MedElevator Войти в исследования
← Поиск публикаций

DOI 10.1093/jscr/rjag517

Медицинская библиотека · Journal of surgical case reports · 2026

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

Ganju Nakul, Ndulue Chukwunonso, Erega Heera Tesfaye, Slater Kristin, Fadul Sarrah, Santiago-Gonzalez Juan Carlos, Aduli Farshad, Shokrani Babak

Клинический случайСтатьяПеревод в очереди

Аннотация

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.

Для цитирования: Ganju Nakul, Ndulue Chukwunonso, Erega Heera Tesfaye, Slater Kristin, Fadul Sarrah, Santiago-Gonzalez Juan Carlos, Aduli Farshad, Shokrani Babak. 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. 2026. DOI: 10.1093/jscr/rjag517.
Подготовка и перевод публикации
Обработка статьи · этапы, ошибки и управление
Загрузка состояния…

История · было → стало

Состояние обновляется каждые 5 секунд, пока блок открыт. Процент показывается только там, где известен объём работы. Пауза останавливает продолжение этапа; текущий запрос к источнику может завершиться.

Тип публикации указан по данным источника. Уровень доказательности автоматически не присваивается.