Anaplastic (pleomorphic) subtype embryonal rhabdomyosarcoma of the cervix

Dipartimento di Patologia Umana, Policlinico Universitario, Messina, Italy.
Archives of Gynecology and Obstetrics (Impact Factor: 1.36). 01/2005; 270(4):278-80. DOI: 10.1007/s00404-003-0504-y
Source: PubMed


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 Where applicable, supplemental and unpublished data from the files of the Armed Forces Institute of Pathology (Washington, DC) are also detailed.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.
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