Ectopic thymus presenting as a thyroid nodule in a patient with the Carney complex

Thyroid. 2009 Mar;19(3):293-6. doi: 10.1089/thy.2008.0404.

Abstract

Ectopic thymic tissue within the thyroid gland is rare. Patients with a complex of myxomas, spotty skin pigmentation, and endocrine overactivity, collectively known as Carney complex (CNC), have a predisposition towards the development of thyroid abnormalities, but there are no reports of thymic defects in CNC. We present the case of a 12-year-old boy with CNC and a growing thyroid nodule. The patient had the c.682 C > T (Arg228X) pathogenic PRKAR1A mutation. Hemithyroidectomy for a Hürthle cell adenoma led to the confirmation of distinct intrathyroidal ectopic thymic tissue. Thymic abnormalities have not been previously reported in CNC.

Publication types

  • Case Reports
  • Research Support, N.I.H., Extramural
  • Research Support, N.I.H., Intramural

MeSH terms

  • Adenoma, Oxyphilic / pathology
  • Child
  • Choristoma / pathology*
  • Cyclic AMP-Dependent Protein Kinase RIalpha Subunit / genetics
  • DNA / biosynthesis
  • DNA / genetics
  • Diagnosis, Differential
  • Humans
  • In Situ Hybridization, Fluorescence
  • Lentigo / complications
  • Lentigo / pathology*
  • Male
  • Multiple Endocrine Neoplasia / complications
  • Multiple Endocrine Neoplasia / pathology*
  • Pigmentation Disorders / complications
  • Pigmentation Disorders / pathology*
  • Syndrome
  • Thymus Gland / pathology*
  • Thyroid Nodule / pathology*
  • Thyroidectomy

Substances

  • Cyclic AMP-Dependent Protein Kinase RIalpha Subunit
  • PRKAR1A protein, human
  • DNA