Gastric Adenocarcinoma Presenting as Thrombotic Microangiopathy in a 14-year-old Girl

Abstract

Gastric adenocarcinoma is rare in childhood and often presents with disseminated malignancy at diagnosis due to aspecific symptoms leading to delay in diagnosis. A familial predisposition for gastrointestinal cancer is suggested for the development of this early-onset adenocarcinoma. We report the case of a 14-year-old girl with a familial history of colorectal, liver, and breast cancers affected by metastatic gastric adenocarcinoma, who first presented with thrombotic microangiopathy. Thrombotic microangiopathy as first clinical presentation of metastatic gastric cancer is an exceptional event in childhood and represents a challenge for pediatricians. Gastric adenocarcinoma should be suspected in young patients with a significant familial history and also in the absence of initial specific signs, so as to provide correct diagnosis and appropriate treatment.

Two pediatric cases of gastric adenocarcinoma and review of the literature

“…Patients' admission findings are mostly nonspecific, and cases with vomiting, hematemesis, abdominal pain, anemia, weight loss, pulmonary hypertension, thrombotic microangiopathy, and massive ascites have been reported (2,(5)(6)(7). Patıroğlu et al (8) reported that they diagnosed gastric signet ring cell carcinoma in an 18-year-old patient with ataxia telangiectasia and Hashimato thyroiditis.…”

An Adolescent With Cough and Dyspepsia

“…Our review of the literature identified 42 published cases of pediatric GAC, with 21 of these cases published in the past decade. [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15] at diagnosis of the most recent 21 cases ranged from 21 months to 17 years, with a mean of 11.7 years. Twelve of these patients were males.…”

“…Unfortunately, no genetic testing was performed in those instances. 1,2,6,7 Chang et al reported metastatic GAC in a 14-year-old boy with a strong family history of colon cancer. Whole exome sequencing identified a TP53 mutation, consistent with Li-Fraumeni syndrome.…”

“…[1][2][3][4][5][6][7][8][9][10][11][12][13][14][15] Three patients presented with dyspnea, pulmonary hypertension, and subsequent insufficiency from pulmonary tumor thrombotic microangiopathy. 7,9,10 Hematologic findings most commonly included iron deficiency anemia; however, 2 cases of pernicious anemia and 1 case of hemolytic anemia were described. 1,5 Thus, high clinical suspicion is needed to establish the diagnosis of GAC in pediatric patients.…”

“…Distal metastasis locations included liver, colon, lungs, vertebral body, pericardium, myocardium, bone marrow, and skin. 1,2,6,7,[9][10][11] Pediatric GAC management is based on guidelines for adult GAC due to the rarity of the condition in children and lack of supporting evidence. Ten patients (47.6%) had local involvement at diagnosis, which qualified them for surgical resection.…”