1/149. Epithelioid sarcoma of the vulva.We report a case of a 23-year-old woman diagnosed as having an epithelioid sarcoma of the vulva. She was treated by a clitoris-sparing hemivulvectomy and lymph node sampling of the ipsilateral groin. Vulvar reconstruction was performed with a rectus abdominis myocutaneous flap. Four years after the operation there is no evidence of disease and the patient has a normal sex life. The English literature on this subject is reviewed with special attention to the biological behavior and therapeutic approach.- - - - - - - - - - ranking = 1keywords = sarcoma (Clic here for more details about this article) |
2/149. 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 = 0.20380388455539keywords = sarcoma, spindle (Clic here for more details about this article) |
3/149. 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 = 0.2keywords = sarcoma (Clic here for more details about this article) |
4/149. dermatofibrosarcoma protuberans of the vulva: clinicopathologic and immunohistochemical analysis of four cases, one with fibrosarcomatous change, and review of the literature.Only 18 cases of dermatofibrosarcoma protuberans (DFSP) of the vulva have been reported. This article describes the clinicopathologic and immunohistochemical findings of four additional vulvar DFSPs. The median patient age was 54 years (range, 36 to 69 years) and the most common clinical presentation was a slowly growing vulvar mass. The tumors occurred in the left paraclitoral area (1 case), right labium majus (1 case), left labium majus (1 case), and mons pubis (1 case). The tumor size, known in three cases, ranged from 1.2 to 5.0 cm in greatest dimension. Microscopically, the tumors were composed of bland spindle cells with oval or wavy nuclei arranged in a cartwheel pattern; in one case, fibrosarcomatous areas were apparent. CD34 immunostaining was positive in the three cases in which it was performed; positive staining was also seen in the area of fibrosarcoma. Estrogen and progesterone receptor immunostains were negative in three cases. Of the three patients who underwent surgery, one had tumor recurrences every 2 to 4 years for 18 to 20 years but has remained free of tumor in the 7 years since the last excision, one was free of tumor for 12 years before dying of an unrelated cause, and the other has been free of disease for 6 months. The fourth patient refused treatment and has persistent disease in the vulva. This study shows that the behavior of DFSP in the vulva is similar to its behavior in its more common locations.- - - - - - - - - - ranking = 2.2134341521559keywords = sarcoma, spindle cell, spindle (Clic here for more details about this article) |
5/149. Primary vulvar and vaginal extraosseous Ewing's sarcoma/peripheral neuroectodermal tumor: diagnostic confirmation with CD99 immunostaining and reverse transcriptase-polymerase chain reaction.Two cases of extraosseous Ewing's sarcoma/peripheral neuroectodermal tumor arising in unusual, superficial sites are reported. One tumor involved the vaginal wall of a 35-year-old woman, and the other neoplasm arose in the dermis of the vulva in a 28-year-old woman. The tumors showed characteristic microscopic features of Ewing's sarcoma/peripheral neuroectodermal tumor with nodular monotonous proliferations of undifferentiated, small, round, hyperchromatic cells with a low mitotic index. Rare rosette-like formations were apparent only in the vulvar neoplasm. The tumors displayed intense immunoreactivity in a membranous pattern for CD99, the cell surface glycoprotein encoded by the MIC2 gene. Genetically, the tumors expressed the EWS/FLI-1 chimeric transcript, derived from the t(11;22)(q24;q12) chromosomal translocation. Both patients had localized disease treated with wide local excision; one received postoperative chemotherapy, and the other received chemotherapy and radiotherapy. To date, 18 and 19 months after diagnosis, neither patient has had clinical evidence of local recurrence or metastasis. To our knowledge, these are the first reported cases of vaginal and vulvar Ewing's sarcoma/peripheral neuroectodermal tumor, confirmed with molecular genetic analysis, in the English literature.- - - - - - - - - - ranking = 1.4keywords = sarcoma (Clic here for more details about this article) |
6/149. Atypical solitary fibrous tumor of the vulva.An atypical solitary fibrous tumor (SFT) was encountered as a slow-growing, 15-cm, well-demarcated, vulvar tumor in a 70-year-old woman. The tumor was highly cellular and composed predominantly of hemangiopericytomatous and capillary hemangioma-like proliferations and short fascicular arrangements of spindled cells. Multinucleated giant cells and tumor necrosis also were present. The tumor cells were positive for vimentin, CD34, progesterone receptors, and bcl-2 and were diploid by flow cytometry. The patient was well without disease 9 months after surgery. awareness of the occurrence of atypical SFT in the vulva is important so that confusion with other neoplasms can be avoided.- - - - - - - - - - ranking = 0.0038038845553935keywords = spindle (Clic here for more details about this article) |
7/149. The vulvar mass in the prepubertal child.The differential diagnosis of the vulvar mass in the prepubertal patient is extensive and reported cases of a vulvar hamartoma in the literature are limited. The case presented in this work and review of the literature demonstrate that when considering the differential diagnosis of vulvar masses in the prepubertal female, hamartoma should be included. This review outlines the differential diagnosis of the vulvar mass and gives a comprehensive review of benign masses arising from embryonic remnants, those of mesenchymal origin as well as sarcoma botryoides-embryonal rhabdomyosarcoma and the embryonal sinus tumor.- - - - - - - - - - ranking = 0.4keywords = sarcoma (Clic here for more details about this article) |
8/149. dermatofibrosarcoma protuberans of the vulva: a case report and review of the literature.OBJECTIVE: The purpose of this study is to describe the management of a patient with dermatofibrosarcoma protuberans (DFSP) of the vulva and to review the literature. methods: A 39-year-old was referred by a district hospital for incomplete excision of a vulval mass. The lesion involved the left labium major and measured 8 x 12 cm. The lesion was reexcised with a 3-cm margin of normal skin. RESULTS: The patient made an uneventful postoperative recovery. histology confirmed a diagnosis of DFSP with clear margins. immunohistochemistry was positive for CD34 glycoprotein. CONCLUSIONS: DFSP of the vulva is a rare fibrous tumor of intermediate grade malignancy, with a tendency for local recurrence. However, it rarely metastasizes. Management should be multidisciplinary and Mohs' micrographic surgery is generally advocated to ensure precise margin control. survival rates range from 91 to 100%, and local recurrence rates of 20 to 49% have been reported. Therefore close follow-up is recommended.- - - - - - - - - - ranking = 1keywords = sarcoma (Clic here for more details about this article) |
9/149. A case of dermatofibrosarcoma protuberans of the vulva with a COL1A1/PDGFB fusion identical to a case of giant cell fibroblastoma.dermatofibrosarcoma protuberans (DFSP) is a highly recurrent low-grade soft tissue sarcoma, which is usually located on the trunk. Presentation in the vulva is rare, with only 13 cases being reported to date, none of which have been investigated at the cytogenetic or molecular level. Specific cytogenetic abnormalities, involving chromosomes 17 and 22, are characteristic features of DFSP and giant cell fibroblastoma (GCF), a tumor closely related to DFSP. These chromosomal rearrangements result in the fusion of the COL1A1 and PDGFB genes in both lesions and show wide variation in the position of the fusion point in COL1A1. Here, we describe a case of DFSP of the vulva with a typical monotonous storiform pattern, with no foci of multinucleated giant cells. cytogenetic analysis showed a 47,XX, r karyotype in 50% of the cells, and molecular investigation disclosed the presence of a transcript fusing COL1A1 exon 37 to PDGFB exon 2. This is the first case of DFSP showing such a fusion point, which is intriguingly identical to that found in a GCF case, indicating that the COL1A1/PDGFB fusion point position does not seem to affect tumor morphology. This finding further underlines the very close relationship between these two morphologically distinct entities.- - - - - - - - - - ranking = 1.2002116723008keywords = sarcoma, soft (Clic here for more details about this article) |
10/149. A successful pregnancy outcome in treated vulval rhabdomyosarcoma.A 14 year old girl presented in 1986 with a huge perineal swelling which was progressively increasing in size and associated with loss of weight and loss of appetite. biopsy from the mass revealed rhabdomyosarcoma of the vulva. She was treated with chemotherapy and radium implant. She responded well to the regime. fibrosis of the vulva and vagina caused difficulty in consummation. Once it was corrected, she conceived easily and proceeded to a normal pregnancy and delivery.- - - - - - - - - - ranking = 1keywords = sarcoma (Clic here for more details about this article) |
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