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PMID: 42368654 已发表 · epublish 英语

Gastric GIST with life-threatening anaemia and paraganglioma: a rare presentation of suspected Carney-Stratakis syndrome in a young adult.

Journal of surgical case reports ·第 2026 卷 ·第 6 期 ·2026-06-00

Ganju N, Ndulue C, Erega HT, Slater K, Fadul S, Santiago-Gonzalez JC, Aduli F, Shokrani B

摘要

Gastrointestinal stromal tumours (GISTs) rarely occur in young adults; in this age group, succinate dehydrogenase (SDH)-deficient subtypes predominate and may signal hereditary tumor syndromes. We present a 23-year-old male with a prior carotid paraganglioma who developed life-threatening iron deficiency anaemia (haemoglobin 4.0 g/dl) as the presenting manifestation of a high-grade gastric GIST. Subtotal gastrectomy with Billroth II reconstruction achieved R0 resection. Molecular testing was negative for KIT and PDGFRA mutations. Given the patient's age, mixed epithelioid/spindle histology, wild-type molecular profile, and paraganglioma history, Carney-Stratakis syndrome was suspected. SDHB immunohistochemistry confirmed an SDH-deficient phenotype, and germline testing identified a pathogenic heterozygous SDHx variant, establishing the diagnosis of Carney-Stratakis syndrome. At follow-up, the patient was clinically stable and enrolled in a multidisciplinary surveillance program. This case highlights the importance of recognizing atypical hematologic presentations of GIST and the need for comprehensive molecular and genetic evaluation in young patients.

关键词
Carney–Stratakis syndrome GIST SDH-deficient iron deficiency anaemia paraganglioma subtotal gastrectomy
文献信息
期刊
Journal of surgical case reports
期刊简称
J Surg Case Rep
ISSN
2042-8812
发表日期
2026-06-00
语言
英语
国家/地区
England
NLM ID
101560169
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