ABSTRACT
Medullary thyroid carcinoma (MTC) constitutes around 5% of all thyroid cancers with a worse prognosis. It accounts for 13% ofthyroid cancer-related deaths. A 23-year-old male presented with a 4-year history of progressively increasing thyroid swellingwith similar family history. On examination, butterfly-shaped firm swelling of size 7 × 3 cm in the right and 7 × 4 cm in the leftseen on the anterior aspect of neck with regular margins and nodular surface moving with deglutition extending from the thyroidcartilage to clavicle head and laterally beyond the sternocleidomastoid into the posterior triangle muscle. Pemberton’s signwas negative. Computed tomography neck showed enlarged both thyroid lobes with areas of cystic degeneration and 15 mmretrosternal extension of the left lobe of thyroid with bilateral IB, II, and V lymphadenopathy. Serum calcitonin level was 4435 pg/ml.Fine-needle aspiration cytology favored features of MTC. Total thyroidectomy with central compartment neck dissection wasdone. Intraoperative frozen sections of bilateral level III were found to be tumor free, so proceeded with thyroid excision andcentral compartment neck dissection. Histopathology revealed MTC with bilateral multifocal capsular and lymphovascularinvasion and metastatic foci in the right central compartment lymph node. Hence, early diagnosis in family members offers ahigher likelihood of cure and long-term survival.