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胸膜孤立性纤维瘤所致的多伊奇-波特综合征

DOEGE-POTTER SYNDROME BY PLEURAL SOLITARY FIBROUS TUMOR.

作者信息

Alves Ana Sofia, Antunes Natalie, Santos Joana, Reis João Eurico, Calvinho Paulo

机构信息

Radiology Department, Centro Hospitalar e Universitário de Lisboa Central, Lisbon, Portugal.

Pathology Department, Centro Hospitalar e Universitário de Lisboa Central, Lisbon, Portugal.

出版信息

Port J Card Thorac Vasc Surg. 2022 Jan 4;28(4):51-53. doi: 10.48729/pjctvs.225.

Abstract

Solitary fibrous tumor of the pleura (SFTP) is a rare neoplasm that accounts for less than 5% of all pleural tumors. We present the case of a 73-year-old man with a history of recurrent episodes of severe hypoglycemia secondary to a large malignant SFTP. This paraneoplastic manifestation of SFTP occurs in less than 5% of cases and is referred to as Doege-Potter syndrome. Although rare, this is an important and reversible cause of hypoglycemia, which is resolved by complete surgical resection of the tumor. We describe the pathogenesis, diagnosis, and treatment of Doege-Potter syndrome. Key imaging findings and pathologic correlation are shown.

摘要

胸膜孤立性纤维瘤(SFTP)是一种罕见的肿瘤,占所有胸膜肿瘤的比例不到5%。我们报告一例73岁男性病例,其患有巨大恶性SFTP继发严重低血糖反复发作史。SFTP的这种副肿瘤表现发生在不到5%的病例中,被称为多伊格-波特综合征。尽管罕见,但这是低血糖的一个重要且可逆转的原因,通过肿瘤的完整手术切除可得以解决。我们描述了多伊格-波特综合征的发病机制、诊断和治疗。展示了关键的影像学表现及病理相关性。

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