Anaplastic (pleomorphic) subtype embryonal rhabdomyosarcoma of the cervix.
ABSTRACT INTRODUCTION: Embryonal rhabdomyosarcoma (RMS) of the female genital tract usually occurs in the vagina during childhood. In rare cases, RMS can originate in the uterine cervix, with an incidence peak in the second decade. Recent studies have suggested that it is possible to limit surgery to local excision in stage I cases. CASE REPORT: We present the case of a 13-year-old girl diagnosed with an anaplastic (pleomorphic) subtype embryonal RMS of the endocervix, who was treated successfully with polypectomy followed by ifosfamide-vincristine-actinomycin combination chemotherapy and brachytherapy. The patient exhibits no evidence of recurrence and has normal menstrual function 36 months following initial diagnosis.
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ABSTRACT: In general, mesenchymal tumors are only rarely encountered in the uterine cervix, where they constitute less than 1% of all malignancies. In this report, the salient clinicopathologic features of the reported cases of primary mesenchymal lesions of the cervix are comprehensively reviewed. Included are lesions displaying skeletal muscle, smooth muscle, adipocytic, myofibroblastic, fibrohistiocytic, fibroblastic, neural, nerve sheath, neuroectodermal and vascular/pericytic differentiation, as well as those whose line(s) of differentiation is (are) currently uncertain. Where applicable, supplemental and unpublished data from the files of the Armed Forces Institute of Pathology (Washington, DC) are also detailed. For the published cases, the overall ratio of benign to malignant cases is approximately 1.9:1. The most commonly reported mesenchymal malignancies, listed in order of decreasing frequency, are embryonal rhabdomyosarcoma (approximately 150 cases), leiomyosarcoma (approximately 30 cases), undifferentiated endocervical sarcoma (17 cases), alveolar soft part sarcoma (11 cases), Ewing sarcoma (9 cases), malignant peripheral nerve sheath tumor (8 cases), and liposarcoma (4 cases). The most commonly reported benign mesenchymal tumors, reported with almost equal frequency, are hemangioma and leiomyoma. Each of the aforementioned lesions is associated with a relatively distinct clinicopathologic profile. Generalizations about the poor prognosis associated with “cervical sarcomas” are therefore no longer appropriate, and each case must be evaluated within the context of the reported cases of that specific histologic subtype.Pathology Case Reviews 04/2006; 11(3):140-152. DOI:10.1097/01.pcr.0000217868.84282.77
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ABSTRACT: In its classical form, embryonal rhabdomyosarcoma (ERMS, botryoid type) is a vaginal neoplasm occurring in infants and young girls and is often not considered in the differential diagnosis of uterine corpus and cervical spindle cell tumors in adult women. Clinicopathologic and immunohistochemical features of 25 cases of ERMS in women 20 years of age or older were analyzed. Patient age ranged from 20 to 89 years (mean, 44.4 y; median, 46 y), with 8 patients aged 20 to 39 years, 14 patients aged 40 to 59 years, and 3 patients older than 60 years of age. Tumors originated in the cervix in 20 cases and in the uterine corpus in 5. They were characterized by an edematous hypocellular spindle cell proliferation, typically with cellular condensation beneath epithelial surfaces (cambium layer), in which tightly packed hypercellular foci were scattered. Neoplastic cells had hyperchromatic nuclei and minimal cytoplasm, usually with delicate cytoplasmic processes. Occasionally, elongated or globular cells with eosinophilic cytoplasm (rhabdomyoblasts) were evident, but cytoplasmic cross-striations were only rarely identified. Apoptotic bodies and mitotic figures were usually identified in the hypercellular foci. Hemorrhage was common, often making recognition of the hypercellular foci difficult. Desmin and myogenin were coexpressed in 22 of 23 (95.6%) tumors evaluated. Proliferative activity, as assessed by Ki-67 expression, was notably elevated in all tumors evaluated, typically concentrated in the hypercellular foci. Estrogen and progesterone receptors were expressed focally in only 3 of 12 (25%) and 1 of 8 (12.5%) tumors evaluated, respectively. Follow-up was available in 7 cases. Five patients were alive without evidence of disease with follow-up of 3 to 8 years, and 1 patient was alive with disease at 5 months. One patient died at 5 months with pulmonary nodules, but it was not determined whether this was due to metastatic ERMS or the patient's known ductal breast carcinoma. ERMS has a broader clinical profile than classically expected and should be considered in the differential diagnosis of a uterine corpus or cervical spindle cell tumor, regardless of patient age. Recognition can be rendered difficult by the hypocellular background, which can suggest a benign polyp or low-grade tumor, and hemorrhage, which can obscure the characteristic hypercellular foci. Identification of hypercellular foci in which mitotic activity and apoptotic bodies are found, desmin and myogenin are coexpressed, proliferative activity is notably elevated, and hormone receptor expression is usually absent is very useful for establishing the diagnosis.The American journal of surgical pathology 01/2013; 37(3). DOI:10.1097/PAS.0b013e31826e0271 · 4.59 Impact Factor
Journal of Obstetrics and Gynaecology 10/2012; 32(7):709-11. DOI:10.3109/01443615.2012.698663 · 0.60 Impact Factor