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病例报告:多伊奇-波特综合征:一种导致严重低血糖的巨大孤立性纤维性胸膜肿瘤。

Case Report: Doege-Potter syndrome: a giant solitary fibrous pleural tumor causing severe hypoglycemia.

作者信息

De Vico Christelle, Tappero Carlo, Fontana Enzo, Lutz Jon Andri, Rouiller Benoît

机构信息

Unit of Thoracic Surgery, Department of Surgery, Fribourg Cantonal Hospital, Fribourg, Switzerland.

Faculty of Science and Medicine, University of Fribourg, Fribourg, Switzerland.

出版信息

Front Oncol. 2025 Jun 4;15:1550343. doi: 10.3389/fonc.2025.1550343. eCollection 2025.

Abstract

Doege-Potter syndrome or non-islet cell tumor hypoglycemia (NICTH) is a rare entity usually due to a solitary fibrous tumor of the pleura (pSFT). The diagnosis of NICTH is challenging because of its non-specific clinical presentation and the rarity of pSFT. We present a case report of a 44-year-old woman diagnosed with NICTH to demonstrate the difficulty of establishing this diagnosis and the importance of preoperative preparation based on the characteristics of the lesion. We performed an embolization followed by an en bloc resection with diaphragmatic patch and lung wedge resection, and we subsequently reconstructed the diaphragm with Prolene mesh through a left hemiclamshell incision. The postoperative course was favorable, particularly the resolution of hypoglycemia, which was concordant with the diagnosis. Pathological examination revealed a pSFT with clean margins and no features of malignancy detected by immunohistochemistry. This case report highlights the fact that diagnosing Doege-Potter syndrome is not always easy and often takes time after the patient's initial presentation, the importance of preoperative planning, and the benefits of preoperative embolization of the arterial feeder.

摘要

多伊格-波特综合征或非胰岛细胞瘤低血糖症(NICTH)是一种罕见病症,通常由胸膜孤立性纤维瘤(pSFT)引起。NICTH的诊断具有挑战性,因为其临床表现不具特异性且pSFT罕见。我们报告一例44岁女性被诊断为NICTH的病例,以说明确立该诊断的困难以及基于病变特征进行术前准备的重要性。我们先进行了栓塞,然后行整块切除并使用膈肌补片和肺楔形切除术,随后通过左半蛤壳形切口用普理灵网片重建膈肌。术后过程顺利,尤其是低血糖症得到缓解,这与诊断相符。病理检查显示为pSFT,切缘干净,免疫组化未检测到恶性特征。本病例报告强调了以下事实:诊断多伊格-波特综合征并不总是容易,在患者初次就诊后往往需要时间,术前规划很重要,以及术前对动脉供血支进行栓塞的益处。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7800/12174128/b455546636ac/fonc-15-1550343-g001.jpg

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