Cases reported "Sarcoma"

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1/72. Extranodal follicular dendritic cell sarcoma of the palate.

    Follicular dendritic cell tumors are uncommon and usually occur in lymph nodes. We report the case of a follicular dendritic cell tumor that occurred in the palate of a 14-year-old boy and manifested itself as a nodular mass. Histologically, the neoplasm consisted of spindle-shaped or oval-shaped cells with eosinophilic cytoplasms and nuclei with delicate, dispersed chromatin. The lesional cells were principally arranged in diffuse, fascicular patterns with vaguely whorled or storiform areas. Focal multinucleate tumor giant cells and lymphocytes were observed throughout the neoplasm. Immunohistochemically, tumor cells were positive for the follicular dendritic cell markers CD21, CD35, and CD23 and for S-100 protein, CD68, and muscle-specific actin. Tumor cells were negative for LCA, CD20, EMA, CK (AE1/AE3), HMB45, and CD34. lymphocytes were positive for LCA and CD45RO. Although follicular dendritic cell sarcoma is a very uncommon tumor, it should be included in the differential diagnosis of tumors in this location.
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2/72. Sarcomatoid carcinoma of the prostate: progression from adenocarcinoma is associated with p53 over-expression.

    BACKGROUND: The pathogenesis of sarcomatoid metaplasia of prostatic adenocarcinoma is uncertain. The histologic features of sarcomatoid carcinoma arising in two patients with previously irradiated prostatic adenocarcinoma are reported and the relationship between prostatic adenocarcinoma and subsequent sarcomatoid carcinoma is investigated by immunohistochemical detection of epithelial and soft tissue tumor markers, and p53 protein. methods AND RESULTS: Two patients, aged 72 and 67 years, underwent localized radiotherapy for prostatic adenocarcinoma and re-presented with sarcomatoid carcinoma 41 months and 60 months later, respectively. In both cases the tumor consisted of anaplastic spindle cells with occasional osteoclast-like giant cells. The initial tumors showed immunohistochemical staining typical of prostatic adenocarcinoma with absence of expression of p53 protein. The subsequent sarcomatoid carcinomas were positive for vimentin and negative for epithelial cell markers. In both cases serial biopsies showed a temporal increase in tumor expression of p53 protein. CONCLUSIONS: The development of sarcomatoid carcinoma in prostatic adenocarcinoma is associated with progressive accumulation of p53. This is suggestive of increasing clonal dominance of dedifferentiated tumor cells carrying p53 mutations.
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3/72. 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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4/72. Granulocytic sarcoma as the cause of giant abdominal mass: diagnosis by fine needle aspiration and review of the literature.

    Granulocytic sarcoma (GS) or chloroma is a neoplasia consisting of myeloid precursors in an extramedullary site. It is generally associated with myeloproliferative disorders especially with myeloid neoplasias. A young woman with huge abdominal mass due to GS associated with chronic myelocytic leukemia (CML) has been reported and literature is reviewed.
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5/72. Sarcomatoid carcinoma of the renal pelvis: a case report.

    A case of sarcomatoid carcinoma of the renal pelvis is reported. A 72-year-old male was admitted with a chief complaint of right flank pain. Clinical imaging studies revealed marked dilation of the right renal pelvis caused by a tumor at the pyeloureteric region. Right nephrectomy was performed in June 1999. The 8.4 x 6.5 cm tumor was grossly polypoid in appearance and protruded into the renal pelvis. Histologically, sarcomatoid spindle cells predominated over the carcinomatous component (mainly transitional cell carcinoma, partly associated with squamous cell and adenocarcinoma components). Osteoclast-like CD68-positive multinucleated giant cells were scattered. The sarcomatoid component was immunoreactive for both cytokeratin and vimentin. Sarcomatoid cells negative for cytokeratin were also noted. In both the sarcomatoid and carcinomatous components, nuclear overexpression of p53 oncoprotein was confirmed. The histogenesis of sarcomatoid carcinoma of the renal pelvis is discussed.
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6/72. Gastrointestinal autonomic nerve sarcoma presenting as a giant intra-abdominal cyst.

    Gastrointestinal autonomic nerve sarcomas are rare tumours. A 36-year-old man presented with abdominal pain, distension and constipation. Abdominal ultrasound and CT scanning demonstrated a giant cystic intra-abdominal mass. laparotomy confirmed a large cystic mass arising from the ileum with multiple metastases. Immunohistochemical staining was positive for vimentin, neuronal-specific enolase and PGP9.5. This is the first reported case to present as a giant intra-abdominal cyst. Specialist histopathological and immunohistochemical analysis is essential to establish the diagnosis of this rare tumour.
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7/72. Myxoid liposarcoma of the oral cavity with involvement of the periodontal tissues.

    BACKGROUND, AIMS: liposarcoma is the 2nd most frequent soft tissue sarcoma in adults, but it is extremely rare in the head and neck and, particularly, in the oral cavity. We report on a 25-year-old female who presented with a periodontal mass, extended from the right upper 3rd molar to the right upper 2nd premolar, covered by intact oral mucosa. The clinical differential diagnosis included peripheral giant cell granuloma, salivary gland neoplasms, squamous cell carcinoma of the gingiva, sarcoma and malignant lymphoma. methods: To accurately plan subsequent treatment, an excisional biopsy was performed and a myxoid liposarcoma was diagnosed. Consequently, the patient underwent wide excision of the neoplasm with maxillary en-block resection. RESULTS: The post-operative course was uneventful and the patient is alive and well 8 years after the original diagnosis. The authors stress the importance of considering soft tissue sarcomas in the diagnostic approach to patients with unusual periodontal neoplasms and to plan adequate surgical sampling of the lesion (i.e. excisional biopsy). CONCLUSIONS: This appears of pivotal importance as more limited specimens may result in inaccurate pre-operative diagnosis.
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8/72. Recurrent malignant variant of phosphaturic mesenchymal tumor with oncogenic osteomalacia.

    Phosphaturic mesenchymal tumor is a rare neoplasm which causes osteomalacia or rickets. The tumor typically follows a benign clinical course. Even in the rare malignant cases, local recurrence and distant metastasis are uncommon. We report on an example of a malignant phosphaturic mesenchymal tumor which recurred several times over 16 years concurrently causing hypophosphatemia, bone pain, and osteomalacia. Following each surgery, symptoms and hypophosphatemia improved. The patient died of disease 17 years after the first surgery. Histologically, the initial tumor was composed of small spindle cells with clusters of giant cells, prominent blood vessels, poorly formed cartilaginous areas, and crystalline material. Cytological atypia was minimal. Following multiple recurrences, the tumor demonstrated areas of high-grade sarcoma exhibiting marked pleomorphism, numerous mitotic figures, and p53 overexpression. This case illustrates the potential lethality of incompletely removed phosphaturic mesenchymal tumors.
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9/72. Hyalinizing spindle cell tumor with giant rosettes of the broad ligament.

    BACKGROUND: Hyalinizing spindle cell tumor with giant rosettes (HSTGR) is a rare, recently described tumor that most commonly occurs in the peripheral deep soft tissues. CASE: A 53-year-old woman was operated on because of a mass in the broad ligament which was first noted 17 years previously. The tumor showed typical features of HSTGR. Two years after surgery, the patient is alive with no evidence of disease. CONCLUSIONS: To our knowledge, the present case is the first description of HSTGR occurring in the broad ligament. Despite its bland morphology, HSTGR is a low-grade sarcoma, most probably a variant of low-grade fibromyxoid sarcoma. A wide resection of the tumor and prolonged follow-up are needed because patients may develop late metastases.
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10/72. Extranodal follicular dendritic cell sarcoma of the head and neck region: three new cases, with a review of the literature.

    Extranodal follicular dendritic cell (FDC) sarcoma of the head and neck region is uncommon, with 16 well-documented cases previously reported (four in the tonsil, four in the pharynx, two in the palate, five in the soft tissue, and one in the thyroid). We here report an additional three cases of extranodal FDC sarcoma in the tonsil (two cases) and pharynx (one case). In these new cases, the neoplastic cells were arranged in diffuse, fascicular, and vaguely whorled growth patterns. A background lymphocytic infiltrate was sprinkled throughout the neoplasms, with focal prominent perivascular cuffing. Scattered multinucleated giant cells were present. Immunohistochemically, tumor cells were strongly and diffusely positive for follicular dendritic cell markers CD21 and CD35. Tumor cells were diffusely positive for fascin and negative for leukocyte common antigen, S-100 protein, cytokeratin, and Epstein-Barr virus (EBV) latent membrane protein-1 (EBV-LMP). EBV was also not detected in the tumor cells by in situ hybridization for EBV-encoded RNAs. FDC sarcomas are probably an underrecognized neoplasm, especially when they occur in extranodal sites in the head and neck region. Two of the three new cases we report were initially misdiagnosed, and five cases of extranodal FDC sarcoma in the head and neck region reported in the recent literature were initially misdiagnosed. Our aim is to complement the current understanding of this neoplasm and alert pathologists to this rare entity in this region to avoid misdiagnosis. Recognition of extranodal FDC sarcoma requires a high index of suspicion, but this tumor has numerous distinctive histological features that should bring the neoplasm into the differential diagnosis. Confirmatory immunohistochemical staining with follicular dendritic cell markers such as CD21 and/or CD35 is essential for the diagnosis. Correct characterization of this neoplasm is imperative given its potential for recurrence and metastasis.
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