International Nuclear Information System (INIS)

    Wallis, W.J.; Simkin, P.A.; Nelp, W.B.; Foster, D.M.

    1985-01-01

    Intraarticular volumes were measured by radiolabeled albumin (RISA) distribution in chronic knee effusions from 11 rheumatoid arthritis patients and 9 osteoarthritis patients. Volumes of synovial fluid obtained at joint aspiration were substantially less than those found by RISA dilution. Up to 24 hours was needed for full distribution of RISA throughout the intraarticular compartment. Measured 123I and RISA radioactivity over the knee described monoexponential rate constants, lambda (minute-1). The clearance of 123I and RISA from synovial effusions was derived by the formulation volume (ml) X lambda (minute-1) = clearance (ml/minute). RISA clearance in rheumatoid effusions was significantly greater than that found in osteoarthritis effusions. Intraarticular volume and isotope clearance were easily quantified and provide measures for further evaluating the microvascular physiology of synovial effusions

  19. Newly Characterized Murine Undifferentiated Sarcoma Models Sensitive to Virotherapy with Oncolytic HSV-1 M002

    Directory of Open Access Journals (Sweden)

    Eric K. Ring

    2017-12-01

    Full Text Available Despite advances in conventional chemotherapy, surgical techniques, and radiation, outcomes for patients with relapsed, refractory, or metastatic soft tissue sarcomas are dismal. Survivors often suffer from lasting morbidity from current treatments. New targeted therapies with less toxicity, such as those that harness the immune system, and immunocompetent murine sarcoma models to test these therapies are greatly needed. We characterized two new serendipitous murine models of undifferentiated sarcoma (SARC-28 and SARC-45 and tested their sensitivity to virotherapy with oncolytic herpes simplex virus 1 (HSV-1. Both models expressed high levels of the primary HSV entry molecule nectin-1 (CD111 and were susceptible to killing by interleukin-12 (IL-12 producing HSV-1 M002 in vitro and in vivo. M002 resulted in a significant intratumoral increase in effector CD4+ and CD8+ T cells and activated monocytes, and a decrease in myeloid-derived suppressor cells (MDSCs in immunocompetent mice. Compared to parent virus R3659 (no IL-12 production, M002 resulted in higher CD8:MDSC and CD8:T regulatory cell (Treg ratios, suggesting that M002 creates a more favorable immune tumor microenvironment. These data provide support for clinical trials targeting sarcomas with oncolytic HSV-1. These models provide an exciting opportunity to explore combination therapies for soft tissue sarcomas that rely on an intact immune system to reach full therapeutic potential.

  20. Ewing sarcoma of the left big toe with trans-articular skip lesion

    Directory of Open Access Journals (Sweden)

    Ahmad F. Kamal

    2009-06-01

    Full Text Available We report the case of the patient who had Ewing Sarcoma in whom radiological and hystopathological appearances revealed a tumor mass in the left big toe along with trans-artikular skip lesion on the left diaphysis of tibia. In Cipto Mangunkusomo Hospital since 1995 until 2004 we have found 20 Ewing sarcoma cases, but only one skip lesion Ewing sarcoma was found. The diagnosis of transarticular skip lesion in association of Ewing sarcoma was confirmed in clinicopathological conferrence. The initial evaluation of all patients included the recording of the medical history, physical examination, and hematological studies. Radiographs of the chest and the site of the primary tumor were made routinely. Systemic staging was performed with use of total-body bone scan. Ray amputation of left big toe and open biopsy from mass of mid-shaft of tibia had been done to confirm the diagnosis. The patient underwent induction chemotherapy and above knee amputation. Ten months after diagnosis, he died because of advanced-distant metastasis. (Med J Indones 2008; 18: 139-44Key words: Ewing sarcoma, trans-articular skip lesion