Endometrial Stromal Sarcoma with Rhabdoid Differentiation

Fitko, Rosanne; Brainer, James; Schink, Julian C.; August, Carey Z.

doi:10.1097/00004347-199010000-00010

Cited by 32 publications

(12 citation statements)

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“…In contrast, we did not identify cytoplasmic cross-striations, immunoreactivity for myoglobin (or other skeletal muscle markers), or sarcomeric differentiation in the rhabdoid cells of our cases. Pure malignant rhabdoid tumors of the uterus are rare (5)(6)(7)(8)(9)(10), and this diagnosis is one of exclusion as a rhabdoid phenotype has been reported in several uterine tumors, including endometrial stromal sarcomas, epithelioid smooth muscle tumors, leiomyosarcomas, and malignant mixed mullerian tumors (16)(17)(18)(19)(20)(21)(22)(23)(24). Although all of the tumors described in this article exhibited clearly benign features and had a background population of typical leiomyoma cells, the rounded cells of these tumors were reminiscent of those of an extrarenal rhabdoid tumor.…”

Section: Discussionmentioning

confidence: 99%

Skeletal Muscle-like and Rhabdoid Cells in Uterine Leiomyomas

Parker

Young²,

Clement³

2005

International Journal of Gynecological Pathology

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We describe eight unusual uterine leiomyomas characterized by a component of cells that suggested skeletal muscle differentiation or resembled the rhabdoid cells of extrarenal rhabdoid tumors. All of the tumors were referred because of problems in differential diagnosis, particularly distinction from an epithelioid smooth muscle tumor, a smooth muscle tumor of uncertain malignant potential, or a tumor with skeletal muscle differentiation. The patients were aged 27 to 50 (mean, 38) years, and the presenting clinical features and gross appearance of the tumors were similar to those of typical uterine leiomyomas. On microscopic examination, seven of the tumors were well circumscribed, whereas one showed slight irregularity of its margin. The characteristic feature of the tumors was a variable number of rounded, polygonal, or strap-shaped cells with abundant deeply eosinophilic cytoplasm and fibrillar, or occasionally hyaline, intracytoplasmic globules. Cytoplasmic cross-striations were not identified. The cells usually had eccentric, round-to-oval nuclei with conspicuous nucleoli. A variable number of the rhabdoid/skeletal muscle-like cells, as well as cells without these features, contained multiple or multilobed, pleomorphic, hyperchromatic nuclei, thus qualifying the tumors as leiomyomas with bizarre nuclei. Foci of hydropic change were present in all of the tumors. The mitotic index was low (<1 mitotic figure/10 high-power fields) and necrosis was absent in all the tumors. The rhabdoid/skeletal muscle-like cells were immunoreactive for desmin and h-caldesmon, but not for cytokeratin (AE1/AE3) or skeletal muscle markers (myoglobin, Myo-D1, or myogenin). Intracytoplasmic whorls of intermediate filaments were observed in the cells of one case examined by electron microscopy; there was no ultrastructural evidence of skeletal muscle differentiation. The histologic, immunohistochemical, and ultrastructural features indicated that the peculiar cells in these leiomyomas likely represented smooth muscle cells with an unusual phenotype rather than the cells of uterine tumors with skeletal muscle differentiation, extrarenal rhabdoid tumors, or epithelioid smooth muscle tumors. An association with leiomyomas with bizarre nuclei also was suggested.

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Section: Discussionmentioning

confidence: 99%

Skeletal Muscle-like and Rhabdoid Cells in Uterine Leiomyomas

Parker

Young²,

Clement³

2005

International Journal of Gynecological Pathology

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“…In the uterus, both pure MERTs (5) and CERTs have been described. The CERTs have included adenocarcinomas (9) , endometrial stromal sarcomas (4,6,7,10) , or malignant mixed müllerian tumors (MMMT) (8) . In several cases of CERT occasional cells may also express muscle markers, however, this is not as strong and diffuse as encountered in smooth muscle tumors.…”

Section: Discussionmentioning

confidence: 99%

“…Thus, their histopathologic identification is clinically important. In the female genital tract, rhabdoid areas have been noted in tumors of the vulva (2,3) and uterine corpus (4–9) but (to the best of our knowledge) this is the first description of primary cervical carcinomas with rhabdoid phenotype.…”

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confidence: 85%

Cervical carcinoma with rhabdoid phenotype: Report of two cases

Donnellan¹,

Chetty²

2000

Int J Gynecol Cancer

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This report documents two cases of cervical carcinomas having a prominent rhabdoid cell component. In one instance, rhabdoid cells were present as a component of endocervical adenocarcinoma, whereas in the other case, an undifferentiated cervical carcinoma developed a prominent rhabdoid phenotype on recurrence. Like tumors containing rhabdoid elements which have been described at other sites, these neoplasms behaved in an aggressive manner.

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“…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).…”

Section: Pleomorphic Rhabdomyosarcoma Of the Uterine Corpusmentioning

confidence: 99%

“…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…”

Section: Pleomorphic Rhabdomyosarcoma Of the Uterine Corpusmentioning

confidence: 99%

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

Fadare

Bonvicino

Martel

et al. 2010

International Journal of Gynecological Pathology

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We report the clinicopathologic features of 4 cases of pure pleomorphic rhabdomyosarcoma of the uterine corpus with an emphasis on their frequent expression of CD10 and CD56, review the relevant literature, and discuss differential diagnostic considerations. The patients ranged from 51 to 79 years (mean 68 y). All were FIGO stage IIIC to IV at initial surgical staging, and 3 were dead from the disease at an average of 8.6 months follow-up. In addition to the expected findings, other notable morphologic features included tumor giant cells (4/4), osteoclast-like giant cells (1/4), patchy myxoid stroma (4/4), and only infrequent cytoplasmic cross striations (1/4). The tumors in all 4 cases were positive for myogenin, myo-D1, smooth muscle actin, desmin, muscle-specific actin (HHF-35), and CD10; 3 (75%) of 4 cases were positive for calponin and CD56; all cases were negative for cytokeratin 7, synaptophysin, epithelial membrane antigen, placental-like alkaline phosphatase, chromogranin, and a pan-keratin. Twenty-three cases have been reported earlier in the English-language literature between 1969 and 2009. In combination with the current 4, the 27 patients had an age range of 35 to 87 years (mean 66.33 y). Only 1 patient was deemed inoperable; most had staging operations. Following their initial evaluations, 16 (59%) were found to have extrauterine extension of disease. At follow-up, 73% (19/27) were dead from the disease and 19.2% had no evidence of recurrence. Ten (53%) of the 19 deaths occurred within 6.5 months of initial evaluation. Stage at presentation did not have any significant impact on outcome: 73% of the 11 patients with uterus-confined disease at presentation were dead from the disease at follow-up, a rate of disease-associated death that was nearly identical to the 75% in the 16 patients with extrauterine disease at presentation. A wide variety of neoadjuvant and adjuvant therapies were administered, which did not appear to significantly impact outcomes. These data indicate that pleomorphic rhabdomyosarcoma of the uterine corpus is a highly aggressive, rapidly progressive tumor with a high case-fatality rate.

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Endometrial Stromal Sarcoma with Rhabdoid Differentiation

Cited by 32 publications

References 0 publications

Skeletal Muscle-like and Rhabdoid Cells in Uterine Leiomyomas

Skeletal Muscle-like and Rhabdoid Cells in Uterine Leiomyomas

Cervical carcinoma with rhabdoid phenotype: Report of two cases

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

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