Epithelioid leiomyosarcoma with rhabdoid features

Abstract

Leiomyosarcomas (LMS) are the most common type of uterine sarcoma. Most LMS have typical histologic features, and variants such as epithelioid LMS, myxoid LMS, LMS with osteoclast-like giant cells and LMS with rhabdoid features occur only rarely. Rhabdoid cells were first described in rhabdoid tumor, a distinctive renal neoplasm of infancy. Such tumors are composed of diffuse proliferation of rhabdoid cells that are round or polygonal in shape with eccentric nuclei, prominent nucleoli and glassy eosinophilic cytoplasm containing hyaline-like inclusion bodies. In the literature, extrarenal localizations of malign rhabdoid tumors have been described in a variety of primary sites such as the central nervous system, liver, skin and soft tissues. These characteristic rhabdoid cells have been reported in sarcomas and carcinomas of various types and in a few cases of uterine sarcomas. The presence of rhabdoid cells in tumors is considered to be a predictor of aggressive tumor behavior. Our case is that of a 56-year-old woman who was admitted to the state hospital with left inguinal mass. Microscopically the tumor was admixed of three different types of cell with spindle, epithelioid or rhabdoid features. Immunopositive cytoplasmic staining for myoglobulin and desmin was seen in rhabdoid cells, and cytokeratin immunopositivity was observed in epithelioid and some rhabdoid cells. Epithelioid cells and spindle cells were also SMA positive. The histopathologic and immunohistochemical findings support the diagnosis of epithelioid LMS with rhabdoid features. We report this very uncommon LMS variant; to the best of our knowledge there are only a few cases in the English literature.

Prognostic significance of preoperative 18F-FDG PET/CT in uterine leiomyosarcoma

“…LMS is characterized histologically as epithelial, myxoid, and rhabdoid, containing osteoclast-like giant cells which may account for variable clinical behavior [5]. Reported symptoms in patients with LMS are irregular bleeding and rapidly enlarging pelvic mass.…”

Pleomorphic Rhabdomyosarcoma of the Uterine Corpus: A Clinicopathologic Study of 4 Cases and a Review of the Literature

“…Furthermore, unlike pleomorphic rhabdomyosarcoma, MERT lack expression of Myo-D1 and myogenin, and frequently express cytokeratins. Epithelioid leiomyosarcoma (48,49), pleomorphic leiomyosarcoma (50), ESS (56)(57)(58), uterine tumors resembling ovarian sex cord tumor (55), and atypical leiomyomas (60), have all been reported to contain varying amounts of rhabdoid cells, and therefore fall within the diagnostic umbrella of uterine composite extrarenal rhabdoid tumors. All can be distinguished from the rhabdomyosarcoma by recognizing the morphologic and immunophenotypic properties…”

“…The differential diagnosis for pleomorphic rhabdomyosarcoma of the uterus includes rhabdomyosarcomatous overgrowth of a mu¨llerian adenosarcoma (42)(43)(44)(45), carcinosarcoma with rhabdomyosarcomatous heterologous elements (46,47), epithelioid leiomyosarcoma with rhabdoid features (48,49), pleomorphic leiomyosarcoma with or without rhabdoid cells (50), malignant extrarrenal rhabdoid tumor of the uterus (51-55), endometrial stromal sarcoma (ESS) with rhabdoid features (56)(57)(58), hybrid leiomyosarcoma/ rhabdomyosarcoma (59), uterine tumors resembling ovarian sex cord tumor with rhabdoid cells (55), atypical leiomyoma (leiomyoma with bizarre nuclei) with skeletal muscle-like or rhabdoid cells (60), and anaplastic embryonal or anaplastic alveolar rhabdomyosarcoma (61,62).…”

Heterologous and Rare Homologous Sarcomas of the Uterine Corpus

“…199,200 A diverse array of uterine tumors have been reported to contain rhabdoid cells, including epithelioid leiomyosarcoma, pleomorphic leiomyosarcoma, endometrial stromal sarcoma, endometrioid adenocarcinoma, carcinosarcoma, uterine tumors resembling ovarian sex cord tumor, and atypical leiomyomas. [99][100][101][102][103][104][105][106][107][108][109][110][111]201,202 Six uterine sarcomas that fit the MERT designation have been reported, in women ranging in age from 39 to 71 years. [103][104][105][106][107]202 Most had displayed extrauterine extension at presentation, and the disease was ultimately fatal in most.…”