Cases reported "Retroperitoneal Neoplasms"

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1/309. Paraneoplastic pemphigus associated with follicular dendritic cell sarcoma arising from Castleman's tumor.

    Paraneoplastic pemphigus (PNP) is associated with various forms of underlying neoplasia. We report a patient with PNP associated with an intraabdominal follicular dendritic cell sarcoma that developed from a Castleman's tumor that had preexisted for 40 years.
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ranking = 1
keywords = sarcoma
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2/309. Embryonal rhabdomyosarcoma with only numerical chromosome changes. Case report and review of the literature.

    An embryonal rhabdomyosarcoma, presenting as a retroperitoneal mass in a 15-year-old girl, is reported. The histological and immunohistochemical picture was typical, except for the presence of focal chondroid differentiation. Interestingly, expression of the "muscle markers" desmin and alpha-sarcomeric actin was present in the latter areas. cytogenetic analysis showed a hyperdiploid karyotype without structural chromosome changes. The pertinent literature on the subject is reviewed. Hyperdiploidy of the clonal type seems to occur frequently, but no characteristic karyotype is so far emerging.
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ranking = 1
keywords = sarcoma
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3/309. Obstructive uropathy due to primary retroperitoneal tumour (leiomyosarcoma): report of 2 cases and review of the literature.

    Two cases of primary retroperitoneal leiomyosarcoma presenting with obstructive uropathy which were successfully treated by surgical excision are reported. The classification of primary retroperitoneal tumours and their symptomatology, treatment and prognosis are discussed in detail based on a review of the literature and the current experience of treating these tumours by combination therapy. It is felt that with advances in the diagnosis and therapy the prognosis is likely to improve.
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ranking = 1
keywords = sarcoma
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4/309. Cervical sarcoma botryoides. A case report.

    BACKGROUND: Sarcoma botryoides is a rare gynecologic malignancy. Reported treatment protocols range from localized resection of the tumor to radical surgery and adjuvant chemotherapy. Recently, limited excisional surgery combined with chemotherapy has contributed to excellent survival rates and preserved bladder and rectal function. CASE: We report a case of cervical sarcoma botryoides with minimal invasion. The patient had primary surgical treatment with complete resection of the tumor without adjuvant chemotherapy. Four months after surgery she presented with extensive metastatic retroperitoneal disease and died two weeks later. CONCLUSION: The clinical course of this patient suggests that complete surgical resection in cases of localized disease is not adequate therapy for sarcoma botryoides. Adjuvant chemotherapy should always be used, even at a very early stage.
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ranking = 1.2
keywords = sarcoma
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5/309. Simulation of renal function by tumor and its effect on the calculated glomerular filtration rate.

    A patient with a retroperitoneal sarcoma near the left kidney was to undergo radiotherapy. Renal assessment with Tc-99m DTPA to determine renal function before radiotherapy resulted in what was thought to be a low glomerular filtration rate based on the Gates computer method and the Russell two-blood-sample method. Most likely this resulted from the observed significant accumulation of DTPA in the tumor for the first few minutes with washout for the rest of the imaging time, simulating renal function.
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ranking = 0.2
keywords = sarcoma
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6/309. A rare chimeric TLS/FUS-CHOP transcript in a patient with multiple liposarcomas: a case report.

    Myxoid liposarcomas harbor a unique and specific t(12;16)(q13,p11) chromosomal translocation. The breakpoint has recently been identified, and involvement of the TLS/FUS gene on chromosome 16 and the CHOP gene on chromosome 12 was demonstrated. We report a case of a 45-year-old woman who developed multiple malignant lipomatous tumors of unknown origin and myxoid/round cell histology at different locations. To examine the diagnostic potential of this translocation and to develop a hypothesis on the origin of the tumors, we used cytogenetic and molecular cytogenetic methods (reverse transcription polymerase chain reaction, RT-PCR). We identified a chimeric rna transcript in the second recurrence in the thigh/groin, as well as in another tumor in the mediastinum, which has an additional sequence of 33 bp, known as fusion transcript type III. cytogenetic analysis of another tumor in retroperitoneal space revealed a rare type of unbalanced translocation der(16)t(12;16). We hypothesize that these tumors are metastases rather than multicentric tumors. The detection of the chimeric message in the present case is not only useful for differential diagnosis, but also for analyzing the origin of multiple neoplasms.
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ranking = 1
keywords = sarcoma
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7/309. Primary pure intratesticular fibrosarcoma.

    Testicular sarcoma appears to be a rare stromal tumor usually of indolent course with potential for distant metastases. A pure primary intratesticular fibrosarcoma in a 71-year-old male is presented. By the time the tumor became evident metastases had already occurred widely. The course was rapidly fatal. A review of the literature yielded only one case reported previously. The present case illustrates that pure fibrosarcoma occurs occasionally as a stromal tumor and may be associated with a dismal prognosis. The diagnosis of pure fibrosarcoma should be made only after extensive sampling of the testicular tumor to rule out an associated germ cell component. The differential diagnosis also includes the fibroma of gonadal stroma origin and the unclassified sex cord-stromal tumor with a predominance of spindle cells.
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ranking = 1.6
keywords = sarcoma
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8/309. Treatment of a malignant enterocutaneous fistula with octreotide acetate.

    An enterocutaneous malignant fistula developed in a patient who had a retroperitoneal angiosarcoma. He was treated with octreotide acetate subcutaneously. drainage decreased and ceased after 2 weeks of therapy. The closure of this malignant fistula suggests that palliative therapy with octreotide acetate merits further study in view of the grave prognosis of this complication.
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ranking = 0.2
keywords = sarcoma
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9/309. Late recurrence of liposarcoma simulating adenoma of the duodenum.

    A large pedunculated, polypoid mass in the duodenum of a patient with asymptomatic anaemia, with mucosal biopsies indicating a villous adenoma, turned out to be a liposarcoma during laparotomy. The patient had had a completely resected retroperitoneal liposarcoma 8 years before. liposarcoma recurrence should be highly suspected even in case of atypical presentation and long disease free interval.
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ranking = 1.4
keywords = sarcoma
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10/309. Case of retroperitoneal dedifferentiated mixed-type liposarcoma: comparison of proliferative activity in specimens from four operations.

    In a case of retroperitoneal dedifferentiated mixed-type liposarcoma, a dedifferentiated component was observed in the so-called mixed-type liposarcoma consisting of well-differentiated and myxoid components. The proliferative activity was compared among the different components of the tumor by immunohistochemical study using the proliferating cell nuclear antigen (PCNA) and MIB-1 monoclonal antibodies. The dedifferentiated component showed higher positivity than the well-differentiated and myxoid components, and tumor progression was most advanced in the dedifferentiated component. In the chronological examination of each component, the labeling indices of PCNA and MIB-1 were significantly higher at the third recurrence than in the primary lesion in all types, indicating that the proliferative activity of the tumor cells increased gradually. Considering the surgical treatment of liposarcoma, an extended resection encompassing normal adjacent tissues is required in cases containing the dedifferentiated component in comparison to the cases containing only well-differentiated or myxoid components.
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ranking = 1.4
keywords = sarcoma
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