Gastric Adenocarcinoma in the Setting of IPEX Syndrome | Zendy

David Steffin | Zendy; Saleh Bhar | Zendy; Douglas S. Fishman | Zendy; Nicholas L. Rider | Zendy; Bindi NaikMathuria | Zendy; Caridad Martinez | Zendy; Rajkumar Venkatramani | Zendy

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Gastric Adenocarcinoma in the Setting of IPEX Syndrome

Author(s) -

David Steffin,

Saleh Bhar,

Douglas S. Fishman,

Nicholas L. Rider,

Bindi NaikMathuria,

Caridad Martinez,

Rajkumar Venkatramani

Publication year - 2021

Publication title -

case reports in immunology

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 0.125

H-Index - 3

eISSN - 2090-6609

pISSN - 2090-6617

DOI - 10.1155/2021/9967198

Subject(s) - enteropathy , immune dysregulation , medicine , foxp3 , cancer , autoimmunity , adenocarcinoma , gastric adenocarcinoma , immunology , immune system , disease

Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome is a rare X-linked disorder caused by a loss of function mutation in the FOXP3 gene. It manifests early in infancy with clinical symptoms including autoimmune enteropathy, type 1 diabetes mellitus, and eczema. While aberrant FOXP3 expression has been associated with several types of cancer, little is known regarding the risk of cancer in patients with IPEX harboring the characteristic FOXP3 mutation. Here, we present a unique case of a primary signet ring gastric adenocarcinoma in a pediatric patient with IPEX syndrome.

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