• Carney complex;
  • ear tumors;
  • pediatrics;
  • PRKAR1A mutation;
  • protein kinase A

Myxomas of the ear are extremely uncommon, especially in young children. A 23-month-old girl born to a family with known Carney complex, a condition that predisposes to multiple myxomas, presented with a large, cauliflower-like mass located on the back of her right ear. Histopathologically, the lesion was composed of scattered spindle-shaped or stellate cells with abundant associated mucin and a very sparse inflammatory infiltrate containing lymphocytes and neutrophils. The patient was a carrier of a protein kinase A regulatory subunit 1A (PRKAR1A) mutation; PRKAR1A mutations cause Carney complex in most patients with this rare disorder. This is the earliest presentation of an ear lobe myxoma reported in the literature.

Briassoulis G, Quezado M, Lee C-CR, Xekouki P, Keil M, Stratakis CA. Myxoma of the ear lobe in a 23-month-old girl with Carney complex.