Imaging features of Carney complex with external auditory canal myxoma.

Abstract

A 46-year-old man, with Carney complex (CC) and a history of recurrent atrial and ventricular myxomas, experienced left ear fullness for 18 months. He complained of hearing difficulties, temporarily resolved by ear canal manipulation, with no vestibular symptoms, earache or ear drainage. The patient also had a family history of cardiac myxoma and biochemical evidence of adrenal cortex autonomy. Physical examination and dedicated head and neck imaging showed a nearly occlusive soft tissue nodule in the left external auditory canal (Fig. 1A and B). Surgical resection and histopathologic examination revealed a myxoma (Fig. 1C). CC is a rare genetic syndrome related to inactivating mutation, either of random occurrence or from autosomal dominant transmission, of the protein kinase A (PRKAR1A) located on chromosome 17. CC is characterized by cardiac myxoma (Fig. 2A and B), cutaneous or mucosal myxoma, lentiginosis, multiple blue nevi, endocrinopathies with dominant...