Cases reported "Sarcoma"

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1/21. 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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2/21. Follicular dendritic cell tumor presenting in the lung: a case report.

    An example of extranodal follicular dendritic cell sarcoma (FDCS) presenting in the lung, a heretofore unreported site, is described. Macroscopically, a 9.5-cm, tan-white, dominant mass and multiple smaller parenchymal and pleural nodules were identified. Microscopically, the tumor was composed of spindled cells with uniform cytologic features arranged in short, intersecting fascicles and intermixed small lymphocytes and plasma cells. One of 4 peribronchial and hilar lymph nodes evaluated microscopically was focally involved by the process. Immunohistochemically, the neoplastic spindled cells expressed complement receptors CD21 and CD35 and low-affinity nerve growth factor receptor but did not express keratin (AE1/AE3 and CAM5.2), CD45 (leukocyte common antigen), CD20 (L26), S-100 protein, muscle-specific actin, or gp100 protein (HMB45). Ultrastructurally, the tumor cells have complex interdigitating cell surface processes and desmosomes. Epstein-Barr virus (EBV) was not detected in the tumor cells by in situ hybridization for EBV-encoded rna or by polymerase chain reaction for viral dna. FDCS should be considered in the differential diagnosis of any spindled-cell tumor with interspersed chronic inflammatory cells occurring in the lung. An immunohistochemical panel, including anti-CD21 and -CD35, can assist in its diagnosis, especially with small bronchial biopsy specimens. 2001 by W.B. Saunders Company.
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3/21. Squamous carcinoma of bladder with pseudosarcomatous stroma.

    A polypoid squamous cell carcinoma with pseudosarcomatous stroma of the urinary bladder was studied electron microscopically. The epithelial component was a typical squamous carcinoma that consisted of cells with abundant bundles of tonofilaments that converged toward well-developed desmosomes; keratohyalin granules were also seen. The stroma consisted of fusiform cells with dilated rough endoplasmic-reticulum cisternae and irregular cytoplasmic projections that were suggestive of active fibroblasts. No structures suggestive of an epithelial origin or of advanced mesenchymal differentiation were recognized. These observations are consistent with the notion that the pseudosarcomatous stroma represents a reactive process that is probably related to the growth of the epithelial neoplasm. Given the differences in behavior and prognosis between carcinomas with pseudosarcomatous stroma and true carcinosarcomas, efforts at separation of these entities are warranted.
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4/21. Sarcoma of follicular dendritic cells in the dorsal mediastinum.

    Follicular dendritic cell sarcomas (FDCSs) are very rare and usually originate in lymph nodes. We report an exceedingly rare case with localization in the dorsal mediastinum and, for the first time, provide positron emission tomography (PET) data for this tumor. This report describes the case of a 76-year-old man with a clinically aggressive tumor in the dorsal mediastinum. Computed tomography scan revealed displacement of soft tissue and lymph nodes. PET showed that the tumor had a high proliferation rate. Investigation of the successfully removed tumor mass revealed reactivity of the tumor cells for follicular dendritic cell markers and desmosomes linking adjacent tumor cells at the ultrastructural level. Marked atypia, a high mitotic rate, and areas of coagulative necrosis were found. The tumor in our case revealed the typical features and thus was classified as FDCS. In contrast to previous reports in the literature, preoperative imaging, histology, and immunohistochemistry studies indicated at least an intermediate degree of malignancy. Nevertheless, the patient made a good postoperative recovery and remained apparently disease-free 2 years later.
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5/21. Follicular dendritic cell sarcoma of the pharyngeal region: histologic, cytologic, immunohistochemical, and ultrastructural study of three cases.

    Follicular dendritic cell sarcoma is a tumor of recent description and characterization; it is often underdiagnosed because it is easily confused with other entities. Three cases of follicular dendritic cell sarcoma are described in the present article. The first occurred in the parapharyngeal space in a 29-year-old woman who developed multiple recurrences over the span of 10 years. The second was located in the left tonsil in a 48-year-old man, and the third case developed in the parapharyngeal space in a 26-year-old man. All cases were positive for CD21 and CD35 and ultrastructurally they displayed a morphologic spectrum. The first case featured spindle cells with interdigitated long cell processes joined by well-developed desmosomes. In the other two cases there were round to ovoid cells with interwoven processes connected by occasional desmosomes. Including these three cases, a total of 20 follicular dendritic cell sarcoma of the pharyngeal region have been reported to date. The clinical behavior of these tumors is similar to other low-grade sarcomas.
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6/21. Follicular dendritic cell sarcoma of lymph node--a rare entity.

    Follicular dendritic cells (FDC) are non-lymphoid, non-phagocytic accessory cells in the immune system that are essential for antigen presentation and germinal center reaction regulation. These cells are CD21 , CD35 , CD1a- and S100 protein /- and they show desmosomes ultrastructurally. The most commonly involved sites by FDC tumors are lymph nodes but may arise at a variety of extranodal sites including oral cavity, tonsil, gastrointestinal tract and liver. Most studies represent single case reports or case series. Our patient presented with tumor in the lymph nodes. histology revealed tumor cells with abundant eosinophilic cytoplasm, hyperchromatic and pleomorphic nuclei, and prominent nucleoli. The tumor cells were found to be positive for CD21 which is a specific marker for follicular dendritic cells.
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7/21. Interwoven dendritic processes of follicular dendritic cell sarcoma demonstrated on ultrafast papanicolaou-stained smears: a case report.

    BACKGROUND: Follicular dendritic cell (FDC) tumor is a rare tumor derived from accessory cells in the lymphoid follicles. FDC tumors are typically diagnosed on histology based on immunoreactivity to at least 1 of the FDC markers (CD21, CD23 or CD35) or based on the characteristic ultrastructural feature of long, interwoven, cytoplasmic, dendritic processes connected by desmosomes. CASE: We observed novel cytologic features of FDC sarcoma in a liver fine needle aspirate of a 46-year-old man status post surgery and chemotherapy for FDC sarcoma, originating in the gastrointestinal tract with metastases to the liver, pancreas and spleen. In the Diff-Quik- and Papanicolaou-stained smears, the tumor cells presented in syncytial fragments as well as single cells, as previously reported in the cytologic literature. However, the single cells were interconnected to neighboring single cells via long, thin, threadlike cytoplasmic processes in ultrafast Papanicolaou (UFP)-stained smears. The tumor cells possessed multipolar cytoplasmic processes rather than unipolar ones, as previously reported. CONCLUSION: The ultrastructural features of a web of interwoven, dendritic, cytoplasmic processes of FDC tumor was demonstrated for the first time on cytology. observation of this feature may allow the diagnosis to be made on cytology prior to histology, immunohistochemistry or electron microscopy.
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8/21. Sarcomatoid renal cell carcinoma metastatic to the heart: report of a case.

    An unusual case of metastatic sarcomatoid renal cell carcinoma is presented. Fifteen months after nephrectomy for a typical clear cell carcinoma, a 63-year-old man presented with bilateral pleural effusions, cardiomegaly, and tamponade. A pericardial biopsy showed an anaplastic spindle cell tumor that was strongly keratin positive and showed desmosomes ultrastructurally. The patient died shortly thereafter, and the autopsy revealed massive tumor infiltration of the heart, pulmonary and adrenal metastases, and tumor nodules at the incision site of his nephrectomy. The differential diagnosis of sarcomatoid renal cell carcinoma is discussed.
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9/21. Common leukocyte antigen staining of a primitive sarcoma.

    A 4-year-old boy presented with symptoms of tracheal obstruction and was found to have a polypoid tracheal mass, which was studied by biopsy. light microscopy showed a tumor composed of small cells with round to oval dark nuclei, clumped chromatin, one to two nucleoli, and small, variable amounts of indistinct pink cytoplasm. In other areas the tumor had a loose, spindle appearance, with some cells showing more elongated nuclei, and fibrillar pink cytoplasm consistent with strap cells. Cross striations were not found. Electron microscopy showed desmosomes and 7 to 10 nm cytoplasmic filaments forming dense bodies. The findings are most consistent with a primitive sarcoma, probably rhabdomyosarcoma. Immunoperoxidase with three monoclonal antibodies for common leukocyte antigen showed diffuse membraneous staining with fresh-frozen tissue. All other lymphocyte and monocyte marker studies were negative. We believe that this case of anticommon leukocyte antigen staining, a rhabdomyosarcoma, represents the first report of a false positive reaction with monoclonal antibody to common leukocyte antigen.
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10/21. A light microscopic and ultrastructural study of spindle-shaped hepatocellular carcinoma.

    Spindle-cell hepatocellular carcinoma is an unusual morphologic variant of hepatocellular carcinoma with a typical sarcomatous appearance. The exact diagnosis of this tumor may be difficult when only small biopsies are available and in the absence of ultrastructural studies. We describe two cases of hepatocellular carcinoma: one was entirely composed of spindle-shaped cells, and the other was a typical hepatocellular carcinoma with only a small area of sarcomatous, fusiform cells. In the first case, ultrastructural studies demonstrated desmosomes and many mallory bodies and confirmed the epithelial nature of the neoplasm. In the second case, no ultrastructural studies were available, but the presence of gradual transition from liver cell carcinoma to spindle-cell carcinoma excluded the diagnosis of carcino-sarcoma.
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