Uterine/Cervical Embryonal Rhabdomyosarcoma in Adult Women.
Fanghong Li, Mamta Gupta, Brigitte M Ronnett. Johns Hopkins Hospital, Baltimore, MD
Background: In its classical form, embryonal rhabdomyosarcoma (ERMS) is a vaginal tumor occurring in young girls and is often not considered in the differential diagnosis of uterine and cervical spindle cell tumors in adult women. Experience with consultation cases indicates a need for improved recognition of this tumor.
Design: Clinicopathologic and immunohistochemical features of 12 cases of ERMS identified in women >20 years of age were evaluated, with the goal of improving recognition of this tumor outside of its classical setting.
Results: Patient age ranged from 23 to 86 years (mean, 49.4 ; median,48.5), with 2 patients aged 20-29 years, no patients aged 30-39 years, 5 patients aged 40-49 years, and 5 patients >50 years of age. Tumors originated in the cervix in 7 cases and in the endometrium in 5. These were diagnosed by biopsy in 4, curettage in 1, polypectomy in 6, and hysterectomy in 1. All endometrial tumors and 5 of 7 cervical tumors occurred in women >40 years of age. Tumors were characterized by a spindle cell proliferation in which tightly packed hypercellular foci composed of cells with minimal cytoplasm were scattered throughout an edematous hypocellular spindle cell proliferation, often with condensation beneath epithelial surfaces. Apoptotic bodies and mitotic figures were evident, usually in the hypercellular foci. In 4 tumors, rare to few entrapped endocervical or endometrial type glands were present but these tumors lacked the classical features of adenosarcoma. All tumors coexpressed desmin and myogenin to some degree (desmin was diffuse in 9 and focal in 3; myogenin was diffuse in 6 and focal in 6). Proliferative activity, as assessed by Ki-67 expression, was notably elevated in all tumors, typically concentrated in the hyperpcellular foci.
Conclusions: ERMS has a broader clinical profile than classically expected and can occur as a uterine (endometrial) or cervical tumor in adult women. Thus, ERMS should be considered in the differential diagnosis of uterine/cervical spindle cell tumors, regardless of patient age. The hypocellular background can suggest a low-grade tumor but recognition of characteristic hyperceullular foci in which desmin and myogenin are coexpressed and proliferative activity is elevated is useful for establishing the diagnosis.
Category: Gynecologic & Obstetrics
Wednesday, March 2, 2011 1:00 PM
Poster Session VI # 188, Wednesday Afternoon