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子宫高级别肺泡状横纹肌肉瘤:一例报告

Advanced alveolar rhabdomyosarcoma of the uterus: a case report.

作者信息

Odoi A T, Dassah E T, Darkey D E, Owusu-Afriyie O, Valkov A Y

机构信息

Department of Obstetrics and Gynaecology, Komfo Anokye Teaching Hospital, Kumasi, Ghana.

出版信息

Afr J Reprod Health. 2009 Mar;13(1):167-73.

Abstract

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.

摘要

肺泡状横纹肌肉瘤是一种罕见的恶性软组织肿瘤,很少见于女性生殖道,预后很差,尤其是在成年人中。一名44岁的绝经前女性因下腹部肿块、间歇性无故阴道出血和体重减轻接受评估。检查发现有一个巨大的腹盆腔肿块,宫颈有一个不规则的菜花状肿块,并从中取了活检。剖腹探查显示子宫肿瘤广泛播散,形成“网膜饼”,几乎累及所有腹部脏器。发现减瘤手术无益处,遂取了子宫和网膜活检。宫颈、子宫和网膜活检均诊断为肺泡状横纹肌肉瘤。在患者术后17天死于暴发性肿瘤播散之前,既无法开始化疗也无法开始放疗。虽然这种罕见肿瘤的最佳治疗方法尚不清楚,但早期识别和诊断以及迅速采用手术、化疗和放疗的多模式治疗方法提供了最佳的治愈机会。

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