Malignant extrarenal rhabdoid tumor of the vulva in an adult

Abstract

Sarcomas of the vulva account for only 1-3% of all vulvar malignancies. Most common vulvar sarcomas are leiomyosarcomas, malignant fibrohistiocytomas, and aggressive angiomyxomas. Malignant rhabdoid tumor (MRT) of the kidney is a distinctive clinicopathological entity that is recognized as a highly aggressive renal tumor of childhood. Extrarenal malignant rhabdoid tumors have been proposed to exist at several sites, including soft parts. MRT of the vulva is a rare and very aggressive neoplasm. Median survival reported in other studies is 9 months. Only 10 cases have been reported thus far in the English literature. We are reporting the 11 th case who remains disease free 30 months following surgery and radiotherapy till the time of reporting.

Primary Vulval Rhabdoid Tumor in an Adult: A Case Report, Immunohistochemical Profile and Literature Review

“…The age of the patient, the site of the tumor, the clinical presentation, and the microscopic findings in our case were compatible with the 12 previously  reported cases (Table 1) [1–10]. The IHC profile varied between cases, and the only universal finding was immunoreactivity to vimentin.…”

“…EMRTs were described in various anatomical sites, and the use of INI1 immunostain has recently emerged as an important adjunct for identifying these tumors. An extensive online search of the literature revealed very few reported cases of EMRT of the vulva [1–10]. We hereby report another case and the first in which the INI1, ER, and PR immunostains were utilized.…”

Vulvar sarcomas: Short guideline for histopathological recognition and clinical management. Part 1

“…41 It is usually observed in the labia majora, mons pubis, and clitoris. 37 It clinically presents as an asymptomatic, well-circumscribed, solid tumor mass, with size in the range of 2-10 cm. 39 The diagnosis is confirmed histologically by the characteristic finding of diffuse proliferation of 'rhabdoid cells'; that is, rounded or polygonal cells with eccentric nuclei, prominent nucleoli, and glassy eosinophilic cytoplasm containing hyalinelike inclusion bodies, arranged in sheets and nests.…”

“…39 Most MRTs express vimentin, followed by EMA, cytokeratin, and CD 99, but not desmin. 37 The differentiation of the MRT from epitheloid sarcoma is difficult, sometimes impossible, because of their overlapping clinical and histological features. 42 According to some authors, MRT is a variant of epithelioid sarcoma, with a more aggressive course and a poor prognosis.…”

Cutaneous Malignant Rhabdoid Tumor in the Palm of an Adult

“…Histologically, it showed a solid arrangement of large, rhabdoid-like cells 7 . Distinction from proximal-type epitheloid sarcoma is difficult, because both of these mesenchymal tumors exhibit epithelial profiles such as immunohistochemical expression of cytokeratin and EMA 8 . They also share ultrastructural features such as specialized junctions, tonofilaments, and microvilli indicative of epithelial differentiation 9 .…”