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外阴脂肪性血管肌纤维母细胞瘤:诊断与组织发生学考量

Lipomatous angiomyofibroblastoma of the vulva: diagnostic and histogenetic considerations.

作者信息

Magro G, Salvatorelli L, Angelico G, Vecchio G M, Caltabiano R

出版信息

Pathologica. 2014 Dec;106(4):322-6.

PMID:25845047
Abstract

We report a rare case of angiomyofibroblastoma (AMFB) of the vulva, composed predominantly of a mature fatty component, representing approximately 60% of the entire tumour. The tumour, designated as "lipomatous AMFB", should be interpreted as the morphological result of an unbalanced bidirectional differentiation of the presumptive precursor stromal cell resident in the hormonally-responsive stroma of the lower genital tract, with the adipocytic component overwhelming the fibroblastic/myofibroblastic one. The close admixture of adipocytes with spindled/epithelioid cells of the conventional AMFB resulted, focally, in a pseudo-infiltrative growth pattern and pseudo-lipoblast-like appearance, raising problems in differential diagnosis, especially with well-differentiated lipoma-like liposarcoma and spindle cell liposarcoma. Awareness of the possibility that vulvo-vaginal AMFB may contain large amount of lipomatous component is crucial to avoid confusion with other bland-looking spindle cell tumours containing infiltrating fat.

摘要

我们报告了一例罕见的外阴血管肌纤维母细胞瘤(AMFB),其主要由成熟脂肪成分构成,约占整个肿瘤的60%。该肿瘤被命名为“脂肪瘤样AMFB”,应被理解为下生殖道激素反应性基质中假定的前体基质细胞双向分化失衡的形态学结果,其中脂肪细胞成分压倒了成纤维细胞/肌成纤维细胞成分。脂肪细胞与传统AMFB的梭形/上皮样细胞紧密混合,局部形成假浸润性生长模式和假脂母细胞样外观,这在鉴别诊断中引发了问题,尤其是与高分化脂肪瘤样脂肪肉瘤和梭形细胞脂肪肉瘤的鉴别。认识到外阴阴道AMFB可能含有大量脂肪成分这一可能性,对于避免与其他含有浸润性脂肪的外观平淡的梭形细胞肿瘤相混淆至关重要。

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