Primary metastasized extraskeletal Ewing sarcoma of the vulva: Report of a case and review of the literature

Department of Obstetrics and Gynecology, University Hospital of Schleswig-Holstein, Campus Luebeck, Ratzeburger Allee 160, 23538 Luebeck, Germany.
Archives of Gynecology (Impact Factor: 1.36). 07/2011; 285(3):785-9. DOI: 10.1007/s00404-011-2011-x
Source: PubMed


Ewing's sarcoma (ES) and primitive neuroectodermal tumor (PNET) are closely related malignant neoplasms, usually affecting the skeletal system. Extraosseous ES/PNETs are uncommon, with occasional reports of tumors affecting the genitourinary tract. Only few cases of primary vulvar Ewing's sarcoma/PNET have previously been reported.
We present a patient with primary vulvar Ewing's sarcoma with pulmonary metastasis who presented at the Department of Gynecology and Obstetrics, University of Schleswig-Holstein, Campus Luebeck.
The present report is the third case of a primary vulvar Ewing's sarcoma and the first constellation with pulmonary metastasis at diagnosis in the current literature. We present the diagnostic and therapeutic management including surgery, chemotherapy and radiation.
The treatment of the Ewing's sarcomas and PNETs requires a multidisciplinary systemic approach. Despite its rarity, the differential diagnosis of vulvar sarcoma must be considered in young women.

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    ABSTRACT: Ewing sarcoma/primitive neuroectodermal tumors (ES/PNET) are aggressive malignancies characterized by translocation involving the EWS gene. Primary extraskeletal ES/PNET rarely affects the female genital tract. We report the case of a 20-year-old female, who presented with a rapidly growing painful mass arising from the left labium majus for six months. Multiple lesions in bones and nodules in lungs were considered metastasis. The diagnosis was established by immunohistochemical stain and reverse transcriptase polymerase chain reaction analysis. The patient ceased treatment and died of respiratory failure one month after discharge. Vulvar ES/PNET mainly affects adolescent females. ES/PNET should be considered in the differential diagnosis of tumors involving the lower gynecologic tract in adolescent female.
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    ABSTRACT: To study the clinicopathologic features of primitive neuroectodermal tumor (PNET) in female genital tract. Six cases of PNET arising in female genital tract were retrospectively reviewed. The clinicopathologic features, immunohistochemical findings and EWS gene translocation study results were analyzed. The age of patients ranged from 10 to 27 years (mean = 20 years). The sites of involvement included ovary (1 case), uterus (1 case), vulva (2 cases) and vagina (2 cases). The greatest diameter of the tumor ranged from 2 to 10 cm (mean = 5.4 cm). The tumor had nodular appearance and showed grayish-pink fleshy cut surface, accompanied by foci of hemorrhage and necrosis. Histologically, the tumor was composed of malignant small round cells with indistinct cell borders, hyperchromatic nuclei, dense chromatin, tiny nucleoli and scanty cytoplasm. The tumor cells were arranged in sheets or lobules. Homer-Wright rosettes were identified in 1 case. Immunohistochemical study showed that the tumor cells were positive for CD99, FLI-1 and CD56 (6/6). Focal expression of vimentin (5/6), NSE (5/6), nestin (4/6), synaptophysin (4/6), S-100 protein (2/6) and chromogranin A (1/6) was also demonstrated. EWS gene translocation was detected in 5 cases studied. Follow-up information was available in 2 patients (7 and 17 months of follow up, respectively). One of them died of tumor metastasis 17 months after diagnosis. The other patient was still alive. PNET arising in female genital tract is rare. It mainly involves ovary, uterus, vulva and vagina. Immunohistochemical study using a panel of antibodies and fluorescence in-situ hybridization play an important role in definitive diagnosis of this rare malignancy.
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    ABSTRACT: Malignant tumors of the female reproductive system are a serious health and social problem, as they are the second most common cause of death among women, after breast cancer. Vulvar tumors represent only 4% of all gynecological neoplasms, and they are fourth in frequency after tumors of the cervix, uterus, and ovary. Ninety-eight percent of all vulvar tumors are benign and only 2% are malignant. Sarcomas of the vulva comprise approximately 1-3% of all vulvar cancers. They are characterized by rapid growth, high metastatic potential, frequent recurrences, aggressive behavior, and high mortality rate. In Part 1 of this paper, we presented the most common forms of sarcoma of the vulva: leiomyosarcoma, epithelioid sarcoma, malignant rhabdoid tumor, and rhabdomyosarcoma. The second part of this review will focus mainly on the rarest variants of vulvar sarcoma: low-grade fibromyxoid sarcoma, synovial sarcoma, monophasic synovial sarcoma, carcinosarcoma, Ewing sarcoma, myeloid sarcoma, dermatofibrosarcoma protuberans, malignant fibrous histiocytoma, angiomatoid fibrous histiocytoma, liposarcoma, malignant peripheral nerve sheath tumor, and malignant mesothelioma. © The Author(s) 2015.
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