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一名患有胸膜孤立性纤维瘤患者的多伊格-波特综合征和皮埃尔-玛丽-班贝格综合征:1例罕见病例并文献复习

Doege-Potter Syndrome and Pierre-Marie-Bamberger Syndrome in a Patient With Pleural Solitary Fibrous Tumor: A Rare Case With Literature Review.

作者信息

Solsi Anup, Pho Karen, Shojaie Shiva, Findakly Dawood, Noori Tarreq

机构信息

Internal Medicine, Creighton University Arizona Health Education Alliance/Valleywise Health Medical Center, Phoenix, USA.

Internal Medicine, University of Arizona College of Medicine, Phoenix, USA.

出版信息

Cureus. 2020 May 1;12(5):e7919. doi: 10.7759/cureus.7919.

DOI:10.7759/cureus.7919
PMID:32494533
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7263730/
Abstract

Solitary fibrous tumors (SFT) represent a unique subset of mostly benign heterogeneous tumors with mesenchymal cell origins. These tumors have been reported in the past as being mostly indolent, with a slowly evolving clinical course and low potential for malignancy. Although found systemically, the incidence of SFT arising intrathoracically, from the pleura of the lung, is relatively poorly documented in the medical literature. SFT is a rare phenomenon, but in even rarer circumstances, these tumors are associated with distinctive paraneoplastic syndromes, such as Pierre-Marie-Bamberger syndrome (PMBS) and Doege-Potter syndrome (DPS). PMBS presents as digital clubbing and hypertrophic pulmonary osteoarthropathy. DPS has been characterized as a non-islet cell tumor hypoglycemia due to the ectopic secretion of insulin-like growth factor 2 (IGF-2), a pattern seen in fewer than 5% of cases of SFT. Treatment is typically through surgical resection. In our research of the medical literature, we found only very few cases in which the association with SFT and both paraneoplastic syndromes were described. Here, we report an uncommon case of a 68-year-old male patient found to have an incidental right hemithoracic tumor with digital clubbing and intermittent severe episodes of fasting hypoglycemia after initially presenting with a syncopal episode.

摘要

孤立性纤维性肿瘤(SFT)是一组独特的肿瘤,大多为良性,起源于间充质细胞,具有异质性。过去报道这些肿瘤大多生长缓慢,临床病程进展缓慢,恶性潜能低。尽管SFT可发生于全身各处,但医学文献中关于起源于肺胸膜的胸腔内SFT的发病率记载相对较少。SFT是一种罕见的现象,但在更为罕见的情况下,这些肿瘤会伴有独特的副肿瘤综合征,如皮埃尔 - 玛丽 - 班贝格综合征(PMBS)和多伊格 - 波特综合征(DPS)。PMBS表现为杵状指和肥厚性肺骨关节病。DPS的特征是由于胰岛素样生长因子2(IGF - 2)的异位分泌导致的非胰岛细胞瘤低血糖症,这种情况在不到5%的SFT病例中可见。治疗通常通过手术切除。在我们对医学文献的研究中,我们发现仅有极少数病例描述了SFT与这两种副肿瘤综合征的关联。在此,我们报告一例罕见病例,一名68岁男性患者最初因晕厥发作就诊,偶然发现右半胸肿瘤,并伴有杵状指和间歇性严重空腹低血糖发作。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1e9e/7263730/5d3333612d33/cureus-0012-00000007919-i06.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1e9e/7263730/1221af97cb70/cureus-0012-00000007919-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1e9e/7263730/1e6c6935e228/cureus-0012-00000007919-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1e9e/7263730/a0851809ea34/cureus-0012-00000007919-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1e9e/7263730/56f6af915a63/cureus-0012-00000007919-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1e9e/7263730/738ec056ed93/cureus-0012-00000007919-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1e9e/7263730/5d3333612d33/cureus-0012-00000007919-i06.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1e9e/7263730/1221af97cb70/cureus-0012-00000007919-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1e9e/7263730/1e6c6935e228/cureus-0012-00000007919-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1e9e/7263730/a0851809ea34/cureus-0012-00000007919-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1e9e/7263730/56f6af915a63/cureus-0012-00000007919-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1e9e/7263730/738ec056ed93/cureus-0012-00000007919-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1e9e/7263730/5d3333612d33/cureus-0012-00000007919-i06.jpg

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