Carcinoma of dual ectopic thyroid gland in a patient with cyanotic congenital heart disease—A case report and review of literature

  • Umashankar K. Ballehaninna
    Department of Surgery, Brooklyn, NY 11219. Tel.: +1 718 283 7683; fax: +1 718 635 7399.
    Department of Surgery, All India Institute of Medical Sciences, Ansarinagar, New Delhi, India
    Department of Surgery, Maimonides Medical Center, Brooklyn, NY, USA
    Search for articles by this author


      Carcinoma arising from an ectopic thyroid gland in the absence of a normal thyroid gland is uncommon. There is a clinically relevant and unique association of ectopic thyroid with congenital heart disease which in turn is related to thyroid embryogenesis. The median thyroid anlage develops adjacent to the newly differentiated myocardium during the 3rd week of intrauterine life. The downward pull exerted by continued descent of heart is postulated to assist in the descent of the thyroid to its final location in the lower cervical position. Ectopic thyroid tissue can result from abnormal heart development or abnormal interaction between thyroid primordium and heart. We present a rare association of cancer of the ectopic thyroid gland in a patient with congenital heart disease.

      Key words

      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


      Subscribe to Journal of Pediatric Surgery
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


        • Fish J.
        • Moore R.M.
        Ectopic thyroid tissue and ectopic thyroid carcinoma.
        Ann Surg. 1963; 157: 213-222
        • Massine R.E.
        • Durning S.J.
        • Koroscil T.M.
        Lingual thyroid carcinoma: a case report and review of the literature.
        Thyroid. 2001; 11: 1191-1196
        • Klubo-Gwiezdzinska J.
        • Manes R.P.
        • Chia S.H.
        • et al.
        Clinical review: ectopic cervical thyroid carcinoma—review of the literature with illustrative case series.
        J Clin Endocrinol Metab. 2011; 96: 2684-2691
        • Ryu H.S.
        • Chung Y.J.
        • Chong S.
        • et al.
        Ectopic intrapulmonary thyroid tissue mimicking metastatic tissue.
        Thyroid. 2012; 22: 755-759
        • Landeta F.
        • Hoffmeier A.
        • Fuchs M.
        • et al.
        Ectopic thyroid mass in the heart.
        Lancet. 2012; 379: 1762
        • Patel S.G.
        • Escrig M.
        • Shaha A.R.
        • et al.
        Management of well-differentiated thyroid carcinoma presenting within a thyroglossal duct cyst.
        J Surg Oncol. 2002; 79: 134-139
        • Plaza C.P.
        • Lopez M.E.
        • Carrasco C.E.
        • et al.
        Management of well-differentiated thyroglossal remnant thyroid carcinoma: time to close the debate? Report of five new cases and proposal of a definitive algorithm for treatment.
        Ann Surg Oncol. 2006; 13: 745-752
      1. Braverman L.E. Utiger R.D. Werner and Ingbar's the thyroid: a fundamental and clinical text. 9th ed. Lippincott–Williams & Wilkins, Philadelphia2005
        • Kreisner E.
        • Neto N.C.
        • Gross J.L.
        High prevelance of extrathyroid malformations in a cohort of Brazilian patients with permanent primary congenital hypothyroidism.
        Thyroid. 2005; 15: 165-169
        • De Felice M.
        • Di Lauro R.
        Thyroid development and its disorders: genetics and molecular mechanisms.
        Endocr Rev. 2004; 25: 722-746
        • Hirnle T.
        • Szymczak J.
        • Ziółkowski P.
        • et al.
        Ectopic thyroid malignancy in the right ventricle of the heart.
        Eur J Cardiothorac Surg. 1997; 12: 147-149
        • Wildi-Runge S.
        • Stoppa-Vaucher S.
        • Lambert R.
        • et al.
        A high prevalence of dual thyroid ectopy in congenital hypothyroidism: evidence for insufficient signaling gradients during embryonic thyroid migration or for the polyclonal nature of the thyroid gland.
        J Clin Endocrinol Metab. 2012; 97: E978-E981
        • Pradeep P.V.
        • Jayashree B.
        Ectopic thyroid with Hashimoto's thyroiditis presenting as goitrous hypothyroidism.
        Eur J Pediatr Surg. 2011; 21: 339-340
        • Karakoc-Aydiner E.
        • Turan S.
        • Akpinar I.
        • et al.
        Pitfalls in the diagnosis of thyroid dysgenesis by thyroid ultrasonography and scintigraphy.
        Eur J Endocrinol. 2012; 166: 43-48
        • Sood A.
        • Sood V.
        • Sharma D.R.
        • et al.
        Thyroid scintigraphy in detecting dual ectopic thyroid: a review.
        Eur J Nucl Med Mol Imaging. 2008; 35: 843-846