Ben Rejeb A, Jabbes H, Essoussi M, Othmani S, Alaya M B, Bahri M
Service d'Anatomie Pathologique, Hôpital militaire de Tunis, Tunisie.
Ann Pathol. 1994;14(3):163-7.
We report a second case of gallbladder embryonal rhabdomyosarcoma diagnosed in a 54 year old female. We had previously published a first similar case in a 48 year old male. In the review of the literature 5 other cases were collected. Five of the 7 cases occurred in 4 females and one male adult mean aged 63.4 years, and 2 in young girls aged 40 months. Three cases were of botryoid type, three were of alveolar type and one was of undetermined type. Immunohistochemistry demonstrated intra-cytoplasmic positivity of tumoral cells with anti-vimentin, anti-desmin and anti-actin anti-bodies. The prognosis remains bad. Death occurred, in 5 patients, in a period going from 18 days to 8 months after clinical diagnosis with a 5 months mean survival. The shortest survival was observed in a 40 month old child with AIDS. Only one case with surgical resection followed by radiochemotherapy had a 9 month survival.
我们报告了第二例在一名54岁女性中诊断出的胆囊胚胎性横纹肌肉瘤。我们之前曾发表过第一例类似病例,患者为一名48岁男性。在文献回顾中,我们收集到了另外5例病例。7例病例中,5例发生在4名女性和1名男性成年人中,平均年龄为63.4岁,2例发生在40个月大的年轻女孩中。3例为葡萄状型,3例为肺泡型,1例类型未确定。免疫组织化学显示肿瘤细胞胞浆内对波形蛋白、结蛋白和肌动蛋白抗体呈阳性。预后仍然很差。5例患者在临床诊断后的18天至8个月内死亡,平均生存时间为5个月。最短生存期出现在一名患有艾滋病的40个月大儿童中。只有1例手术切除后接受放化疗的患者存活了9个月。