Carney’s triad in an adult male from a tertiary care center in India: a case report

Background Carney’s triad is a rare syndrome comprising gastrointestinal stromal tumor, extra-adrenal paraganglioma, and pulmonary chondroma along with newer additions of adrenal adenoma and esophageal leiomyoma. The triad is completely manifest in only 25–30% cases, with most patients presenting with two out of three parts of the syndrome. Wild-type succinate-dehydrogenase-deficient gastric gastrointestinal stromal tumor forms the most common component of Carney’s triad and is usually multicentric and multifocal. It usually demonstrates indolent behavior and resistance to imatinib; hence, the management remains predominantly surgical. Pulmonary chondromas are commonly unilateral and multiple with slow-growing nature, which allows for conservative management. Adrenocortical adenomas are found in 20% of patients and are usually detected as incidentalomas. Case presentation A 49-year-old Asian male presented with upper gastrointestinal bleed and was diagnosed with multiple gastric succinate-dehydrogenase-deficient gastrointestinal stromal tumors. On evaluation, he was found to have left pulmonary chondroma and non-secretory adrenal adenoma, thus completing the Carney’s triad. He underwent surgery with sleeve gastrectomy and excision of the antral tumor nodule, while the adrenal and pulmonary tumors have been under close follow-up. Conclusion Literature regarding Carney’s triad is scarce, especially from the Indian setting. Our report aims to highlight the various manifestations of this syndrome with emphasis on management of wild-type succinate-dehydrogenase-deficient gastrointestinal stromal tumor. Radical gastric surgeries do not offer a survival advantage in this condition; hence, more conservative modalities of resection can be adopted.

Hamartoma and Benign Tumor-like Lesions

Pulmonary hamartomas are benign neoplasms comprised of various mesenchymal components. The presence of chondroid calcification (popcorn appearance) and/or macroscopic fat can be diagnostic of hamartoma. Very slow growth is an expected finding. Treatment of pulmonary hamartoma is usually conservative. Surgical resection can be considered if the CT findings are nondiagnostic, if biopsy is inconclusive, or if the lesion demonstrates rapid growth. Surgery should also be considered in patients with central lesions and postobstructive atelectasis or infection. Hamartomas account for the majority of benign lung neoplasms. Other benign neoplasms occurring in the lungs and airways include lipoma, chondroma, and leiomyoma and are very rare. If a pulmonary chondroma is diagnosed, additional investigation should be considered to exclude concurrent leiomyosarcoma and extraadrenal paraganglioma (Carney Triad).

Computed Tomography Imaging Findings of Pulmonary Chondroma

Purpose. To characterize the computed tomography (CT) imaging findings in patients with pulmonary chondroma. Methods. We examined CT imaging findings of eight patients with histopathologically verified pulmonary chondroma. We assessed the location, size, shape, margins, amount of calcification, calcification pattern, and attenuation on precontrast and enhancement CT. Results. All patients exhibited solitary, mildly lobulated pulmonary masses, which were located in the right lung in four cases and the left lung in four cases. The mean lesion size was 3.7 cm (range 0.9–10.7 cm). All eight tumours had a well-defined margin. On plain CT images, seven of the cases (87.5%) showed a mass with varying degrees of calcification, which included strip-like punctate (n=5) and ring (n=2) patterns. One patient with a large lesion (10.7 cm) showed chest wall adhesion. On contrast-enhanced CT images, all lesions demonstrated slight inhomogeneous enhancement ≤14 HU. Conclusion. CT is the reference standard diagnostic technique for locating pulmonary chondroma. In most cases, CT findings show some characteristics that are important in the diagnosis, surgical planning, and follow-up of the tumour.

Carney Triad

1. Pulmonary chondroma 2. Gastrointestinal stromal tumor (GIST), formerly, gastric epithelioid leiomyosarcoma 3. Functioning extra-adrenal paraganglioma • Triad eventually present in more than half of patients • 〉90% of patients are female • Mean age: 17 years • Mechanism: unknown (disease absent in 1 identical twin)...