Yan FF, Huang BK, Chen YL, Zhuang YZ, You XY, Liu CQ, Li XJ. Coexistence of ovarian serous papillary cystadenofibroma and type A insulin resistance syndrome in a 14-year-old girl: A case report. World J Clin Cases 2020; 8(15): 3334-3340 [PMID: 32874990 DOI: 10.12998/wjcc.v8.i15.3334]
Corresponding Author of This Article
Xue-Jun Li, MD, PhD, Chief Doctor, Professor, Department of Endocrinology and Diabetes, the First Affiliated Hospital of Xiamen University, No. 55 Zhenhai Road, Siming District, Xiamen 361003, Fujian Province, China. xmlixuejun@163.com
Research Domain of This Article
Endocrinology & Metabolism
Article-Type of This Article
Case Report
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Fang-Fang Yan, Bing-Kun Huang, Yin-Ling Chen, Chang-Qin Liu, Xue-Jun Li, Department of Endocrinology and Diabetes, The First Affiliated Hospital of Xiamen University, Xiamen 361003, Fujian Province, China
Yan-Zhen Zhuang, Xue-Ye You, Department of Pathology, The First Affiliated Hospital of Xiamen University, Xiamen 361003, Fujian Province, China
Author contributions: Yan FF and Huang BK wrote the manuscript draft; Chen YL created the figures; Zhuang YZ and You XY conducted the staining and pathological diagnosis; Liu CQ collected the clinical information; Li XJ designed the study and revised the submission; all authors contributed to the discussion and approved the final manuscript for the version to be submitted.
Informed consent statement: Informed written consent was obtained from the father of the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflict of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Xue-Jun Li, MD, PhD, Chief Doctor, Professor, Department of Endocrinology and Diabetes, the First Affiliated Hospital of Xiamen University, No. 55 Zhenhai Road, Siming District, Xiamen 361003, Fujian Province, China. xmlixuejun@163.com
Received: March 27, 2020 Peer-review started: March 30, 2020 First decision: April 24, 2020 Revised: May 27, 2020 Accepted: July 14, 2020 Article in press: July 14, 2020 Published online: August 6, 2020 Processing time: 132 Days and 6.8 Hours
Abstract
BACKGROUND
Type A insulin resistance syndrome is a rare disorder caused by mutations in the gene encoding the insulin receptor. Its coexistence with ovarian serous papillary cystadenofibroma is even rarer.
CASE SUMMARY
A 14-year-old girl developed type A insulin resistance syndrome and showed high fasting insulin, glucose, and hemoglobin A1c (HbA1c) levels. The girl suffered from ovarian serous papillary cystadenofibroma. The laboratory results were as follows: fasting insulin was 2624.90 pmol/L and HbA1c was 8.5%. A heterozygous missense mutation on exon 20 of the insulin receptor gene (c.3601C>T, Arg1201Trp) was observed. The histopathological diagnosis was a cystic lesion that extended to the upper right uterus, indicating a right ovarian serous papillary cystadefibroma accompanied by focal interstitial hyperplasia. The patient was treated with metformin for over 6 mo. Additionally, laparoscopic resection (bilateral) of the ovarian lesion and laparoscopic intestinal adhesiolysis were performed under general anesthesia. Diet therapy combined with exercise was then initiated. The patient had an uneventful recovery. The patient also showed improved blood glucose control, with reduced levels of fasting insulin (857.84 pmol/L) and HbA1c (7.0%).
CONCLUSION
Insulin resistance may play a significant role in the induction of tumors. It is important to investigate further the association between insulin resistance and tumors and the underlying mechanism.
Core tip: Type A insulin resistance syndrome (TAIRS) is a rare disorder that results from mutations in the gene encoding the insulin receptor. We present a rare case of TAIRS coexisting with an ovarian serous papillary cystadenofibroma in a 14-year-old girl caused by gene mutation. This case demonstrates that metformin has partial efficacy in the treatment of TAIRS. Furthermore, insulin may play a critical role in the development and progression of ovarian cystadenofibroma.
