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Keywords:

  • multiple endocrine neoplasia type 2;
  • pheochromocytoma;
  • screening;
  • adrenalectomy

Abstract

Childhood pheochromocytoma in the setting of multiple endocrine neoplasia type 2 (MEN2) remains rare and has not been reported under the age of 12. We present an 8-year-old female with known MEN 2A, C634Y RET mutation, diagnosed with a 6 cm pheochromocytoma requiring laparoscopic adrenalectomy. Given this patient's age at diagnosis, screening guidelines should recommend annual screening beginning at age 8 for patients with MEN 2B or MEN 2A codons 630 or 634 RET mutations. J. Surg. Oncol. 2013 108:203–206. © 2013 Wiley Periodicals, Inc.