Pulmonary chondroma

Definition

A benign tumour composed of (myxo)hyaline cartilage, usually forming part of Carney's triad along with gastrointestinal stromal tumour and paraganglioma^1,2. Benign adrenocortical tumors² and possibly other tumours³ may also be part of the syndrome.

Synonyms

Osteochondroma

Epidemiology

Most cases occur in young women².

Clinical features

Most cases are asymptomatic

Radiology

The lesions are peripheral, forming well demarcated masses with "popcorn" calcification.

Macroscopic appearances

The lesions are circumscribed, often enucleate and are calcified.

Histopathology

The nodules of cartilage are hypocellular, with no evidence of malignancy.

Immunohistochemistry

Ultrastructure

Differential diagnosis

Pulmonary hamartoma: there are clefts lined by respiratory epithelium. There may be other differentiated mesenchymal elements.
Chondroblastoma
Metastatic chondrosarcoma: has features of malignancy.

Management

Local excision.

Prognosis

Local excision is curative, but patients are likely to develop the other features of Carney's triad.

References

¹ Carney JA The triad of gastric epithelioid leiomyosarcoma, functioning extra-adrenal paraganglioma, and pulmonary chondroma. Cancer 1979; 43:374-82

² Carney JA Gastric stromal sarcoma, pulmonary chondroma, and extra-adrenal paraganglioma (Carney Triad): natural history, adrenocortical component, and possible familial occurrence. Mayo Clin Proc 1999; 74:543-52

³ Scopsi L, Collini P,Muscolino G A new observation of the Carney's triad with long follow-Up period and additional tumors. Cancer Detect Prev 1999; 23:435-43

This page last revised 6.5.2005.