Amyloid goiter as an unusual presentation of primary systemic amyloidosis: A case report


Nuhoğlu İ., Civan N., Ucuncu O., KOÇAK M., COŞKUN H., TURGUTALP H., ...More

61th European Congress of Endocrinology, Wroclaw, Poland, 3 - 07 May 2014, pp.353

  • Publication Type: Conference Paper / Full Text
  • City: Wroclaw
  • Country: Poland
  • Page Numbers: pp.353

Abstract

Introduction: Deposition of amyloid substance within the thyroid gland causing clinically appearent enlargement of the gland is described as amyloid goiter. Although microscopic deposition of amyloid substance in thyroid gland is common in systemic amyloidosis, occurence of amyloid goiter is rare.

Case: A 55 years old male patient, who had been diagnosed with multinodular goiter for 10 years, was admitted to our clinic with complaint of increased swelling in his neck during the last 3 months. Physical examination revealed grade 3 palpable thyroid gland. Laboratory testing confirmed euthyroid status. On USG of the neck extensive enlargement of thyroid gland and multiple nodules, largest being 35 mm in size, was evident. On neck CT, the thyroid gland was extented into upper mediastinum and narrowing the trachea was evident (Default 1). Bilateral total thyroidectomy was performed and 380 g 15×11×5 cm sized thyroid mass was excised. Histopathological examination revealed deposition of homogenous eosinophylic appearanced amyloid substance in thyroid gland stroma. Immunhistochemical examination was negative for Amyloid A. Biopsy sample of bone marrow revelaed hypocelluler bone marrow with amyloid deposition and the patient was treated with VAD (vincristine, doxorubicin, dexhamethasone) for the diagnosis of primary amyloidoza.

Conclusion: Amyloid goiter as first sign of systemic amyloidozis is very rare condition. Thyroid gland amyloid deposition is more often occurs secondary and rarely to the primary amyloidozis. Presence of goiter have been reported in 0.04% of the cases with primary systemic amyloidozis. In cases with sudden enlargement of thyroid glands, amyloidosis should be considered as another possible cause alongside with malignancies.