Duygu AYAZ, Demet ETİT, Süheyla CUMURCU, Yetkin KOCA, Ali SAYAN

Çocukta Tiroid Medüller Mikrokarsinom: Olgu Sunumu

Medüller tiroid karsinomu, parafolliküler C hücrelerinden oluşan nöroendokrin kökenli nadir bir tümördür. Tüm tiroid kanserlerinin %3-12’sini oluşturur. Medüller tiroid kanserlerinin yaklaşık %75’i sporadiktir. Geri kalan vakalar kalıtsal multipl endokrin neoplazi (MEN) 2A, 2B veya ailesel medüller karsinom sendromu ile ilişkilidir. Medüller karsinomun kalıtsal formları RET proto-onkogeninin otozomal dominant mutasyonuna bağlıdır. Aile öyküsü olan 6 yaşındaki erkek çocukta RET proto-onkogen mutasyonu saptanması üzerine profilaktik tiroidektomi yapıldı. Histopatolojik incelemede, bilateral medüller mikrokarsinom ve C hücre hiperplazisi saptandı. Bu olguyu, nadir görülmesi nedeniyle literatür bilgileri eşliğinde sunuyoruz.

Thyroid Medullary Microcarcinoma in Child: Case Report

Medullary carcinoma is an uncommon neuroendocrine tumour consisting of parafollicular C cells. It is representing 3-12% of all thyroid cancers. About 75% of medullary thyroid cancers are sporadic. Other cases are heritable, being associated with multipl endocrine neoplasia (MEN) 2A, MEN 2B or with the familial medullary thyroid carcinoma syndrome. Inherited forms of medullary thyroid carcinoma are due to autosomal dominant mutations of the RET protooncogene. Prophylactic thyroidectomy was performed upon detection of RET protooncogen mutation in a 6-years-old boy with family history. Histopatological examination revealed bilateral medullary microcarcinoma and C cell hyperplasia. We wanted to present this case because of it’s rare frequency.

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