Hepatic paraganglioma and multifocal gastrointestinal stromal tumor in a female: Incomplete Carney triad

The Carney triad (CT) describes the coexistence of multiple neoplasms including gastrointestinal stromal tumors (GISTs), extra-adrenal paraganglioma and pulmonary chondroma. At least two neoplastic tumors are required for diagnosis. In most cases, however, CT is incomplete. We report a case of an incomplete CT in a 34-year-old woman with a multifocal GIST and non-functional paraganglioma of the liver. Preoperative evaluation with a gastrofiberscope and abdominal computed tomography revealed multiple gastric tumors resembling GISTs and a single liver lesion which was assumed to have metastasized from the gastric tumors. The patient underwent total gastrectomy and partial hepatectomy. Histologic findings confirmed multiple gastric GISTs and paraganglioma of the liver. We report a case of a patient with incomplete expression of CT.