title: Ectopic thyroid tissue in the adrenal gland: a report of two cases with pathogenetic implications.
abstract: BACKGROUND
  Ectopic thyroid tissue is usually found anywhere along the embryonic descent pathway of the medial thyroid anlage from the tongue to the trachea (Wölfler area). However, ectopic thyroid tissue in the adrenal gland (ETTAG) is not easy to understand on the basis of thyroid embryology; because it is so rare, the possibility of metastasis should first be considered. Here, we describe two cases of ETTAG with pathogenetic implications and review the associated literature.
  
  
  METHODS
  Two cases of ETTAG presented as incidental cystic adrenal masses in adult females, one having a congenital hernia of Morgagni. The ETTAG was histologically indistinguishable from normal orthotopic thyroid tissue, and its follicular nature was confirmed by immunohistochemical positivity for thyroglobulin, thyroperoxidase, thyroid transcription factor-1 (TTF-1/Titf-1/Nkx2.1), cytokeratin AE1/AE3, cytokeratin 7, pendrin, human sodium iodide symporter, paired box gene 8, and forkhead box E1 (TTF-2), as well as positivity for the messenger RNA of the thyroglobulin gene by in situ hybridization analysis. No C cells (negativity for calcitonin, chromogranin, and synaptophysin) were present. Neither BRAF nor KRAS mutations were detected with real-time polymerase chain reaction analysis. Further work-up did not show evidence of thyroid malignancy.
  
  
  CONCLUSIONS
  ETTAG is a rare finding, with only seven cases reported; women are much more frequently affected than men (8:1), and it usually presents in the fifth decade (mean age 54, range 38-67) as a cystic adrenal mass incidentally discovered on abdominal ultrasonography and/or in computed tomography images. ETTAG is composed of normal follicular cells without C cells. The expression of some transcription factors (TTF-1, paired box gene 8, and FOXE1) involved in development and/or migration of the medial thyroid anlage is preserved. Coexistence of a congenital hernia of Morgagni in one patient suggests an overdescent of medial thyroid anlage-derived cells in its pathogenesis.
  
  
  CONCLUSIONS
  Although ETTAG pathogenesis remains unknown, the lack of C cells together with the coexistence of a congenital defect of the anterior diaphragm (hernia of Morgagni) in one of our patients could suggest an overdescent of medial thyroid anlage-derived cells in the origin of this heterotopia.
authors.affiliation: 1 National Cancer Institute , Bogota, Colombia .
authors.forename: Alfredo
authors.initials: A
authors.lastname: Romero-Rojas
authors.forename: María Rosa
authors.initials: MR
authors.lastname: Bella-Cueto
authors.forename: Ivonne A
authors.initials: IA
authors.lastname: Meza-Cabrera
authors.forename: Angeles
authors.initials: A
authors.lastname: Cabezuelo-Hernández
authors.forename: Darío
authors.initials: D
authors.lastname: García-Rojo
authors.forename: Hernando
authors.initials: H
authors.lastname: Vargas-Uricoechea
authors.forename: José
authors.initials: J
authors.lastname: Cameselle-Teijeiro
mesh_terms: D000307:Adrenal Gland Diseases / Q000473:pathology* / Q000601:surgery; D000328:Adult; D002828:Choristoma / Q000473:pathology* / Q000601:surgery; D005260:Female; D006801:Humans; D008875:Middle Aged; D013961:Thyroid Gland