Published online Aug 6, 2020. doi: 10.12998/wjcc.v8.i15.3334
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
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.
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%).
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.