Poncelet C, Boccara J, Walker-Combrouze F, Féraud O, Madelenat P
Service de gynécologie-obstétrique, hôpital Bichat-Claude-Bernard, 46, rue Henri-Huchard, 75018 Paris, France.
Gynecol Obstet Fertil. 2004 Jan;32(1):46-8. doi: 10.1016/j.gyobfe.2003.11.003.
Liposarcoma of the vulva is a rare entity. This unusual localization with atypical clinical and histological appearance may induce diagnostic and treatment delay. We report the 13th case shown in the literature in a 31-year-old woman initially treated for a vulvar lipoma. Arguments based on clinical short term recurrence, histological infiltrating adipocytes, and cytogenentical findings evoked well-differentiated liposarcoma. Even though cytogenetic abnormalities, involving MDM2 and CDK4 genes, have been found, a certainty in malignity diagnosis could be difficult. In these cases, treatment decision may be uneasy. This case report recalls difficulties encountered in uterine hypercellular leiomyomas.
外阴脂肪肉瘤是一种罕见的疾病。这种不寻常的部位伴有非典型的临床和组织学表现，可能导致诊断和治疗延误。我们报告了文献中第13例病例，患者为一名31岁女性，最初因外阴脂肪瘤接受治疗。基于临床短期复发、组织学上浸润性脂肪细胞以及细胞遗传学结果，提示为高分化脂肪肉瘤。尽管已发现涉及MDM2和CDK4基因的细胞遗传学异常，但恶性诊断仍可能存在困难。在这些病例中，治疗决策可能不易做出。本病例报告回顾了子宫高细胞平滑肌瘤中遇到的困难。
