Surgical resection of a solitary fibrous tumour causing refractory hypoglycaemia: Doege-Potter syndrome.

IF 0.6 Q3 MEDICINE, GENERAL & INTERNAL
Ryan D Rosen, Matthew O'Brien, Ramya Ersala, John M Barnwell
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引用次数: 0

Abstract

Solitary fibrous tumours (SFTs) are rare soft tissue masses that are often clinically silent until they cause mass effect. A paraneoplastic syndrome manifesting as persistent hypoglycaemia, termed Doege-Potter syndrome (DPS), can be associated with these lesions. Surgical treatment is recommended for the management of these tumours.A case of a man in his 60s who was admitted for altered mental status and hypoglycaemia is herein described. Physical examination revealed a distended, asymmetric abdomen with a firm palpable mass in the left upper quadrant. Imaging demonstrated a 24 cm abdominal mass, confirmed to be an SFT on pathology. Biochemical evaluation was consistent with DPS. Surgical excision was performed; however, its large size and retroperitoneal location complicated intraoperative visualisation, ultimately requiring aortic reconstruction, multiple extremity fasciotomy, nephrectomy and prolonged intensive care unit stay.Few publications describe DPS. While tumour excision can be curative, this case report describes challenges that may arise with surgical management.

手术切除引起难治性低血糖的孤立性纤维肿瘤:多格-波特综合征。
孤立性纤维性肿瘤(SFTs)是一种罕见的软组织肿块,通常在临床上无症状,直到它们引起肿块效应。一种表现为持续低血糖的副肿瘤综合征,称为多吉-波特综合征(DPS),可能与这些病变有关。建议对这些肿瘤进行手术治疗。一个60多岁的男子因精神状态改变和低血糖而入院的病例在此描述。体格检查显示腹部膨胀,不对称,左侧上象限有可触及的硬肿块。影像学显示腹部24厘米肿块,病理证实为SFT。生化评价与DPS一致。手术切除;然而,其较大的体积和腹膜后位置使术中观察复杂,最终需要主动脉重建、多肢筋膜切开术、肾切除术和延长重症监护病房的住院时间。很少有出版物描述DPS。虽然肿瘤切除可以治愈,但本病例报告描述了手术治疗可能出现的挑战。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
BMJ Case Reports
BMJ Case Reports Medicine-Medicine (all)
CiteScore
1.40
自引率
0.00%
发文量
1588
期刊介绍: BMJ Case Reports is an important educational resource offering a high volume of cases in all disciplines so that healthcare professionals, researchers and others can easily find clinically important information on common and rare conditions. All articles are peer reviewed and copy edited before publication. BMJ Case Reports is not an edition or supplement of the BMJ.
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