Cases reported "Sarcoma"

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1/141. Myxoid variant of follicular dendritic cell sarcoma arising in the breast.

    Follicular dendritic cell sarcoma is a malignant tumor of the follicular dendritic cell which can arise in extranodal sites. We present here a case arising as a mass in the breast of a 41-year-old woman. The tumor was composed of mildly pleomorphic spindly cells with pale ovoid nuclei and cell processes intimately admixed with mature lymphocytes. In much of the lesion the cells were dispersed in cords in a myxoid stroma, and elsewhere there were solid sheets. The neoplastic cells were immunoreactive for CD21, CD35, EMA, and S100 protein, but not for other lymphoid markers or cytokeratin. Electron microscopy showed interdigitating cytoplasmic processes with junctions but no external lamina. The differential diagnosis includes carcinoma, lymphomas, and a variety of myxoid sarcomas. The tumor recurred within a few months and displayed increased nuclear pleomorphism and lymphatic invasion but the patient appears free of disease 3 years after the further excision. This case extends the spectrum of follicular dendritic cell sarcoma in soft tissue sites.
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2/141. 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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3/141. Penile epithelioid sarcoma: MR imaging findings.

    magnetic resonance imaging findings of a 38-year-old man with epithelioid sarcoma of the penis is presented. It started as a firm, painless and slowly growing nodule at the base of his penis 6 months previously which caused pain radiating to the testis during coitus. It has been well known that sarcomas may well mimic reactive processes. Initial presentation of epithelioid sarcoma may provoke considerable diagnostic difficulty, and its differentiation from benign lesions, such as Peyronie's disease and chronic inflammation, may be a clinical problem. In our present report the MR findings are compared with those of the epithelioid sarcomas of various locations reported in the literature and differential diagnosis of the entity is discussed. To our knowledge, this is the first report regarding the MR findings of the epithelioid sarcoma of penis.
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4/141. 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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5/141. Alveolar soft-part sarcoma in the oral cavity: report of a case.

    This case presents a rare tumor of the oral cavity--an alveolar soft-part sarcoma. Difficulties developed in the diagnosis, and therefore, consultations were required. Because of the radioresistant qualities of the lesion, wide surgical excision was performed. The patient's condition has been followed for 40 months without evidence of recurrence. The conclusion arrived at from review of the literature is that this is an indolent but malignant tumor and has so far proved to be 100% fatal. However, it may be 20 years before it proves fatal.
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6/141. Granular cell traumatic neuroma: a lesion occurring in mastectomy scars.

    BACKGROUND: Granular cell changes can be observed in a variety of benign and malignant tumors, and are seen more commonly in granular cell tumors, which in about 5% of cases develop in the breast. Granular cells also have been observed in sites of previous trauma, such as surgery, and are found to be inflammatory reactions of histiocytic origin. methods AND RESULTS: We investigated, morphologically and immunohistochemically, 2 granular cell lesions occurring in mastectomy scars after surgery for carcinoma. Both lesions were composed of strands and nests of large granular cells, haphazardly set in a background of fibrous tissue, with sparse inflammatory infiltrates. Several tortuous hypertrophic nerve bundles were also embedded in the fibrous tissue. A few of these nerve bundles showed degenerative changes and contained granular cells. Immunohistochemically, granular cells were positive for S100 protein, neuron-specific enolase, vimentin, and CD68 antigen. CONCLUSIONS: We consider these proliferative lesions of peripheral nerves to have the features of both granular cell tumor and traumatic neuroma. These cases indicate that traumatic neuroma can undergo extensive granular cell changes and constitute a previously unrecognized entity, which we provisionally label granular cell traumatic neuroma. Granular cell traumatic neuroma has to be taken into consideration when evaluating lesions occurring at mastectomy scars and should be differentiated from malignant tumors with granular cells, such as apocrine carcinoma and alveolar soft part sarcoma.
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7/141. 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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8/141. Interdigitating dendritic cell sarcoma. A report of four cases and review of the literature.

    To better define the clinical and pathologic features of interdigitating dendritic cell sarcoma (IDCS), we report 4 cases, including the first reported in the tonsil. There were 2 male and 2 female patients (mean age, 70 years). Sites of tumor included 1 case each in the right cervical lymph node, left axillary lymph node, right tonsil, and right inguinal lymph node. Histologically, all showed diffuse effacement of the lymphoid tissue by pleomorphic round to spindled cells with convoluted nuclei and abundant eosinophilic cytoplasm. All were immunoreactive for S-100, CD68, lysozyme, and vimentin. CD45 was positive in 3 cases and CD1a in 1 case. Fascin was positive in 3 cases. Other immunostains, including CD3, CD20, CD21, CD30, actin, cytokeratin, and HMB-45, were negative. Ultrastructurally, the tumor cells were elongated and showed indented nuclei, variable numbers of lysosomes, and interdigitating cytoplasmic processes. Follow-up was available for all cases. One patient died of widespread disease 2 months after diagnosis. One was alive with metastatic lung disease at 12 months. Two patients were disease free at 5 and 9 months.
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9/141. A primary cardiac sarcoma with unusual histology and clinical course.

    A left atrial tumor, in which radical resection was impossible, demonstrated two processes: An inflammatory pseudotumor and cellular atypia suggestive of a sarcoma. immunohistochemistry (proliferating cell nuclear antigen [PCNA], MIB-1 [Ki-67 antibody], bcl-2 positive; p53 negative, focal loss of nm23) was supportive for a malignant tumor. Despite no further therapy because of uncertainty in tumor classification, the patient remained in remission for 28 months. Thereafter, spine metastases and local regrowth were found, and the patient died 15 months later, after temporary remission by radiotherapy. This case stresses the impact immunohistochemistry may have on diagnosis of malignancy and the difficulty in predicting the biological behavior of cardiac sarcomas.
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10/141. Development of follicular dendritic cell sarcoma in hyaline-vascular Castleman's disease of the nasopharynx: tracing its evolution by sequential biopsies.

    AIMS: Hyaline-vascular Castleman's disease (HVCD) and follicular dendritic cell (FDC) sarcoma occurring in the nasopharynx are both extremely rare. We report the first case of transformation of the former into the latter as documented by sequential biopsies. The steps involved in the transformation were described in detail and the possible role of p53 studied. methods AND RESULTS: The patient presented at the age of 23 years with nasopharyngeal HVCD. Hyaline- vascular Castleman's disease with FDC overgrowth was diagnosed in a recurrence 8 years later, and a frank FDC sarcoma developed at the same site 11 years after initial presentation. The patient remained disease-free 3 years after excision and adjuvant chemotherapy. The FDC sarcoma comprised swirling fascicles of spindly cells with indistinct cell borders. The tumour cells expressed the FDC markers CD21, CD35 and CNA.42 and in-situ hybridization for Epstein-Barr virus-encoded RNAs was negative. Over-expression of p53 protein was observed in the FDC sarcoma and an increased number of weakly p53-positive spindly cells could also be demonstrated in the HVCD specimen. This finding suggested a possible role of p53 in the evolution from HVCD to FDC sarcoma. Critical analysis of the literature shows that, among the 13 reported cases of FDC sarcoma associated with Castleman's disease, possible progression from the latter to the former is documented in only two cases. CONCLUSIONS: The sequential changes observed in the current case provide further evidence to strengthen the role of HVCD as a possible precursor of FDC sarcoma. There is a possible role of p53 in the transformation process but confirmation by future studies is needed.
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