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胸廓外:以腹膜后腹部肿块形式表现的多伊格-波特综合征。

Outside the Thorax: Doege-Potter Syndrome Presenting as a Retroperitoneal Abdominal Mass.

作者信息

Berrebi Daniel M, Symczyk Oksana, Cater Taylor, Adelanwa Ayodele, Bacaj Patrick, Thomay Alan A, Haider Adnan

机构信息

School of Medicine, West Virginia University, Morgantown, WV, USA.

Department of Endocrinology, Department of Medicine, West Virginia University, Morgantown, WV, USA.

出版信息

Case Rep Endocrinol. 2021 May 19;2021:9919321. doi: 10.1155/2021/9919321. eCollection 2021.

DOI:10.1155/2021/9919321
PMID:34104499
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8159632/
Abstract

OBJECTIVE

We present a case of refractory hypoglycemia, weight loss, and retroperitoneal solitary fibrous tumor. . A 68-year-old female presented with symptomatic hypoglycemia, weight loss, and abdominal mass identified on CT scan of the abdomen. Blood work during symptomatic hypoglycemia was consistent with an IGF-2-producing tumor. The abdominal mass pathology was consistent with solitary fibrous tumor surrounding the adrenal gland, and resection resulted in complete resolution of hypoglycemia. . Understanding the biochemical mechanisms behind glucose regulation is necessary to diagnose and adequately treat Doege-Potter syndrome, a paraneoplastic syndrome observed in patients with solitary fibrous tumors. Solitary fibrous tumors can be characterized by specific histologic and immunohistochemical studies.

CONCLUSION

This report describes the clinical workup of a patient presenting with hypoglycemia and a retroperitoneal tumor. This case is unique because of its presentation with severe, refractory hypoglycemia and the tumor's location in the retroperitoneum, given the majority of solitary fibrous tumors are found in the lungs originating from the pleura.

摘要

目的

我们报告一例难治性低血糖、体重减轻和腹膜后孤立性纤维瘤的病例。一名68岁女性出现症状性低血糖、体重减轻,腹部CT扫描发现腹部肿块。症状性低血糖期间的血液检查结果与产生胰岛素样生长因子-2(IGF-2)的肿瘤一致。腹部肿块病理与肾上腺周围的孤立性纤维瘤一致,切除后低血糖完全缓解。了解葡萄糖调节背后的生化机制对于诊断和充分治疗多伊奇-波特综合征(Doege-Potter syndrome)至关重要,这是一种在孤立性纤维瘤患者中观察到的副肿瘤综合征。孤立性纤维瘤可通过特定的组织学和免疫组织化学研究进行特征性描述。

结论

本报告描述了一名出现低血糖和腹膜后肿瘤患者的临床检查情况。该病例独特之处在于其表现为严重难治性低血糖,且肿瘤位于腹膜后,因为大多数孤立性纤维瘤发现于起源于胸膜的肺部。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a8b6/8159632/3760f8d980aa/CRIE2021-9919321.004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a8b6/8159632/6cbdc6402eb0/CRIE2021-9919321.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a8b6/8159632/7468238d322a/CRIE2021-9919321.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a8b6/8159632/7e72dad1445d/CRIE2021-9919321.003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a8b6/8159632/3760f8d980aa/CRIE2021-9919321.004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a8b6/8159632/6cbdc6402eb0/CRIE2021-9919321.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a8b6/8159632/7468238d322a/CRIE2021-9919321.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a8b6/8159632/7e72dad1445d/CRIE2021-9919321.003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a8b6/8159632/3760f8d980aa/CRIE2021-9919321.004.jpg

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本文引用的文献

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BMJ Case Rep. 2020 Aug 25;13(8):e235549. doi: 10.1136/bcr-2020-235549.
2
Doege-Potter syndrome: A review of the literature including a new case report.多伊格-波特综合征:文献综述并附一例新病例报告
Medicine (Baltimore). 2017 Jul;96(27):e7417. doi: 10.1097/MD.0000000000007417.
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Histologic variability in solitary fibrous tumors reflects angiogenic and growth factor signaling pathway alterations.
孤立性纤维性肿瘤的组织学变异性反映了血管生成和生长因子信号通路的改变。
Hum Pathol. 2015 Jul;46(7):1015-26. doi: 10.1016/j.humpath.2015.03.014. Epub 2015 Apr 15.
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Abdom Imaging. 2014 Dec;39(6):1323-9. doi: 10.1007/s00261-014-0155-4.
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Management of endocrine disease: a clinical update on tumor-induced hypoglycemia.内分泌疾病的管理:肿瘤诱导性低血糖的临床最新进展。
Eur J Endocrinol. 2014 Mar 14;170(4):R147-57. doi: 10.1530/EJE-13-1012. Print 2014 Apr.
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Immunohistochemistry of soft tissue tumours - review with emphasis on 10 markers.软组织肿瘤的免疫组织化学——重点介绍 10 种标志物的综述。
Histopathology. 2014 Jan;64(1):101-18. doi: 10.1111/his.12298. Epub 2013 Nov 28.
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IGF2 and cancer.IGF2 与癌症。
Endocr Relat Cancer. 2013 Oct 24;20(6):R321-39. doi: 10.1530/ERC-13-0231. Print 2013 Dec.
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Nuclear expression of STAT6 distinguishes solitary fibrous tumor from histologic mimics.STAT6 的核表达可将孤立性纤维性肿瘤与组织学模拟物区分开来。
Mod Pathol. 2014 Mar;27(3):390-5. doi: 10.1038/modpathol.2013.164. Epub 2013 Sep 13.
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Tumors, IGF-2, and hypoglycemia: insights from the clinic, the laboratory, and the historical archive.肿瘤、IGF-2 和低血糖:来自临床、实验室和历史档案的见解。
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