Definition
A benign tumour composed of (myxo)hyaline cartilage, usually forming part of Carney's triad along with gastrointestinal stromal tumour and paraganglioma1,2. Benign adrenocortical tumours2 and possibly other tumours3 may also be part of the syndrome.
Osteochondroma
Most cases occur in young women2.
Most cases are asymptomatic
The lesions are peripheral, forming well demarcated masses with central or "popcorn" calcification. They are often multiple, sometimes numerous4. Rarely they appear to replace an entire lung4. The radiological differential diagnosis includes metastatic gastrointestinal stromal tumour.
The lesions are circumscribed, often enucleate at at surgery and are calcified. They are often bosselated with a cartilaginous cut surface4. Less often, they are gelatinous, cystic, haemorrhagic or necrotic4.
The nodules of cartilage are myxoid and hypocellular, with no cytological features of malignancy. They have a thin pseudocapsule4.
Pulmonary hamartoma: most often occur in older men. They have a component of fat or smooth muscle. Calcification is less common than in chondromas. There are clefts lined by respiratory epithelium. They lack a pseudocapsule. Solitary pulmonary chondromas, unassociated with Carney's triad, occur, usually in older men, and are usually not calcified.
Chondroblastoma
Metastatic chondrosarcoma: has features of malignancy.
Local excision.
Further pulmonary nodule soften develop but metastases do not occur. Patients are likely to develop the other features of Carney's triad.
This page last revised 7.3.2008.
©SMUHT/PW Bishop