Advanced alveolar rhabdomyosarcoma of the uterus: a case report.
Alveolar rhabdomyosarcoma is an uncommon malignant soft tissue tumour rarely found in the female genital tract and carries a very poor prognosis especially in adults. A 44 year old premenopausal woman was evaluated for a lower abdominal mass, intermittent unprovoked vaginal bleeding and weight loss. Examination showed a huge abdomino-pelvic mass, and an irregular cauliflower mass protruding from the cervix, from which a biopsy was taken. Exploratory laparotomy revealed a widespread disseminated uterine tumour with an "omental cake" and involving virtually all the abdominal viscera. Debulking surgery was found to be non-beneficial and uterine and omental biopsies were taken. The cervical, uterine and omental biopsies were all diagnosed alveolar rhabdomyosarcoma. Neither chemotherapy nor radiotherapy could be started before the client died of fulminant neoplastic dissemination 17 days postoperatively. While the optimal management of this rare tumour is unknown, early recognition and diagnosis, and a prompt multimodality treatment approach of surgery, chemotherapy and radiotherapy offers the best chance of cure.
Odoi
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Department of Obstetrics and Gynaecology, Komfo Anokye Teaching Hospital, Kumasi, Ghana. dratodoi@yahoo.com
Dassah
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Darkey
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Owusu-Afriyie
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Valkov
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D000328:Adult; D017809:Fatal Outcome; D005260:Female; D005869:Ghana; D006801:Humans; D007150:Immunohistochemistry; D009852:Omentum / Q000473:pathology*; D015514:Oncogene Proteins, Fusion / Q000378:metabolism; D010534:Peritoneal Neoplasms / Q000473:pathology*; D018232:Rhabdomyosarcoma, Alveolar / Q000473:pathology*; D014594:Uterine Neoplasms / Q000473:pathology*
D015514:Oncogene Proteins, Fusion; C523321:PAX3-FKHR fusion protein, human