The Carney triad (gastrointestinal stromal tumor [GIST], pulmonary chondroma, and extraadrenal paraganglioma) is a very rare non-hereditary multitumor syndrome characterized by female predominance (85%) and early symptom onset. When paraganglioma is absent, it is called “incomplete Carney triad”. A 23-year-old woman presented with a 3-month history of epigastric pain and weight loss of 6 kg. Her history included distal gastrectomy for a gastric antrum tumor (leiomyoma) 6 years earlier. Positive diagnosis was established by upper gastrointestinal endoscopy and abdominal contrast-enhanced computed tomography (CT), which revealed 5 exophytic tumors from 1.0 to 6.0 cm in the distal part of the stomach (Fig. 1 A and B). A chest CT showed a calcified mass (3.9 cm in diameter) in the right lower lobe (S9) consistent with a benign pulmonary chondroma (Fig. 1C). The presumed diagnosis was incomplete Carney triad syndrome, and the patient underwent subtotal gastrectomy (Billroth II) (Fig. 1D). The pathology report was consistent with a mixed-cell type GIST with 4 mitoses per 50 high-power fields (HPFs). Immunohistochemical staining was positive for CD117/c-KIT (Fig. 1E) and CD34. The patient returned to the clinic 2 years later to undergo symptomatic pulmonary chondroma removal. After a 10-year follow-up, the patient is asymptomatic and without disease progression or metastasis.

Figure 1.

Axial view (A) and coronal view (B) of abdominal contrast-enhanced CT showing multifocal tumors in the distal part of the stomach. C) Chest CT shows well-circumscribed mass compatible with lung chondroma. D) Multiple tumor nodules in the gastric antrum. E) Tumor cells are positive for anti-CD117 antibody (×100).