Patients with multiple chondromatous hamartomas of the lung should be evaluated for Carney syndrome, particularly examining the stomach and nervous system.
Multiple pulmonary chondromatous hamartomas may signal Carney syndrome in young females; hypothesis-generating and should not yet change practice.
BACKGROUND: Multiple chondromatous hamartomas of the lung, which are very rare, are a feature of Carney syndrome. The relation between the two entities is not clear. METHODS: A patient with multiple chondromatous hamartomas of the lung is described in this article. The literature was reviewed with special reference to the relation between multiple chondromatous hamartomas of the lung and Carney syndrome as well as the triad of gastric epithelioid leiomyosarcoma, functioning extra-adrenal paraganglioma, and pulmonary chondroma. RESULTS: A total of 15 cases of multiple chondromatous hamartomas of the lung have been published worldwide. Two cases exhibited two other features of Carney syndrome, namely, gastric leiomyogenic neoplasms and extra-adrenal paragangliomas, and three other cases demonstrated only gastric leiomyomatous neoplasms. These five patients were all young females. CONCLUSIONS: Some patients with multiple chondromatous hamartomas of the lung have a history of Carney syndrome. Patients with multiple chondromatous hamartomas require further examination of other sites, particularly the stomach and nervous system.
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Kiryu et al. (1999) studied this question.
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