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

Abstract

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.

Amelanotic Malignant Melanoma of Unknown Primary Origin Metastasizing to the Bone Marrow: A Case Report and Review of the Literature

“…CD56 is a cell adhesion protein called NCAM1 (neural cell adhesion molecule 1) that is known to be expressed in hematological malignancies, such as NK/T cell neoplasms, malignant myeloma and some cases of acute leukemia. However, CD56 is also expressed in some nonhematological malignancies, such as melanoma (17), small cell carcinoma (18), rhabdomyosarcoma (19), Merkel cell carcinoma (20), neuroblastoma (21) and neuroendocrine tumors/carcinoma (22).…”

Primary Ovarian Rhabdomyosarcoma in a Dog

“…In man and animals rhabdomyosarcomas are classified as embryonal, botryoid, alveolar or pleomorphic on the basis of microscopical appearance (Cooper and Valentine, 2002). Human rhabdomyosarcomas generally arise in the head, neck and extremities (MacArthur et al, 1992;Casanova et al, 2009;Aslam et al, 2010;Bolger et al, 2010;Wagemans et al, 2010); however, rhabdomyosarcomas can also arise in organs that do not have striated muscle such as the genitourinary tract, including the ovaries (Barris and Shaw, 1929;Chan et al, 1989;Allende and Yang, 2008;Childs et al, 2008;Cribbs et al, 2008;Fadare et al, 2010). In domestic animals there have been reports of rhabdomyosarcoma in the genitourinary tract (Kim et al, 1996;Kuwamura et al, 1998;Suzuki et al, 2006;Bae et al, 2007), but not in the ovaries.…”

Misdiagnosis of primary pleomorphic rhabdomyosarcoma of the right thigh in a young adult: A case report

“…PRMS was first described by Stout in 1946 (14). Primary PRMS is relatively rare and primarily affects adults in the fifth decade of life (15). Its occurrence in young adults, as in the present case, is extremely rare.…”