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Doege–Potter syndrome: A review of the literature including a new case report
RATIONALE: We reviewed 76 published cases of Doege–Potter syndrome, and non-islet cell tumor hypoglycemia (NICTH) secondary to a solitary fibrous tumor (SFT) between 1989 and 2016, to study disease pathogenesis, diagnosis, and treatment of this rare paraneoplastic disease. Further, we report 1 new c...
Autores principales: | , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Wolters Kluwer Health
2017
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5502173/ https://www.ncbi.nlm.nih.gov/pubmed/28682900 http://dx.doi.org/10.1097/MD.0000000000007417 |
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author | Han, Guiyan Zhang, Zhimin Shen, Xingbin Wang, Kunpeng Zhao, Yang He, Jianqiu Gao, Yu Shan, Xiujie Xin, Guohua Li, Chunhui Liu, Xiaoyan |
author_facet | Han, Guiyan Zhang, Zhimin Shen, Xingbin Wang, Kunpeng Zhao, Yang He, Jianqiu Gao, Yu Shan, Xiujie Xin, Guohua Li, Chunhui Liu, Xiaoyan |
author_sort | Han, Guiyan |
collection | PubMed |
description | RATIONALE: We reviewed 76 published cases of Doege–Potter syndrome, and non-islet cell tumor hypoglycemia (NICTH) secondary to a solitary fibrous tumor (SFT) between 1989 and 2016, to study disease pathogenesis, diagnosis, and treatment of this rare paraneoplastic disease. Further, we report 1 new case of a patient presenting with Doege–Potter syndrome. PATIENTS CONCERNS: The tumors originated from the pleural cavity, lung, pelvis, liver, retroperitoneum, kidney, mediastinal, the sella, uterus, bladder, intestine, mandibular, and the thigh. The most common location was the pleural cavity (left 12 cases and right 28 cases). Moreover, 28/71 (39.4%) were benign and 43/71 (60.6%) were malignant. SFTs with NICTH were more likely to be malignant and present at a higher rate than previously published (5%–10.4%). The malignancy rate of extrathoracic SFTs was higher than that of thoracic SFTs, 20 (66.7%) as compared with 23 (56.1%). Age of onset varied from 24 to 85 years (mean 59 years), with 47 males and 28 females, and gender unavailable for 1 case. When comparing clinical characteristics of patients with benign as compared malignant tumors, no significant differences in the age of onset, gender, or size of tumor were seen. Among 15/19 cases, the insulin-like growth factor II (IGF-II)/IGF-I ration was >10.0. Complete tumor resection remained the only definitive treatment. OUTCOMES AND LESSENS: Glucocorticoids dose-dependently reduce the frequency and severity of hypoglycemic episodes. Low doses of prednisone were ineffective at relieving hypoglycemia. The effect of neoadjuvant treatment, consisting of chemoradiation, and consecutive selective embolization of vessels feeding the tumor were not identified. |
format | Online Article Text |
id | pubmed-5502173 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2017 |
publisher | Wolters Kluwer Health |
record_format | MEDLINE/PubMed |
spelling | pubmed-55021732017-07-18 Doege–Potter syndrome: A review of the literature including a new case report Han, Guiyan Zhang, Zhimin Shen, Xingbin Wang, Kunpeng Zhao, Yang He, Jianqiu Gao, Yu Shan, Xiujie Xin, Guohua Li, Chunhui Liu, Xiaoyan Medicine (Baltimore) 5700 RATIONALE: We reviewed 76 published cases of Doege–Potter syndrome, and non-islet cell tumor hypoglycemia (NICTH) secondary to a solitary fibrous tumor (SFT) between 1989 and 2016, to study disease pathogenesis, diagnosis, and treatment of this rare paraneoplastic disease. Further, we report 1 new case of a patient presenting with Doege–Potter syndrome. PATIENTS CONCERNS: The tumors originated from the pleural cavity, lung, pelvis, liver, retroperitoneum, kidney, mediastinal, the sella, uterus, bladder, intestine, mandibular, and the thigh. The most common location was the pleural cavity (left 12 cases and right 28 cases). Moreover, 28/71 (39.4%) were benign and 43/71 (60.6%) were malignant. SFTs with NICTH were more likely to be malignant and present at a higher rate than previously published (5%–10.4%). The malignancy rate of extrathoracic SFTs was higher than that of thoracic SFTs, 20 (66.7%) as compared with 23 (56.1%). Age of onset varied from 24 to 85 years (mean 59 years), with 47 males and 28 females, and gender unavailable for 1 case. When comparing clinical characteristics of patients with benign as compared malignant tumors, no significant differences in the age of onset, gender, or size of tumor were seen. Among 15/19 cases, the insulin-like growth factor II (IGF-II)/IGF-I ration was >10.0. Complete tumor resection remained the only definitive treatment. OUTCOMES AND LESSENS: Glucocorticoids dose-dependently reduce the frequency and severity of hypoglycemic episodes. Low doses of prednisone were ineffective at relieving hypoglycemia. The effect of neoadjuvant treatment, consisting of chemoradiation, and consecutive selective embolization of vessels feeding the tumor were not identified. Wolters Kluwer Health 2017-07-07 /pmc/articles/PMC5502173/ /pubmed/28682900 http://dx.doi.org/10.1097/MD.0000000000007417 Text en Copyright © 2017 the Author(s). Published by Wolters Kluwer Health, Inc. http://creativecommons.org/licenses/by-nc-nd/4.0 This is an open access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal. http://creativecommons.org/licenses/by-nc-nd/4.0 |
spellingShingle | 5700 Han, Guiyan Zhang, Zhimin Shen, Xingbin Wang, Kunpeng Zhao, Yang He, Jianqiu Gao, Yu Shan, Xiujie Xin, Guohua Li, Chunhui Liu, Xiaoyan Doege–Potter syndrome: A review of the literature including a new case report |
title | Doege–Potter syndrome: A review of the literature including a new case report |
title_full | Doege–Potter syndrome: A review of the literature including a new case report |
title_fullStr | Doege–Potter syndrome: A review of the literature including a new case report |
title_full_unstemmed | Doege–Potter syndrome: A review of the literature including a new case report |
title_short | Doege–Potter syndrome: A review of the literature including a new case report |
title_sort | doege–potter syndrome: a review of the literature including a new case report |
topic | 5700 |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5502173/ https://www.ncbi.nlm.nih.gov/pubmed/28682900 http://dx.doi.org/10.1097/MD.0000000000007417 |
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