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

Abstract

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.

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Figure 1: Endoscopic ultrasound (X marks the probe) revealed a submucosal multinodular tumor (white arrows) of about 100 mm in diameter spanning from the gastric angulus to the prepyloric gastric antrum
Figure 2: Histology of the resected gastrointestinal stromal tumor
Figure 3: [18F]-2-fluoro-2-deoxy-D-glucose-PET/CT scan of the gastrointestinal stromal tumor revealed a single lesion of 44 mm × 17 mm × 55 mm adjacent to the posterior wall of the heart that seemed to protrude into the lumen of the left ventricle
Figure 4: Cardiac MRI and CT scans
Figure 5: CT angiography of the tumor (white arrows)
Figure 6: Coronary angiography of the tumor revealed that it was supplied by a branch of the left circumflex artery
Figure 7: Histomorphological analysis revealed a neuroendocrine lesion of ectodermal origin with epithelioid cells

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Acknowledgements

V Kächele and S Pauls contributed equally to this work.

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Correspondence to Thomas Seufferlein.

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Kächele, V., Pauls, S., Mottaghy, F. et al. A 25-year-old woman with a gastric GIST and a PET-positive epicardial tumor. Nat Rev Clin Oncol 4, 197–201 (2007). https://doi.org/10.1038/ncponc0743

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