Cases reported "Vulvar Neoplasms"

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1/32. Malignant myoepithelioma of the vulva resembling a rhabdoid tumour.

    AIMS: We report an example of malignant myoepithelioma of the vulva, which has not been hitherto described. We discuss the differential diagnosis and briefly review the literature. methods AND RESULTS: The lesion was found in an 81-year-old woman as an indolent 40 mm tumour. The neoplastic cells showed a myoid, spindled, epithelioid and plasmacytoid phenotype. Hyalinization of extracellular material and myxoid changes were present. There was a partly solid and microcystic pattern and a tight cohesiveness of cells was lacking. The circumscribed multinodular tumour somewhat resembled an extrarenal rhabdoid tumour, having large tumour cells with prominent nucleoli and large amounts of acidophilic cytoplasm. Immunohistochemically, the tumour cells were immunoreactive for cytokeratin, vimentin, muscle-specific actin, alpha-smooth muscle actin, and S100 protein, but not for desmin, epithelial membrane antigen, factor viii-related antigen, CD34 and CD31. CONCLUSIONS: The histological and cytomorphological appearance of the tumour well as the immunohistochemical findings suggest the diagnosis of malignant myoepithelioma, possibly derived from minor vestibulary glands or ectopic breast tissue. Differential diagnoses are, in particular, extrarenal rhabdoid tumour and 'proximal type' epithelioid sarcoma. Differentiation is important, because the tumours show a different behaviour and prognosis.
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2/32. Malignant rhabdoid tumor of the vulva. Case report.

    The poor prognosis of malignant rhabdoid tumor is emphasized and histopathological criteria for distinction from epithelial sarcoma of the vulva are discussed. Immunohistochemical analyses were performed by using nine different antigens including vimentin, cytokeratin, epithelial membrane antigen, carcinoembryonic antigen, desmin, muscle-specific actin, S-100 protein, AP-15, neuron specific enolase. This is the sixth reported case of a malignant rhabdoid tumor of the vulva. The patient died eight months after the initial diagnosis in spite of a combination of surgery, adjuvant chemotherapy and external radiotherapy.
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3/32. Merkel cell (neuroendocrine) carcinoma of the vulva. A case report with immunohistochemical and ultrastructural findings and review of the literature.

    A new case of primary Merkel cell carcinoma (MCC) of the vulva is reported and the literature reviewed for noting its clinical presentation, microscopic, immunohistochemical and ultrastructural features, as well as for establishing the role of immunohistochemistry in the ultimate diagnosis of this uncommon and aggressive tumor. The lesion occurred in a 79 year old patient. Histologically, the tumor was composed of intradermal small cells with high mitotic index and frequent apoptosis. The immunohistochemical study showed positivity for wide spectrum and low molecular weight cytokeratins, epithelial membrane antigen, neurofilaments, neuron specific enolase and chromogranin a. Electron microscopy revealed intermediate filaments in a typical globular paranuclear arrangement. The coexpression of cytokeratins (including cytokeratin 20) and neurofilaments, both in typical globular paranuclear arrangement, made possible the diagnosis of MCC, differentiating it from other malignant small cell tumors such as neuroendocrine metastatic carcinoma.
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4/32. leiomyosarcoma of the vulva. A case report.

    BACKGROUND: leiomyosarcoma of the vulva is a rare mesenchymal tumor. Biologic features of a low grade tumor were investigated by an immunohistochemical workup. CASE: A 38-year-old woman presented with a slowly growing vulvar mass. Surgical treatment was performed, and a low grade leiomyosarcoma of the vulva was diagnosed. Immunohistochemical reactions were performed with monoclonal antibodies against desmin, vimentin, smooth muscle actin, cytokeratin, S-100 protein, estrogen, progesterone and androgen receptor, p53 protein, ki-67 antigen, leukocyte common antigen and polyclonal antibodies to factor viii-related antigen. Expression of estrogen, progesterone and androgen receptor was present in addition to a moderate number of Ki-67-positive cells and absence of p53 protein overexpression and lymphatic cell infiltration besides adequate microvessel density for smooth muscle tumors. Since the immunohistochemical markers indicated a less aggressive tumor, any further adjuvant therapy was rejected. The patient was without recurrence 24 months later. CONCLUSION: The immunohistologic profile proved the low histologic grade of vulvar leiomyosarcoma. The findings helped to estimate prognosis and plan therapy.
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5/32. Mucinous adenocarcinoma of Bartholin gland treated with radiation therapy: a case report.

    adenocarcinoma of Bartholin gland is a very rare disease and its molecular pathological features are poorly delineated. A 92-year-old woman with mucinous adenocarcinoma of Bartholin gland with metastasis to the right inguinal lymph node was treated with radiation therapy alone. Despite intensive radiation therapy, the tumor was locally recurrent and the patient died 10 months after radiation therapy. We searched for the presence of human papillomavirus 16 and 18 dna and the expression of p53 protein, CA19-9, CEA and MIB-1 antigen. Immunohistochemical study showed that mucinous adenocarcinoma of Bartholin gland produced CA19-9 and CEA.
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6/32. Primary vulvar adenocarcinoma of cloacogenic origin.

    A 43-year-old woman who had a vulvar mass associated with mild discomfort was found to have a rare primary vulvar adenocarcinoma of probable cloacal origin. The tumor was contiguous with the surface epithelium of the vulva and was a well to moderately differentiated adenocarcinoma of colonic type. Stains of the neoplastic cells were positive for both acid and neutral mucin, and periodic acid-Schiff (PAS) was positive after diastase reaction. The neoplastic cells were strongly positive for carcinoembryonic antigen, broad spectrum cytokeratin, and p-53 antigen. Clinical evaluation failed to show any primary tumor in colon, lung, or breast. The patient was disease free 18 months after operation.
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7/32. Angiomyofibroblastoma of the vulva.

    A case of angiomyofibroblastoma of the vulva in a 53-year-old woman was examined by means of immunohistochemistry. Histological examination of the tumor revealed typical characteristics of vulval angiomyofibroblastoma. immunohistochemistry of the tumor cells revealed diffuse immunoreactivity for estrogen receptors, progesterone receptors, vimentin, and CD34. The stains for cytokeratin, epithelial membrane antigen, desmin, smooth muscle alpha-actin, muscle-specific actin, and S-100 were negative. These results were mostly consistent with those of previous reports and suggest that the tumors cells were derived from primitive mesenchymal cells which occur normally in this lesion and which show the potential for diverse lines of myoid differentiation.
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8/32. Human papillomavirus-negative spindle cell carcinoma of the vulva associated with lichen sclerosus: case report and literature review.

    Although spindle cell carcinoma (SC) is a common neoplasm in the oral cavity, upper respiratory tract, and other head and neck areas, its occurrence in the vulva is rare. We report a case of this rare condition with immunohistochemical, ultrastructural, and human papillomavirus (HPV) testing. The neoplastic cells were positive for vimentin, keratins (AE1-AE3, keratin 902, and keratin 903), and epithelial membrane antigen. Ultrastructurally, they showed primitive junctions and tonofilaments. HPV testing by polymerase chain reaction was negative. In addition, we review the clinicopathologic findings of the four well-documented cases of vulvar SC that have been reported previously in the English language literature.
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9/32. myoepithelioma of the vulva.

    The histologic diagnosis of myoepithelioma is often problematic. We here describe a case of myoepithelioma, composed exclusively of neoplastic myoepithelial cells, in the vulva of a 52-year-old female. The vulva is a very rare site for this neoplasm. A subcutaneous tumor measuring 3.0 x 2.5 x 2.0 cm displayed a multinodular growth pattern. Histologically, it was characterized by epithelioid, trabecular, cord, solid, or reticular arrangements of tumor cells and markedly hyalinized stroma. The tumor cells were large and polygonal with eccentrically located round to oval nuclei with prominent nucleoli and eosinophilic cytoplasm. Some cells had clear cytoplasm. Moderate cellular atypia was seen and there were 4 mitotic figures per 10 high-power fields. No ductal architecture was found and there were no areas of chondroid or osseous differentiation. There was no destructive invasive growth. Immunohistochemically the tumor was positive for vimentin, epithelial membrane antigen, wide keratin, alpha-smooth muscle actin, S-100 protein, and glial fibrillary acidic protein. The patient was well and free of disease at 6 months. This neoplasm should be distinguished from other epithelial and mesenchymal neoplasms as it shows a different clinical behavior.
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10/32. Cellular angiofibroma of the vulva: a clinicopathological study of two cases with documentation of some unusual features and review of the literature.

    BACKGROUND: Cellular angiofibroma (CA) of the vulva is a recently described condition, whose clinical and pathological features are poorly known. methods: We have encountered two cases of this very unusual tumor. Their clinical and pathological features were analyzed and compared to those reported in the literature. RESULTS: Both patients were middle-aged women. In each case, the lesion had the clinical appearance of a vulvar cyst, located in the lateral aspect of the clitoris and the right labium majus, respectively. Microscopically, the lesions were well circumscribed but not truly encapsulated. Both were composed of small spindle cells arranged in short fascicles and mixed up with relatively abundant small- or medium-sized rounded vessels. While mitotic activity was perceptible in both cases, no cellular atypia could be demonstrated. A striking feature seen in one case was the presence of pseudoangiomatous changes in the stroma, similar to those occasionally found in spindle cell lipoma. Phenotypically, the tumor cells consistently expressed vimentin, CD99, and both estrogen and progesterone receptors. A discrete CD34 or smooth muscle actin immunoreactivity was also found in one case. No expression of S-100 protein, Bcl-2 protein, CD117 (c-kit gene product), epithelial membrane antigen, desmin, or h-caldesmon could be demonstrated. CONCLUSION: This study further illustrates that CA of the vulva has distinct clinical and pathologic features that set it apart from the other soft tissue conditions involving this area. However, like many soft tissue neoplasms, this tumor also exhibits some variation in its histological or immunohistochemical features.
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