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手术治愈的继发于胸膜孤立性纤维瘤的低血糖症:病例报告及对多伊格-波特综合征的最新综述

Surgically cured hypoglycemia secondary to pleural solitary fibrous tumour: case report and update review on the Doege-Potter syndrome.

作者信息

Kalebi Ahmed Y, Hale Martin J, Wong Michelle L, Hoffman Tessa, Murray Jill

机构信息

Department of Anatomical Pathology, National Health Laboratory Service [NHLS], Johannesburg, South Africa.

出版信息

J Cardiothorac Surg. 2009 Aug 18;4:45. doi: 10.1186/1749-8090-4-45.

DOI:10.1186/1749-8090-4-45
PMID:19689813
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC2739517/
Abstract

The association of paraneoplastic hypoglycemia [Doege-Potter syndrome] and finger clubbing [Pierre-Marie-Bamberg syndrome] with pleural solitary fibrous tumour is rare. We present a previously unpublished but typical example of this rare occurrence together with a detailed updated literature review of previously published cases of pleural SFT discussing the histopathology of SFT; pathophysiology of the hypoglycemia and finger clubbing; treatment and outcome of pleural SFT. The patient, a 57-year-old African male was admitted at our hospital with recurrent episodes of hypoglycemia. He was found to have digital clubbing and decreased breath sounds in the right lower chest but no other significant clinical findings. His insulin level measured during an episode of hypoglycemia was undetectable. Chest radiograph and CT-scan revealed a lobulated mass in the right chest which was diagnosed to be SFT on histology. Surgical excision of the mass resulted in cure of the hypoglycemic episodes and rapid regression of the clubbing. Less than 65 cases of pleural SFT manifesting with hypoglycemia with or without finger-clubbing have been published in the English literature. The mean diameter of these tumours manifesting with hypoglycemia is 20 cm, 54% being benign while 42% were malignant. They predominantly present in the 6th-8th decade, average age of 64 years and a slight male preponderance at 58%. Complete surgical resection remains the most important predictor of clinical outcome in terms of recurrence and metastases, while providing instant cure for the hypoglycemia and rapid resolution of the finger clubbing.

摘要

副肿瘤性低血糖症(多伊格 - 波特综合征)和杵状指(皮埃尔 - 玛丽 - 班贝格综合征)与胸膜孤立性纤维瘤的关联较为罕见。我们呈现了一个此前未发表但典型的此类罕见病例,并对既往发表的胸膜孤立性纤维瘤病例进行了详细的文献综述更新,讨论了孤立性纤维瘤的组织病理学、低血糖症和杵状指的病理生理学、胸膜孤立性纤维瘤的治疗及预后。该患者为一名57岁的非洲男性,因反复低血糖发作入住我院。发现其有杵状指,右下胸部呼吸音减弱,但无其他显著临床发现。在一次低血糖发作期间测得其胰岛素水平无法检测到。胸部X线片和CT扫描显示右胸有一个分叶状肿块,组织学诊断为孤立性纤维瘤。手术切除肿块后低血糖发作得以治愈,杵状指迅速消退。英文文献中发表的胸膜孤立性纤维瘤伴或不伴杵状指表现为低血糖症的病例少于65例。这些表现为低血糖症的肿瘤平均直径为20厘米,54%为良性,42%为恶性。它们主要出现在60至80岁,平均年龄64岁,男性略占优势,占58%。就复发和转移而言,完整的手术切除仍然是临床预后最重要的预测因素,同时能立即治愈低血糖症并使杵状指迅速消退。

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