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[宫颈葡萄状横纹肌肉瘤。1例临床病理研究]

[Botryoid rhabdomyosarcoma of the cervix. Clinico-pathologic study of a case].

作者信息

Mainguene C, Hugol D, Caulet S, Ayel S, de Saint-Maur P P, Poitout P, Diebold J

机构信息

Laboratoire central J. Delarue d'Anatomie et de Cytologie Pathologiques, Hôtel-Dieu, Paris.

出版信息

Ann Pathol. 1993;13(1):40-4.

PMID:8489649
Abstract

The botryoid rhabdomyosarcoma of the cervix is a rare tumour occurring in young woman or during genital activity. It mainly causes vaginal bleeding or appears as a polypoid grape-like mass with a gelatinous cut-surface. The diagnosis is based on the presence of a submucosal cambium layer and a rhabdomyoblastic differentiation, corresponding to an intracytoplasmic double cross-striation. Metaplastic cartilaginous islands are sometimes observed. The immunostaining ensures the muscular origin of the tumour, characterized by the expression of actin and desmin. Apart from mullerian adenosarcomas, the main differential diagnosis is represented by the benign polypoid formations of the cervix: the genital rhabdomyoma and the fibroblastic lesion, called fibro-epithelial polyp with atypical stroma. The treatment actually includes both chemotherapy and surgery which is often limited to a conization.

摘要

宫颈葡萄状横纹肌肉瘤是一种罕见肿瘤,发生于年轻女性或性活跃期。它主要引起阴道出血,或表现为息肉样葡萄状肿物,切面呈胶冻状。诊断依据是存在黏膜下层生发层和横纹肌母细胞分化,表现为胞质内双横纹。有时可见化生的软骨岛。免疫组化可确定肿瘤的肌肉起源,其特征为肌动蛋白和结蛋白表达。除苗勒管腺肉瘤外,主要鉴别诊断包括宫颈的良性息肉样病变:生殖性横纹肌瘤和成纤维细胞病变,即伴有非典型间质的纤维上皮息肉。目前的治疗包括化疗和手术,手术通常限于锥切术。

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