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Sample records for extra-abdominal desmoid tumors

  1. Extra-Abdominal Desmoid Tumors Associated with Familial Adenomatous Polyposis

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    George T. Calvert

    2012-01-01

    Full Text Available Extra-abdominal desmoid tumors are a significant cause of morbidity in patients with familial adenomatous polyposis syndrome. Understanding of the basic biology and natural history of these tumors has increased substantially over the past decade. Accordingly, medical and surgical management of desmoid tumors has also evolved. This paper analyzes recent evidence pertaining to the epidemiology, molecular biology, histopathology, screening, and treatment of extra-abdominal desmoid tumors associated with familial adenomatous polyposis syndrome.

  2. Multicentric extra-abdominal desmoid tumors arising in bilateral lower limbs

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    Nobuhiro Fukushima

    2010-03-01

    Full Text Available Extra-abdominal desmoid tumors preferentially affect the shoulders, arms, backs, buttocks, and thighs of young adults. Multicentric occurrence is rather rare but seems to be another distinctive feature of extra-abdominal desmoid tumors. In this article we report a rare case of multicentric extra-abdominal desmoid tumors arising in bilateral lower limbs.

  3. Extra-Abdominal Desmoid Tumors (Aggressive Fibromatoses)

    Science.gov (United States)

    ... Centers Broken Bones & Injuries Diseases & Conditions Arthritis Tumors Sports Injuries & Prevention Children Bone Health Health & Safety Treatment ... tissue. Because of this, they are also called aggressive fibromatoses. The tumors ... erratic behavior makes treatment challenging. Treatment for a tumor depends ...

  4. [Extra-abdominal desmoid tumors. Case report and literature review].

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    Ridders, J; Ernst, A; Todt, I; Seidl, R O

    2005-07-01

    Musculoaponeurotic fibromatosis or desmoid tumors are rare. We report the case of a 57 year old woman with a slowly growing tumor behind the sternocleidomastoid muscle, which was completely removed. Histological examination confirmed the clinical suspicion of a desmoid tumor. Desmoid tumors are aggressive, locally infiltrating, non-metastasizing tumors with a high local recurrence. Genetic, endocrine and physical factors have been implicated as causative agents. The diagnosis is made histologically, reactive fibromatosis and fibrosarcoma must be eliminated in differential diagnosis. By the combination of different radiographic techniques, it is possible to describe the tumors and differentiate between vessels, nerves and bones. The therapy of the choice is the surgical resection. Adjuvant therapy, such as x-ray treatment, chemo- and hormone therapy, are indicated when the tumor is inoperable or too extensive for surgery.

  5. Transition of Treatment for Patients with Extra-Abdominal Desmoid Tumors: Nagoya University Modality

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    Nishida, Yoshihiro, E-mail: ynishida@med.nagoya-u.ac.jp; Tsukushi, Satoshi [Department of Orthopaedic Surgery, Nagoya University Graduate School of Medicine, 65-Tsurumai, Showa, Nagoya 466-8550 (Japan); Shido, Yoji [Department of Orthopaedic Surgery, Hamamatsu University School of Medicine, 1-20-1 Handayama, Higashi-ku, Hamamatsu, Shizuoka 431-3192 (Japan); Urakawa, Hiroshi; Arai, Eisuke; Ishiguro, Naoki [Department of Orthopaedic Surgery, Nagoya University Graduate School of Medicine, 65-Tsurumai, Showa, Nagoya 466-8550 (Japan)

    2012-02-07

    Treatment modalities for desmoid tumors have been changed because of the high recurrence rate, even after wide resection, and some cases experience spontaneous self-regression during clinical course. The treatment modality in our institutions before 2003 was surgical resection with wide surgical margin, however, meloxicam, which is a NSAID and a selective COX-2 inhibitor has been applied consecutively since 2003. We reviewed the previously reported outcomes of surgical and conservative treatment in our institutions. Among 30 patients receiving surgical treatment, 16 (53%) recurred. Younger age (p < 0.05) was a significant poor factor. According to RECIST for meloxicam treatment, CR was in one, PR in 10, SD in eight, PD in one evaluated at 2011. Older age (p < 0.01) was significantly associated with good outcome for meloxicam treatment. Results of the previous study indicated that surgical treatment alone could not control desmoid tumors, even with negative surgical margin. Considering the functional impairment resulting from surgery with negative surgical margin, a conservative and effective treatment modality with fewer complications is desired. Conservative treatment with meloxicam is a promising novel modality for patients with extra-abdominal desmoid tumors.

  6. Transition of Treatment for Patients with Extra-Abdominal Desmoid Tumors: Nagoya University Modality

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    Naoki Ishiguro

    2012-02-01

    Full Text Available Treatment modalities for desmoid tumors have been changed because of the high recurrence rate, even after wide resection, and some cases experience spontaneous self-regression during clinical course. The treatment modality in our institutions before 2003 was surgical resection with wide surgical margin, however, meloxicam, which is a NSAID and a selective COX-2 inhibitor has been applied consecutively since 2003. We reviewed the previously reported outcomes of surgical and conservative treatment in our institutions. Among 30 patients receiving surgical treatment, 16 (53% recurred. Younger age ( p < 0.05 was a significant poor factor. According to RECIST for meloxicam treatment, CR was in one, PR in 10, SD in eight, PD in one evaluated at 2011. Older age ( p < 0.01 was significantly associated with good outcome for meloxicam treatment. Results of the previous study indicated that surgical treatment alone could not control desmoid tumors, even with negative surgical margin. Considering the functional impairment resulting from surgery with negative surgical margin, a conservative and effective treatment modality with fewer complications is desired. Conservative treatment with meloxicam is a promising novel modality for patients with extra-abdominal desmoid tumors.

  7. Local control of extra-abdominal desmoid tumors: systematic review and meta-analysis

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    Michelle A. Ghert

    2013-02-01

    Full Text Available The local control of desmoid tumors constitutes a continuing treatment dilemma due to its high recurrence rates. The purpose of this systematic review was to critically examine the current treatment of these rare tumors and to specifically evaluate the local failure and response rates of surgery, radiation and systemic therapy. We comprehensively searched the literature for relevant studies across Cinahl, Embase, Medline and the Cochrane databases. Articles were categorized as surgery, radiation, surgery + radiation and systemic therapy (including cytotoxic and non cytotoxic. Methodological quality of included studies was assessed using the Newcastle-Ottawa Scale. Pooled odd ratios (OR for comparative studies and weighted proportions with 95% confidence intervals (CI are reported. Thirty-five articles were included in the final analysis. Weighted mean local failure rates were 22% [95% CI (16-28%], 35% [95% CI (26-44%] and 28% [95% CI (18-39%] for radiation alone, surgery alone and surgery + radiation respectively. In the analysis of comparative studies, surgery and radiation in combination had lower local failure rates than radiation alone [OR 0.7 (0.4, 1.2] and surgery alone [OR 0.7 (0.4, 1.0]. Weighted mean stable disease rates were 91% [95% CI (85-96%] and 52% [95% CI (38-65%] for non cytotoxic and cytotoxic chemotherapy respectively. The current evidence suggests that surgery alone has a consistently high rate of local recurrence in managing extra-abdominal desmoid tumors. Radiation therapy in combination with surgery improves local control rates. However, the limited data on systemic therapy for this rare tumor suggests the benefit of using both cytotoxic and non cytotoxic chemotherapy to achieve stable disease.

  8. Early experience with percutaneous cryoablation of extra-abdominal desmoid tumors

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    Kujak, Jennifer L.; Liu, Patrick T. [Mayo Clinic Arizona, Department of Radiology, Phoenix, AZ (United States); Johnson, Geoffrey B.; Callstrom, Matthew R. [Mayo Clinic Rochester, Department of Radiology, Rochester, MN (United States)

    2010-02-15

    Surgical resection, radiation therapy and chemotherapy are all accepted as standard treatments for extra-abdominal desmoid (EAD) tumors, but their effectiveness has been limited by frequent local recurrence. The purpose of this article is to describe our early experiences with using percutaneous cryoablation for local control of extra-abdominal desmoid tumors in five patients whose tumors had failed to respond to standard therapy. In a retrospective search of our institution's radiology database for patients who had undergone percutaneous cryoablation for treatment of EAD tumors between June 2004 and July 2007, we identified five patients (three female and two male). No patients were excluded from this review. Three of these patients had been referred for cryoablation for local tumor control, and two had been referred for palliation of inoperable tumors. The age range of the patients at the time of cryoablation was 9-41 years. The treated EAD tumors were located in the neck, shoulders and trunk and ranged in size from 3.0 cm to 10.0 cm. Medical records were reviewed for short-term and long-term follow-up, and patients were contacted for additional follow-up. Patients were asked to rate their pain as absent, mild, moderate or severe, and to compare it with their levels before cryoablation, describing it as improved, unchanged or worsened. Radiology records were reviewed to follow the size of the EAD tumors before and after cryotherapy. For the three patients referred for local control of EAD tumors, complete tumor coverage with the ablation zones was achieved. Two of these patients, with masses 3.0 cm and 4.9 cm in diameter, reported complete absence of pain at both short-term and long-term follow-up at 13 months and 49 months. Their tumors had completely resolved on long-term imaging follow-up at 19 months and 43 months. The third patient, with a 6.1 cm mass, reported improved mild pain at 6 months, and imaging showed a moderate decrease of tumor size. For the

  9. Extra-Abdominal Desmoid Tumours: A Review of the Literature

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    A. P. Molloy

    2012-01-01

    Full Text Available Extra-abdominal desmoid lesions, otherwise known as aggressive fibromatosis, are slow-growing benign lesions which may be encountered in clinical practice. Recent controversies exist regarding their optimal treatment. Given their benign nature, is major debulking surgery justified, or is it worth administering chemotherapy for a disease process which unusually defies common teaching and responds to such medications? We present a literature review of this particular pathology discussing the aetiology, clinical presentation, and various current controversies in the treatment options.

  10. Extra-abdominal desmoid fibromatosis: A review of management, current guidance and unanswered questions.

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    Eastley, N; McCulloch, T; Esler, C; Hennig, I; Fairbairn, J; Gronchi, A; Ashford, R

    2016-07-01

    Extra abdominal desmoid fibromatosis is a complex condition with many recognised treatments including active observation, hormonal therapy, chemotherapy, radiotherapy and surgical resection. There is large variation in the natural history of individual desmoid tumours, with some cases progressing aggressively and others regressing spontaneously when observed alone. This combined with an absence of accurate clinical predictors of a desmoid tumour's behaviour has led to difficulties in identifying which patients would benefit most from aggressive treatment, and which could be adequately managed with a policy of active observation alone. This review explores the aetiology and common presentation of extra-abdominal desmoid fibromatosis including the condition's histopathological, clinical and radiological characteristics. The current evidence for potential predictors of desmoid tumour behaviour is also reviewed, along with the indications and evidence for the multitude of treatments available. We also summarise the published guidelines that are currently available for oncologists and surgeons managing extra-abdominal desmoid fibromatosis, and highlight some of the unanswered questions that need to be addressed to optimise the management of this condition. Copyright © 2016 Elsevier Ltd. All rights reserved.

  11. Primary or recurring extra-abdominal desmoid fibromatosis: assessment of treatment by observation only.

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    Barbier, O; Anract, P; Pluot, E; Larouserie, F; Sailhan, F; Babinet, A; Tomeno, B

    2010-12-01

    Extra-abdominal desmoid fibromatosis (EADF) is a benign tumoral condition, classically managed by more or less radical and sometimes mutilating excision. This treatment strategy is associated with a recurrence rate of nearly 50% according to various reports. EADF may show spontaneous stabilization over time. A retrospective series of 26 cases of EADF managed by simple observation was studied to assess spontaneous favorable evolution and identify possible factors impacting evolution. Eleven cases were of primary EADF with no treatment or surgery, and 15 of recurrence after surgery with no adjuvant treatment. MRI was the reference examination during follow-up. Twenty-four cases showed stabilization at a median 14 months; there were no cases of renewed evolution after stabilization. One primary tumor showed spontaneous regression, and one recurrence still showed evolution at end of follow-up (23 months). The sole factor impacting potential for evolution was prior surgery. No radiologic or pathologic criteria of evolution emerged from analysis. The present series, one of the largest dedicated to EADF managed by observation, confirmed recent literature findings: a conservative "wait-and-see" attitude is reasonable and should be considered when large-scale resection would entail significant functional or esthetic impairment. Level IV, retrospective study. Copyright © 2010 Elsevier Masson SAS. All rights reserved.

  12. Desmoid tumor in Gardner's Syndrome presented as acute abdomen

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    Rizos Spiros

    2006-03-01

    Full Text Available Abstract Background Gardner's syndrome can occasionally be complicated with intra-abdominal desmoid tumor. These tumors usually remain asymptomatic but can exhibit symptoms due to intestinal, vascular and ureteral compression and obstruction. Case presentation A rare case of a 41-year-old male patient with Gardner's syndrome complicated with intra-abdominal desmoid tumor, which first presented as acute abdomen, is presented. Conclusion Extra-abdominal manifestations of Gardner's syndrome along with a palpable abdominal mass would raise suspicion for the presence of a desmoid tumor in the majority of cases. In life-threatening cases, surgical treatment should be considered as a palliative approach, though the extent of excision remains debatable

  13. Updates on abdominal desmoid tumors

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    Desmoid tumor is a monoclonal, fibroblastic proliferation arising in musculoaponeurotic structures. This connective tissue hyperplasia infiltrates locally, recurs frequentiy after resection but does not metastasize. Abdominal desmoid occurs sporadically, in association with some familial syndromes and often represents a clinical dilemma for surgeons. The enigmatic biology and anatomical location of abdominal desmoids make treatment recommendations difficult. This distinct pathological entity is reviewed with a specific focus on aetiology and management.

  14. Desmoid Tumor of the Pancreas

    DEFF Research Database (Denmark)

    Gerleman, Roxana; Mortensen, Michael Bau; Detlefsen, Sönke

    2015-01-01

    Desmoid tumors, also known as desmoid-type fibromatoses or aggressive fibromatoses, are clonal fibroblastic proliferations that arise in the deep soft tissues. They are characterized by infiltrative growth, a tendency toward local recurrence and the inability to metastasize. We present a case of ...

  15. Bilateral desmoid tumor of the breast: case seriesand literature review

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    Wongmaneerung, Phanchaporn; Somwangprasert, Areewan; Watcharachan, Kirati; Ditsatham, Chagkrit

    2016-01-01

    Background Desmoid tumor of the breast is very rare and locally aggressive but has no distant metastasis. Bilateral lesions are extremely rare, found in only 4% of patients. Two cases of bilateral desmoid tumor of the breast are reported. The clinical presentation, diagnosis, imaging, treatment, and follow-up outcomes of recurrence as well as a brief literature review are provided. Case reports Case 1 is a 31-year-old woman who presented with nipple retraction. An ultrasound revealed BIRAD V in both breasts. She underwent a bilateral excisional biopsy under ultrasound mark with the pathology result of extra-abdominal desmoid tumor in both breasts. The patient had a bilateral mastectomy with silicone implantation due to the involved margins by excision. She remained tumor free after 7-year follow-up. Case 2 is a 28-year-old woman who presented with a lump on her right breast that she had discovered ~2 months earlier. An ultrasound showed a spiculated mass in the right breast and some circumscribed hypoechoic masses in both breasts. A bilateral breast excision was done. The pathology result was an extra-abdominal desmoid tumor. She had recurrence on both sides and underwent a mastectomy and silicone implantation. The tumor has not recurred after 1-year follow-up. Conclusion Imaging cannot distinguish between benign breast lesions and malignancy. Pathology results are helpful in making a definitive diagnosis. Given that the desmoid tumor is locally aggressive, a local excision with clear margins is recommended. Chemotherapy and hormonal treatment are controversial. PMID:27578999

  16. Genetics Home Reference: desmoid tumor

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    ... The most common symptom of desmoid tumors is pain. Other signs and symptoms, which are often caused ... occur during a person's lifetime, called somatic mutations. A somatic mutation in one copy of ...

  17. Treatment of Extra — Abdominal Desmoid Tumors with Chemotherapy

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    H. Thomas Temple

    2011-08-01

    Full Text Available Fibromatosis, or extra-abdominal desmoid tumor, is a benign disease which often has an aggressive clinical course that can be difficult to treat. We performed a retrospective review of 16 patients (12 females and four males with a mean age of 34.2 years treated with methotrexate and vinblastine for newly diagnosed or recurrent extra-abdominal desmoid tumor. The mean age of our patient cohort was 34.2 years (range 11–70, and the mean tumor size was 11.5 cm (range 2.5–21.2 cm. The mean duration of therapy was 12 months with an average follow-up of 43 months (range 1–149 months. Fourteen of 16 patients demonstrated a clinical response to treatment. Eight of 14 patients demonstrated a radiologic decrease in tumor size. Only one patient progressed on therapy. Six patients developed recurrent symptoms after discontinuation of treatment. Chemotherapy-related symptoms including neutropenia, nausea, and vomiting were common and observed in most patients, however these side effects were mild and transient. Five patients developed peripheral neuropathy that prompted a change from vinblastine to vinorelbine during treatment. One potentially life-threatening complication (pneumocystis pneumonia occurred which was diagnosed early and successfully treated. The use of methotrexate and vinblastine/vinorelbine in the management of fibromatosis appears to be an effective treatment with minimal treatment-related side effects.

  18. Treatment of Extra — Abdominal Desmoid Tumors with Chemotherapy

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    Montgomery, Corey [Rockefeller Cancer Institute, University of Arkansas for Medical Sciences, Little Rock, AR 72211 (United States); Emory, Cynthia [Wake Forest School of Medicine, Medical Center Blvds, Winston-Salem, NC 27157 (United States); Adams, Sheila [Department of Orthopaedics and Rehabilitation, University of Miami Miller School of Medicine, Cedars Medical Center, 1400 NW 12th Avenue (R-12), Miami, FL 33136 (United States); Cohen, Jonathan [Division of Psychology, University of Miami Miller School of Medicine, 1695 N.W. 9th Ave. (D-29), Miami, FL 33136 (United States); Pitcher, John David [Department of Orthopaedics and Rehabilitation, University of Miami School of Medicine, 1400 NW 12th Avenue (R-12), Miami, FL 33136 (United States); Potter, Benjamin Kyle [Department of Orthopaedic Surgery, Walter Reed Army Medical Center, 6900 Georgia Avenue North West, Washington, D.C., 20307 (United States); Temple, H. Thomas [Department of Orthopaedic Surgery, University of Miami Miller School of Medicine, 1600 N.W. 10th Avenue (R-12), Miami, FL 33136 (United States)

    2011-08-25

    Fibromatosis, or extra-abdominal desmoid tumor, is a benign disease which often has an aggressive clinical course that can be difficult to treat. We performed a retrospective review of 16 patients (12 females and four males) with a mean age of 34.2 years treated with methotrexate and vinblastine for newly diagnosed or recurrent extra-abdominal desmoid tumor. The mean age of our patient cohort was 34.2 years (range 11–70), and the mean tumor size was 11.5 cm (range 2.5–21.2 cm). The mean duration of therapy was 12 months with an average follow-up of 43 months (range 1–149 months). Fourteen of 16 patients demonstrated a clinical response to treatment. Eight of 14 patients demonstrated a radiologic decrease in tumor size. Only one patient progressed on therapy. Six patients developed recurrent symptoms after discontinuation of treatment. Chemotherapy-related symptoms including neutropenia, nausea, and vomiting were common and observed in most patients, however these side effects were mild and transient. Five patients developed peripheral neuropathy that prompted a change from vinblastine to vinorelbine during treatment. One potentially life-threatening complication (pneumocystis pneumonia) occurred which was diagnosed early and successfully treated. The use of methotrexate and vinblastine/vinorelbine in the management of fibromatosis appears to be an effective treatment with minimal treatment-related side effects.

  19. Desmoid Tumors in the Pediatric Population

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    Joshua N. Honeyman

    2012-03-01

    Full Text Available Desmoid tumors are benign soft tissue tumors associated with locally aggressive growth and high rates of morbidity, but they do not metastasize via lymphatic or hematogenous routes. While most of the data on desmoid tumors originates in the adult literature, many of the findings have been applied to the management of pediatric patients. This article discusses the epidemiology, etiology, clinical presentation, pathology, and treatment of this rare tumor in the pediatric population and includes a literature review of the most recent large series of pediatric patients with desmoid tumors.

  20. RARE CASE OF DESMOID TUMOR OF URINARY BLADDER

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    Chalapathy

    2015-08-01

    Full Text Available Desmoid tumor is a benign soft tissue tumor which belongs to a family of myofibroblastic fibromatoses. Occasionally, desmoid tumors have an unusual site of occurrence . We describe a case of incisional hernia in postmenopausal women with an intra operative incidental finding of a desmoid tumor from anterior wall of urinary bladder for which a wide excision was performed

  1. Tumor desmoide abdômino-pélvico Abdominopelvic desmoid tumor

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    Fernando Antonio Mourão Valejo

    2009-01-01

    Full Text Available O tumor desmoide é uma neoplasia não capsulada, localmente agressiva, originária dos fibroblastos dos tecidos músculo-aponeuróticos. Embora ele não tenha comportamento maligno, tal como capacidade de gerar metástases ou de invasão, o tumor desmoide tem uma alta capacidade de crescimento local, causando deformidades nos órgãos adjacentes, dor e, eventualmente, disfunção orgânica, dependendo da área envolvida. Relatamos o caso de um tumor desmoide intra-abdominal de grandes proporções, invadindo órgãos pélvicos em uma paciente de 53 anos. A neoplasia foi totalmente extirpada e, atualmente, cinco anos após a cirurgia, a paciente encontra-se sem sinal clínico ou radiológico de recidiva da lesão.Desmoid tumor is a noncapsulated neoplasia, locally aggressive, originated from the fibroblasts of the musculo-aponeurotic tissues. Even though with no malignant behavior, such as the ability of generating metastasis or of invasion, the desmoid tumor has a high tendency for local growth, causing deformities in the adjacent organs, pain and sometimes organ dysfunction, depending on the area involved. We report the case of a large intra-abdominal desmoid tumor, invading pelvic organs in a 53 year-old patient. The neoplasia has been totally extirpated and, at the moment, five years after the surgery, the patient presents no clinical or radiologic sign of lesion relapse.

  2. Desmoid tumors of the abdominal wall: A case report

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    Textor Hans Jochen

    2003-07-01

    Full Text Available Abstract Background Desmoid tumors are slow growing deep fibromatoses with aggressive infiltration of adjacent tissue but without any metastatic potential. Case Presentation We report on two female patients with desmoid tumor of the abdominal wall who underwent primary resection. Both patients had a history of an earlier abdominal surgery. Preoperative evaluation included abdominal ultrasound, magnetic resonance imaging and computed tomography. The histology in both cases revealed a desmoid tumor. Conclusion Complete surgical resection is the first line management of this tumor entity.

  3. Desmoplastic small round cell tumor: Extra abdominal and abdominal presentations and the results of treatment

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    Biswas G

    2005-01-01

    Full Text Available BACKGROUND: Desmoplastic small round cell tumor (DSRCT is a rare malignant neoplasm of adolescent males. Current multimodality treatment prolongs life and rarely achieves cure. Aim: To review the presenting features, histopathology and outcome of 18 patients with DSRCT treated at a single institution. Setting and Design: This is a retrospective observational study of patients with DSRCT who presented at the Tata Memorial Hospital between January 1994 to January 2005. Materials and Methods: Eighteen patients of DSRCT seen during this period were evaluated for their clinical presentation, response to chemotherapy and other multimodality treatment and overall survival. The cohort of 18 patients included 11 males (61% and 7 females (39% with a mean age of 16 years (Range 1½ - 30 years. Majority (83% presented with abdomino-pelvic disease. The others, involving chest wall and extremities. There were 6 patients (33% with metastatic disease at presentation. Results:The treatment primarily included a multimodality approach using a combination of multiagent chemotherapy with adjuvant surgery and radiotherapy as applicable. A response rate of 39% (CR-1, PR-6, with chemotherapy was observed. The overall response rate after multimodality treatment was 39% (CR-5, PR-2. The overall survival was poor except in patients who had complete excision of the tumor. Conclusion: Abdomino-pelvic site was the commonest presentation, the disease can occur at other non-serosal surfaces also. Despite aggressive treatment the outcome was poor. However, complete surgical excision seems to provide a better survival.

  4. Desmoid tumor of bone with enchondromatous nodules, mistaken for chondrosarcoma

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    Bahk, Won-Jong [Musculoskeletal Oncology Study Group, Catholic University of Korea (Korea); Department of Orthopaedic Surgery, Uijongbu St. Mary' s Hospital, 65-1 Geumohdong, Uijongbu, Gyunggido, 480-130 (Korea); Kang, Yong-Koo; Lee, An-Hee [Musculoskeletal Oncology Study Group, Catholic University of Korea (Korea); Mirra, Joseph M. [Orthpaedic Oncology, Orthopaedic Hospital, Los Angeles, CA (United States)

    2003-04-01

    Desmoid tumor of bone, also termed desmoplastic fibroma or aggressive fibromatosis, is a rare, locally aggressive fibroblastic tumor. We present a 16-year-old male with a huge desmoid tumor involving the iliac wing. It was associated with enchondromatous nodules mimicking malignancy. The tumor in this patient was mistaken for chondrosarcoma and hemipelvectomy was performed. To our knowledge, such a case has not previously been documented fully in the English literature. The radiographic and pathologic findings and a possible mechanism of enchondromatous nodule formation in fibrous bone tumors are discussed. (orig.)

  5. Huge Facial Desmoid Tumors with Neck Extension: A Case Report

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    Ali Ghazipour

    2014-07-01

    Full Text Available Introduction: Desmoid tumors are very rare, benign fibrous neoplasms arise from the musculoaponeurotic structures throughout the body.   Case Report: The patient was a   seven-year old boy with a large mandibular mass growing over a period of six months. His CT-scan showed a large mass, 13 cm in diameter in the cheek area extending to the neck and trachea. Biopsy was compatible with desmoid fibromatosis.  He was given neoadjovant treatment with vinblastin and methotrexate. The patient underwent a tracheostomy. Then a complete hemimandibulectomy and submandibular gland excision was performed.  Finally reconstruction with latisimus dorsi free flap was performed.   Conclusion: Despite rarity desmoid tumors should be kept in mind of an otorhinolaryngologist as a differential diagnosis in children with head and neck mass.  

  6. Multiple desmoid tumors in a patient with familial adenomatous polyposis caused by the novel W421X mutation Tumor desmoide múltiple en un paciente con poliposis adenomatosa familiar originada por la nueva mutación W421X

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    Orestis Ioannidis

    2012-03-01

    Full Text Available Familial adenomatous polyposis (FAP is a rare syndrome characterized by the presence of hundreds to thousands of colorectal adenomas and is responsible for less than 1% of all colorectal cancers. The syndrome is also characterized by extra-colorectal features including amongst others upper gastrointestinal tract polyps and desmoid tumors. The syndrome is inherited by an autosomal dominant gene, the adenomatous polyposis coli (APC gene. We present the physical history, clinical presentation, diagnosis and treatment of a patient with a novel germline APC mutation, the W421X mutation, which resulted in FAP presenting with about a hundred colorectal polyps, gastric hyperplastic polyps and multiple aggressive intra-abdominal and extra-abdominal desmoid tumors.

  7. Diagnostic problems of abdominal desmoid tumors in various locations

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    Kreuzberg, B. [Department of Diagnostic Radiology, Faculty Hospital and Medical Faculty of Charles University, Alej Svobody 80, 30460 Pilsen (Czech Republic)]. E-mail: kreuzberg@fnplzen.cz; Koudelova, J. [Department of Diagnostic Radiology, Faculty Hospital and Medical Faculty of Charles University, Alej Svobody 80, 30460 Pilsen (Czech Republic); Ferda, J. [Department of Diagnostic Radiology, Faculty Hospital and Medical Faculty of Charles University, Alej Svobody 80, 30460 Pilsen (Czech Republic); Treska, V. [Department of Surgery, Faculty Hospital and Medical Faculty of Charles University, Alej Svobody 80, 30460 Pilsen (Czech Republic); Spidlen, V. [Department of Surgery, Faculty Hospital and Medical Faculty of Charles University, Alej Svobody 80, 30460 Pilsen (Czech Republic); Mukensnabl, P. [Sikl' s Department of Pathology, Faculty Hospital and Medical Faculty of Charles University, Alej Svobody 80, 30460 Pilsen (Czech Republic)

    2007-05-15

    Background: Abdominal desmoid tumor is one of the forms of deep (musculoaponeurotic) fibromatosis. It occurs more often as a desmoid tumor in the abdominal wall, less often in various intra-abdominal locations. In this work, we performed retrospective study concerning diagnostic problems of this disease with the use of imaging techniques. Method: Four patients (three females and one male) were examined by postcontrast CT (computed tomography) and one of them also by MR (magnetic resonance). All findings were correlated with the operational findings and histologic examination. Results: The findings were typical only in the case with the lesion located in the abdominal wall, three described cases of the intra-abdominal desmoid provided a broad range of differentially diagnostic possibilities (metastases, GIT tumors, lymphomas, etc.). In particular, the findings in infiltrative processes in intra-abdominal location and retroperitoneal involvement were less typical. Ultimately, the histological findings were decisive. Conclusion: The possibility of the occurrence of intra-abdominal desmoid tumor must be considered particularly in younger individuals with rapidly growing tumorous process, which does not immediately arise from the surrounding organs (digestive tract, internal genitalia, etc.), and is located in the abdominal wall or in the abdominal cavity.

  8. Abdominal desmoid tumor: difficulties in the clinical and radiological diagnosis; Tumor desmoide abdominal: dificuldades no diagnostico clinico e radiologico

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    Lima, Marco Fabio Prata; Melo, Ana Lucia Kefalas Oliveira; Sousa Batista Leite, Luciana de; Cavasini, Alexandre Marcos [Faculdade de Medicina do Triangulo Mineiro (FMTM), Uberaba, MG (Brazil)

    1998-05-01

    The authors report a serious case of abdominal desmoid tumor with important pelvic implication and comment about image diagnostic difficulties involving this issue. It is commented about the clinical covering of this disease, and some epidemiologic, diagnostic and therapeutic aspects are discussed involving the clinical data related in this case. (author) 10 refs., 3 figs.

  9. Biology and Treatment of Aggressive Fibromatosis or Desmoid Tumor.

    Science.gov (United States)

    Skubitz, Keith M

    2017-06-01

    Aggressive fibromatosis, also known as desmoid-type fibromatosis (DTF) or desmoid tumor, is an uncommon locally invasive tumor. Because of its low incidence and variable behavior, DTF is often first seen by physicians who are not familiar with it, and recent advances in understanding this disease have led to changes in treatment approaches. The Wnt (β-catenin) pathway appears to play a key role in DTF pathogenesis, and recent studies of DTF biology suggest a possible model of DTF pathogenesis. Histologically, DTF shows a poorly circumscribed proliferation of myofibroblast-like cells with variable collagen deposition, similar to the proliferative phase of wound healing, and DTF has been associated with trauma and pregnancy. Desmoid-type fibromatosis may be a useful model of the tumor stroma in carcinomas as well as other fibrosing diseases such as progressive pulmonary fibrosis. The clinical course of DTF can vary greatly among patients, complicating the determination of the optimal treatment approach. Treatment options include surgery, nonsteroidal anti-inflammatory drugs with or without hormonal manipulation, chemotherapy, radiation therapy, and other forms of local therapy. Many treatments have been used, but these are not without toxicities. Because of the variable nature of the disease and the potential morbidity of treatment, some cases of DTF may do better without treatment; simple observation is often the best initial treatment. This review used a PubMed search from January 1, 1980, through October 31, 2016, using the terms fibromatosis and desmoid and discusses DTF disease characteristics, pathophysiology, and treatment options as well as examines several cases illustrating key points in the biology and treatment of this heterogeneous disease. Copyright © 2017 Mayo Foundation for Medical Education and Research. Published by Elsevier Inc. All rights reserved.

  10. Giant Desmoid Tumor of the Anterior Abdominal Wall in a Young Female: A Case Report

    Directory of Open Access Journals (Sweden)

    Mahim Koshariya

    2013-01-01

    Full Text Available Desmoid tumors (also called desmoids fibromatosis are rare slow growing benign and musculoaponeurotic tumors. Although these tumors have a propensity to invade surrounding tissues, they are not malignant. These tumors are associated with women of fertile age, especially during and after pregnancy. We report a young female patient with a giant desmoid tumor of the anterior abdominal wall who underwent primary resection. The patient had no history of an earlier abdominal surgery. Preoperative evaluation included abdominal ultrasound, computed tomography, and magnetic resonance imaging. The histology revealed a desmoid tumor. Primary surgical resection with immediate reconstruction of abdominal defect is the best management of this rarity. To the best of our knowledge and PubMed search, this is the first case ever reported in the medical literature of such a giant desmoid tumor arising from anterior abdominal wall weighing 6.5 kg treated surgically with successful outcome.

  11. Current Perspectives on Desmoid Tumors: The Mayo Clinic Approach

    Directory of Open Access Journals (Sweden)

    Scott Okuno

    2011-08-01

    Full Text Available Desmoid tumors are a rare group of locally aggressive, non malignant tumors of fibroblastic origin that can lead to significant morbidity due to local invasion. Despite advances in the understanding of these tumors, their natural history is incompletely understood and the optimal treatment is still a matter of debate. Local control is the main goal of treatment and there has been a change in philosophy regarding the management of these tumors from aggressive surgical resection to function preservation. A multidisciplinary approach is essential to plan local control with acceptable morbidity. The current Mayo Clinic algorithm for the treatment of these tumors is based on institutional experience and the available evidence in the literature: asymptomatic/non progressive lesions away from vital structures are managed with observation and regular imaging; primary or recurrent desmoid tumors which are symptomatic or progressive or near vital structures are managed with wide surgical resection when wide surgical margins are possible with minimal functional and cosmetic loss. When positive or close surgical margins are likely, surgical resection with adjuvant radiotherapy or definitive radiotherapy is preferred. If likely functional or cosmetic deficit is unacceptable, radiotherapy is the treatment of choice. Unresectable lesions are considered for radiotherapy, chemotherapy or newer modalities however an unresectable lesion associated with a painful, functionless, infected extremity is managed with an amputation.

  12. Current Perspectives on Desmoid Tumors: The Mayo Clinic Approach

    Energy Technology Data Exchange (ETDEWEB)

    Joglekar, Siddharth B. [Musculoskeletal Oncology, Mayo Clinic, Rochester, MN55905 (United States); Rose, Peter S.; Sim, Franklin, E-mail: sim.franklin@mayo.edu [Department of Orthopedics, Mayo Clinic, 200 1st ST SW, Rochester, MN 55905 (United States); Okuno, Scott [Department of Oncology, Mayo Clinic, Rochester, MN55905 (United States); Petersen, Ivy [Department of Radiation Oncology, Mayo Clinic, Rochester, MN55905 (United States)

    2011-08-08

    Desmoid tumors are a rare group of locally aggressive, non malignant tumors of fibroblastic origin that can lead to significant morbidity due to local invasion. Despite advances in the understanding of these tumors, their natural history is incompletely understood and the optimal treatment is still a matter of debate. Local control is the main goal of treatment and there has been a change in philosophy regarding the management of these tumors from aggressive surgical resection to function preservation. A multidisciplinary approach is essential to plan local control with acceptable morbidity. The current Mayo Clinic algorithm for the treatment of these tumors is based on institutional experience and the available evidence in the literature: asymptomatic/non progressive lesions away from vital structures are managed with observation and regular imaging; primary or recurrent desmoid tumors which are symptomatic or progressive or near vital structures are managed with wide surgical resection when wide surgical margins are possible with minimal functional and cosmetic loss. When positive or close surgical margins are likely, surgical resection with adjuvant radiotherapy or definitive radiotherapy is preferred. If likely functional or cosmetic deficit is unacceptable, radiotherapy is the treatment of choice. Unresectable lesions are considered for radiotherapy, chemotherapy or newer modalities however an unresectable lesion associated with a painful, functionless, infected extremity is managed with an amputation.

  13. MR findings of extra abdominal fibromatosis

    Energy Technology Data Exchange (ETDEWEB)

    Park, Hee Jin; Lee, Sung Moon; Rhee, Chang Soo; Sohn, Chul Ho; Lee, Hee Jung; Kim, Jung Sik; Kim, Hong [Dongsan Medical Center, Keimyung Univ. College of Medicine, Taegu (Korea, Republic of); Seo, Kyung Jin [Suh Joo MRI center, Seoul (Korea, Republic of); Jo, Kil Ho [Youngnam Univ. College of Medicine, Kyongsan (Korea, Republic of)

    1999-12-01

    To evaluate the MR findings of extra-abdominal fibromatosis and the role of MRI in primary diagnosis Fifteen cases in of histologically proven extra-abdominal fibromatosis in 13 patients were retrospectively reviewed. T1-weighted and T2-weighted images were obtained in axial, coronal and sagittal planes. Gd-enhancement was performed in 14 cases, and dynamic enhancement studies in two. All lesions were evaluated for mass shape and margin definition. Among the 15 cases, tumors of the buttock accounted for five, and tumor of the thigh for two. in eight cases tumors were intermuscular and in six cases were intramuscular. In ten cases (67%) the mass extended along the long axis of the body and in 14 of 15 cases (93%) focal infiltration of adjacent structures was visible. The signal intensity of the lesion was in all cases inhomogeneous on both T1 and T2 weighted images. As seen on Gd-DTPA enhanced scans, the masses were inhomogeneously enhanced. In all cases MRI revealed star-shaped linear strands or a band-like low signal area in the mass. These features were not enhanced and were arranged along the long axis of the mass. MR findings of extra-abdominal fibromatosis were relatively characteristic and helpful for primary diagnosis of the condition.

  14. Functional and Aesthetic Thorax Reconstruction after Desmoid Tumor Resection

    Science.gov (United States)

    Acosta-Ponce de Leon, Eric; Sanchez-Sosa, Sergio

    2017-01-01

    Summary: This study describes a case report of a 31-year-old patient who presented with a left thoracic tumor on costal cartilages 5 and 6 that was diagnosed as a desmoid tumor 3 years after receiving retropectoral breast implants for cosmetic reasons. The integral reconstruction of the thoracic wall, functional and aesthetic, was planned for a single surgical period. The defect secondary to the tumor resection, which left the pericardium and lung exposed, was closed using the pectoral muscle as a “pre-expanded” flap by the breast implant, and the breast aesthetic was treated bilaterally with new implants in the retromammary position. After 12 months, the patient remained free from tumor recurrence and had a satisfactory aesthetic result. PMID:28280682

  15. Intraperitoneal desmoid tumor: achievements in CT. Tumor demoide intraperitoneal: hallazgos en TC

    Energy Technology Data Exchange (ETDEWEB)

    Soto Aguilar, M.C.; Navarro Sanchis, E.; Sanchez-Lafuente Rubio, J.; Vicente Romo, J.; Fuentes Limpiaez, J.J. (Hospital Regional Carlos Haya. Servicio de Radiodiagnostico. Malaga (Spain))

    1993-01-01

    We present two cases of intraperitoneal desmoid tumor studied by means of CT. Both presented in the form of solid masses with involvement of small bowel loops in asympthmatic patients. CT is useful for locating the mass and studying possible tumor recurrences, but there is no semiological data to differentiate them from other intraperitoneal solid neoplasms. (Author)

  16. Aggressive fibromatosis (desmoid tumor) is derived from mesenchymal progenitor cells.

    Science.gov (United States)

    Wu, Colleen; Amini-Nik, Saeid; Nik-Amini, Saied; Nadesan, Puviindran; Stanford, William L; Alman, Benjamin A

    2010-10-01

    The cellular origins from which most tumors arise are poorly defined, especially in mesenchymal neoplasms. Aggressive fibromatosis, also known as desmoid tumor, is a locally invasive soft tissue tumor that has mesenchymal characteristics. We found that aggressive fibromatosis tumors express genes and cell surface markers characteristic of mesenchymal stem cells (MSC). In mice that are genetically predisposed to develop aggressive fibromatosis tumors (Apc(wt/1638N)), we found that the number of tumors formed was proportional to the number of MSCs present. Sca-1(-/-) mice, which develop fewer MSCs, were crossed with Apc(wt/1638N) mice. Doubly mutant mice deficient in Sca-1 developed substantially fewer aggressive fibromatosis tumors than wild-type (WT) littermates, but Sca-1 deficiency had no effect on the formation of epithelial-derived intestinal polyps. MSCs isolated from Apc(wt/1638N) mice (or mice expressing a stabilized form of β-catenin) induced aberrant cellular growth reminiscent of aggressive fibromatosis tumors after engraftment to immunocompromised mice, but WT cells and mature fibroblasts from the same animals did not. Taken together, our findings indicate that aggressive fibromatosis is derived from MSCs, and that β-catenin supports tumorigenesis by maintaining mesenchymal progenitor cells in a less differentiated state. Protecting this progenitor cell population might prevent tumor formation in patients harboring a germline APC mutation, where fibromatosis is currently the leading cause of mortality. © 2010 AACR.

  17. Anterior Abdominal Wall Desmoids Tumor in a Five Year Old Girl – A ...

    African Journals Online (AJOL)

    xp

    Pediatric Surgery Unit, Department of Surgery, Lagos University Teaching Hospital, Idi Araba, Lagos. ABSTRACT: ... by the parents. ... history of weight loss, fever or swelling in other parts .... management of desmoid tumors: how important is a.

  18. The role of APC and beta-catenin in the aetiology of aggressive fibromatosis (desmoid tumors)

    NARCIS (Netherlands)

    Lips, D.J.; Barker, N.; Clevers, H.; Hennipman, A.

    2009-01-01

    BACKGROUND: Aggressive fibromatosis (syn. desmoid tumor) is a sporadically occurring neoplastic proliferation of fibroblasts originating from musculoaponeurotic planes, forming invasively growing masses without the capability to metastasize. The choice of treatment remains surgical resection with or

  19. Surgical Treatment of Intra-Abdominal Desmoid Tumors Resulting In Short Bowel Syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Wheeler, Matthew; Mercer, David; Grant, Wendy; Botha, Jean; Langnas, Alan; Thompson, Jon, E-mail: jthompso@unmc.edu [Department of Surgery, University of Nebraska Medical Center, The Nebraska Medical Center 3280, Omaha, NE 68198 (United States)

    2012-01-19

    Advanced intra-abdominal desmoids tumors present with severe symptoms, complications or rapid growth, which lead to adverse outcomes. Our aim was to evaluate the treatment and outcome of patients with advanced intra-abdominal desmoids tumors, and develop guidelines for surgical management of these patients. We reviewed the clinical courses of 21 adult patients with advanced stage intra-abdominal desmoid tumors who presented to an intestinal rehabilitation and transplantation program. Patients with massive intestinal resection presented in two groups. The first group had a short small intestinal remnant after resection (<60 cm). These patients were poor rehabilitation candidates and eventually met criteria for transplant. The second had longer intestinal remnants and were more successfully rehabilitated and have not had complications that would lead to transplantation. Advanced intra-abdominal desmoid tumors have outcomes after resection that merit aggressive resection and planned intestinal rehabilitation and intestinal transplantation as indicated.

  20. An unusual case of fatty liver in a patient with desmoid tumor

    Institute of Scientific and Technical Information of China (English)

    Francesca De Felice; Daniela Musio; Rossella Caiazzo; Bartolomeo Dipalma; Lavinia Grapulin; Camilla Proietti Semproni; Vincenzo Tombolini

    2012-01-01

    A desmoid tumor,also known as aggressive fibromatosis,is a rare benign neoplasm that arises from fascial or musculoaponeurotic tissues.It can occur in any anatomical location,most commonly the abdominal wall,shoulder girdle and retroperitoneum.The typical clinical presentation is a painless mass with a slow and progressive invasion of contiguous structures.It is associated with a high local recurrence rate after resection.Many issues regarding the optimal treatment of desmoid tumors remain controversial.Aggressive surgical resection with a wide margin (2-3 cm) remains the gold standard treatment with regard to preserving quality of life.Radiotherapy alone has been shown to be effective for the control of unresectable or recurrent lesions.Desmoid tumors tend to be locally infiltrative,therefore,the fields must be generous to prevent marginal recurrence.The radiation dose appropriate for treating desmoid tumors remains controversial.We present a 25-year-old Caucasian man with local recurrence of a desmoid tumor after repeated surgical resection,treated with radiotherapy.The patient achieved complete tumor regression at 4 mo after radiotherapy,and he is clinically free of disease at 12 mo after the end of treatment,with an acceptable quality of life.The patient developed short bowel syndrome as a complication of second surgical resection.Consequently,radiotherapy might have worsened an already present malabsorption and so led to steatohepatitis.

  1. Desmoid Tumor of the Anterior Abdominal Wall in Female Patients: Comparison with Endometriosis

    Directory of Open Access Journals (Sweden)

    H. Krentel

    2012-01-01

    Full Text Available In female patients presenting a tumor of the lower abdominal wall especially after cesarian section, an endometriotic tumor as well as an aggressive desmoid tumor should be considered. Symptoms in correlation with the monthly period can facilitate the presurgical differentiation between endometriosis and fibromatosis. Ultrasound reveals the typical location of both tumors and its remarkable sonographic appearance. In the clinical practice, the desmoid fibromatosis of the lower abdominal wall is a very rare disease. We present a case of a 25-year-old pregnant and discuss diagnostic and therapeutic options by a PubMed literature review. With the knowledge of the prognosis of the desmoid fibromatosis and the respective treatment options including wait and see, complete surgical resection with macroscopically free margins and adjuvant approaches is essential to avoid further interventions and progression of the locally destructive tumor.

  2. Family history, surgery, and APC mutation are risk factors for desmoid tumors in familial adenomatous polyposis: an international cohort study

    DEFF Research Database (Denmark)

    Nieuwenhuis, Marry H; Lefevre, Jérémie H; Bülow, Steffen

    2011-01-01

    Ability to identify patients with familial adenomatous polyposis who have a high risk of developing desmoid tumors may affect decisions in clinical practice.......Ability to identify patients with familial adenomatous polyposis who have a high risk of developing desmoid tumors may affect decisions in clinical practice....

  3. Abdominal Wall Desmoid Tumor in a Pregnant Woman and Cesarean Section Managment

    Directory of Open Access Journals (Sweden)

    M Mojibian

    2013-08-01

    Full Text Available Increased risk of sporadic desmoid tumor occurs in increased estrogen level (pregnancy and surgical incisions (abdominal and thorasic. The frequency of desmoid tumors in the general population is 2.4 to 4.3 cases. The case is 30 year old woman with history of previous cesarean section. In fourth month of pregnancy,ultrasonography revealed a 5×7 cm mass in lower segment of the uterus(leiomyoma. The tumor diameter was 20 cm in term gestation. The time of cesarean , incision of skin was done above the umbilicus and below the sternum and incision of uterus was done from fondus vertically down. After delivery, the mass which was separated from uterus and located in the abdominal wall was extracted. The histological investigation diagnosed a desmoid tumor.

  4. A case of a desmoid tumor with an uretertumoral fisttula detected on renal scintigraphy

    Energy Technology Data Exchange (ETDEWEB)

    Yoo, Hyun Woo; Lee, Kyu Taek; Lee, Sang Mi [Soonchunhyang University Cheonan Hospital, Cheonan (Korea, Republic of)

    2016-11-15

    Desmoid tumors are rare benign tumors with aggressive fibroblastic proliferation. Although desmoid tumors do not metastasize, they have locally aggressive features and can cause a urinary fistula. Here, we report a case of a 35-year-old woman with Gardner syndrome who was diagnosed with an intra-abdominal desmoid tumor 1 year previously and who presented with a newly developed cystic mass lesion on a computed tomography scan. The cystic mass lesion was clinically diagnosed as an urinoma from the right ureterotumoral fistula; thus, surgical resection of the mass lesion was planned. However, Tc-99m diethylenetriamine pentaacetic acid renal scintigraphy revealed bilateral ureterotumoral fistulas; hence, the treatment plan was changed to conservative management.

  5. Morphologic Spectrum of Desmoid-Type Fibromatosis.

    Science.gov (United States)

    Zreik, Riyam T; Fritchie, Karen J

    2016-03-01

    Desmoid-type fibromatosis is a locally aggressive neoplasm composed of long sweeping fascicles of bland fibroblasts/myofibroblasts. Tumors with classic features are easy to identify, but the morphologic spectrum of this entity spans beyond the conventional histologic pattern. Since sampling of an unrecognized histologic variant may lead to misdiagnosis, especially on small needle biopsy specimens, we sought to fully characterize the morphologic spectrum of this entity. We examined 165 cases of desmoid-type fibromatosis and catalogued the morphologic patterns and percentage of those patterns identified in each case. Clinicopathologic variables, including age, sex, location, and recurrence, were analyzed and correlated with pattern frequency. Seven morphologic patterns were identified, including conventional, hyalinized/hypocellular, staghorn vessel, myxoid, keloidal, nodular fasciitis-like, and hypercellular. The mean number of patterns per case was two, but some cases harbored up to five patterns. The greatest morphologic variability was seen in the intra-abdominal lesions, with these tumors having a significantly higher percentage of the keloidal and staghorn patterns compared with extra-abdominal and abdominal wall sites. No significant correlation between patient outcome (recurrence) and presence of variant morphologic pattern was observed (P = .549). The morphologic spectrum of desmoid-type fibromatosis is deceptively broad, and awareness of the variety of histologic patterns is critical for accurate diagnosis. © American Society for Clinical Pathology, 2016. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

  6. Desmoid tumors in a dutch cohort of patients with familial adenomatous polyposis.

    NARCIS (Netherlands)

    Nieuwenhuis, M.H.; Cappel, W De Vos Tot Nede; Botma, A.; Nagengast, F.M.; Kleibeuker, J.H.; Mathus-Vliegen, E.M.H.; Dekker, E. den; Dees, J.; Wijnen, J.; Vasen, H.F.

    2008-01-01

    BACKGROUND & AIMS: Desmoid tumors are a severe extracolonic manifestation in familial adenomatous polyposis (FAP). Identification of risk factors might be helpful in the management of FAP patients with such tumors. The aim of this study was to assess potential risk factors for the development of des

  7. Desmoid tumors in a dutch cohort of patients with familial adenomatous polyposis

    NARCIS (Netherlands)

    Nieuwenhuis, M.H.; Vos to Nederveen Cappel, de W.; Botma, A.; Nagengast, F.M.; Kleibeuker, J.H.; Mathus-Vliegen, E.M.; Dekker, E.; Dees, J.; Wijnen, J.; Vasen, H.F.

    2008-01-01

    Background & Aims: Desmoid tumors are a severe extracolonic manifestation in familial adenomatous polyposis (FAP). Identification of risk factors might be helpful in the management of FAP patients with such tumors. The aim of this study was to assess potential risk factors for the development of

  8. An Intra-Abdominal Desmoid Tumor, Embedded in the Pancreas, Preoperatively Diagnosed as an Extragastric Growing Gastrointestinal Stromal Tumor

    Directory of Open Access Journals (Sweden)

    Mari Mizuno

    2017-04-01

    Full Text Available A 45-year-old woman was found to have a pancreatic tumor by abdominal ultrasound performed for a medical check-up. Abdominal contrast-enhanced computed tomography showed a hypovascular tumor measuring 30 mm in diameter in the pancreatic tail. Endoscopic ultrasound-guided fine needle aspiration was performed. An extragastric growing gastrointestinal stromal tumor was thereby diagnosed preoperatively, and surgical resection was planned. Laparoscopic surgery was attempted but conversion to open surgery was necessitated by extensive adhesions, and distal pancreatectomy, splenectomy, and partial gastrectomy were performed. The histological diagnosis was an intra-abdominal desmoid tumor. A desmoid tumor is a fibrous soft tissue tumor arising in the fascia and musculoaponeurotic tissues. It usually occurs in the extremities and abdominal wall, and only rarely in the abdominal cavity. We experienced a case with an intra-abdominal desmoid tumor that was histologically diagnosed after laparotomy, which had been preoperatively diagnosed as an extragastric growing gastrointestinal stromal tumor. Although rare, desmoid tumors should be considered in the differential diagnosis of intra-abdominal tumors. Herein, we report this case with a literature review.

  9. Dacarbazine-Doxorubicin therapy ameliorated an extremely aggressive mesenteric desmoid tumor associated with familial adenomatous polyposis: report of a case.

    Science.gov (United States)

    Ezumi, Koji; Yamamoto, Hirofumi; Takemasa, Ichiro; Nomura, Masaya; Ikeda, Masataka; Sekimoto, Mitsugu; Monden, Morito

    2008-03-01

    A 30-year-old man with familial adenomatous polyposis (FAP) underwent prophylactic proctocolectomy by laparoscopy-assisted surgery. After 10 months, we found an intra-abdominal tumor, which grew rapidly to 25 cm in diameter. We performed an emergency operation, which revealed that it was a desmoid tumor derived mainly from colorectal mesenterium. The tumor was removed with three short segments of intestine and the left ureter. A computed tomography (CT) scan done 3 months later showed a 10 cm mesenteric desmoid tumor at the beginning of jejunum, approaching the root of the superior mesenteric artery (SMA). Fortunately, we were able to remove the tumor without injuring the SMA. To our distress, however, another recurrent mesenteric desmoid tumor was discovered in the pelvis one month later, which grew rapidly from 5 cm to 16 cm within 4 months. During this period, we gave the patient several regimens, including antiestrogen (tamoxifen), a nonsteroidal anti-inflammtory drug and imatinib mesylate (Gleevec), which had little or no effect. Finally, when the desmoid occupied the pelvic space, we gave the patient dacarbazine (DTIC) and doxorubicin (DOX). After seven courses, the mesenteric tumor showed an almost complete response (CR). The chemotherapy caused grade 3 to 4 leukocytopenia, but without any hazardous events. No evidence of further recurrence of mesenteric desmoid has been seen for 4 years. This combination chemotherapy is a promising strategy, even against an extremely aggressive, life-threatening mesenteric desmoid associated with FAP.

  10. Giant Desmoid Tumor and Gardner Syndrome. Case Report and Literature Review

    Directory of Open Access Journals (Sweden)

    Etcheverry MG

    2016-06-01

    Full Text Available Gardner´s syndrome represents a variant of the genetic disorder called familial adenomatous polyposis (FAP. The inherited pattern is autosomal dominant, however 20-25% of cases may represent new mutations. It is characterized by colonic polyposis with extracolonic manifestations as gastro-duodenal polyposis, osteomas, dental abnormalities and desmoid tumors.We report a case of a 25 years old man with family history of multiple surgeries caused by desmoid tumors without personal history. He visited our hospital complaining of a large tumor in the abdominal wall, and during the preoperative studies we identified colonic and gastroduodenal polyposis. Tumor resection was performed with safety margins that included the entire abdominal wall with total colectomy, ileal-rectal anastomosis and abdominal wall replacement with a protection visceral mesh. Gardner´s syndrome is a rare entity that is important to identify when we have a patient presenting with a desmoid tumor as in this case. Its association with colonic polyposis with high risk of malignant change demand an early aggressive treatment that will determine the survival of the patient.

  11. A Patient With Desmoid Tumors and Familial FAP Having Frame Shift Mutation of the APC Gene

    Directory of Open Access Journals (Sweden)

    Sanambar Sadighi

    2017-02-01

    Full Text Available Desmoids tumors, characterized by monoclonal proliferation of myofibroblasts, could occur in 5-10% of patients with familial adenomatous polyposis (FAP as an extra-colonic manifestation of the disease. FAP can develop when there is a germ-line mutation in the adenomatous polyposis coli gene. Although mild or attenuated FAP may follow mutations in 5΄ extreme of the gene, it is more likely that 3΄ extreme mutations haveamore severe manifestation of thedisease. A 28-year-old woman was admitted to the Cancer Institute of Iran with an abdominal painful mass. She had strong family history of FAP and underwent prophylactic total colectomy. Pre-operative CT scans revealed a large mass. Microscopic observation showed diffuse fibroblast cell infiltration of the adjacent tissue structures. Peripheral blood DNA extraction followed by adenomatous polyposis coli gene exon by exon sequencing was performed to investigate the mutation in adenomatous polyposis coli gene. Analysis of DNA sequencing demonstrated a mutation of 4 bpdeletions at codon 1309-1310 of the exon 16 of adenomatous polyposis coli gene sequence which was repeated in 3 members of the family. Some of them had desmoid tumor without classical FAP history. Even when there is no familial history of adenomatous polyposis, the adenomatous polyposis coli gene mutation should be investigated in cases of familial desmoids tumors for a suitable prevention. The 3΄ extreme of the adenomatous polyposis coli gene is still the best likely location in such families.

  12. Tailoring Desmoid Treatment

    NARCIS (Netherlands)

    D.L.M. van Broekhoven (Danique)

    2017-01-01

    markdownabstractDesmoid-type fibromatosis (DF) is a rare disease, also known as aggressive fibromatosis or desmoid tumor. DF is characterized by infiltrative growth with a tendency toward local recurrence but an inability to metastasize. The incidence of DF is low. The pathogenesis of this rare dise

  13. Radiation-induced Sarcomas Occurring in Desmoid-type Fibromatosis Are Not Always Derived From the Primary Tumor.

    Science.gov (United States)

    Verschoor, Arie J; Cleton-Jansen, Anne-Marie; Wijers-Koster, Pauline; Coffin, Cheryl M; Lazar, Alexander J; Nout, Remi A; Rubin, Brian P; Gelderblom, Hans; Bovée, Judith V M G

    2015-12-01

    Desmoid-type fibromatosis is a rare, highly infiltrative, locally destructive neoplasm that does not metastasize, but recurs often after primary surgery. Activation of the Wnt/β-catenin pathway is the pathogenic mechanism, caused by an activating mutation in exon 3 of CTNNB1 (85% of the sporadic patients). Radiotherapy is a frequent treatment modality with a local control rate of approximately 80%. In very rare cases, this may result in the development of radiation-induced sarcoma. It is unclear whether these sarcomas develop from the primary tumor or arise de novo in normal tissue. In 4 tertiary referral centers for sarcoma, 6 cases of desmoid-type fibromatosis that subsequently developed sarcoma after radiotherapy were collected. The DNA sequence of CTNNB1 exon 3 in the desmoid-type fibromatosis and the subsequent postradiation sarcoma was determined. Sarcomas developed 5 to 21 years after the diagnosis of desmoid-type fibromatosis and included 2 osteosarcomas, 2 high-grade undifferentiated pleomorphic sarcomas, 1 fibrosarcoma, and 1 undifferentiated spindle cell sarcoma. Three patients showed a CTNNB1 hotspot mutation (T41A, S45F, or S45N) in both the desmoid-type fibromatosis and the radiation-induced sarcoma. The other 3 patients showed a CTNNB1 mutation in the original desmoid-type fibromatosis (2 with a T41A and 1 with an S45F mutation), which was absent in the sarcoma. In conclusion, postradiation sarcomas that occur in the treatment area of desmoid-type fibromatosis are extremely rare and can arise through malignant transformation of CTNNB1-mutated desmoid fibromatosis cells, but may also originate from CTNNB1 wild-type normal cells lying in the radiation field.

  14. Radiotherapy in desmoid tumors. Treatment response, local control, and analysis of local failures

    Energy Technology Data Exchange (ETDEWEB)

    Santti, Kirsi; Beule, Annette; Tuomikoski, Laura; Jaeaeskelaeinen, Anna-Stina; Saarilahti, Kauko; Tarkkanen, Maija; Blomqvist, Carl [Helsinki University Hospital and University of Helsinki, Comprehensive Cancer Center, Helsinki (Finland); Roenty, Mikko [HUSLAB and University of Helsinki, Department of Pathology, Helsinki (Finland); Ihalainen, Hanna [Helsinki University Hospital and University of Helsinki, Department of Plastic Surgery, Helsinki (Finland)

    2017-04-15

    Desmoid tumors (aggressive fibromatosis) are rare soft tissue tumors which frequently recur after surgery. Desmoid tumors arise from musculoaponeurotic tissue in the extremities, head and neck, abdominal wall, or intra-abdominally. Our aim was to examine the outcome of radiotherapy of desmoid tumors in a single institution series. We evaluated 41 patients with desmoid tumors treated with 49 radiotherapies between 1987 and 2012. Radiologic images for response evaluation were reassessed and responses to treatment registered according to RECIST criteria 1.1. For patients with local failures radiation dose distribution was determined in each local failure volume using image co-registration. Recurrences were classified as in-target, marginal, or out-of-target. Prognostic factors for radiotherapy treatment failure were evaluated. Radiotherapy doses varied from 20-63 Gy (median 50 Gy) with a median fraction size of 2 Gy. The objective response rate to definitive radiotherapy was 55% (12/22 patients). Median time to response was 14 months. A statistically significant dose-response relation for definitive and postoperative radiotherapy was observed both in univariate (p-value 0.002) and in multivariate analysis (p-value 0.02) adjusted for potential confounding factors. Surgery before radiotherapy or surgical margin had no significant effect on time to progression. Nine of 11 (82%) local failures were classified as marginal and two of 11 (18%) in-target. None of the recurrences occurred totally out-of-target. Radiotherapy is a valuable option for treating desmoid tumors. Radiotherapy dose appears to be significantly associated to local control. (orig.) [German] Desmoide (aggressive Fibromatosen) sind seltene Weichteiltumore der muskulaeren Membranen von Kopf, Hals, Extremitaeten und Bauchwand. Ziel war es, die Wirksamkeit der Strahlentherapie bei aggressiver Fibromatose an einer einzelnen Klinik zu untersuchen. Ausgewertet wurden 41 Patienten mit aggressiver Fibromatose, die

  15. ABDOMINAL WALL DESMOID TUMOUR OVER APPENDICECTOMY SCAR

    OpenAIRE

    Vijaya; Sarbeshwar; Gogoi

    2015-01-01

    BACKGROUND: Desmoid tumors are slow growing deep fibromatoses with aggressive infiltration of adjacent tissue but without any metastatic potential . (1,2,3) CASE PRESENTATION: We report a female patient with desmoid tumor of the abdominal wall over appendicectomy scar w ho underwent primary resection. Preoperative evaluation incl uded abdominal ultrasound, and computed tomography. The histology of this cases revealed a desmoid tumor. CONCLUSION: ...

  16. Synchronous multicentric desmoid tumors (aggressive fibromatosis) of the extremities

    Energy Technology Data Exchange (ETDEWEB)

    Sundaram, M.; Duffrin, H.; Vas, W.; McGuire, M.H.

    1988-01-01

    Synchronous multicentric aggressive fibromatosis does not appear to have been previously reported. Two such cases are described. The tumors were identified by magnetic resonance (MR) imaging. The incidence of synchronous multicentric aggressive fibromatosis is not known. It is anticipated that increased use of coronal MR imaging will reveal more tumors of this type, both synchronous and metachronous. In a patient with known or suspected aggressive fibromatosis, every other soft tissue nodule or mass in the same limb has to be regarded as an additional tumor of the same histology. (orig.)

  17. [Proctocolectomy with ileoanal anastomoses and desmoid tumor treated with resection. One case of familial adenomatous polyposis].

    Science.gov (United States)

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

    2014-01-01

    Introducción: la poliposis adenomatosa familiar (PAF) es una rara enfermedad causada por una mutación en el gen de la poliposis adenomatosa coli (APC). Caso clínico: mujer de 32 años, con dolor y aumento del perímetro abdominal además de evacuaciones melénicas y pérdida de peso. La paciente presentó un tumor de 12 cm de diámetro en la fosa iliaca derecha. Tras la administración de medio de contraste, en una tomografía se apreció el tumor abdominal con reforzamiento compatible con sarcoma frente a tumor desmoide. Se realizó colonoscopia, por medio de la que se encontraron pólipos en el recto y el colon. La biopsia reportó adenomas túbulo-vellosos. Una panendoscopía demostró pólipos en fondo y cuerpo gástrico; el duodeno se encontraba en estado normal. Se realizó resección del tumor en pared abdominal y reconstrucción con malla además de proctocolectomía restaurativa con un reservorio íleo-anal con una ileostomía temporal. Se reportó tumor desmoide en la pared abdominal y se identificaron 152 pólipos túbulo-vellosos que afectaban todas las porciones del colon y el recto. Conclusiones: la PAF es una enfermedad autosómica dominante causada por una mutación en el gen APC que da como resultado el desarrollo de múltiples pólipos tanto en el colon como en el recto. Descrito en 1991, el gen APC se localiza en el cromosoma 5q21. Sin cirugía profiláctica, todos los pacientes desarrollarán cáncer colorrectal en la tercera década de la vida. Los tumores desmoides y los pólipos duodenales son ahora la causa de muerte en los pacientes con PAF.

  18. [A recurrent desmoid tumor of the popliteal fossa with vascular compromise].

    Science.gov (United States)

    Díaz Hernández, M; Febles, G; Domínguez del Toro, M; Reboso Morales, L E; Llopart López, J R; Gambra Michel, L E

    1993-01-01

    We report a case of a 18-year-old male patient with his fourth relapse of a desmoid tumour of the left popliteal hollow after a traumatism in this area. Arterial displacement and extrinsic compression were showed by arteriography. Tumoration was extirpated including popliteal artery, in this way a terminal by-pass from distal femoral to peroneo-tibial trunks with contralateral great saphena was made. Diagnosis of intestinal polyposis and of the little osseous malformations, was discarded these pathologies frequently are related with these tumours. The research for 8 years and 4 months doesn't show any relapse, nor local neither multicentral. Furthermore, some considerations about this theme are reported.

  19. Role of technetium-99m pertechnetate scintigraphy in the management of extra-abdominal fibromatosis

    Energy Technology Data Exchange (ETDEWEB)

    Terui, Shoji [Div. of Nuclear Medicine, National Cancer Hospital, Tokyo (Japan); Terauchi, Takashi [Div. of Nuclear Medicine, National Cancer Hospital, Tokyo (Japan); Abe, Hiroyuki [Div. of Nuclear Medicine, National Cancer Hospital, Tokyo (Japan); Muramatsu, Yukio [Div. of Diagnostic Radiology, National Cancer Hospital, Tokyo (Japan); Fukuma, Hisatoshi [Div. of Orthopedics, National Cancer Center Hospital, Tokyo (Japan); Beppu, Yasuo [Div. of Orthopedics, National Cancer Center Hospital, Tokyo (Japan); Yokoyama, Ryohei [Div. of Orthopedics, National Cancer Center Hospital, Tokyo (Japan)

    1995-07-01

    The purpose of this study was to investigate technetium-99m pertechnetate (Tc-99m) as a tumorscanning agent in patients with extra-abdominal fibromatosis, and to establish the sensitivity of this type of scintigraphy. Eleven patients with extra-abdominal fibromatosis were studied: all but one having postsurgical recurrences. Of the 11 patients, diagnosed histologically, 5 underwent repeated Tc-99m scintigraphic follow-up examinations. The injected 370 MBq Tc-99m gave us an early scintigram within 10 min and a delayed one 2 h later. For adequate comparison, the region of interest (ROI) of the scintigram was placed over the tumor. The tumor-to-background (T/BG) count ratio was computed. Extra-abdominal fibromatoses, even recurrences, were demonstrated scintigraphically in both the early and the delayed phase, in all 11 patients. The average T/BG ratio was 2.11 in the early scintigram and 2.15 in the delayed one. The sensitivity and the specificity were both 100%. Tc-99m scintigraphy has proved useful in detecting extra-abdominal fibromatoses and in the follow-up of patients. (orig.)

  20. CTNNB1 S45F mutation predicts poor efficacy of meloxicam treatment for desmoid tumors: a pilot study.

    Science.gov (United States)

    Hamada, Shunsuke; Futamura, Naohisa; Ikuta, Kunihiro; Urakawa, Hiroshi; Kozawa, Eiji; Ishiguro, Naoki; Nishida, Yoshihiro

    2014-01-01

    We hypothesized that patterns of CTNNB1 (β-catenin) mutations would affect the outcome of conservative therapy in patients with desmoid tumors. This study aimed to determine the significance of CTNNB1 (β-catenin) mutations in predicting the treatment outcome in patients with desmoid tumors treated with meloxicam, a cyclooxygenase-2 (COX-2) selective inhibitor. Between 2003 and 2012, consecutive thirty-three patients with extra-peritoneal sporadic desmoid tumors were prospectively treated with meloxicam as the initial systemic medical therapy. The efficacy of meloxicam was evaluated according to Response Evaluation Criteria in Solid Tumors (RECIST). DNA was isolated from frozen tissue or formalin-fixed materials. CTNNB1 mutation analysis was performed by direct sequencing. Positivity of nuclear β-catenin staining by immunohistochemistry was compared with the status of CTNNB1 mutations. The correlation between the efficacy of meloxicam treatment and status of CTNNB1 mutations was analyzed. Of the 33 patients with meloxicam treatment, one showed complete remission (CR), 7 partial remission (PR), 12 stable disease (SD), and 13 progressive disease (PD). The following 3 point mutations were identified in 21 of the 33 cases (64%): T41A (16 cases), S45F (4 cases) and S45P (one case). The nuclear expression of β-catenin correlated significantly with CTNNB1 mutation status (p = 0.035); all four cases with S45F mutation exhibited strong nuclear expression of β-catenin. S45F mutation was significantly associated with a poor response (all cases; PD) (p = 0.017), whereas the other mutations had no impact on efficacy. The CTNNB1 mutation status was of significant prognostic value for meloxicam treatment in patients with sporadic desmoid tumors.

  1. CTNNB1 S45F mutation predicts poor efficacy of meloxicam treatment for desmoid tumors: a pilot study.

    Directory of Open Access Journals (Sweden)

    Shunsuke Hamada

    Full Text Available We hypothesized that patterns of CTNNB1 (β-catenin mutations would affect the outcome of conservative therapy in patients with desmoid tumors. This study aimed to determine the significance of CTNNB1 (β-catenin mutations in predicting the treatment outcome in patients with desmoid tumors treated with meloxicam, a cyclooxygenase-2 (COX-2 selective inhibitor. Between 2003 and 2012, consecutive thirty-three patients with extra-peritoneal sporadic desmoid tumors were prospectively treated with meloxicam as the initial systemic medical therapy. The efficacy of meloxicam was evaluated according to Response Evaluation Criteria in Solid Tumors (RECIST. DNA was isolated from frozen tissue or formalin-fixed materials. CTNNB1 mutation analysis was performed by direct sequencing. Positivity of nuclear β-catenin staining by immunohistochemistry was compared with the status of CTNNB1 mutations. The correlation between the efficacy of meloxicam treatment and status of CTNNB1 mutations was analyzed. Of the 33 patients with meloxicam treatment, one showed complete remission (CR, 7 partial remission (PR, 12 stable disease (SD, and 13 progressive disease (PD. The following 3 point mutations were identified in 21 of the 33 cases (64%: T41A (16 cases, S45F (4 cases and S45P (one case. The nuclear expression of β-catenin correlated significantly with CTNNB1 mutation status (p = 0.035; all four cases with S45F mutation exhibited strong nuclear expression of β-catenin. S45F mutation was significantly associated with a poor response (all cases; PD (p = 0.017, whereas the other mutations had no impact on efficacy. The CTNNB1 mutation status was of significant prognostic value for meloxicam treatment in patients with sporadic desmoid tumors.

  2. Giant desmoid tumor of the abdominal wall in a patient with Gardner Syndrome

    Directory of Open Access Journals (Sweden)

    Daniel Paulino Santana

    2012-09-01

    Full Text Available Gardner syndrome (GS is a rare entity characterized by a triad of familial colonic polyposis, multiple osteomas and soft tissue tumors, including desmoid tumor (DT. This is a case report of a 30 year-old patient with GS who developed giant DT in the abdominal wall after undergoing several laparotomies. The patient has taken a long time to search for medical care, and at first he saw another team that refused to operate him by judging the lesion unresectable. The surgery in our department was performed in three steps. Initially, we resected the lesion with macroscopic margins, and as there were small bowel adhesions in the tumor, we performed enterectomy and closed using the "Bogotá" technique, with skin closure on the bag. On the fourth postoperative day (POD, we reoperated the abdomen without identifying any signs of fistula. On the seventh POD there was another surgical intervention, this time to insert a double-sided mesh. The patient recovered well, and had no debilitating motor deficit, despite the extensive resection of the abdominal muscles. Curative treatment of DT is based on surgical resection and only sequential surveillance allows us an early diagnosis, when the lesion is still resectable.Tumor desmoide gigante de parede abdominal em paciente portador da Síndrome de Gardner. A Síndrome de Gardner (SG é uma entidade rara caracterizada pela tríade polipose colônica familial, múltiplos osteomas e tumores de tecidos moles, dentre eles o tumor desmoide (TD. Tratou-se de um relato de caso de um paciente de 30 anos, com SG que evoluiu com TD gigante em parede abdominal, após ser submetido a diversas laparotomias prévias. O paciente levou longo tempo para procurar o serviço de cirurgia, passando por outra equipe que se negou a abordá-lo por julgar a lesão irressecável. A cirurgia no nosso serviço se deu em três tempos. Inicialmente, foi feita a ressecção da lesão com margens macroscópicas e, por haver aderências de al

  3. Desmoid Tumor Associated With Familial Adenomatous Polyposis: Evaluation With 64-Detector CT Enterography

    Directory of Open Access Journals (Sweden)

    Oktay Algin

    2012-03-01

    Full Text Available Desmoid tumors (DTs are benign tumors which are not seen very often, and most of the radiologists and clinicians do not know the characteristics of them very well. Correct and early diagnosis of DTs is important for decreasing mortality and morbidity. Computed tomography enterography (CTE is a new modality for small bowel imaging which combines the improved spatial and temporal resolution of multidetector computed tomography (CT with large volumes of ingested enteric contrast material to permit evaluation of the small bowel wall and lumen and also the entire abdomen. We report a familial adenomatous polyposis (FAP patient with localized mesentery and abdominal wall DTs. We showed the exact location of the DTs and their relation with the small bowel by CTE. In conclusion, CTE is a useful technique for DT localization, the degree of extension and invasion to local structures, presence of partial and complete small bowel obstruction, and the relationship of the tumors with vasculature and whether ischemia ha s occurred as a result or not.

  4. Desmoid fibromatosis: MRI features of response to systemic therapy

    Energy Technology Data Exchange (ETDEWEB)

    Sheth, Pooja J.; Subhawong, Ty K. [University of Miami Miller School of Medicine/Jackson Memorial Hospital, Department of Radiology, Miami, FL (United States); Del Moral, Spencer; Wilky, Breelyn A.; Trent, Jonathan C. [University of Miami Miller School of Medicine/Sylvester Comprehensive Cancer Center, Division of Hematology/Oncology, Department of Medicine, Miami, FL (United States); Cohen, Jonathan [Oncology and Radiation Associates, Miami, FL (United States); Rosenberg, Andrew E. [University of Miami Miller School of Medicine, Department of Pathology, Miami, FL (United States); Temple, H.T. [Center for Orthopedic Innovations, Miami, FL (United States)

    2016-10-15

    Imaging criteria for measuring the response of desmoid fibromatosis to systemic therapy are not well established. We evaluated a series of patients with desmoids who underwent systemic therapy to document magnetic resonance imaging (MRI) features associated with a positive clinical response. This Institutional Review Board-approved retrospective study included 23 patients (mean age 40.5) with 29 extra-abdominal tumors. Therapeutic regimens included cytotoxic chemotherapy (n = 19), targeted therapy (n = 3), and nonsteroid anti-inflammatory drugs (NSAIDS; n = 1). Clinical effects were categorized as progressive disease, stable, or partial response. Maximum tumor dimension (D{sub max}), approximate tumor volume (V{sub Tumor}), and quantitative tumor T2 hyperintensity and contrast enhancement (relative to muscle) for pre- and post-treatment MRIs were compared. Three lesions progressed, 5 lesions were stable, whereas 21 showed a clinical response. D{sub max} decreased more in responders (mean -11.0 %) than in stable/progressive lesions (mean -3.6 and 0 % respectively, p = 0.28, ANOVA); by Response Evaluation Criteria in Solid Tumors (RECIST 1.1) 27 out of 29 lesions were ''stable,'' including the 3 progressive lesions. In responders, V{sub Tumor} change averaged -29.4 %, but -19.2 % and +32.5 % in stable and progressive lesions respectively (p = 0.002, ANOVA); by 3D criteria 14 out of 29 lesions showed a partial response. T2 hyperintensity decreased by 50-54 % in partial response/stable disease, but only by 10 % in progressive lesions (p = 0.049, t test). Changes in contrast enhancement ranged from -23 % to 0 %, but were not statistically significant among response groups (p = 0.37). Change in T2 hyperintensity showed a positive correlation with volumetric change (r = 0.40). Decreases in volume and T2 hyperintensity reflect the positive response of desmoid fibromatosis to systemic therapy; RECIST 1.1 criteria are not sensitive to clinically

  5. Long-term outcome of sporadic and FAP-associated desmoid tumors treated with high-dose selective estrogen receptor modulators and sulindac: a single-center long-term observational study in 134 patients.

    Science.gov (United States)

    Quast, Daniel Robert; Schneider, Ralph; Burdzik, Emanuel; Hoppe, Steffen; Möslein, Gabriela

    2016-01-01

    Aim of this study is to evaluate the outcome of long-term conservative treatment with sulindac and high-dose selective estrogen receptor modulators (SERMs) for sporadic and FAP-associated desmoid tumors. Desmoids are very rare tumors in the general population but occur frequently in FAP patients, being encountered in 23-38 %. Treatment of desmoids is still most controversial since response cannot be predicted and they are prone to develop recurrence. This study included all desmoid patients that were treated and followed at our institution and had completed at least 1 year of treatment. Response was defined as stable size or regression of desmoid size between two CT or MRI scans. A total of 134 patients were included. 64 (47.8 %) patients had a confirmed diagnosis of FAP, 69 (51.5 %) patients were sporadic. Overall 114 (85.1 %) patients showed regressive or stable desmoid size. Patients with previous history of multiple desmoid-related surgeries showed less-favorable response. The mean time to reach at least stable size was 14.9 (±9.1) months. After regression or stabilization, medication was tapered in 69 (60.5 %) of the treated patients with only one long-term recurrence after >10 years. The results of this study fortify the role of sulindac and high-dose SERMs as an effective and safe treatment for both, sporadic and FAP-associated desmoid tumors. While invasive treatment frequently results in high recurrence rates, high morbidity and high mortality, this conservative treatment is successful in most patients. The recurrence rate is negligible with no desmoid-related mortality in this large series. Therefore surgical resection, especially for mesenteric desmoids, should be deferred favoring this convincingly effective, well tolerated regimen.

  6. Malignant granular cell tumor of the abdominal wall mimicking desmoid tumor: A case report with CT imaging findings and literature review

    Energy Technology Data Exchange (ETDEWEB)

    Yoon, Je Hong; Ahn, Sung Eun; Lee, Dong Ho; Park, Seong Jin; Moon, Sung Kyoung; Lim, Joo Won [Dept. Radiology, Kyung Hee University Hospital, Kyung Hee University School of Medicine, Seoul (Korea, Republic of)

    2016-08-15

    Granular cell tumors (GCTs) are extremely rare mesenchymal neoplasms of Schwann cell origin. Malignant GCTs (MGCTs) comprise 0.5-2% of all GCTs. In the present report, we describe a case of a 66-year-old man with MGCT of the abdominal wall. The patient visited our hospital due to a recently growing palpable soft tissue mass in the abdominal wall. Computed tomography scan revealed a 4.3 × 4.1 × 2.9 cm sized mass arising from the left abdominal wall, which was contemplated as a desmoid tumor before surgical excision. Histopathological examination confirmed MGCT.

  7. Case report 358: Desmoid tumor of the greater wing of the right sphenoid bone

    Energy Technology Data Exchange (ETDEWEB)

    Crisi, G.; Calo, M.; Mauri, C.

    1986-03-01

    In summary, a case has been presented of a 26-year-old man who on admission showed a mild right proptosis and swelling of the right temple. Roentgenograms of the skull demonstrated loss of the right innominate line. CT studies after contrast demonstrated a sharply-demarcated, destructive area of the right greater sphenoid wing with an enhancing, spindle-shaped, soft tissue mass observed around the bony defect. Increased uptake was demonstrated on a radionuclide scan. The lesion was surgically removed and proved to be a desmoid. (orig./SHA).

  8. [A Case of Familial Adenomatous Polyposis with a Desmoid Tumor Probably Communicating to the Intestinal Lumen That Was Successfully Treated with Non-Surgical Therapy].

    Science.gov (United States)

    Ito, Tetsuya; Chika, Noriyasu; Yamamoto, Azusa; Ogura, Toshiro; Amano, Kunihiko; Ishiguro, Toru; Fukuchi, Minoru; Kumagai, Youichi; Ishibashi, Keiichiro; Eguchi, Hidetaka; Okazaki, Yasushi; Mochiki, Erito; Ishida, Hideyuki

    2016-11-01

    A 44-year-old man with familial adenomatous polyposis underwent laparoscopic-assistedtotal proctocolectomy with ilealpouch anal anastomosis(IPAA). Computed tomography conducted 21 months after IPAA demonstrated bilateral hydronephrosis andan intra-abdominal mass with a maximal diameter of 22 cm, leading to a diagnosis of stage IV desmoid disease, according to the classification by Church and associates. Six courses of combination chemotherapy with doxorubicin plus dacarbazine were administered. Computed tomography after chemotherapy demonstrated marked shrinkage of the desmoidtumor with intraabdominal air andfluidcollection extending just below the skin of the ileostomy closure site. Stoollike fluidoverflowedspontaneously through the site of the ileostomy closure andthe abscess cavity was successfully drained. The patient was discharged 30 days after the start of drainage. The patient is doing well 10 months after the drainage without regrowth of the desmoid tumor, even though a cavity-like lesion encapsulatedby a thick wall remains.

  9. Positron Emission Tomography as a Surrogate Marker for Evaluation of Treatment Response in Patients with Desmoid Tumors under Therapy with Imatinib

    Directory of Open Access Journals (Sweden)

    Bernd Kasper

    2013-01-01

    Full Text Available We used 2-deoxy-2-[18F] fluoro-D-glucose (FDG positron emission tomography (PET to evaluate patients with desmoid tumors undergoing therapy with imatinib. The study included 22 patients with progressive disease (PD of a biopsy proven desmoid tumor treated orally with imatinib 800 mg daily. Patients were examined using PET prior to onset of therapy and during treatment. Restaging was performed in parallel using computed tomography (CT and/or magnetic resonance imaging (MRI. Outcome of 22 evaluable patients was as follows: five patients with partial response (PR; twelve patients with stable disease (SD accounting for 77% with non-progressive disease; five patients showed PD. A 30% decrease of the mean average standardized uptake value (SUV of sequential PET examinations could be demonstrated; no patient demonstrated a substantial increase in SUV. Patients with PR/SD were matched to a group of nonprogressive disease and tested versus PD. The initial average SUV and seem to be candidates for a response prediction with an approximate -value of 0.06553 and 0.07785, respectively. This is the first larger series of desmoid patients monitored using PET showing that early SUV changes may help to discriminate responders from nonresponders and, thus, to decide whether imatinib therapy should be continued.

  10. Tumor desmóide da parede abdominal durante a gravidez: relato de caso Desmoid tumor of the abdominal wall during pregnancy: a case report

    Directory of Open Access Journals (Sweden)

    Denise Gonçalves Priolli

    2005-05-01

    Full Text Available Tumores desmóides são neoplasias do tecido conjuntivo, caracterizadas por apresentarem crescimento exclusivamente loco-regional, recorrência freqüente e mínimo potencial metastático. Acometem principalmente portadores de polipose adenomatosa familial dos cólons, sendo sua ocorrência isolada extremamente rara. São mais freqüentes nas mulheres em idade reprodutiva e durante a gravidez. Descreve-se um caso de tumor desmóide de grandes proporções, localizado na parede abdominal, que surgiu a partir da 17ª semana em gestante sem antecedentes de polipose adenomatosa familial. A neoplasia foi totalmente extirpada utilizando-se prótese de polipropileno para reconstituição da parede abdominal. Atualmente a doente encontra-se bem, um ano após a cirurgia, em uso de antiinflamatório não hormonal para prevenção de recidivas.Desmoid tumors are neoplasms of the conjunctive tissue that are characterized by exclusive locoregional growth, frequent recurrence and minimal metastatic potential. They mainly affect individuals with familial adenomatous polyposis of the colon, and rarely occur isolated. The single form of this neoplasm most frequently appears in women of reproductive age, and during pregnancy. A case of a desmoid tumor of large proportions located in the abdominal wall is described. It appeared at the 17th week of pregnancy in a woman without any history of familial adenomatous polyposis. The neoplasm was totally extirpated, with the use of a polypropylene prosthesis for reconstitution of the abdominal wall. One year after the surgery, the patient continues to be well, while using non-steroidal anti-inflammatory drugs for the prevention of relapses.

  11. Multiple desmoid tumors in a patient with familial adenomatous polyposis caused by the novel W421X mutation Tumor desmoide múltiple en un paciente con poliposis adenomatosa familiar originada por la nueva mutación W421X

    OpenAIRE

    Orestis Ioannidis; George Paraskevas; Stavros Chatzopoulos; Anastasios Kotronis; Nikolaos Papadimitriou; Athina Konstantara; Apostolos Makrantonakis; Emmanouil Kakoutis

    2012-01-01

    Familial adenomatous polyposis (FAP) is a rare syndrome characterized by the presence of hundreds to thousands of colorectal adenomas and is responsible for less than 1% of all colorectal cancers. The syndrome is also characterized by extra-colorectal features including amongst others upper gastrointestinal tract polyps and desmoid tumors. The syndrome is inherited by an autosomal dominant gene, the adenomatous polyposis coli (APC) gene. We present the physical history, clinical presentation,...

  12. Proton therapy versus photon radiation therapy for the management of a recurrent desmoid tumor of the right flank: a case report

    Directory of Open Access Journals (Sweden)

    Kil Whoon

    2012-10-01

    Full Text Available Abstract Desmoid tumors are benign mesenchymal tumors with a strong tendency for local recurrence after surgery. Radiotherapy improves local control following incomplete resection, but nearby organs at risk may limit the dose to the target volume. The patient in this report presented with a recurrent desmoid tumor of the right flank and underwent surgery with microscopically positive margins. Particular problems presented in this case included that the tumor bed was situated in close proximity to the liver and the right kidney and that the right kidney was responsible for 65% of the patient’s renal function. Intensity-modulated radiation therapy plans delivering 54 Gy necessarily exposed the right kidney to a V18 of 98% and the liver to a V30 of 55%. Proton therapy plans significantly reduced the right kidney V18 to 32% and the liver V30 to 28%. In light of this, the proton plan was utilized for treatment of this patient. Proton therapy was tolerated without gastrointestinal discomfort or other complaints. Twenty-four months after initiation of proton therapy, the patient is without clinical or radiographic evidence of disease recurrence. In this setting, the improved dose distribution associated with proton therapy allowed for curative treatment of a patient who arguably could not have been safely treated with intensity-modulated radiation therapy or other methods of conventional radiotherapy.

  13. Extra-abdominal venous thromboses at unusual sites.

    Science.gov (United States)

    Martinelli, Ida; De Stefano, Valerio

    2012-09-01

    Venous thrombosis typically involves the lower extremities. Rarely, it can occur in cerebral, splanchnic, or renal veins, with a frightening clinical impact. Other rare manifestations are upper-extremity deep vein thrombosis, that can complicate with pulmonary embolism and post-thrombotic syndrome, and retinal vein occlusion, significantly affecting the quality of life. This review is focused on venous thromboses at unusual extra-abdominal sites. Local infections or cancer are frequent in cerebral sinus-venous thrombosis. Upper-extremity deep vein thrombosis is mostly due to catheters or effort-related factors. Common risk factors are inherited thrombophilia and oral contraceptive use. Acute treatment is based on heparin; in cerebral sinus-venous thrombosis, local or systemic fibrinolysis should be considered in case of clinical deterioration. Vitamin-K antagonists are recommended for 3-6 months; indefinite anticoagulation is suggested for recurrent thrombosis or unprovoked thrombosis and permanent risk factors. However, such recommendations mainly derive from observational studies; there are no data about long-term treatment of retinal vein occlusion.

  14. Study of Ultrasound in the Diagnosis of Desmoid-type Fibromatoses%韧带样型纤维瘤病的超声诊断探讨

    Institute of Scientific and Technical Information of China (English)

    杨薇; 朱亦菲; 伍海翔

    2012-01-01

    目的 本文通过分析韧带样型纤维瘤病的超声声像图表现,从而提高超声对该病的认识和诊断水平.方法 回顾性分析经手术病理证实的13例韧带样型纤维瘤病的超声特征,其中腹外型6例,腹壁型6例,腹内型1例.结果 13例肿瘤的内部回声欠均匀,无囊性变,内部血供均较丰富.其中,腹壁及腹外型病变质硬、固定,腹内型肠系膜病变质硬、活动度大.浸润性生长者形态不规则、边界欠清、无明显包膜.膨胀性生长者形态规则、边界较清楚、可见部分包膜.结论 韧带样型纤维瘤病有一定的超声声像图特征,结合病史、体征,可对该病的诊断或鉴别诊断提供重要依据.%Objective; Through analyzing the ultras on ography characteristics of desmoid-type fibromatoses , to promote the diagnostic accuracy and understanding of this kind of disease. Methods; Ultrasound image features of 13 cases of desmoid-type fibromatoses confirmed surgically and pathologically were analyzed retrospectively. Six cases were the extra-abdominal type,6 cases were in abdominal wall muscles and 1 was the intra-abdominal type. ResultS:The common features of 13 cases were uneven internal echo, no cystic changes and abundant color doppler blood flow. Among these cases, the features of extra-abdominal type and abdominal wall type were hard and fixed, as for the intra-abdominal type, the mass in the mesentery was hard and with large range of movement. The aggressive-growing cases had irregular figure, ill-defined border and had no obvious coat. The expansive growing cases had regular figure, well-defined border and part of the capsule was visible. Conclusion; Desmoid-type fibromatoses has certain sonographic characteristics. Combined with clinical history and physical sign,the sonographic characteristics provide important basis tor diagnosis or dinerenti-al diagnosis of this tumor.

  15. Differential diagnostic considerations of desmoid-type fibromatosis.

    Science.gov (United States)

    Goldstein, Jeffery A; Cates, Justin M M

    2015-07-01

    Fibrous and myofibroblastic tumors of soft tissue often present the surgical pathologist with a difficult differential diagnosis because of the number of diagnostic possibilities and morphologic similarities among cytologically bland spindle-cell tumors. Prototypical in this regard is desmoid-type fibromatosis. In a review of 320 surgical specimens diagnosed as desmoid tumor, 94 (29%) were discovered to be misclassified as such. The most common lesions in this series were Gardner fibroma, scar tissue, superficial fibromatosis, nodular fasciitis, myofibroma, and collagenous fibroma. Four sarcomas were also misinterpreted as desmoid-type fibromatosis (3 low-grade fibromyxoid sarcomas and 1 unclassified spindle-cell sarcoma). We take this opportunity to compare and contrast desmoid tumor and several of the soft tissue tumors that should be considered in the differential diagnosis thereof.

  16. Desmoid-type fibromatosis-associated Gardner fibromas: prevalence and impact on local recurrence.

    Science.gov (United States)

    Cates, Justin M M; Stricker, Thomas P; Sturgeon, Duveen; Coffin, Cheryl M

    2014-10-28

    Although Gardner fibroma is a precursor lesion of desmoid tumor, the prevalence and prognostic importance of Gardner fibroma associated with desmoid tumors has not been systematically studied in adults. From 129 patients with desmoid-type fibromatosis, 170 specimens were re-examined for the presence of an associated Gardner fibroma. Clinicopathologic features of Gardner fibroma-associated desmoid-type fibromatosis were compared to desmoid tumors without associated Gardner fibroma. Recurrence-free survival was compared using multivariate Cox proportional hazard regression to account for known confounding factors. Of 104 evaluable primary desmoid tumor resections, 25 (24%) had an associated Gardner fibroma. When previous incisional biopsies and resection specimens of locally recurrent desmoid tumors were also examined, the overall prevalence of associated Gardner fibroma was 37%. Desmoid tumors arising in high risk anatomic sites (extremities or deep soft tissues of the back and chest wall) were more often associated with Gardner fibroma than tumors at other sites. Median recurrence-free survival for patients with Gardner fibroma-associated desmoid-type fibromatosis was 3.2 years, whereas median survival for patients without associated Gardner fibroma was >25 years (hazard ratio 2.8; P = 0.001). Although the presence of Gardner fibroma had no impact on the recurrence rate of desmoid tumors arising at high risk anatomic sites, associated Gardner fibroma increased the risk of recurrence 4-fold for desmoid tumors at low risk anatomic sites. Associated Gardner fibroma is under-recognized in desmoid-type fibromatosis and increases the risk of local recurrence for a subgroup of patients. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  17. A large extra-abdominal prevesical pseudo-cyst in a newborn with posterior urethral valves.

    NARCIS (Netherlands)

    Binkhorst, M.; Gier, R.P.E. de

    2010-01-01

    A male newborn is described, in whom a large extra-abdominal prevesical pseudo-cyst as well as prune-belly features were present, both of which were supposedly secondary to posterior urethral valves. It is postulated that the subvesical obstruction caused pressure build-up in the urinary tract, foll

  18. Recidivant desmoide fibromatosis of anterior abdominal wall et desmoide fibromatosis of retroperitoneum: Case report

    Directory of Open Access Journals (Sweden)

    Marijanović G.

    2015-01-01

    Full Text Available Desmoide or aggressive fibromatosis belong to the group of benign tumor characterized by a locally infiltrative growth and tendency to recur and which do not metastasize. Here is shown 46 years old woman who is coming for examination because of recurrent tumors of the anterior abdominal wall, PH earlier verified as desmoide fibromatosis. With a tumor of the anterior abdominal wall measuring 14.7 x 7.0 x 12.5 cm, CT diagnostics discovered a tumor size of 5.8 x 8.8 cm 6,2x in the lodge earlier removed right kidney, which is in close communication with v. cava inferior (VCI and A. Illiaca comunis dex. (AIC dex.. Compression of VCI and AIC has resulted in swelling of the right leg as a whole who retired after excision of the tumor. The postoperative course was uneventful.

  19. Abdominal Wall Desmoid during Pregnancy: Diagnostic Challenges

    Directory of Open Access Journals (Sweden)

    Johnny Awwad

    2013-01-01

    Full Text Available Background. Desmoids are benign tumors, with local invasive features and no metastatic potential, which have rarely been described to be pregnancy associated. Case. We described the rapid growth of an anterior abdominal wall mass in a 40-year-old pregnant woman. Due to its close proximity to the enlarged uterus, it was misdiagnosed to be a uterine leiomyoma by ultrasound examination. Final tissue diagnosis and radical resection were done at the time of abdominal delivery. Conclusion. Due to the diagnostic limitations of imaging techniques, desmoids should always be considered when the following manifestations are observed in combination: progressive growth of a solitary abdominal wall mass during pregnancy and well-delineated smooth tumor margins demonstrated by imaging techniques. This case emphasizes the importance of entertaining uncommon medical conditions in the differential diagnosis of seemingly common clinical manifestations.

  20. Time trends in the incidence and treatment of extra-abdominal and abdominal aggressive fibromatosis: a population-based study.

    Science.gov (United States)

    van Broekhoven, Danique L M; Grünhagen, Dirk J; den Bakker, Michael A; van Dalen, Thijs; Verhoef, Cornelis

    2015-09-01

    Aggressive fibromatosis (AF) is a locally infiltrating soft-tissue tumor. In a population-based study in the Netherlands, we evaluated time trends for the incidence and treatment of AF. In PALGA: Dutch Pathology Registry, all patients diagnosed between 1993 and 2013 as having extra-abdominal or abdominal wall aggressive fibromatosis were identified and available pathology data of the patients were evaluated. Epidemiological and treatment-related factors were analyzed with χ (2)and regression analysis. During the study period, 1134 patients were identified. The incidence increased from 2.10 to 5.36 per million people per year. Median age at the time of diagnosis increased annually by B 0.285 (P = 0.001). Female gender prevailed and increased over time [annual odds ratio (OR) 1.022; P = 0.058]. All anatomic localizations, but in particular truncal tumors, became more frequent. During the study period diagnostic histological biopsies were performed more often (annual OR 1.096; P fibromatosis was observed. The workup of patients improved and a trend towards a nonsurgical treatment policy was observed.

  1. High frequency of beta-catenin heterozygous mutations in extra-abdominal fibromatosis: a potential molecular tool for disease management.

    Science.gov (United States)

    Dômont, J; Salas, S; Lacroix, L; Brouste, V; Saulnier, P; Terrier, P; Ranchère, D; Neuville, A; Leroux, A; Guillou, L; Sciot, R; Collin, F; Dufresne, A; Blay, J-Y; Le Cesne, A; Coindre, J-M; Bonvalot, S; Bénard, J

    2010-03-16

    Fibromatosis comprises distinct clinical entities, including sporadic extra-abdominal fibromatosis, which have a high tendency for recurrence, even after adequate resection. There are no known molecular biomarkers of local recurrence. We searched for beta-catenin mutations in a European multicentre series of fibromatosis tumours to relate beta-catenin mutational status to disease outcome. Direct sequencing of exon 3 beta-catenin gene was performed for 155 frozen fibromatosis tissues from all topographies. Correlation of outcome with mutation rate and type was performed on the extra-abdominal fibromatosis group (101 patients). Mutations of beta-catenin were detected in 83% of all cases. Among 101 extra-abdominal fibromatosis, similar mutation rates (87%) were observed, namely T41A (39.5%), S45P (9%), S45F (36.5%), and deletion (2%). None of the clinico-pathological parameters were found to be significantly associated with beta-catenin mutational status. With a median follow-up of 62 months, 51 patients relapsed. Five-year recurrence-free survival was significantly worse in beta-catenin-mutated tumours regardless of a specific genotype, compared with wild-type tumours (49 vs 75%, respectively, P=0.02). A high frequency (87%) of beta-catenin mutation hallmarks extra-abdominal fibromatosis from a large multicentric retrospective study. Moreover, wild-type beta-catenin seems to be an interesting prognostic marker that might be useful in the therapeutic management of extra-abdominal fibromatosis.

  2. Diffusion tensor imaging (DTI) of desmoid tumours in familial adenomatous polyposis: Initial experience

    Energy Technology Data Exchange (ETDEWEB)

    Bhandari, Santosh, E-mail: s.bhandari10@imperial.ac.uk [Polyposis Registry, St. Mark' s Hospital, Watford Road, Harrow, Middlesex HA1 3UJ (United Kingdom); Sinha, Ashish, E-mail: asinha@imperial.ac.uk [Polyposis Registry, St. Mark' s Hospital, Watford Road, Harrow, Middlesex HA1 3UJ (United Kingdom); Tam, Emily, E-mail: Emily.wy.tam@gmail.com [Paul Strickland Scanner Centre, Mount Vernon Hospital, Northwood, Middlesex HA6 2RN (United Kingdom); Stirling, J. James, E-mail: james.stirling@stricklandscanner.org.uk [Paul Strickland Scanner Centre, Mount Vernon Hospital, Northwood, Middlesex HA6 2RN (United Kingdom); Simcock, Ian, E-mail: ian.simcock@stricklandscanner.org.uk [Paul Strickland Scanner Centre, Mount Vernon Hospital, Northwood, Middlesex HA6 2RN (United Kingdom); Clark, Sue, E-mail: s.clark8@nhs.net [Polyposis Registry, St. Mark' s Hospital, Watford Road, Harrow, Middlesex HA1 3UJ (United Kingdom); Goh, Vicky, E-mail: Vicky.goh@kcl.ac.uk [Division of Imaging Sciences and Biomedical Engineering, King' s College London, Imaging 2, Level 1, Lambeth Wing, St. Thomas' Hospital, Lambeth Palace Road, London, SE1 7EH (United Kingdom)

    2012-11-15

    Purpose: To assess the feasibility of diffusion tensor imaging (DTI) of desmoid tumours in familial adenomatous polyposis (FAP). Materials and methods: Following ethical approval and informed consent, FAP patients with desmoids underwent DTI. Fractional anisotropy (FA), relative anisotropy (RA) and apparent diffusion coefficient (ADC) were compared to control muscle using Mann-Whitney test; and to tumour site and signal intensity using one way analysis of variance (ANOVA). Imaging was repeated after 1 year. Results: 15 desmoids (6 intra-abdominal; 6 abdominal wall, 3 extra-abdominal; size range: 1.6-22.9 cm) were evaluated in 9 patients. DTI was successful in 12/15 desmoid tumours. Median (range) of RA, FA and ADC were 0.23 Multiplication-Sign 10{sup -3} mm{sup 2}/s (0.17-0.26); 0.27 Multiplication-Sign 10{sup -3} mm{sup 2}/s (0.21-0.31); and 1.65 Multiplication-Sign 10{sup -3} mm{sup 2}/s (1.39-1.91) for desmoids, significantly different from muscle: 0.27 Multiplication-Sign 10{sup -3} mm{sup 2}/s (0.23-0.40), 0.32 Multiplication-Sign 10{sup -3} mm{sup 2}/s (0.28-0.46), and 1.45 Multiplication-Sign 10{sup -3} mm{sup 2}/s (0.92-1.63) (p = 0.0001, p = 0.0001, p = 0.0016, respectively). There was no difference in RA, FA or ADC between tumour sites, or signal intensity (p > 0.05). One year later, 2 patients had died. Tumour increased in size in 1 patient (+61%). DTI quantification was possible in only 8/13 tumours. FA, RA and ADC were not significantly different from baseline (p = 0.77, 0.71 and 0.34, respectively). Conclusions: Assessment of water diffusion has the potential to provide insight into tumour microstructure and is feasible in desmoids. Desmoid tumours demonstrate anisotropy but diffusion is less restricted and less directional than in muscle.

  3. Les tumeurs desmoides de la paroi thoracique : à propos de 12 cas Dermoid tumors of the thoracic wall: about 12 cases

    Directory of Open Access Journals (Sweden)

    Abdellatif Benosman

    2009-11-01

    Full Text Available Les tumeurs desmoides sont des tumeurs rares des tissus mous qui peuvent être très agressives localement. A travers l’expérience de notre service, nous analyserons les résultats du traitement chirurgical de ces tumeurs. De 1980 à 2008, 12 patients ont été opérés pour tumeur desmoide de la paroi thoracique. Le diagnostic a été suspecté sur la base des signes cliniques et radiologiques. Aucun patient n’avait un syndrome de Gardner. L’abord chirurgical a été souvent électif à l’aplomb de la tumeur. La résection a été complète dans 11 cas. La confirmation diagnostique a été apportée par l’étude histologique de la pièce opératoire. La durée du suivi post opératoire variait entre 24 et 180 mois. Une patiente était décédée par insuffisance cardiaque et rénale. 7 cas avaient récidivé, et qui ont été traités par simple résection complète dans 5 cas, dont un avait nécessité une greffe myocutanée ; par ailleurs, deux cas ont été traités par résection incomplète associée à une radiothérapie adjuvante. La chirurgie des tumeurs desmoides de la paroi thoracique doit être aussi large que possible pour diminuer le risque de récidive.

  4. Desmoid Type Fibromatosis in the Facet Joint of Lumbar Spine: Case Report and Review of Literature

    Science.gov (United States)

    Kim, So Jung; Lee, Sang Min; Kang, Haeyoun

    2013-01-01

    Desmoid type fibromatosis is a benign fibroblastic tumor arising from the fascia or musculoaponeurosis. It may occur in various locations, but most commonly in the shoulder girdle and neck; to our knowledge, there has been no reported case originating from a facet joint of the spine. We report CT and MR imaging findings of a desmoid type fibromatosis, involving the facet joint of the L3-4 spine with bone involvement. PMID:24043979

  5. Desmoid type fibromatosis in the facet joint of lumbar spine: Case report and review of literature

    Energy Technology Data Exchange (ETDEWEB)

    Kim, So Jung; Ha, Doo Hoe; Lee, Sang Min; Kang, Hae Youn [CHA Bundang Medical Center, CHA University, Seongnam (Korea, Republic of)

    2013-10-15

    Desmoid type fibromatosis is a benign fibroblastic tumor arising from the fascia or musculoaponeurosis. It may occur in various locations, but most commonly in the shoulder girdle and neck; to our knowledge, there has been no reported case originating from a facet joint of the spine. We report CT and MR imaging findings of a desmoid type fibromatosis, involving the facet joint of the L3-4 spine with bone involvement.

  6. High frequency of β-catenin heterozygous mutations in extra-abdominal fibromatosis: a potential molecular tool for disease management

    Science.gov (United States)

    Dômont, J; Salas, S; Lacroix, L; Brouste, V; Saulnier, P; Terrier, P; Ranchère, D; Neuville, A; Leroux, A; Guillou, L; Sciot, R; Collin, F; Dufresne, A; Blay, J-Y; Le Cesne, A; Coindre, J-M; Bonvalot, S; Bénard, J

    2010-01-01

    Background: Fibromatosis comprises distinct clinical entities, including sporadic extra-abdominal fibromatosis, which have a high tendency for recurrence, even after adequate resection. There are no known molecular biomarkers of local recurrence. We searched for β-catenin mutations in a European multicentre series of fibromatosis tumours to relate β-catenin mutational status to disease outcome. Methods: Direct sequencing of exon 3 β-catenin gene was performed for 155 frozen fibromatosis tissues from all topographies. Correlation of outcome with mutation rate and type was performed on the extra-abdominal fibromatosis group (101 patients). Results: Mutations of β-catenin were detected in 83% of all cases. Among 101 extra-abdominal fibromatosis, similar mutation rates (87%) were observed, namely T41A (39.5%), S45P (9%), S45F (36.5%), and deletion (2%). None of the clinico-pathological parameters were found to be significantly associated with β-catenin mutational status. With a median follow-up of 62 months, 51 patients relapsed. Five-year recurrence-free survival was significantly worse in β-catenin-mutated tumours regardless of a specific genotype, compared with wild-type tumours (49 vs 75%, respectively, P=0.02). Conclusion: A high frequency (87%) of β-catenin mutation hallmarks extra-abdominal fibromatosis from a large multicentric retrospective study. Moreover, wild-type β-catenin seems to be an interesting prognostic marker that might be useful in the therapeutic management of extra-abdominal fibromatosis. PMID:20197769

  7. [Expression of beta-catenin and estrogen receptor in desmoid-type fibromatosis].

    Science.gov (United States)

    Zhang, Hong-Ying; Ke, Qi; Zhang, Zhang; Zhang, Rui; Fu, Jing; Chen, Hui-Jiao; Wei, Bing; Bu, Hong

    2010-01-01

    To detect the expression of beta-catenin and Estrogen Receptor in desmoid-type fibromatosis. Nuclear beta-catenin expression was detected by immunohistochemistry in 77 lesions with desmoid-type fibromatosis and 171 other spindle cell lesions, including superficial fibromatosis (n = 18), nodular fasciitis (n = 36), keloid (n = 16), scar (n = 10), granulation tissue (n = 9), synovial sarcoma (n = 38), neufibroma (n = 13), solitary fibrous tumor (n =12), gastrointestinal stromal tumor (n = 10), low-grade myxofibrosarcoma (n = 3), low-grade fibromyxoid sarcoma (n = 3), and smooth muscle tumor (n = 10). In addition, the immunohistochemical expressions of ER-alpha, ER-beta and Ki-67 were examined in all of the lesions with desmoid-type fibromatosis. The nuclear immunohistochemical staining for nuclear beta-catenin and ER-beta was graded as high level ( > or = 25% of cells), low level (5%-25%) or none. High-level nuclear beta-catenin staining was detected in a very limited subset of tissue types, which included 70.1% of lesions with desmoid-type fibromatosis (54/77) and 6.3% of lesions with keloid (1/16). No high-level nuclear beta-catenin staining was seen in any of the other lesions. None of the lesions with desmoid-type fibromatosis expressed ER-alpha. However, 62 (80.5%) of the lesions with desmoids-type fibromatosis were positive in ER-beta, which included 52 (67.5%) with high-level expression, and 10 (13%) with low-level expression. The Spearman correlation analysis suggested that the expression of beta-catenin was positively correlated (r = 0.867, P fibromatosis had very low Ki-67 positive rate. The recurrence of desmoids-type fibromatosis was not correlated independently with beta-catenin, ER-beta or Ki-67. High-level nuclear beta-catenin staining serves as a useful diagnostic tool for desmoid-type fibromatosis. The high expression of ER-beta in desmoid-type fibromatosis provides a biological mechanism for the antiestrogenic compounds to treat fibromatosis. There

  8. A new extra-abdominal channel alternative to the mitrofanoff principle: experimental and preliminary clinical experience

    Directory of Open Access Journals (Sweden)

    Antonio Macedo Jr.

    2009-04-01

    Full Text Available INTRODUCTION: The appendix is the gold-standard channel for the Mitrofanoff principle in pediatric urology, but the search for alternatives is justified considering it may not be available or preferably used for colonic stomas (Malone antegrade continence enema. The aim of this study is to report on technical feasibility of a new approach for creating catheterizable channels in a rabbit model and to present our preliminary clinical experience. MATERIAL AND METHODS: We configured a tube from two rectangular skin flaps 1x4 cm opposite each other in the middle line of the lower inferior abdomen. The channel was anastomosed to the bladder dome with embedding sutures to create a valvular mechanism. The experimental study consisted of 12 rabbits, divided in 4 groups according to the sacrifice schedule at 2, 4, 8 and 12 weeks. At 30th postoperative day, an urodynamic evaluation was performed to record continence of the stoma. A histological analysis of the specimens stained with hematoxylin-eosin, Masson trichrome and Picrosirius red was also done in group 2 (sacrifice at 4 weeks postoperatively. We used this method in 3 patients with congenital non-neurogenic bladder disease presenting with massive residual volumes without compliance deficits. RESULT: The technique proved feasible in all animals, 9 of 12 could be easily catheterized and underwent urodynamic study. No stoma leakage was observed in 7 animals at high bladder pressures (> 50 cm H20 and only 2 animals had some leakage at 40 cm H20. Urodynamics performed through the stoma showed urethral leakage at 20 cm H20, therefore demonstrating the efficacy of the valvular mechanism. Histological analysis confirmed good integration between the tube and the bladder. Mean follow-up of the clinical series (3 patients was 7.2 months. Two patients remained continent up to 4 hours, whereas 1 patient had some leakage after 2 hours. CONCLUSION: We were able to confirm feasibility of a new extra-abdominal

  9. Evaluation of management of desmoid tumours associated with familial adenomatous polyposis in Dutch patients

    NARCIS (Netherlands)

    Nieuwenhuis, M. H.; Mathus-Vliegen, E. M.; Baeten, C. G.; Nagengast, F. M.; van der Bijl, J.; van Dalsen, A. D.; Kleibeuker, J. H.; Dekker, E.; Langers, A. M.; Vecht, J.; Peters, F. T.; van Dam, R.; van Gemert, W. G.; Stuifbergen, W. N.; Schouten, W. R.; Gelderblom, H.; Vasen, H. F. A.

    2011-01-01

    BACKGROUND: The optimal treatment of desmoid tumours is controversial. We evaluated desmoid management in Dutch familial adenomatous polyposis (FAP) patients. METHODS: Seventy-eight FAP patients with desmoids were identified from the Dutch Polyposis Registry. Data on desmoid morphology, management,

  10. Desmoid Fibromatosis of the Abdominal Wall: Surgical Resection and Reconstruction with Biological Matrix Egis®

    Directory of Open Access Journals (Sweden)

    Saveria Tropea

    2017-02-01

    Full Text Available Desmoid tumor is a rare monoclonal fibroblast proliferation that is regarded as benign. The clinical management of desmoid tumors is very complex and requires a multidisciplinary approach because of the unpredictable disease course. For those cases localized in the anterior abdominal wall, symptomatic and unresponsive to medical treatment, radical resection and reconstruction with a prosthetic device are indicated. We present here a case of desmoid fibromatosis of the left anterolateral abdominal wall with a marked increase of the mass that required a large excision followed by reconstruction with biological matrix. The fact that it can be incorporated in patient tissue without a fibrotic response and that it can resist future infections, together with a very competetive price, made the new collagen matrix Egis® our first choice.

  11. Desmoid-type fibromatosis of the head and neck in children: a case report and review of the literature.

    Science.gov (United States)

    Miyashita, Hidetaka; Asoda, Seiji; Soma, Tomoya; Munakata, Kanako; Yazawa, Masaki; Nakagawa, Taneaki; Kawana, Hiromasa

    2016-06-10

    Desmoid-type fibromatosis is defined as an intermediate tumor that rarely occurs in the head and neck of children. There is no doubt as to the value of complete surgical excision for desmoid-type fibromatosis. However, in pediatric patients, surgeons may often be concerned about making a wide excision because of the potential for functional morbidity. Some studies have reported a lack of correlation between margin status and recurrence. Therefore, we discussed our findings with a focus on the state of surgical margins. We report an unusual case of a 9-month-old Japanese girl who prior to presenting at our hospital underwent debulking surgery twice with chemotherapy for desmoid-type fibromatosis of the tongue at another hospital. We performed a partial glossectomy and simultaneous reconstruction with local flap and achieved microscopic complete resection. We also reviewed available literature of pediatric desmoid-type fibromatosis in the head and neck. We described successful treatment for the refractory case of pediatric desmoid-type fibromatosis. The review results showed that some microscopic incomplete resections of tumors in pediatric patients with desmoid-type fibromatosis tended to be acceptable with surgical treatment.

  12. Familial adenomatous polyposis-associated desmoids display significantly more genetic changes than sporadic desmoids.

    Directory of Open Access Journals (Sweden)

    Els Robanus-Maandag

    Full Text Available Desmoid tumours (also called deep or aggressive fibromatoses are potentially life-threatening fibromatous lesions. Hereditary desmoid tumours arise in individuals affected by either familial adenomatous polyposis (FAP or hereditary desmoid disease (HDD carrying germline mutations in APC. Most sporadic desmoids carry somatic mutations in CTNNB1. Previous studies identified losses on 5q and 6q, and gains on 8q and 20q as recurrent genetic changes in desmoids. However, virtually all genetic changes were derived from sporadic tumours. To investigate the somatic alterations in FAP-associated desmoids and to compare them with changes occurring in sporadic tumours, we analysed 17 FAP-associated and 38 sporadic desmoids by array comparative genomic hybridisation and multiple ligation-dependent probe amplification. Overall, the desmoids displayed only a limited number of genetic changes, occurring in 44% of cases. Recurrent gains at 8q (7% and 20q (5% were almost exclusively found in sporadic tumours. Recurrent losses were observed for a 700 kb region at 5q22.2, comprising the APC gene (11%, a 2 Mb region at 6p21.2-p21.1 (15%, and a relatively large region at 6q15-q23.3 (20%. The FAP-associated desmoids displayed a significantly higher frequency of copy number abnormalities (59% than the sporadic tumours (37%. As predicted by the APC germline mutations among these patients, a high percentage (29% of FAP-associated desmoids showed loss of the APC region at 5q22.2, which was infrequently (3% seen among sporadic tumours. Our data suggest that loss of region 6q15-q16.2 is an important event in FAP-associated as well as sporadic desmoids, most likely of relevance for desmoid tumour progression.

  13. Infantile desmoid-type fibromatosis with involvement of temporal bone: case report

    Energy Technology Data Exchange (ETDEWEB)

    Hagen, T. [Dept. of Neuroradiology, Saarland Univ., Homburg (Germany); Feiden, W. [Dept. of Neuropathology, Saarland Univ., Homburg (Germany); Piepgras, U. [Dept. of Neuroradiology, Saarland Univ., Homburg (Germany)

    1995-12-31

    Infantile desmoid-type fibromatosis is a rare, benign, infiltratively growing tumor of the soft tissue. The histological classification is often confusing due to various patterns. Although surgical excision is the treatment of choice, local recurrence is high. Ultrasound, CT, and MRI cannot differentiate the different types of fibrous lesions, but are very essential, showing the localization and infiltrated tissues. We report an unusual case of desmoid-type fibromatosis destroying the temporal bone in a now-5-year-old boy. The literature contains only few reports of radiological findings in patients with infantile fibromatosis. (orig.)

  14. A case of desmoid-type fibromatosis arising after thoracotomy for lung cancer with a review of the english and Japanese literature.

    Science.gov (United States)

    Mori, Takeshi; Yamada, Tatsuya; Ohba, Yasuomi; Yoshimoto, Kentaro; Ikeda, Koei; Shiraishi, Kenji; Suzuki, Makoto

    2014-01-01

    Chest wall desmoid-type fibromatoses are rare, locally aggressive tumors that occasionally arise from previous thoracotomy sites. Tumors arising from previous sites of thoracotomy to treat malignant disease should be discriminated from the pleural dissemination of the previous malignancy. In this study, we report a case of desmoid-type fibromatosis arising from a site for thoracotomy to treat lung cancer. Additionally we reviewed 15 reported cases of desmoid-type fibromatosis following thoracotomy and summarized their features. A 62-year-old woman was found to have a tumor on computed tomography (CT) at a 1-year routine checkup for lung cancer. The tumor (diameter, 3.4 cm) was located at the previous thoracotomy site. Positron emission tomography (PET) revealed mild (18)F-fluorodeoxyglucose (FDG) accumulation in the tumor, with a maximal standardized uptake value (SUVmax) of 1.9. CT-guided biopsy revealed only fibrous tissue. Eighteen months after the biopsy, CT revealed apparent tumor growth, and a biopsy revealed the same histology observed previously. The tumor was removed and diagnosed as desmoid-type fibromatosis. Currently, the patient is alive without recurrence 4 years after desmoid surgery.

  15. Clinical and diagnostic problems of desmoid-type fibromatosis of the mesentery: case report and review of the literature.

    Science.gov (United States)

    La Greca, Gaetano; Santangelo, Antonio; Primo, Stefano; Sofia, Maria; Latteri, Saverio; Russello, Domenico; Magro, Gaetano

    2014-12-12

    In accordo con la “World Health Organization”, il tumore desmoide viene definito come una “proliferazione di tipo clonale dei fibroblasti che origina dai tessuti molli posti più in profondità e che risulta essere caratterizzata da una crescita infiltrante con la tendenza a dare delle recidive locali ma con l’incapacità di metastatizzare”. Noi riportiamo il caso di una giovane donna di 33 anni che presentava una sintomatologia aspecifica, con la presenza di una massa palpabile in sede addominale. La prima diagnosi, eseguita in altra Unità Operativa con US, evidenziava una neoformazione classificata come Leiomioma uterino. Il reperto intraoperatorio durante intervento di isterectomia evidenziava, invece, una neoformazione a partenza dal mesentere. L’esecuzione di due esami bioptici estemporanei non ha portato a nessuna diagnosi di certezza. Veniva quindi eseguita una resezione “en bloc”, allargata con criteri oncologici, della massa, comprendendo digiuno, ileo e colon destro. La diagnosi istologica definitiva è stata poi di Tumore Desmoide. La revisione della letteratura condotta con 25 casi di tumore desmoide del mesentere, ha confermato definitivamente come la diagnosi è molto difficile e quasi mai disponibile prima o durante l’intervento chirurgico. Appare quindi chiaro che la prima scelta di trattamento di questa neoplasia rimane quella chirurgica con margini di resezione R0, pur esistendo la possibilità di un trattamento conservativo farmacologico o radioterapico. Il trattamento del Tumore Desmoide rimane comunque difficile e richiede esperienza ed collaborazione interdisciplinare.

  16. Slow growing desmoid-type fibromatosis of the breast: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Park, Ah Reum; Kim, Ji Young; Kim, Hyun Jung; KIm, Jae Hyung; Jeong, Myeong Ja; Kim, Soung Hee; Kim, Soo Hyun; Kang, Mi Jin; Lee, Ji Hae; Bae, Kyung Eun [Sanggye Paik Hospital, Inje University College of Medicine, Seoul (Korea, Republic of)

    2017-04-15

    Fibromatosis or desmoid tumor of the breast is a rare benign entity that has no metastatic potential but has significant risk of local recurrence. Its association with previous surgical or accidental trauma and Gardner's syndrome has been reported. Awareness of this lesion is important as the diagnosis is often confused with breast carcinoma. We report a case of a 44-year-old woman who presented with a palpable mass in her left breast, close to the axilla since a few months ago. She had undergone excisional biopsy for her left breast mass 15 months ago, and the diagnosis was confirmed as intraductal papilloma with atypical ductal hyperplasia. Subsequent ultrasound and core needle biopsy revealed stromal fibrosis. After 9 months, the mass showed an increase in its size and the anteroposterior to width ratio on ultrasonography compared to the previous examination, and final excisional biopsy confirmed the diagnosis of desmoid-type fibromatosis.

  17. Desmoid Fibromatosis of Submandibular Region

    African Journals Online (AJOL)

    Head and Neck Surgery, Mater Misericordiae University ... and neck. With tumors in this region, patients usually present with trismus ..... Cancer. 2004;100:612‑20. 17. Poon R, Smits R, Li C, Jagmohan‑Changur S, Kong M, Cheon S, et al.

  18. An interesting journey of an ingested needle: a case report and review of the literature on extra-abdominal migration of ingested Foreign bodies

    Directory of Open Access Journals (Sweden)

    Gezen Fazli C

    2011-05-01

    Full Text Available Abstract Swallowed foreign bodies encounter a major problem especially in children, but fortunately they mostly do not cause any related complication and are easily passed with the stool. In this paper, an interesting journey of a needle is presented. A 20-year old female admitted to our emergency service after she had swallowed a sewing machine needle, which is initially observed in the stomach in the plain abdominal radiography. During the follow-up period, the needle traveled through bowels, and surprisingly was observed in the left lung on 10th day of the follow-up. It was removed with a thoracotomy and pneumotomy under the fluoroscopic guidance. The postoperative period was uneventful and the patient was discharged from the hospital on the day 5. We also review the literature on interesting extra-abdominal migrations of swallowing foreign bodies.

  19. Desmoid tumor of the mesentery: a case report; Tumor desmoide mesenterico: relato de caso

    Energy Technology Data Exchange (ETDEWEB)

    Santos, Jose Eduardo Mourao [Universidade Federal de Sao Paulo (UNIFESP/EPM), SP (Brazil). Dept. de Diagnostico por Imagem; Mucerino, Donato R. [Sao Paulo Univ., SP (Brazil). Faculdade de Medicina; D' Ippolito, Giuseppe [Hospital Sao Luiz, Sao Paulo, SP (Brazil). Setor de Diagnostico por Imagem]. E-mail: giuseppe_dr@uol.com.br

    2005-01-15

    A case of a 37 years old male patient with histological diagnosis of intra-abdominal fibromatosis who underwent laparotomy for excision of a painless mass in the left iliac fossa is reported. This is a rare disease of difficult therapeutic planning that is locally aggressive and often recurs, which exhibits high morbidity and lethality rates well described in the literature. We discuss the imaging methods used and their role in the diagnosis of this patient. (author)

  20. Cortical desmoid of the humerus: radiographic and MRI correlation

    Energy Technology Data Exchange (ETDEWEB)

    Kay, Matthew; Counsel, Peter [Princess Margaret Hospital for Children, Department of Diagnostic Imaging, Perth (Australia); Perth Radiological Clinic, Perth (Australia); Wood, David [Princess Margaret Hospital for Children, Department of Orthopedic Surgery, Perth (Australia); Breidahl, William [Perth Radiological Clinic, Perth (Australia)

    2017-07-15

    Cortical desmoids are self-limiting fibro-osseous lesions commonly occurring at the medial supracondylar femur in active adolescents, at either the origin of the medial head of the gastrocnemius or at the insertion of the adductor magnus aponeurosis. Less commonly, in a similar demographic, cortical desmoids may occur in the proximal humerus medially at the insertion of the pectoralis major muscle or laterally at the insertion of the deltoid. The radiographic appearance of the proximal humerus cortical desmoid has been described previously, but not the MRI appearance. We present the radiographic and MRI appearances of a proximal humerus cortical desmoid in a young adolescent who presented for investigation of right shoulder pain. (orig.)

  1. Nationwide trends in the current management of desmoid (aggressive) fibromatosis.

    Science.gov (United States)

    Eastley, N C; Hennig, I M; Esler, C P; Ashford, R U

    2015-06-01

    The optimal management of desmoid fibromatosis remains unclear, leading to significant variability in patient management. To assess this problem, the current approach of clinicians managing this complex condition in the UK was investigated. A hypothetical case of intramuscular limb girdle desmoid fibromatosis in a fit 65-year-old patient was devised. Surgical and non-surgical oncology members of the British Sarcoma Group were questioned on how they would manage this case in three scenarios: primary disease with function-sparing surgery possible, primary disease with neurovascular involvement and disease recurrence after a previous R0 resection. Initial management, management of symptomatic disease progression, follow-up preferences and any differences in respondents' management choices in a younger case were investigated. The responses from 14 sarcoma surgeons and 23 oncologists (14 clinical, nine medical) were analysed. Desmoid fibromatosis management is generally shared by surgeons and oncologists within sarcoma multidisciplinary teams in the UK. Variation exists in the chosen initial management of primary desmoid fibromatosis in the UK, with function-sparing surgery possible (observation 51%, resection 51%), primary desmoid fibromatosis with neurovascular involvement (hormone therapy with non-steroidal anti-inflammatory drugs 51%, radiotherapy 27%, observation 22%) and for cases of desmoid fibromatosis recurrence (radiotherapy 41%, hormone therapy and non-steroidal anti-inflammatory drugs 27%, observation 24%). There was a clear preference of surgical resection of symptomatic disease progression in cases of primary desmoid fibromatosis without neurovascular involvement (60%). By contrast, radiotherapy was the preferred treatment for progression in cases with neurovascular involvement (47%) or cases of recurrence after a previous R0 resection (34%). Clinical follow-up was selected 3 months after intervention in 68% of scenarios. Follow-up imaging was selected 3

  2. Time Trends in the Incidence and Treatment of Extra-Abdominal and Abdominal Aggressive Fibromatosis: A Population-Based Study

    NARCIS (Netherlands)

    D.L.M. van Broekhoven (Danique); D.J. Grunhagen (Dirk Jan); M.A. den Bakker (Michael); T. van Dalen (Thijs); C. Verhoef (Kees)

    2015-01-01

    textabstractBackground: Aggressive fibromatosis (AF) is a locally infiltrating soft-tissue tumor. In a population-based study in the Netherlands, we evaluated time trends for the incidence and treatment of AF. Methods: In PALGA: Dutch Pathology Registry, all patients diagnosed between 1993 and 2013

  3. Cholangiocarcinoma accompanied by desmoid-type fibromatosis

    Institute of Scientific and Technical Information of China (English)

    Nan Xu; Zhe-Yu Chen; Lu-NanYan; Jia-YingYang; Wen-TaoWang; Shu-Guang Jing

    2011-01-01

    BACKGROUND: Cholangiocarcinoma complicated by intra-abdominal desmoid-type fibromatosis (DTF) is uncommon. There are no reports on patients with this type of fibromatosis, in which the pre-operative treatment (including diagnosis), surgical approach, post-operative pathologic reports, and prognosis are discussed. METHOD: The clinicopathological features of a 49-year-old man were retrospectively analyzed. RESULTS: Cholangiocarcinoma located in the inferior segment of the bile duct was considered pre-operatively on the basis of clinical findings. At the time of pancreaticoduodenectomy, the mesojejunum was stiff without nodules or a mass at a distance of approximately 80 cm from the ligament of Treitz. Complete excision of the entire lesion of the intestinal mesenteric contracture and its subsidiary was performed. Post-operative pathologic findings confirmed an adenocarcinoma located at the extremity of the common bile duct and infiltrating the full thickness of the common bile duct as well as the deep muscular layer of the duodenum. The contracted jejunal mesentery was shown to have DTF. The patient was alive with no evidence of recurrence after a follow-up of 6 months. CONCLUSIONS: The patient had a rare hereditary disease with intra-abdominal DTF, which manifests the characteristics of an aggressive growth pattern and a high rate of local recurrence;conservative therapy is recommended. Complete excision of the fibromatous lesion during pancreaticoduodenectomy may maximally decrease the risk of local recurrence.

  4. Desmoid tumour in familial adenomatous polyposis. A review of literature

    DEFF Research Database (Denmark)

    Knudsen, Anne Louise; Bülow, Steffen

    2001-01-01

    Desmoid tumours (DT) are rare benign tumours that do not metastasise, but tend to invade locally. DT are frequently seen in patients with familial adenomatous polyposis (FAP), and diagnosis and treatment are often difficult. Surgical trauma, genetic predisposition and hormonal factors are conside...

  5. Long-Term Follow-Up of Desmoid Fibromatosis Treated with PF-03084014, an Oral Gamma Secretase Inhibitor.

    Science.gov (United States)

    Villalobos, Victor Manuel; Hall, Francis; Jimeno, Antonio; Gore, Lia; Kern, Kenneth; Cesari, Rossano; Huang, Bo; Schowinsky, Jeffrey T; Blatchford, Patrick Judson; Hoffner, Brianna; Elias, Anthony; Messersmith, Wells

    2017-09-08

    Desmoid fibromatosis is a fibroblastic neoplasm driven by aberrations within the WNT pathway, exhibiting mutations in β-catenin or APC. We review the long-term follow-up of patients in a phase I study treated with an oral gamma secretase inhibitor, PF-03084014. PF-03084014 was administered orally at doses ranging from 20 to 330 mg twice daily. Tumor assessments were performed using computed tomography/magnetic resonance imaging (CT/MRI) within 4 weeks of study entry, and every other cycle through cycle 9. After cycle 9, patients were evaluated as clinically indicated. Seven patients with desmoid fibromatosis were treated between December 2009 and December 2016 at the University of Colorado. Five patients (71.4%, 95% confidence interval [CI] 29.0-96.3%) achieved a partial response (PR), with a mean time to achieving response of 11.9 months (95% CI 2.5-21.4 months). All patients who achieved a PR continue to maintain responses between 47.9 and 73+ months. Four patients stopped treatment yet remain free of progression between 11 and 53+ months. One patient had PFS of 42+ months, with a 17% decrease in the target lesion. A biopsy performed at the end of the study showed decreased tumoral cellularity compared with previous biopsies. Effective treatment doses ranged from 80 to 330 mg administered orally twice daily. PF-03084014 was effective in treating desmoid tumors, with an objective response rate of 71.4% (95% CI 29.0-96.3%) in this small cohort of patients. PF-03084014 exhibits promising activity, even at relatively low doses (80 mg twice daily), with high tolerability leading to prolonged disease control even after therapy discontinuation.

  6. Desmoids in familial adenomatous polyposis are monoclonal proliferations.

    Science.gov (United States)

    Middleton, S B; Frayling, I M; Phillips, R K

    2000-02-01

    Desmoids are poorly-understood, locally aggressive, non-metastasizing fibromatoses that occur with disproportionate frequency in patients with familial adenomatous polyposis (FAP). Their nature is controversial with arguments for and against a neoplastic origin. Neoplastic proliferations are by definition monoclonal, whereas reactive processes originate from a polyclonal background. We examined clonality of 25 samples of desmoid tissue from 11 female FAP patients by assessing patterns of X-chromosome inactivation to calculate a clonality ratio. Polymerase chain reaction (PCR) amplification of a polymorphic CAG short tandem repeat (STR) sequence adjacent to a methylation-sensitive restriction enzyme site within the human androgen receptor (HUMARA) gene using fluorescent-labelled primers enabled analysis of PCR products by Applied Biosystems Genescan II software. Twenty-one samples from nine patients were informative for the assay. Samples from all informative cases comprised a median of 66% (range 0-75%) clonal cells but from the six patients with a clonality ratio < or =0.5 comprised a median of 71% (65-75%) clonal cells. FAP-associated desmoid tumours are true neoplasms. This may have implications in the development of improved treatment protocols for patients with these aggressive tumours.

  7. Desmoplastic Fibroma of Bone: Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Sang Yun; Ha, Dong Ho; Kim, Dong Won [Dept. of Radiology, Dong A University College of Medicine, Seoul (Korea, Republic of); Kim, Su Jin [Dept. of Pathology, Dong A University College of Medicine, Seoul (Korea, Republic of)

    2011-05-15

    Desmoplastic fibroma of bone is a rare benign primary bone tumor that histologically resembles the extra-abdominal desmoid tumor of soft tissues. It is a nonmetastasizing, but locally aggressive tumor that is similar to a desmoid tumor of the soft tissues, and so it is considered 'semimalignant'. According to a previous report on a series of bone tumors, the incidence rate of desmoplastic fibroma was 0.1-0.3%. Its rarity results in radiologists having a tendency of overlooking the possibility of desmoplastic fibroma of bone during the imaging readings. We report on the imaging findings of desmoplastic fibroma of bone with a review of the relevant literature.

  8. 韧带样纤维瘤病的CT及MRI特征及病理对照%CT and MRI appearance of desmoid-type fibromatosis with pathologic correlation

    Institute of Scientific and Technical Information of China (English)

    袁焕初; 郑晓林; 肖利华; 王刚; 黎群弟

    2014-01-01

    Objective To assess the CT and MRI appearance and pathologic features of desmoid type fibromatosis(DF). Methods 27 patients with pathologically confirmed DF underwent contrast-enhanced CT(12)and MRI(14)with 1 patient having both CT and MRI examinations.Results Of 30 soft tissue DF,14 were located in extra-abdominal and 16 in abdominal wall muscles. All tumors presented as solid masses growing along the long axis of muscles.20 tumors were fusiform or round and 10 were irregular in shape.The margins were well-defined(7)or ill-defined(23).On unenhanced CT,3 tumors were isodense and 10 hypodense relative to the muscle.After intravenous contrast injection,9 tumors showed moderate progressive enhancement and 4 tumors enhanced heterogeneously.On MRI,the lesions were homogeneously T1 isointense(9)or slightly hypointense(9),slightly T2 hyperintense (15)or heterogeneous on T2-weighted images(3).All lesions were hyperintense on STIR-weighted images with bands of low signal intensities in 15.Heterogeneous moderate progressive contrast enhancement was observed in all tumors with non-enhancing T1-and T2-hypointense bands in 12 tumors.Histopathology showed infiltration of fibroblasts and myofibroblasts with compact fibrous and collagen tissues as well as mucus sedimentation.Conclusion DF has CT and MRI features of ill-defined margin,low CT density or MR signal intensity without necrosis and progressive contrast enhancement.%目的:回顾性分析韧带样纤维瘤的CT及MRI特征表现及病理基础。方法收集经手术病理证实的韧带样纤维瘤共27例影像学及病理资料,CT检查12例,MRI检查14例,另1例行CT及MRI检查;全部病例均行增强扫描。结果27例共30个病灶,16个病灶发生于腹壁,14个病灶发生于腹壁外。形态:全部病灶均沿肌纤维长轴生长,20个呈梭形或类圆形,10个不规则形;7个边界较清,23个边界不清。与肌肉密度、信号相比:CT上10个病灶呈稍低密度,3

  9. A CASE OF DESMOID TUMOUR – ABDOMINAL FIBROMATOSIS IN A YOUNG FEMALE PRESENTING WITH ABDOMINAL PAIN

    Directory of Open Access Journals (Sweden)

    Achu Jacob Philip

    2016-06-01

    Full Text Available Desmoid tumours are rare; they account for about 0.03 percent of all neoplasms and less than 3 percent of all soft tissue tumours. The estimated incidence in the general population is two to four per million population per year. Here reporting a case of a 34-year-old female presenting with pain abdomen and swelling chest, excised mass diagnosed to be a desmoid tumour.

  10. Positron emission tomography in patients with aggressive fibromatosis/desmoid tumours undergoing therapy with imatinib

    Energy Technology Data Exchange (ETDEWEB)

    Kasper, Bernd; Hohenberger, Peter [University of Heidelberg, Sarcoma Unit, ITM - Interdisciplinary Tumor Center Mannheim, Mannheim University Medical Center, Mannheim (Germany); Dimitrakopoulou-Strauss, Antonia; Strauss, Ludwig G. [German Cancer Research Center, Clinical Cooperation Unit Nuclear Medicine, Heidelberg (Germany)

    2010-10-15

    We used {sup 18}F-FDG PET to evaluate the FDG uptake in patients with aggressive fibromatosis (AF, also known as desmoid tumours) undergoing therapy with imatinib (imatinib mesylate, Glivec). The pilot study included nine patients with progressive AF receiving oral treatment with imatinib at a daily dose of 800 mg. Patients were examined using PET prior to the start of therapy and during imatinib treatment. Restaging according to the Response Evaluation Criteria in Solid Tumors (RECIST) was performed in parallel using CT and/or MRI and served as reference. The clinical outcomes in nine evaluable patients were as follows: seven patients with stable disease, and two patients with progressive disease. A 27% decrease in the median average standardized uptake value (SUV) of the sequential PET examinations was demonstrated in all evaluable patients with three patients (33%) showing a decrease in SUV of more than 40% (48%, 52% and 54%, respectively); no patient showed a substantial increase in SUV. To our knowledge, this is the first series of AF patients undergoing treatment with imatinib and monitored using sequential PET imaging, that allows detection of SUV changes after imatinib induction, thus helping to decide whether treatment should be continued or not. (orig.)

  11. Gene Expression Profiling in Familial Adenomatous Polyposis Adenomas and Desmoid Disease

    Directory of Open Access Journals (Sweden)

    Bowden Nikola A

    2007-06-01

    Full Text Available Abstract Gene expression profiling is a powerful method by which alterations in gene expression can be interrogated in a single experiment. The disease familial adenomatous polyposis (FAP is associated with germline mutations in the APC gene, which result in aberrant β-catenin control. The molecular mechanisms underlying colorectal cancer development in FAP are being characterised but limited information is available about other symptoms that occur in this disorder. Although extremely rare in the general population, desmoid tumours in approximately 10% of FAP patients. The aim of this study was to determine the similarities and differences in gene expression profiles in adenomas and compare them to those observed in desmoid tumours. Illumina whole genome gene expression BeadChips were used to measure gene expression in FAP adenomas and desmoid tumours. Similarities between gene expression profiles and mechanisms important in regulating formation of FAP adenomas and desmoid tumours were identified. This study furthers our understanding of the mechanisms underlying FAP and desmoid tumour formation.

  12. A RARE CASE OF DESMOID TUMOUR AND NEUROFIBROMA WITH POSTOPERATIVE COMPLICATION AND MANAGEMENT: CASE REPORT

    Directory of Open Access Journals (Sweden)

    Rahul Raj C. L

    2016-09-01

    Full Text Available BACKGROUND Desmoid tumour is included under group of aggressive fibromatosis. It is an uncommon neoplasm that occurs sporadically or as a part of syndrome (Familial Adenomatous Polyposis. It has a high propensity for recurrence even after surgical removal. Occur with a frequency of 2.4 to 4.3 cases per million population. Occur in young women during gestation or more from one year of childbirth. We are here by reporting a 36-year-old female who diagnosed with desmoid tumour and underwent surgical excision and her postoperative complications.

  13. Testosterone regulates cell proliferation in aggressive fibromatosis (desmoid tumour)

    Science.gov (United States)

    Hong, H; Nadesan, P; Poon, R; Alman, B A

    2011-01-01

    Background: Aggressive fibromatosis (desmoid tumour) is a locally invasive tumour caused by mutations resulting in β-catenin protein stabilisation. Apc1638N mice are predisposed to developing aggressive fibromatosis tumours, and male mice develop greater numbers of tumours than female mice, suggesting a role for androgens in this tumour type. Methods: Human aggressive fibromatosis tumours were examined for the expression of the androgen receptor, and primary human tumour cell cultures were treated with testosterone. Orchidectomised Apc1638N mice were investigated for the development of tumours, and were treated with testosterone to study the effect of tumour formation and the level of β-catenin. Results: Androgen receptors are universally expressed in human aggressive fibromatosis tumours. Testosterone increased the proliferation rate and β-catenin protein level in a dose-dependent manner in human aggressive fibromatosis tumours. Orchiectomy reduced the number and size of tumours that formed in male Apc1638N mice to a similar level as observed in female mice. Testosterone treatment increased the number of tumours that formed in orchidectomised male mice, and resulted in a marked increase in β-catenin protein levels. Conclusion: Testosterone regulates β-catenin protein level and proliferation rate in this mesenchymal tumour. This work identifies the therapeutic use of testosterone blockade in aggressive fibromatosis as an area for further investigation. PMID:21468052

  14. Retroperitoneal abscess: an extra-abdominal manifestation

    OpenAIRE

    Mallia, Alvin James; Ashwood, Neil; Arealis, George; Galanopoulos, Ilias

    2015-01-01

    Retroperitoneal abscesses are unusual occurrences with occult and insidious presentations. There is often a lack of abdominal signs, leading to delays in drainage and high mortality rates. We report a case of thigh emphysema in an 88-year-old patient with diabetes. Prior to admission the patient reported a vague 4-week history of left thigh pain and an inability to fully weight bear. She presented to our emergency department with sepsis and acute kidney impairment. An X-ray of her left femur ...

  15. Retroperitoneal abscess: an extra-abdominal manifestation.

    Science.gov (United States)

    Mallia, Alvin James; Ashwood, Neil; Arealis, George; Galanopoulos, Ilias

    2015-01-09

    Retroperitoneal abscesses are unusual occurrences with occult and insidious presentations. There is often a lack of abdominal signs, leading to delays in drainage and high mortality rates. We report a case of thigh emphysema in an 88-year-old patient with diabetes. Prior to admission the patient reported a vague 4-week history of left thigh pain and an inability to fully weight bear. She presented to our emergency department with sepsis and acute kidney impairment. An X-ray of her left femur revealed widespread gas between muscular planes. A retroperitoneal abscess involving the left renal fossa, psoas, iliacus and upper thigh muscles was revealed on an urgent CT scan. The patient was transferred to intensive care unit (ICU) and underwent an emergency drainage. Despite ICU the patient died 2 days after admission.

  16. The challenge of extraabdominal desmoid tumour management in patients with Gardner's syndrome: radiofrequency ablation, a promising option.

    Science.gov (United States)

    Cobianchi, Lorenzo; Ravetta, Valentina; Viera, Francesca Torello; Filisetti, Claudia; Siri, Barbara; Segalini, Edoardo; Maestri, Marcello; Dominioni, Tommaso; Alessiani, Mario; Rossi, Sandro; Dionigi, Paolo

    2014-11-27

    Desmoid tumours are benign, myofibroblastic stromal neoplasms common in Gardner's syndrome, which is a subtype of familial adenomatous polyposis characterized by colonic polyps, osteomas, thyroid cancer, epidermoid cysts, fibromas and sebaceous cysts. The primary treatment is surgery, followed by adjuvant radiotherapy, but the local recurrence rate is high, and wide resection can result in debilitating loss of function. We report the case of a 39-year-old man with Gardner's syndrome who had already undergone a total prophylactic colectomy. He developed desmoid tumours localized in the mesenteric root, abdominal wall and dorsal region, which were treated from 2003 through 2013 with several surgical procedures and percutaneous radiofrequency ablation. In 2008 and 2013, RFA was applied under ultrasonographic guidance to two desmoid tumours localized in the dorsal thoracic wall. The outcomes were low-grade pain and one case of superficial skin necrosis, but so far there has been no recurrence of desmoid tumours in these locations. Surgical resection remains the first-line therapy for patients with desmoid tumours, but wide resection may lead to a poor quality of life. Radiofrequency ablation is less invasive and expensive and is a possible therapeutic option for desmoid tumours in patients with Gardner's syndrome.

  17. [Pseudo-tumoral lesions of dense conjuntival tissue. Attempt at pathogenic interpretation].

    Science.gov (United States)

    Bonenfani, J L; Lagacé, R

    1975-01-01

    The histogenesis of pseudotumors of dense connective tissue has been studied. These lesions may be classified as mucoid (synovial cyst and periungueal myxoma), collagenous (desmoid tumor, desmoid fibroblastoma and fibromatosis colli), elastic (elastofibroma dorsi), cellular (fibroblastic: fibromatosis, fasciitis and myositis; histiocytic: giant-cell tumor of tendon sheath, fibrous histiocytoma and atypical fibroxanthoma), metaplastic (ossifying fasciitis, ossifying myositis and juvenile chondroid fibroma) and hamartomatous nature (fibrous hamartoma). It must be emphasized that these lesions show a variable and polymorphouse cellular composition and then can simulate sarcoma.

  18. Tumorer

    DEFF Research Database (Denmark)

    Prause, J.U.; Heegaard, S.

    2005-01-01

    oftalmologi, øjenlågstumorer, conjunctivale tumorer, malignt melanom, retinoblastom, orbitale tumorer......oftalmologi, øjenlågstumorer, conjunctivale tumorer, malignt melanom, retinoblastom, orbitale tumorer...

  19. Desmoid-Type Fibromatosis of the Thorax: CT, MRI, and FDG PET Characteristics in a Large Series From a Tertiary Referral Center

    Science.gov (United States)

    Xu, Hai; Koo, Hyun Jung; Lim, Soyeoun; Lee, Jae Wook; Lee, Han Na; Kim, Dong Kwan; Song, Joon Seon; Kim, Mi Young

    2015-01-01

    Abstract The purpose of this study was to describe the radiologic findings of computed tomography (CT), magnetic resonance (MR) imaging, and 18F-fluorodeoxy glucose positron emission tomography (FDG PET) in desmoid-type fibromatosis of the thorax. We retrospectively evaluated 47 consecutive patients with pathologically proven desmoid-type fibromatosis from January 2005 to March 2015. Patients underwent CT (n = 36) and/or MR (n = 32), and 13 patients also underwent FDG PET. Based on CT and MR, the sizes, locations, margins, contours, presence of surrounding fat, extra-compartment extension, bone involvement, and neurovascular involvement of the tumors were recorded. The attenuation, signal intensity, enhancement pattern, and presence of internal low signal band or signal void of the tumors were evaluated. Initial image findings were then compared between 2 groups of tumors: group 1 with recurrence or progression, and group 2 with no recurrence or stable without treatment. Median age at diagnosis of the tumors was 45 years, range 4 to 96, female-to-male ratio 1.8. Median tumor long diameter was 65 mm (range, 22–126 mm). The most common locations were chest wall (42.6%), followed by supraclavicular area, shoulder or axillary area, and mediastinum. The tumors had well-defined margins (83.0%), lobulated in contours (66.0%) surrounding fat (63.8%), extra-compartment extensions (42.6%), bone involvements (42.6%), and neurovascular involvements (27.7%). On CT, tumors had low attenuation (60.0%) with mild enhancement (median 24 HU, range 0–52). On MR, they showed iso-signal intensity (SI) (96.9%) on T1-weighted images (WI), and high SI (90.6%) on T2WI images, with strong (87.5%) and heterogeneous (96.9%) enhancement. Internal low signal bands (84.4%) and signal voids (68.8%) were noted. The median value of maxSUV was 3.1 (range, 2.0–7.3). In group 1 (n = 19, 40.4%), 13 patients suffered recurrence and 6 experienced progression. Group 2 (n = 28, 59

  20. [Case report: Rapidly growing abdominal wall giant desmoid tumour during pregnancy].

    Science.gov (United States)

    Palacios-Zertuche, Jorge Tadeo; Cardona-Huerta, Servando; Juárez-García, María Luisa; Valdés-Flores, Everardo; Muñoz-Maldonado, Gerardo Enrique

    Desmoid tumours are one of the rarest tumours worldwide, with an estimated yearly incidence of 2-4 new cases per million people. They are soft tissue monoclonal neoplasms that originate from mesenchymal stem cells. It seems that the hormonal and immunological changes occurring during pregnancy may play a role in the severity and course of the disease. The case is presented on 28-year-old female in her fifth week of gestation, in whom an abdominal wall tumour was found attached to left adnexa and uterus while performing a prenatal ultrasound. The patient was followed up under clinical and ultrasonographic surveillance. When she presented with abnormal uterine activity at 38.2 weeks of gestation, she was admitted and obstetrics decided to perform a caesarean section. Tumour biopsy was taken during the procedure. Histopathology reported a desmoid fibromatosis. A contrast enhanced abdominal computed tomography scan was performed, showing a tumour of 26×20.5×18cm, with well-defined borders in contact with the uterus, left adnexa, bladder and abdominal wall, with no evidence of infiltration to adjacent structures. A laparotomy, with tumour resection, hysterectomy and left salpingo-oophorectomy, components separation techniques, polypropylene mesh insertion, and drainage was performed. The final histopathology report was desmoid fibromatosis. There is no evidence of recurrence after 6 months follow-up. Desmoid tumours are locally aggressive and surgical resection with clear margins is the basis for the treatment of this disease, using radiotherapy, chemotherapy and hormone therapy as an adjunct in the treatment. Copyright © 2016 Academia Mexicana de Cirugía A.C. Publicado por Masson Doyma México S.A. All rights reserved.

  1. Desmoid-type fibromatosis of the head and neck region in the paediatric population: a clinicopathological and genetic study of seven cases

    NARCIS (Netherlands)

    Flucke, U.E.; Tops, B.B.J.; Diest, P.J. van; Slootweg, P.J.

    2014-01-01

    AIMS: Desmoid-type fibromatosis (desmoid) is a locally aggressive (myo)fibroblastic lesion. It represents one of the more common fibrous tumours in children and adolescents. The head and neck region is more often involved than in adults. METHODS AND RESULTS: We investigated the clinicopathological a

  2. Desmoid-type fibromatosis of the head and neck region in the paediatric population: a clinicopathological and genetic study of seven cases

    NARCIS (Netherlands)

    Flucke, U.E.; Tops, B.B.J.; Diest, P.J. van; Slootweg, P.J.

    2014-01-01

    AIMS: Desmoid-type fibromatosis (desmoid) is a locally aggressive (myo)fibroblastic lesion. It represents one of the more common fibrous tumours in children and adolescents. The head and neck region is more often involved than in adults. METHODS AND RESULTS: We investigated the clinicopathological a

  3. Intra-thoracic desmoid tumour in a patient with a previous aortocoronary bypass

    Directory of Open Access Journals (Sweden)

    Iuzzolino Paolo

    2006-07-01

    Full Text Available Abstract Background Intra-thoracic desmoid tumours with mediastinal invasion are very rare. Although rare they have to be taken into account in the differential diagnosis of a thoracic mass and therapeutic options have to be weighted since surgical treatment may require wide excision. Case presentation A 48-year-old male diabetic, dyslipidaemic, former heavy smoker with psychiatric illness was operated by sternotomy for a triple aorto-coronary bypass 4 years before, presented with complains of recent onset such as constant and oppressive chest pain. At surgery a mass extending from the aortic arch into the entire anterior mediastinum and to most of the right pleural cavity was found. The mass was separated from sternal periosteum and vessels of aorto-coronary by pass were isolated starting from the aortic arch up to the pericardium. The histological examination revealed aggressive fibromatosis. Conclusion Although technically demanding, radical surgical excision is actually the most indicated therapeutic approach for intra-thoracic desmoid tumours.

  4. Multicentric synchronous recurrent aggressive fibromatosis

    Directory of Open Access Journals (Sweden)

    Kavita Kohli

    2012-01-01

    Full Text Available Extra-abdominal desmoid tumors are known as aggressive fibromatosis (AFM. Synchronous and metachronous multicentric aggressive fibromatosis are rare lesions and pose dilemma in diagnosis and management. A rare and interesting case of recurrent multicentric, synchronous AFM is presented which to the best of our knowledge has not been reported earlier. A young male presented with well defined, hard, fixed swelling on the thigh. Resected tumor mass on histopathology was diagnosed as an extra abdominal fibromatosis. He presented again after two months with swelling at the same site; and two more swellings on the foot. Fine needle aspiration cytology (FNAC from all three sites was performed; and was suggestive of benign spindle cell lesion of fibrogenic origin with the possibility of multicentric synchronous recurrent aggressive fibromatosis.

  5. AGGRESSIVE FIBROMATOSIS (NONFAMILIAL DESMOID TUMOR) - THERAPEUTIC PROBLEMS AND THE ROLE OF ADJUVANT RADIOTHERAPY

    NARCIS (Netherlands)

    PLUKKER, JT; VANOORT, [No Value; VERMEY, A; MOLENAAR, [No Value; HOEKSTRA, HJ; PANDERS, AK; DOLSMA, WV; KOOPS, HS

    1995-01-01

    Aggressive fibromatosis is a rare soft tissue tumour with a high tendency to local recurrence, even after apparently adequate resection. Wide local excision with a margin of at least 3 cm, depending on the anatomical location, should be performed to improve rates of recurrent disease. There is no co

  6. Diagnostic and therapeutic dilemmas in intra-abdominal desmoid tumors: A case report and literature review

    Directory of Open Access Journals (Sweden)

    Austin D. Williams, MD MSEd

    2016-01-01

    Conclusion: DTs present a clinical challenge in their diagnosis and management, and despite providing standard medical and surgical treatment, recurrence rates are high and continued surveillance is crucial.

  7. Tumor

    Institute of Scientific and Technical Information of China (English)

    2008-01-01

    2008479 Preliminary study of MR elastography in brain tumors. XU Lei(徐磊), et al.Neurosci Imaging Center, Beijing Tiantan Hosp, Capital Med Univ, Beijing 100050.Chin J Radiol 2008;42(6):605-608. Objective To investigate the potential values of magnetic resonance elastography (MRE) for evaluating the brain tumor consistency in vivo. Methods Fourteen patients with known solid brain tumor (5 male, 9 female; age range: 16-63 years)

  8. Primary desmoplastic small round cell tumor of the femur

    Energy Technology Data Exchange (ETDEWEB)

    Yoshida, Akihiko; Garcia, Joaquin [Memorial Sloan-Kettering Cancer Center, Department of Pathology, New York, NY (United States); Edgar, Mark A. [Memorial Sloan-Kettering Cancer Center, Department of Pathology, New York, NY (United States); Weill Medical College of Cornell University, New York, NY (United States); Meyers, Paul A. [Weill Medical College of Cornell University, New York, NY (United States); Memorial Sloan-Kettering Cancer Center, Department of Pediatrics, New York, NY (United States); Morris, Carol D. [Weill Medical College of Cornell University, New York, NY (United States); Memorial Sloan-Kettering Cancer Center, Department of Surgery, Orthopaedic Service, New York, NY (United States); Panicek, David M. [Weill Medical College of Cornell University, New York, NY (United States); Memorial Sloan-Kettering Cancer Center, Department of Radiology, New York, NY (United States)

    2008-09-15

    Desmoplastic small round cell tumor (DSRCT) is a rare malignant neoplasm typically involving the abdominal cavity of a young male. Extra-abdominal occurrence of this tumor is very rare. We report a 10-year-old girl with primary DSRCT arising within the left femur. The patient presented with knee pain, and radiological findings were strongly suggestive of osteogenic sarcoma. In addition to the typical microscopic appearance and immunophenotype, RT-PCR demonstrated the chimeric transcript of EWS-WT1, which is diagnostic of DSRCT. Pulmonary metastases were present at initial staging studies, but no abdominal or pelvic lesion was present. Despite chemotherapy and complete tumor excision, the patient developed progressive lung and bone metastases and died 3 years after initial presentation. This is the second reported case of primary DSRCT of bone with genetic confirmation. (orig.)

  9. Desmoid-type fibromatosis of the head and neck region in the paediatric population: a clinicopathological and genetic study of seven cases.

    Science.gov (United States)

    Flucke, Uta; Tops, Bastiaan B J; van Diest, Paul J; Slootweg, Pieter J

    2014-05-01

    Desmoid-type fibromatosis (desmoid) is a locally aggressive (myo)fibroblastic lesion. It represents one of the more common fibrous tumours in children and adolescents. The head and neck region is more often involved than in adults. We investigated the clinicopathological and genetic characteristics of seven paediatric desmoids at this anatomical site, including two cases of desmoplastic fibroma located in the mandible. There were two females and five males with an age range of 1.5-8 years. The sites of the soft tissue lesions were sinonasal (n = 4) and paramandibular (n = 1). All cases showed typical morphology and nuclear β-catenin expression. CTNNB1 gene sequencing, performed successfully in five cases, revealed mutations in three cases with one p.T41A (bone lesion), one p.S37A and one novel mutation, p.D32V (sinonasal soft tissue lesions). Six patients were treated by excision with positive margins in five cases. Follow-up, available for six patients (median 4 years), showed no evidence of disease in four cases, slow progression in one case, and recurrence with stable disease in the last case. Our study provides evidence of genetic similarities in desmoid and desmoplastic fibroma. Additionally, we expanded the spectrum of mutations in CTNNB1 with one novel desmoid mutation. © 2013 John Wiley & Sons Ltd.

  10. Unusual ultrasonography findings of recurred mammary fibermatosis mimicking subareolar mastitis: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Sung, Hwa Sung; Kim, Young Seon; Bae, Young Kyung [College of Medicine, Yeungnam University, Daegu (Korea, Republic of)

    2016-09-15

    Fibromatosis, also known as an extra-abdominal desmoid tumor, rarely occurs in the breast and is often mistaken for carcinoma, clinically and radiologically. Desmoid tumor is a monoclonal myofibroblastic neoplasm which is locally aggressive, but rarely metastasizes. We herein report a case of a 64-year-old woman who experienced two episodes of recurrence of mammary fibromatosis. The mass was initially detected by screening mammography. It appeared as an irregularly shaped mass which was confined within the mammary zone. Recurrences were excised from the right breast 10 and 17 months later. The second recurrence occurred in the subareolar area accompanied by skin thickening and showed an anechoic component on ultrasonography, which mimicked subareolar mastitis with an abscess.

  11. Breast fibromatosis associated with breast implants.

    Science.gov (United States)

    Seo, Yoon Nae; Park, Young Mi; Yoon, Hye Kyoung; Lee, Sun Joo; Choo, Hye Jung; Ryu, Ji Hwa

    2015-09-01

    Fibromatosis refers to an extra-abdominal desmoid tumor or aggressive fibromatosis. Breast fibromatosis can develop in association with the capsule around a breast implant, although reports of cases of fibromatosis associated with breast implants are rare. As the demand for breast augmentation has increased, it is important to understand the diseases associated with breast implants. In the present report, we describe a case of breast fibromatosis that developed adjacent to a breast implant and demonstrated a relatively well-defined border even though it invaded the surrounding structures. We also explore the specific imaging features for diagnosing breast fibromatosis in association with implants by reviewing previous literature.

  12. 韧带样型纤维瘤病的X线平片、CT、MR表现及病理对照分析%Comparative analysis of X-ray, CT, MR diagnosis and pathology in desmoid-type fibromatoses

    Institute of Scientific and Technical Information of China (English)

    王成华; 周祖平

    2013-01-01

    Objective To investigate the imaging manifestations and diagnostic value of imageology of the effect of desmoid fibromatosis (desmoid-type fibromatoses,DF).Methods 16 patients with DF confirmed by operation and pathology,which included,7 males and 9 females,were examined by CT and MRI.12 cases underwent CT examination (9 cases with enhanced),4 cases MRI examination (1 case with enhanced),and 4 cases X-ray examination.Retrospective analysis of CT and MRI of desmoid fibromatosis was analyzed and compared with pathology.Results 16 patients with 16 lesions were detected and divided into three types,extra-abodmen in 11 cases,abdominal wall in 3 cases and intra-abdomen in 2 cases.CT scans showed that the lesions presented homogeneous low density as soft tissue and boundary is not clear.X-ray plain film and CT showed that the skeletal lesions hadexpansile,osteolytic change,trabecular formation within lesions,soap bubble,oppressed absorption of bone and irregular bone ridge.Most lesions had not calcification andossification.Compared with the muscle signal,the lesions showed slightly low signal on T1 WI and showed highand low mixed signal on T2 WI.After contrast enhancement,the lesions showed moderate enhancement.The stripe and patchy non-enhanced structures with low signal intensity on both T1 WI and T2WI were seen.Conclusion Desmoid fibromatosis is a kind of aggressive benign tumor.X-ray can observe the integrity of bone lesions,CT examination can help understand the features of destruction of bone; stripe structure in the lesions on T1 WI,T2 WI and no enhancement of the lesions were a feature of this disease; Therefore,multiple imaging modalities can make a correct diagnosis of desmoids fibromatosis in most cases.%目的 探讨韧带样型纤维瘤病(desmoid-type fibromatoses,DF)的影像学表现及其影像学诊断价值.方法 搜集经手术病理证实的DF患者16例,男7例,女9例.12例行CT检查(增强9例),4例行MRI检查(增强1例,4例均同时行X线平

  13. Giant desmoid tumour of the thorax following latissimus dorsi and implant breast reconstruction: case report and review of the literature

    LENUS (Irish Health Repository)

    Collins, AM

    2017-03-01

    The case of a giant thoracic desmoid tumour in a 44-year-old woman, who presented two years following a breast reconstruction with a latissimus dorsi (LD) flap and implant, is reported. Clinical findings included a rapidly growing, painless mass. Computed tomography (CT) suggested skin and intercostal soft tissue invasion. The tumour was resected en bloc with the LD muscle, implant capsule and underlying rib segments. The resultant thoracic and abdominal wall defects were reconstructed with Dualmesh® and polypropylene meshes respectively. There was no evidence of recurrence at thirty-six months follow-up.

  14. Development of multiple myeloma in a patient with gastrointestinal stromal tumor treated with imatinib mesylate: A case report

    Institute of Scientific and Technical Information of China (English)

    D Tzilves; A Gatopoulou; K Zervas; E Katodritou; F Patakiouta; A Tarpagos; I Katsos

    2007-01-01

    Gastrointestinal stromal tumors (GISTs) are rare tumors,which represent approximately 1% of the neoplasms of the gastrointestinal tract. These tumors rarely give extra-abdominal metastases. However, their clinical outcome is potentially adverse. In some rare cases, coexistance of GISTs with other malignancies has been reported. Here we present a case of a 74-year old male with GIST, which was managed by surgical resection.Fourteen months later, the patient presented with liver metastases and imatinib mesylated was administered.During treatment, the patient reported skeletal pain and plane X-rays revealed osteolytic bone lesions. Further investigation revealed the presence of multiple myeloma.To the best of our knowledge, this is the first report of the co-existence of multiple myeloma (MM) with GIST.

  15. Tumors about the knee in children.

    Science.gov (United States)

    Gebhardt, M C; Ready, J E; Mankin, H J

    1990-06-01

    Tumors are rare causes of knee symptoms in children but must be considered in the differential diagnosis of pediatric knee pain in order to avoid errors in treatment that could result in loss of limb or even life. Experience with 199 bone and soft-tissue tumors about the knee in children are reviewed. The majority of lesions were benign bone tumors (n = 101), with osteocartilaginous exostoses, nonossifying fibromas, and chondroblastomas predominating. Malignant bone tumors (n = 59) were less frequent, and osteosarcoma (n = 48) was by far the most common sarcoma. Soft-tissue lesions (n = 31) were much less frequent and included rhabdomyosarcoma, synovial sarcoma, fibrosarcoma, and desmoid tumors. A careful history, physical examination, and review of roentgenograms are essential to avoid errors in diagnosis. Malignant tumors require roentgenograms and laboratory studies in sequence to stage the patient. A properly performed biopsy established the diagnosis in most instances. Popliteal cysts, stress fractures, infection, myositis ossificans, histiocytosis, and other lesions can mimic tumors and delay correct diagnosis.

  16. Mesenchymal Tumors Can Derive from Ng2/Cspg4-Expressing Pericytes with β-Catenin Modulating the Neoplastic Phenotype

    Directory of Open Access Journals (Sweden)

    Shingo Sato

    2016-07-01

    Full Text Available The cell of origin for most mesenchymal tumors is unclear. One cell type that contributes to this lineages is the pericyte, a cell expressing Ng2/Cspg4. Using lineage tracing, we demonstrated that bone and soft tissue sarcomas driven by the deletion of the Trp53 tumor suppressor, or desmoid tumors driven by a mutation in Apc, can derive from cells expressing Ng2/Cspg4. Deletion of the Trp53 tumor suppressor gene in these cells resulted in the bone and soft tissue sarcomas that closely resemble human sarcomas, while stabilizing β-catenin in this same cell type caused desmoid tumors. Comparing expression between Ng2/Cspg4-expressing pericytes lacking Trp53 and sarcomas that arose from deletion of Trp53 showed inhibition of β-catenin signaling in the sarcomas. Activation of β-catenin inhibited the formation and growth of sarcomas. Thus, pericytes can be a cell of origin for mesenchymal tumors, and β-catenin dysregulation plays an important role in the neoplastic phenotype.

  17. Mesenchymal Tumors Can Derive from Ng2/Cspg4-Expressing Pericytes with β-Catenin Modulating the Neoplastic Phenotype.

    Science.gov (United States)

    Sato, Shingo; Tang, Yuning J; Wei, Qingxia; Hirata, Makoto; Weng, Angela; Han, Ilkyu; Okawa, Atsushi; Takeda, Shu; Whetstone, Heather; Nadesan, Puvindran; Kirsch, David G; Wunder, Jay S; Alman, Benjamin A

    2016-07-26

    The cell of origin for most mesenchymal tumors is unclear. One cell type that contributes to this lineages is the pericyte, a cell expressing Ng2/Cspg4. Using lineage tracing, we demonstrated that bone and soft tissue sarcomas driven by the deletion of the Trp53 tumor suppressor, or desmoid tumors driven by a mutation in Apc, can derive from cells expressing Ng2/Cspg4. Deletion of the Trp53 tumor suppressor gene in these cells resulted in the bone and soft tissue sarcomas that closely resemble human sarcomas, while stabilizing β-catenin in this same cell type caused desmoid tumors. Comparing expression between Ng2/Cspg4-expressing pericytes lacking Trp53 and sarcomas that arose from deletion of Trp53 showed inhibition of β-catenin signaling in the sarcomas. Activation of β-catenin inhibited the formation and growth of sarcomas. Thus, pericytes can be a cell of origin for mesenchymal tumors, and β-catenin dysregulation plays an important role in the neoplastic phenotype.

  18. Management of sporadic desmoid-type fibromatosis: A European consensus approach based on patients' and professionals' expertise - A Sarcoma Patients EuroNet and European Organisation for Research and Treatment of Cancer/Soft Tissue and Bone Sarcoma Group initiative

    NARCIS (Netherlands)

    Kasper, B.; Baumgarten, C.; Bonvalot, S.; Haas, R; Haller, F.; Hohenberger, P.; Moreau, G.; Graaf, W.T.A. van der; Gronchi, A.

    2015-01-01

    Desmoid-type fibromatosis (DF) is a rare monoclonal, fibroblastic proliferation characterised by a variable and often unpredictable clinical course. It may affect nearly all parts of the body including extremities, trunk and abdomen. Considering the variable clinical presentations, anatomic location

  19. Intramuscular Myxoma – A Rare Tumor

    Directory of Open Access Journals (Sweden)

    Vishwanath Yaligod

    2013-10-01

    Full Text Available Introduction: Intramuscular myxoma is a rare benign soft tissue tumor involving the musculoskeletal system. The incidence is reported as varying from 0.1 to 0.13 per 100,000 population. Most patients present between the fifth and sixth decade of life. The swelling commonly occurs in the large muscles of the thigh, shoulder, buttocks and arms. The tumor can be diagnosed with certainty only with histopathological examination. Local recurrence is rare after excision. Case Report: A 73 year old male patient presented to us with a swelling of the anterior aspect of the middle third of the right thigh measuring about 8 x 4 centimeters. He was thoroughly investigated and the swelling excised in toto. The soft cystic swelling excised was found on histopathology to be an intramuscular myxoma. Conclusion: This case report is being presented since the tumor is rare and also an important consideration in the differential diagnosis of sarcomas, intramuscular lipoma, hemangioma, hematoma, and desmoid tumor. Another important feature is that it can be diagnosed with certainty only after excision. Keywords: Intramuscular myxoma, soft tissue swelling, benign.

  20. Recurrent angio-fibroma of breast masquerading as phyllodes tumor.

    Science.gov (United States)

    Chaurasia, Jai K; Alam, Feroz; Shadan, Mariam; Naim, Mohammed

    2015-01-01

    A young Indian female presented with a recurring tumor in the right breast masquerading as phyllodes tumor. Patient had history of five times excision and recurrences of the tumor, diagnosed as fibrous phyllodes of the breast. Presently, a well-circumscribed tumor of about 10 cm size, comprising of benign fibrous-angiomatous tissue with evidence of foci of pyogenic vasculitis was observed. Immuno-histochemical markers for the myo-epithelial and epithelial elements excluded the possibility of fibrous phyllodes, inflammatory myofibroblastic tumor, desmoid fibromatosis, and metaplastic carcinoma. The present findings were diagnostic of an inflammatory angio-fibroma of the right breast, not reported in the earlier literature. The observations indicated that the female breast may be susceptible to spontaneous productive and common-antibiotic-resistant focal septic vascular inflammation giving rise to angio-fibromatous proliferation producing a well-defined tumor mass in the breast, distinguishable from the other breast lesions by the connective tissue stains and immuno-histochemical markers.

  1. Solid tumors of the peritoneum, omentum, and mesentery in children: radiologic-pathologic correlation: from the radiologic pathology archives.

    Science.gov (United States)

    Chung, Ellen M; Biko, David M; Arzamendi, Aaron M; Meldrum, Jaren T; Stocker, J Thomas

    2015-01-01

    Intraperitoneal solid tumors are far less common in children than in adults, and the histologic spectrum of neoplasms of the peritoneum and its specialized folds in young patients differs from that in older patients. Localized masses may be caused by inflammatory myofibroblastic tumor, Castleman disease, mesenteric fibromatosis, or other mesenchymal masses. Inflammatory myofibroblastic tumor is a mesenchymal tumor of borderline biologic potential that appears as a solitary circumscribed mass, possibly with central calcification. Castleman disease is an idiopathic lymphoproliferative disorder that appears as a circumscribed, intensely enhancing mass in the mesentery. Mesenteric fibromatosis, or intra-abdominal desmoid tumor, is a benign tumor of mesenchymal origin associated with familial adenomatous polyposis. Mesenteric fibromatosis appears as a mildly enhancing, circumscribed solitary mass without metastases. Diffuse peritoneal disease may be due to desmoplastic small round cell tumor (DSRCT), non-Hodgkin lymphoma, or rhabdomyosarcoma. DSRCT is a rare member of the small round blue cell tumor family that causes diffuse peritoneal masses without a visible primary tumor. A dominant mass is typically found in the retrovesical space. Burkitt lymphoma is a pediatric tumor that manifests with extensive disease because of its short doubling time. The bowel and adjacent mesentery are commonly involved. Rhabdomyosarcoma may arise as a primary tumor of the omentum or may spread from a primary tumor in the bladder, prostate, or scrotum. Knowledge of this spectrum of disease allows the radiologist to provide an appropriate differential diagnosis and suggest proper patient management.

  2. Expression of annexin Ⅱ, TGF-β1 and TGF-βRI in desmoid-type fibromatosis%韧带样型纤维瘤病中Annexin Ⅱ、TGF-β1和TGF-βRI的表达

    Institute of Scientific and Technical Information of China (English)

    苗亚静; 宋魏; 李家印; 仇晓菲

    2013-01-01

    Purpose To investigate the expression of annexin Ⅱ , TGF-p, and TGF-βRI, and their possible roles in desmoid-type fibromatosis ( DTF ). Methods The expression of annexin II , TGF-p, and TGF-βRI was detected in 34 DTF tumors and matched surrounding tissues by immunohistochemical staining. Results Annexin Ⅱ was expressed in 29 of 34 cases ( 85. 3% ) of DTF, which were significantly higher than that of surrounding tissues ( P < 0. 05 ). The positive tumor cells were mainly distributed in infiltrating region. The average percentage of annexin Ⅱ positive cells in DTF was significantly higher than that of surrounding tissues ( P < 0. 05 ), and that in infiltrating areas was significantly higher than that in non-infiltrated ones ( P < 0. 05 ). The positive expression rates of TGF-β1, and TGF-βRI in DTF were 79. 4% ( 27/34 ) and 82. 4% ( 28/34 ), respectively, which were remarkably higher than those of surrounding tissues ( P <0. 05 ). The expression of TGF-pRI was positively correlated with TGF-β1( P <0. 05 ). Conclusions Annexin Ⅱ may participate in the development of DTF and the infiltrative growth of tumor cells, and may be a potential target for anti-DTF therapy. In addition, there may be existed a TGF-p, autocrine loops in DTF.%目的 探讨Annexin Ⅱ、TGF-β1和TGF-βRI在韧带样型纤维瘤病(desmoid-type fibromatosis,DTF)组织中的表达及意义.方法 采用免疫组化PV-9000两步法检测34例DTF和周围正常组织中Annexin Ⅱ、TGF-β1和TGF-βRI的表达及分布.结果 (1)Annexin Ⅱ在DTF中的阳性率为85.3%(29/34),明显高于周围正常组织(P<0.05),阳性表达在浸润区尤为突出.Annexin Ⅱ在DTF中的平均阳性细胞率明显高于周围组织(P<0.05);在浸润区平均阳性细胞率明显高于非浸润区(P<0.05).(2)DTF肿瘤组织中TGF-β1和TGF-βRI的阳性率分别为79.4%(27/34)和82.4%(28/34),明显高于周围正常组织(P<0.05);TGF-β1与TGF-βRI表达呈正相关(P<0.05).结论 (1)Annexin Ⅱ可能

  3. Fibroblastic and myofibroblastic tumors of the head and neck: Comprehensive imaging-based review with pathologic correlation

    Energy Technology Data Exchange (ETDEWEB)

    Hourani, Roula, E-mail: rh64@aub.edu.lb [Department of Diagnostic Radiology, American University of Beirut Medical Center, Beirut (Lebanon); Taslakian, Bedros, E-mail: bt05@aub.edu.lb [Department of Diagnostic Radiology, American University of Beirut Medical Center, Beirut (Lebanon); Shabb, Nina S., E-mail: ns04@aub.edu.lb [Department of Pathology and Laboratory Medicine, American University of Beirut Medical Center, Beirut (Lebanon); Nassar, Lara, E-mail: ln07@aub.edu.lb [Department of Diagnostic Radiology, American University of Beirut Medical Center, Beirut (Lebanon); Hourani, Mukbil H., E-mail: mh17@aub.edu.lb [Department of Diagnostic Radiology, American University of Beirut Medical Center, Beirut (Lebanon); Moukarbel, Roger, E-mail: rm17@aub.edu.lb [Department of Otolaryngology – Head and Neck Surgery, American University of Beirut Medical Center, Beirut (Lebanon); Sabri, Alain, E-mail: as71@aub.edu.lb [Department of Otolaryngology – Head and Neck Surgery, American University of Beirut Medical Center, Beirut (Lebanon); Rizk, Toni, E-mail: tonirisk@hotmail.com [Department of Neurosurgery, Hôtel-Dieu de France, Saint-Joseph University, Beirut (Lebanon)

    2015-02-15

    Highlights: • Almost all fibroblastic tumors are evaluated with non-invasive imaging. • Radiologists should be familiar with the imaging appearance of fibroblastic tumors. • Most appropriate initial examination when fibromatosis coli suspected is ultrasound. • Most common location of ossifying fibromas is the tooth-bearing regions. - Abstract: Fibroblastic and myofibroblastic tumors of the head and neck are a heterogeneous group of disorders characterized by the proliferation of fibroblasts, myofibroblasts, or both. These tumors may be further subclassified on the basis of their behavior as benign, intermediate with malignant potential, or malignant. There are different types of fibroblastic and myofibroblastic tumors that can involve the head and neck including desmoid-type fibromatosis, solitary fibrous tumor, myofibroma/myofibromatosis, nodular fasciitis, nasopharyngeal angiofibroma, fibrosarcoma, dermatofibrosarcoma protuberans, fibromatosis coli, inflammatory myofibroblastic tumor, ossifying fibroma, fibrous histiocytoma, nodular fasciitis, fibromyxoma, hyaline fibromatosis and fibrous hamartoma. Although the imaging characteristics of fibroblastic and myofibroblastic tumors of the head and neck are nonspecific, imaging plays a pivotal role in the noninvasive diagnosis and characterization of these tumors, providing information about the constitution of tumors, their extension and invasion of adjacent structures. Correlation with the clinical history may help limit the differential diagnosis and radiologists should be familiar with the imaging appearance of these tumors to reach an accurate diagnosis.

  4. Primary intraosseous desmoplastic small round cell tumor of the calvarium: Case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Vadim Khachaturov, MD

    2015-03-01

    Full Text Available Desmoplastic small round cell tumor (DSRCT is a rare, aggressive malignancy typically occurring intra-abdominally in young adolescent males. Rare extra-abdominal primaries have been reported in the mediastinum, head and neck area, central nervous system, paratesticular region, visceral organs, and soft tissue. We report a primary intraosseous DSRCT of the calvarium in a 6-year-old male who presented with right ear pain and swelling. Imaging showed an aggressive-appearing permeative mixed lytic and sclerotic lesion of the right sphenoid and temporal bones with extensive periosteal reaction, clinically concerning for osteosarcoma. An open biopsy was performed, and the tumor was composed of primitive round cells with perinuclear cytoplasmic clearing, arranged in diffuse sheets and ill-defined nests and surrounded by a prominent desmoplastic stroma. Immunohistochemically the tumor cells were reactive for desmin (dot-like, CD99 (membranous and cytokeratin AE1/3 (focal. EWSR1-WT1 chimeric fusion transcript was detected by reverse transcriptase-polymerase chain reaction. Sequencing of the fusion transcript revealed a rare in-frame junction of EWSR1 exon 10 to WT1 exon 8. This is the third documented case of an intraosseous DSRCT with molecular confirmation, but it is the first reported case to arise in the calvarium. While the diagnosis of DSRCT is usually straightforward in the classic clinical setting of an intra-abdominal mass, awareness that this entity may present as a bone primary is necessary to prevent misclassification as osteosarcoma or other malignancy.

  5. MRI manifestation and correlation with pathologic findings in the diagnosis of desmoid-type fibromatosis%韧带样纤维瘤病MRI特征表现及其病理基础

    Institute of Scientific and Technical Information of China (English)

    陈德周; 俞顺; 包强; 苏家威; 杜瑞宾

    2014-01-01

    目的:探讨韧带样纤维瘤病(DF)的MRI特点,重点分析MRI特征影像及其病理基础,评价MRI在DF诊断中的价值。方法回顾性分析经手术病理证实14例韧带样纤维瘤病的M RI表现(14个病灶均行M RI增强及延迟扫描),与术后病理结果进行比较,并探讨M RI特征影像的病理基础。结果14例均为单发病灶(腹外型7例、腹壁型4例、腹内型3例),其中有2例为术后复发,肿瘤沿着肌肉长轴生长。T1 WI呈等或稍低于肌肉的信号,T2 WI呈高于肌肉信号,增强扫描病灶均呈不均匀强化,以延迟强化为明显,病灶内可见条状或带状 T1 WI及 T2 WI低信号,增强后未见强化。14例病灶内均未见坏死、囊变及瘤周水肿。病理组织学上肿瘤由不同比例的成纤维细胞、纤维组织和胶原纤维束组成,纤维细胞及成纤维细胞密集区域T2 WI信号增高,而肿瘤内胶原纤维区域在 T1 WI、T2 WI均为低信号。肿瘤内见较多血管壁完整的毛细血管,这是病灶延迟强化的病理基础。结论病灶内可见T1 WI、T2 WI均呈带状低信号且增强扫描不强化以及病灶延迟强化是DF的特征性M RI表现。M RI可以很好评估肿瘤侵犯范围及其与周围结构的关系,有助于DF的诊断和鉴别诊断。%Objective To explore the magnetic resonance imaging (MRI) features of desmoid-type fibromatosis (DF) ,to analyze emphatically the correction of M RI characteristic features and pathological basis of DF ,and to evaluate the value of MRI in the diagnosis of DF .Methods The MRI imaging characteristics of 14 patients with DF confirmed pathologically were retrospectively analyzed (all the patients were scaned with contrast enhance ) and compared with findings of patholo-gy .Results 14 cases were single lesion (7 lesions were extre-abdominal ,4 lesions were located in the abdominal wall ,oth-ers were intra-abdominal) ,two of which were postoperative

  6. Fibroblastic and myofibroblastic tumors of the head and neck: comprehensive imaging-based review with pathologic correlation.

    Science.gov (United States)

    Hourani, Roula; Taslakian, Bedros; Shabb, Nina S; Nassar, Lara; Hourani, Mukbil H; Moukarbel, Roger; Sabri, Alain; Rizk, Toni

    2015-02-01

    Fibroblastic and myofibroblastic tumors of the head and neck are a heterogeneous group of disorders characterized by the proliferation of fibroblasts, myofibroblasts, or both. These tumors may be further subclassified on the basis of their behavior as benign, intermediate with malignant potential, or malignant. There are different types of fibroblastic and myofibroblastic tumors that can involve the head and neck including desmoid-type fibromatosis, solitary fibrous tumor, myofibroma/myofibromatosis, nodular fasciitis, nasopharyngeal angiofibroma, fibrosarcoma, dermatofibrosarcoma protuberans, fibromatosis coli, inflammatory myofibroblastic tumor, ossifying fibroma, fibrous histiocytoma, nodular fasciitis, fibromyxoma, hyaline fibromatosis and fibrous hamartoma. Although the imaging characteristics of fibroblastic and myofibroblastic tumors of the head and neck are nonspecific, imaging plays a pivotal role in the noninvasive diagnosis and characterization of these tumors, providing information about the constitution of tumors, their extension and invasion of adjacent structures. Correlation with the clinical history may help limit the differential diagnosis and radiologists should be familiar with the imaging appearance of these tumors to reach an accurate diagnosis.

  7. Bone tumor

    Science.gov (United States)

    Tumor - bone; Bone cancer; Primary bone tumor; Secondary bone tumor; Bone tumor - benign ... The cause of bone tumors is unknown. They often occur in areas of the bone that grow rapidly. Possible causes include: Genetic defects ...

  8. The impact of radiotherapy in the treatment of desmoid tumours. An international survey of 110 patients. A study of the Rare Cancer Network

    Directory of Open Access Journals (Sweden)

    Maurer Robert

    2007-03-01

    Full Text Available Abstract Purpose A multi-centre study to assess the value of combined surgical resection and radiotherapy for the treatment of desmoid tumours. Patients and methods One hundred and ten patients from several European countries qualified for this study. Pathology slides of all patients were reviewed by an independent pathologist. Sixty-eight patients received post-operative radiotherapy and 42 surgery only. Median follow-up was 6 years (1 to 44. The progression-free survival time (PFS and prognostic factors were analysed. Results The combined treatment with radiotherapy showed a significantly longer progression-free survival than surgical resection alone (p smaller than 0.001. Extremities could be preserved in all patients treated with combined surgery and radiotherapy for tumours located in the limb, whereas amputation was necessary for 23% of patients treated with surgery alone. A comparison of PFS for tumour locations proved the abdominal wall to be a positive prognostic factor and a localization in the extremities to be a negative prognostic factor. Additional irradiation, a fraction size larger than or equal to 2 Gy and a total dose larger than 50 Gy to the tumour were found to be positive prognostic factors with a significantly lower risk for a recurrence in the univariate analysis. This analysis revealed radiotherapy at recurrence as a significantly worse prognostic factor compared with adjuvant radiotherapy. The addition of radiotherapy to the treatment concept was a positive prognostic factor in the multivariate analysis. Conclusion Postoperative radiotherapy significantly improved the PFS compared to surgery alone. Therefore it should always be considered after a non-radical tumour resection and should be given preferably in an adjuvant setting. It is effective in limb preservation and for preserving the function of joints in situations where surgery alone would result in deficits, which is especially important in young patients.

  9. Non-odontogenic tumors of the facial bones in children and adolescents: role of multiparametric imaging

    Energy Technology Data Exchange (ETDEWEB)

    Becker, Minerva; Stefanelli, Salvatore; Poletti, Pierre Alexandre; Merlini, Laura [University of Geneva, Division of Radiology, Department of Imaging and Medical Informatics, Geneva University Hospital, Geneva (Switzerland); Rougemont, Anne-Laure [University of Geneva, Division of Clinical Pathology, Department of Genetic and Laboratory Medicine, Geneva University Hospital, Geneva (Switzerland)

    2017-04-15

    Tumors of the pediatric facial skeleton represent a major challenge in clinical practice because they can lead to functional impairment, facial deformation, and long-term disfigurement. Their treatment often requires a multidisciplinary approach, and radiologists play a pivotal role in the diagnosis and management of these lesions. Although rare, pediatric tumors arising in the facial bones comprise a wide spectrum of benign and malignant lesions of osteogenic, fibrogenic, hematopoietic, neurogenic, or epithelial origin. The more common lesions include Langerhans cell histiocytosis and osteoma, while rare lesions include inflammatory myofibroblastic and desmoid tumors; juvenile ossifying fibroma; primary intraosseous lymphoma; Ewing sarcoma; and metastases to the facial bones from neuroblastoma, Ewing sarcoma, or retinoblastoma. This article provides a comprehensive approach for the evaluation of children with non-odontogenic tumors of the facial skeleton. Typical findings are discussed with emphasis on the added value of multimodality multiparametric imaging with computed tomography (CT), magnetic resonance imaging (MRI) with diffusion-weighted imaging (DWI), positron emission tomography CT (PET CT), and PET MRI. Key imaging findings and characteristic histologic features of benign and malignant lesions are reviewed and the respective role of each modality for pretherapeutic assessment and post-treatment follow-up. Pitfalls of image interpretation are addressed and how to avoid them. (orig.)

  10. Management of sporadic desmoid-type fibromatosis: a European consensus approach based on patients' and professionals' expertise - a sarcoma patients EuroNet and European Organisation for Research and Treatment of Cancer/Soft Tissue and Bone Sarcoma Group initiative.

    Science.gov (United States)

    Kasper, B; Baumgarten, C; Bonvalot, S; Haas, R; Haller, F; Hohenberger, P; Moreau, G; van der Graaf, W T A; Gronchi, A

    2015-01-01

    Desmoid-type fibromatosis (DF) is a rare monoclonal, fibroblastic proliferation characterised by a variable and often unpredictable clinical course. It may affect nearly all parts of the body including extremities, trunk and abdomen. Considering the variable clinical presentations, anatomic locations and biological behaviours, an individualised treatment approach is required. No established or evidence-based approach for the treatment of this neoplasm is available as of today. Therefore, we propose a consensus treatment algorithm based on a round table meeting bringing together sarcoma experts from the European Organisation for Research and Treatment of Cancer (EORTC) Soft Tissue and Bone Sarcoma Group (STBSG) with patient advocates from Sarcoma Patients EuroNet (SPAEN). The aim of the meeting was to develop - for the first time ever - a consensus approach based on professionals' AND patients' expertise. As a fundamental prerequisite, all patients should be discussed in a multidisciplinary setting in centres or professional networks with a specific expertise in the disease.

  11. Wilms Tumor

    Science.gov (United States)

    Wilms tumor is a rare type of kidney cancer. It causes a tumor on one or both kidneys. It usually affects ... are at risk should be screened for Wilms tumor every three months until they turn eight. Symptoms ...

  12. Fibromatosis of the Sigmoid Colon With CTNNB1 (β-Catenin) Gene Mutation, Arising at the Site of Ileocolic Anastomosis for Resection of Gastrointestinal Stromal Tumor.

    Science.gov (United States)

    Thway, Khin; Abou Sherif, Sara; Riddell, Angela M; Mudan, Satvinder

    2016-05-01

    We describe a case of intra-abdominal fibromatosis, which occurred in a 44-year-old woman who had a previous history of gastrointestinal stromal tumor (GIST) of the sigmoid mesocolon, which was treated with imatinib and resection. A mass was detected at the site of ileocolic anastomosis of the previous small bowel resection and sigmoid colectomy, nearly 3 years later. Clinically, this was suspected to represent recurrent GIST and was excised, but histology and mutational analysis showed desmoid-type fibromatosis with a mutation in codon 41 of exon 3 of the CTNNB1 (β-catenin) gene. The occurrence of fibromatosis at the site of excision of GIST is very rare, but its recognition is important as the treatment of the two neoplasms differs significantly. As imaging cannot reliably distinguish between these 2 entities, histological diagnosis is crucial for correct clinical management. © The Author(s) 2015.

  13. Pituitary Tumors

    Science.gov (United States)

    ... institutes of the National Institutes of Health (NIH) conduct research related to brain tumors, including pituitary tumors, in their laboratories at ... institutes of the National Institutes of Health (NIH) conduct research related to brain tumors, including pituitary tumors, in their laboratories at ...

  14. Wilms Tumor

    Science.gov (United States)

    ... Wilms tumor is 1 pound at diagnosis. Some children also may have nausea, stomach pain, high blood pressure (hypertension), blood in the urine, loss of appetite, or fever. Even though Wilms tumors often are ...

  15. Hypothalamic tumor

    Science.gov (United States)

    Complications of brain surgery may include: Bleeding Brain damage Death (rarely) Infection Seizures can result from the tumor or from any surgical procedure on the brain. Hydrocephalus can occur with some tumors and ...

  16. 3'-end sequencing for expression quantification (3SEQ from archival tumor samples.

    Directory of Open Access Journals (Sweden)

    Andrew H Beck

    Full Text Available Gene expression microarrays are the most widely used technique for genome-wide expression profiling. However, microarrays do not perform well on formalin fixed paraffin embedded tissue (FFPET. Consequently, microarrays cannot be effectively utilized to perform gene expression profiling on the vast majority of archival tumor samples. To address this limitation of gene expression microarrays, we designed a novel procedure (3'-end sequencing for expression quantification (3SEQ for gene expression profiling from FFPET using next-generation sequencing. We performed gene expression profiling by 3SEQ and microarray on both frozen tissue and FFPET from two soft tissue tumors (desmoid type fibromatosis (DTF and solitary fibrous tumor (SFT (total n = 23 samples, which were each profiled by at least one of the four platform-tissue preparation combinations. Analysis of 3SEQ data revealed many genes differentially expressed between the tumor types (FDR<0.01 on both the frozen tissue (approximately 9.6K genes and FFPET (approximately 8.1K genes. Analysis of microarray data from frozen tissue revealed fewer differentially expressed genes (approximately 4.64K, and analysis of microarray data on FFPET revealed very few (69 differentially expressed genes. Functional gene set analysis of 3SEQ data from both frozen tissue and FFPET identified biological pathways known to be important in DTF and SFT pathogenesis and suggested several additional candidate oncogenic pathways in these tumors. These findings demonstrate that 3SEQ is an effective technique for gene expression profiling from archival tumor samples and may facilitate significant advances in translational cancer research.

  17. Urogenital tumors

    Energy Technology Data Exchange (ETDEWEB)

    Weller, R.E.

    1994-03-01

    An overview is provided for veterinary care of urogenital tumors in companion animals, especially the dog. Neoplasms discussed include tumors of the kidney, urinary bladder, prostate, testis, ovary, vagina, vulva and the canine transmissible venereal tumor. Topics addressed include description, diagnosis and treatment.

  18. Brain Tumors

    Science.gov (United States)

    A brain tumor is a growth of abnormal cells in the tissues of the brain. Brain tumors can be benign, with no cancer cells, ... cancer cells that grow quickly. Some are primary brain tumors, which start in the brain. Others are ...

  19. Odontogenic Tumors

    OpenAIRE

    TAHSİNOĞLU, Melih

    2013-01-01

    DefinitionThe neoplasms that consist of the cells considered specialized for odontogenesis, and their product (dentin, enamel, cementum) are called odontogenic tumors.ClassificationTo initiate odontogenesis, epithelium is a must. Same rule holds for the odontogenic tumors: without odontogenic epithelium, odontogenic tumors cannot be, without the induction of odontogenic epithelium odontogenic mesenchyme cannot develop.

  20. Pindborg tumor

    Science.gov (United States)

    Caliaperoumal, Santhosh Kumar; Gowri, S.; Dinakar, J.

    2016-01-01

    Calcifying epithelial odontogenic tumor (CEOT), also known as Pindborg tumor, is a rare odontogenic epithelial neoplasm. So far, nearly 200 cases have been reported in the literature. We are reporting a case of CEOT in a 42-year-old male patient with painless bony swelling in the mandible. The clinical, radiographic, and histopathologic features are discussed with relevant references. PMID:27041911

  1. Pindborg tumor

    Directory of Open Access Journals (Sweden)

    Santhosh Kumar Caliaperoumal

    2016-01-01

    Full Text Available Calcifying epithelial odontogenic tumor (CEOT, also known as Pindborg tumor, is a rare odontogenic epithelial neoplasm. So far, nearly 200 cases have been reported in the literature. We are reporting a case of CEOT in a 42-year-old male patient with painless bony swelling in the mandible. The clinical, radiographic, and histopathologic features are discussed with relevant references.

  2. Ear Tumors

    Science.gov (United States)

    ... Japanese Espaniol Find information on medical topics, symptoms, drugs, procedures, news and more, written in everyday language. * This is ... the Ears, Nose, and Throat Additional Content Medical News Ear Tumors ... NOTE: This is the Consumer Version. DOCTORS: Click ...

  3. Pituitary tumor

    Science.gov (United States)

    ... enough of its hormones. This condition is called hypopituitarism . The causes of pituitary tumors are unknown. Some ... Cyst Endocrine glands Gigantism Growth hormone test Hyperthyroidism Hypopituitarism Multiple endocrine neoplasia (MEN) I Prolactin blood test ...

  4. Wilms' Tumor

    Science.gov (United States)

    ... team and have training in child development, recreation, psychology or social work. If your child must remain ... conditions/wilms-tumor/basics/definition/CON-20043492 . Mayo Clinic Footer Legal Conditions and Terms Any use of ...

  5. Tumor Grade

    Science.gov (United States)

    ... Other Funding Find NCI funding for small business innovation, technology transfer, and contracts Training Cancer Training at ... much of the tumor tissue has normal breast (milk) duct structures Nuclear grade : an evaluation of the ...

  6. Pituitary Tumors

    Science.gov (United States)

    ... impairment, and behavioral changes. Tumors can also either produce excessive amounts of hormone or limit how much hormone is produced. The hormones most commonly affected include: growth hormone (regulates body height and structure), prolactin (controls ...

  7. Ependymal tumors.

    Science.gov (United States)

    Moynihan, Timothy J

    2003-12-01

    Ependymal tumors are rare malignancies that arise from the cells that line the ventricles and central canal of the spinal cord. Although they are more common in children, adults may also be effected by ependymal tumors. Prognosis is dependent on tumor location, histology, especially for myxopapillary tumors that tend to occur in the lumbar spine, extent of surgical resection, and stage of disease. Standard therapy consists of complete resection when feasible. The exact role of adjuvant radiotherapy in patients with radiographically confirmed complete resection is poorly defined. Patients with known residual disease may benefit from local radiation therapy, but the extent of radiation field and total dose are controversial. Even in patients treated with involved field radiotherapy, most relapses occur within the original tumor bed, thus local control remains the biggest obstacle to effective therapy. Chemotherapy has little impact against this tumor and has no role in the adjuvant setting, outside of a well designed clinical trial, with the possible exception of children younger than 5 years in an effort to delay radiation. A minority of patients may respond to one of several chemotherapy regimens at the time of recurrence, but the impact of this therapy is limited. Newer treatment strategies are needed.

  8. Tumor Types: Understanding Brain Tumors

    Science.gov (United States)

    ... Classification of Tumors of the Central Nervous System Purchase WHO Blue Book NBTS Official Statement Questions and ... Privacy Copyright Site Search Search term Submit Submit Facebook Twitter YouTube Flickr

  9. Testicular tumors

    Directory of Open Access Journals (Sweden)

    Giovanni Rosti

    2011-12-01

    Full Text Available Germ cell tumors of the testes represent a unique paradigm of diseases which can be cured even in extremely advanced phase. Unfortunately, this makes them unique among adult solid tumors. Seminoma and non seminoma are relatively rare with approximatively 25,000 patients in Europe per year, but numbers are increasing world wide. Different strategies are needed depending on stage and prognostic scores. Seminoma is extremely sensitive to radiation therapy and chemotherapy, while all germ cell tumors show a very good response to chemotherapy. Clinical stage I seminoma is currently treated with radiation, single course carboplatin or surveillance policy. Clinical stage I non seminoma can also be approached with different strategies such as retroperitoneal lymph node dissection, observation or one-two courses of standard chemotherapy. Stage II seminoma may be treated with either radiation or chemotherapy, while for all advanced stages chemotherapy is mandatory. Since the mid-eighties PEB (Cisplatin, Etoposide and Bleomycin is the regimen of choice and no other schedule has proved superior in terms of efficacy. Surgery on the residual disease is crucial to the whole strategy and should be performed or attempted in all cases. Consequently, the correct treatment strategy for these tumors does not depend only on the ability of a single physician, but on a skilled team specialized in this particular tumor. Second line therapies (VeIP, PEI, TIP can cure 25%–40% of patients, but improved strategies for resistant tumors are desperately needed. High-dose chemotherapy has shown very good results in some studies while being less impressive in others. In any case, it should remain an option for relapsing patients and could be used in some cases of upfront chemotherapy in patients with slow marker decline, but this should only be considered in referring centers.

  10. Tumor thrombus

    DEFF Research Database (Denmark)

    Ravina, Mudalsha; Hess, Søren; Chauhan, Mahesh Singh

    2014-01-01

    PURPOSE: Thrombosis in cancer may manifest itself as venous thromboembolic disease or tumor thrombosis (TT). We present our experience with incidentally detected TT on FDG PET/CT in 21 oncologic patients. PATIENTS AND METHODS: We retrospectively reviewed all FDG PET/CT examinations during a 5-year......), but most other major branches of the venous vasculature was represented, and some patients had thrombi in several vessels. FDG uptake was linear in 7 patients, linear with a dilated vessel in 6 patients, and focal in 7 patients. The mean SUVmax of the primary tumors was 10.3 (range, 2.6-31.2; median, 6...

  11. Superior sulcus tumors (Pancoast tumors).

    Science.gov (United States)

    Marulli, Giuseppe; Battistella, Lucia; Mammana, Marco; Calabrese, Francesca; Rea, Federico

    2016-06-01

    Superior Sulcus Tumors, frequently termed as Pancoast tumors, are a wide range of tumors invading the apical chest wall. Due to its localization in the apex of the lung, with the potential invasion of the lower part of the brachial plexus, first ribs, vertebrae, subclavian vessels or stellate ganglion, the superior sulcus tumors cause characteristic symptoms, like arm or shoulder pain or Horner's syndrome. The management of superior sulcus tumors has dramatically evolved over the past 50 years. Originally deemed universally fatal, in 1956, Shaw and Paulson introduced a new treatment paradigm with combined radiotherapy and surgery ensuring 5-year survival of approximately 30%. During the 1990s, following the need to improve systemic as well as local control, a trimodality approach including induction concurrent chemoradiotherapy followed by surgical resection was introduced, reaching 5-year survival rates up to 44% and becoming the standard of care. Many efforts have been persecuted, also, to obtain higher complete resection rates using appropriate surgical approaches and involving multidisciplinary team including spine surgeon or vascular surgeon. Other potential treatment options are under consideration like prophylactic cranial irradiation or the addition of other chemotherapy agents or biologic agents to the trimodality approach.

  12. Understanding Brain Tumors

    Science.gov (United States)

    ... to Know About Brain Tumors . What is a Brain Tumor? A brain tumor is an abnormal growth
 ... Tumors” from Frankly Speaking Frankly Speaking About Cancer: Brain Tumors Download the full book Questions to ask ...

  13. Brain tumor - primary - adults

    Science.gov (United States)

    ... Vestibular schwannoma (acoustic neuroma) - adults; Meningioma - adults; Cancer - brain tumor (adults) ... Primary brain tumors include any tumor that starts in the brain. Primary brain tumors can start from brain cells, ...

  14. Brain tumor - children

    Science.gov (United States)

    ... children; Neuroglioma - children; Oligodendroglioma - children; Meningioma - children; Cancer - brain tumor (children) ... The cause of primary brain tumors is unknown. Primary brain tumors may ... (spread to nearby areas) Cancerous (malignant) Brain tumors ...

  15. Adrenal Gland Tumors: Statistics

    Science.gov (United States)

    ... Gland Tumor: Statistics Request Permissions Adrenal Gland Tumor: Statistics Approved by the Cancer.Net Editorial Board , 03/ ... primary adrenal gland tumor is very uncommon. Exact statistics are not available for this type of tumor ...

  16. Pediatric brain tumors

    Energy Technology Data Exchange (ETDEWEB)

    Poussaint, Tina Y. [Department of Radiology, Boston, MA (United States); Panigrahy, Ashok [Children' s Hospital of Pittsburgh of University of Pittsburgh Medical Center, Department of Radiology, Pittsburgh, PA (United States); Huisman, Thierry A.G.M. [Charlotte R. Bloomberg Children' s Center, Johns Hopkins Hospital, Division of Pediatric Radiology and Pediatric Neuroradiology, Baltimore, MD (United States)

    2015-09-15

    Among all causes of death in children from solid tumors, pediatric brain tumors are the most common. This article includes an overview of a subset of infratentorial and supratentorial tumors with a focus on tumor imaging features and molecular advances and treatments of these tumors. Key to understanding the imaging features of brain tumors is a firm grasp of other disease processes that can mimic tumor on imaging. We also review imaging features of a common subset of tumor mimics. (orig.)

  17. Teratoid Wilms′ tumor - A rare renal tumor

    Directory of Open Access Journals (Sweden)

    Biswanath Mukhopadhyay

    2011-01-01

    Full Text Available Teratoid Wilms′ tumor is an extremely rare renal tumor. We report a case of unilateral teratoid Wilms′ tumor in a 4-year-old girl. The patient was admitted with a right-sided abdominal mass. The mass was arising from the right kidney. Radical nephrectomy was done and the patient had an uneventful recovery. Histopathology report showed teratoid Wilms′ tumor.

  18. Cholecystokinin expression in tumors

    DEFF Research Database (Denmark)

    Rehfeld, Jens F

    2016-01-01

    in different neuroendocrine tumors; cerebral gliomas and astrocytomas and specific pediatric tumors. Tumor hypersecretion of CCK was recently reported in a patient with a metastatic islet cell tumor and hypercholecystokininemia resulting in a novel tumor syndrome, the cholecystokininoma syndrome. This review...... presents an overview of the cell-specific biogenesis of CCK peptides, and a description of the CCK expression in tumors and of the cholecystokininoma syndrome. Finally, assays for the diagnosis of CCK-producing tumors are reviewed....

  19. Bronchial carcinoid tumors: A rare malignant tumor

    African Journals Online (AJOL)

    2015-02-03

    Feb 3, 2015 ... Key words: Bronchial carcinoid tumor, diagnosis, outcome, treatment, West .... They can present with carcinoid syndrome, Cushing's ... 2012 reported BCT as a rare differential of pulmonary ... Management of carcinoid tumors.

  20. DCB - Tumor Metastasis Research

    Science.gov (United States)

    Tumor metastasis research examines the mechanisms that allow cancer cells to leave the primary tumor and spread to another part of the body. Learn about recent tumor metastasis research studies supported by the Division of Cancer Biology.

  1. Brain Tumors (For Parents)

    Science.gov (United States)

    ... Old Feeding Your 1- to 2-Year-Old Brain Tumors KidsHealth > For Parents > Brain Tumors Print A ... radiation therapy or chemotherapy, or both. Types of Brain Tumors There are many different types of brain ...

  2. Childhood Brain Tumors

    Science.gov (United States)

    Brain tumors are abnormal growths inside the skull. They are among the most common types of childhood ... still be serious. Malignant tumors are cancerous. Childhood brain and spinal cord tumors can cause headaches and ...

  3. Tumors and Pregnancy

    Science.gov (United States)

    Tumors during pregnancy are rare, but they can happen. Tumors can be either benign or malignant. Benign tumors aren't cancer. Malignant ones are. The most common cancers in pregnancy are breast cancer, cervical cancer, lymphoma, and melanoma. ...

  4. Neuroendocrine Tumor: Statistics

    Science.gov (United States)

    ... Tumor > Neuroendocrine Tumor: Statistics Request Permissions Neuroendocrine Tumor: Statistics Approved by the Cancer.Net Editorial Board , 11/ ... the United States are diagnosed with Merkel cell skin cancer each year. Almost all people diagnosed with the ...

  5. [Desmoid fibromatosis of the posterior mediastinum].

    Science.gov (United States)

    Ayadi-Kaddour, A; Chaabouni, N; Smati, B; Mehouachi, R; Djilani, H; El Mezni, F

    2008-01-01

    Fibromatosis are uncommon connective tissue tumours arising from musculo-aponeurotic tissue and characterised by spindle cell fibroblastic and myofibroblastic proliferation. The exact aetiology is unknown but several factors are considered to be positively correlated with their development and growth (genetic and hormonal factors and trauma). Although they are considered histologically benign they behave aggressively locally and relapse repeatedly after surgical excision. Mediastinal localisation is very rare. We describe a case of de novo fibromatosis of the posterior mediastinum in a 61 year old man with no history of thoracotomy or trauma. Although mediastinal fibromatosis is very uncommon physicians should be aware of this disease in order to ensure appropriate surgical treatment.

  6. Updates in Orbital Tumors

    Institute of Scientific and Technical Information of China (English)

    Nila; F.Moeloek

    1993-01-01

    Orbital anatomy, the clinical features of orbital tumors, the recent development of the diagnosis and management of orbital tumors were described. The incidence of orbital tumors in Dr. Cipto Mangunkusumo Hospital in the past years were introduced. The principle of management of orbital tumors and their prognosis were discussed.

  7. Endocrine Tumor: Overview

    Science.gov (United States)

    ... are here Home > Types of Cancer > Endocrine Tumor Endocrine Tumor This is Cancer.Net’s Guide to Endocrine Tumor. Use the menu below to choose the ... social workers, and patient advocates. Cancer.Net Guide Endocrine Tumor Introduction Statistics Risk Factors Symptoms and Signs ...

  8. Supratentorial tumors; Supratentorielle Tumoren

    Energy Technology Data Exchange (ETDEWEB)

    Grunwald, I.; Dillmann, K.; Roth, C.; Backens, M.; Reith, W. [Universitaetsklinikum Saarland, Homburg (Germany). Klinik fuer Diagnostische und Interventionelle Neuroradiologie

    2007-06-15

    Magnetic resonance imaging is a routine diagnostic measure for a suspected intracerebral mass. Computed tomography is usually also indicated. Further diagnostic procedures as well as the interpretation of the findings vary depending on the tumor location. This contribution discusses the symptoms and diagnostics for supratentorial tumors separated in relation to their intra- or extracranial location. Supratentorial tumors include astrocytoma, differentiated by their circumscribed and diffuse growth, ganglioglioma, ependyoma, neurocytoma, primitive neuroectodermal tumors (PNET), oligodendroglioma, dysembryoplastic neuroepithelial tumors (DNET), meningoangiomatosis, pineal tumors, hamartoma, lymphoma, craniopharyngeoma and metastases. The supratentorial extracranial tumors include the choroid plexus, colloid cysts, meningeoma, infantile myofibromatosis and lipoma. The most common subforms, especially of astrocytoma, will also be presented. (orig.)

  9. Epidemiology of Brain Tumors.

    Science.gov (United States)

    McNeill, Katharine A

    2016-11-01

    Brain tumors are the commonest solid tumor in children, leading to significant cancer-related mortality. Several hereditary syndromes associated with brain tumors are nonfamilial. Ionizing radiation is a well-recognized risk factor for brain tumors. Several industrial exposures have been evaluated for a causal association with brain tumor formation but the results are inconclusive. A casual association between the common mutagens of tobacco, alcohol, or dietary factors has not yet been established. There is no clear evidence that the incidence of brain tumors has changed over time. This article presents the descriptive epidemiology of the commonest brain tumors of children and adults.

  10. Cardiac Tumors; Tumeurs cardiaques

    Energy Technology Data Exchange (ETDEWEB)

    Laissy, J.P.; Fernandez, P. [Centre Hospitalier Universitaire Bichat Claude Bernard, Service d' Imagerie, 76 - Rouen (France); Mousseaux, E. [Hopital Europeen Georges Pompidou (HEGP), Service de Radiologie Cardio Vasculaire et Interventionnelle, 75 - Paris (France); Dacher, J.N. [Centre Hospitalier Universitaire Charles Nicolle, 75 - Rouen (France); Crochet, D. [Centre Hospitalier Universitaire, Hopital Laennec, Centre Hemodynamique, Radiologie Thoracique et Vasculaire, 44 - Nantes (France)

    2004-04-01

    Metastases are the most frequent tumors of the heart even though they seldom are recognized. Most primary cardiac tumors are benign. The main role of imaging is to differentiate a cardiac tumor from thrombus and rare pseudo-tumors: tuberculoma, hydatid cyst. Echocardiography is the fist line imaging technique to detect cardiac tumors, but CT and MRl arc useful for further characterization and differential diagnosis. Myxoma of the left atrium is the most frequent benign cardiac tumor. It usually is pedunculated and sometimes calcified. Sarcoma is the most frequent primary malignant tumor and usually presents as a sessile infiltrative tumor. Lymphoma and metastases are usually recognized by the presence of known tumor elsewhere of by characteristic direct contiguous involvement. Diagnosing primary and secondary pericardial tumors often is difficult. Imaging is valuable for diagnosis, characterization, pre-surgical evaluation and follow-up. (author)

  11. Evaluation of {sup 68}Ga-DOTA-TOC PET/CT for the detection of duodenopancreatic neuroendocrine tumors in patients with MEN1

    Energy Technology Data Exchange (ETDEWEB)

    Morgat, Clement; Mazere, Joachim; Hindie, Elif; Fernandez, Philippe [CNRS, INCIA, Bordeaux (France); University of Bordeaux, INCIA, Bordeaux (France); University Hospital of Bordeaux, Department of Nuclear Medicine, Bordeaux (France); Velayoudom-Cephise, Fritz-Line; Nunes, Marie-Laure; Tabarin, Antoine [USN Haut-Leveque, Department of Endocrinology, Pessac (France); Schwartz, Paul; Guyot, Martine [University Hospital of Bordeaux, Department of Nuclear Medicine, Bordeaux (France); Gaye, Delphine [University Hospital of Bordeaux, Department of Radiology, Pessac (France); Vimont, Delphine; Schulz, Juergen [CNRS, INCIA, Bordeaux (France); University of Bordeaux, INCIA, Bordeaux (France); Smith, Denis [University Hospital of Bordeaux, Department of Oncology, Bordeaux (France)

    2016-07-15

    Somatostatin receptor scintigraphy with {sup 111}In-pentetreotide (SRS) is used to detect duodenopancreatic neuroendocrine tumors (dpNETs) in multiple endocrine neoplasia type 1 (MEN1). However, SRS has limited sensitivity for this purpose. Positron emission tomography/computed tomography (PET/CT) with {sup 68}Ga-DOTA-TOC has a higher rate of sporadic dpNETs detection than SRS but there is little data for dpNETs detection in MEN1. To compare the performances of {sup 68}Ga-DOTA-TOC PET/CT, SRS and contrast-enhanced computed tomography (CE-CT) to diagnose dpNETs in MEN1. Single-institution prospective comparative study Nineteen consecutive MEN1 patients (aged 47 ± 13 years) underwent {sup 68}Ga-DOTA-TOC PET/CT, SRS, and CE-CT within 2 months in random order. Blinded readings of images were performed separately by experienced physicians. Unblinded analysis of CE-CT, combined with additional magnetic resonance imaging, endoscopic-ultrasound, {sup 18}F-2-fluoro-deoxy-d-glucose ({sup 18}F-FDG) PET/CT or histopathology results served as reference standard for dpNETs diagnosis. The sensitivity of {sup 68}Ga-DOTA-TOC PET/CT, SRS, and CE-CT was 76, 20, and 60 %, respectively (p < 0.0001). All the true-positive lesions detected by SRS were also depicted on {sup 68}Ga-DOTA-TOC PET/CT. {sup 68}Ga-DOTA-TOC PET/CT detected lesions of smaller size than SRS (10.7 ± 7.6 and 15.2 ± 5.9 mm, respectively, p < 0.03). False negatives of {sup 68}Ga-DOTA-TOC PET/CT included small dpNETs (<10 mm) and {sup 18}F-FDG PET/CT positive aggressive dpNETs. No false positives were recorded. In addition, whole-body mapping with {sup 68}Ga-DOTA-TOC PET/CT identified extra-abdominal MEN1-related tumors including one neuroendocrine thymic carcinoma identified by the three imaging procedures, one bronchial carcinoid undetected by CE-CT and three meningiomas undetected by SRS. Owing to higher diagnostic performance, {sup 68}Ga-DOTA-TOC PET/CT (or alternative {sup 68}Ga-labeled somatostatin analogues

  12. Gastrointestinal Neuroendocrine Tumors: Pancreatic Endocrine Tumors

    OpenAIRE

    2008-01-01

    Pancreatic endocrine tumors (PETs) have long fascinated clinicians and investigators despite their relative rarity. Their clinical presentation varies depending upon whether the tumor is functional or not and also according to the specific hormonal syndrome produced. Tumors may be sporadic or inherited but little is known about their molecular pathology, especially the sporadic forms. Chromogranin A appears to be the most useful serum marker for diagnosis, staging and monitoring. Initially, t...

  13. Recurring fibromatosis of breast following tumorectomy: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Kum Rae; Park, Won Kyu; Hwang, Mi Soo; Cho, Jae Ho; Bae, Young Kyung [College of Medicine, Yeungnam University, Daegu (Korea, Republic of)

    2008-02-15

    A fibromatosis or desmoid tumor of the breast is an extremely rare benign tumor, which is often presented clinically and radiologically as a malignant tumor. Although benign, fibromatosis or desmoid tumors can grow aggressively in local infiltrating patterns. The recurrence is relatively common; hence a wide excision with a clear margin around the tumor is necessary. We report a case of a young female patient with a recurring fibromatosis of the breast after a local excision.

  14. Metaphyseal giant cell tumor

    Energy Technology Data Exchange (ETDEWEB)

    Pereira, L.F.; Hemais, P.M.P.G.; Aymore, I.L.; Carmo, M.C.R. do; Cunha, M.E.P.R. da; Resende, C.M.C.

    Three cases of metaphyseal giant cell tumor are presented. A review of the literature is done, demostrating the lesion is rare and that there are few articles about it. Age incidence and characteristics of the tumor are discussed.

  15. Tumor suppressor ARF

    Science.gov (United States)

    Través, Paqui G.; Luque, Alfonso; Hortelano, Sonsoles

    2012-01-01

    ARF (alternative reading frame) is one of the most important tumor regulator playing critical roles in controlling tumor initiation and progression. Recently, we have demonstrated a novel and unexpected role for ARF as modulator of inflammatory responses. PMID:23162766

  16. Pediatric Brain Tumor Foundation

    Science.gov (United States)

    ... you insights into your child's treatment. LEARN MORE Brain tumors and their treatment can be deadly so ... Cancer Foundation joins the PBTF Read more >> Pediatric Brain Tumor Foundation 302 Ridgefield Court, Asheville, NC 28806 ...

  17. Renal primitive neuroectodermal tumors.

    Science.gov (United States)

    Bartholow, Tanner; Parwani, Anil

    2012-06-01

    Primitive neuroectodermal tumors exist as a part of the Ewing sarcoma/primitive neuroectodermal tumor family. These tumors most commonly arise in the chest wall and paraspinal regions; cases with a renal origin are rare entities, but have become increasingly reported in recent years. Although such cases occur across a wide age distribution, the average age for a patient with a renal primitive neuroectodermal tumor is the mid- to late 20s, with both males and females susceptible. Histologically, these tumors are characterized by pseudorosettes. Immunohistochemically, CD99 is an important diagnostic marker. Clinically, these are aggressive tumors, with an average 5-year disease-free survival rate of only 45% to 55%. Given that renal primitive neuroectodermal tumor bears many similarities to other renal tumors, it is important to review the histologic features, immunostaining profile, and genetic abnormalities that can be used for its correct diagnosis.

  18. Pituitary Tumors: Condition Information

    Science.gov (United States)

    ... and metabolism. Thyroid-stimulating hormone is involved in growth, body temperature, and heart rate. Nonfunctioning pituitary tumors (also called nonsecretory tumors) do not produce hormones. They can press on or damage the pituitary ...

  19. Labial salivary gland tumors.

    Science.gov (United States)

    Neville, B W; Damm, D D; Weir, J C; Fantasia, J E

    1988-05-15

    A study was conducted on labial salivary gland tumors from four oral pathology laboratories. Of the 103 identified tumors, 87 (84.5%) were from the upper lip, whereas 16 (15.5%) were from the lower lip. Of the 87 upper lip tumors, 80 (92.0%) were benign. Forty-three of these were monomorphic adenomas and 37 were pleomorphic adenomas. Seven malignant tumors of the upper lip were as follows: four adenoid cystic carcinomas, two acinic cell carcinomas, and one adenocarcinoma. Of the 16 lower lip tumors, 15 (93.8%) were malignant. Thirteen of these were mucoepidermoid carcinomas and two were acinic cell carcinomas. The only benign lower lip tumor was an intraductal papilloma. These results confirm the findings of previous investigations, showing that minor salivary gland tumors are much more common in the upper lip than the lower lip, but that lower lip tumors are more likely to be malignant.

  20. Aggressive malignant phyllodes tumor

    OpenAIRE

    Nathan Roberts; Dianne M. Runk

    2015-01-01

    Introduction: Originally described in 1838 by Muller, phyllodes tumor is a rare fibroepithelial neoplasm which represents roughly 0.3–0.9% of all breast cancers. Phyllodes tumor are divided into benign, borderline and malignant histologic categories. Malignant phyllodes tumor represent anywhere from 10–30% of all phyllodes tumors. This group has both the potential to recur locally and metastasize, however not all malignant phyllodes behave this way. The challenge lays in predicting which tumo...

  1. Rhabdoid Tumor Mimicking Hemangioma

    NARCIS (Netherlands)

    Assen, Yvette J.; Madern, Gerard C.; de Laat, Peter C. J.; den Hollander, Jan C.; Oranje, Arnold P.

    2011-01-01

    We report a young boy with a malignant tumor, which remained unrecognized for 8 months because it was assumed to be a hemangioma. The presentation of a rhabdoid tumor mimicking hemangioma is very rare. It was reported only on two earlier occasions. Rhabdoid tumors are one of the most aggressive type

  2. Liver Tumors (For Parents)

    Science.gov (United States)

    ... Teaching Kids to Be Smart About Social Media Liver Tumors KidsHealth > For Parents > Liver Tumors Print A A A What's in this ... Malignant (Cancerous) Tumors Symptoms Diagnosis Treatment Coping The liver is the body's largest solid organ. Lying next ...

  3. Rhabdoid Tumor Mimicking Hemangioma

    NARCIS (Netherlands)

    Assen, Yvette J.; Madern, Gerard C.; de Laat, Peter C. J.; den Hollander, Jan C.; Oranje, Arnold P.

    2011-01-01

    We report a young boy with a malignant tumor, which remained unrecognized for 8 months because it was assumed to be a hemangioma. The presentation of a rhabdoid tumor mimicking hemangioma is very rare. It was reported only on two earlier occasions. Rhabdoid tumors are one of the most aggressive

  4. Calcifying epithelial odontogenic tumor (Pindborg tumor)

    Science.gov (United States)

    Singh, Neeraj; Sahai, Sharad; Singh, Sourav; Singh, Smita

    2011-01-01

    The calcifying epithelial odontogenic tumor (CEOT) is a rare entity and represents less than 1% of all odontogenic tumors. Dr. J J Pindborg (1958) first described four cases of this unusual lesion; subsequently Shafer et al coined the term Pindborg tumor. This lesion is a locally aggressive benign odontogenic neoplasm arising from epithelial tissue. It occurs most commonly in 4th-5th-6th decade of life and bears no gender predilection. A case of CEOT in a 50-year-old male arising in the left body region is described. PMID:22639521

  5. Tumor penetrating peptides

    Directory of Open Access Journals (Sweden)

    Tambet eTeesalu

    2013-08-01

    Full Text Available Tumor-homing peptides can be used to deliver drugs into tumors. Phage library screening in live mice has recently identified homing peptides that specifically recognize the endothelium of tumor vessels, extravasate, and penetrate deep into the extravascular tumor tissue. The prototypic peptide of this class, iRGD (CRGDKGPDC, contains the integrin-binding RGD motif. RGD mediates tumor homing through binding to αv integrins, which are selectively expressed on various cells in tumors, including tumor endothelial cells. The tumor-penetrating properties of iRGD are mediated by a second sequence motif, R/KXXR/K. This C-end Rule (or CendR motif is active only when the second basic residue is exposed at the C-terminus of the peptide. Proteolytic processing of iRGD in tumors activates the cryptic CendR motif, which then binds to neuropilin-1 activating an endocytic bulk transport pathway through tumor tissue. Phage screening has also yielded tumor-penetrating peptides that function like iRGD in activating the CendR pathway, but bind to a different primary receptor. Moreover, novel tumor-homing peptides can be constructed from tumor-homing motifs, CendR elements and protease cleavage sites. Pathologies other than tumors can be targeted with tissue-penetrating peptides, and the primary receptor can also be a vascular zip code of a normal tissue. The CendR technology provides a solution to a major problem in tumor therapy, poor penetration of drugs into tumors. The tumor-penetrating peptides are capable of taking a payload deep into tumor tissue in mice, and they also penetrate into human tumors ex vivo. Targeting with these peptides specifically increases the accumulation in tumors of a variety of drugs and contrast agents, such as doxorubicin, antibodies and nanoparticle-based compounds. Remarkably the drug to be targeted does not have to be coupled to the peptide; the bulk transport system activated by the peptide sweeps along any compound that is

  6. Tumors in invertebrates

    Directory of Open Access Journals (Sweden)

    F Tascedda

    2014-06-01

    Full Text Available Tumors are ectopic masses of tissue formed by due to an abnormal cell proliferation. In this review tumors of several invertebrate species are examined. The description of tumors in invertebrates may be a difficult task, because the pathologists are usually inexperienced with invertebrate tissues, and the experts in invertebrate biology are not familiar with the description of tumors. As a consequence, the terminology used in defining the tumor type is related to that used in mammalian pathology, which can create misunderstandings in some occasions.

  7. Benign ear cyst or tumor

    Science.gov (United States)

    Osteomas; Exostoses; Tumor - ear; Cysts - ear; Ear cysts; Ear tumors; Bony tumor of the ear canal ... bony tumors of the ear canal (exostoses and osteomas) are caused by excess growth of bone. Repeated ...

  8. Tumor interstitial fluid

    DEFF Research Database (Denmark)

    Gromov, Pavel; Gromova, Irina; Olsen, Charlotta J.

    2013-01-01

    Tumor interstitial fluid (TIF) is a proximal fluid that, in addition to the set of blood soluble phase-borne proteins, holds a subset of aberrantly externalized components, mainly proteins, released by tumor cells and tumor microenvironment through various mechanisms, which include classical...... secretion, non-classical secretion, secretion via exosomes and membrane protein shedding. Consequently, the interstitial aqueous phase of solid tumors is a highly promising resource for the discovery of molecules associated with pathological changes in tissues. Firstly, it allows one to delve deeper...... into the regulatory mechanisms and functions of secretion-related processes in tumor development. Secondly, the anomalous secretion of molecules that is innate to tumors and the tumor microenvironment, being associated with cancer progression, offers a valuable source for biomarker discovery and possible targets...

  9. Epilepsy and Brain Tumors

    Institute of Scientific and Technical Information of China (English)

    Zhi-yi Sha

    2009-01-01

    @@ Epidemiology It is estimated 61,414 new cases of primary brain tumors are expected to be diagnosed in 2009 in the U.S. The incidence statistic of 61,414 persons diagnosed per year includes both malignant (22,738) and non-malignant (38,677) brain tumors. (Data from American Brain Tumor Association). During the years 2004-2005, approximately 359,000 people in the United States were living with the diagnosis of a primary brain or central nervous system tumor. Specifically, more than 81,000 persons were living with a malignant tumor, more than 267,000 persons with a benign tumor. For every 100,000 people in the United States, approximately 131 are living following the diagnosis of a brain tumor. This represents a prevalence rate of 130.8 per 100,000 person years[1].

  10. Genital soft tissue tumors.

    Science.gov (United States)

    Schoolmeester, John K; Fritchie, Karen J

    2015-07-01

    Mesenchymal neoplasms of the vulvovaginal and inguinoscrotal regions are among the most diagnostically challenging specimens in the pathology laboratory owing largely to their unique intersection between general soft tissue tumors and relatively genital-specific mesenchymal tumors. Genital stromal tumors are a unique subset of soft tissue tumors encountered at this location, and this group includes fibroepithelial stromal polyp, superficial (cervicovaginal) myofibroblastoma, cellular angiofibroma, mammary-type myofibroblastoma, angiomyofibroblastoma and aggressive angiomyxoma. Aside from the striking morphologic and immunophenotypic similarity that is seen with these entities, there is evidence that a subset of genital stromal tumors may be linked genetically. This review will focus on simplifying this group of tumors and provide the pathologist or dermatopathologist with practical management information. Smooth muscle tumors of the external genitalia will also be discussed.

  11. Targeted tumor radiotherapy

    Directory of Open Access Journals (Sweden)

    Unak Perihan

    2002-01-01

    Full Text Available Targeted tumor radiotherapy is selectively delivery of curative doses of radiation to malignant sites. The aim of the targeted tumor radiotherapy is to use the radionuclides which have high LET particle emissions conjugated to appropriate carrier molecules. The radionuclides are selectively collected by tumor cells, depositing lethal doses to tumor cells while no admission occur to normal cells. In theory, targeted radiotherapy has several advantages over conventional radiotherapy since it allows a high radiation dose to be administered without causing normal tissue toxicity, although there are some limitations in the availability of appropriate targeting agents and in the calculations of administered doses. Therefore, for routine clinical applications more progress is still needed. In this article, the potential use of targeted tumor radiotherapy is briefly reviewed. More general aspects and considerations, such as potential radionuclides, mechanisms of tumor targeting was also outlined.

  12. Uterine primitive neuroectodermal tumor.

    Science.gov (United States)

    Aminimoghaddam, Soheila; Seifirad, Soroush; Abbasi Dezfouli, Golbahar; Abbasi, Neda; Zare Mehrjardi, Ali; Razavi, Seyed Mohsen; Mahmoudzadeh, Fatemeh

    2015-04-01

    Primitive neuroectodermal tumors are fairly rare in uterus. A case of uterine body primitive neuroectodermal tumor in a 32-year-old Iranian woman is presented. The patient was admitted with abdominal pain and fever and underwent emergency exploratory surgery with total abdominal hysterectomy, bilateral salpingo-oophorectomy, and pelvic lymph node dissection. Posterior wall of the uterus was necrotic and ruptured and a huge tumor disrupted the uterine body. The tumor was strongly positive for CD99, NSE, and chromogranin; No reaction was seen for CD10, CD45 and myogenin. To the best of our knowledge, this is the first report of an uterine body primitive neuroectodermal tumor and the second report of uterine primitive neuroectodermal tumor from Iran.

  13. Dentinogenic ghost cell tumor

    Directory of Open Access Journals (Sweden)

    Singhaniya Shikha

    2009-01-01

    Full Text Available Dentinogenic ghost cell tumor (DGCT is a rare tumorous form of calcifying odontogenic cyst and only a small number of cases have been described. It is a locally invasive neoplasm that is characterized by ameloblastoma-like epithelial islands, ghost cells and dentinoid. The present report describes a case of a 21-year-old male with a tumor in the posterior region of the mandible, showing features of DGCT.

  14. Olfactory ensheathing cell tumor

    Directory of Open Access Journals (Sweden)

    Ippili Kaushal

    2009-01-01

    Full Text Available Olfactory ensheathing cells (OECs are found in the olfactory bulb and olfactory nasal mucosa. They resemble Schwann cells on light and electron microscopy, however, immunohistochemical staining can distinguish between the two. There are less than 30 cases of olfactory groove schwannomas reported in the literature while there is only one reported case of OEC tumor. We report an OEC tumor in a 42-year-old male and discuss the pathology and origin of this rare tumor.

  15. TUMORES ANEXIALES Y EMBARAZO

    OpenAIRE

    Tapia M.,Mauricio; Orellana H.,Ricardo; Cisterna C,Patricio; Gazitúa P,Raimundo; Sepúlveda A.,Rodrigo

    2005-01-01

    Objetivo: Evaluar la frecuencia de tumores anexiales en el embarazo, la histología tumoral y los resultados perinatales. Pacientes y método: Análisis retrospectivo de 33 pacientes con diagnóstico de tumor anexial y embarazo atendidas en el Servicio de Obstetricia del Hospital San Juan de Dios entre febrero de 2001 a julio de 2004. Resultados: La asociación tumor anexial y embarazo fue 1 en 424 embarazos. El tipo histológico más frecuente fue el cistoadenoma seroso (19,2%). La cirugía no alter...

  16. Canine mammary gland tumors.

    Science.gov (United States)

    Sorenmo, Karin

    2003-05-01

    The National Consensus Group recommends that all women with tumors larger than 1 cm be offered chemotherapy regardless of tumor histology of lymph node status. This recommendation is to ensure that everyone at risk for failing, even though the risk may be low in women with relatively small tumors and favorable histology, has a choice and receives the benefit of adjuvant chemotherapy. This type of treatment recommendation may also be made in dogs based on recognized, well-accepted prognostic factors such as tumor size, stage, type, and histologic differentiation. Based on the limited clinical information available in veterinary medicine, the drugs that are effective in human breast cancer, such as cyclophosphamide, 5-fluorouracil, and doxorubicin, may also have a role in the treatment of malignant mammary gland tumors in dogs. Randomized prospective studies are needed, however, to evaluate the efficacy of chemotherapy in dogs with high-risk mammary gland tumors and to determine which drugs and protocols are the most efficacious. Until such studies are performed, the treatment of canine mammary gland tumors will be based on the individual oncologist's understanding of tumor biology, experience, interpretation of the available studies, and a little bit of gut-feeling. Table 2 is a proposal for treatment guidelines for malignant canine mammary gland tumors according to established prognostic factors, results from published veterinary studies, and current recommendations for breast cancer treatment in women.

  17. Tumor cell metabolism

    Science.gov (United States)

    Romero-Garcia, Susana; Lopez-Gonzalez, Jose Sullivan; B´ez-Viveros, José Luis; Aguilar-Cazares, Dolores

    2011-01-01

    Cancer is a genetic disease that is caused by mutations in oncogenes, tumor suppressor genes and stability genes. The fact that the metabolism of tumor cells is altered has been known for many years. However, the mechanisms and consequences of metabolic reprogramming have just begun to be understood. In this review, an integral view of tumor cell metabolism is presented, showing how metabolic pathways are reprogrammed to satisfy tumor cell proliferation and survival requirements. In tumor cells, glycolysis is strongly enhanced to fulfill the high ATP demands of these cells; glucose carbons are the main building blocks in fatty acid and nucleotide biosynthesis. Glutaminolysis is also increased to satisfy NADPH regeneration, whereas glutamine carbons replenish the Krebs cycle, which produces metabolites that are constantly used for macromolecular biosynthesis. A characteristic feature of the tumor microenvironment is acidosis, which results from the local increase in lactic acid production by tumor cells. This phenomenon is attributed to the carbons from glutamine and glucose, which are also used for lactic acid production. Lactic acidosis also directs the metabolic reprogramming of tumor cells and serves as an additional selective pressure. Finally, we also discuss the role of mitochondria in supporting tumor cell metabolism. PMID:22057267

  18. Diagnostic value of PET/CT for the staging and restaging of pediatric tumors

    Energy Technology Data Exchange (ETDEWEB)

    Kleis, Margit [University of California (UCSF), Department of Radiology, San Francisco, CA (United States)]|[Technical University of Munich, Department of Radiology, Munich (Germany); Daldrup-Link, Heike; Lu, Ying; Schreck, Carole; Chu, Philip W.; Hawkins, Randall A.; Franc, Benjamin L. [University of California (UCSF), Department of Radiology, San Francisco, CA (United States); Matthay, Katherine [University of California, Department of Pediatrics, Division of Pediatric Hemato-Oncology, San Francisco, CA (United States); Goldsby, Robert [University of California, Department of Pediatric Oncology, San Francisco, CA (United States); Schuster, Tibor [Technical University of Munich, Department of Biostatistics and Epidemiology, Munich (Germany)

    2009-01-15

    The objective of this retrospective study was to compare the diagnostic value of 2-[{sup 18}F]fluoro-2-deoxy-d-glucose positron emission tomography ({sup 18}F-FDG PET)/CT versus {sup 18}F-FDG PET and CT alone for staging and restaging of pediatric solid tumors. Forty-three children and adolescents (19 females and 24 males; mean age, 15.2 years; age range, 6-20 years) with osteosarcoma (n = 1), squamous cell carcinoma (n = 1), synovial sarcoma (n = 2), germ cell tumor (n = 2), neuroblastoma (n = 2), desmoid tumor (n = 2), melanoma (n = 3), rhabdomyosarcoma (n = 5), Hodgkin's lymphoma (n = 7), non-Hodgkin-lymphoma (n = 9), and Ewing's sarcoma (n = 9) who had undergone {sup 18}F-FDG PET/CT imaging for primary staging or follow-up of metastases were included in this study. The presence, location, and size of primary tumors was determined separately for PET/CT, PET, and CT by two experienced reviewers. The diagnosis of the primary tumor was confirmed by histopathology. The presence or absence of metastases was confirmed by histopathology (n = 62) or clinical and imaging follow-up (n = 238). The sensitivities for the detection of solid primary tumors using integrated {sup 18}F-FDG PET/CT (95%), {sup 18}F-FDG PET alone (73%), and CT alone (93%) were not significantly different (p > 0.05). Seventeen patients showed a total of 153 distant metastases. Integrated PET/CT had a significantly higher sensitivity for the detection of these metastases (91%) than PET alone (37%; p < 0.05), but not CT alone (83%; p > 0.05). When lesions with a diameter of less than 0.5 cm were excluded, PET/CT (89%) showed a significantly higher specificity compared to PET (45%; p < 0.05) and CT (55%; p < 0.05). In a sub-analysis of pulmonary metastases, the values for sensitivity and specificity were 90%, 14%, 82% and 63%, 78%, 65%, respectively, for integrated PET/CT, stand-alone PET, and stand-alone CT. For the detection of regional lymph node metastases, {sup 18}F-FDG PET/CT, {sup 18}F

  19. Adenomatoid odontogenic tumor, an uncommon tumor

    Directory of Open Access Journals (Sweden)

    K Vasudevan

    2012-01-01

    Full Text Available Here we report a case of adenomatoid odontogenic tumor (AOT in the maxilla in a young girl aged 14 years and its surgical management. We also review the literature and variations in the nomenclature and classifications of this interesting tumor. The review of literature gives an interesting picture regarding terminologies in the past and dilemma in classifying this tumor. The introduction of the name adenomatoid odontogenic tumour has resulted in the simpler and fruitful surgical management like enucleation and curettage with no reports of recurrences. In the past, similar lesion with the terminology like adeno ameloblastoma has resulted in unnecessary mutilating surgery. The conflicting views whether the lesion is being neoplasm or an anomalous hamartomatous growth is also being discussed.

  20. Tumor carcinoide apendicular Appendiceal carcinoid tumor

    Directory of Open Access Journals (Sweden)

    Julio Vázquez Palanco

    2008-12-01

    Full Text Available El objetivo de este trabajo fue dar a conocer un interesante caso de tumor carcinoide que se presentó con cuadro clínico de apendicitis aguda. El paciente fue un varón de 8 años de edad, al cual se realizó apendicectomía a causa de una apendicitis aguda. El resultado anatomopatológico confirmó un tumor de células endocrinas (argentafinoma, tumor carcinoide en el tercio distal del órgano, que infiltraba hasta la serosa, y apendicitis aguda supurada. El paciente fue enviado a un servicio de oncohematología para tratamiento oncoespecífico. Por lo inusual de estos tumores en edades tempranas y por lo que puede representar para el niño una conducta no consecuente, decidimos presentar este caso a la comunidad científica nacional e internacional. Es extremadamente importante el seguimiento de los pacientes con apendicitis aguda y de las conclusiones del examen histológico, por lo que puede representar para el niño una conducta inadecuada en una situación como esta.The objective of this paper was to make known an interesting case of carcinoid tumor that presented a clinical picture of acute appendicitis.The patient was an eight-year-old boy that underwent appendectomy due to an acute appendicitis. The anatomopathological report confirmed an endocrine cell tumor (argentaffinoma, carcinoid tumor in the distal third of the organ that infiltrated up to the serosa, and acute suppurative appendicitis. The patient was referred to an oncohematology service for oncospecific treatment. As it is a rare tumor at early ages, and taking into account what a inconsequent behavior may represent for the child, it was decided to present this case to the national and international scientific community. The follow-up of the patients with acute appendicitis and of the conclusions of the histological examination is extremely important considering what an inadequate conduct may represent for the child in a situation like this.

  1. Renal inflammatory myofibroblastic tumor

    DEFF Research Database (Denmark)

    Heerwagen, S T; Jensen, C; Bagi, P

    2007-01-01

    Renal inflammatory myofibroblastic tumor (IMT) is a rare soft-tissue tumor of controversial etiology with a potential for local recurrence after incomplete surgical resection. The radiological findings in renal IMT are not well described. We report two cases in adults with a renal mass treated...

  2. Atypically localized glomus tumors

    Directory of Open Access Journals (Sweden)

    Meric Ugurlar

    2016-12-01

    Conclusion: When a painful mass is found in the body, glomus tumors should be kept in mind. The consideration of symptoms, including pain, temperature sensitivity, point tenderness, and discoloration, common characteristics of glomus tumors, may aid diagnosis. [Hand Microsurg 2016; 5(3.000: 112-117

  3. Pseudoanaplastic tumors of bone

    Energy Technology Data Exchange (ETDEWEB)

    Bahk, Won-Jong [Uijongbu St. Mary Hospital, The Catholic University of Korea, Department of Orthopaedic Surgery, Gyunggido, 480-821 (Korea); Mirra, Joseph M. [Orthopaedic Hospital, Orthopedic Oncology, Los Angeles, California (United States)

    2004-11-01

    To discuss the concept of pseudoanaplastic tumors of bone, which pathologically show hyperchromatism and marked pleomorphism with quite enlarged, pleomorphic nuclei, but with no to extremely rare, typical mitoses, and to propose guidelines for their diagnosis. From a database of 4,262 bone tumors covering from 1971 to 2001, 15 cases of pseudoanaplastic bone tumors (0.35% of total) were retrieved for clinical, radiographic and pathologic review. Postoperative follow-up after surgical treatment was at least 3 years and a maximum of 7 years. There were eight male and seven female patients. Their ages ranged from 10 to 64 years with average of 29.7 years. Pathologic diagnoses of pseudoanaplastic variants of benign bone tumors included: osteoblastoma (4 cases), giant cell tumor (4 cases), chondromyxoid fibroma (3 cases), fibrous dysplasia (2 cases), fibrous cortical defect (1 case) and aneurysmal bone cyst (1 case). Radiography of all cases showed features of a benign bone lesion. Six cases, one case each of osteoblastoma, fibrous dysplasia, aneurysmal bone cyst, chondromyxoid fibroma, giant cell tumor and osteoblastoma, were initially misdiagnosed as osteosarcoma. The remaining cases were referred for a second opinion to rule out sarcoma. Despite the presence of significant cytologic aberrations, none of our cases showed malignant behavior following simple curettage or removal of bony lesions. Our observation justifies the concept of pseudoanaplasia in some benign bone tumors as in benign soft tissue tumors, especially in their late evolutionary stage when bizarre cytologic alterations strongly mimic a sarcoma. (orig.)

  4. Vanishing tumor in pregnancy

    Directory of Open Access Journals (Sweden)

    M V Vimal

    2012-01-01

    Full Text Available A patient with microprolactinoma, who had two successful pregnancies, is described for management issues. First pregnancy was uneventful. During the second pregnancy, the tumor enlarged to macroprolactinoma with headache and blurring of vision which was managed successfully with bromocriptine. Post delivery, complete disappearance of the tumor was documented.

  5. [Prognosis in tumor diagnosis].

    Science.gov (United States)

    Bruns, G

    1983-01-01

    This essay on prognosis in tumor diagnosis pathology resp. tumor etiology, cancerogenesis and molecular oncology is the authors personal opinion. Generally tumor diagnosis improves with progress in histo- and cytological methods for example in tumors of the APUD-system and precancerous lesions especially of the breast. Fundamental principle of developed tumor diagnosis is the knowledge of malignant transformation. Its profits favours the etiology in cancerogenesis and e.g. non Hodgkin lymphomas (Burkitt lymphoma, adult T-cell lymphoma) and to result from new methods in molecular biology and viral genetics (DNS-hybridizing, -recombination and gene technology). With the beginning century a stepwise fitted diagnosis of malignant lymphomas is evident up to monoclonal dedifferentiated lymphoid cells and their multifarious phenotypical markers. This concept may be of general significance in tumor diagnosis already indicated in prelymphomas. Finally the present prognosis of tumor diagnosis is evaluated by contents, tasks and strategies of its corresponding research lines in clinical and experimental tumor pathology resp. its organization.

  6. [Circulating "tumor markers" in gastrointestinal tumors].

    Science.gov (United States)

    Borlinghaus, P; Lamerz, R

    1991-09-01

    Tumor markers (TM) of the neoplastic cell can be divided into non-shedded substances and antigens shedded in blood, urine or other body fluids. For clinicians circulating TM are more important. All relevant circulating TM are not useful in screening of asymptomatic patients because of insufficient sensitivity and specificity. With caution they are useful in the observation of risk groups. Circulating TM have their main significance as additional parameters in monitoring symptomatic patients with malignancies. Several follow up determinations are more important than one single measurement. During follow up of tumor patients TM should not be checked automatically if there are no diagnostic or therapeutical consequences. The clinically most important circulating TM in non-hormone secreting tumors of the gastrointestinal tract are the oncofetal antigens CEA and AFP and antigens defined by monoclonal antibodies e. g. CA 19-9 and CA 72-4. AFP is the primary TM in hepatocellular carcinoma, often elevated in hepatoblastoma and always normal in cholangiocellular carcinoma. CEA is the TM of first choice in patients with colorectal carcinomas and liver metastasis. CA 19-9 is TM of first choice in pancreatic carcinoma and additionally of diagnostic value in cholangiocellular carcinoma and tumors of the bile ducts. In cancer of the stomach CA 19-9 and CEA are secondary TM in combination with CA 72-4 as primary TM. Care should be taken that slight and moderate elevations of TM can be observed in benign diseases of liver, pancreas and bowel.

  7. Pulmonary neuroendocrine (carcinoid) tumors

    DEFF Research Database (Denmark)

    Caplin, M E; Baudin, E; Ferolla, P

    2015-01-01

    carried out in PubMed for the terms 'pulmonary neuroendocrine tumors', 'bronchial neuroendocrine tumors', 'bronchial carcinoid tumors', 'pulmonary carcinoid', 'pulmonary typical/atypical carcinoid', and 'pulmonary carcinoid and diagnosis/treatment/epidemiology/prognosis'. A systematic review...... of the relevant literature was carried out, followed by expert review. RESULTS: PCs are well-differentiated neuroendocrine tumors and include low- and intermediate-grade malignant tumors, i.e. typical (TC) and atypical carcinoid (AC), respectively. Contrast CT scan is the diagnostic gold standard for PCs......, particularly of low-grade TC and AC. Locoregional or radiotargeted therapies should be considered for metastatic disease. Systemic chemotherapy is used for progressive PCs, although cytotoxic regimens have demonstrated limited effects with etoposide and platinum combination the most commonly used, however...

  8. Familial pituitary tumor syndromes.

    Science.gov (United States)

    Elston, Marianne S; McDonald, Kerrie L; Clifton-Bligh, Roderick J; Robinson, Bruce G

    2009-08-01

    The vast majority of pituitary tumors are benign and occur sporadically; however, they can still result in significant morbidity and even premature mortality through mass effects and hormone dysfunction. The etiology of sporadic tumors is still poorly understood; by contrast, advances have been made in our understanding of familial pituitary adenoma syndromes in the past decade. Currently, four genes are known to be associated with familial pituitary tumor syndromes: MEN1, CDKN1B, PRKAR1A and AIP. The first three genes are associated with a variety of extrapituitary pathologies, for example, primary hyperparathyroidism with multiple endocrine neoplasia type 1, which might aid identification of these syndromes. By contrast, AIP mutations seem to occur in the setting of isolated familial pituitary adenomas, particularly of the growth-hormone-secreting subtype. Awareness and identification of familial pituitary tumor syndromes is important because of potential associated pathologies and important implications for family members. Here, we review the current knowledge of familial pituitary tumor syndromes.

  9. Merkel cell tumor.

    Science.gov (United States)

    Kitazawa, M; Watanabe, H; Kobayashi, H; Ohnishi, Y; Shitara, A; Nitto, H

    1987-06-01

    A Merkel cell tumor appeared on the left cheek of an 83-year-old female was reported. The tumor was located mainly in the dermis and infiltrated to the subcutaneous adipose tissue with an involvement of the blood vessels and lymphatics at the periphery. Electron-microscopically, few of the dense-cored granules and the single globular aggregates of intermediate filaments at the nuclear indentations were observed. Electron-microscopic uranaffin reaction proved positive reaction on the dense-cored granules. Half of the cytoplasmic border was smooth, while the rest had short projections. Desmosomes or junctional complexes were not detected among the tumor cells. Immunohistochemically, the cytoplasm of tumor cell showed positive reaction to both neuron-specific enolase (NSE) and keratin. The single globular positive spots of the latter were localized in accordance with the aggregates of intermediate filaments. These findings suggested a neurogenic origin with double differentiation, epithelial and neuroendocrine, of the Merkel cell tumor.

  10. Gastrointestinal carcinoid tumors Tumores carcinoides digestivos

    Directory of Open Access Journals (Sweden)

    M. J. Varas Lorenzo

    2010-09-01

    Full Text Available Objective: carcinoid tumors (CTs represent the commonest neuroendocrine tumors. Those in the gastrointestinal tract are diagnosed in surgical specimens, clinically, and using imaging techniques (endoscopy, echoendoscopy, CT, Octreoscan, etc.. The goal of this retrospective study was to review a personal series of gastrointestinal carcinoid tumors, and to compare it to those in the literature. Patients and methods: the medical records of 40 Caucasian patients with over 50 gastrointestinal carcinoid tumors (including multiple cases who were seen for a period of 16 years (1994-2009 were reviewed. Results: mean age at presentation was 52 years, 50% were females, and mean tumor size was 9.9 mm. Most were gastroduodenal (42.5% or rectal (30%, and were treated endoscopically. Metastases and carcinoid syndrome (CS were seen in 5% of patients. Survival at study endpoint was 85%. Conclusions: age and gender were consistent with the literature. There was an increase in gastroduodenal (multifocal and rectal carcinoids, likely because the series was essentially endoscopical in nature (bias. There was a lower rate of CS and higher survival, likely due to earlier diagnosis and treatment.Objetivo: los tumores carcinoides (TC son los tumores neuroendocrinos más frecuentes. Los digestivos se diagnostican en las piezas quirúrgicas, en la clínica, y mediante los métodos de imagen (endoscopia, ecoendoscopia, TAC y Octreoscan, etc.. El objetivo de este trabajo retrospectivo fue revisar una serie personal de tumores carcinoides digestivos y compararla con la literatura. Pacientes y métodos: se revisaron las historias clínicas de 40 pacientes de raza blanca con más de 50 tumores carcinoides digestivos, algunos múltiples, observados durante 16 años (1994-2009. Resultados: la edad media de presentación fue 52 años, 50% mujeres, con un tamaño medio del tumor de 9,9 mm. La mayoría eran gastroduodenales (42,5% y rectales (30% y fueron tratados por vía endosc

  11. Parallel evolution of tumor subclones mimics diversity between tumors

    DEFF Research Database (Denmark)

    Martinez, Pierre; Birkbak, Nicolai Juul; Gerlinger, Marco

    2013-01-01

    Intratumor heterogeneity (ITH) may foster tumor adaptation and compromise the efficacy of personalized medicines approaches. The scale of heterogeneity within a tumor (intratumor heterogeneity) relative to genetic differences between tumors (intertumor heterogeneity) is unknown. To address this, ...

  12. Iatrogenic Tumor Implantation

    Institute of Scientific and Technical Information of China (English)

    Ying Ma; Ping Bai

    2008-01-01

    Iatrogenic tumor implantation is a condition that results from various medical procedures used during diagnosis or treatment of a malignancy. It involves desquamation and dissemination of tumor cells that develop into a local recurrence or distant metastasis from the tumor under treatment. The main clinical feature of the condition is nodules at the operation's porous channel or incision, which is easily diagnosed in accordance with the case history. Final diagnosis can be made based on pathological examination. Tumor implantation may occur in various puncturing porous channels, including a laparoscopic port, abdominal wall incision, and perineal incision, etc. Besides a malignant tumor,implantation potential exists with diseases, such as a borderline tumor and endometriosis etc. Once a tumor implantation is diagnosed, or suspected, surgical resection is usually conducted.During the diagnosis and treatment of diseases, avoiding and reducing iatrogenic implantation and dissemination has been regarded as an important principle for surgical treatment of tumors. In a clinical practice setting, if possible, excisional biopsy should be employed, if a biopsy is needed. Repeated puncturing should be avoided during a paracentesis. In a laparoscopic procedure, the tissue is first put into a sample bag and then is taken out from the point of incision. After a laparoscopic procedure, the peritoneum, abdominal muscular fasciae, and skin should be carefully closed, and/or the punctured porous channel be excised. In addition, the sample/tissue should be rinsed with distilled water before surgical closure of the abdominal cavity,allowing the exfoliated tumor cells to swell and rupture in the hypo-osmolar solution. Then surgical closure can be conducted following a change of gloves and equipment. The extent of hysteromyomectomy should as far as possible be away from the uterine cavity. The purpose of this study is to make clinicians aware of the possibility of tumor implantation

  13. The management of parotid tumors

    Institute of Scientific and Technical Information of China (English)

    Guang Yan Yu

    2008-01-01

    @@ The majority of salivary gland tumors are of epithelial origin. Parotid gland is the most common location of the tumors. Surgery is the main modality for the management of parotid tumors. Radiotherapy, chemotherapy etc are the auxiliary modalities.

  14. General Information about Pituitary Tumors

    Science.gov (United States)

    ... Tumors Treatment Pituitary Tumors Treatment (PDQ®)–Patient Version General Information About Pituitary Tumors Go to Health Professional ... by many other glands in the body. Enlarge Anatomy of the inside of the brain, showing the ...

  15. Epilepsy and brain tumors

    Science.gov (United States)

    ENGLOT, DARIO J.; CHANG, EDWARD F.; VECHT, CHARLES J.

    2016-01-01

    Seizures are common in patients with brain tumors, and epilepsy can significantly impact patient quality of life. Therefore, a thorough understanding of rates and predictors of seizures, and the likelihood of seizure freedom after resection, is critical in the treatment of brain tumors. Among all tumor types, seizures are most common with glioneuronal tumors (70–80%), particularly in patients with frontotemporal or insular lesions. Seizures are also common in individuals with glioma, with the highest rates of epilepsy (60–75%) observed in patients with low-grade gliomas located in superficial cortical or insular regions. Approximately 20–50% of patients with meningioma and 20–35% of those with brain metastases also suffer from seizures. After tumor resection, approximately 60–90% are rendered seizure-free, with most favorable seizure outcomes seen in individuals with glioneuronal tumors. Gross total resection, earlier surgical therapy, and a lack of generalized seizures are common predictors of a favorable seizure outcome. With regard to anticonvulsant medication selection, evidence-based guidelines for the treatment of focal epilepsy should be followed, and individual patient factors should also be considered, including patient age, sex, organ dysfunction, comorbidity, or cotherapy. As concomitant chemotherapy commonly forms an essential part of glioma treatment, enzyme-inducing anticonvulsants should be avoided when possible. Seizure freedom is the ultimate goal in the treatment of brain tumor patients with epilepsy, given the adverse effects of seizures on quality of life. PMID:26948360

  16. Laryngeal inflammatory myofibroblastic tumor.

    Science.gov (United States)

    Girardi, Fábio M; Fontana, Ciro W; Kroef, Ricardo G; Barra, Marinez B; Detânico, Felipe O; Herter, Nilton T

    2014-12-01

    Inflammatory myofibroblastic tumor seldom involves the larynx, as only about 50 to 60 cases have been described in the literature. Even though these tumors are often not aggressive, they have the potential for invasion and local recurrence. We describe the case of a 27-year-old man who was admitted to an emergency department with signs of upper airway obstruction secondary to an obstructive mass. Histology identified the mass as an inflammatory myofibroblastic tumor of the subglottis. The patient underwent an emergency tracheotomy followed by a partial laryngectomy. During 14 months of follow-up, he remained free of active disease.

  17. Targeting the tumor microenvironment

    Energy Technology Data Exchange (ETDEWEB)

    Kenny, P.A.; Lee, G.Y.; Bissell, M.J.

    2006-11-07

    Despite some notable successes cancer remains, for the most part, a seemingly intractable problem. There is, however, a growing appreciation that targeting the tumor epithelium in isolation is not sufficient as there is an intricate mutually sustaining synergy between the tumor epithelial cells and their surrounding stroma. As the details of this dialogue emerge, new therapeutic targets have been proposed. The FDA has already approved drugs targeting microenvironmental components such as VEGF and aromatase and many more agents are in the pipeline. In this article, we describe some of the 'druggable' targets and processes within the tumor microenvironment and review the approaches being taken to disrupt these interactions.

  18. Uterine mesenchymal tumors

    Directory of Open Access Journals (Sweden)

    Nikhil A Sangle

    2011-01-01

    Full Text Available Uterine mesenchymal tumors are a heterogeneous group of neoplasms that can frequently be diagnostically challenging. Differentiation between the benign and malignant counterparts of mesenchymal tumors is significant due to differences in clinical outcome, and the role of the surgical pathologist in making this distinction (especially in the difficult cases cannot be underestimated. Although immunohistochemical stains are supportive toward establishing a final diagnosis, the morphologic features trump all the other ancillary techniques for this group of neoplasms. This review therefore emphasizes the key morphologic features required to diagnose and distinguish uterine mesenchymal tumors from their mimics, with a brief description of the relevant immunohistochemical features.

  19. SURVIVIN AND TUMOR

    Institute of Scientific and Technical Information of China (English)

    宋文哲; 宋燕; 叶剑桥; 邱东涛

    2003-01-01

    As a new member of IAP (inhibitors of apoptosis protein) family, survivin has potent anti-apoptotic activities, and involves in the mitosis and angiogenesis. Researches have demonstrated that surviving is a tumor-specific anti-apoptotic factor, expressed in fetal tissues, and common human cancers, while not in normal, terminally differentiated adult tissues. The overexpression of survivin in tumor tissues is correlated with poor prognosis of the patients. Survivin can be used as a prognostic factor and a new target in tumor targeting therapy.

  20. Myoepithelial Tumors: An Update.

    Science.gov (United States)

    Jo, Vickie Y

    2015-09-01

    Primary myoepithelial neoplasms of soft tissue are uncommon, and have been increasingly characterized by clinicopathologic and genetic means. Tumors are classified as mixed tumor/chondroid syringoma, myoepithelioma, and myoepithelial carcinoma, and they share morphologic, immunophenotypic, and genetic features with their salivary gland counterparts. However, soft tissue myoepithelial tumors are classified as malignant based on the presence of cytologic atypia, in contrast to the criterion of invasive growth in salivary gland sites. This review discusses the clinicopathologic and morphologic characteristics, distinct variants, and currently known genetic alterations of myoepithelial neoplasms of soft tissue, skin, and bone.

  1. Tumor-Associated Macrophages and Neutrophils in Tumor Microenvironment

    Directory of Open Access Journals (Sweden)

    Jaehong Kim

    2016-01-01

    Full Text Available Distinct tumor microenvironment forms in each progression step of cancer and has diverse capacities to induce both adverse and beneficial consequences for tumorigenesis. It is now known that immune cells can be activated to favor tumor growth and progression, most probably influenced by the tumor microenvironment. Tumor-associated macrophages and tumor-associated neutrophils can exert protumoral functions, enhancing tumor cell invasion and metastasis, angiogenesis, and extracellular matrix remodeling, while inhibiting the antitumoral immune surveillance. Considering that neutrophils in inflammatory environments recruit macrophages and that recruited macrophages affect neutrophil functions, there may be various degrees of interaction between tumor-associated macrophages and tumor-associated neutrophils. Platelets also play an important role in the recruitment and regulation of monocytic and granulocytic cells in the tumor tissues, suggesting that platelet function may be essential for generation of tumor-associated macrophages and tumor-associated neutrophils. In this review, we will explore the biology of tumor-associated macrophages and tumor-associated neutrophils and their possible interactions in the tumor microenvironment. Special attention will be given to the recruitment and activation of these tumor-associated cells and to the roles they play in maintenance of the tumor microenvironment and progression of tumors.

  2. Primary hepatic carcinoid tumor

    Directory of Open Access Journals (Sweden)

    Gao Jinbo

    2011-11-01

    Full Text Available Abstract Primary hepatic carcinoid tumor is rare and poses a challenge for diagnosis and management. We presented a case of primary hepatic carcinoid tumor in a 53-year-old female with a complaint of right upper abdominal pain. Computer tomography scans revealed a hypervascular mass in segment 4 of the liver. An ultrasonography-guided biopsy showed a carcinoid tumor. No other lesions were found by the radiological investigations. Surgery resection was performed and histopathological examination revealed a primary hepatic carcinoid tumor. Three years later, recurrence was found and transcatheter arterial chemoembolization was performed. After transcatheter arterial chemoembolization, the patient has been free of symptom and had no radiological disease progression for over 6 months. Surgical resection combination with transcatheter arterial chemoembolization is effective to offer excellent palliation.

  3. Benign Liver Tumors

    Science.gov (United States)

    ... A Life After Diagnosis Support for Chronic Illness Corporate Partnerships Interview with Kristen Hanks Liver Lowdown July ... Disease , Liver Transplant , Liver Cancer , Liver Tumor , Liver Failure Help Fight Liver Disease We rely upon donations ...

  4. GASTROENTEROPANCREATIC NEUROENDOCRINE TUMORS ...

    African Journals Online (AJOL)

    biology and natural history of a high grade malig- nancy and a ... tumors arise in the gastrointestinal tract and collectively, they are ... jaundice, intra-abdominal mass and bleeding. Patients ... tion of the primary lesion and definition of the extent.

  5. [Radiation induced tumors].

    Science.gov (United States)

    Gutiérrez Bayard, L; Delgado López, L; Tirado Bejarano, C; Gómez Puerto, A; García Fernández, J L

    1998-04-01

    Radiations at cellular level produce different effects, depending on type of radiation and irradiated tissue. The radiation-induced cancers are associated to non-letals genetics mutations, and to classify like radiation induced tumors is necessary that appear in the treatment volume, a long latency period (years), histolo-different to the primary lesion, enough doses quantitatively and that exists a greater incidence in the irradiated populations. The genetics mutations affect at tumoral suppressors gen(Gen RB I, p53, BRCA I, BRCA 2) and repressors gen (hMSH 2, hMLH I,...), they could be longer and multifocals mutations, and produce lack of cellular control and a greater predisposition to develop tumors and a probable risk of increment of radiosensitivity. We present some of the more representatives studies about radiation-induced tumors.

  6. Nonislet Cell Tumor Hypoglycemia

    Directory of Open Access Journals (Sweden)

    Johnson Thomas

    2013-01-01

    Full Text Available Nonislet cell tumor hypoglycemia (NICTH is a rare cause of hypoglycemia. It is characterized by increased glucose utilization by tissues mediated by a tumor resulting in hypoglycemia. NICTH is usually seen in large mesenchymal tumors including tumors involving the GI tract. Here we will discuss a case, its pathophysiology, and recent advances in the management of NICTH. Our patient was diagnosed with poorly differentiated squamous cell carcinoma of esophagus. He continued to be hypoglycemic even after starting continuous tube feeds and D5W. General workup for hypoglycemia was negative and insulin-like growth factor II (IGF II was in the normal range. Hypoglycemia secondary to “big” IGF II was considered, and patient was started on steroids. His hypoglycemia resolved within a day of treatment with steroids. Initially patient had hypoglycemia unawareness, which he regained after maintaining euglycemia for 48 hours.

  7. Osteochondroma (Bone Tumor)

    Science.gov (United States)

    ... to be the most common benign bone tumor, accounting for 35% to 40% of all benign bone ... imaging scans. Doctors may also request computed tomography (CT) scans or magnetic resonance imaging (MRI) scans to ...

  8. Brain Tumors and Fatigue

    Science.gov (United States)

    ... can help calm the mind. Meditation, guided imagery, music therapy, and yoga are just a few worth investigating. Home Donor and Privacy Policies Find Resources Disclaimer Donate Subscribe Login American Brain Tumor Association 8550 W. Bryn Mawr Ave. Ste ...

  9. [Metastatic bronchial carcinoid tumors].

    Science.gov (United States)

    Bouledrak, K; Walter, T; Souquet, P J; Lombard-Bohas, C

    2016-02-01

    Bronchial carcinoids are uncommon pulmonary neoplasms and represent 1 to 2 % of all lung tumors. In early stage of disease, the mainstay and only curative treatment is surgery. Bronchial carcinoids are generally regarded as low-grade carcinomas and metastatic dissemination is unusual. The management of the metastatic stage is not currently standardized due to a lack of relevant studies. As bronchial carcinoids and in particular their metastatic forms are rare, we apply treatment strategies that have been evaluated in gastrointestinal and pancreatic neuroendocrine tumors. However, bronchial carcinoids have their own characteristic. A specific therapeutic feature of these metastatic tumors is that they require a dual approach: both anti-secretory for the carcinoid syndrome, and anti-tumoral.

  10. Pituitary tumors containing cholecystokinin

    DEFF Research Database (Denmark)

    Rehfeld, J F; Lindholm, J; Andersen, B N

    1987-01-01

    We found small amounts of cholecystokinin in the normal human adenohypophysis and therefore examined pituitary tumors from 87 patients with acromegaly, Cushing's disease, Nelson's syndrome, prolactinoma, or inactive pituitary adenomas. Five adenomas associated with Nelson's syndrome contained...

  11. Overview of Heart Tumors

    Science.gov (United States)

    ... develop in the sac that surrounds the heart (pericardium). Tumors in the pericardium may squeeze (constrict) the heart, preventing it from ... Heart wall Fibroma Hemangioma Rhabdomyoma Outside surface Lipoma Pericardium (outer sac covering heart) Pericardial cyst Base of ...

  12. GASTROINTESTINAL STROMAL TUMOR (GIST

    Directory of Open Access Journals (Sweden)

    Luigi eTornillo

    2014-11-01

    Full Text Available Gastrointestinal stromal tumors are the most frequent mesenchymal tumors of the gastrointestinal tract. The discovery that these tumors, formerly thought of smooth muscle origin, are indeed better characterized by specific activating mutation in genes coding for the receptor tyrosine kinases CKIT and PDGFRA and that these mutations are strongly predictive for the response to targeted therapy with receptor tyrosine kinase inhibitors has made GISTs the typical example of the integration of basic molecular knowledge in the daily clinical activity. The information on the mutational status of these tumors is essential to predict (and subsequently to plan the therapy. As resistant cases are frequently wild-type, other possible oncogenic events, defining other entities, have been discovered (e.g. succinil dehydrogenase mutation/dysregulation, insuline growth factor expression, mutations in the RAS-RAF-MAPK pathway. The classification of disease must nowadays rely on the integration of the clinico-morphological characteristics with the molecular data.

  13. Brain Tumor Surgery

    Science.gov (United States)

    ... Meningitis Brain swelling Stroke Excess fluid in the brain Coma Death Recovery Time Recovery time depends on: The procedure performed. The part of the brain where the tumor is/was located. The areas ...

  14. Neuropathic pain due to fibromatosis: Does anticancer treatment help?

    Directory of Open Access Journals (Sweden)

    David Mathew

    2011-01-01

    Full Text Available Desmoid fibromatosis, although histologically benign, infiltrates local structures. The involvement of neural structures can lead to difficult neuropathic pain and the escalating use of analgesics. We report a patient with desmoid fibromatosis of the chest wall causing brachial plexus infiltration. As the tumor was locally invasive and unresectable, he was treated with radiation therapy and oral tamoxifen. On follow-up, there was significant pain relief, sustained reduction in the tumor size, and reduced analgesic requirement. Antineoplastic treatments like local radiation therapy and targeted systemic therapy with hormones or other agents can be considered in the management of selected unresectable desmoid fibromatosis to improve symptom control and reduce polypharmacy.

  15. Tracheal and bronchial tumors.

    Science.gov (United States)

    Varela, Patricio; Pio, Luca; Brandigi, Elisa; Paraboschi, Irene; Khen-Dunlop, Nazhia; Hervieux, Erik; Muller, Cecile; Mattioli, Girolamo; Sarnacki, Sabine; Torre, Michele

    2016-12-01

    Although primary tracheobronchial tumors are extremely rare in children, recurrent respiratory symptoms resistant to conventional therapy require further investigations to exclude possible malignant obstructive causes. As the matter of fact, early diagnosis may allow minimally invasive surgeries, improving the standard of living and the globally survival rate. The aim of this article is to provide an overview of diagnosis and management of tracheobronchial tumors in the early age, since only few reports are reported in the worldwide literature.

  16. Perlecan and tumor angiogenesis

    DEFF Research Database (Denmark)

    Jiang, Xinnong; Couchman, John R

    2003-01-01

    Perlecan is a major heparan sulfate proteoglycan (HSPG) of basement membranes (BMs) and connective tissues. The core protein of perlecan is divided into five domains based on sequence homology to other known proteins. Commonly, the N-terminal domain I of mammalian perlecan is substituted with thr...... have unwanted promoting effects on tumor cell proliferation and tumor angiogenesis. Understanding of these attributes at the molecular level may offer opportunities for therapeutic intervention....

  17. Pituitary tumors containing cholecystokinin

    DEFF Research Database (Denmark)

    Rehfeld, J F; Lindholm, J; Andersen, B N

    1987-01-01

    We found small amounts of cholecystokinin in the normal human adenohypophysis and therefore examined pituitary tumors from 87 patients with acromegaly, Cushing's disease, Nelson's syndrome, prolactinoma, or inactive pituitary adenomas. Five adenomas associated with Nelson's syndrome contained......'s disease and 7 acromegaly with adenomas containing ACTH. The cholecystokinin peptides from the tumors were smaller and less sulfated than cholecystokinin from normal pituitary glands. We conclude that ACTH-producing pituitary cells may also produce an altered form of cholecystokinin....

  18. Adrenocortical tumors in children

    Directory of Open Access Journals (Sweden)

    R.C. Ribeiro

    2000-10-01

    Full Text Available Childhood adrenocortical tumors (ACT are rare. In the USA, only about 25 new cases occur each year. In Southern Brazil, however, approximately 10 times that many cases are diagnosed each year. Most cases occur in the contiguous states of São Paulo and Paraná. The cause of this higher rate has not been identified. Familial genetic predisposition to cancer (p53 mutations and selected genetic syndromes (Beckwith-Wiedemann syndrome have been associated with childhood ACT in general but not with the Brazilian counterpart. Most of the affected children are young girls with classic endocrine syndromes (virilizing and/or Cushing. Levels of urinary 17-ketosteroids and plasma dehydroepiandrosterone sulfate (DHEA-S, which are abnormal in approximately 90% of the cases, provide the pivotal clue to a diagnosis of ACT. Typical imaging findings of pediatric ACT consist of a large, well-defined suprarenal tumor containing calcifications with a thin capsule and central necrosis or hemorrhage. The pathologic classification of pediatric ACT is troublesome. Even an experienced pathologist can find it difficult to differentiate carcinoma from adenoma. Surgery is the single most important procedure in the successful treatment of ACT. The role of chemotherapy in the management of childhood ACT has not been established although occasional tumors are responsive to mitotane or cisplatin-containing regimens. Because of the heterogeneity and rarity of the disease, prognostic factors have been difficult to establish in pediatric ACT. Patients with incomplete tumor resection or with metastatic disease at diagnosis have a dismal prognosis. In patients with localized and completely resected tumors, the size of the tumor has predictive value. Patients with large tumors have a much higher relapse rate than those with small tumors.

  19. Tumores cartilaginosos da laringe

    Directory of Open Access Journals (Sweden)

    Thomé Robert

    2001-01-01

    Full Text Available Introdução: Os tumores cartilaginosos da laringe são raros, sendo a cartilagem cricóide a mais acometida. A raridade desses tumores tem como conseqüência experiência limitada e, portanto, o conhecimento a respeito destas neoplasias apresenta pontos obscuros. Objetivo: Apresentar os resultados cirúrgicos bem como o seguimento a longo prazo de 6 pacientes com tumores cartilaginosos da laringe, 4 condrossarcomas de baixa malignidade e 2 condromas. Forma de estudo: Retrospectivo não randomizado. Material e método: Cinco dos tumores acometiam a cartilagem cricóide e 1 a tireóide. Os pacientes foram submetidos a laringectomia parcial (4 e total (2, com seguimento de 6 a 30 anos (média-19,5 anos. Resultados: Nenhum dos pacientes apresentou metástase ou morte relacionada ao tumor. A sobrevida alcançada por 5 anos foi de 100% e a por 20 anos, 67%, sendo a comorbidade responsável por tal decréscimo. Conclusões: Nossos resultados reforçam o conceito de que a laringectomia conservadora seja a modalidade ideal de tratamento, reservando a ressecção total para tumores extensos ou casos de recorrência. Há indícios de que 5 anos pode não ser tempo suficiente para observar recorrência no condrossarcoma de baixo grau de malignidade.

  20. Giant Intradiverticular Bladder Tumor

    Science.gov (United States)

    Noh, Mohamad Syafeeq Faeez Md; Aziz, Ahmad Fuad Abdul; Ghani, Khairul Asri Mohd; Siang, Christopher Lee Kheng; Yunus, Rosna; Yusof, Mubarak Mohd

    2017-01-01

    Patient: Male, 74 Final Diagnosis: Giant intradiverticular bladder tumor with metastasis Symptoms: Hematuria Medication:— Clinical Procedure: — Specialty: Urology Objective: Rare disease Background: Intradiverticular bladder tumors are rare. This renders diagnosis of an intradiverticular bladder tumor difficult. Imaging plays a vital role in achieving the diagnosis, and subsequently staging of the disease. Case Report: A 74-year-old male presented to our center with a few months history of constitutional symptoms. Upon further history, he reported hematuria two months prior to presentation, which stopped temporarily, only to recur a few days prior to coming to the hospital. The patient admitted to having lower urinary tract symptoms. However, there was no dysuria, no sandy urine, and no fever. Palpation of his abdomen revealed a vague mass at the suprapubic region, which was non tender. In view of his history and the clinical examination findings, an ultrasound of the abdomen and computed tomography (CT) was arranged. These investigations revealed a giant tumor that seemed to be arising from a bladder diverticulum, with a mass effect and hydronephrosis. He later underwent operative intervention. Conclusions: Intradiverticular bladder tumors may present a challenge to the treating physician in an atypical presentation; thus requiring a high index of suspicion and knowledge of tumor pathophysiology. As illustrated in our case, CT with its wide availability and multiplanar imaging capabilities offers a useful means for diagnosis, disease staging, operative planning, and follow-up. PMID:28246375

  1. Wilms tumors: genotypes and phenotypes

    NARCIS (Netherlands)

    H. Segers (Heidi)

    2013-01-01

    textabstractWilms tumor, or nephroblastoma, represents about 90% of all pediatric renal tumors and about 7% of all pediatric malignancies. Most Wilms tumors are unilateral, although in 5-10 % of the patients both kidneys are infected. Wilms tumor typically occurs between the age of 2 and 4 years,

  2. [Enophthalmos in an orbital tumor].

    Science.gov (United States)

    Szabo, Bianca; Szabo, I; Nicula, Cristina; Popescu, Livia Adriana

    2013-01-01

    Enophtalmus is an unusual sign of the orbital tumors often represented by proptosis. One patient with enophtalmus and intraorbital tumor and aplasy is presented. The treatment of choice of orbital tumor is complete surgical excision and careful follow-up. Considering the more aggressive course followed by recurrent tumor, correct diagnosis and management is essential.

  3. Brain tumors in infants

    Directory of Open Access Journals (Sweden)

    Seyyed Mohammad Ghodsi

    2015-01-01

    Full Text Available Background: Brain tumors in infants have different clinical presentations, anatomical distribution, histopathological diagnosis, and clinical prognosis compared with older children. Materials and Methods: A retrospective analysis was done in patients <12 months old who were operated on for primary brain tumor in Children's Hospital Medical Center since 2008 to 2014. Results: Thirty-one infants, 20 males and 11 females, with the mean age of 7.13 months (0.5–12 were enrolled. There were 16 supratentorial and 15 infratentorial tumors. The presenting symptoms included increased head circumference (16; bulge fontanel (15; vomiting (15; developmental regression (11; sunset eye (7; seizure (4; loss of consciousness (4; irritability (3; nystagmus (2; visual loss (2; hemiparesis (2; torticollis (2; VI palsy (3; VII, IX, X nerve palsy (each 2; and ptosis (1. Gross total and subtotal resection were performed in 19 and 11 cases, respectively. Fourteen patients needed external ventricular drainage in the perioperative period, from whom four infants required a ventriculoperitoneal shunt. One patient underwent ventriculoperitoneal shunting without tumor resection. The most common histological diagnoses were primitive neuroectodermal tumor (7, followed by anaplastic ependymoma (6 and grade II ependymoma. The rate of 30-day mortality was 19.3%. Eighteen patients are now well-controlled with or without adjuvant therapy (overall survival; 58%, from whom 13 cases are tumor free (disease free survival; 41.9%, 3 cases have residual masses with fixed or decreased size (progression-free survival; 9.6%, and 2 cases are still on chemotherapy. Conclusion: Brain tumors in infants should be treated with surgical resection, followed by chemotherapy when necessary.

  4. Metastatic Brain Tumors

    Directory of Open Access Journals (Sweden)

    Ersin Haciyakupoglu

    2014-04-01

    Full Text Available Metastatic tumor is secondary spread to the central nervous system of primer systemic cancers originating from tissues other than the central nervous system. In adults; there are metastases respectively from lungs, breasts, malign melanoma, renal cell carcinoma, colon and thyroid cancers. 30-60% of lung cancers metastasis to the brain. In children there are quite a few cerebral metastases. Most commonly leukemia, lymphoma, osteogenic sarcoma, rhabdomyosarcoma and germ cell tumors metastasis to the brain. %50 of malign melanoma, lung, breast and colon cancers intend to make multipl metastases but renal cell cancers intend to make solitary metastasis.While lung cancers metastasis to brain in 6-9 months after the definitive diagnosis, renal cancers in 1 year, colon cancers in 2 years, breast cancers and malign melanoma in 3 years metastasis to brain. In 6% of cases there are cerebral metastasis while there isn’t a symptom of a primary tumor. For treatment corticosteroids, surgery, Radiotherapy(RT, Chemotherapy(CT and Stereotactic Radiosurgery(SRS can be implemented. Small cell lung cancers, lymphoma, germ cell tumors are sensitive to RT and CT. Non small cell lung cancers, renal, colon cancers and malign melanoma are radioresistant. The purposes in the surgery of the metastatic brain tumors are; total resection of tumors without neurologic deficits, decreasing the intracranial pressure and decreasing the dose of postoperative radiotherapy. Key Words: Metastatic brain tumors, Stereotactic radiosurgery, Malign melanoma, Lung cancers, Renal cell carcinoma, Radiotherapy, Chemotherapy [Cukurova Med J 2014; 39(2.000: 191-202

  5. Tumores neonatales bucomaxilofaciales Neonatal buccomaxillofacial tumors

    Directory of Open Access Journals (Sweden)

    Zoila del S. López Díaz

    2007-12-01

    Full Text Available Se realiza un estudio descriptivo, lineal y retrospectivo por un período de 10 años, de 11 recién nacidos (edad 0-30 días, quienes al nacer presentan en la región bucomaxilofacial un tumor que les ocasiona de manera determinante compromiso para la ventilación y/o alimentación, por lo que se hace necesario realizarles a todos de manera inmediata, tratamiento quirúrgico para preservarles la vida. Se analizaron las variables edad, sexo, color de la piel, diagnóstico, tumoraciones que se presentaron con mayor frecuencia, compromiso para la ventilación y/o alimentación, procederes y mortalidad. Los datos se recogieron en una planilla confeccionada al efecto, lo que permitió establecer resultados y confeccionar tablas. Se concluye que en nuestro estudio este tipo de tumoración afectó con mayor frecuencia al sexo femenino y a niños de piel blanca; y el tipo de tumoración observada con mayor frecuencia fueron las malformaciones vasculares de tipo linfático (linfangiomas gigantes o higromas quísticos, así como y los teratomas bucofaríngeos, con una mortalidad de alrededor del 27,3 % en estas edades neonatales.A descriptive, lineal and retrospective study of 11 newborn infants aged 0-30 days was conducted. They presented a tumor in the buccomaxillofacial region that compromised their ventilation and/or nutrition, which made necessary to immediately perform surgery to preserve their lives. The following variables were analyzed: age, sex, colour of the skin, diagnosis, the most common tumours, compromise for ventilation and/or nutrition, procedures and mortality. Data were collected in a form that allowed to establish results and to make tables. It was concluded that this type of tumor affected mostly females and white children. The most commonly observed tumors were vascular lymphatic malformations (giant lymphangiomas or cystic hygromas, as well as buccopharyngeal teratomas, with a mortality around 27.3 % at these neonatal ages.

  6. Wilms tumors: genotypes and phenotypes

    OpenAIRE

    Segers, Heidi

    2013-01-01

    textabstractWilms tumor, or nephroblastoma, represents about 90% of all pediatric renal tumors and about 7% of all pediatric malignancies. Most Wilms tumors are unilateral, although in 5-10 % of the patients both kidneys are infected. Wilms tumor typically occurs between the age of 2 and 4 years, and 90% of the patients are diagnosed before the age of 7 years. Above the age of 18 years, Wilms tumor is rare, representing less than 1% of all adult renal tumors. Most pediatric Wilms tumor patien...

  7. Pituitary gland tumors; Hypophysentumoren

    Energy Technology Data Exchange (ETDEWEB)

    Jesser, J.; Schlamp, K.; Bendszus, M. [Radiologische Klinik, Universitaetsklinikum Heidelberg, Abteilung fuer Neuroradiologie, Heidelberg (Germany)

    2014-10-15

    This article gives an overview of the most common tumors of the pituitary gland and the differential diagnostics with special emphasis on radiological diagnostic criteria. A selective search of the literature in PubMed was carried out. Pituitary adenomas constitute 10-15 % of all intracranial tumors and are the most common tumors of the sellar region. Tumors smaller than 1 cm in diameter are called microadenomas while those larger than 1 cm in diameter are called macroadenomas. Approximately 65 % of pituitary gland adenomas secrete hormones whereby approximately 50 % secrete prolactin, 10 % secrete growth hormone (somatotropin) and 6 % secrete corticotropin. Other tumors located in the sella turcica can also cause endocrinological symptoms, such as an oversecretion of pituitary hormone or pituitary insufficiency by impinging on the pituitary gland or its stalk. When tumors spread into the space cranial to the sella turcica, they can impinge on the optic chiasm and cause visual disorders. A common differential diagnosis of a sellar tumor is a craniopharyngeoma. In children up to 10 % of all intracranial tumors are craniopharyngeomas. Other differential diagnoses for sellar tumors are metastases, meningiomas, epidermoids and in rare cases astrocytomas, germinomas or Rathke cleft cysts As these tumors are located in an anatomically complex region of the skull base and are often very small, a highly focused imaging protocol is required. The currently favored modality is magnetic resonance imaging (MRI) with the administration of a contrast agent. The sellar region should be mapped in thin slices. In cases of suspected microadenoma the imaging protocol should also contain a sequence with dynamic contrast administration in order to assess the specific enhancement characteristics of the tumor and the pituitary gland. (orig.) [German] Diese Arbeit ist eine Uebersicht ueber die haeufigsten Hypophysentumoren und deren Differenzialdiagnosen mit Augenmerk auf die

  8. Thermoradiotherapy of malignant tumors

    Energy Technology Data Exchange (ETDEWEB)

    Hatano, Kazuo; Itami, Jun; Arimizu, Noboru (Chiba Univ. (Japan). School of Medicine); Uno, Takashi; Toita, Takafumi; Shiina, Takeki; Mikuriya, Shuuichi; Yamada, Tsunehisa

    1991-06-01

    From October 1986 to June 1989, 79 patients with malignant tumors were treated by radiation therapy combined with hyperthermia at National Medical Center Hospital. Seventy two patients (male: 48, female: 23) were evaluable. Average age was 62.4 years old (ranged 20-81 years old). Irradiation was delivered twice to fifth weekly in tumor doses of 50 to 60 Gy (TDF 82-122). We used 3 types of hyperthermic equipment, Thermotron RF8, BSD 1000 System and Endoradiotherm 100A. Hyperthermia was initiated within 30 minutes following irradiation, most of the patients being treated with adequate equipment, 41-44degC, for 60-70 minutes every 72 hours. Fifteen of 71 patients (20.8%) showed a complete response and 36 patients (50%) showed a partial response, so effective rate was 70.8% of all. In superficial tumors, 4 of 21 patients (21.5%) showed a complete response and 8 of 21 patients showed a partial response, effective rate was 63.2% of all. In deep seated tumor, 10 of 46 patients (21.7%) showed a complete response and 25 of all patients showed a partial response, so effective rate was 76.1% of all. Five patients were heated following only intra-tumor injection of OK-432 and its effective rate was 75%. We think that this modality of therapy will be effective in cases which heating area had been irradiated over tolerable doses. CR rate of superficial tumors according to intra-tumor center temperature tends to higher in the cases of higher tumor temperature. In deep seated tumor, 11 patients (23.9%) had reached over 43degC, 29 patients (63.0%) heated 41-43degC, 6 patients (13.0%) heated under 41degC and CR rate of each group were 36.4%, 17.2%, 16.7%, respectively. We think that thermotherapy with irradiation is an effective therapy in the treatment of malignancies but the improvement of heating equipment will be expected. (author).

  9. CNS Tumors in Neurofibromatosis.

    Science.gov (United States)

    Campian, Jian; Gutmann, David H

    2017-07-20

    Neurofibromatosis (NF) encompasses a group of distinct genetic disorders in which affected children and adults are prone to the development of benign and malignant tumors of the nervous system. The purpose of this review is to discuss the spectrum of CNS tumors arising in individuals with NF type 1 (NF1) and NF type 2 (NF2), their pathogenic etiologies, and the rational treatment options for people with these neoplasms. This article is a review of preclinical and clinical data focused on the treatment of the most common CNS tumors encountered in children and adults with NF1 and NF2. Although children with NF1 are at risk for developing low-grade gliomas of the optic pathway and brainstem, individuals with NF2 typically manifest low-grade tumors affecting the cranial nerves (vestibular schwannomas), meninges (meningiomas), and spinal cord (ependymomas). With the identification of the NF1 and NF2 genes, molecularly targeted therapies are beginning to emerge, as a result of a deeper understanding of the mechanisms underlying NF1 and NF2 protein function. As we enter into an era of precision oncology, a more comprehensive awareness of the factors that increase the risk of developing CNS cancers in affected individuals, coupled with a greater appreciation of the cellular and molecular determinants that maintain tumor growth, will undoubtedly yield more effective therapies for these cancer predisposition syndromes.

  10. Extraovarian granulosa cell tumor

    Directory of Open Access Journals (Sweden)

    Paul Prabir

    2009-04-01

    Full Text Available Extraovarian granulosa cell tumor (GCT is a very uncommon tumor, assumed to arise from the ectopic gonadal tissue along the embryonal route of the genital ridge. One such rare case of extraovarian GCT was encountered in a 58-year-old female who presented with a large intraabdominal lump. Computerized tomography revealed one large retroperitoneal mass measuring 15cm x 16cm and another mesenteric mass of 8cm x 5cm size. The patient had a history of hysterectomy with bilateral salpingooophorectomy 20 years ago for uterine leiomyoma. Ultrasonography-guided aspiration smears revealed cytological features suggestive of GCT. Histopathological examination of the excised masses showed features of adult-type GCT. Because metastatic epithelial tumors, particularly from the ovaries, may show identical morphology, immunostains for inhibin and epithelial membrane antigen (EMA were performed. The tumor showed positivity for inhibin while EMA was negative thus confirming the diagnosis of GCT. As this patient had no previous history of GCT and was oophorectomized 20 years ago, the tumor was considered as extraovarian. A diagnosis of extraovarian GCT should be carried out after excluding any previous history of GCT of the ovary. Immunostains help to differentiate GCTs from other neoplasms.

  11. Endolymphatic sac tumor : a rare cerebellopontine angle tumor.

    Directory of Open Access Journals (Sweden)

    Joseph B

    2002-10-01

    Full Text Available Endolymphatic sac tumors (ELST are rare papillary tumors of the temporal bone. Previously named as aggressive papillary middle ear tumors, they have recently been shown to arise from the endolymphatic sac. They are a rare in cerebello-pontine angle (CPA. We present a case of an ELST who presented as a CPA tumor with hydrocephalus. He underwent a ventriculo-peritoneal shunt initially. On exploration of the CP angle, the tumor was found to be extremely vascular. He was re-explored following embolization, and a subtotal excision of the tumor was done. Extensive petrous bone infiltration and vascularity of the tumor makes total excision almost impossible with high risk of cranial nerve deficits, excessive blood loss and CSF leak. This tumor should be considered in the differential diagnosis of vascular CPA tumors which erode the petrous temporal bone. The relevant literature is reviewed.

  12. Aggressive fibromatosis of the neck in a patient with Gardner's syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Rai, A.T.; Nguyen, T.P.; Hogg, J.P.; Gabriele, F.J. [Dept. of Radiology, West Virginia Univ. Hospitals, Morgantown, WV (United States)

    2001-08-01

    We report a patient with Gardner's syndrome who, in addition to a total colectomy, had multiple excisions of desmoid tumors in both thighs. He presented with left-sided neck swelling and pain. MRI was highly suggestive of desmoid tumors in multiple neck muscles. To our knowledge this is the first description of diffuse fibromatosis of the neck in association with Gardner's syndrome. (orig.)

  13. Upper urinary tract tumors

    DEFF Research Database (Denmark)

    Gandrup, Karen L; Nordling, Jørgen; Balslev, Ingegerd

    2014-01-01

    BACKGROUND: Computed tomography urography (CTU) is used widely in the work-up of patients with symptoms of urinary tract lesions. Preoperative knowledge of whether a tumor is invasive or non-invasive is important for the choice of surgery. So far there are no studies about the distinction...... of invasive and non-invasive tumors in ureter and renal pelvis based on the enhancement measured with Hounsfield Units. PURPOSE: To examine the value of CTU using split-bolus technique to distinguish non-invasive from invasive urothelial carcinomas in the upper urinary tract. MATERIAL AND METHODS: Patients...... obtained at CTU could distinguish between invasive and non-invasive lesions. No patients had a CTU within the last year before the examination that resulted in surgery. CONCLUSION: A split-bolus CTU cannot distinguish between invasive and non-invasive urothelial tumors in the upper urinary tract...

  14. Benign notochordal cell tumors.

    Science.gov (United States)

    Martínez Gamarra, C; Bernabéu Taboada, D; Pozo Kreilinger, J J; Tapia Viñé, M

    2017-08-01

    Benign notochordal cell tumors (TBCN) are lesions with notochordal differentiation which affect the axial skeleton. They are characterized by asymptomatic or non-specific symptomatology and are radiologically unnoticed because of their small size, or because they are mistaken with other benign bone lesions, such as vertebral hemangiomas. When they are large, or symptomatic, can be differential diagnosis with metastases, primary bone tumors and chordomas. We present a case of a TBCN in a 50-year-old woman, with a sacral lesion seen in MRI. A CT-guided biopsy was scheduled to analyze the lesion, finding that the tumor was not clearly recognizable on CT, so the anatomical references of MRI were used to select the appropriate plane. The planning of the approach and the radio-pathological correlation were determinant to reach the definitive diagnosis. Copyright © 2017 SERAM. Publicado por Elsevier España, S.L.U. All rights reserved.

  15. Renal Tumor Biopsy Technique

    Institute of Scientific and Technical Information of China (English)

    Lei Zhang; Xue-Song Li; Li-Qun Zhou

    2016-01-01

    Objective:To review hot issues and future direction of renal tumor biopsy (RTB) technique.Data Sources:The literature concerning or including RTB technique in English was collected from PubMed published from 1990 to 2015.Study Selection:We included all the relevant articles on RTB technique in English,with no limitation of study design.Results:Computed tomography and ultrasound were usually used for guiding RTB with respective advantages.Core biopsy is more preferred over fine needle aspiration because of superior accuracy.A minimum of two good-quality cores for a single renal tumor is generally accepted.The use of coaxial guide is recommended.For biopsy location,sampling different regions including central and peripheral biopsies are recommended.Conclusion:In spite of some limitations,RTB technique is relatively mature to help optimize the treatment of renal tumors.

  16. Aggressive malignant phyllodes tumor

    Directory of Open Access Journals (Sweden)

    Nathan Roberts

    2015-01-01

    Conclusion: Despite biopsy proven malignant phyllodes tumor, it was near impossible to predict such a rapid course of disease progression in our patient. Our case illustrates the unpredictable nature of this disease in general and it possibly sheds light on a variant of the disease which had undergone an aggressive transformation.

  17. Ghost Cell Tumors.

    Science.gov (United States)

    Sheikh, Jason; Cohen, Molly D; Ramer, Naomi; Payami, Ali

    2017-04-01

    Ghost cell tumors are a family of lesions that range in presentation from cyst to solid neoplasm and in behavior from benign to locally aggressive or metastatic. All are characterized by the presence of ameloblastic epithelium, ghost cells, and calcifications. This report presents the cases of a 14-year-old girl with a calcifying cystic odontogenic tumor (CCOT) and a 65-year-old woman with a peripheral dentinogenic ghost cell tumor (DGCT) with dysplastic changes, a rare locally invasive tumor of odontogenic epithelium. The first patient presented with a 1-year history of slowly progressing pain and swelling at the left body of the mandible. Initial panoramic radiograph displayed a mixed radiolucent and radiopaque lesion. An incisional biopsy yielded a diagnosis of CCOT. Decompression of the mass was completed; after 3 months, it was enucleated and immediately grafted with bone harvested from the anterior iliac crest. The second patient presented with a 3-month history of slowly progressing pain and swelling at the left body of the mandible. Initial panoramic radiograph depicted a mixed radiolucent and radiopaque lesion with saucerization of the buccal mandibular cortex. An incisional biopsy examination suggested a diagnosis of DGCT because of the presence of ghost cells, dentinoid, and islands of ameloblastic epithelium. Excision of the mass with peripheral ostectomy was completed. At 6 and 12 months of follow-up, no evidence of recurrence was noted.

  18. Targeting thapsigargin towards tumors

    DEFF Research Database (Denmark)

    Doan, Nhu Thi Quynh; Paulsen, Eleonora Sandholdt; Sehgal, Pankaj

    2015-01-01

    substrates for either prostate specific antigen (PSA) or prostate specific membrane antigen (PSMA) prodrugs were created, which selectively affect prostate cancer cells or neovascular tissue in tumors. One of the prodrug is currently tested in clinical phase II. The prodrug under clinical trial has been...

  19. Serodiagnosis for Tumor Viruses

    Science.gov (United States)

    Morrison, Brian J.; Labo, Nazzarena; Miley, Wendell J.; Whitby, Denise

    2015-01-01

    The known human tumor viruses include the DNA viruses Epstein-Barr virus, Kaposi sarcoma herpesvirus, Merkel cell polyomavirus, human papillomavirus, and hepatitis B virus. RNA tumor viruses include Human T-cell lymphotrophic virus type-1 and hepatitis C virus. The serological identification of antigens/antibodies in plasma serum is a rapidly progressing field with utility for both scientists and clinicians. Serology is useful for conducting seroepidemiology studies and to inform on the pathogenesis and host immune response to a particular viral agent. Clinically, serology is useful for diagnosing current or past infection and for aiding in clinical management decisions. Serology is useful for screening blood donations for infectious agents and for monitoring the outcome of vaccination against these viruses. Serodiagnosis of human tumor viruses has improved in recent years with increased specificity and sensitivity of the assays, as well as reductions in cost and the ability to assess multiple antibody/antigens in single assays. Serodiagnosis of tumor viruses plays an important role in our understanding of the prevalence and transmission of these viruses and ultimately in the ability to develop treatments/preventions for these globally important diseases. PMID:25843726

  20. Radioembolization of liver tumors

    NARCIS (Netherlands)

    van den Hoven, AF

    2016-01-01

    Radioembolization is a therapy during which radioactive microspheres are administered through a microcatheter placed in the hepatic arterial vasculature in order to irradiate liver tumors from within. In the past decennium, this treatment has evolved as a safe and effective treatment option for pati

  1. Tumor ablations in IMRI

    Institute of Scientific and Technical Information of China (English)

    Roberto Blanco Sequeiros

    2002-01-01

    @@ IntroductionMagnetic resonance imaging based guidance control and monitoring of minimally invasive intervention has developed from a hypothetical concept to a practical possibility. Magnetic-resonance-guided interstitial therapy in principle is defined as a treatment technique for ablating deepseated tumors in the human body.

  2. Intracardiac tumor causing

    Directory of Open Access Journals (Sweden)

    Christopher P. Jordan

    2016-07-01

    Full Text Available The following report describes the case of newborn girl with an asymptomatic systolic murmur, which on imaging revealed a nearly obstructive mass in the left-ventricular outflow tract. The mass was resected and found to be consistent with a rhabdomyoma. Here, we describe the pathologic and clinical characteristics of this tumor.

  3. [2 infrequent vulvar tumors].

    Science.gov (United States)

    Aguilar, G; Benavides, A; Villanueva, M E

    1994-01-01

    Two cases of malignant vulvar tumors are presented: a malignant Melanoma and a case of Carcinoma of the Bartholin Gland. Both were diagnosticated during 1993 in the Obstetrics and Gynecology Department of Dr. Félix Bulnes Cerda Hospital. Clinical and anatomophatological aspects are studied.

  4. NO, Immunosuppression and Tumor Immmunotherapy

    OpenAIRE

    Hegardt, Pontus

    2002-01-01

    This study aims at clarifying the role of NO in the immunosuppression induced by in vivo tumor growth and by tumor immunotherapy, and determining whether the inhibition of NO production can be used as an adjuvant in tumor immunotherapy. We have shown previously that tumor cells, glioma (N32) and colon carcinoma (H1D2), when genetically engineered to express such immune stimulatory cytokines as IFN-g and IL-18, induce strong anti-tumor immune response, in immunized tumor-free rats, whereas onl...

  5. Rare and Challenging Tumor Entity: Phyllodes Tumor of the Prostate

    Directory of Open Access Journals (Sweden)

    Andreas Bannowsky

    2009-01-01

    Full Text Available Cystic epithelial-stromal tumors of the prostate are rare, with 82 cases reported in literature. These cases have been published under a variety of diagnoses, including phyllodes tumor and prostatic stromal proliferation of uncertain malignant potential as well as a malignant tumor called “prostatic stromal sarcoma”. We report a case of a 60-year-old man with the histological diagnosis of phyllodes tumor of the prostate in transurethral resection specimen.

  6. Rare and Challenging Tumor Entity: Phyllodes Tumor of the Prostate

    OpenAIRE

    Andreas Bannowsky; Andreas Probst; Helmut Dunker; Tillmann Loch

    2009-01-01

    Cystic epithelial-stromal tumors of the prostate are rare, with 82 cases reported in literature. These cases have been published under a variety of diagnoses, including phyllodes tumor and prostatic stromal proliferation of uncertain malignant potential as well as a malignant tumor called “prostatic stromal sarcoma”. We report a case of a 60-year-old man with the histological diagnosis of phyllodes tumor of the prostate in transurethral resection specimen.

  7. Advances of Tumor Hyperthermia and Tumor Immunology in Translational Medicine

    OpenAIRE

    Hooshang Lahooti

    2015-01-01

    Hyperthermia is another important method in the treatment of tumors, secondary to surgery, radiotherapy, chemotherapy and biotherapy. It has been demonstrated the efficacy and versatility of hyperthermia in a lot of randomized trials across various primary cancers. Both heat shock proteins (HSPs) and dendritic cells (DCs) are greatly affected by hyperthermia and closely related to the tumor immunology. Nowadays, tumor hyperthermia and tumor immunology have been attached much attention in the ...

  8. Epidemiological features of brain tumors

    Directory of Open Access Journals (Sweden)

    Živković Nenad

    2013-01-01

    Full Text Available Brain tumors account for 1.4% of all cancers and 2.4% of all cancer-related deaths. The incidence of brain tumors varies and it is higher in developed countries of Western Europe, North America, Australia and New Zealand. In Serbia, according to data from 2009, malignant brain tumors account for 2. 2 of all tumors, and from all cancer­related deaths, 3.2% is caused by malignant brain tumors. According to recent statistical reports, an overall incidence of brain tumors for benign and malignant tumors combined is 18.71 per 100,000 persons/year. The most common benign brain tumor in adults is meningioma, which is most present in women, and the most common malignant tumor is glioblastoma, which is most present in adult men. Due to high mortality, especially in patients diagnosed with glioblastoma and significant brain tumor morbidity, there is a constant interest in understanding its etiology in order to possibly prevent tumor occurrence in future and enable more efficient treatment strategies for this fatal brain disease. Despite the continuously growing number of epidemiological studies on possible factors of tumor incidence, the etiology remains unclear. The only established environmental risk factor of gliomas is ionizing radiation exposure. Exposure to radiofrequency electromagnetic fields via cell phone use has gained a lot of attention as a potential risk factor of brain tumor development. However, studies have been inconsistent and inconclusive, so more definite results are still expected.

  9. Neuroendocrine tumors of the pancreas.

    LENUS (Irish Health Repository)

    Davies, Karen

    2009-04-01

    Pancreatic endocrine tumors are rare neoplasms accounting for less than 5% of pancreatic malignancies. They are broadly classified into either functioning tumors (insulinomas, gastrinomas, glucagonomas, VIPomas, and somatostatinomas) or nonfunctioning tumors. The diagnosis of these tumors is difficult and requires a careful history and examination combined with laboratory tests and radiologic imaging. Signs and symptoms are usually related to hormone hypersecretion in the case of functioning tumors and to tumor size or metastases with nonfunctioning tumors. Surgical resection remains the treatment of choice even in the face of metastatic disease. Further development of novel diagnostic and treatment modalities offers potential to greatly improve quality of life and prolong disease-free survival for patients with pancreatic endocrine tumors.

  10. Beta-2 Microglobulin Tumor Marker

    Science.gov (United States)

    ... services. Advertising & Sponsorship: Policy | Opportunities Beta-2 Microglobulin Tumor Marker Share this page: Was this page helpful? Also ... Microglobulin, Serum, Urine, or CSF Related tests: Albumin , Tumor Markers , CSF Analysis All content on Lab Tests Online ...

  11. Neuroendocrine tumors of the pancreas.

    LENUS (Irish Health Repository)

    Davies, Karen

    2012-02-01

    Pancreatic endocrine tumors are rare neoplasms accounting for less than 5% of pancreatic malignancies. They are broadly classified into either functioning tumors (insulinomas, gastrinomas, glucagonomas, VIPomas, and somatostatinomas) or nonfunctioning tumors. The diagnosis of these tumors is difficult and requires a careful history and examination combined with laboratory tests and radiologic imaging. Signs and symptoms are usually related to hormone hypersecretion in the case of functioning tumors and to tumor size or metastases with nonfunctioning tumors. Surgical resection remains the treatment of choice even in the face of metastatic disease. Further development of novel diagnostic and treatment modalities offers potential to greatly improve quality of life and prolong disease-free survival for patients with pancreatic endocrine tumors.

  12. Primary tumors of the patella

    National Research Council Canada - National Science Library

    Song, Mingzhi; Zhang, Zhen; Wu, Yuxuan; Ma, Kai; Lu, Ming

    2015-01-01

    The patella is an uncommon location for cancerous occurrence and development. The majority of tumors of the patella are benign, with a significant incidence of giant cell tumors and chondroblastoma...

  13. Biopsy in Musculoskeletal Tumors

    Directory of Open Access Journals (Sweden)

    Mohammad Gharehdaghi

    2014-09-01

    Full Text Available Diagnosis of bone tumors is based on careful evaluation of clinical, imaging and a pathologic findings. So the biopsy of bone and soft tissue sarcomas is the final step in evaluation and a fundamental step in the diagnosis of the lesion. It should not be performed as a shortcut to diagnosis (1. The biopsy should be performed in order to confirm the diagnosis and differentiate among few diagnoses after careful staged studies. Real and artificial changes in imaging studies will be superimposed after performing biopsy, which may alter the interpretation if done after biopsy is taken (1. The correct management of a sarcoma depends on the accurate diagnosis. Inadequate, inapprppriate, or inaccurate non-representative biopsy leads to poorer outcome in terms of survivorship and limb salvage. An incorrect, unplanned incision and biopsy may unnecessarily contaminate uninvolved compartments which may convert a salvageable limb to amputation. Anatomic approach along with the proper biopsy techniques may lead to success or catastrophe. It is clear that in patients with inappropriate biopsy, the chance of the need to change the treatment to more radical than would originally be expected is significantly higher. Also it is more probable to need to  convert curative to palliative treatment and to require adjuvant radiotherapy in patients with inappropriate biopsies. Patients with sarcoma are best served by early referral to a specialized center where staged investigations and biopsy can be performed with minimal morbidity (3. Open biopsy is still considered the gold standard; however, recent studies suggest comparable results with percutaneous core needle biopsy. Our study on 103 consecutive CNB and open biopsy showed comparable results as well. Surgeons need to answer to two questions prior to performing a biopsy: 1-          Where is the best part of the lesion to be biopsied? 2-          What is the safest route without contaminating

  14. Tumor suppressor and hepatocellular carcinoma

    Institute of Scientific and Technical Information of China (English)

    Juliette Martin; Jean-Frangois Dufour

    2008-01-01

    A few signaling pathways are driving the growth of hepatocellular carcinoma. Each of these pathways possesses negative regulators. These enzymes, which normally suppress unchecked cell proliferation, are circumvented in the oncogenic process, either the over-activity of oncogenes is sufficient to annihilate the activity of tumor suppressors or tumor suppressors have been rendered ineffective. The loss of several key tumor suppressors has been described in hepatocellular carcinoma. Here, we systematically review the evidence implicating tumor suppressors in the development of hepatocellular carcinoma.

  15. Radiological review of pleural tumors

    Directory of Open Access Journals (Sweden)

    Binit Sureka

    2013-01-01

    Full Text Available Tumors of the pleura are not uncommon and diagnosis is clinched by combined imaging and clinical correlation. Malignant tumors are more common than benign tumors. Initial imaging modalities are chest radiography and Computed Tomography (CT. Further characterization may be required using Ultrasoundgraphy (USG, Magnetic resonance Imaging (MRI and PET-CT. Biopsy remains gold standard. This article highlights various common and uncommon tumors of pleura and characteristic imaging findings.

  16. Bednar Tumor: An Uncommon Entity

    OpenAIRE

    Gayathri P Amonkar; Asha Rupani; Ajay Shah; Ramesh Deshpande

    2016-01-01

    Bednar tumor is an uncommon variant of dermatofibrosarcoma protuberans. Also known as pigmented dermatofibrosarcoma protuberans, this tumor is of intermediate grade. It is seen in adults and has a predisposition to affect the shoulder region. We report a rare case of Bednar tumor in a 40-year-old female patient. The diagnosis of Bednar tumor must be considered while reporting pigmented subcutaneous spindle cell lesions.

  17. Phyllodes tumor showing intraductal growth.

    Science.gov (United States)

    Makidono, Akari; Tsunoda, Hiroko; Mori, Miki; Yagata, Hiroshi; Onoda, Yui; Kikuchi, Mari; Nozaki, Taiki; Saida, Yukihisa; Nakamura, Seigo; Suzuki, Koyu

    2013-07-01

    Phyllodes tumor of the breast is a rare fibroepithelial lesion and particularly uncommon in adolescent girls. It is thought to arise from the periductal rather than intralobular stroma. Usually, it is seen as a well-defined mass. Phyllodes tumor showing intraductal growth is extremely rare. Here we report a girl who has a phyllodes tumor with intraductal growth.

  18. Apoptosis in irradiated murine tumors.

    Science.gov (United States)

    Stephens, L C; Ang, K K; Schultheiss, T E; Milas, L; Meyn, R E

    1991-09-01

    Early radiation responses of transplantable murine ovarian (OCaI) and hepatocellular (HCaI) carcinomas were examined at 6, 24, 48, 96, and 144 h after single photon doses of 25, 35, or 45 Gy. Previous studies using tumor growth delay and tumor radiocurability assays had shown OCaI tumors to be relatively radiosensitive and HCaI tumors to be radioresistant. At 6 h, approximately 20% of nuclei in OCaI tumors showed aberrations characteristic of cell death by apoptosis. This contrasted to an incidence of 3% in HCaI tumors. Mitotic activity was eliminated in OCaI tumors but was only transiently suppressed in HCaI tumors. At 24-96 h, OCaI tumors continued to display apoptosis and progressive necrosis, whereas HCaI tumors responded by exhibiting marked pleomorphism. Factors other than mitotic activity may influence tumor radiosensitivity, and one of these may be susceptibility to induction of apoptosis (programmed cell death), because this was a prominent early radiation response by the radiosensitive OCaI tumors.

  19. Tumor Microenvironment in the Brain

    Energy Technology Data Exchange (ETDEWEB)

    Lorger, Mihaela [Leeds Institute of Molecular Medicine, University of Leeds, St. James’s University Hospital, Beckett Street, Leeds, LS9 7TF (United Kingdom)

    2012-02-22

    In addition to malignant cancer cells, tumors contain a variety of different stromal cells that constitute the tumor microenvironment. Some of these cell types provide crucial support for tumor growth, while others have been suggested to actually inhibit tumor progression. The composition of tumor microenvironment varies depending on the tumor site. The brain in particular consists of numerous specialized cell types such as microglia, astrocytes, and brain endothelial cells. In addition to these brain-resident cells, primary and metastatic brain tumors have also been shown to be infiltrated by different populations of bone marrow-derived cells. The role of different cell types that constitute tumor microenvironment in the progression of brain malignancies is only poorly understood. Tumor microenvironment has been shown to be a promising therapeutic target and diagnostic marker in extracranial malignancies. A better understanding of tumor microenvironment in the brain would therefore be expected to contribute to the development of improved therapies for brain tumors that are urgently required due to a poor availability of treatments for these malignancies. This review summarizes some of the known interactions between brain tumors and different stromal cells, and also discusses potential therapeutic approaches within this context.

  20. Sertoli-Leydig cell tumor

    Science.gov (United States)

    Sertoli-stromal cell tumor; Arrhenoblastoma; Androblastoma; Ovarian cancer - Sertoli-Leydig cell tumor ... The exact cause of this tumor is not known. Changes (mutations) in genes may play a role. SLCT occur most often in young women 20 to 30 ...

  1. Notch Signaling and Brain Tumors

    DEFF Research Database (Denmark)

    Stockhausen, Marie; Kristoffersen, Karina; Poulsen, Hans Skovgaard

    2011-01-01

    Human brain tumors are a heterogenous group of neoplasms occurring inside the cranium and the central spinal cord. In adults and children, astrocytic glioma and medulloblastoma are the most common subtypes of primary brain tumors. These tumor types are thought to arise from cells in which Notch...

  2. Tumor uptake of radioruthenium compounds

    Energy Technology Data Exchange (ETDEWEB)

    Srivastava, S C; Richards, P; Meinken, G E; Larson, S M; Grunbaum, Z

    1980-01-01

    The use of ruthenium-97 as a scintigraphic agent, particularly for tumor localization, is investigated. The tumor uptake of ruthenium chloride and ruthenium-labelled transferrin is evaluated and their application as tumor-imagine agents is compared to gallium-67 citrate. (ACR)

  3. Notch Signaling and Brain Tumors

    DEFF Research Database (Denmark)

    Stockhausen, Marie; Kristoffersen, Karina; Poulsen, Hans Skovgaard

    2011-01-01

    Human brain tumors are a heterogenous group of neoplasms occurring inside the cranium and the central spinal cord. In adults and children, astrocytic glioma and medulloblastoma are the most common subtypes of primary brain tumors. These tumor types are thought to arise from cells in which Notch...

  4. Metastasis and circulating tumor cells

    NARCIS (Netherlands)

    Dalum, van G.; Holland, L.; Terstappen, L.W.M.M.

    2012-01-01

    Cancer is a prominent cause of death worldwide. In most cases, it is not the primary tumor which causes death, but the metastases. Metastatic tumors are spread over the entire human body and are more difficult to remove or treat than the primary tumor. In a patient with metastatic disease, circulati

  5. [Retroperitoneal Tumor: Neurofibroma.

    Science.gov (United States)

    Lada, Paul Eduardo; Marriot, Daniela; Sanchez Tasonne, Carlos; Sanchez, Martin; Caballero, Fabian; Massa, Martin

    2016-01-01

    The neurofibroma is a benign tumor that grows from the sheath of the peripheral nerves, which is often localized on superficial tissues, especially in isolated forms. The neurofibromas can be of two types, localized or diffuse, the last one closely related to Von Recklinghausen disease or NF-1. We described a 37 years old male e patient, 37 years without symptoms, and the computed tomography scan (CT), and magnetic resonance imaging showed a tumor in proximity to the right kidney, the psoas muscle, the spine and compressing the cava vein, but cannot be accurately determinate the invasion of these structures. We review the differential diagnosis, therapeutic and the management of this disease in this clinical case.

  6. [Perihilar cholangiocarcinoma (Klatskin tumor)].

    Science.gov (United States)

    Stavrou, G A; Donati, M; Faiss, S; Jenner, R M; Niehaus, K J; Oldhafer, K J

    2014-02-01

    Perihilar cholangiocarcinoma or Klatskin tumors are a rare entity arising from the extrahepatic bile duct bifurcation. Considering the close anatomical relationship of the bile duct bifurcation with the portal vein bifurcation and hepatic arteries, surgical treatment is demanding. With an incidence of only 2-4 cases/100,000 population/year patients should be referred to a specialized center. The tumors are usually poorly differentiated adenocarcinomas growing diffusely along the duct and also the perineural sheath. Only radical surgery offers a curative option and currently surgical strategy usually consists of en bloc resection of the bile duct, extended liver resection and portal vein resection. Proximal and lateral safety margin R0 resections are technically very demanding procedures because of the local anatomy.

  7. Retroperitoneal inflammatory myofibroblastic tumor

    Directory of Open Access Journals (Sweden)

    Bapsy Poonamalle P

    2005-10-01

    Full Text Available Abstract Background Inflammatory myofibroblastic tumor (IMT is a neoplasm of unknown etiology occurring at various sites. By definition, it is composed of spindle cells (myofibroblasts with variable inflammatory component, hence the name is IMT. Case presentation The present case is of a 46 years old woman presented with a history of flank pain, abdominal mass and intermittent hematuria for last 6 months. The initial diagnosis was kept as renal cell carcinoma. Finally, it turned out to be a case of retroperitoneal IMT. The patient was managed by complete surgical resection of the tumor. Conclusion IMT is a rare neoplasm of uncertain biological potential. Complete surgical resection remains the mainstay of the treatment.

  8. Inflammatory myofibroblastic tumor

    Directory of Open Access Journals (Sweden)

    Sangeeta Palaskar

    2011-01-01

    Full Text Available Inflammatory myofibroblastic tumor is an uncommon lesion of unknown cause. It encompasses a spectrum of myofibroblastic proliferation along with varying amount of inflammatory infiltrate. A number of terms have been applied to the lesion, namely, inflammatory pseudotumor, fibrous xanthoma, plasma cell granuloma, pseudosarcoma, lymphoid hamartoma, myxoid hamartoma, inflammatory myofibrohistiocytic proliferation, benign myofibroblatoma, and most recently, inflammatory myofibroblastic tumor. The diverse nomenclature is mostly descriptive and reflects the uncertainty regarding true biologic nature of these lesions. Recently, the concept of this lesion being reactive has been challenged based on the clinical demonstration of recurrences and metastasis and cytogenetic evidence of acquired clonal chromosomal abnormalities. We hereby report a case of inflammatory pseudotumor and review its inflammatory versus neoplastic behavior.

  9. Pediatric Suprasellar Tumors.

    Science.gov (United States)

    McCrea, Heather J; George, Emilie; Settler, Allison; Schwartz, Theodore H; Greenfield, Jeffrey P

    2016-10-01

    The various childhood suprasellar tumors, while pathologically distinct, present similar clinical and surgical challenges as a result of their common anatomic location. These lesions are in close proximity to or may invade the optic nerve and chiasm, pituitary gland and infundibulum, hypothalamus, and third ventricle, leading to presenting features including visual field loss, impairment in visual acuity, endocrine dysfunction, and hydrocephalus. Though many suprasellar lesions are relatively benign in pathology, treatment may be complicated by high surgical morbidity resulting from damage to the hypothalamic-pituitary axis. Here we review the most frequent pediatric lesions occurring in the suprasellar region: craniopharyngioma, chiasmatic glioma, germ cell tumor, Rathke cleft and arachnoid cysts, pituitary adenoma, and histiocytosis. This review outlines both common presenting features and differentiating aspects of these lesions. It also includes classic radiographic presentations and treatment considerations for each lesion.

  10. Tumor-Induced Hyperlipidemia Contributes to Tumor Growth

    Directory of Open Access Journals (Sweden)

    Jianfeng Huang

    2016-04-01

    Full Text Available The known link between obesity and cancer suggests an important interaction between the host lipid metabolism and tumorigenesis. Here, we used a syngeneic tumor graft model to demonstrate that tumor development influences the host lipid metabolism. BCR-Abl-transformed precursor B cell tumors induced hyperlipidemia by stimulating very low-density lipoprotein (VLDL production and blunting VLDL and low-density lipoprotein (LDL turnover. To assess whether tumor progression was dependent on tumor-induced hyperlipidemia, we utilized the VLDL production-deficient mouse model, carboxylesterase3/triacylglycerol hydrolase (Ces3/TGH knockout mice. In Ces3/Tgh−/− tumor-bearing mice, plasma triglyceride and cholesterol levels were attenuated. Importantly tumor weight was reduced in Ces3/Tgh−/− mice. Mechanistically, reduced tumor growth in Ces3/Tgh−/− mice was attributed to reversal of tumor-induced PCSK9-mediated degradation of hepatic LDLR and decrease of LDL turnover. Our data demonstrate that tumor-induced hyperlipidemia encompasses a feed-forward loop that reprograms hepatic lipoprotein homeostasis in part by providing LDL cholesterol to support tumor growth.

  11. Neurons and tumor suppressors.

    Science.gov (United States)

    Zochodne, Douglas W

    2014-08-20

    Neurons choose growth pathways with half hearted reluctance, behavior that may be appropriate to maintain fixed long lasting connections but not to regenerate them. We now recognize that intrinsic brakes on regrowth are widely expressed in these hesitant neurons and include classical tumor suppressor molecules. Here, we review how two brakes, PTEN (phosphatase and tensin homolog deleted on chromosome 10) and retinoblastoma emerge as new and exciting knockdown targets to enhance neuron plasticity and improve outcome from damage or disease.

  12. Neurons and Tumor Suppressors

    OpenAIRE

    Douglas W Zochodne

    2014-01-01

    Neurons choose growth pathways with half hearted reluctance, behavior that may be appropriate to maintain fixed long lasting connections but not to regenerate them. We now recognize that intrinsic brakes on regrowth are widely expressed in these hesitant neurons and include classical tumor suppressor molecules. Here, we review how two brakes, PTEN (phosphatase and tensin homolog deleted on chromosome 10) and retinoblastoma emerge as new and exciting knockdown targets to e...

  13. TRUS Findings of Prostate Tumor or Tumor Like Lesions

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Hak Jong; Jang, Jung Min; Kim, Seung Hyup [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    2006-03-15

    Tumors or tumor-like lesions in the prostate raise questions concerning their histogenesis and they may have prognoses dissimilar to those of prostatic adenocarcinoma. Several neoplasms involving the prostate have been described and characterized in recent years. In addition to adenocarcinoma, they include mucinous cyst adenocarcinoma, neuroendocrine cancer, lymphoma, spindle cell neoplasm, squamous cell carcinoma, transitional cell carcinoma, and benign prostatic hyperplasia (BPH) mimicking malignancy. In addition, infectious conditions such as tuberculosis and some stages of prostatic abscess can also mimic prostate tumors. Radiologic findings overlap and have limited roles in the diagnoses of these entities. However, knowledge of these variable tumors and tumor-like conditions is helpful when making accurate radiologic diagnoses, which have important clinical implications for treatment and prognosis. Transrectal ultrasound (TRUS) and available pathologic images of unusual tumors and tumor- like lesions are demonstrated in this article

  14. Retroperitoneal calcifying fibrous tumor mimicking an adrenal tumor.

    Science.gov (United States)

    Prochaska, Erica C; Sciallis, Andrew P; Miller, Barbra S

    2016-06-01

    Establishing the etiology of a retroperitoneal tumor may be difficult due to close proximity of multiple organs. Evaluation of retroperitoneal tumors often leads to surgery, many times to obtain a definitive diagnosis and rule out malignancy. Calcifying fibrous tumors (CFT) are very rare soft tissue tumors occurring most often in young patients. They are most often found arising in the thoracic cavity, mediastinum, abdominal cavity and extremities and usually have a benign clinical course. Macrocscopically, the tumors are well circumscribed and firm with a white-tan appearance. Histologically, CFT comprised a hypocellular proliferation of bland spindle cells, densely hyalinized collagen, chronic lymphoplasmacytic inflammation and dystrophic calcifications. Other considerations in the pathologic differential diagnosis include solitary fibrous tumor and inflammatory myofibroblastic tumor.

  15. Characterization of the Tumor Secretome from Tumor Interstitial Fluid (TIF).

    Science.gov (United States)

    Gromov, Pavel; Gromova, Irina

    2016-01-01

    Tumor interstitial fluid (TIF) surrounds and perfuses bodily tumorigenic tissues and cells, and can accumulate by-products of tumors and stromal cells in a relatively local space. Interstitial fluid offers several important advantages for biomarker and therapeutic target discovery, especially for cancer. Here, we describe the most currently accepted method for recovering TIF from tumor and nonmalignant tissues that was initially performed using breast cancer tissue. TIF recovery is achieved by passive extraction of fluid from small, surgically dissected tissue specimens in phosphate-buffered saline. We also present protocols for hematoxylin and eosin (H&E) staining of snap-frozen and formalin-fixed, paraffin-embedded (FFPE) tumor sections and for proteomic profiling of TIF and matched tumor samples by high-resolution two-dimensional gel electrophoresis (2D-PAGE) to enable comparative analysis of tumor secretome and paired tumor tissue.

  16. Signal transduction pathway analysis in fibromatosis: receptor and nonreceptor tyrosine kinases☆

    Science.gov (United States)

    Cates, Justin M. M.; Black, Jennifer O.; Itani, Doha M.; Fasig, John H.; Keedy, Vicki L.; Hande, Kenneth R.; Whited, Brent W.; Homlar, Kelly C.; Halpern, Jennifer L.; Holt, Ginger E.; Schwartz, Herbert S.; Coffin, Cheryl M.

    2014-01-01

    Summary Despite reports of receptor tyrosine kinase activation in desmoid-type fibromatosis, therapeutic benefits of kinase inhibitor therapy are unpredictable. Variability in signal transduction or cellular kinases heretofore unevaluated in desmoid tumors may be responsible for these inconsistent responses. In either case, a better understanding of growth regulatory signaling pathways is necessary to assess the theoretical potential of inhibitor therapy. Immunohistochemical analysis of tyrosine kinases and activated isoforms of downstream signal transduction proteins was performed on a tissue microarray containing 27 cases of desmoid-type fibromatosis and 14 samples of scar; 6 whole sections of normal fibrous tissue were studied for comparison. Platelet-derived growth factor receptor, β type, and focal adhesion kinase 1 were expressed in all desmoid tumors and healing scars but only 80% and 50% of nonproliferative fibrous tissue samples, respectively. Hepatocyte growth factor receptor was detected in 89% of desmoids and all scars tested, but not in any of the fibrous tissue samples. Epidermal growth factor receptor was detected in only 12% of desmoids and not in scar or fibrous tissue. Mast/stem cell growth factor receptor, receptor tyrosine–protein kinase erbB-2, and phosphorylated insulin-like growth factor 1 receptor/insulin receptor were negative in all study cases. Variable levels of phosphorylated downstream signal transduction molecules RAC-α/β/γ serine/threonine-protein kinase, mitogenactivated protein kinase, and signal transducer and activator of transcription-3 were observed in desmoids (58%, 62%, and 67%), scar tissues (100%, 86%, and 86%), and fibrous tissue (33%, 17%, and 17%). These results indicate that tyrosine kinase signaling is active in both fibromatosis and healing scar, but not in most nonproliferating fibrous tissues. Although platelet-derived growth factor receptor, β type, is expressed ubiquitously in desmoids, the kinases driving

  17. Signal transduction pathway analysis in fibromatosis: receptor and nonreceptor tyrosine kinases.

    Science.gov (United States)

    Cates, Justin M M; Black, Jennifer O; Itani, Doha M; Fasig, John H; Keedy, Vicki L; Hande, Kenneth R; Whited, Brent W; Homlar, Kelly C; Halpern, Jennifer L; Holt, Ginger E; Schwartz, Herbert S; Coffin, Cheryl M

    2012-10-01

    Despite reports of receptor tyrosine kinase activation in desmoid-type fibromatosis, therapeutic benefits of kinase inhibitor therapy are unpredictable. Variability in signal transduction or cellular kinases heretofore unevaluated in desmoid tumors may be responsible for these inconsistent responses. In either case, a better understanding of growth regulatory signaling pathways is necessary to assess the theoretical potential of inhibitor therapy. Immunohistochemical analysis of tyrosine kinases and activated isoforms of downstream signal transduction proteins was performed on a tissue microarray containing 27 cases of desmoid-type fibromatosis and 14 samples of scar; 6 whole sections of normal fibrous tissue were studied for comparison. Platelet-derived growth factor receptor, β type, and focal adhesion kinase 1 were expressed in all desmoid tumors and healing scars but only 80% and 50% of nonproliferative fibrous tissue samples, respectively. Hepatocyte growth factor receptor was detected in 89% of desmoids and all scars tested, but not in any of the fibrous tissue samples. Epidermal growth factor receptor was detected in only 12% of desmoids and not in scar or fibrous tissue. Mast/stem cell growth factor receptor, receptor tyrosine-protein kinase erbB-2, and phosphorylated insulin-like growth factor 1 receptor/insulin receptor were negative in all study cases. Variable levels of phosphorylated downstream signal transduction molecules RAC-α/β/γ serine/threonine-protein kinase, mitogen-activated protein kinase, and signal transducer and activator of transcription-3 were observed in desmoids (58%, 62%, and 67%), scar tissues (100%, 86%, and 86%), and fibrous tissue (33%, 17%, and 17%). These results indicate that tyrosine kinase signaling is active in both fibromatosis and healing scar, but not in most nonproliferating fibrous tissues. Although platelet-derived growth factor receptor, β type, is expressed ubiquitously in desmoids, the kinases driving cell

  18. Renal neuroendocrine tumors

    Directory of Open Access Journals (Sweden)

    Brian R Lane

    2009-01-01

    Full Text Available Objectives: Neuroendocrine tumors (NETs are uncommon tumors that exhibit a wide range of neuroendocrine differentiation and biological behavior. Primary NETs of the kidney, including carcinoid tumor, small cell carcinoma (SCC, and large cell neuroendocrine carcinoma (LCNEC are exceedingly rare. Materials and Methods: The clinicopathologic features of renal NETs diagnosed at a single institution were reviewed along with all reported cases in the worldwide literature. Results: Eighty renal NETs have been described, including nine from our institution. Differentiation between renal NETs and the more common renal neoplasms (renal cell carcinoma, transitional cell carcinoma can be difficult since clinical, radiographic, and histopathologic features overlap. Immunohistochemical staining for neuroendocrine markers, such as synaptophysin and chromogranin, can be particularly helpful in this regard. Renal carcinoids are typically slow-growing, may secrete hormones, and pursue a variable clinical course. In contrast, SCC and LCNEC often present with locally advanced or metastatic disease and carry a poor prognosis. Nephrectomy can be curative for clinically localized NETs, but multimodality treatment is indicated for advanced disease. Conclusions: A spectrum of NETs can rarely occur in the kidney. Renal carcinoids have a variable clinical course; SCC and LCNEC are associated with poor clinical outcomes. Diagnosis of NETs, especially LCNEC, requires awareness of their rare occurrence and prudent use of immunohistochemical neuroendocrine markers.

  19. [Resection of Klatskin tumors].

    Science.gov (United States)

    Seehofer, D; Kamphues, C; Neuhaus, P

    2012-03-01

    Curative treatment of Klatskin tumors by radical surgical procedures with surgical preparation distant to the tumor region results in 5-year survival rates of 30-50%. This requires mandatory en bloc liver resection and resection of the extrahepatic bile duct often together with vascular resection. Nevertheless, the ideal safety margin of 0.5-1 cm remote from the macroscopic tumor extensions cannot be achieved in all cases. Based on hilar anatomy the probability of an adequate safety margin is higher using extended right hemihepatectomy together with portal vein resection compared to left hemihepatectomy. However, due to severe atrophy of the left liver lobe solely left-sided hepatectomy is feasible in some patients. In cases of eligibility for both procedures right hemihepatectomy is preferentially used due to the higher oncological radicality if sufficient liver function is present. Postoperative hepatic insufficiency and bile leakage after demanding biliary reconstruction, often with several small orifices, contribute to the postoperative complication rate of this complex surgical disease pattern.

  20. Cardiac tumors: echo assessment.

    Science.gov (United States)

    Mankad, Rekha; Herrmann, Joerg

    2016-12-01

    Cardiac tumors are exceedingly rare (0.001-0.03% in most autopsy series). They can be present anywhere within the heart and can be attached to any surface or be embedded in the myocardium or pericardial space. Signs and symptoms are nonspecific and highly variable related to the localization, size and composition of the cardiac mass. Echocardiography, typically performed for another indication, may be the first imaging modality alerting the clinician to the presence of a cardiac mass. Although echocardiography cannot give the histopathology, certain imaging features and adjunctive tools such as contrast imaging may aid in the differential diagnosis as do the adjunctive clinical data and the following principles: (1) thrombus or vegetations are the most likely etiology, (2) cardiac tumors are mostly secondary and (3) primary cardiac tumors are mostly benign. Although the finding of a cardiac mass on echocardiography may generate confusion, a stepwise approach may serve well practically. Herein, we will review such an approach and the role of echocardiography in the assessment of cardiac masses.

  1. Neuroendocrine Tumor, diagnostic difficulties

    Directory of Open Access Journals (Sweden)

    Pedro Oliveira

    2017-06-01

    Full Text Available Ectopic adrenocorticotropic hormone (ACTH secretion is a rare disease. A 51 years old woman, with a Cushing syndrome secondary to ectopic ACTH secretion, diagnosed in 2009, with mediastinal lymphadenopathy, whose biopsy was compatible with lung small cell carcinoma, staged as IIIB using TNM classification. No other lesions were found in patient study. The patient was submitted to chemotherapy, associated to ketoconazole 200 mg twice daily, with partial remission of both conditions. Three years later was admitted with an aggravation of Cushing syndrome. There was no evidence of progression of pulmonary disease. A cystic lesion in the pancreatic uncinated process was found by abdominal CT scan and with avid uptake by DOTANOC PET discreet in anterior mediastinal lymphadenopathy. Biopsy of pancreatic mass revealed a neuroendocrine tumor. Pulmonary masses were biopsied again and was in favor of neuroendocrine tumor. It was assumed the diagnosis of pancreatic neuroendocrine tumor with mediastinal metastasis. The patient initiated lanreotid (120 mg, monthly, subcutaneous in association with ketoconazole. After 5 months of therapy, patient died with sepsis secondary to pneumonia. Neuroendocrine tumours are rare, difficult to diagnose and with poor prognosis when associated with ectopic ACTH secreting Cushing syndrome.

  2. Cardiac tumors: echo assessment

    Directory of Open Access Journals (Sweden)

    Rekha Mankad MD

    2016-12-01

    Full Text Available Cardiac tumors are exceedingly rare (0.001–0.03% in most autopsy series. They can be present anywhere within the heart and can be attached to any surface or be embedded in the myocardium or pericardial space. Signs and symptoms are nonspecific and highly variable related to the localization, size and composition of the cardiac mass. Echocardiography, typically performed for another indication, may be the first imaging modality alerting the clinician to the presence of a cardiac mass. Although echocardiography cannot give the histopathology, certain imaging features and adjunctive tools such as contrast imaging may aid in the differential diagnosis as do the adjunctive clinical data and the following principles: (1 thrombus or vegetations are the most likely etiology, (2 cardiac tumors are mostly secondary and (3 primary cardiac tumors are mostly benign. Although the finding of a cardiac mass on echocardiography may generate confusion, a stepwise approach may serve well practically. Herein, we will review such an approach and the role of echocardiography in the assessment of cardiac masses.

  3. Mouse Leydig Tumor Cells

    Directory of Open Access Journals (Sweden)

    Bo-Syong Pan

    2011-01-01

    Full Text Available Cordycepin is a natural pure compound extracted from Cordyceps sinensis (CS. We have demonstrated that CS stimulates steroidogenesis in primary mouse Leydig cell and activates apoptosis in MA-10 mouse Leydig tumor cells. It is highly possible that cordycepin is the main component in CS modulating Leydig cell functions. Thus, our aim was to investigate the steroidogenic and apoptotic effects with potential mechanism of cordycepin on MA-10 mouse Leydig tumor cells. Results showed that cordycepin significantly stimulated progesterone production in dose- and time-dependent manners. Adenosine receptor (AR subtype agonists were further used to treat MA-10 cells, showing that A1, A 2A , A 2B , and A3, AR agonists could stimulate progesterone production. However, StAR promoter activity and protein expression remained of no difference among all cordycepin treatments, suggesting that cordycepin might activate AR, but not stimulated StAR protein to regulate MA-10 cell steroidogenesis. Meanwhile, cordycepin could also induce apoptotic cell death in MA-10 cells. Moreover, four AR subtype agonists induced cell death in a dose-dependent manner, and four AR subtype antagonists could all rescue cell death under cordycepin treatment in MA-10 cells. In conclusion, cordycepin could activate adenosine subtype receptors and simultaneously induce steroidogenesis and apoptosis in MA-10 mouse Leydig tumor cells.

  4. [Intraductal papillary mucinous pancreas tumor].

    Science.gov (United States)

    Maev, I V; Kaziulin, A N; Kucheriavyĭ, Iu A

    2008-01-01

    Data of the literature on the epidemiology, patogenesis, diagnosis, peculiarities of the symptoms and the treatment of the intraduct papillar pancreatic tumor, are analyzed in this review. These tumors are rare, there are up to 1% of the exocrine pancreatic tumors. Intraduct proliferation of the mucin producing cells, that are disposed as papillars is typical of these tumors. There are the symptoms of the acute or chronic pancreatitis, sometimes the diagnosis of this tumor is accidental. The main diagnostic methods are ultrasound (US) and computed tomography (CT). Endoluminal ultrasound (EUS) and magnetic resonance cholangiopancreatography (MRCP) are the main methods to reveal the intraduct growth. The surgical treatment is necessary for these patients.

  5. Cellular Potts modeling of tumor growth, tumor invasion and tumor evolution

    NARCIS (Netherlands)

    A. Szabó (Andras); R.M.H. Merks (Roeland)

    2013-01-01

    htmlabstractDespite a growing wealth of available molecular data, the growth of tumors, invasion of tumors into healthy tissue, and response of tumors to therapies are still poorly understood. Although genetic mutations are in general the first step in the development of a cancer, for the mutated

  6. Laser therapy in intraocular tumors

    Science.gov (United States)

    Carstocea, Benone D.; Gafencu, Otilia L.; Apostol, Silvia

    1995-01-01

    Intraocular tumors present special problems of diagnosis and treatment. Diagnostic methods include, in addition to systemic and ophthalmological examinations, ancillary examinations such as transillumination, fluorescein angiography, ultrasonography, radioactive phosphorus uptake test, radiology, computerized tomography, and fine-needle aspiration biopsy with cytological analyses. Previously, enucleation of the involved eye was generally accepted as management of malignant tumors. Improved therapeutic methods such as photocoagulation and better surgical techniques now provide a variety of therapeutical alternatives. This study consists of 21 cases of intraocular tumors that were managed by Argon laser photocoagulation. Four cases were intraocular metastasis and 17 cases were primitive intraocular tumors. Argon laser therapy proved to be totally ineffective for the intraocular metastasis and a very adequate therapy for the primitive tumors. Tumor extirpations (choroidal, cillary body, or iris tumors) using laser lancet proved to be more suitable than classic surgery.

  7. TumorHoPe: a database of tumor homing peptides.

    Directory of Open Access Journals (Sweden)

    Pallavi Kapoor

    Full Text Available BACKGROUND: Cancer is responsible for millions of immature deaths every year and is an economical burden on developing countries. One of the major challenges in the present era is to design drugs that can specifically target tumor cells not normal cells. In this context, tumor homing peptides have drawn much attention. These peptides are playing a vital role in delivering drugs in tumor tissues with high specificity. In order to provide service to scientific community, we have developed a database of tumor homing peptides called TumorHoPe. DESCRIPTION: TumorHoPe is a manually curated database of experimentally validated tumor homing peptides that specifically recognize tumor cells and tumor associated microenvironment, i.e., angiogenesis. These peptides were collected and compiled from published papers, patents and databases. Current release of TumorHoPe contains 744 peptides. Each entry provides comprehensive information of a peptide that includes its sequence, target tumor, target cell, techniques of identification, peptide receptor, etc. In addition, we have derived various types of information from these peptide sequences that include secondary/tertiary structure, amino acid composition, and physicochemical properties of peptides. Peptides in this database have been found to target different types of tumors that include breast, lung, prostate, melanoma, colon, etc. These peptides have some common motifs including RGD (Arg-Gly-Asp and NGR (Asn-Gly-Arg motifs, which specifically recognize tumor angiogenic markers. TumorHoPe has been integrated with many web-based tools like simple/complex search, database browsing and peptide mapping. These tools allow a user to search tumor homing peptides based on their amino acid composition, charge, polarity, hydrophobicity, etc. CONCLUSION: TumorHoPe is a unique database of its kind, which provides comprehensive information about experimentally validated tumor homing peptides and their target cells. This

  8. [Surgical therapy of proximal extrahepatic bile duct tumors (Klatskin tumors)].

    Science.gov (United States)

    Timm, S; Gassel, H-J; Thiede, A

    2007-08-01

    Due to their anatomical position, the tendency of early infiltrative growth and their poor prognosis without treatment, klatskin tumors are challenging concerning diagnosis and therapy. In contrast to other tumors of the gastrointestinal tract, for which exact diagnostic and stage dependent therapeutic guidelines could be formulated, clear recommendations for klatskin tumors are missing. Thus, survival rates after local resection, e. g. resection of the bile duct bifurcation alone, show high rates of R1/2 resection and early tumor recurrence. With an additional hepatic resection formally curative resections and long-term survival can be improved. Extended liver resections including the portal vein provide the highest rates of R0 resections for hilar carcinomas of the extrahepatic bile duct. Survival rates after liver transplantation for klatskin tumors are not yet convincing. Promising first results have been reported for the combination of neoadjuvant treatment and liver transplantation and might show future perspectives for the treatment of klatskin tumors.

  9. Breast Tumor Angiogenesis and Tumor-Associated Macrophages: Histopathologist's Perspective

    Directory of Open Access Journals (Sweden)

    Ewe Seng Ch'ng

    2011-01-01

    Full Text Available Much progress has been made since the conceptualization of tumor angiogenesis—the induction of growth of new blood vessels by tumor—as a salient feature of clinically significant primary or metastatic cancers. From a practicing histopathologist's point of view, we appraise the application of this concept in breast cancer with particular reference to the evaluation of proangiogenic factors and the assessment of new microvessels in histopathological examination. Recently, much focus has also been centered on the active roles played by tumor-associated macrophages in relation to tumor angiogenesis. We review the literature; many data supporting this facet of tumor angiogenesis were derived from the breast cancer models. We scrutinize the large body of clinical evidence exploring the link between the tumor-associated macrophages and breast tumor angiogenesis and discuss particularly the methodology and limitations of incorporating such an assessment in histopathological examination.

  10. Angiogenesis and tumor

    Directory of Open Access Journals (Sweden)

    Kamran Mansouri

    2010-12-01

    Full Text Available Angiogenesis, the process of new blood vessel formation from existing ones, plays an important role in the physiologic circumstances such as embryonic development, placenta formation, and wound healing. It is also crucial to progress of pathogenic processes of a variety of disorders, including tumor growth and metastasis. In general, angiogenesis process is a multi-factorial and highly structured sequence of cellular events comprising migration, proliferation and differentiation of endothelial cells and finally vascular formation, maturation and remodeling.Thereby, angiogenesis inhibition as a helping agent to conventional therapies such as chemotherapy and radiation has attracted the scientists’ attentions studying in this field.

  11. Imaging in Pediatric Infratentorial Tumors

    Directory of Open Access Journals (Sweden)

    S. Hajiahmadi

    2008-01-01

    Full Text Available Intracranial tumors are the second cause of malignancies in childhood following leukemia. The overall incidence varies between 1:20000 and 1:100000 in different series. They are the most common solid tumors that occur in childhood .The most important diagnostic feature of an intracranial mass is its location. They can be supratentorial or infratentorial. With the exception of the first year of life, infratentorial brain tumors are more frequent than supratentorial tumors in the first decade of life. In particular, these are cerebellar low-grade astrocytomas, medulloblastomas, brain stem gliomas and ependymomas of the fourth ventricle. .Posterior fossa tumors also are readily identified with both CT and MRI. Spectroscopy in the analysis of brain tumors has recently come on the scene but may be of limited practical value when it comes to differentiating tumors. However, CT and especially MRI are the primary imaging modalities for the investigation of brain tumors. Sonography can be used in the neonates. With modern imaging, it is relatively easy to detect the presence of a tumor in most patients. The purpose of this essay is to illustrate the imaging features of various infratentorial brain tumors to make a clue for differentiation them by these features.

  12. Mechanisms of Metastatic Tumor Dormancy

    Directory of Open Access Journals (Sweden)

    Mary Osisami

    2013-09-01

    Full Text Available Tumor metastasis can occur years after an apparent cure due to a phenomenon known as metastatic tumor dormancy; in which tumor masses or individual tumor cells are growth restricted for extended periods of time. This period of dormancy is induced and maintained by several mechanisms, including: (1 Tumor microenvironment factors such as cytokine expression, immunosurveillance and angiogenesis; (2 Metastasis suppressor gene activity; and (3 Cancer therapeutics. Disseminated tumor cells (DTC are the key cells that result in dormant tumors. However, many challenges exist towards isolating DTCs for mechanistic studies. The main DTC that may represent the dormant cell is the cancer stem cells (CSC as they have a slow proliferation rate. In addition to limited knowledge regarding induction of tumor dormancy, there are large gaps in knowledge regarding how tumors escape from dormancy. Emerging research into cancer stem cells, immunotherapy, and metastasis suppressor genes, may lead to new approaches for targeted anti-metastatic therapy to prevent dormancy escape. Overall, an enhanced understanding of tumor dormancy is critical for better targeting and treatment of patients to prevent cancer recurrence.

  13. Imaging tumors of the patella

    Energy Technology Data Exchange (ETDEWEB)

    Casadei, R., E-mail: roberto.casadei@ior.it [Department of Orthopaedic Oncology, Istituto Ortopedico Rizzoli, Bologna (Italy); Kreshak, J., E-mail: j.kreshak@yahoo.com [Department of Orthopaedic Oncology, Istituto Ortopedico Rizzoli, Bologna (Italy); Department of Pathology, Istituto Ortopedico Rizzoli, Bologna (Italy); Rinaldi, R. [Department of Radiology, Istituto Ortopedico Rizzoli, Bologna (Italy); Rimondi, E., E-mail: eugenio.rimondi@ior.it [Department of Radiology, Istituto Ortopedico Rizzoli, Bologna (Italy); Bianchi, G., E-mail: giuseppe.bianchi@ior.it [Department of Orthopaedic Oncology, Istituto Ortopedico Rizzoli, Bologna (Italy); Alberghini, M., E-mail: marco.alberghini@ior.it [Department of Pathology, Istituto Ortopedico Rizzoli, Bologna (Italy); Ruggieri, P. [Department of Orthopaedic Oncology, Istituto Ortopedico Rizzoli, Bologna (Italy); Vanel, D., E-mail: daniel.vanel@ior.it [Department of Radiology, Istituto Ortopedico Rizzoli, Bologna (Italy); Department of Pathology, Istituto Ortopedico Rizzoli, Bologna (Italy)

    2013-12-01

    Background: Patellar tumors are rare; only a few series have been described in the literature and radiographic diagnosis can be challenging. We reviewed all patellar tumors at one institution and reviewed the literature. Materials and methods: In an evaluation of the database at one institution from 1916 to 2009, 23,000 bone tumors were found. Of these, 41 involved the patella. All had imaging studies and microscopic diagnostic confirmation. All medical records, imaging studies, and pathology were reviewed. Results: There were 15 females and 26 males, ranging from 8 to 68 years old (average 30). There were 30 benign tumors; eight giant cell tumors, eight chondroblastomas, seven osteoid osteomas, two aneurysmal bone cysts, two ganglions, one each of chondroma, exostosis, and hemangioma. There were 11 malignant tumors: five hemangioendotheliomas, three metastases, one lymphoma, one plasmacytoma, and one angiosarcoma. Conclusion: Patellar tumors are rare and usually benign. As the patella is an apophysis, the most frequent lesions are giant cell tumor in the adult and chondroblastoma in children. Osteoid osteomas were frequent in our series and easily diagnosed. Metastases are the most frequent malignant diagnoses in the literature; in our series malignant vascular tumors were more common. These lesions are often easily analyzed on radiographs. CT and MR define better the cortex, soft tissue extension, and fluid levels. This study presents the imaging patterns of the more common patellar tumors in order to help the radiologist when confronted with a lesion in this location.

  14. What is a pediatric tumor?

    Directory of Open Access Journals (Sweden)

    Mora J

    2012-11-01

    Full Text Available Jaume Mora1,21Department of Oncology, 2Developmental Tumor Biology Laboratory, Hospital Sant Joan de Deu, Fundacio Sant Joan de Deu, Barcelona, SpainAbstract: Working together with medical oncologists, the question of whether a Ewing sarcoma in a 25-year-old is a pediatric tumor comes up repeatedly. Like Ewing's, some tumors present characteristically at ages that cross over what has been set as the definition of pediatrics (15 years, 18 years, or 21 years?. Pediatric oncology textbooks, surprisingly, do not address the subject of defining a pediatric tumor. They all begin with an epidemiology chapter defining the types of tumors appearing at distinct stages of childhood, adolescence, and young adulthood. Describing the epidemiology of tumors in relation to age, it becomes clear that the disease is related to the phenomenon of aging. The question, however, remains: is there a biological definition of what pediatric age is? And if so, will tumors occurring during this period of life have anything to do with such biological definition? With the aim of finding an objective definition, the fundamental concepts of what defines "pediatrics" was reviewed and then the major features of tumors arising during development were analyzed. The tumors were explored from the perspective of a host immersed in the normal process of growth and development. This physiological process, from pluripotential and undifferentiated cells, makes possible the differentiation, maturation, organization, and function of tissues, organs, and apparatus. A biological definition of pediatric tumors and the infancy–childhood–puberty classification of developmental tumors according to the infancy–childhood–puberty model of normal human development are proposed.Keywords: growth and development, pediatric tumor, infant, childhood and adolescence, pubertal tumors

  15. Multiparametric classification links tumor microenvironments with tumor cell phenotype.

    Directory of Open Access Journals (Sweden)

    Bojana Gligorijevic

    2014-11-01

    Full Text Available While it has been established that a number of microenvironment components can affect the likelihood of metastasis, the link between microenvironment and tumor cell phenotypes is poorly understood. Here we have examined microenvironment control over two different tumor cell motility phenotypes required for metastasis. By high-resolution multiphoton microscopy of mammary carcinoma in mice, we detected two phenotypes of motile tumor cells, different in locomotion speed. Only slower tumor cells exhibited protrusions with molecular, morphological, and functional characteristics associated with invadopodia. Each region in the primary tumor exhibited either fast- or slow-locomotion. To understand how the tumor microenvironment controls invadopodium formation and tumor cell locomotion, we systematically analyzed components of the microenvironment previously associated with cell invasion and migration. No single microenvironmental property was able to predict the locations of tumor cell phenotypes in the tumor if used in isolation or combined linearly. To solve this, we utilized the support vector machine (SVM algorithm to classify phenotypes in a nonlinear fashion. This approach identified conditions that promoted either motility phenotype. We then demonstrated that varying one of the conditions may change tumor cell behavior only in a context-dependent manner. In addition, to establish the link between phenotypes and cell fates, we photoconverted and monitored the fate of tumor cells in different microenvironments, finding that only tumor cells in the invadopodium-rich microenvironments degraded extracellular matrix (ECM and disseminated. The number of invadopodia positively correlated with degradation, while the inhibiting metalloproteases eliminated degradation and lung metastasis, consistent with a direct link among invadopodia, ECM degradation, and metastasis. We have detected and characterized two phenotypes of motile tumor cells in vivo, which

  16. Advances of Tumor Hyperthermia and Tumor Immunology in Translational Medicine

    Institute of Scientific and Technical Information of China (English)

    Hooshang Lahooti

    2015-01-01

    Hyperthermia is another important method in the treatment of tumors, secondary to surgery, radiotherapy, chemotherapy and biotherapy. It has been demonstrated the efifcacy and versatility of hyperthermia in a lot of randomized trials across various primary cancers. Both heat shock proteins (HSPs) and dendritic cells (DCs) are greatly affected by hyperthermia and closely related to the tumor immunology. Nowadays, tumor hyperthermia and tumor immunology have been attached much attention in the field of translational medicine. In this article, the action mechanism and immunological effects of hyperthermia, activation of HSPs and DCs as well as HSP- and DC-based cancer vaccine were reviewed from the perspective of translational medicine.

  17. Advances of Tumor Hyperthermia and Tumor Immunology in Translational Medicine

    Directory of Open Access Journals (Sweden)

    Hooshang Lahooti

    2015-09-01

    Full Text Available Hyperthermia is another important method in the treatment of tumors, secondary to surgery, radiotherapy, chemotherapy and biotherapy. It has been demonstrated the efficacy and versatility of hyperthermia in a lot of randomized trials across various primary cancers. Both heat shock proteins (HSPs and dendritic cells (DCs are greatly affected by hyperthermia and closely related to the tumor immunology. Nowadays, tumor hyperthermia and tumor immunology have been attached much attention in the field of translational medicine. In this article, the action mechanism and immunological effects of hyperthermia, activation of HSPs and DCs as well as HSP- and DC-based cancer vaccine were reviewed from the perspective of translational medicine.

  18. A rare tumor of the lung: inflammatory myofibroblastic tumor

    Directory of Open Access Journals (Sweden)

    Hammas Nawal

    2012-07-01

    Full Text Available Abstract Inflammatory myofibroblastic tumor is a rare benign lesion whose tumor origin is now proven. It represents 0.7% of all lung tumors. We report the case of a three-year-old child who suffered from a chronic cough with recurrent respiratory infections. Chest X-ray and computed tomography revealed the presence of a left lower lobe lung mass. After pneumonectomy, histological examination combined with immunohistochemical study discovered an inflammatory myofibroblastic tumor. The virtual slide(s for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/8722069326962972.

  19. NANOROBOTS IN BRAIN TUMOR

    Directory of Open Access Journals (Sweden)

    Sayyed Tarannum, Garje Dattatray H

    2011-02-01

    Full Text Available Nanomedicine is the process of diagnosing, treating, and preventing disease and traumatic injury, of relieving pain, and of preserving and improving human health, using molecular tools and molecular knowledge of the human body. In the relatively near term, nanomedicine can address many important medical problems by using nanoscale-structured materials and simple nanodevices that can be manufactured today, including the interaction of nanostructured materials with biological systems. The authors predict that technology-assisted medicine and robotics in particular, will have a significant impact over the next few decades. Robots will augment the surgeon’s motor performance, diagnosis capability, and senses with haptics (feel, augmented reality (sight, and ultrasound (sound. Robotic devices have been used in cardiac surgery, urology, fetal surgery, pediatrics, neurosurgery, orthopedics, and many other medical disciplines. In this article, we present the Nanorobot drug delivery to brain tumor, paying special attention to the transformation trends of organizations, and the integration of robots in brain tumor and underscoring potential repercussions which may deserve more attention and further research.

  20. Tumor triquilemal proliferante

    Directory of Open Access Journals (Sweden)

    Fulin Yu-Tseng

    2015-09-01

    Full Text Available El tumor triquilemal proliferante es una dermatosis tumoral poco frecuente y derivada de la capa externa de la raíz del folículo piloso. Generalmente, es de características benignas, tiende a ser recidivante y se han reportado casos de transformaciones malignas en la bibliografía mundial. El caso trata de una paciente femenina de 59 años, con una neoformación exofítica nodular lobulada de crecimiento lento en piel cabelluda, de 5 años de evolución, con recidivas después de 3 cirugías. No se documentó compromiso óseo ni cerebral en los estudios de gabinete. La biopsia mostró proliferación de nidos de células escamosas en dermis, separados por bandas de tejido fibroso de la epidermis. El abordaje quirúrgico es el tratamiento de elección de esta patología.

  1. Imaging Tumor Necrosis with Ferumoxytol.

    Directory of Open Access Journals (Sweden)

    Maryam Aghighi

    Full Text Available Ultra-small superparamagnetic iron oxide nanoparticles (USPIO are promising contrast agents for magnetic resonance imaging (MRI. USPIO mediated proton relaxation rate enhancement is strongly dependent on compartmentalization of the agent and can vary depending on their intracellular or extracellular location in the tumor microenvironment. We compared the T1- and T2-enhancement pattern of intracellular and extracellular USPIO in mouse models of cancer and pilot data from patients. A better understanding of these MR signal effects will enable non-invasive characterizations of the composition of the tumor microenvironment.Six 4T1 and six MMTV-PyMT mammary tumors were grown in mice and imaged with ferumoxytol-enhanced MRI. R1 relaxation rates were calculated for different tumor types and different tumor areas and compared with histology. The transendothelial leakage rate of ferumoxytol was obtained by our measured relaxivity of ferumoxytol and compared between different tumor types, using a t-test. Additionally, 3 patients with malignant sarcomas were imaged with ferumoxytol-enhanced MRI. T1- and T2-enhancement patterns were compared with histopathology in a descriptive manner as a proof of concept for clinical translation of our observations.4T1 tumors showed central areas of high signal on T1 and low signal on T2 weighted MR images, which corresponded to extracellular nanoparticles in a necrotic core on histopathology. MMTV-PyMT tumors showed little change on T1 but decreased signal on T2 weighted images, which correlated to compartmentalized nanoparticles in tumor associated macrophages. Only 4T1 tumors demonstrated significantly increased R1 relaxation rates of the tumor core compared to the tumor periphery (p<0.001. Transendothelial USPIO leakage was significantly higher for 4T1 tumors (3.4±0.9x10-3 mL/min/100cm3 compared to MMTV-PyMT tumors (1.0±0.9x10-3 mL/min/100 cm3. Likewise, ferumoxytol imaging in patients showed similar findings with

  2. Pericytes limit tumor cell metastasis

    DEFF Research Database (Denmark)

    Xian, Xiaojie; Håkansson, Joakim; Ståhlberg, Anders

    2006-01-01

    Previously we observed that neural cell adhesion molecule (NCAM) deficiency in beta tumor cells facilitates metastasis into distant organs and local lymph nodes. Here, we show that NCAM-deficient beta cell tumors grew leaky blood vessels with perturbed pericyte-endothelial cell-cell interactions...... and deficient perivascular deposition of ECM components. Conversely, tumor cell expression of NCAM in a fibrosarcoma model (T241) improved pericyte recruitment and increased perivascular deposition of ECM molecules. Together, these findings suggest that NCAM may limit tumor cell metastasis by stabilizing...... the microvessel wall. To directly address whether pericyte dysfunction increases the metastatic potential of solid tumors, we studied beta cell tumorigenesis in primary pericyte-deficient Pdgfb(ret/ret) mice. This resulted in beta tumor cell metastases in distant organs and local lymph nodes, demonstrating a role...

  3. Primary renal primitive neuroectodermal tumor.

    Science.gov (United States)

    Goel, V; Talwar, V; Dodagoudar, C; Singh, S; Sharma, A; Patnaik, N

    2015-01-01

    Primitive Neuroectodermal Tumor of the kidney is a rare entity. Very few cases of primary renal PNET have been reported to date. Most literature about rPNET is isolated case reports. We report a case of rPNET in a 39-year-old male with a pre-operative diagnosis of renal cell carcinoma with renal vein thrombosis. The patient underwent radical nephrectomy with thrombolectomy, and histopathological examination revealed a highly aggressive tumor composed of monotonous sheets of round cells. Tumor cells were positive for CD 99 and FLI-1, hence confirming the diagnosis of Primitive Neuroectodermal Tumor. Post-surgery, patient was given VAC/IE-based adjuvant chemotherapy. In view of highly aggressive nature of this tumor, prompt diagnosis and imparting effective chemotherapy regimen to the patient is required, and it is important to differentiate PNET from other small round-cell tumors because of different therapeutic approach.

  4. Differentiated thyroid tumors: surgical indications.

    Science.gov (United States)

    Lucchini, R; Monacelli, M; Santoprete, S; Triola, R; Conti, C; Pecoriello, R; Favoriti, P; Di Patrizi, M S; Barillaro, I; Boccolini, A; Avenia, S; D'Ajello, M; Sanguinetti, A; Avenia, N

    2013-01-01

    Thyroid gland tumors represent 1% of malignant tumors. In Italy their incidence is in constant growth. The aggressiveness depends on the histological type. The relative non-aggressive grade of different forms of tumors is the basis for discussing the treatment of choice: total thyroidectomy vs lobectomy with or without lymphadenectomy of the sixth level in the absence of metastasis. Authors report about their experience, and they advocate, given the high percentage of multicentric forms, total thyroidectomy as treatment of choice.

  5. Glutamate antagonists limit tumor growth

    OpenAIRE

    2001-01-01

    Neuronal progenitors and tumor cells possess propensity to proliferate and to migrate. Glutamate regulates proliferation and migration of neurons during development, but it is not known whether it influences proliferation and migration of tumor cells. We demonstrate that glutamate antagonists inhibit proliferation of human tumor cells. Colon adenocarcinoma, astrocytoma, and breast and lung carcinoma cells were most sensitive to the antiproliferative effect of the N...

  6. Self-scaling tumor growth

    DEFF Research Database (Denmark)

    Schmiegel, Jürgen

    We study the statistical properties of the star-shaped approximation of in vitro tumor profiles. The emphasis is on the two-point correlation structure of the radii of the tumor as a function of time and angle. In particular, we show that spatial two-point correlators follow a cosine law....... Based on this similarity, we provide a Lévy based model that captures the correlation structure of the radii of the star-shaped tumor profiles....

  7. Glutathione Levels in Human Tumors

    Science.gov (United States)

    Gamcsik, Michael P.; Kasibhatla, Mohit S.; Teeter, Stephanie D.; Colvin, O. Michael

    2013-01-01

    This review summarizes clinical studies in which glutathione was measured in tumor tissue from patients with brain, breast, gastrointestinal, gynecological, head and neck and lung cancer. Glutathione tends to be elevated in breast, ovarian, head and neck and lung cancer and lower in brain and liver tumors compared to disease-free tissue. Cervical, colorectal, gastric and esophageal cancers show both higher and lower levels of tumor glutathione. Some studies show an inverse relationship between patient survival and tumor glutathione. Based on this survey, we recommend approaches that may improve the clinical value of glutathione as a biomarker. PMID:22900535

  8. Tumors of the lacrimal gland

    DEFF Research Database (Denmark)

    Von Holstein, Sarah Linéa; Rasmussen, Peter Kristian; Heegaard, Steffen

    2016-01-01

    Tumors of the lacrimal gland comprise a wide spectrum, of which the most common demonstrate epithelial and lymphoid differentiation. The diagnosis of lacrimal gland tumors depends primarily on histological evaluation, as do the choice of treatment and prognosis. For some lacrimal gland neoplasms......, such as adenoid cystic carcinoma, the outlook is grave. Optimal treatment for several lacrimal gland tumors is also a matter of controversy. However, recent progress has been made in the molecular and genetic understanding of tumorigenesis for such lesions. This article presents an overview of the histopathology...... of lacrimal gland tumors, together with their epidemiological features, clinical characteristics, and treatment strategies....

  9. Tumors of the sublingual gland

    DEFF Research Database (Denmark)

    Andreasen, Simon; Bjørndal, K; Agander, T K

    2016-01-01

    Tumors of the salivary glands are a heterogeneous group of diseases most often originating in the major salivary glands. Only a minor proportion of mainly malignant tumors arise in the sublingual gland. Due to the rarity of sublingual gland tumors (SGTs), little is known about the clinicopathologic...... (ACC). Patient demography was similar to salivary gland tumors in other locations. All fine needle aspiration cytologies (FNACs) interpreted as benign were from ACCs. Metastatic disease was found in 12.5 % of ACCs at diagnosis with one third of all ACC patients having metastases at the end of follow...

  10. Subarachnoid hemorrhage in pituitary tumor

    Directory of Open Access Journals (Sweden)

    Ashis Patnaik

    2013-01-01

    Full Text Available Subarachnoid hemorrhage (SAH is the bleeding into the subarachnoid space containing cerebrospinal fluid. The most common cause of SAH is trauma. Rupture of aneurysms, vascular anomalies, tumor bleeds and hypertension are other important etiologies. SAH in the setting of pituitary tumor can result from various causes. It can be due to intrinsic tumor related pathology, injury to surrounding the vessel during the operative procedure or due to an associated aneurysm. We discuss the pathological mechanisms and review relevant literature related to this interesting phenomenon. Early and accurate diagnosis of the cause of the SAH in pituitary tumors is important, as this influences the management.

  11. Surgical Treatment in Uveal Tumors

    Directory of Open Access Journals (Sweden)

    Kaan Gündüz

    2014-09-01

    Full Text Available Surgical treatment in uveal tumors can be done via iridectomy, partial lamellar sclerouvectomy (PLSU and endoresection. Iridectomy is done in iris tumors without angle and ciliary body involvement. PLSU is performed in tumors with ciliary body and choroidal involvement. For this operation, a partial thickness scleral flap is dissected, the intraocular tumor is excised, and the flap is sutured back in position. PLSU surgery is done in iridociliary and ciliary body tumors with less than 3 clock hours of iris and ciliary body involvement and in choroidal tumors with a base diameter less than 15 mm. However, it can be employed in any size tumor for biopsy purposes. Potential complications of PLSU surgery include vitreous hemorrhage, cataract, retinal detachment, and endophthalmitis. Endoresection is a technique whereby the intraocular tumor is excised using vitrectomy techniques. The rationale for performing endoresection is based on the fact that irradiated uveal melanomas may be associated with exudation and neovascular glaucoma and removing the dead tumor tissue may contribute to better visual outcome. There are some centers where endoresection is done without prior radiotherapy. Allegedly, avoidance of radiation retinopathy and papillopathy are the main advantages of using endoresection without prior radiotherapy. (Turk J Ophthalmol 2014; 44: Supplement 29-34

  12. Notch Signaling and Brain Tumors

    DEFF Research Database (Denmark)

    Stockhausen, Marie; Kristoffersen, Karina; Poulsen, Hans Skovgaard

    2011-01-01

    Human brain tumors are a heterogenous group of neoplasms occurring inside the cranium and the central spinal cord. In adults and children, astrocytic glioma and medulloblastoma are the most common subtypes of primary brain tumors. These tumor types are thought to arise from cells in which Notch...... signaling plays a fundamental role during development. Recent findings have shown that Notch signaling is dysregulated, and contributes to the malignant potential of these tumors. Growing evidence point towards an important role for cancer stem cells in the initiation and maintenance of glioma...

  13. Monitoring Radiographic Brain Tumor Progression

    Directory of Open Access Journals (Sweden)

    John H. Sampson

    2011-03-01

    Full Text Available Determining radiographic progression in primary malignant brain tumors has posed a significant challenge to the neuroncology community. Glioblastoma multiforme (GBM, WHO Grade IV through its inherent heterogeneous enhancement, growth patterns, and irregular nature has been difficult to assess for progression. Our ability to detect tumor progression radiographically remains inadequate. Despite the advanced imaging techniques, detecting tumor progression continues to be a clinical challenge. Here we review the different criteria used to detect tumor progression, and highlight the inherent challenges with detection of progression.

  14. Pediatric Mediastinal Tumors and Tumor-Like Lesions.

    Science.gov (United States)

    Singh, Achint K; Sargar, Kiran; Restrepo, Carlos S

    2016-06-01

    This article reviews the imaging findings of pediatric mediastinal tumors and tumor-like lesions. The classification of the mediastinum is discussed with normal imaging appearance of the thymus in pediatric age group followed by a discussion on multiple mediastinal lesions in different compartments with emphasis on their imaging characteristics.

  15. Therapeutic Trial for Patients With Ewing Sarcoma Family of Tumor and Desmoplastic Small Round Cell Tumors

    Science.gov (United States)

    2016-08-25

    Desmoplastic Small Round Cell Tumor; Ewing Sarcoma of Bone or Soft Tissue; Localized Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor

  16. Cathepsins mediate tumor metastasis

    Institute of Scientific and Technical Information of China (English)

    Gong-Jun; Tan; Zheng-Ke; Peng; Jin-Ping; Lu; Fa-Qing; Tang

    2013-01-01

    Cathepsins are highly expressed in various human cancers, associated with tumor metastasis. It is superfamily, concluding A, B, C, D, E, F, G, H, L, K, O, S, V, and W family members. As a group of lysosomal proteinases or endopeptidases, each member has a different function, playing different roles in distinct tumorigenic processes such as proliferation, angiogenesis, metastasis, and invasion. Cathepsins belong to a diverse number of enzyme subtypes, including cysteine proteases, serine proteases and aspartic proteases. The contribution of cathepsins to invasion in human cancers is well documented, although the precise mechanisms by which cathepsins exert their effects are still not clear. In the present review, the role of cathepsin family members in cancer is discussed.

  17. Desmoid tumour in familial adenomatous polyposis. A review of literature

    DEFF Research Database (Denmark)

    Knudsen, Anne Louise; Bülow, Steffen

    2001-01-01

    in combination with tamoxifen. Surgery may be considered in case of a small and well-defined DT with no signs of invasion of vital structures, and in cases of imminent bowel ischaemia or obstruction. The prognosis in mesenteric DT is serious, and improvement of the therapeutic strategy awaits current...

  18. Desmoid tumour in familial adenomatous polyposis. A review of literature

    DEFF Research Database (Denmark)

    Knudsen, Anne Louise; Bülow, Steffen

    2001-01-01

    in combination with tamoxifen. Surgery may be considered in case of a small and well-defined DT with no signs of invasion of vital structures, and in cases of imminent bowel ischaemia or obstruction. The prognosis in mesenteric DT is serious, and improvement of the therapeutic strategy awaits current...... are considered to be correlated with the development and growth of DT. In patients with FAP, 50% of the tumours are localised intra-abdominally, and 85-100% of these are mesenteric. DT frequently present as non- tender, slowly growing masses. The symptoms are abdominal pain, vomiting, diarrhoea or haematochezia...

  19. Spontaneous ureteric rupture secondary to an invasive desmoid tumour

    Directory of Open Access Journals (Sweden)

    Peter Daechul Yoon

    2014-01-01

    CONCLUSION: Spontaneous rupture of the ureter is often misdiagnosed as other conditions. History taking and examination can be unreliable, hence a high level of suspicion and further investigations should be utilised. Once the diagnosis is made, treatment can be individualised based on aetiology.

  20. Solitary fibrous tumor

    Directory of Open Access Journals (Sweden)

    Manlio Mencoboni

    2010-12-01

    Full Text Available Solitary fibrous tumor (SFT is a rare mesenchymal neoplasm which may be found everywhere in the body. It is now distinguished into two forms, pleural and extrapleural, which morphologically resemble each other. Abdomi­nal localizations are quite rare, with 10 cases only reported in bladder; rarely they can be source of paraneoplastic syndromes (i.e., hypoglycemia secondary to insulin-like growth factor. In April 2006 a 74-year-old white male presented with chills, diaphoresis and acute abdominal pain with hematuria. At admission in emergency he underwent an abdominal X-ray (no pathological findings and an ultrasound examination of the kidneys and urinary tract, which revealed a pelvic hyperechogenic neoformation measuring approximately 10¥8¥7 cm, compressing the bladder. Blood chemistry at admission revealed only a mild neutrophilic leucocytosis (WBC 16600, N 80%, L 11%, elevated fibrinogen and ESR, and hypoglycemia (38 mg/dL. Macro­scopic hematuria was evident, while urinocolture was negative. Contrast enhanced CT scan of the abdomen and pelvic region revealed a large round neoformation dislocating the bladder, with an evident contrast-enhanced periphery and a central necrotic area. Continuous infusion of glucose 5% solution was necessary in order to maintain blood glucose levels above 50 mg/dL. The patient underwent complete surgical resection of an ovoidal mass coated by adipose tissue, with well delimited margins; histological findings were consistent with solitary fibrous tumor (SFT. Hypoglycemia resolved completely with removal of the growth. In this case report we describe a SFT growing in the bladder, a quite rare localization, which presented a unique hypoglycemia. In contrast to the majority of cases reported in the literature, the behavior of this SFT was not aggressive, and, since the patient is still alive, surgical resection was considered conclusive.

  1. Childhood brain tumor epidemiology: a brain tumor epidemiology consortium review.

    Science.gov (United States)

    Johnson, Kimberly J; Cullen, Jennifer; Barnholtz-Sloan, Jill S; Ostrom, Quinn T; Langer, Chelsea E; Turner, Michelle C; McKean-Cowdin, Roberta; Fisher, James L; Lupo, Philip J; Partap, Sonia; Schwartzbaum, Judith A; Scheurer, Michael E

    2014-12-01

    Childhood brain tumors are the most common pediatric solid tumor and include several histologic subtypes. Although progress has been made in improving survival rates for some subtypes, understanding of risk factors for childhood brain tumors remains limited to a few genetic syndromes and ionizing radiation to the head and neck. In this report, we review descriptive and analytical epidemiology childhood brain tumor studies from the past decade and highlight priority areas for future epidemiology investigations and methodological work that is needed to advance our understanding of childhood brain tumor causes. Specifically, we summarize the results of a review of studies published since 2004 that have analyzed incidence and survival in different international regions and that have examined potential genetic, immune system, developmental and birth characteristics, and environmental risk factors. Cancer Epidemiol Biomarkers Prev; 23(12); 2716-36. ©2014 AACR.

  2. [Local treatment of liver tumors

    DEFF Research Database (Denmark)

    Pless, T.K.; Skjoldbye, Bjørn Ole

    2008-01-01

    Local treatment of non-resectable liver tumors is common. This brief review describes the local treatment techniques used in Denmark. The techniques are evaluated according to the evidence in literature. The primary local treatment is Radiofrequency Ablation of both primary liver tumors and liver...

  3. Tumors STING adaptive antitumor immunity.

    Science.gov (United States)

    Bronte, Vincenzo

    2014-11-20

    Immunotherapy is revolutionizing the treatment of cancer patients, but the molecular basis for tumor immunogenicity is unclear. In this issue of Immunity, Deng et al. (2014) and Woo et al. (2014) provide evidence suggesting that dendritic cells detect DNA from tumor cells via the STING-mediated, cytosolic DNA sensing pathway.

  4. Tumors of the optic nerve

    DEFF Research Database (Denmark)

    Lindegaard, Jens; Heegaard, Steffen

    2009-01-01

    A variety of lesions may involve the optic nerve. Mainly, these lesions are inflammatory or vascular lesions that rarely necessitate surgery but may induce significant visual morbidity. Orbital tumors may induce proptosis, visual loss, relative afferent pupillary defect, disc edema and optic...... tumor-specific histologic features are given. Finally, treatment modalities and prognosis are discussed....

  5. Management of salivary gland tumors

    NARCIS (Netherlands)

    Andry, Guy; Hamoir, Marc; Locati, Laura D.; Licitra, Lisa; Langendijk, Johannes A.

    2012-01-01

    Surgery after proper imaging (MRI or CT scan) is the main stay of treatment for salivary gland tumors. Although excision margins should be >= 5 mm for malignant tumors in cases of parotid gland carcinoma, the facial nerve should be preserved whenever it is not infiltrated. Adjuvant external radiatio

  6. Surgical strategies in endocrine tumors

    NARCIS (Netherlands)

    Schreinemakers, J.M.J.

    2010-01-01

    Endocrine surgery has become more custom-made throughout the years. Endocrine tumors can be sporadic or develop as part of familial syndromes. Several familial syndromes are known to cause endocrine tumors. The most common are multiple endocrine neoplasia (MEN) syndromes type 1, 2A and 2B. This

  7. Brain Tumor Epidemiology Consortium (BTEC)

    Science.gov (United States)

    The Brain Tumor Epidemiology Consortium is an open scientific forum organized to foster the development of multi-center, international and inter-disciplinary collaborations that will lead to a better understanding of the etiology, outcomes, and prevention of brain tumors.

  8. Primitive neuroectodermal tumor: CT findings

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Chang Soo; Eun, Tchoong Kie; Cha, Seong Sook; Han, Sang Suk; Chung, Duck Hwan [Inje College, Paik Hospital, Busan (Korea, Republic of)

    1987-04-15

    Primitive neuroectodermal tumor is a neoplasm of young individuals that occurs predominantly in the supratentorial compartment. Authors experienced three cases of primitive neuroectodermal tumor. On the brain CT scans, all three cases reveal the large, irregular, hyperdense mass lesions with calcifications and cystic or necrotic areas, and show dense heterogeneous contrast enhancement.

  9. [Intraoperative staging of colorectal tumors].

    Science.gov (United States)

    Abdurakhmonov, Iu B; Mel'nikov, O R; Egorenkov, V V; Moiseenko, V M

    2007-01-01

    The effectiveness of intraoperative staging of tumor by sentinel node staining with lymphotropic dyes was evaluated in 60 patients with colorectal tumors (colon carcinoma -39, rectal cancer- 21). High sensitivity (84.6% and 87.5%, respectively) and specificity (100% and 100%, respectively) for regional lymph node assessment were identified for both colonic and rectal cancer.

  10. TUMOR OVARIUM: PREDIKSI KEGANASAN PRABEDAH

    Directory of Open Access Journals (Sweden)

    I NYOMAN GEDE BUDIANA

    2014-12-01

    Full Text Available Untuk meningkatkan kelangsungan hidup penderita kanker ovarium, dilakukan upaya untukmemprediksi keganasan tumor tersebut sebelum dilakukan pembedahan, karena adanya perbedaanpenanganan pada tumor jinak dan kanker ovarium. Terdapat berbagai modalitas untuk mendeteksikeganasan tumor ovarium prabedah. Mulai dari pemeriksaan klinis melalui anamnesis danpemeriksaan fisik, serta pemeriksaan penunjang seperti petanda tumor dan ultrasonografi. Untukmeningkatkan akurasi dalam mendeteksi keganasan tumor ovarium prabedah, dibuat berbagai sistemskoring. Indeks keganasan Sudaryanto, memakai batasan total skor e” 3 menunjukkan risiko keganasantinggi, sedangkan total skor < 3 menunjukkan risiko keganasan rendah. Melalui pemeriksaan USG,dibuat sistem skoring morfologi berdasarkan struktur permukaan dalam tumor, ketebalan dinding,septa, dan echogenitas tumor yang disebut indeks morfologi Sassone-Timor Tritsch. Denganmenggunakan batas skor 9, ditetapkan skor < 9 menunjukkan prediksi jinak dan skor e” 9 menunjukkanprediksi ganas. Batasan ini memiliki sensitifitas 94%, spesifisitas 87%, nilai duga positif 60%, dannilai duga negatif 93,6%. Metode lainnya adalah Risk of Malignancy Index (RMI. RMI mengintegrasikanstatus menopause penderita, temuan USG, dan kadar CA 125 serum. Nilai cut-off 200 digunakanuntuk membedakan tumor ovarium yang jinak dan ganas serta mempunyai sensitifitas 87% danspesifisitas 97%. The Risk of Ovarian Malignancy Algorithm (ROMA merupakan upaya koreksi RMIdengan menambahkan biomarker human protein epididymis 4 (HE4 dan menghilangkan USG. Untukmemprediksi kanker ovarium tipe epitel, ROMA mempunyai sensitifitas dan spesifisitas masingmasingsebesar 89% dan 83%. Jadi, terdapat berbagai modalitas untuk memprediksi keganasantumorovarium dengan akurasi berbeda-beda. Penerapannya disesuaikan dengan sarana dan prasaranayangtersedia sesuai dengan kondisi tempat pelayanan kesehatan.

  11. Tumor microenvironment and therapeutic response.

    Science.gov (United States)

    Wu, Ting; Dai, Yun

    2017-02-28

    The tumor microenvironment significantly influences therapeutic response and clinical outcome. Microenvironment-mediated drug resistance can be induced by soluble factors secreted by tumor or stromal cells. The adhesion of tumor cells to stromal fibroblasts or to components of the extracellular matrix can also blunt therapeutic response. Microenvironment-targeted therapy strategies include inhibition of the extracellular ligand-receptor interactions and downstream pathways. Immune cells can both improve and obstruct therapeutic efficacy and may vary in their activation status within the tumor microenvironment; thus, re-programme of the immune response would be substantially more beneficial. The development of rational drug combinations that can simultaneously target tumor cells and the microenvironment may represent a solution to overcome therapeutic resistance. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  12. [Radiological evaluation of congenital tumors].

    Science.gov (United States)

    Aguado del Hoyo, A; Ruiz Martín, Y; Lancharro Zapata, Á; Marín Rodríguez, C; Gordillo Gutiérrez, I

    2015-01-01

    In this article, we consider tumors that are diagnosed during pregnancy or in the first three months of life. This is a heterogeneous group of neoplasms with special biological and epidemiological characteristics that differentiate them from tumors arising in children or adults. In the last two decades, the prenatal detection of congenital tumors has increased due to the generalized use of prenatal sonographic screening. Advances in imaging techniques, especially in fetal magnetic resonance imaging, have enabled improvements in the diagnosis, follow-up, clinical management, and perinatal treatment of these tumors. This image-based review of the most common congenital tumors describes their histologic types, locations, and characteristics on the different imaging techniques used.

  13. Benign mixed tumor of the lacrimal sac

    Directory of Open Access Journals (Sweden)

    Jong-Suk Lee

    2015-01-01

    Full Text Available Neoplasms of the lacrimal drainage system are uncommon, but potentially life-threatening and are often difficult to diagnose. Among primary lacrimal sac tumors, benign mixed tumors are extremely rare. Histologically, benign mixed tumors have been classified as a type of benign epithelial tumor. Here we report a case of benign mixed tumor of the lacrimal sac.

  14. Pathogenesis and progression of fibroepithelial breast tumors

    NARCIS (Netherlands)

    Kuijper, Arno

    2006-01-01

    Fibroadenoma and phyllodes tumor are fibroepithelial breast tumors. These tumors are biphasic, i.e. they are composed of stroma and epithelium. The behavior of fibroadenomas is benign, whereas phyllodes tumors can recur and even metastasize. Classification criteria for both tumors show considerable

  15. The history of tumor virology.

    Science.gov (United States)

    Javier, Ronald T; Butel, Janet S

    2008-10-01

    In the century since its inception, the field of tumor virology has provided groundbreaking insights into the causes of human cancer. Peyton Rous founded this scientific field in 1911 by discovering an avian virus that induced tumors in chickens; however, it took 40 years for the scientific community to comprehend the effect of this seminal finding. Later identification of mammalian tumor viruses in the 1930s by Richard Shope and John Bittner, and in the 1950s by Ludwik Gross, sparked the first intense interest in tumor virology by suggesting the possibility of a similar causal role for viruses in human cancers. This change in attitude opened the door in the 1960s and 1970s for the discovery of the first human tumor viruses--EBV, hepatitis B virus, and the papillomaviruses. Such knowledge proved instrumental to the development of the first cancer vaccines against cancers having an infectious etiology. Tumor virologists additionally recognized that viruses could serve as powerful discovery tools, leading to revolutionary breakthroughs in the 1970s and 1980s that included the concept of the oncogene, the identification of the p53 tumor suppressor, and the function of the retinoblastoma tumor suppressor. The subsequent availability of more advanced molecular technologies paved the way in the 1980s and 1990s for the identification of additional human tumor viruses--human T-cell leukemia virus type 1, hepatitis C virus, and Kaposi's sarcoma virus. In fact, current estimates suggest that viruses are involved in 15% to 20% of human cancers worldwide. Thus, viruses not only have been shown to represent etiologic agents for many human cancers but have also served as tools to reveal mechanisms that are involved in all human malignancies. This rich history promises that tumor virology will continue to contribute to our understanding of cancer and to the development of new therapeutic and preventive measures for this disease in the 21st century.

  16. Improving drug delivery to solid tumors: priming the tumor microenvironment.

    Science.gov (United States)

    Khawar, Iftikhar Ali; Kim, Jung Ho; Kuh, Hyo-Jeong

    2015-03-10

    Malignant transformation and growth of the tumor mass tend to induce changes in the surrounding microenvironment. Abnormality of the tumor microenvironment provides a driving force leading not only to tumor progression, including invasion and metastasis, but also to acquisition of drug resistance, including pharmacokinetic (drug delivery-related) and pharmacodynamic (sensitivity-related) resistance. Drug delivery systems exploiting the enhanced permeability and retention (EPR) effect and active targeting moieties were expected to be able to cope with delivery-related drug resistance. However, recent evidence supports a considerable barrier role of tumors via various mechanisms, which results in imperfect or inefficient EPR and/or targeting effect. The components of the tumor microenvironment such as abnormal tumor vascular system, deregulated composition of the extracellular matrix, and interstitial hypertension (elevated interstitial fluid pressure) collectively or cooperatively hinder the drug distribution, which is prerequisite to the efficacy of nanoparticles and small-molecule drugs used in cancer medicine. Hence, the abnormal tumor microenvironment has recently been suggested to be a promising target for the improvement of drug delivery to improve therapeutic efficacy. Strategies to modulate the abnormal tumor microenvironment, referred to here as "solid tumor priming" (vascular normalization and/or solid stress alleviation leading to improvement in blood perfusion and convective molecular movement), have shown promising results in the enhancement of drug delivery and anticancer efficacy. These strategies may provide a novel avenue for the development of new chemotherapeutics and combination chemotherapeutic regimens as well as reassessment of previously ineffective agents. Copyright © 2014 Elsevier B.V. All rights reserved.

  17. A New Type of Uterine Trophoblastic Tumor: Epithelioid Trophoblastic Tumor

    Institute of Scientific and Technical Information of China (English)

    Langdi Fan; Zhanhong Wang; Xiurong Wang; Yingge Xing

    2005-01-01

    OBJECTIVE To describe a case of a patient with an epithelioid trophoblastic tumor (ETT) and review the literature regarding this new type of uterine trophoblastic tumor which is being reported with increasing frequency.There have been only 42 cases described in the world literature.METHODS Routine sections of the tumor were prepared and stained with H&E. Using the SP method, immunohistochemical staining, for AE1/AE3,hPL, PLAP, and α-inhibin antigens was conducted.RESULTS The patient was a 34 years old female who had delivered 12 years previously. She presented with amenorrhea for three months and vaginal irregular bleeding for 2 months. Her serum hCG level was 2,240 IU/L. After diagnostic curettage, an ETT was identified, and total abdominal hysterectomy and bilateral salpingo-oophorectomy (TAHBSO) performed.On microscopic examination it was found that the tumor was composed of chorionic-type intermediate- trophoblastic cells. The tumor cell nests were distributed in a geographical pattern. Some cells were filled with an eosinophilic hyalinized degenerative material. Study of the immunophenotype of the tumor showed that AE1/AE3, hPL, hCG, and α-inhibin were positively expressed.CONCLUSION This is the 4th case report of an ETT in China. The tumor was identified as a new type of trophoblastic tumor combined with a focal chorioepithelioid carcinoma, a condition that is extremely rare. It consists of chorionic-type intermediate-trophoblastic cells, and is considered to have a Iowgrade of malignancy. ETT should be differentiated from a placenta-site trophoblastic tumor, placenta-site nodule, chorioepithelioid carcinoma, and cervical squamous cell carcinoma.

  18. IMRT in hypopharyngeal tumors

    Energy Technology Data Exchange (ETDEWEB)

    Studer, G.; Luetolf, U.M.; Davis, J.B.; Glanzmann, C. [Dept. of Radiation Oncology, Univ. Hospital, Zurich (Switzerland)

    2006-06-15

    Background and purpose: intensity-modulated radiation therapy (IMRT) data on hypopharyngeal cancer (HC) are scant. In this study, the authors report on early results in an own HC patient cohort treated with IMRT. A more favorable outcome as compared to historical data on conventional radiation techniques was expected. Patients and methods: 29 consecutive HC patients were treated with simultaneous integrated boost (SIB) IMRT between 01/2002 and 07/2005 (mean follow-up 16 months, range 4-44 months). Doses of 60-71 Gy with 2.0-2.2 Gy/fraction were applied. 26/29 patients were definitively irradiated, 86% received simultaneous cisplatin-based chemotherapy. 60% presented with locally advanced disease (T3/4 Nx, Tx N2c/3). Mean primary tumor volume measured 36.2 cm{sup 3} (4-170 cm{sup 3}), mean nodal volume 16.6 cm{sup 3} (0-97 cm{sup 3}). Results: 2-year actuarial local, nodal, distant control, and overall disease-free survival were 90%, 93%, 93%, and 90%, respectively. In 2/4 patients with persistent disease (nodal in one, primary in three), salvage surgery was performed. The mean dose to the spinal cord (extension of > 5-15 mm) was 26 Gy (12-38 Gy); the mean maximum (point) dose was 44.4 Gy (26-58.9 Gy). One grade (G) 3 dysphagia and two G4 reactions (laryngeal fibrosis, dysphagia), both following the schedule with 2.2 Gy per fraction, have been observed so far. Larynx preservation was achieved in 25/26 of the definitively irradiated patients (one underwent a salvage laryngectomy); 23 had no or minimal dysphagia (G0-1). Conclusion: excellent early disease control and high patient satisfaction with swallowing function in HC following SIB IMRT were observed; these results need to be confirmed based on a longer follow-up period. In order to avoid G4 reactions, SIB doses of < 2.2 Gy/fraction are recommended for large tumors involving laryngeal structures. (orig.)

  19. Initiative action of tumor-associated macrophage during tumor metastasis

    Directory of Open Access Journals (Sweden)

    Saroj Singh

    2017-06-01

    In this review article, we present an overview of mechanisms responsible for TAMs recruitment and highlight the roles of TAMs in the regulation of tumor angiogenesis, invasion, metastasis, immunosuppression, and chemotherapeutic resistance. We describe the interplay between Th17 cells and other immune cells in the tumor microenvironment, and we assess both the potential antitumorigenic and pro-tumorigenic activities of Th17 cells and their associated cytokines. Understanding the nature of Th17 cell responses in the tumor microenvironment will be important for the design of more efficacious cancer immunotherapies. Finally, we discuss TAM-targeting therapy as a promising novel strategy for an indirect cancer therapy.

  20. SIRT1, Is It a Tumor Promoter or Tumor Suppressor?

    Directory of Open Access Journals (Sweden)

    Chu-Xia Deng

    2009-01-01

    Full Text Available SIRT1 has been considered as a tumor promoter because of its increased expression in some types of cancers and its role in inactivating proteins that are involved in tumor suppression and DNA damage repair. However, recent studies demonstrated that SIRT1 levels are reduced in some other types of cancers, and that SIRT1 deficiency results in genetic instability and tumorigenesis, while overexpression of SIRT1 attenuates cancer formation in mice heterozygous for tumor suppressor p53 or APC. Here, I review these recent findings and discuss the possibility that activation of SIRT1 both extends lifespan and inhibits cancer formation.

  1. Tumor cerebral e gravidez Brain tumors and pregnancy

    Directory of Open Access Journals (Sweden)

    José Carlos Lynch

    2007-12-01

    Full Text Available O diagnóstico de um tumor cerebral durante a gravidez é um fato raro que coloca a mãe e o concepto em risco de vida. OBJETIVO: Avaliar a melhor forma de conduzir uma paciente grávida portadora de um tumor cerebral. MÉTODO: Realizamos análise retrospectiva dos prontuários e imagens de seis pacientes grávidas portadoras de tumor cerebral. RESULTADOS: Vários tipos histológicos de tumor cerebral podem estar associados à gravidez. O meningioma é o mais freqüente. Nessa série não observamos óbito cirúrgico materno. Em duas pacientes, o parto ocorreu antes da craniotomia e em outras quatro o parto foi realizado após a neurocirurgia. CONCLUSÃO: O momento mais adequado para a realização da craniotomia para remoção tumoral irá depender da gravidade do quadro neurológico, do tipo histológico presumível da lesão, e da idade gestacional do embrião.BACKGROUND: Despite not being a common fact, the occurrence of brain tumors during pregnancy poses a risk to both the mother and infant. AIM: To identify the best medical procedure to be followed for a pregnant patient harboring a brain tumor. METHOD: The records of 6 patients with brain tumors, diagnosed during pregnancy were examined. RESULTS: Several types of brain tumors have been associated with pregnancy, but the meningioma is, by far, the most frequent. It seems that pregnancy aggravates the clinical course of intracranial tumors. There were no operative mortality in these series. In 2 patients the labor occurred before the craniotomy and in others, the delivery occurred after the surgery. CONCLUSION: The best moment to recommend the craniotomy and the neurosurgical removal of the tumor will depend of the mother’s neurological condition, the tumor histological type as well as the gestational age.

  2. Cushing syndrome due to adrenal tumor

    Science.gov (United States)

    Adrenal tumor - Cushing syndrome ... Cushing syndrome is a disorder that occurs when your body has a higher than normal level of the ... or cancerous (malignant). Noncancerous tumors that can cause ... Adrenal adenomas Micronodular hyperplasia Cancerous tumors that ...

  3. What Are the Symptoms of Pituitary Tumors?

    Science.gov (United States)

    ... Clinical Trials Resources and Publications What are the symptoms of pituitary tumors? Skip sharing on social media links Share this: Page Content Symptoms of Functioning Tumors The symptoms of functioning tumors ...

  4. Tumor targeting via integrin ligands

    Directory of Open Access Journals (Sweden)

    Udaya Kiran eMarelli

    2013-08-01

    Full Text Available Selective and targeted delivery of drugs to tumors is a major challenge for an effective cancer therapy and also to overcome the side effects associated with current treatments. Overexpression of various receptors on tumor cells is a characteristic structural and biochemical aspect of tumors and distinguishes them from physiologically normal cells. This abnormal feature is therefore suitable for selectively directing anticancer molecules to tumors by using ligands that can preferentially recognize such receptors. Several subtypes of integrin receptors that are crucial for cell adhesion, cell signaling, cell viability and motility have been shown to have an upregulated expression on cancer cells. Thus, ligands that recognize specific integrin subtypes represent excellent candidates to be conjugated to drugs or drug carrier systems and be targeted to tumors. In this regard, integrins recognizing the RGD cell adhesive sequence have been extensively targeted for tumor specific drug delivery. Here we review key recent examples on the presentation of RGD-based integrin ligands by means of distinct drug delivery systems, and discuss the prospects of such therapies to specifically target tumor cells.

  5. Laser therapy in ocular tumors

    Science.gov (United States)

    Carstocea, Benone D.; Gafencu, Otilia L.; Apostol, Silvia; Ionita, Marcel A.; Moroseanu, A.; Dascalu, Traian; Lupei, Voicu; Ionita-Manzatu, V.

    1998-07-01

    The medical laser equipments made at NILPRP have been exploited intensively for more than 10 years at CMH. The availability and reliability of the first like-on equipment have increased, following improvements in optical delivery system and cooling circuit. This paper shows the impact of technical advances on the development of ophthalmologic laser therapy. Intraocular tumors pose special problems of diagnosis and treatment. Diagnostic methods include addition to systemic and ophthalmologic examinations, ancillary examinations, such as transillumination, fluorescence angiography, ultrasonography, radioactive phosphorus uptake tests, radiology, computerized tomography and fine-needle aspiration biopsy with cytological analyses. The enucleation of the involved eye used to be a generally accepted management of malignant tumors. Improved therapeutic methods such as photocoagulation and better surgical techniques now provide a variety of therapeutic alternatives. This study covers 31 cases of intraocular tumors that were managed either by Argon Laser photocoagulation and/or by Nd:YAG laser surgical treatment. Four cases were intraocular metastasse and 17 cases were primitive intraocular tumors. Argon laser therapy proved to be totally ineffective for intraocular metastasse but very adequate therapy for primitive tumors. Tumor extirpations (choroidal, cillary body or iris tumors) using Nd:YAG laser lancet proved to be more suitable than classic surgery.

  6. [Orbital tumor emergencies in childhood].

    Science.gov (United States)

    Morax, S; Desjardins, L

    2009-05-01

    Emergencies in childhood orbital tumorals are rare. The absolute emergency involves malignant primary orbital tumors, such as rhabdomyosarcoma or secondary malignant tumors (metastatic neuroblastoma, leukemia), involving a vital prognosis requiring prompt diagnosis. Delayed emergencies are usually vascular lesions. Among these lesions, immature orbital hemangioma, with a good prognosis, must be distinguished from orbital adnexal lymphangiomas, which are less frequent but can lead to dramatic cosmetic and functional disorders. In rare cases, they can be responsible for sudden, painful proptosis, due to orbital hemorrhage, with a risk of optic nerve compression, requiring emergency surgical treatment. Neurogenous lesions, either isolated, such as in gliomas, or associated with a systemic disease, such as Recklinghausen neurofibromatosis, threaten the functional prognosis. Diagnosis of pediatric orbital tumors is based on a good clinical examination, precise imaging investigations, and evaluation of the locoregional extension of the tumor. Biopsy is required in emergency situations, when rhabdomyosarcoma is suspected, in order to start the chemotherapy. However, the biopsy can be superfluous, and even useless or dangerous, when clinical and imaging investigations are sufficient to provide a diagnosis of capillary hemangioma, lymphangioma, or metastatic tumor from an abdominal malignancy. Treatment is closely related to the etiopathogenesis of the tumor. The outcomes are vital, functional and cosmetic. They may require orbital surgery (biopsy, tumoral resection, orbital decompression in case of a compressive hemorrhage), systemic corticotherapy (as in immature adnexal hemangioma), radiation, and chemotherapy (rhabdomyosarcoma, secondary malignant tumor). These diseases require a pediatric ophthalmological medical center specializing in orbital surgery, with close collaboration of multiple specialists such as onco-pediatricians and neurosurgeons.

  7. [Phyllodes tumor: diagnosis and treatment].

    Science.gov (United States)

    Uribe, A; Bravo, G; Uribe, A; Viada, R; Capetillo, M; Villarroel, T

    1995-01-01

    We reviewed 1.178 benign tumors treated between 1981/93 among which 39 appeared with a Phylodes Tumors diagnosis, disregarding 5 of them because they did not have a precise description and histologic classification, studying 34 proved cases which represented 2.89% of all benign tumors; if we add 89% cancers in these years, we have 2.074 and the relation becomes 1.64% of the total. We found 22 benign phylodes (64.7%) 7 border line (20.5%) and 5 malignant (14.8%) whose clinic, histologic and evolutive characteristics are presented in this paper.

  8. Solid tumor cytogenetics: current perspectives.

    Science.gov (United States)

    Nanjangud, Gouri; Amarillo, Ina; Rao, P Nagesh

    2011-12-01

    Conventional cytogenetics in conjunction with Fluorescence in Situ Hybridization (FISH) continues to remain an important and integral component in the diagnosis and management of solid tumors. The ability to effectively detect the vast majority of clinically relevant chromosomal aberrations with a rapid-to-acceptable turnaround time makes them the most cost-effective screening/detection tool currently available in modern pathology. In this review, we describe a representative set of solid tumors in which chromosomal analysis and/or FISH plays a significant role in the routine clinical management of solid tumors.

  9. Intraosseous calcifying cystic odontogenic tumor

    Directory of Open Access Journals (Sweden)

    Kler Shikha

    2009-01-01

    Full Text Available The calcifying odontogenic cyst was first reported by Gorlin et al . in 1962. It had been classified as a neoplasm related to the odontogenic apparatus because of its histological complexity and morphological diversity until it was renamed as a calcifying cystic odontogenic tumor by the WHO, in 2005. Here we describe a case of mandibular calcifying cystic odontogenic tumor in a 75-year-old male, which was present since five years, with a history of occurrence after the extraction of teeth in the involved region. The lesion was surgically removed and a histopathological examination revealed a cystic tumor with predominance of ghost cells and some amount of dentinoid tissue.

  10. Uncommon liver tumors

    Science.gov (United States)

    Wu, Chia-Hung; Chiu, Nai-Chi; Yeh, Yi-Chen; Kuo, Yu; Yu, Sz-Shian; Weng, Ching-Yao; Liu, Chien-An; Chou, Yi-Hong; Chiou, Yi-You

    2016-01-01

    Abstract Background: Beside hepatocellular carcinoma, metastasis, and cholangiocarcinoma, the imaging findings of other relatively uncommon hepatic lesions are less discussed in the literature. Imaging diagnosis of these lesions is a daily challenge. In this article, we review the imaging characteristics of these neoplasms. Methods: From January 2003 to December 2014, 4746 patients underwent liver biopsy or hepatic surgical resection in our hospital. We reviewed the pathological database retrospectively. Imaging of these lesions was reviewed. Results: Imaging findings of uncommon hepatic lesions vary. We discuss the typical imaging characteristics with literature review. Clinical and pathological correlations are also described. Primary hepatic lymphoma consists only of 1% of the extranodal non-Hodgkin lymphoma, and is defined as the one involving only the liver and perihepatic lymph nodes within 6 months after diagnosis. Combined hepatocellular and cholangiocarcinoma (cHCC-CC) shares some overlapping imaging characteristics with both HCC and cholangiocarcinoma because of being an admixture of them. Angiosarcoma is the most common hepatic mesenchymal tumor and is hypervascular in nature. Inflammatory pseudotumor is often heterogeneous on ultrasonography and with enhanced septations and rims in the portovenous phase after contrast medium. Angiomyolipoma (AML) typically presents with macroscopic fat components with low signal on fat-saturated magnetic resonance imaging (MRI) and presence of drainage vessels. Intraductal papillary neoplasm of the bile duct (IPNB) is thought of as a counterpart to the pancreatic intraductal papillary mucinous neoplasm. Most of the IPNBs secrete mucin and cause disproportional dilatation of the bile ducts. Mucinous cystic neoplasm (MCN) contains proteinaceous and colloidal components without ductal communication and characterizes with hyperintensity on T1-weighted imaging. Other extremely rare lesions, including epithelioid

  11. Daemoplastic fiberblastoma of the foot: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Ji Ye; Park, Noh Hyuck; Park, Chan Sub; Seong, Su Ok; Kwon, Tae Jung [Myongji Hospital, Goyang (Korea, Republic of)

    2014-03-15

    The desmoplastic fibroblastoma is a unique fibrous soft tissue tumor which shows clinically and morphologically distinct characteristics. Most of the desmoplastic fibroblastomas arise from the extremities, shoulder girdle, posterior neck, upper back or abdominal wall. This tumor resembles histological a desmoid tumor. However, it is much better microscopic circumscribed. The clinical outcome after a surgical resection is better than that of a desmoid tumor. We will report about imaging findings of a desmoplastic fibroblastoma which raised in the foot with a review of the relevant literature.

  12. Primary tracheobronchial tumors in children.

    Science.gov (United States)

    Varela, Patricio; Pio, Luca; Torre, Michele

    2016-06-01

    Primary tracheobronchial tumors are rare lesions that can be benign or malignant, with different location along the airway tree. Symptoms may include wheezing, chronic pneumonia, asthma, chest pain, recurrent cough, atelectasis, haemoptysis, and weight loss. Due to the heterogeneity of symptoms, diagnosis can be difficult and the airway involvement can lead progressively to a bronchial or tracheal obstruction. Due to the rarity of primary tracheobronchial tumors in children, there are not any oncological guidelines on pre-operative work-up, treatment, and follow-up. Only few reports and multicentric studies are reported. In most cases, surgical resection seems to be the treatment of choice. Brachytherapy, endoscopic treatment, and chemotherapy are rarely described. In this article we present an overview on these rare tumors, including pathological aspects, clinical presentation, imaging assessment, and endoscopic or open surgical treatments. We discuss different surgical approaches, according with tumor location. Copyright © 2016 Elsevier Inc. All rights reserved.

  13. Brain tumor survivors speak out.

    Science.gov (United States)

    Carlson-Green, Bonnie

    2009-01-01

    Although progress has been made in the treatment of childhood brain tumors,work remains to understand the complexities of disease, treatment, and contextual factors that underlie individual differences in outcome. A combination of both an idiographic approach (incorporating observations made by adult survivors of childhood brain tumors) and a nomothetic approach (reviewing the literature for brain tumor survivors as well as childhood cancer survivors) is presented. Six areas of concern are reviewed from both an idiographic and nomothetic perspective, including social/emotional adjustment, insurance, neurocognitive late effects, sexuality and relationships, employment, and where survivors accessed information about their disease and treatment and possible late effects. Guidelines to assist health care professionals working with childhood brain tumor survivors are offered with the goal of improving psychosocial and neurocognitive outcomes in this population.

  14. What Are Gastrointestinal Stromal Tumors?

    Science.gov (United States)

    ... system, also known as the digestive system. The gastrointestinal system The gastrointestinal (GI) system (or digestive system) processes ... in “ How are gastrointestinal stromal tumors diagnosed? ” Other gastrointestinal tract cancers It is important to understand that GISTs ...

  15. Tumoral calcinosis mimicking recurrent osteosarcoma

    National Research Council Canada - National Science Library

    Petscavage, Jonelle M; Richardson, Michael L

    2009-01-01

    We report a case of a tumoral calcinosis mimicking the appearance of recurrent osteosarcoma of the left femur and tibia in a 29-year-old woman with a history of osteosarcoma and chronic renal failure...

  16. Drugs Approved for Wilms Tumor

    Science.gov (United States)

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

  17. Infantile pericardial round cell tumor

    Directory of Open Access Journals (Sweden)

    K H Sridevi

    2015-01-01

    Full Text Available Cardiac malignancies presenting in infancy are rare. Desmoplastic small round cell tumor (DSRCT is a rare occurrence in this age group. No case of intrapericardial DSRCT has been reported in the literature in infants.

  18. How Are Pituitary Tumors Diagnosed?

    Science.gov (United States)

    ... secreting tumor. Levels of related hormones, such as estrogen, progesterone, and testosterone, are often checked as well. Thyrotropin-secreting adenoma Tests to measure blood levels of thyrotropin (TSH) and thyroid hormones can usually identify people with a thyrotropin- ...

  19. Ovarian Germ Cell Tumors Treatment

    Science.gov (United States)

    ... ovarian germ cell tumor are swelling of the abdomen or vaginal bleeding after menopause. Ovarian germ cell ... if you have either of the following: Swollen abdomen without weight gain in other parts of the ...

  20. Radiofrequency Ablation of Lung Tumors

    Science.gov (United States)

    ... Rotating around you, the x-ray tube and electronic x-ray detectors are located opposite each other ... breathe is impaired, such as current or former cigarette smokers. When part of the tumor persists after ...

  1. Tumor Acidity as Evolutionary Spite

    Directory of Open Access Journals (Sweden)

    Mohammed E. A. Shayoub

    2011-01-01

    Full Text Available Most cancer cells shift their metabolic pathway from a metabolism reflecting the Pasteur-effect into one reflecting the Warburg-effect. This shift creates an acidic microenvironment around the tumor and becomes the driving force for a positive carcinogenesis feedback loop. As a consequence of tumor acidity, the tumor microenvironment encourages a selection of certain cell phenotypes that are able to survive in this caustic environment to the detriment of other cell types. This selection can be described by a process which can be modeled upon spite: the tumor cells reduce their own fitness by making an acidic environment, but this reduces the fitness of their competitors to an even greater extent. Moreover, the environment is an important dimension that further drives this spite process. Thus, diminishing the selective environment most probably interferes with the spite process. Such interference has been recently utilized in cancer treatment.

  2. Tumor Acidity as Evolutionary Spite

    Energy Technology Data Exchange (ETDEWEB)

    Alfarouk, Khalid O., E-mail: khalid.alfarouk@act.sd [Department of Biotechnology, Africa City of Technology, Khartoum (Sudan); Department of Pharmaceutics, Faculty of Pharmacy, University of Khartoum, Khartoum (Sudan); Muddathir, Abdel Khalig [Department of Pharmacognosy, Faculty of Pharmacy, University of Khartoum, Khartoum (Sudan); Shayoub, Mohammed E. A. [Department of Pharmaceutics, Faculty of Pharmacy, University of Khartoum, Khartoum (Sudan)

    2011-01-20

    Most cancer cells shift their metabolic pathway from a metabolism reflecting the Pasteur-effect into one reflecting the Warburg-effect. This shift creates an acidic microenvironment around the tumor and becomes the driving force for a positive carcinogenesis feedback loop. As a consequence of tumor acidity, the tumor microenvironment encourages a selection of certain cell phenotypes that are able to survive in this caustic environment to the detriment of other cell types. This selection can be described by a process which can be modeled upon spite: the tumor cells reduce their own fitness by making an acidic environment, but this reduces the fitness of their competitors to an even greater extent. Moreover, the environment is an important dimension that further drives this spite process. Thus, diminishing the selective environment most probably interferes with the spite process. Such interference has been recently utilized in cancer treatment.

  3. Tracking Tumor Evolution through Mathematics.

    Science.gov (United States)

    Leslie, Mitch

    2016-04-01

    A recent study suggests that many cancers undergo neutral evolution, with all key mutations present at the start of malignancy. New mutations acquired along the way don't confer any advantages on tumor cells.

  4. Laparoscopic Nephrectomy for Wilms Tumor

    OpenAIRE

    Andolfi C; Randi B; Ruggeri G.; Lima M.

    2014-01-01

    Wilms tumor is the most frequent primary renal malignancy in children. The surgical resection is traditionally performed through laparotomy. The advent of laparoscopic surgery for benign renal lesions has led the surgeon to use a minimal invasive approach for the nephroblastoma. We describe a 9-months-old girl who presented with a left renal mass. A laparoscopic resection of the tumor with left radical nephroureterectomy was performed. The specimens were removed in an endoscopic bag through a...

  5. Ossifying renal tumor of infancy.

    Science.gov (United States)

    Schelling, Johannes; Schröder, Annette; Stein, Raimund; Rösch, Wolfgang H

    2007-06-01

    A renal ossifying tumor of infancy is a rare event with few cases having been published, and the etiology has not yet been established. We report on two new cases of this unusual neoplasm. A 2-year-old boy presented with intermittent painless gross hematuria. After several diagnostic procedures, an open pyelolithotomy was performed and the histological diagnosis of renal tumor of infancy was finally made. The history of the second case is very similar. An 8-week-old infant presented with gross hematuria. As in the first case, an open pyelolithotomy was performed and a tumor entirely covered with blood clots was found in the renal pelvis and completely removed. A histological diagnosis of renal ossifying tumor of infancy was made. Using the literature available, the histological criteria and biological behavior are discussed, together with the diagnostic and therapeutic algorithm for this tumor. In infants with gross hematuria and a calcified (non-)invasive mass in the pelvi-calceal system, renal ossifying tumor should be considered in the differential diagnosis. MRI or CT scan offers a good diagnostic guide.

  6. Laparoscopic adrenalectomy for adrenal tumors.

    Science.gov (United States)

    Chuan-Yu, Sun; Yat-Faat, Ho; Wei-Hong, Ding; Yuan-Cheng, Gou; Qing-Feng, Hu; Ke, Xu; Bin, Gu; Guo-Wei, Xia

    2014-01-01

    Objective. To evaluate the indication and the clinical value of laparoscopic adrenalectomy of different types of adrenal tumor. Methods. From 2009 to 2014, a total of 110 patients were diagnosed with adrenal benign tumor by CT scan and we performed laparoscopic adrenalectomy. The laparoscopic approach has been the procedure of choice for surgery of benign adrenal tumors, and the upper limit of tumor size was thought to be 6 cm. Results. 109 of 110 cases were successful; only one was converted to open surgery due to bleeding. The average operating time and intraoperative blood loss of pheochromocytoma were significantly more than the benign tumors (P < 0.05). After 3 months of follow-up, the preoperative symptoms were relieved and there was no recurrence. Conclusions. Laparoscopic adrenalectomy has the advantages of minimal invasion, less blood loss, fewer complications, quicker recovery, and shorter hospital stay. The full preparation before operation can decrease the average operating time and intraoperative blood loss of pheochromocytomas. Laparoscopic adrenalectomy should be considered as the first choice treatment for the resection of adrenal benign tumor.

  7. Unraveling tumor grading and genomic landscape in lung neuroendocrine tumors.

    Science.gov (United States)

    Pelosi, Giuseppe; Papotti, Mauro; Rindi, Guido; Scarpa, Aldo

    2014-06-01

    Currently, grading in lung neuroendocrine tumors (NETs) is inherently defined by the histological classification based on cell features, mitosis count, and necrosis, for which typical carcinoids (TC) are low-grade malignant tumors with long life expectation, atypical carcinoids (AC) intermediate-grade malignant tumors with more aggressive clinical behavior, and large cell NE carcinomas (LCNEC) and small cell lung carcinomas (SCLC) high-grade malignant tumors with dismal prognosis. While Ki-67 antigen labeling index, highlighting the proportion of proliferating tumor cells, has largely been used in digestive NETs for assessing prognosis and assisting therapy decisions, the same marker does not play an established role in the diagnosis, grading, and prognosis of lung NETs. Next generation sequencing techniques (NGS), thanks to their astonishing ability to process in a shorter timeframe up to billions of DNA strands, are radically revolutionizing our approach to diagnosis and therapy of tumors, including lung cancer. When applied to single genes, panels of genes, exome, or the whole genome by using either frozen or paraffin tissues, NGS techniques increase our understanding of cancer, thus realizing the bases of precision medicine. Data are emerging that TC and AC are mainly altered in chromatin remodeling genes, whereas LCNEC and SCLC are also mutated in cell cycle checkpoint and cell differentiation regulators. A common denominator to all lung NETs is a deregulation of cell proliferation, which represents a biological rationale for morphologic (mitoses and necrosis) and molecular (Ki-67 antigen) parameters to successfully serve as predictors of tumor behavior (i.e., identification of pathological entities with clinical correlation). It is envisaged that a novel grading system in lung NETs based on the combined assessment of mitoses, necrosis, and Ki-67 LI may offer a better stratification of prognostic classes, realizing a bridge between molecular alterations

  8. Posterior Fossa Tumor in Children

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    Seyed Mahmoud TABATABAEI

    2012-06-01

    Full Text Available How to Cite this Article: Tabatabaei SM, Seddighi A, Seddighi AS. Posterior Fossa Tumor in Children. Iran. J. Child. Neurol 2012;6(2: 19-24. Objective Primary brain tumors are the most common solid neoplasms of childhood, representing 20% of all pediatric tumors. The best current estimates place the incidence between 2.76 and 4.28/100,000 children per year. Compared with brain tumors in adults, a much higher percentage of pediatric brain tumors arise in the posterior fossa. Infratentorial tumors comprise as many as two thirds of all pediatric brain tumors in some large series. Tumor types that most often occur in the posterior fossa include medulloblastoma, ependymoma, cerebellar astrocytoma and brainstem glioma. Materials & Methods All pediatric cases of posterior fossa tumor that were considered for surgery from 1981 to 2011 were selected and the demographic data including age, gender and tumor characteristics along with the location and pathological diagnosis were recorded. The surgical outcomes were assessed according to pathological diagnosis. Results Our series consisted of 84 patients (52 males, 32 females. Cerebellar symptoms were the most common cause of presentation (80.9% followed by headache (73.8% and vomiting (38.1%. The most common histology was medulloblastoma (42.8% followed by cerebellar astrocytoma (28.6%, ependymoma (14.3%, brainstem glioma (7.2% and miscellaneous pathologies (e.g., dermoid,  andtuberculoma (7.2%. Conclusion The diagnosis of brain tumors in the general pediatric population remains challenging. Most symptomatic children require several visits to a physician before the correct diagnosis is made. These patients are often misdiagnosed for gastrointestinal disorders. Greater understanding of the clinical presentation of these tumors and judicious use of modern neuroimaging techniques should lead to more efficacious therapies.References 1. Mehta V, Chapman A, McNeely PD, Walling S, Howes WJ. Latency between

  9. Necessity of Microdissecting Different Tumor Components in Pulmonary Tumor Pyrosequencing

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    Dahui Qin

    2016-01-01

    Full Text Available Microdissection is a useful method in tissue sampling prior to molecular testing. Tumor heterogeneity imposes new challenges for tissue sampling. Different microdissecting methods have been employed in face of such challenge. We improved our microdissection method by separately microdissecting the morphologically different tumor components. This improvement helped the pyrosequencing data analysis of two specimens. One specimen consisted of both adenocarcinoma and neuroendocrine components. When both tumor components were sequenced together for KRAS (Kirsten rat sarcoma viral oncogene homolog gene mutations, the resulting pyrogram indicated that it was not a wild type, suggesting that it contained KRAS mutation. However, the pyrogram did not match any KRAS mutations and a conclusion could not be reached. After microdissecting and testing the adenocarcinoma and neuroendocrine components separately, it was found that the adenocarcinoma was positive for KRAS G12C mutation and the neuroendocrine component was positive for KRAS G12D mutation. The second specimen consisted of two morphologically different tumor nodules. When microdissected and sequenced separately, one nodule was positive for BRAF (v-raf murine sarcoma viral oncogene homolog B1 V600E and the other nodule was wild type at the BRAF codon 600. These examples demonstrate that it is necessary to microdissect morphologically different tumor components for pyrosequencing.

  10. Multiple rectal carcinoid tumors in monozygotic twins.

    Science.gov (United States)

    Doi, Momoko; Ikawa, Osamu; Taniguchi, Hiroki; Kawamura, Takuji; Katsura, Kanade

    2016-08-01

    We report multiple rectal carcinoid tumors in monozygotic twins who, respectively, had 42 and 36 carcinoid tumors in the lower rectum. This is the first report about carcinoid tumors in monozygotic twins. Both twins developed a similar number of rectal carcinoids with a similar distribution. Investigation of their genetic background may provide information about the origin of these tumors.

  11. 9 CFR 381.87 - Tumors.

    Science.gov (United States)

    2010-01-01

    ... 9 Animals and Animal Products 2 2010-01-01 2010-01-01 false Tumors. 381.87 Section 381.87 Animals... § 381.87 Tumors. Any organ or other part of a carcass which is affected by a tumor shall be condemned... by the size, position, or nature of the tumor, the whole carcass shall be condemned. ...

  12. Tumor suppressor molecules and methods of use

    Science.gov (United States)

    Welch, Peter J.; Barber, Jack R.

    2004-09-07

    The invention provides substantially pure tumor suppressor nucleic acid molecules and tumor suppressor polypeptides. The invention also provides hairpin ribozymes and antibodies selective for these tumor suppressor molecules. Also provided are methods of detecting a neoplastic cell in a sample using detectable agents specific for the tumor suppressor nucleic acids and polypeptides.

  13. Kidney Tumors | Office of Cancer Genomics

    Science.gov (United States)

    Pediatric kidney tumors fall into four primary categories: Wilms tumors (~85% of all cases), clear cell sarcomas of the kidney (~5%), congenital mesoblastic nephromas (~4%), and rhabdoid tumors of the kidney (~3%). The TARGET initiative is investigating three of these tumor types.

  14. Neuroendocrine Tumors of the Lung

    Energy Technology Data Exchange (ETDEWEB)

    Fisseler-Eckhoff, Annette, E-mail: Annette.Fisseler-Eckhoff@hsk-wiesbaden.de; Demes, Melanie [Department of Pathology und Cytology, Dr. Horst-Schmidt-Kliniken (HSK), Wiesbaden 65199 (Germany)

    2012-07-31

    Neuroendocrine tumors may develop throughout the human body with the majority being found in the gastrointestinal tract and bronchopulmonary system. Neuroendocrine tumors are classified according to the grade of biological aggressiveness (G1–G3) and the extent of differentiation (well-differentiated/poorly-differentiated). The well-differentiated neoplasms comprise typical (G1) and atypical (G2) carcinoids. Large cell neuroendocrine carcinomas as well as small cell carcinomas (G3) are poorly-differentiated. The identification and differentiation of atypical from typical carcinoids or large cell neuroendocrine carcinomas and small cell carcinomas is essential for treatment options and prognosis. Pulmonary neuroendocrine tumors are characterized according to the proportion of necrosis, the mitotic activity, palisading, rosette-like structure, trabecular pattern and organoid nesting. The given information about the histopathological assessment, classification, prognosis, genetic aberration as well as treatment options of pulmonary neuroendocrine tumors are based on own experiences and reviewing the current literature available. Most disagreements among the classification of neuroendocrine tumor entities exist in the identification of typical versus atypical carcinoids, atypical versus large cell neuroendocrine carcinomas and large cell neuroendocrine carcinomas versus small cell carcinomas. Additionally, the classification is restricted in terms of limited specificity of immunohistochemical markers and possible artifacts in small biopsies which can be compressed in cytological specimens. Until now, pulmonary neuroendocrine tumors have been increasing in incidence. As compared to NSCLCs, only little research has been done with respect to new molecular targets as well as improving the classification and differential diagnosis of neuroendocrine tumors of the lung.

  15. Focus on treatment of lung carcinoid tumor

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    Noel-Savina E

    2013-10-01

    Full Text Available Elise Noel-Savina,1 Renaud Descourt2 1Pulmonary Service, 2Thoracic Oncology Service, Hospital de la Cavale Blanche, CHU – Brest, Brest, France Abstract: Bronchial typical carcinoid tumors are neuroendocrine bronchopulmonary tumors with a low-grade malignancy, and an atypical carcinoid is an intermediate form of these tumors. There is a lack of knowledge on the optimal treatment for these tumors. The surgical treatment of choice consists of a lobectomy supplemented by dissection. The benefit of chemotherapy and radiotherapy is unclear. Targeted therapy could be used in this condition, but there is a lack of research recommending it. Keywords: carcinoid tumor, neuroendocrine tumor, bronchopulmonary tumor, treatment

  16. Mediastinal tumors. Report of 29 operated patients.

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    Antonio Ríos Rodríguez

    2008-08-01

    Full Text Available Background: Surgery is the unique possibility of cure of survival with life quality for patients with tumor or mediastinal mass. Objective: To describe the results of surgical procedures in patients with mediastinal tumor. Methods: Descriptive, retrospective study of 29 patients operated after being diagnosed with mediastinal tumor in a period of 17 years (1986-2002. Studied variables were: tumor´s locus in the mediastinum, tumor nature, histological diagnosis and strategies regarding surgical approach. Findings: There was a prevalence of benign tumors (81,8% and the most frequent locus was anterior mediastinum. Conclusions: These findings agree with previous studies, mainly regarding tumors´ locus and nature.

  17. Glomus tumor of penis- A rare case

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    Madhuri S. Kate

    2013-01-01

    Full Text Available Glomus tumors are rare, painful, and benign soft tissue tumors of the skin arising from the glomus body, an arteriovenous thermoregulatory structure. This lesion is usually found on the extremities. Glomus tumor involving the external genital organs including penis is extremely rare. Until now, only few cases have been reported in the available literature. A 22 year old male patient presented with a painful nodule over the penis. The pathologic diagnosis was glomus tumor of the glans penis. We report herewith a case of glomus tumor of penis diagnosed on histopathologic examination. Glomus tumor, being a benign neoplasm, complete extirpation of the glomus tumor is the treatment of choice.

  18. The ING tumor suppressor genes: status in human tumors.

    Science.gov (United States)

    Guérillon, Claire; Bigot, Nicolas; Pedeux, Rémy

    2014-04-01

    ING genes (ING1-5) were identified has tumor suppressor genes. ING proteins are characterized as Type II TSGs since they are involved in the control of cell proliferation, apoptosis and senescence. They may also function as Type I TSGs since they are also involved in DNA replication and repair. Most studies have reported that they are frequently lost in human tumors and epigenetic mechanisms or misregulation of their transcription may be involved. Recently, studies have described that this loss may be caused by microRNA inhibition. Here, we summarize the current knowledge on ING functions, their involvement in tumor suppression and, in order to give a full assessment of the current knowledge, we review all the studies that have examined ING status in human cancers.

  19. Testicular tumors with tumor thrombosis within the inferior vena cava: Two case reports

    Energy Technology Data Exchange (ETDEWEB)

    Park, Ji Eun; Moon, Sung Kyoung; Lim, Joo Won; Park, Seong Jin [Kyung Hee Univ. Hospital/College of Medicine/Kyung Hee Univ. Seoul (Korea, Republic of)

    2012-09-15

    Testicular tumors are almost all malignant tumors and can develop in younger age groups. Testicular tumors are mostly curable, with reported cases of tumor thrombosis within the inferior vena cava being rare. Two patients, aged 35 years and 37 years old complaining of testicular pain and lower abdominal pain were diagnosed with testicular tumors by ultrasound. In addition, tumor thrombus of the inferior vena cava was diagnosed concomitantly at the time of the diagnosis by computed tomography and magnetic resonance imaging. Here, we report testicular tumors accompanied by tumor thrombus, which is an extremely rare finding, with limited reports available. Pathologic diagnoses were seminoma and mixed germ cell tumors, respectively.

  20. Recurrence of Solid Pseudopapillary Tumor: A Rare Pancreatic Tumor

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    Chandra Punch

    2016-01-01

    Full Text Available Solid pseudopapillary tumor of the pancreas (SPTP is a rare disease of young females that does not usually recur after resection. Here we report a case of an elderly female with history of SPTP ten years ago who presented with anorexia and a palpable left lower quadrant abdominal mass. Imaging revealed metastatic disease and US-guided biopsy of the liver confirmed the diagnosis of SPTP. Due to her advanced age and comorbidities, she elected to undergo hospice care. The objective of this case report is to increase awareness of this tumor and its possibility of recurrence, necessitating further guidelines for follow-up.

  1. Circulating Fibronectin Controls Tumor Growth

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    Anja von Au

    2013-08-01

    Full Text Available Fibronectin is ubiquitously expressed in the extracellular matrix, and experimental evidence has shown that it modulates blood vessel formation. The relative contribution of local and circulating fibronectin to blood vessel formation in vivo remains unknown despite evidence for unexpected roles of circulating fibronectin in various diseases. Using transgenic mouse models, we established that circulating fibronectin facilitates the growth of bone metastases by enhancing blood vessel formation and maturation. This effect is more relevant than that of fibronectin produced by endothelial cells and pericytes, which only exert a small additive effect on vessel maturation. Circulating fibronectin enhances its local production in tumors through a positive feedback loop and increases the amount of vascular endothelial growth factor (VEGF retained in the matrix. Both fibronectin and VEGF then cooperate to stimulate blood vessel formation. Fibronectin content in the tumor correlates with the number of blood vessels and tumor growth in the mouse models. Consistent with these results, examination of three separate arrays from patients with breast and prostate cancers revealed that a high staining intensity for fibronectin in tumors is associated with increased mortality. These results establish that circulating fibronectin modulates blood vessel formation and tumor growth by modifying the amount of and the response to VEGF. Furthermore, determination of the fibronectin content can serve as a prognostic biomarker for breast and prostate cancers and possibly other cancers.

  2. Tumor ablation with irreversible electroporation.

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    Bassim Al-Sakere

    Full Text Available We report the first successful use of irreversible electroporation for the minimally invasive treatment of aggressive cutaneous tumors implanted in mice. Irreversible electroporation is a newly developed non-thermal tissue ablation technique in which certain short duration electrical fields are used to permanently permeabilize the cell membrane, presumably through the formation of nanoscale defects in the cell membrane. Mathematical models of the electrical and thermal fields that develop during the application of the pulses were used to design an efficient treatment protocol with minimal heating of the tissue. Tumor regression was confirmed by histological studies which also revealed that it occurred as a direct result of irreversible cell membrane permeabilization. Parametric studies show that the successful outcome of the procedure is related to the applied electric field strength, the total pulse duration as well as the temporal mode of delivery of the pulses. Our best results were obtained using plate electrodes to deliver across the tumor 80 pulses of 100 micros at 0.3 Hz with an electrical field magnitude of 2500 V/cm. These conditions induced complete regression in 12 out of 13 treated tumors, (92%, in the absence of tissue heating. Irreversible electroporation is thus a new effective modality for non-thermal tumor ablation.

  3. Disseminated typical bronchial carcinoid tumor

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    Novković Dobrivoje

    2013-01-01

    Full Text Available Introduction. Bronchial carcinoids belong to a rare type of lung tumors. If they do not expose outstanding neuroendocrine activity, they develop without clearly visible symptoms. They are often detected during a routine examination. According to their clinical pathological features, they are divided into typical and atypical tumors. Typical bronchial carcinoids metastasize to distant organs very rarely. Localized forms are effectively treated by surgery. The methods of conservative treatment should be applied in other cases. Case report. We presented a 65-year-old patient with carcinoid lung tumor detected by a routine examination. Additional analysis (chest X-ray, computed tomography of the chest, ultrasound of the abdomen, skeletal scintigraphy, bronhoscopy, histopathological analysis of the bioptate of bronchial tumor, as well as bronchial brushing cytology and immunohistochemical staining performed with markers specific for neuroendocrine tumor proved a morphologically typical lung carcinoid with dissemination to the liver and skeletal system, which is very rarely found in typical carcinoids. Conclusion. The presented case with carcinoid used to be showed morphological and pathohistological characteristics of typical bronchial carcinoid. With its metastasis to the liver and skeletal system it demonstrated unusual clinical course that used to be considered as rare phenomenon. Due to its frequent asymptomatic course and varied manifestation, bronchial carcinoid could be considered as a diagnostic challenge requiring a multidisciplinary approach.

  4. Max Wilms and his tumor.

    Science.gov (United States)

    Raffensperger, John

    2015-02-01

    The most common cancer of the kidney in infants and children is named for Max Wilms, a German surgeon. How did this eponym come about? There were excellent reviews of this lesion before Wilms, a second year surgical assistant, published "Die Mischgeschwulste Der Niere" or The Mixed Tumors of the Kidney in 1899. At thirty two years of age, he demonstrated a masterful knowledge of pathology and embryology. Wilms' career was cut short when he became septic after operating on a prisoner of war during WWI. The survival rate for children with Wilms tumor was dismal until William Ladd, at the Boston Children's hospital introduced rational surgical treatment. By mid century, Robert Gross achieved a 47% survival rate with surgery combined with postoperative radiation. Sydney Farber treated Wilms tumors with Actinomycin-d and opened the door to cancer chemotherapy. With protocols developed by the National Wilms Tumor Study Group, the survival rate of children with Wilms tumors reached 90% by the end of the twentieth century.

  5. Awake craniotomy for tumor resection

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    Mohammadali Attari

    2013-01-01

    Full Text Available Surgical treatment of brain tumors, especially those located in the eloquent areas such as anterior temporal, frontal lobes, language, memory areas, and near the motor cortex causes high risk of eloquent impairment. Awake craniotomy displays major rule for maximum resection of the tumor with minimum functional impairment of the Central Nervous System. These case reports discuss the use of awake craniotomy during the brain surgery in Alzahra Hospital, Isfahan, Iran. A 56-year-old woman with left-sided body hypoesthesia since last 3 months and a 25-year-old with severe headache of 1 month duration were operated under craniotomy for brain tumors resection. An awake craniotomy was planned to allow maximum tumor intraoperative testing for resection and neurologic morbidity avoidance. The method of anesthesia should offer sufficient analgesia, hemodynamic stability, sedation, respiratory function, and also awake and cooperative patient for different neurological test. Airway management is the most important part of anesthesia during awake craniotomy. Tumor surgery with awake craniotomy is a safe technique that allows maximal resection of lesions in close relationship to eloquent cortex and has a low risk of neurological deficit.

  6. Askin's Tumor: A Dual Case Study

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    Bikash Shrestha

    2011-01-01

    Full Text Available Askin's tumor is a rare tumor arising from the chest wall. It is a subset of Ewing sarcoma characterized histologically by the presence of small round blue cells. It is a highly malignant tumor with guarded prognosis, which is dependent upon the extension of tumor at the time of diagnosis. A dual paper of Askin's tumors in young boys is being presented here.

  7. Value of diffusion weighted MRI in differentiating benign from malignant bony tumors and tumor like lesions

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    Samir Zaki Kotb

    2014-06-01

    Conclusion: DWI has been proven to be highly useful in the differentiation of benign, malignant bone tumors and tumor like bony lesions. Measurement of ADC values improves the accuracy of the diagnosis of bone tumors and tumor like lesions. Moreover, measurement of ADC values can be used in the follow up of tumors and their response to therapy.

  8. Cellular Potts modeling of tumor growth, tumor invasion and tumor evolution

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    András eSzabó

    2013-04-01

    Full Text Available Despite a growing wealth of available molecular data, the growth of tumors, invasion of tumors into healthy tissue, and response of tumors to therapies are still poorly understood. Although genetic mutations are in general the first step in the development of a cancer, for the mutated cell to persist in a tissue, it must compete against the other, healthy or diseased cells, for example by becoming more motile, adhesive, or multiplying faster. Thus, the cellular phenotype determines the success of a cancer cell in competition with its neighbors, irrespective of the genetic mutations or physiological alterations that gave rise to the altered phenotype.What phenotypes can make a cell successful in an environment of healthy and cancerous cells, and how? A widely-used tool for getting more insight into that question is cell-based modeling. Cell based models constitute a class of computational, agent-based models that mimic biophysical and molecular interactions between cells. One of the most widely used cell-based modeling formalisms is the cellular Potts model (CPM, a lattice-based, multi particle cell-based modeling approach. The CPM has become a popular and accessible method for modeling mechanisms of multicellular processes including cell sorting, gastrulation,or angiogenesis. The CPM accounts for biophysical cellular properties, including cell proliferation, cell motility, and cell adhesion, which play a key role in cancer. Multiscale models are constructed by extending the agents with intracellular processes including metabolism, growth, and signaling. Here we review the use of the CPM for modeling tumor growth, tumor invasion, and tumor progression. We argue that the accessibility and flexibility of the CPM, and its accurate, yet coarse-grained and computationally efficient representation of cell- and tissue biophysics, make the CPM the method of choice for modeling cellular processesin tumor development.

  9. Cellular potts modeling of tumor growth, tumor invasion, and tumor evolution.

    Science.gov (United States)

    Szabó, András; Merks, Roeland M H

    2013-01-01

    Despite a growing wealth of available molecular data, the growth of tumors, invasion of tumors into healthy tissue, and response of tumors to therapies are still poorly understood. Although genetic mutations are in general the first step in the development of a cancer, for the mutated cell to persist in a tissue, it must compete against the other, healthy or diseased cells, for example by becoming more motile, adhesive, or multiplying faster. Thus, the cellular phenotype determines the success of a cancer cell in competition with its neighbors, irrespective of the genetic mutations or physiological alterations that gave rise to the altered phenotype. What phenotypes can make a cell "successful" in an environment of healthy and cancerous cells, and how? A widely used tool for getting more insight into that question is cell-based modeling. Cell-based models constitute a class of computational, agent-based models that mimic biophysical and molecular interactions between cells. One of the most widely used cell-based modeling formalisms is the cellular Potts model (CPM), a lattice-based, multi particle cell-based modeling approach. The CPM has become a popular and accessible method for modeling mechanisms of multicellular processes including cell sorting, gastrulation, or angiogenesis. The CPM accounts for biophysical cellular properties, including cell proliferation, cell motility, and cell adhesion, which play a key role in cancer. Multiscale models are constructed by extending the agents with intracellular processes including metabolism, growth, and signaling. Here we review the use of the CPM for modeling tumor growth, tumor invasion, and tumor progression. We argue that the accessibility and flexibility of the CPM, and its accurate, yet coarse-grained and computationally efficient representation of cell and tissue biophysics, make the CPM the method of choice for modeling cellular processes in tumor development.

  10. Tumors of the orbit; Orbitatumoren

    Energy Technology Data Exchange (ETDEWEB)

    Mueller-Forell, Wibke [Klinikum der Univ. Mainz (Germany). Inst. fuer Neuroradiologie

    2008-09-15

    This short review will present an overview not only of the findings in imaging, but the main clinical symptoms of the different orbital space occupying lesions, (not only different tumors, but even tumor-like lesions), as those should be included in differential diagnostic consideration. This should lead to a targeted diagnostic procedure in the individual patient. Basic knowledge of orbital anatomy is as important as the knowledge of the pros and contra's of computed tomography (CT) and magnetic resonance imaging (MRI), which should influence the examination protocol. Imaging findings of the most important tumors/lesions of the globe, the intraconal and extraconal space as well as of the optic nerve are presented with different examples (without any demand of completeness). (orig.)

  11. Immunological treatment of liver tumors

    Institute of Scientific and Technical Information of China (English)

    Maurizio Chiriva-Internati; Fabio Grizzi; Cynthia A Jumper; Everardo Cobos; Paul L Hermonat; Eldo E Frezza

    2005-01-01

    Although multiple options for the treatment of liver tumors have often been described in the past, including liver resection, radiofrequency ablation with or without hepatic pump insertion, laparoscopic liver resection and the use of chemotherapy, the potential of immunotherapy and gene manipulation is still largely unexplored.Immunological therapy by gene manipulation is based on the interaction between virus-based gene delivery systems and dendritic cells. Using viruses as vectors, it is possible to transduce dendritic cells with genes encoding tumor-associated antigens, thus inducing strong humoral and cellular immunity against the antigens themselves.Both chemotherapy and radiation therapy have the disadvantage of destroying healthy cells, thus causing severe side-effects. We need more precisely targeted therapies capable of killing cancer cells while sparing healthy cells. Our goal is to establish a new treatment for solid liver tumors based on the concept of cytoreduction,and propose an innovative algorithm.

  12. Notch Signaling in Neuroendocrine Tumors

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    Judy Sue Crabtree

    2016-04-01

    Full Text Available Carcinoids and neuroendocrine tumors (NETs are a heterogeneous group of tumors that arise from the neuroendocrine cells of the GI tract, endocrine pancreas and the respiratory system. NETs remain significantly understudied with respect to molecular mechanisms of pathogenesis, particularly the role of cell fate signaling systems like Notch. The abundance of literature on the Notch pathway is a testament to its complexity in different cellular environments. Notch receptors can function as oncogenes in some contexts, and tumor suppressors in others. The genetic heterogeneity of NETs suggests that to fully understand the roles and the potential therapeutic implications of Notch signaling in NETs, a comprehensive analysis of Notch expression patterns and potential roles across all NET subtypes is required.

  13. Malignant renal tumors in children

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    Justin Scott Lee

    2015-05-01

    Full Text Available Renal malignancies are common in children. While the majority of malignant renal masses are secondary to Wilms tumor, it can be challenging to distinguish from more aggressive renal masses. For suspicious renal lesions, it is crucial to ensure prompt diagnosis in order to select the appropriate surgical procedure and treatment. This review article will discuss the common differential diagnosis that can be encountered when evaluating a suspicious renal mass in the pediatric population. This includes clear cell sarcoma of the kidney, malignant rhabdoid tumor, renal medullary carcinoma and lymphoma. 

  14. Tumores Epiteliales Laringo-Traqueales.

    OpenAIRE

    Muñoz Cariñanos, Antonio

    2017-01-01

    Se comienza con un estudio de la historia de este tipo de tumores en la medicina, la incidencia en la población y su comparación en España con otros países del área europea, y también en las provincias de España. Estudio de los tipos histológicos de los diversos tumores que son causa del trabajo de esta tesis.   Un apartado importante de la misma es el estudio de los conocimientos actuales sobre la patogénesis del c&aacu...

  15. Contemporary treatment of renal tumors

    DEFF Research Database (Denmark)

    Nisen, Harry; Järvinen, Petrus; Fovaeus, Magnus

    2017-01-01

    questions on renal tumor management and surgical education was designed and sent to 91 institutions performing renal tumor surgery in 2015. The response rate was 68% (62 hospitals), including 28 academic, 25 central and nine district hospitals. Hospital volume was defined as low (LVH: ...% thermoablations. For RN and PN, the percentages of open, laparoscopic and robotic approaches were 47%, 40%, 13% and 47%, 20%, 33%, respectively. The mean complication rate (Clavien–Dindo 3–5) was 4.9%, and 30 day mortality (TDM) was 0.5%. The median length of hospital stay was 4 days. Training with a simulator...

  16. Small intestinal tophus mimicking tumor

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    Pragya Katoch

    2014-03-01

    Full Text Available A 72 year old male with hypertension, diabetes mellitus type 2 and previous gouty arthritis presented with weight loss, nausea, and vomiting. Ultrasound and CT scanning of the abdomen revealed a circumscribed tumor mass of the jejunum, 3.7 cm in diameter. Microscopic examination of the resected jejunum revealed the tumor to be a gouty tophus. To the best of our knowledge, three cases of tophi in the large intestine have previously been reported but none in the small intestine.

  17. Laparoscopic Nephrectomy for Wilms Tumor

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    Andolfi C

    2014-02-01

    Full Text Available Wilms tumor is the most frequent primary renal malignancy in children. The surgical resection is traditionally performed through laparotomy. The advent of laparoscopic surgery for benign renal lesions has led the surgeon to use a minimal invasive approach for the nephroblastoma. We describe a 9-months-old girl who presented with a left renal mass. A laparoscopic resection of the tumor with left radical nephroureterectomy was performed. The specimens were removed in an endoscopic bag through a low suprapubic incision. The patient had an uncomplicated recovery. Minimally invasive techniques may be considered for resection of nephroblastoma if oncologic principles are carefully followed.

  18. Ectomesenchymal chondromyxoid tumor of tongue

    Directory of Open Access Journals (Sweden)

    Shan-Yin Tsai

    2012-01-01

    Full Text Available Ectomesenchymal chondromyxoid tumor (ECMT is a rare entity of the dorsal tongue first described in 1995. Herein, we report a rare case of lingual ECMT in a 41-year-old man. Patient presented with an asymptomatic, small nodule (0.5 cm in diameter in the anterior tongue. The pathological findings showed uni-lobular proliferation of fusiform cells, arranged in net-like sheets or swirls, in a chondromyxoid background. The tumor cells were immunoreactive for S-100 and glial fibrillary acidic protein (GFAP, but negative for epithelial markers. Familiarity with this entity helps pathologists make a correct diagnosis.

  19. Unarmed, tumor-specific monoclonal antibody effectively treats brain tumors

    Science.gov (United States)

    Sampson, John H.; Crotty, Laura E.; Lee, Samson; Archer, Gary E.; Ashley, David M.; Wikstrand, Carol J.; Hale, Laura P.; Small, Clayton; Dranoff, Glenn; Friedman, Allan H.; Friedman, Henry S.; Bigner, Darell D.

    2000-01-01

    The epidermal growth factor receptor (EGFR) is often amplified and rearranged structurally in tumors of the brain, breast, lung, and ovary. The most common mutation, EGFRvIII, is characterized by an in-frame deletion of 801 base pairs, resulting in the generation of a novel tumor-specific epitope at the fusion junction. A murine homologue of the human EGFRvIII mutation was created, and an IgG2a murine mAb, Y10, was generated that recognizes the human and murine equivalents of this tumor-specific antigen. In vitro, Y10 was found to inhibit DNA synthesis and cellular proliferation and to induce autonomous, complement-mediated, and antibodydependent cell-mediated cytotoxicity. Systemic treatment with i.p. Y10 of s.c. B16 melanomas transfected to express stably the murine EGFRvIII led to long-term survival in all mice treated (n = 20; P < 0.001). Similar therapy with i.p. Y10 failed to increase median survival of mice with EGFRvIII-expressing B16 melanomas in the brain; however, treatment with a single intratumoral injection of Y10 increased median survival by an average 286%, with 26% long-term survivors (n = 117; P < 0.001). The mechanism of action of Y10 in vivo was shown to be independent of complement, granulocytes, natural killer cells, and T lymphocytes through in vivo complement and cell subset depletions. Treatment with Y10 in Fc receptor knockout mice demonstrated the mechanism of Y10 to be Fc receptor-dependent. These data indicate that an unarmed, tumor-specific mAb may be an effective immunotherapy against human tumors and potentially other pathologic processes in the “immunologically privileged” central nervous system. PMID:10852962

  20. Molecular Profiling of Odontogenic Tumors - Pilot Study

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    Gültekin Sibel Elif

    2017-07-01

    Full Text Available Background/Aim: In the pathogenesis of odontogenic tumors which arise from the rests of the dental apparatus in the jaw, several molecular pathways have been shown to play critical roles such as genetic alterations in the hedgehog, BRAF/Ras/MAPK, epidermal growth factor receptor. Next generation genomic sequencing has identified gene mutations in many different tumors. Materials and Methods: Here we report four types of odontogenic tumor including six cases in which five had mutation according to next generation sequencing analysis from archival paraffin blocks that diagnosed previously as ameloblastoma (solid, amloblastoma (unicystic-mural, ameloblastic fibroma, squamous odontogenic tumor, and adenomatoid odontogenic tumor. Results: All ameloblastomatic tumors were shown BRAF mutation and adenomatoid odontogenic tumors were KRAS mutation. Conclusion: This evidence may highlight the poorly understood pathogenesis of odontogenic tumors. Further comparisons need to be made with other benign and malignant odontogenic tumors so that unique odontogenic features may be found.

  1. Localization of spinal tumors by MRI

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    Takahashi, Mutsumasa; Sakamoto, Yuji; Kojima, Ryutaro; Yamashita, Yasuyuki; Bussaka, Hiromasa

    1989-02-01

    Exact localization of the spinal tumors is particularly important for differential diagnosis and surgery. Therefore, it was attempted to evauate the diagnostic accuracy of MRI in localizing the spinal tumors exactly. Nineteen cases of spinal cord tumors, being localized in the intradural extramedullary, extradural and both intradural and extradural spaces, were studied with MRI. Intradural extramedullary tumors showed small CSF spaces just below and above the tumor which were demonstrated as CSF intensity on T1 and T2 weighted images. Although extradural tumors did not show CSF spaces, there was extradural sign or dural density between the tumor and the spinal cord. Intradural and extradural tumors were outlined as having both characteristics. Signal intensities of the spinal tumors were not characteristic for specific histology. Gd-DTPA was quite useful for accurate localization of the tumors. In comparison with myelography, MRI was superior to myelography in extradural tumors and equally useful for intradural and extradural tumors, but it was less diagnostic in intradural extramedullary tumors. In general, MRI was quite useful in localizing the spinal tumors exactly and the accuracy of MRI was quite high. In the near future this technique will replace myelography and other radiologic methods.

  2. Microenvironments Dictating Tumor Cell Dormancy

    Science.gov (United States)

    Bragado, Paloma; Sosa, Maria Soledad; Keely, Patricia; Condeelis, John

    2012-01-01

    The mechanisms driving dormancy of disseminated tumor cells (DTCs) remain largely unknown. Here, we discuss experimental evidence and theoretical frameworks that support three potential scenarios contributing to tumor cell dormancy. The first scenario proposes that DTCs from invasive cancers activate stress signals in response to the dissemination process and/or a growth suppressive target organ microenvironment inducing dormancy. The second scenario asks whether therapy and/or micro-environmental stress conditions (e.g. hypoxia) acting on primary tumor cells carrying specific gene signatures prime new DTCs to enter dormancy in a matching target organ microenvironment that can also control the timing of DTC dormancy. The third and final scenario proposes that early dissemination contributes a population of DTCs that are unfit for immediate expansion and survive mostly in an arrested state well after primary tumor surgery, until genetic and/or epigenetic mechanisms activate their proliferation. We propose that DTC dormancy is ultimately a survival strategy that when targeted will eradicate dormant DTCs preventing metastasis. For these non-mutually exclusive scenarios we review experimental and clinical evidence in their support. PMID:22527492

  3. Tumor Immunotargeting Using Innovative Radionuclides

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    Françoise Kraeber-Bodéré

    2015-02-01

    Full Text Available This paper reviews some aspects and recent developments in the use of antibodies to target radionuclides for tumor imaging and therapy. While radiolabeled antibodies have been considered for many years in this context, only a few have reached the level of routine clinical use. However, alternative radionuclides, with more appropriate physical properties, such as lutetium-177 or copper-67, as well as alpha-emitting radionuclides, including astatine-211, bismuth-213, actinium-225, and others are currently reviving hopes in cancer treatments, both in hematological diseases and solid tumors. At the same time, PET imaging, with short-lived radionuclides, such as gallium-68, fluorine-18 or copper-64, or long half-life ones, particularly iodine-124 and zirconium-89 now offers new perspectives in immuno-specific phenotype tumor imaging. New antibody analogues and pretargeting strategies have also considerably improved the performances of tumor immunotargeting and completely renewed the interest in these approaches for imaging and therapy by providing theranostics, companion diagnostics and news tools to make personalized medicine a reality.

  4. Ghrelin and gastrointestinal stromal tumors

    Science.gov (United States)

    Zhu, Chang-Zhen; Liu, Dong; Kang, Wei-Ming; Yu, Jian-Chun; Ma, Zhi-Qiang; Ye, Xin; Li, Kang

    2017-01-01

    Ghrelin, as a kind of multifunctional protein polypeptide, is mainly produced in the fundus of the stomach and can promote occurrence and development of many tumors, including gastrointestinal tumors, which has been proved by the relevant researches. Most gastrointestinal stromal tumors (GISTs, about 80%), as the most common mesenchymal tumor, also develop in the fundus. Scientific research has confirmed that ghrelin, its receptors and mRNA respectively can be found in GISTs, which demonstrated the existence of a ghrelin autocrine/paracrine loop in GIST tissues. However, no reports to date have specified the mechanism whether ghrelin can promote the occurrence and development of GISTs. Studies of pulmonary artery endothelial cells in a low-oxygen environment and cardiac muscle cells in an ischemic environment have shown that ghrelin can activate the phosphatidylinositol 3-kinase/AKT/mammalian target of rapamycin (PI3K/AKT/mTOR) signaling pathway. Moreover, some studies of GISTs have confirmed that activation of the PI3K/AKT/mTOR pathway can indeed promote the growth and progression of GISTs. Whether ghrelin is involved in the development or progression of GISTs through certain pathways remains unknown. Can we find a new target for the treatment of GISTs? This review explores and summaries the relationship among ghrelin, the PI3K/AKT/mTOR pathway and the development of GISTs.

  5. [The systematization of APUD tumors].

    Science.gov (United States)

    Liubenov, T; Terziev, I

    1995-01-01

    The mechanisms involved in neuroendocrine transmission of peptides, underlying the so-called classification of multiple endocrine neoplasms (MEN), are described. Three cases from the clinical practice are followed up where facilitation of the diagnosis and the results of treatment are related to the tumor markers' values.

  6. Mechanisms of tumor cell necrosis.

    Science.gov (United States)

    Proskuryakov, Sergey Y; Gabai, Vladimir L

    2010-01-01

    Until recently, necrosis, unlike apoptosis, was considered as passive and unregulated form of cell death. However, during the last decade a number of experimental data demonstrated that, except under extreme conditions, necrosis may be a well-regulated process activated by rather specific physiological and pathological stimuli. In this review, we consider mechanisms and the role of necrosis in tumor cells. It became recently clear that the major player in necrotic cascade is a protein kinase RIP1, which can be activated by number of stumuli including TNF, TRAIL, and LPS, oxidative stress, or DNA damage (via poly-ADP-ribose polymerase). RIP1 kinase directly (or indirectly via another kinase JNK) transduces signal to mitochondria and causes specific damage (mitochondrial permeability transition). Mitochondrial collapse activates various proteases (e.g., calpains, cathepsin) and phospholipases, and eventually leads to plasma membrane destruction, a hallmark of necrotic cell death. Necrosis, in contrast to apoptosis, usually evokes powerful inflammatory response, which may participate in tumor regression during anticancer therapy. On the other hand, excessive spontaneous necrosis during tumor development may lead to more aggressive tumors due to stimulatory role of necrosis-induced inflammation on their growth.

  7. Neuroradiology of primitive neuroectodermal tumors

    Energy Technology Data Exchange (ETDEWEB)

    Hinshaw, D.B. Jr.; Thompson, J.R.; Hasso, A.N.; Ashwal, S.

    1983-05-01

    The neuroradiological findings in four cases of primitive neuroectodermal tumor of the cerebrum are described. These highly malignant neoplasms of childhood present as large, enhancing cerebral masses with extensive neovascularity. Cerebrospinal fluid seeding is common and distant extraneural metastases may occur.

  8. TUMORES CEREBRALES ASOCIADOS A EPILEPSIA

    Directory of Open Access Journals (Sweden)

    Dr. Manuel G. Campos

    2017-05-01

    La Resonancia Magnética de cerebro es mandatoria en el estudio de todo paciente con epilepsia, para detectar lesiones estructurales, especialmente en epilepsia focal. Alrededor del 30% de los pacientes operados de epilepsia refractaria presentan tumores. En estos casos el control de crisis post-operatorio llega hasta un 70% en el seguimiento a largo plazo.

  9. Leptomeningeal Metastases in Solid Tumors

    Directory of Open Access Journals (Sweden)

    Marija Karakolevska-Ilova

    2014-12-01

    CONCLUSION: The prognostic factors associated with survival in patients with LM metastasis are not certain as well as right treatment for these patients which is still a big challenge. Due to the lack of randomized studies and especially of studies referring to one specific primary tumor, there is currently no generally accepted standard of care in the treatment of LM metastases.

  10. Evolution of Avian Tumor Viruses

    Science.gov (United States)

    Virus-induced neoplastic diseases of poultry, namely Marek’s disease (MD), induced by a herpesvirus, and the avian leukosis and reticuloendotheliosis induced by retroviruses, can cause significant economic losses from tumor mortality as well as poor performance. Successful control of MD is and has ...

  11. Escape from Tumor Cell Dormancy

    Science.gov (United States)

    2012-10-01

    Pouliot, K. L. Stanley, J. Chia , J. M. Moseley, D. K. Hards and R. L. Anderson: Tumor-specific expression of alphavbeta3 integrin promotes...deep, measured by confocal imaging of microwells filled with 20-mm-diameter fluores - cent beads (Fig. 1). Evaluation of mechanical properties of PEG...

  12. Current immunotherapy for solid tumors.

    Science.gov (United States)

    Barkholt, Lisbeth; Bregni, Marco

    2009-05-01

    Explorative knowledge of cellular and molecular mechanisms of immune function and regulation has provided optimism in developing cancer immunotherapy. However, three decades of experimental and clinical investigations to offer powerful immunotherapeutic strategies against solid tumors, with the possible exception of monoclonal antibody-targeted therapies, have not succeeded in significantly prolonging patient survival. Nonspecific immune approaches, including cytokine-based therapies and allogeneic hematopoietic stem cell transplantation, have so far produced consistent, although limited, results. In this review, we present the developments of cell transfer-based strategies that, in preclinical studies, have demonstrated potential efficacy, but have only established tumor regression in limited numbers of patients. The key to success demands creative combinations of tumor antigens, adjuvance, gene modification and various administration strategies in the development of cell-based therapies together with other cancer-treatment principles, often in a stepwise 'space-rocket-type' approach. Combined efforts of several scientific disciplines, such as tumor biology and immunology, as well as cell and gene research in transplantation, will open new venues. New regulation for clinical trials with advanced therapy medicine products to ensure patient safety will be highlighted.

  13. Surgery of Glomus Jugulare Tumors.

    Science.gov (United States)

    Pareschi, Roberto; Righini, Stefano; Destito, Domenico; Raucci, Aldo Falco; Colombo, Stefano

    2003-08-01

    The treatment of choice for glomus jugulare tumors is still controversial. High rates of morbidity, incomplete resection, and the aggressive behavior of these tumors are the main arguments for advocates of primary radiotherapy. However, constant refinements in skull base techniques have made complete resection of these lesions a realistic goal. The high probability of achieving local control of these tumors by surgery has convinced us to support this option strongly. Between 1993 and 2000 we diagnosed 52 glomus tumors of the temporal bone. Of these patients, only 42 had a class C lesion (glomus jugulare) and were included in this study; 37 of these patients underwent surgery, 10 of whom had intracranial extension of the disease. The overall resection rate was 96 %. Facial nerve function at 1 year was House-Brackmann grade I to II in 52 % of patients and grade III or better in 84 % of patients. Hospitalization was shorter than 14 days in 33 patients (89 %). All patients with pharyngolaryngeal palsy had sufficient compensation at discharge. Twelve vocal chord Teflon injections were performed after surgery to reduce hoarseness and aspiration. No patient died. No relapse was observed (mean follow-up, 4.9 years).

  14. Tumor raro em mediastino: hamartoma

    OpenAIRE

    Roberto Saad Júnior; José Donato de Próspero; Roberto Gonçalves; Jorge Henrique Rivaben; Fabiano Alves Squeff

    2013-01-01

    In the present paper we report a case of mediastinal tumor which we believe has never been reported. Since surgical treatment, the patient presented good evolution. We discuss the presentation, differential diagnosis and therapy. Finally, we stress the importance of the pathologic findings.

  15. Studies on tumor induced angiogenesis.

    Science.gov (United States)

    Ambrus, J L; Ambrus, C M; Forgach, P; Stadler, S; Halpern, J; Sayyid, S; Niswander, P; Toumbis, C

    1992-01-01

    Methods were developed to test angiogenic response to human tumor implants and various biologic agents in the cornea of rabbits and non-human primates (Macaca arctoides). Crude PDGF preparations were found to have significant angiogenic effect. Purified, recombinant PDGF preparations were also effective inhibitors (e.g. pentoxifylline (Px) (which also were found to release PgI2 and t-PA) inhibited human tumor implant induced angiogenesis and reduced spontaneous metastases in 3 transplantable murine tumors (Furth-Columbia Wilms' tumor in Furth-Wistar rats, C-1300 neuroblastoma in A/J mice and HM-Kim mammary carcinoma in Wistar rats) but not in the NIH adenocarcinoma in Balb/c mice. Sodium diethyldithiocarbamate (DDTC), a metal complexing agent with special affinity to copper and anti-thyroid as well as, immune stimulating activity was shown to be anti-angiogenic and to potentiate the effect of Px. The anti-fibrinolytic agents epsilon amino caproic acid (EACA) and tranaxamic acid (t-AMCHA) were anti-angiogenic. DDTC and Px were synergistic from this point of view.

  16. Fever and abdominal tumoral masses

    Directory of Open Access Journals (Sweden)

    Augustin C. Dima

    2016-04-01

    Full Text Available 49 year-old man presented to our clinic for pain in the right hypochondrium, diarrhea, and fever. The clinical examination highlights a tumoral formation in the right side of the abdomen, with firm consistency, poorly defined margins, and present mobility in the deep structures. On biological exams, leukocytosis with neutrophilia, inflammatory syndrome, and hypoalbuminaemia were identified. The first computed tomography exam described parietal thickening of the ascending colon, with infiltrative aspect, and multiple local adenopathies, lomboaortic and interaortocave. Moreover, four nodular liver tumors, with hypodense image in native examination, were identified. The lab tests for infectious diseases were all inconclusives: three hemocultures, three stool samples, and three coproparasitological exams were all negatives. Interdisciplinary examinations, internal medicine and infectious diseases, sustained the diagnosis of colonic neoplasm with peritumoral abscess and liver pseudo-tumoral masses. The colonoscopy did not revealed any bowel lesions relevant for neoplasia. This result as well as the bio-clinical context imposed abstention from surgical intervention. Wide spectrum antibiotics and symptomatic treatment were initiated. But, ten days after hospitalization, the second computed tomography exam showed reduction of the ascending colon wall thickness associated with significant increases of the liver tumors is so revealed. The investigations for other possible etiologies were so continued.

  17. Pediatric liver tumors--a pictorial review.

    Science.gov (United States)

    Jha, Priyanka; Chawla, Soni C; Tavri, Sidhartha; Patel, Chirag; Gooding, Charles; Daldrup-Link, Heike

    2009-01-01

    Hepatic masses constitute about 5-6% of all intra-abdominal masses in children. The majority of liver tumors in children are malignant; these malignant liver tumors constitute the third most common intra-abdominal malignancy in the pediatric age group after Wilms' tumor and neuroblastoma. Only about one third of the liver tumors are benign. A differential diagnosis of liver tumors in children can be obtained based on the age of the child, clinical information (in particular AFP) and imaging characteristics. The purpose of this review is to report typical clinical and imaging characteristics of benign and malignant primary liver tumors in children.

  18. Pediatric liver tumors - a pictorial review

    Energy Technology Data Exchange (ETDEWEB)

    Jha, Priyanka; Tavri, Sidhartha; Patel, Chirag; Gooding, Charles; Daldrup-Link, Heike [University of California, San Francisco, Department of Radiology, San Francisco, CA (United States); Chawla, Soni C. [Department of Radiological Sciences 2D115, Olive View-UCLA Medical Center, Sylmar, CA (United States)

    2009-01-15

    Hepatic masses constitute about 5-6% of all intra-abdominal masses in children. The majority of liver tumors in children are malignant; these malignant liver tumors constitute the third most common intra-abdominal malignancy in the pediatric age group after Wilms' tumor and neuroblastoma. Only about one third of the liver tumors are benign. A differential diagnosis of liver tumors in children can be obtained based on the age of the child, clinical information (in particular AFP) and imaging characteristics. The purpose of this review is to report typical clinical and imaging characteristics of benign and malignant primary liver tumors in children. (orig.)

  19. [Ultrasonographic study of rectal carcinoid tumors].

    Science.gov (United States)

    Nomura, M; Fujita, N; Matsunaga, A; Ando, M; Tominaga, G; Noda, Y; Kobayashi, G; Kimura, K; Yuki, T; Ishida, K; Yago, A; Mochizuki, F; Chonan, A

    1996-11-01

    To compare intraluminal ultrasonographic (ILUS) findings with histological findings of rectal carcinoid tumors, 35 patients with rectal carcinoid tumors were reviewed. The results were as follows: 1) The rectal wall was visualized as a seven- or nine-layer structure by means of ILUS in 81% of the patients. 2) The possibility that the thin hyperechoic third layer above the tumor on ILUS corresponds to the muscularis mucosae and fibrointerstitium above the tumor histologically. 3) In cases with relatively high internal echoes, the amount of fibrointerstitium exceeded that of tumor cells histologically. 4) In cases with nonuniform internal echo patterns, tumor cells were separated by thick fibrointerstitium forming nodular nests.

  20. Surgical management of pediatric brain tumors.

    Science.gov (United States)

    Heuer, Gregory G; Jackson, Eric M; Magge, Suresh N; Storm, Phillip B

    2007-12-01

    Brain tumors are the most common cause of cancer-related death and the second most common form of cancer in pediatric patients. Many of these tumors are treated primarily with surgery, either alone or in combination with radiation or chemotherapy. Recent advances have lead to greater survival and decreased morbidities in childhood brain tumor patients. A full understanding of the biology and primary treatment modalities for the particular tumor are essential for any professional treating these patients, including the neurosurgeon. Each tumor type has features in common with, and unique from, other tumors that need to be understood prior to undertaking a rational treatment plan. This article summarizes some of these features.

  1. Hybrid resection of duodenal tumors.

    Science.gov (United States)

    Poultsides, George A; Pappou, Emmanouil P; Bloom, George Peter; Orlando, Rocco

    2011-09-01

    The aim of this study was to review our experience with the hand-assisted laparoscopic management of duodenal tumors with no or low malignant potential and to compare this approach with published case reports of purely laparoscopic local duodenal resection. Eight cases of hand-assisted laparoscopic local duodenal resection performed from 2000 to 2008 were retrospectively reviewed. Hand-assistance was utilized for complete duodenal mobilization, and local duodenal resection was accomplished extracorporeally through the hand-access incision. Patient and tumor characteristics, operative time, length of stay, and complications were compared with 18 cases of totally laparoscopic local excision of duodenal tumors published since 1997. Patients with ampullary tumors were excluded. Compared with the purely laparoscopic approach, the hand-assisted technique was associated with shorter operative time (179 versus 131 minutes, P=.03) and was more commonly used for lesions located in the third portion of the duodenum (0% versus 37.5%, P=.02). Tumor size (2.9 cm versus 3.2 cm, P=.61) and length of hospital stay (5.9 versus 5.9 days, P=.96) were similar between the two groups. The rate of complications was also comparable (0% versus 12.5%, P=.31); 1 of 8 patients in the hand-assisted group developed an incisional hernia at the hand-access site. Hand-assisted laparoscopic local duodenal resection is a feasible, safe, and effective alternative to the totally laparoscopic approach. In addition to being associated with comparable length of hospital stay, hand-assistance can shorten operative time by facilitating duodenal mobilization as well as extracorporeal duodenal resection and closure.

  2. Palifosfamide in Treating Patients With Recurrent Germ Cell Tumors

    Science.gov (United States)

    2015-06-11

    Adult Central Nervous System Germ Cell Tumor; Adult Teratoma; Malignant Extragonadal Germ Cell Tumor; Malignant Extragonadal Non-Seminomatous Germ Cell Tumor; Extragonadal Seminoma; Recurrent Malignant Testicular Germ Cell Tumor; Recurrent Ovarian Germ Cell Tumor; Stage IV Extragonadal Non-Seminomatous Germ Cell Tumor; Stage IV Extragonadal Seminoma; Stage IV Ovarian Germ Cell Tumor

  3. Pituitary tumor apoplexy Apoplexia em tumor hipofisário

    Directory of Open Access Journals (Sweden)

    Claudia V. Chang

    2009-06-01

    Full Text Available Pituitary tumor apoplexy is a medical emergency due to acute infarction or hemorrhage in the pituitary gland. In this review, the authors discuss the sellar anatomy, the pituitary gland and adenomas' vascularization and the general aspects of the syndrome such as its ethiopatogenesis, predisposing factors, clinical features, treatment and prognosis.A apoplexia em tumor hipofisário é uma emergência médica decorrente do infarto agudo ou hemorrágico na glândula hipófise. Nesta revisão os autores discutem a anatomia da região selar, a vascularização da hipófise e adenomas hipofisários, e demais aspectos da síndrome como etiopatogenia, fatores predisponentes, quadro clínico, tratamento e prognóstico.

  4. Tumor suppressor ARF regulates tissue microenvironment and tumor growth through modulation of macrophage polarization.

    Science.gov (United States)

    Jiménez-García, Lidia; Herranz, Sandra; Higueras, María Angeles; Luque, Alfonso; Hortelano, Sonsoles

    2016-10-11

    Tumor microenvironment has been described to play a key role in tumor growth, progression, and metastasis. Macrophages are a major cellular constituent of the tumor stroma, and particularly tumor associated macrophages (TAMs or M2-like macrophages) exert important immunosuppressive activity and a pro-tumoral role within the tumor microenvironment. Alternative-reading frame (ARF) gene is widely inactivated in human cancer. We have previously demonstrated that ARF deficiency severely impairs inflammatory response establishing a new role for ARF in the regulation of innate immunity. On the basis of these observations, we hypothesized that ARF may also regulates tumor growth through recruitment and modulation of the macrophage phenotype in the tumor microenvironment. Xenograft assays of B16F10 melanoma cells into ARF-deficient mice resulted in increased tumor growth compared to those implanted in WT control mice. Tumors from ARF-deficient mice exhibited significantly increased number of TAMs as well as microvascular density. Transwell assays showed crosstalk between tumor cells and macrophages. On the one hand, ARF-deficient macrophages modulate migratory ability of the tumor cells. And on the other, tumor cells promote the skewing of ARF-/- macrophages toward a M2-type polarization. In conclusion, these results demonstrate that ARF deficiency facilitates the infiltration of macrophages into the tumor mass and favors their polarization towards a M2 phenotype, thus promoting tumor angiogenesis and tumor growth. This work provides novel information about the critical role of ARF in the modulation of tumor microenvironment.

  5. TUMOR CONTAMINATION IN THE BIOPSY PATH OF PRIMARY MALIGNANT BONE TUMORS

    Science.gov (United States)

    Oliveira, Marcelo Parente; Lima, Pablo Moura de Andrade; de Mello, Roberto José Vieira

    2015-01-01

    Objective: To study factors possibly associated with tumor contamination in the biopsy path of primary malignant bone tumors. Method: Thirty-five patients who underwent surgical treatment with diagnoses of osteosarcoma, Ewing's tumor and chondrosarcoma were studied retrospectively. The sample was analyzed to characterize the biopsy technique used, histological type of the tumor, neoadjuvant chemotherapy used, local recurrences and tumor contamination in the biopsy path. Results: Among the 35 patients studied, four cases of contamination occurred (11.43%): one from osteosarcoma, two from Ewing's tumor and one from chondrosarcoma. There was no association between the type of tumor and presence of tumor contamination in the biopsy path (p = 0.65). There was also no association between the presence of tumor contamination and the biopsy technique (p = 0.06). On the other hand, there were associations between the presence of tumor contamination and local recurrence (p = 0.01) and between tumor contamination and absence of neoadjuvant chemotherapy (p = 0.02). Conclusion: Tumor contamination in the biopsy path of primary malignant bone tumors was associated with local recurrence. On the other hand, the histological type of the tumor and the type of biopsy did not have an influence on tumor contamination. Neoadjuvant chemotherapy had a protective effect against this complication. Despite these findings, tumor contamination is a complication that should always be taken into consideration, and removal of the biopsy path is recommended in tumor resection surgery. PMID:27047877

  6. Regulation of invadopodia by the tumor microenvironment

    Science.gov (United States)

    Gould, Christine M; Courtneidge, Sara A

    2014-01-01

    The tumor microenvironment consists of stromal cells, extracellular matrix (ECM), and signaling molecules that communicate with cancer cells. As tumors grow and develop, the tumor microenvironment changes. In addition, the tumor microenvironment is not only influenced by signals from tumor cells, but also stromal components contribute to tumor progression and metastasis by affecting cancer cell function. One of the mechanisms that cancer cells use to invade and metastasize is mediated by actin-rich, proteolytic structures called invadopodia. Here, we discuss how signals from the tumor environment, including growth factors, hypoxia, pH, metabolism, and stromal cell interactions, affect the formation and function of invadopodia to regulate cancer cell invasion and metastasis. Understanding how the tumor microenvironment affects invadopodia biology could aid in the development of effective therapeutics to target cancer cell invasion and metastasis. PMID:24714597

  7. New Microscope Scans Breast Tumors During Surgery

    Science.gov (United States)

    ... https://medlineplus.gov/news/fullstory_166925.html New Microscope Scans Breast Tumors During Surgery The instrument examines ... WEDNESDAY, June 28, 2017 (HealthDay News) -- A new microscope could help surgeons remove breast tumors completely, reducing ...

  8. [Tumors of the ovaries in zebus].

    Science.gov (United States)

    Izquierdo, N; Zhelev, V; Angelov, A K

    1983-01-01

    In a total of 112 slaughtered sterile cows of the Zebu cattle, originating from the Camaguey region, Cuba, 25 tumors were found in 22 animals (in three of the cows the tumors were bilateral). Histologically, the tumors were differentiated as follows: 1 papillary adenoma, 6 papillary cystadenomas, 1 adenocarcinoma, 1 papillary adenocarcinoma, 8 granulosocellular tumors, 1 thecoma, 2 luteomas, 4 hemangiomas, and 1 angiocarcinoma. In two of the cases, beside tumors there were in the ovaries parovarian cysts. The differentiation of the tumors were made on the basis of the international histologic classification of tumors of the World Health Organization. Most frequently, the tumors turned out to be granulosocellular ones as well as papillary cystadenomas and hemangiomas.

  9. Patient-Derived Antibody Targets Tumor Cells

    Science.gov (United States)

    An NCI Cancer Currents blog on an antibody derived from patients that killed tumor cells in cell lines of several cancer types and slowed tumor growth in mouse models of brain and lung cancer without evidence of side effects.

  10. Intrasellar malignant peripheral nerve sheath tumor (MPNST).

    Science.gov (United States)

    Krayenbühl, N; Heppner, F; Yonekawa, Y; Bernays, R L

    2007-02-01

    Intracranial malignant peripheral nerve sheath tumors (MPNST) and intrasellar schwannomas are rare tumors. We describe a case of an intrasellar schwannoma with progression to a MPNST, a finding that, although very rare, extends the differential diagnosis of intrasellar lesions.

  11. Treatment Option Overview (Extragonadal Germ Cell Tumors)

    Science.gov (United States)

    ... Germ Cell Tumors Treatment Extragonadal Germ Cell Tumors Treatment (PDQ®)–Patient Version General Information About Extragonadal Germ ... Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery ) and treatment ...

  12. Stages of Ovarian Low Malignant Potential Tumors

    Science.gov (United States)

    ... potential tumor include pain or swelling in the abdomen. Ovarian low malignant potential tumor may not cause ... include the following: Pain or swelling in the abdomen . Pain in the pelvis. Gastrointestinal problems, such as ...

  13. Stages of Gestational Trophoblastic Tumors and Neoplasia

    Science.gov (United States)

    ... check the liver, kidney, and bone marrow . Serum tumor marker test : A procedure in which a sample of ... increased levels in the body. These are called tumor markers . For GTD, the blood is checked for the ...

  14. Dendritic cells are stressed out in tumor.

    Science.gov (United States)

    Maj, Tomasz; Zou, Weiping

    2015-09-01

    A recently paper published in Cell reports that dendritic cells (DCs) are dysfunctional in the tumor environment. Tumor impairs DC function through induction of endoplasmic reticulum stress response and subsequent disruption of lipid metabolic homeostasis.

  15. Syndrome-Associated Tumors by Organ System.

    Science.gov (United States)

    Gonzalez, Raul S; Riddle, Nicole D

    2016-06-01

    Certain tumors suggest the possibility of a patient harboring a genetic syndrome, particularly in children. Syndrome-associated tumors of the gastrointestinal tract, genitourinary tract, gynecologic tract, heart, lungs, brain, eye, endocrine organs, and hematopoietic system will be briefly discussed.

  16. Biological stoichiometry in tumor micro-environments.

    Directory of Open Access Journals (Sweden)

    Irina Kareva

    Full Text Available Tumors can be viewed as evolving ecological systems, in which heterogeneous populations of cancer cells compete with each other and somatic cells for space and nutrients within the ecosystem of the human body. According to the growth rate hypothesis (GRH, increased phosphorus availability in an ecosystem, such as the tumor micro-environment, may promote selection within the tumor for a more proliferative and thus potentially more malignant phenotype. The applicability of the GRH to tumor growth is evaluated using a mathematical model, which suggests that limiting phosphorus availability might promote intercellular competition within a tumor, and thereby delay disease progression. It is also shown that a tumor can respond differently to changes in its micro-environment depending on the initial distribution of clones within the tumor, regardless of its initial size. This suggests that composition of the tumor as a whole needs to be evaluated in order to maximize the efficacy of therapy.

  17. Biological stoichiometry in tumor micro-environments.

    Science.gov (United States)

    Kareva, Irina

    2013-01-01

    Tumors can be viewed as evolving ecological systems, in which heterogeneous populations of cancer cells compete with each other and somatic cells for space and nutrients within the ecosystem of the human body. According to the growth rate hypothesis (GRH), increased phosphorus availability in an ecosystem, such as the tumor micro-environment, may promote selection within the tumor for a more proliferative and thus potentially more malignant phenotype. The applicability of the GRH to tumor growth is evaluated using a mathematical model, which suggests that limiting phosphorus availability might promote intercellular competition within a tumor, and thereby delay disease progression. It is also shown that a tumor can respond differently to changes in its micro-environment depending on the initial distribution of clones within the tumor, regardless of its initial size. This suggests that composition of the tumor as a whole needs to be evaluated in order to maximize the efficacy of therapy.

  18. Targeting tumor microenvironment: crossing tumor interstitial fluid by multifunctional nanomedicines

    Directory of Open Access Journals (Sweden)

    Yadollah Omidi

    2014-06-01

    Results: We reviewed all relevant literature for the impacts of tumor interstitium and microvasculature within the TME as well as the significance of the implemented strategies. Results: While tumorigenesis initiation seems to be in close relation with an emergence of hypoxia and alterations in epigenetic/genetic materials, large panoplies of molecular events emerge as intricate networks during oncogenesis to form unique lenient TME in favor of tumor progression. Within such irregular interstitium, immune system displays defective surveillance functionalities against malignant cells. Solid tumors show multifacial traits with coadaptation and self-regulation potentials, which bestow profound resistance against the currently used conventional chemotherapy and immunotherapy agents that target solely one face of the disease. Conclusion: The cancerous cells attain unique abilities to form its permissive microenvironment, wherein (a extracellular pH is dysregulated towards acidification, (b extracellular matrix (ECM is deformed, (c stromal cells are cooperative with cancer cells, (d immune system mechanisms are defective, (e non-integrated irregular microvasculature with pores (120-1200 nm are formed, and (h interstitial fluid pressure is high. All these phenomena are against cancer treatment modalities. As a result, to control such abnormal pathophysiologic traits, novel cancer therapy strategies need to be devised using multifunctional nanomedicines and theranostics.

  19. Embolismo tumoral pulmonar microvascular en paciente con tumor urotelial

    Directory of Open Access Journals (Sweden)

    B. de Escalante Yangüela

    2015-08-01

    Full Text Available Los embolismos tumorales pulmonares (ETP representan una causa poco frecuente de disnea en pacientes oncológicos. La mayoría se diagnostican en pacientes con tumores avanzados, sobre todo localizados en mama, pulmón o estómago. Existen pocos casos publicados en pacientes con tumores uroteliales. Presentamos el caso de un varón de 69 años, sin diagnóstico previo de cáncer, que ingresó por disnea subaguda, con sospecha clínica inicial de tromboembolismo pulmonar (TEP. El paciente falleció al 5º día del ingreso. En la autopsia se comprobó la existencia de un tumor en pelvis renal izquierda con metástasis hepáticas y linfoganglionares múltiples y una extensa embolia pulmonar microvascular que afectaba a gran parte de los capilares y vasos sanguíneos de mediano calibre de ambos pulmones. Los ETP se consideraron responsables de la insuficiencia respiratoria progresiva y se interpretaron como la causa última de la muerte. La presentación clínica más frecuente de los ETP es la disnea, a menudo se confunden con el TEP y el diagnóstico no resulta fácil. Su pronóstico es muy malo, con una elevadísima mortalidad y su confirmación habitualmente es post-mortem.

  20. 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

  1. Minimal invasive surgery in pediatric solid tumors.

    Science.gov (United States)

    Chan, Kin Wai; Lee, K H; Tam, Y H; Yeung, C K

    2007-12-01

    There is only limited experience of using the minimally invasive surgery (MIS) technique in resecting pediatric solid tumors. In this paper, we report our experience of using the MIS technique in the management of pediatric solid tumors. A retrospective review was undertaken on all children who had undergone MIS for their solid tumors between 1995 and 2005. Over a 10-year period, there were 38 patients who had undergone MIS for tumor resection. The mean age at the time of surgery was 7.5 years (range, 1 day to 15 years). There were 22 ovarian tumors, 4 sacrococcygeal tumors, 3 adrenal tumors, 3 retroperitoneal tumors, 1 kidney tumor, 1 liver mass, 1 intra-abdominal testicular tumor, and 3 intrathoracic masses. Thirty of 38 patients had undergone a successful resection using the MIS technique (78.9%). Eight patients required a conversion to the open procedure because of limited intraperitoneal space in 7 and excessive bleeding in 1. Of the 28 successfully MIS-resected intra-abdominal tumors, 18 required enlargement of the umbilical incision and 5 required an additional Pfannenstiel incision for tumor retrieval. Enlargement of the thoracic port site for specimen retrieval was required in the 2 successfully MIS-resected intrathoracic masses. The mean operation time was 171 minutes (range, 45-275). There was no postoperative complication encountered. On an average follow-up of 3.1 years, there was no recurrence observed, even in the 7 patients with malignant tumors, and all patients with successful MIS tumor excision had good cosmetic results. With the advance of laparoscopic instruments and techniques, a variety of pediatric solid tumors can be resected safely by the MIS technique. This has the potential benefit of a more rapid postoperative recovery and better cosmetic results. The role of the MIS technique in resecting malignant tumors is uncertain, as the number of cases in the current series is too small to draw any conclusion.

  2. Primary primitive neuroectodermal tumor of the orbit

    Directory of Open Access Journals (Sweden)

    Das Dipankar

    2009-01-01

    Full Text Available Primitive neuroectodermal tumor (PNET is a small round cell malignant tumor of neuroectodermal origin. Most of the PNETs occur in the central nervous system (CNS. PNETs recognized outside of CNS are diagnosed as peripheral PNET (pPNET. This tumor which expresses MIC-2 gene (CD99 seems to be least aggressive after complete tumor resection. We describe a rare case of PNET in a young girl.

  3. Extrahepatic bile duct neurilemmoma mimicking Klatskin tumor.

    Science.gov (United States)

    Kamani, Fereshteh; Dorudinia, Atosa; Goravanchi, Farhood; Rahimi, Farzaneh

    2007-04-01

    Neurilemmoma rarely develops in the biliary tree. Here, we report a 39-year-old Iranian woman with neurilemmoma in the extrahepatic bile duct presenting with progressively deepening jaundice. On the basis of clinical and radiological features, this tumor was initially suspected as Klatskin tumor. Histologically, the tumor was a typical neurilemmoma. Immunostaining showed that tumor cells were strongly and diffusely positive for S-100 protein, which supported the diagnosis of neurilemmoma. Neurilemmoma should be considered in the differential diagnosis of obstructive jaundice.

  4. Imaging Tumor Cell Movement In Vivo

    OpenAIRE

    Entenberg, David; Kedrin, Dmitriy; Wyckoff, Jeffrey; Sahai, Erik; Condeelis, John; Segall, Jeffrey E

    2013-01-01

    This unit describes the methods that we have been developing for analyzing tumor cell motility in mouse and rat models of breast cancer metastasis. Rodents are commonly used both to provide a mammalian system for studying human tumor cells (as xenografts in immunocompromised mice) as well as for following the development of tumors from a specific tissue type in transgenic lines. The Basic Protocol in this unit describes the standard methods used for generation of mammary tumors and imaging th...

  5. Fluorescent Nanoparticle Uptake for Brain Tumor Visualization

    Directory of Open Access Journals (Sweden)

    Rachel Tréhin

    2006-04-01

    Full Text Available Accurate delineation of tumor margins is vital to the successful surgical resection of brain tumors. We have previously developed a multimodal nanoparticle CLIO-Cy5.5, which is detectable by both magnetic resonance imaging and fluorescence, to assist in intraoperatively visualizing tumor boundaries. Here we examined the accuracy of tumor margin determination of orthotopic tumors implanted in hosts with differing immune responses to the tumor. Using a nonuser-based signal intensity method applied to fluorescent micrographs of 9L gliosarcoma green fluorescent protein (GFP tumors, mean overestimations of 2 and 24 µm were obtained using Cy5.5 fluorescence, compared to the true tumor margin determined by GFP fluorescence, in nude mice and rats, respectively. To resolve which cells internalized the nanoparticle and to quantitate degree of uptake, tumors were disaggregated and cells were analyzed by flow cytometry and fluorescence microscopy. Nanoparticle uptake was seen in both CD11b+ cells (representing activated microglia and macrophages and tumor cells in both animal models by both methods. CD11b+ cells were predominantly found at the tumor margin in both hosts, but were more pronounced at the margin in the rat model. Additional metastatic (CT26 colon and primary (Gli36 glioma brain tumor models likewise demonstrated that the nanoparticle was internalized both by tumor cells and by host cells. Together, these observations suggest that fluorescent nanoparticles provide an accurate method of tumor margin estimation based on a combination of tumor cell and host cell uptake for primary and metastatic tumors in animal model systems and offer potential for clinical translation.

  6. Cystic adenomatoid tumor of the uterus

    Directory of Open Access Journals (Sweden)

    Varsha Manucha

    2015-01-01

    Full Text Available We present a case of a cystic adenomatoid tumor in a 40-year-old woman. The tumor was an intramural multicystic mass, histologically similar to a multicystic mesothelioma. Cystic adenomatoid tumors of the uterus are extremely rare. They present with a wide differential diagnosis in radiology. The tumors are known to be benign and awareness of this rare entity is the key to its diagnosis for a pathologist.

  7. Intraosseous glomus tumor of the fibula

    Energy Technology Data Exchange (ETDEWEB)

    Bahk, W.J. [Catholic Univ. of Korea, Gyunggido (Korea). Dept. of Orthopaedic Surgery; Mirra, J.M. [Orthopaedic Oncology, Orthopaedic Hospital, Los Angeles, CA (United States); Anders, K.H. [Dept. of Pathology, Kaiser Permanente, Woodland Hills, CA (United States)

    2000-12-01

    Glomus tumor is a rare, benign vascular tumor and intraosseous glomus tumor, which arises primarily within bone, is even rarer. Fewer than 20 cases have been reported in the literature. We present the case of a 34-year-old woman with glomus tumor primarily in the midshaft of the fibula that radiologically mimicked chondromyxoid fibroma, aneurysmal bone cyst or adamantinoma, together with a review of other reported cases. (orig.)

  8. Glomus Tumor Presenting as Raynaud's Phenomenon

    Directory of Open Access Journals (Sweden)

    M. H. Abdelrahman

    2012-01-01

    Full Text Available Glomus tumors are rare tumors that often include hands and feet; they present characteristically with paroxysmal pain, exquisite point tenderness, and cold sensitivity. Such diagnosis needs to be confirmed by imaging like ultrasound and magnetic resonance imaging (MRI. Surgical excision is the treatment of choice for glomus tumors. There are only few case reports of glomus tumors in association with Raynaud’s phenomenon; this is considered to be the 4th case.

  9. Human neutrophils facilitate tumor cell transendothelial migration.

    LENUS (Irish Health Repository)

    Wu, Q D

    2012-02-03

    Tumor cell extravasation plays a key role in tumor metastasis. However, the precise mechanisms by which tumor cells migrate through normal vascular endothelium remain unclear. In this study, using an in vitro transendothelial migration model, we show that human polymorphonuclear neutrophils (PMN) assist the human breast tumor cell line MDA-MB-231 to cross the endothelial barrier. We found that tumor-conditioned medium (TCM) downregulated PMN cytocidal function, delayed PMN apoptosis, and concomitantly upregulated PMN adhesion molecule expression. These PMN treated with TCM attached to tumor cells and facilitated tumor cell migration through different endothelial monolayers. In contrast, MDA-MB-231 cells alone did not transmigrate. FACScan analysis revealed that these tumor cells expressed high levels of intercellular adhesion molecule-1 (ICAM-1) but did not express CD11a, CD11b, or CD18. Blockage of CD11b and CD18 on PMN and of ICAM-1 on MDA-MB-231 cells significantly attenuated TCM-treated, PMN-mediated tumor cell migration. These tumor cells still possessed the ability to proliferate after PMN-assisted transmigration. These results indicate that TCM-treated PMN may serve as a carrier to assist tumor cell transendothelial migration and suggest that tumor cells can exploit PMN and alter their function to facilitate their extravasation.

  10. An intramedullary tumor presenting with hyperhidrosis

    Directory of Open Access Journals (Sweden)

    Chatterjee Sandip

    2004-07-01

    Full Text Available A case of a cervical intramedullary tumor is reported whose presentation was with disabling hyperhidrosis. The symptom resolved after surgical debulking of the tumor. Hyperhidrosis as a presenting manifestation of an intramedullary tumor has not been reported earlier.

  11. [Klatskin tumors: rational diagnostics and staging].

    Science.gov (United States)

    Denzer, U W; Rösch, T

    2012-03-01

    Klatskin tumors continue to be a challenge for diagnostic assessment and staging due to their longitudinal tumor growth along the perihilar bile ducts. Therefore the rate of non-resectable tumors remains relatively stable despite modern imaging and endoscopic techniques. This article reviews the current diagnostic methods for preoperative staging and the significance for predicting resectability.

  12. Thoracic tumors : prognostic and thearapeutic improvements

    NARCIS (Netherlands)

    Jong, Wouter Karst de

    2008-01-01

    Thoracic tumors are a major burden of disease in many countries. Non-small cell lung cancer and small-cell lung cancer are the most common thoracic malignancies, both tumors are one of the most well-known of the less common thoracic tumors. In this thesis, new ways in estimating the prognosis of pat

  13. Localization of thymosin beta-4 in tumors

    DEFF Research Database (Denmark)

    Larsson, L. -I.; Holck, Susanne

    2007-01-01

    Overexpression of thymosin beta-4 has been linked to malignant progression but the localization of this polypeptide within tumors is incompletely known. We therefore examined breast cancers for thymosin beta-4 using immunofluorescence. Reactive cells were identified with monoclonal cell marker...... in the tumor microenvironment may modulate tumor behavior....

  14. Pulmonary carcinoid tumor associated with nephrotic syndrome.

    Science.gov (United States)

    DePace, N L; Elquezabal, A; Hardenburg, H C

    1980-04-01

    A patient with carcinoid tumor of the lung associated with nephrotic syndrome was treated. Excision of the tumor resulted in remission of marked proteinuria, hypoalbuminemia, and edema. A review of the literature disclosed many neoplasms associated with the nephrotic syndrome; however, no association of the nephrotic syndrome and a carcinoid tumor of the lung has previously been reported, to our knowledge.

  15. The pathobiology of glioma tumors.

    Science.gov (United States)

    Gladson, Candece L; Prayson, Richard A; Liu, Wei Michael

    2010-01-01

    The ongoing characterization of the genetic and epigenetic alterations in the gliomas has already improved the classification of these heterogeneous tumors and enabled the development of rodent models for analysis of the molecular pathways underlying their proliferative and invasive behavior. Effective application of the targeted therapies that are now in development will depend on pathologists' ability to provide accurate information regarding the genetic alterations and the expression of key receptors and ligands in the tumors. Here we review the mechanisms that have been implicated in the pathogenesis of the gliomas and provide examples of the cooperative nature of the pathways involved, which may influence the initial therapeutic response and the potential for development of resistance.

  16. Tumor Metabolism of Malignant Gliomas

    Energy Technology Data Exchange (ETDEWEB)

    Ru, Peng; Williams, Terence M.; Chakravarti, Arnab; Guo, Deliang, E-mail: deliang.guo@osumc.edu [Department of Radiation Oncology, Ohio State University Comprehensive Cancer Center & Arthur G James Cancer Hospital, Columbus, OH 43012 (United States)

    2013-11-08

    Constitutively activated oncogenic signaling via genetic mutations such as in the EGFR/PI3K/Akt and Ras/RAF/MEK pathways has been recognized as a major driver for tumorigenesis in most cancers. Recent insights into tumor metabolism have further revealed that oncogenic signaling pathways directly promote metabolic reprogramming to upregulate biosynthesis of lipids, carbohydrates, protein, DNA and RNA, leading to enhanced growth of human tumors. Therefore, targeting cell metabolism has become a novel direction for drug development in oncology. In malignant gliomas, metabolism pathways of glucose, glutamine and lipid are significantly reprogrammed. Moreover, molecular mechanisms causing these metabolic changes are just starting to be unraveled. In this review, we will summarize recent studies revealing critical gene alterations that lead to metabolic changes in malignant gliomas, and also discuss promising therapeutic strategies via targeting the key players in metabolic regulation.

  17. Tumor Metabolism of Malignant Gliomas

    Directory of Open Access Journals (Sweden)

    Deliang Guo

    2013-11-01

    Full Text Available Constitutively activated oncogenic signaling via genetic mutations such as in the EGFR/PI3K/Akt and Ras/RAF/MEK pathways has been recognized as a major driver for tumorigenesis in most cancers. Recent insights into tumor metabolism have further revealed that oncogenic signaling pathways directly promote metabolic reprogramming to upregulate biosynthesis of lipids, carbohydrates, protein, DNA and RNA, leading to enhanced growth of human tumors. Therefore, targeting cell metabolism has become a novel direction for drug development in oncology. In malignant gliomas, metabolism pathways of glucose, glutamine and lipid are significantly reprogrammed. Moreover, molecular mechanisms causing these metabolic changes are just starting to be unraveled. In this review, we will summarize recent studies revealing critical gene alterations that lead to metabolic changes in malignant gliomas, and also discuss promising therapeutic strategies via targeting the key players in metabolic regulation.

  18. Endoscopic resection of subepithelial tumors

    Institute of Scientific and Technical Information of China (English)

    Arthur; Schmidt; Markus; Bauder; Bettina; Riecken; Karel; Caca

    2014-01-01

    Management of subepithelial tumors(SETs) remains challenging. Endoscopic ultrasound(EUS) has improved differential diagnosis of these tumors but a definitive diagnosis on EUS findings alone can be achieved in the minority of cases. Complete endoscopic resection may provide a reasonable approach for tissue acquisition and may also be therapeutic in case of malignant lesions. Small SET restricted to the submucosa can be removed with established basic resection techniques. However, resection of SET arising from deeper layers of the gastrointestinal wall requires advanced endoscopic methods and harbours the risk of perforation. Innovative techniques such as submucosal tunneling and full thickness resection have expanded the frontiers of endoscopic therapy in the past years. This review will give an overview about endoscopic resection techniques of SET with a focus on novel methods.

  19. Tumor Carcinoide Gástrico.

    OpenAIRE

    Haydelisis Peraza González; Ofelia María Pompa Oliva; Mirian Belkis Nápoles Valdés

    2015-01-01

    Las neoplasias endocrinas se originan en las células del sistema neuroendocrino, también afectan al tubo digestivo; dentro de ellas, el tumor carcinoide gástrico es poco frecuente. El objetivo de la publicación es mostrar la situación de una paciente femenina que presentó características clínicas, endoscópicas, histológicas e inmunohistoquímica de esta variedad de tumor gástrico. Paciente femenina de 45 años de edad con antecedentes de salud, referida de su área de salud al Departamento de G...

  20. Tumor Molecular Imaging with Nanoparticles

    Directory of Open Access Journals (Sweden)

    Zhen Cheng

    2016-03-01

    Full Text Available Molecular imaging (MI can provide not only structural images using traditional imaging techniques but also functional and molecular information using many newly emerging imaging techniques. Over the past decade, the utilization of nanotechnology in MI has exhibited many significant advantages and provided new opportunities for the imaging of living subjects. It is expected that multimodality nanoparticles (NPs can lead to precise assessment of tumor biology and the tumor microenvironment. This review addresses topics related to engineered NPs and summarizes the recent applications of these nanoconstructs in cancer optical imaging, ultrasound, photoacoustic imaging, magnetic resonance imaging (MRI, and radionuclide imaging. Key challenges involved in the translation of NPs to the clinic are discussed.