A 25-year-old woman with a gastric GIST and a PET-positive epicardial tumor

Autor: Felix M. Mottaghy, Hans J. Brambs, Guido Adler, N. M. Blumstein, Thomas F. E. Barth, Martin Höher, Andreas Hannekum, Sven N. Reske, Volker Kächele, Sandra Pauls, Peter Möller, Thomas Seufferlein, Martin Jeltsch
Rok vydání: 2006
Předmět:
Zdroj: Nature clinical practice. Oncology. 4(3)
ISSN: 1743-4262
Popis: Carney's triad is a syndrome consisting of three rare neoplasms: gastric leiomyosarcoma, extra-adrenal paraganglioma and pulmonary chondroma. The article by Kachele et al. describes the case of a young woman who presented with a gastrointestinal stromal tumor and metastatic epicardial paraganglioma. The patient underwent an extended hemigastrectomy and cardiac surgery and remained free of recurrent disease during the follow-up period. This is the first report of an epicardial paraganglioma within a Carney's triad and the authors discuss the diagnostic workup and management of patients with Carney's syndrome. Background A 25-year-old woman presented with a history of abdominal pain. Endoscopy of the upper gastrointestinal tract revealed a tumor that protruded into the prepyloric antrum. After resection, a 'high-risk' gastrointestinal stromal tumor was histologically confirmed. Investigations Endoscopy, endoscopic ultrasound, hemigastrectomy, [18F]-2-fluoro-2-deoxy-D-glucose (FDG)-PET/CT scan, histological examination, immunohistochemistry, cardiac MRI, high-resolution CT with electrocardiogram gating, CT angiography, and cardiac surgery. Diagnosis Gastrointestinal stromal tumor, epicardial paraganglioma, and Carney's syndrome. Management Abdominal ultrasound and endoscopy combined with endoscopic ultrasound, annual FDG-PET/CT scan.
Databáze: OpenAIRE