A Case Report of Familial Medullary Carcinoma Thyroid—Seldom Seen by Surgeons

Abstract

Medullary thyroid carcinoma (MTC) constitutes around 5% of all thyroid cancers with a worse prognosis. It accounts for 13% of thyroid cancer–related deaths. Twenty-three-year-old male presented with 4-year history of progressively increasing thyroid swelling with similar family history. On examination, butterfly-shaped firm swelling of size 7 × 3 cm in the right and 7 × 4 cm in the left can be seen on the anterior aspect of neck with regular margins and nodular surface moving with deglutition extending from the thyroid cartilage to clavicle head and laterally beyond the sternocleidomastoid into the posterior triangle muscle. Pemberton’s sign is negative. CT neck showed enlarged both thyroid lobes with areas of cystic degeneration and 15 mm retrosternal extension of left lobe of thyroid with bilateral IB, II, and V lymphadenopathy. Serum calcitonin level was 4435 pg/ml. FNAC favoured the features of MTC. Total thyroidectomy with central compartment neck dissection was done. Intraoperative frozen sections of bilateral level III were found to be tumour-free, so proceeded with thyroid excision and central compartment neck dissection. Histopathology revealed MTC with bilateral multifocal capsular and lymphovascular invasion and metastatic foci in the right central compartment lymph node. Hence, early diagnosis in family members offers a higher likelihood of cure and long-term survival.