Cases reported "Sarcoma"

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1/100. Low-grade fibromyxoid sarcoma: clinicopathologic case report with review of the literature.

    Low-grade fibromyxoid sarcoma is a rare, benign-appearing soft tissue neoplasm with an aggressive clinical course characterized by multiple local recurrences over several years, with ultimate spread to lung and occasionally to bone. Thus far, a total of 24 cases of low-grade fibromyxoid sarcoma have been reported in the literature. The authors present an additional case that grossly and microscopically emphasizes a pronounced lobular pattern of contrasting areas of cellularity showing high proliferative activity, as demonstrated by a proliferation marker, Ki 67 with MIB-1, and hypocellular areas with prominent myxoid component and abundant collagen fibrils. There was predominance of delicate capillary-sized stromal vessels with collagenized walls in both cellular and myxoid areas. The unusual features in this case were osseous metaplasia, prominent intranuclear pseudoinclusions, dna tetraploidy, and membrane-bound intracytoplasmic fat vacuoles. The immunoprofile and cytologic and ultrastructural features are described. After the excision of the tumor, the patient was treated with radiotherapy without chemotherapy. The patient has been observed for 26 months and is alive without the evidence of disease. The postoperative follow-up with axial computed tomography at 24 months showed no evidence of disease, except postsurgical fibrotic changes.
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2/100. Proximal-type epithelioid sarcoma in the pelvic soft tissues.

    A case of proximal-type epithelioid sarcoma arising in the pelvic soft tissues of a 46-year-old man is presented. The tumor showed a predominantly epithelioid component with rhabdoid features, cord-like arrangement, small nests, abundant eosinophilic cytoplasm containing intracytoplasmic, paranuclear, hyaline-like globules, large vesicular nuclei, and prominent nucleoli. There were foci with alveolar arrangement and a spindle-cell proliferation. The tumor cells were positive for cytokeratin CAM5.2, vimentin, epithelial membrane antigen, and CD34. flow cytometry showed a diploid dna content with high S-phase fraction. The patient had pelvic lymph-node metastases. He died of the disease 5 months after diagnosis. It is important to include proximal-type epithelioid sarcoma in the differential diagnosis of pelvic tumors.
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3/100. Epithelioid sarcoma: report of two cases diagnosed by fine-needle aspiration biopsy with immunocytochemical correlation.

    Epithelioid sarcoma (ES) is an aggressive soft-tissue malignant tumor generally arising in the distal extremities of young adults. The microscopic diagnosis of ES is often difficult because of its rarity and its possible confusion with other malignant tumors or even with benign granulomatous processes. Two cases of ES and a recurrence of one of these tumors, diagnosed by fine-needle aspiration biopsy (FNAB), are reported. Cytologic smears were quite similar in both cases including the relapse, showing single or loosely arranged groups of medium to large atypical cells. Single cells had well-defined eosinophilic cytoplasms and one or more atypical, eccentrically located nuclei, resulting in a plasmacytoid appearance. Nuclei had fine granular chromatin and one or two large nucleoli. The cells sometimes palisaded along the edges of necrotic material. The immunocytochemical stains showed diffuse cytoplasmic positivity for cytokeratins (CAM 5.2) and both cytoplasmic and cell membrane positivity for vimentin, while S-100 protein and HMB 45 immunostaining were negative, thus supporting the cytological diagnosis of ES, which was subsequently proven on the surgical samples. Diagn. Cytopathol. 1999;21:405-408.
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4/100. Sarcomatoid renal cell carcinoma with scant carcinomatous components.

    A 30-year-old male underwent radical nephrectomy for a right renal tumor 15 cm in diameter. On microscopic examination of initial 17 sections, the tumor consisted of pleomorphic giant cells and spindle neoplastic cells. There was no carcinomatous component. Immunohistochemically, the neoplastic cells were negative for keratin and epithelial membrane antigen but positive for vimentin. The giant cells were also scatteringly, weakly positive for myoglobin. At that time a diagnosis of rhabdomyosarcoma of the kidney was made. However, further microscopic examination of another eight sections revealed small areas of clear cell-type renal cell carcinoma (RCC) which transited to sarcomatous components and led to a diagnosis of sarcomatoid RCC. The patient underwent three cycles of adjuvant chemotherapy. He has been free of the disease for 14 months after nephrectomy.
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5/100. Epithelioid sarcoma of the penis: report of an unusual case and review of the literature.

    Epithelioid sarcoma is a rare, slowly growing soft tissue tumor that uncommonly involves the penis, with only 11 previously reported cases. We present a case of penile epithelioid sarcoma in a 39-year-old man that mimicked Peyronie's disease, which was diagnosed 13 years following initial presentation. Preoperative magnetic resonance imaging showed multiple peripherally enhancing low signal intensity nodules involving the corpora cavernosa bilaterally. Following penectomy, histologic examination showed the typical features of epithelioid sarcoma, with a prominent pseudogranulomatous pattern. Immunohistochemically, the neoplastic cells demonstrated strong and diffuse staining for cytokeratins (AE1/AE3 and CAM 5.2), vimentin, epithelial membrane antigen, and CD34. Stains for S-100 protein, desmin, smooth muscle actin, and CD31 were negative. Electron microscopy demonstrated abundant intracytoplasmic intermediate filaments, scattered tonofilaments, and interdigitating filopodia. The present study is the first to describe magnetic resonance imaging and comprehensive immunohistochemical findings in penile epithelioid sarcoma. The majority of cases reported in the literature have demonstrated features similar to those typically found in epithelioid sarcoma involving the distal extremities. Consideration of epithelioid sarcoma in the differential diagnosis of a penile nodule or obstructive urinary symptoms may lead to early diagnosis and treatment.
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6/100. Epithelioid sarcoma arising on the nose of a child: a case report and review of the literature.

    A 4-year-old boy presented with a 6-month history of a red papule on the nasal septum. physical examination was otherwise unremarkable. A biopsy specimen showed an epithelioid sarcoma characterized by nodular collections of epithelioid tumor cells with central, tumor cell necrosis. By immunohistochemistry the tumor cells were positive for cytokeratin, epithelial membrane antigen, vimentin, and CD34, but negative for S-100, CD31, factor viii-related antigen, CD68, actin, desmin and myoglobin. Epithelioid sarcoma is an uncommon tumor of uncertain histogenesis that typically arises in the extremities of young adults. Both the age of our patient and the location of his tumor are unusual, emphasizing the spectrum of presentations that may occur with epithelioid sarcoma. Epithelioid sarcoma should be considered in the differential diagnosis of granulomatous diseases and epithelioid tumors of children, even in unusual locations.
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7/100. Epithelioid sarcoma of the penis: a case report and review of the literature.

    A rare case of epithelioid sarcoma (ES) of the penis is presented. The patient, a 35-year-old man, was initially treated as having Peyronie's disease, but the subsequent appearance of a subcutaneous nodule displayed a 'distal-type' ES. At immunohistochemical phenotypification, the tumor was positive for vimentin, cytokeratins and epithelial membrane antigen (EMA), as well as for some other multidirectional antibodies, including a membranous reaction for CD99. The review of 11 similar cases so far reported in the literature led to the conclusion that the clinicopathological characteristics of penile ES are basically the same as those of tumors in more classical locations: the age at diagnosis ranged from 23 to 43 years, the interval between first manifestations and diagnosis averaged 37 months (delayed diagnosis being common because of the slow growth rate and the harmless appearance of the lesion), the typical sign was a superficial nodule or mass, usually accompanied (better preceded) by urethral stenosis, dysuria and erectile disturbances. Total or partial penectomy was the treatment of choice in most patients, but lack of adequate follow up did not permit any definitive conclusion to be reached regarding its efficacy.
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8/100. Chromophobe renal cell carcinoma with sarcomatoid change. A case report.

    Chromophobe renal cell carcinoma (RCC) is a newly established entity of renal neoplasm with histological and molecular biological features different from those of common RCCs. Chromophobe RCC shows characteristically cloudy and reticular cytoplasm and cellular features resembling distal nephron. Its prognosis has been reported to be more favorable than that of common RCCs. Recently, however, several cases have been reported which showed sarcomatoid change to present poor prognosis. Here we present a case of chromophobe RCC with sarcomatoid change which was once resected surgically. The surgically resected tumor was histologically composed of chromophobe epithelial cell sheets and sarcomatoid elements. The former showed positivity for colloid iron staining, and was immunohistochemically positive for E-cadherin and epithelial membrane antigen (EMA), whereas the latter was positive for vimentin instead of colloid iron and E-cadherin. EMA was focally positive in the sarcomatoid element. The patient died with systemic metastases 14 months after the operation. Histologically, the metastatic tumors were composed only of sarcomatoid element lacking epithelial element. Based on these findings and previous reports, this case supports the existence of a tumor progression pathway from chromophobe to sarcomatoid RCC. It is necessary to perform careful postoperative investigation of chromophobe RCC due to its possible histological progression to the sarcomatoid subtype.
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9/100. Follicular dendritic cell sarcoma of the colon mimicking stromal tumour.

    AIMS: Follicular dendritic cell tumours are very rare neoplasms that often occur in lymph nodes. We report here a case in the colon, a hitherto unreported site, in a 37-year-old female. The differentiation from gastrointestinal stromal tumour is emphasized. methods and RESULTS: The tumour was tan, elastic and solid with surface ulceration. Microscopically, it was composed of oval to spindle tumour cells with syncytial cytoplasm arranged in fascicular and whorled patterns. There were many infiltrating lymphocytes. The histological appearance resembled gastrointestinal stromal tumour, thymoma or meningioma. Distinct from the stromal tumour, the lymph node was also involved by the tumour. Immunohistochemically, the tumour cells were positive for CD21, CD35 and CD68, but negative for cytokeratin, CD34, smooth muscle actin, desmin, S100 protein, epithelial membrane antigen, leukocyte common antigen, HMB-45 and c-kit. In-situ hybridization study was negative for Epstein-Barr virus rna sequences. Ultrastructurally, the tumour cells possessed cytoplasmic processes joined by desmosomes. CONCLUSIONS: This entity should be considered in the list of differential diagnoses for gastrointestinal stromal tumour. The lymph node metastasis and immunohistochemical features are of value for identification of this rare neoplasm.
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10/100. Sarcomatoid chordoma: chordoma with a massive malignant spindle-cell component.

    We report a case of chordoma containing a spindle cell sarcomatoid component with a gradual transition from conventional chordoma. Immunohistochemically, many tumor cells in both conventional chordoma and sarcomatoid components were positive for cytokeratins (AE1/AE3, CAM5.2) and epithelial membrane antigen as well as vimentin. This report provides a rare example of sarcomatoid chordoma. Familiarity with this type of bone tumor should help to avoid confusion with dedifferentiated chordoma and other spindle cell sarcomas or carcinomas.
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