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双侧弥漫性假血管瘤样间质增生(PASH)导致一名33岁孕妇巨乳症:病例报告

Bilateral Diffuse Pseudoangiomatous Stromal Hyperplasia (PASH) Causing Gigantomastia in a 33-Year-Old Pregnant Woman: Case Report.

作者信息

Krawczyk Natalia, Fehm Tanja, Ruckhäberle Eugen, Mohrmann Svjetlana, Riemer Jasmin, Braunstein Stefan, Hoffmann Jürgen

机构信息

Department of Obstetrics and Gynaecology, Heinrich-Heine University, Düsseldorf, Germany, Düsseldorf, Germany.

Department of Pathology, Heinrich-Heine University, Düsseldorf, Germany.

出版信息

Breast Care (Basel). 2016 Oct;11(5):356-358. doi: 10.1159/000450867. Epub 2016 Oct 24.

Abstract

Pseudoangiomatous stromal hyperplasia (PASH) is a benign proliferation of mammary stroma mostly described as an incidental microscopic finding. Clinically, it can manifest as a palpable, well-circumscribed breast mass or in rare cases as a diffuse bilateral process causing massive breast enlargement. The most postulated theory for aetiology of this condition is hormonal stimulation of mammary myofibroblasts, particularly by progesterone. A definite diagnosis of PASH is based on typical pathological findings like stromal hyperplasia and empty slit-like channels positive for myofibroblastic and negative for endothelial markers. The main clinical differential diagnosis is a fibroadedoma or phylloid tumour, and histologically a low-grade angiosarcoma. There are less than 200 cases of tumorous PASH and less than 20 of diffuse PASH reported so far. Here we present a case report of huge diffuse PASH, that is, to our knowledge, the first in a pregnant woman.

摘要

假血管瘤样间质增生(PASH)是一种乳腺间质的良性增生,大多作为偶然的显微镜下发现被描述。临床上,它可表现为可触及的、边界清楚的乳腺肿块,或在罕见情况下表现为导致乳腺大量增大的弥漫性双侧病变。关于这种情况的病因,最被假定的理论是激素对乳腺肌成纤维细胞的刺激,尤其是孕酮。PASH的明确诊断基于典型的病理表现,如间质增生以及肌成纤维细胞呈阳性而内皮标志物呈阴性的空的裂隙样通道。主要的临床鉴别诊断是纤维腺瘤或叶状肿瘤,在组织学上是低级别血管肉瘤。到目前为止,报道的肿瘤性PASH病例少于200例,弥漫性PASH病例少于20例。在此,我们报告一例巨大弥漫性PASH病例,据我们所知,这是首例发生在孕妇身上的病例。

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