TY - JOUR A2 - Plebani, Alessandro AU - Steffin, David AU - Bhar, Saleh AU - Fishman, Douglas S. AU - Rider, Nicholas L. AU - Naik-Mathuria, Bindi AU - Martinez, Caridad AU - Venkatramani, Rajkumar PY - 2021 DA - 2021/06/28 TI - Gastric Adenocarcinoma in the Setting of IPEX Syndrome SP - 9967198 VL - 2021 AB - Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome is a rare X-linked disorder caused by a loss of function mutation in the FOXP3gene. It manifests early in infancy with clinical symptoms including autoimmune enteropathy, type 1 diabetes mellitus, and eczema. While aberrant FOXP3expression has been associated with several types of cancer, little is known regarding the risk of cancer in patients with IPEX harboring the characteristic FOXP3mutation. Here, we present a unique case of a primary signet ring gastric adenocarcinoma in a pediatric patient with IPEX syndrome. SN - 2090-6609 UR - https://doi.org/10.1155/2021/9967198 DO - 10.1155/2021/9967198 JF - Case Reports in Immunology PB - Hindawi KW - ER -