Abstract

A case of wuchernde Struma of the thyroid associated with dyshormonogenetic goiter is reported. A 75-year-old woman was seen at the hospital because of enlarging tumor on the neck and dysphagia. There was a previous history of undergoing right thyroid lobectomy for congenital goiter. A fist-sized tumor was palpabled in the left anterior neck. Dyshormonogenetic goiter was diagnosed by perchlorate positive discharge. On suspicion of thyroid carcinoma due to findings of neck X-ray, ultrasonogram and computed tomography, total thyroidectomy was performed. The resected thyroid was 15.3×7.6×7.0 cm (220 g) and showed the solid appearance with many hard tumors in the fibous capsule. Histologically, the thyroid was like a adenomatous goiter, partially associated with poorly differentiated follicular carcinoma (wuchernds Struma).Dyshormonogenetic goiter is defined as defect in the steps involved in thyroid hormone synthesis due to inborn errors of metabolism which in turn causes hyperplasia of the thyroid. Ten cases of thyroid carcinoma associated with dyshormonogenetic goiter including this case have been reported in the world.

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