Tracheoesophageal fistula with esophageal atresia (TEF/EA) carries long-term gastrointestinal morbidity, including strictures, gastroesophageal reflux disease (GERD), and dysmotility, all of which predispose to chronic inflammation. Gastric signet-ring-cell adenocarcinoma (SRC) is rare in young adults and unusual in the context of congenital foregut anomalies. A female in her twenties with repaired TEF/EA had progressive dysphagia, recurrent food impactions, and severe distal esophageal stricture requiring frequent dilations. During routine surveillance esophagogastroduodenoscopy (EGD), SRC was incidentally identified. Biopsy handling errors, multiple specimens placed on a single slide labeled "stomach" obscured precise tumor localization. Staging PET-CT showed no metastatic disease. A hereditary cancer panel (ATM, CDH1, CTNNA1, MLH1, MSH2, PMS2, TP53) was negative. Given uncertain tumor location and negative imaging, the patient elected endoscopic surveillance over total gastrectomy. An advanced endoscopist successfully traversed and treated the high-risk stricture, restoring patency and eliminating need for repeat dilations. Over 12 months, serial EGDs demonstrated no residual or recurrent malignancy, and the patient reported marked functional and psychosocial improvement. This case highlights the interplay of congenital foregut anomalies, chronic inflammation, and SRC development, and underscores the critical importance of meticulous biopsy handling and personalized endoscopic care. Tailored surveillance may be appropriate in select early-stage SRC cases where localization is uncertain and imaging is negative.
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