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Rhabdomyosarcoma of the uterine cervix. Sarcoma botryoides

✍ Scribed by Ely Brand; Jonathan S. Berek; Roberta K. Nieberg; Neville F. Hacker


Publisher
John Wiley and Sons
Year
1987
Tongue
English
Weight
931 KB
Volume
60
Category
Article
ISSN
0008-543X

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✦ Synopsis


Twenty-one cases of sarcoma botryoides of the uterine cervix, including four previously unreported cases, are reviewed. The age of the patients ranged from 5 months to 48 years, with a peak incidence in the group aged 14 to 18 years. Eighty percent of the patients are alive, with a mean follow-up period of 68 months. Seventy-five percent of the patients had Group I disease, of whom 88% are alive. Eleven of 14 patients (79%) receiving vincristine and dactinomycin based chemotherapy are alive. There were five patients with recurrent disease (24%) of whom two (40%) are alive. The prognosis for cervical sarcoma botryoides is similar to that of other female genital tract embryonal rhabdomyosarcomas. Primary therapy should consist of vincristine and dactinomycin based chemotherapy. Surgery should be guided by the response to initial chemotherapy and should attempt to conserve the function of the bladder, rectum, vagina, and ovaries.

Cancer 609552-1 560, 1987.

HABDOMYOSARCOMA is one of the commonest soft R tissue tumors in children. After the head and neck, genitourinary rhabdomyosarcoma is the second most common site.' There are four major types of rhabdomyosarcoma: embryonal, alveolar, pleomorphic, and undifferentiated. A subtype of embryonal rhabdomyosarcoma is often denoted by the descriptive term "sarcoma botryoides," because of a layer of spindle cells pushing up beneath the mucosa in polypoid masses.

Rhabdomyosarcomas are heterologous tumors because skeletal muscle is not normally found in the genital tract. The skeletal muscle or precursor myoblasts may sometimes be mixed with other mesenchymal elements, usually cartilage. Twenty percent of rhabdomyosarcomas arise in the genitourinary tract, with slightly more than half being embryonal rhabdomyosarcomas.','

One commonly thinks of a grape-like mass protruding from the introitus of a young infant upon hearing the term "sarcoma botryoides." However, there is a second peak in the bimodal incidence curve among adolescents. Simpson, and later Ober, theorized that rhabdomyosarcomas arise higher in the genital tract at each major From the


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