1/9. 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.- - - - - - - - - - ranking = 1keywords = rhabdoid tumour, rhabdoid (Clic here for more details about this article) |
2/9. 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.- - - - - - - - - - ranking = 0.090109941140277keywords = rhabdoid (Clic here for more details about this article) |
3/9. Malignant rhabdoid tumor of the vulva: case report and review of the literature with emphasis on clinical management and outcome.BACKGROUND: Malignant extrarenal rhabdoid tumors (ERTs) of the vulva are rare but aggressive neoplasms. We describe the eighth reported case and the first to have long-term survival despite initial rapid recurrence. CASE: A 40-year-old woman presented with a painless lump on the mons pubis. It was excised and revealed an ERT. One month later, the tumor had regrown. A wide local excision and superficial right groin node dissection was performed. Local recurrence was noted 11 months after the initial excision and was treated by wide excision and radiotherapy. She is without evidence of disease 61 months after initial presentation. CONCLUSION: A review of previously reported cases of ERT reveals that the median survival is 9 months. Local recurrences are common and the appearance of distant metastases is almost invariably rapidly fatal. Chemotherapy and radiotherapy do not appear to be effective in controlling disease. Initial surgical extirpation may offer the best chance of cure.- - - - - - - - - - ranking = 0.075091617616897keywords = rhabdoid (Clic here for more details about this article) |
4/9. Fine-needle aspiration cytology of "proximal-type" epithelioid sarcoma.The cytologic and immunocytologic findings in a case of recurrent "proximal-type" epithelioid sarcoma (ES) of the vulva are presented. This is a recently described neoplasm that differs clinically and morphologically from conventional ES. Cytologic smears showed a dissociated population of large, atypical neoplastic cells with bi- and multinucleated cells, abundant cytoplasm, and rhabdoid-like morphology. Due to its different clinical management it must be differentiated from metastatic carcinoma and melanoma. From a practical perspective, its differentiation from other epithelial-like sarcomas is less important. In conclusion the cytopathologic findings of "proximal-type" ES show a good correlation with histopathology, permitting the diagnosis of recurrences and metastases. When accompanied by adequate clinical information and ancillary studies, a specific preoperative recognition seems possible.- - - - - - - - - - ranking = 0.015018323523379keywords = rhabdoid (Clic here for more details about this article) |
5/9. Malignant rhabdoid tumor of the clitoris in an elderly patient: report of a case.Malignant rhabdoid tumors of the vulva are rare neoplasms which most of the time show aggressive behavior and a dismal prognosis. We report a case of malignant rhabdoid tumor of the clitoris occurring in an elderly patient. Due to the similarities that these neoplasms show with other low-differentiated tumors, immunohistochemical and ultrastructural assessment should always be conducted so that accurate diagnosis is achieved. Individualized extensive surgical treatment might decrease relapsing disease.- - - - - - - - - - ranking = 0.090109941140277keywords = rhabdoid (Clic here for more details about this article) |
6/9. Undifferentiated carcinoma of the vulva mimicking epithelioid sarcoma.We report an undifferentiated sweat gland carcinoma of the vulva in an 80-year-old woman. The tumor, which was located in the right labium majus, resembled an epithelioid sarcoma histologically; it had a granulomatous appearance with multiple tumor nodules containing epithelioid tumor cells. The tumor also contained rhabdoid cells; a large cluster of them showed histological features indistinguishable from those of a malignant rhabdoid tumor. Immunohistochemically, the tumor cells reacted not only for epithelial markers such as cytokeratins, EMA, and CEA, which are known to be expressed by epithelioid sarcoma, but also for CA125 and with monoclonal antibodies recognizing sweat gland structures--namely, EKH5 and EKH6. For comparison, two epithelioid sarcomas and two extrarenal malignant rhabdoid tumors were also studied. Of these tumors, only one extrarenal rhabdoid tumor reacted with EKH5, and none reacted for CA125. Electron-microscopic examination of the present tumor showed the presence of discontinuous basal laminae and tonofibril-like structures as well as primitive cell junctions and interdigitating filopodia. From these findings, we conclude that the tumor was an undifferentiated sweat gland carcinoma mimicking an epithelioid sarcoma. Findings in this case support the idea of the diverse histogenesis of extrarenal malignant rhabdoid tumors and indicate that electron microscopy is important for differentiating epithelioid sarcoma from skin adnexal carcinoma.- - - - - - - - - - ranking = 0.075091617616897keywords = rhabdoid (Clic here for more details about this article) |
7/9. Malignant rhabdoid tumor of the vulva: is distinction from epithelioid sarcoma possible? A pathologic and immunohistochemical study.Epithelioid sarcoma (ES) and malignant rhabdoid tumor (MRT) have heretofore been regarded as two separate clinicopathologic entities. However, they have some histologic similarities, and both represent histogenetic and phenotypic enigmas. This study reports the pathologic and immunohistochemical findings of four vulvar neoplasms occurring in young women that represented diagnostic dilemmas because of their similarity to both ES and MRT. Only one case had the classic histologic features of ES, whereas, in our opinion, the other three cases fulfilled the histologic criteria of MRT, despite the fact that two of the three cases were reported earlier as examples of ES. Neither electron microscopy nor immunohistochemistry has been found to be helpful in separating ES from MRT, mainly because they share several ultrastructural and immunophenotypic features. The behavior of these vulvar tumors--ours and the few published examples of ES--is generally aggressive, more in keeping with MRT than classic ES. We believe that some, if not most, putative ES of the vulva are in fact MRT, a neoplasm with an unfavorable prognosis.- - - - - - - - - - ranking = 0.075091617616897keywords = rhabdoid (Clic here for more details about this article) |
8/9. Malignant rhabdoid tumor of the vulva: a case report and review of the literature.Malignant rhabdoid tumor (MRT) is an uncommon aggressive neoplasm which usually occurs in the kidney of children, but it has also been found in extrarenal sites. MRT arising in the vulva is extremely rare. Only four cases of MRT of the vulva have been reported in the English literature. We herein present another case. The diagnosis and management of vulva MRT are reviewed.- - - - - - - - - - ranking = 0.075091617616897keywords = rhabdoid (Clic here for more details about this article) |
9/9. Malignant rhabdoid tumor of the vulva: case report with cytological, immunohistochemical, ultrastructural and dna ploidy studies and a review of the literature.A case of malignant rhabdoid tumor of the vulva in a 25-year-old female was examined. The patient presented with a subcutaneous nodule in the left labium majus. Smears of the material obtained by percutaneous fine-needle aspiration demonstrated clusters of atypical cells with prominent nucleoli. The tumor measured 6 x 5 x 5 cm and appeared tan to brown on the cut surface and partly cystic. Pathological findings obtained from intraoperative frozen tissue sections had been originally interpreted as rhabdomyosarcoma. light microscopic examination revealed that polygonal tumor cells having vesicular nuclei with prominent nucleoli were arranged in sheets and the great majority of the tumor cells contained an eosinophilic globular paranuclear cytoplasmic inclusion. Ultrastructurally, this cytoplasmic inclusion corresponds to whirls of intermediate filaments. vimentin immunoreactivity was detected in both the cytoplasm and cytoplasmic inclusion of almost all the tumor cells. No cytokeratin and desmin immunoreactivity were detected in the tumor cells. The Ki-67 labeling index was 36% and the dna content of the tumor cells, which was examined by image cytometry, demonstrated diploidy (dna index = 0.95).- - - - - - - - - - ranking = 0.075091617616897keywords = rhabdoid (Clic here for more details about this article) |