Journal of Endocrinological Investigation

, Volume 28, Issue 3, pp 66–71 | Cite as

Thyroid hemiagenesis and incidentally discovered papillary thyroid cancer: Case report and review of the literature

  • A. M. Pizzini
  • G. Papi
  • S. Corrado
  • C. Carani
  • E. Roti
Case Report


Thyroid hemiagenesis (TH) is a rare congenital abnormality in which one thyroid lobe fails to develop. Its prevalence is uncertain, because the absence of one thyroid lobe does not usually cause clinical symptoms. The detection of TH is usually incidental when the evaluation of other thyroid disorders is requested. It is more frequently found in female than in male patients (3:1 ratio) and in the left lobe compared to the right lobe. We report the case of a 54-yr-old man, presenting with a large multinodular right-sided goiter, with mediastinal extension and dysphagia. Thyroid scan and ultrasound study showed the absence of the left lobe. The patient underwent surgery for compressive symptoms, and the operation confirmed the absence of the left lobe. Histological examination demonstrated a multinodular goiter with papillary carcinoma. To our knowledge, this case represents the first reported case of association between TH and papillary thyroid carcinoma in a male patient, and the second in which the tumor arose in the right lobe.


Thyroid hemiagenesis papillary thyroid carcinoma etiopathogenesis epidemiology 


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Melnick JC, Stemkowski PE. Thyroid hemiagenesis (hockey stick sign): a review of the world literature and a report of four cases. J Clin Endocrinol Metab 1981, 52: 247–51.PubMedCrossRefGoogle Scholar
  2. 2.
    Shabana W, Delange F, Freson M, Osteaux M, De Shepper J. Prevalence of thyroid hemiagenesis: ultrasound screening in normal children. Eur J Pediatr 2000, 59: 456–8.CrossRefGoogle Scholar
  3. 3.
    Ozaki O, Ito K, Mimura T, et al. Hemiaplasia of the thyroid associated with Graves’ disease: report of three cases and a review of the literature. Surgery Today 1994, 24: 164–9.PubMedCrossRefGoogle Scholar
  4. 4.
    Vakili R, Mazlouman SJ. Dyshormonogenic hypothyroidism with normal neurological development, unexplained short stature and facial anomalies in three siblings. Clin Dysmorphol 2003, 12: 21–7.PubMedCrossRefGoogle Scholar
  5. 5.
    Lee IT, Sheu WH, Lin SY. Familial form of thyroid dysgenesis: report of thyroid hemiagenesis with accompanying Graves’ disease in a woman whose daughter has thyroid agenesis. Horm Res 2003, 59: 47–9.PubMedCrossRefGoogle Scholar
  6. 6.
    McLean R, Howard N, Murray IP. Thyroid dysgenesis in monozygotic twins: variants identified by scintigraphy. Eur J Nucl Med 1985, 10: 346–8.PubMedCrossRefGoogle Scholar
  7. 7.
    Rajmil Ho, Rodriguez-Espinosa J, Soldevila J, Ordonez-Llanos J. Thyroid hemiagenesis in two sisters. J Endocrinol Invest 1984, 7: 393–4.PubMedCrossRefGoogle Scholar
  8. 8.
    Maiorana R, Carta A, Floriddia G, et al. Thyroid hemiagenesis: prevalence in normal children and effect on thyroid function. J Clin Endocrinol Metab 2003, 88: 1534–6.PubMedCrossRefGoogle Scholar
  9. 9.
    Mikosch P, Gallowitsch HJ, Kresnik E, Molnar M, Gomez I, Lind P. Thyroid hemiagenesis in an endemic goiter area diagnosed by ultrasonography: report of sixteen patients. Thyroid 1999, 9: 1075–84.PubMedCrossRefGoogle Scholar
  10. 10.
    McHenry CR, Walfish PG, Rosen IB, Lawrence AM, Paloyan E. Congenital thyroid hemiagenesis. Am Surg 1995, 61: 634–8, discussion 638–9.PubMedGoogle Scholar
  11. 11.
    Letonturier P, Hazard J, Tourneur R, Perlemuter L, Angel R. Thyroid hemiagenesis (single thyroid lobe). 8 cases. Nouv Presse Med 1979, 8: 1227–9.PubMedGoogle Scholar
  12. 12.
    Karabay N, Comlekci A, Canda MS, Bayraktar F, Degirmenci B. Thyroid hemiagenesis with multinodular goiter: a case report and review of the literature. Endocr J 2003, 50: 409–13.PubMedCrossRefGoogle Scholar
  13. 13.
    Braga-Basaria M, Basaria S, Mesa C Jr, Stolf AR, Graf H. Sonographically guided percutaneous ethanol treatment of a symptomatic complex nodule with a large cystic component in a patient with thyroid hemiagenesis. J Clin Ultrasound 2002, 30: 445–9.PubMedCrossRefGoogle Scholar
  14. 14.
    Attipou K, Cheynel N, Aubry K, et al. Bilobar thyroid agenesis. Ann Endocrinol (Paris) 2000, 61: 509–11.Google Scholar
  15. 15.
    Folsom DL, Gauderer MW, Dahms WT. Nodular hyperplasia, black thyroid, and chronic minocyclin ingestion in a teenager. Arch Surg 1992, 127: 1476–7.PubMedCrossRefGoogle Scholar
  16. 16.
    Graber AL. Multinodular goiter in thyroi hemiagenesis. J Tenn Med Ass 1983, 76: 74.Google Scholar
  17. 17.
    Vaswani K, Vitellas, KM, Bennett WF. Case 1. Thyroid hemiagenesis with adenoma. AJR Am J Roentgenol 2000, 175: 895.PubMedCrossRefGoogle Scholar
  18. 18.
    Piera J, Garriga J, Calabuig R, Bargallo D. Thyroidal hemiagenesis. Am J Surg 1986, 151: 419–21.PubMedCrossRefGoogle Scholar
  19. 19.
    Greening WP, Sarker SK, Osborne MP. Hemiagenesis of the thyroid gland. Br J Surg 1980, 67: 446–8.PubMedCrossRefGoogle Scholar
  20. 20.
    Mariani G, Molea N, Toni MG, Bianchi R. Thyroid hemiagenesis: a review of thirteen consecutive cases. J Nucl Med Allied Sci 1980, 24: 183–7.PubMedGoogle Scholar
  21. 21.
    Ozgen AG, Saygili F, Kabalak T. Thyroid hemiagenesis associated with Graves’ disease and Graves’ ophthalmopathy: case report. Thyroid 2004, 14: 75–7.PubMedCrossRefGoogle Scholar
  22. 22.
    Hervas Benito I, Vera Espallardo F, Rivas Sanchez A, et al. Association of thyroid hemiagenesis and Graves disease. Rev Esp Med Nucl 2001, 20: 116–9.PubMedCrossRefGoogle Scholar
  23. 23.
    Zangeneh F, Miller JL, Intenzo CM. Thyroid gland hemiagenesis with Graves’ disease. Thyroid 2001, 11: 603.PubMedCrossRefGoogle Scholar
  24. 24.
    Veliz J, Pineda G. Thyroid hemiagenesis associated with Basedow-Graves disease.Report of a case. Rev Med Chil 2000, 128: 896–8.PubMedGoogle Scholar
  25. 25.
    Bando Y, Nagai Y, Ushiogi Y, Toya D, Tanaka N, Fujisawa M. Development of Graves’ hyperthyroidism from primary hypothyroidism in a case of thyroid hemiagenesis. Thyroid 1999, 9: 183–7.PubMedCrossRefGoogle Scholar
  26. 26.
    Rashid HI, Yassin J, Owen WJ. A case of Graves’ disease in association with hemiagenesis of the thyroid gland. Int J Clin Pract 1998, 52: 515–6.PubMedGoogle Scholar
  27. 27.
    Shechner C, Kraiem Z, Zuckerman E, Dickstein G. Toxic Graves’ disease with thyroid hemiagenesis: diagnosis using thyroid-stimulating immunoglobulin measurements. Thyroid 1992, 2: 133–5.PubMedCrossRefGoogle Scholar
  28. 28.
    Sasaki H, Futata T, Ninomiya H, Higashi Y, Okumura M. CT and MR imagings of single thyroid lobe (thyroid hemiagenesis) with Graves’ disease. Postgrad Med J 1991, 67: 701.PubMedCentralPubMedCrossRefGoogle Scholar
  29. 29.
    Levy EG. Graves’ disease in a patient with thyroid hemiagenesis. Thyroidology 1989, 1: 49–50.PubMedGoogle Scholar
  30. 30.
    Matsumura LK, Russo EM, Dib SA, Maciel RM, Chacra AR. Hemiagenesis of the thyroid gland and T3 hyperthyroidism. Postgrad Med J 1982, 58: 244–6.PubMedCentralPubMedCrossRefGoogle Scholar
  31. 31.
    Mortimer PS, Tomlinson IW, Rosenthal FD. Hemiaplasia of the thyroid with thyrotoxicosis. J Clin Endocrinol Metab 1981, 52: 152–5.PubMedCrossRefGoogle Scholar
  32. 32.
    Burman KD, Adler RA, Wartofsky L. Hemiagenesis of the thyroid gland. Am J Med 1975, 58: 143–6.PubMedCrossRefGoogle Scholar
  33. 33.
    Papi G, Salvatori R, Ferretti G, Roti E. Thyroid hemiagenesis and autoimmune polyglandular syndrome type III. J Endocrinol Invest 2003, 26: 1160–1.PubMedCrossRefGoogle Scholar
  34. 34.
    Sharma R, Mondal A, Popli M, Sahoo M, Malhotra N, Soni S. Hemiagenesis of the thyroid associated with chronic lymphocytic thyroiditis. Clin Nucl Med 2001, 26: 506–8.PubMedCrossRefGoogle Scholar
  35. 35.
    Lazzarin M, Benati F, Menini C. Agenesis of the thyroid lobe associated with Hashimoto’s thyroiditis. Minerva Endocrinol 1997, 22: 75–7.PubMedGoogle Scholar
  36. 36.
    Hopwood NJ, Carroll RG, Kenny FM, Foley TP Jr. Functioning thyroid masses in childhood and adolescence. Clinical, surgical, and pathologic correlations. J Pediatr 1976, 89: 710–8.PubMedCrossRefGoogle Scholar
  37. 37.
    Shibutani Y, Inoue D, Koshiyama H, Mori T. Thyroid hemiagenesis with subacute thyroiditis. Thyroid 1995, 5: 133–5.PubMedCrossRefGoogle Scholar
  38. 38.
    Kreisner E, Camargo-Neto E, Maia CR, Gross JL. Accuracy of ultrasonography to establish the diagnosis and aetiology of permanent primary congenital hypothyroidism. Clin Endocrinol (Oxf) 2003, 59: 361–5.CrossRefGoogle Scholar
  39. 39.
    Leger J, Marinovic D, Garel C, Bonaiti-Pellie C, Polak M, Czernichow P. Thyroid developmental abnormalities in first degree relatives of children with congenital hypothyroidism. J Clin Endocrinol Metab 2002, 87: 575–80.PubMedCrossRefGoogle Scholar
  40. 40.
    Bergami G, Barbuti D, Di Mario M. Echographic diagnosis of thyroid hemiagenesis. Minerva Endocrinol 1995, 20: 195–8.PubMedGoogle Scholar
  41. 41.
    Devos H, Rodd C, Gagne N, Laframboise R, Van Vliet G. A search for the possible molecular mechanisms of thyroid dysgenesis: sex ratios and associated malformations. J Clin Endocrinol Metab 1999, 84: 2502–6.PubMedCrossRefGoogle Scholar
  42. 42.
    Huang SM, Chen HD, Wen TY, Kun MS. Right thyroid hemiagenesis associated with papillary thyroid cancer and an ectopic prelaryngeal thyroid: a case report. J Formos Med Assoc 2002, 101: 368–71.PubMedGoogle Scholar
  43. 43.
    Hsu CY, Wang SJ. Thyroid hemiagenesis accompanying an ectopic sublingual thyroid. Clin Nucl Med 1994, 19: 546.PubMedCrossRefGoogle Scholar
  44. 44.
    Tsang SK, Maher J. Thyroid hemiagenesis accompanying a thyroglossal duct cyst: a case report. Clin Nucl Med 1998, 23: 229–32.PubMedCrossRefGoogle Scholar
  45. 45.
    Maganini RJ, Narendran K. Hyperparathyroidism in a patient with thyroid hemiagenesis. IMJ Ill Med J 1977, 151: 368–70.PubMedGoogle Scholar
  46. 46.
    Duh QY, Ciulla TA, Clark OH. Primary parathyroid hyperplasia associated with thyroid hemiagenesis and agenesis of the isthmus. Surgery 1994, 115: 257–63.PubMedGoogle Scholar
  47. 47.
    Woods RH, Loury M. Thyroid hemiagenesis with a parathyroid adenoma. Otolaryngol Head Neck Surg 1992, 107: 469–71.PubMedGoogle Scholar
  48. 48.
    Cammareri V, Vignati G, Nocera G, Beck-Peccoz P, Persani L. Thyroid hemiagenesis and elevated thyrotropin levels in a child with Williams syndrome. Am J Med Genet 1999, 85: 491–4.PubMedCrossRefGoogle Scholar
  49. 49.
    Leiba S, Shani M, Zahavi I, Samuel R, Borohowsky S, Ber A. Secondary pituitary insufficiency: report of three cases of ectopia or hemiagenesis of the thyroid gland. Arch Intern Med 1976, 136: 1010–5.PubMedCrossRefGoogle Scholar
  50. 50.
    Rodriguez Cuartero A, Hernandez Burruezo JJ. Marfan’s syndrome and thyroid hemiagenesis. An Med Interna 1993, 10: 154.PubMedGoogle Scholar
  51. 51.
    Konno N, Kanaya A. Thyroid hemiagenesis associated with the right aortic arch. J Endocrinol Invest 1988, 11: 685–7.PubMedCrossRefGoogle Scholar
  52. 52.
    Shaha AR, Gujarati R. Thyroid hemiagenesis. J Surg Oncol 1997, 65: 137–40.PubMedCrossRefGoogle Scholar
  53. 53.
    Khatri VP, Espinosa MH, Harada WA. Papillary adenocarcinoma in thyroid hemiagenesis. Head Neck 1992, 14: 312–5.PubMedCrossRefGoogle Scholar
  54. 54.
    Harada T, Nichikawa Y, Ito K. Aplasia of one thyroid lobe. Am J Surg 1972, 123: 617.CrossRefGoogle Scholar
  55. 55.
    Hamburger JI, Hamburger SW. Thyroidal hemiagenesis. Arch Surg 1970, 100: 319–20.PubMedCrossRefGoogle Scholar
  56. 56.
    Rosenberg T, Gilhoa Y. Familial thyroid ectopy and hemiagenesis. Arch Dis Child 1980, 8: 6–39.Google Scholar
  57. 57.
    Macchia PE. Recent advances in understanding the molecular basis of primary congenital hypothyroidism. Mol Med Today 2000, 6: 36–42.PubMedCrossRefGoogle Scholar
  58. 58.
    Van Vliet G. Development of the thyroid gland: lessons from congenitally hypothyroid mice and men. Clin Genet 2003, 63: 445–55.PubMedCrossRefGoogle Scholar
  59. 59.
    Manley NR, Capecchi MR. The role of Hoxa-3 in mouse thymus and thyroid development. Development 1995, 121: 1989–2003.PubMedGoogle Scholar
  60. 60.
    Aghini-Lombardi F, Antonangeli L, Martino E, et al. The spectrum of thyroid disorders in an iodine-deficient community: the Pescopagano survey. J Clin Endocrinol Metab 1999, 84: 561–6.PubMedGoogle Scholar
  61. 61.
    Kao SY, Tu H, Chang RC, Yang AH, Chang KW, Lee CH. Primary ectopic thyroid papillary carcinoma in the floor of the mouth and tongue: a case report. Br J Oral Maxillofac Surg 2002, 40: 213–5.PubMedCrossRefGoogle Scholar

Copyright information

© Italian Society of Endocrinology (SIE) 2005

Authors and Affiliations

  • A. M. Pizzini
    • 1
  • G. Papi
    • 1
  • S. Corrado
    • 2
  • C. Carani
    • 1
  • E. Roti
    • 3
  1. 1.Endocrinology Unit, Department of Internal MedicineUniversity of Modena and Reggio EmiliaModena and Reggio EmiliaItaly
  2. 2.Section of Pathology, Department of Forensic and Morphological SciencesUniversity of Modena and Reggio EmiliaModena and Reggio EmiliaItaly
  3. 3.Institute of EndocrinologyUniversity of MilanMilanItaly

Personalised recommendations