ABSTRACT

Introduction 453

History 453

Incidence of medullary thyroid cancer 453

Pathology 454

Hereditary medullary thyroid cancer 455

Clinical features of sporadic and hereditary MTC 456

Diagnosis of medullary thyroid cancer 457

Preoperative investigations 458

Surgery for MTC 460

Persistent/recurrent hypercalcitonaemia and recurrent MTC 462

Outcome and prognosis 463

Non-surgical treatment of MTC 464

Prophylactic surgery for hereditary MTC 465

Case histories 466

Key evidence 466

Key learning points 466

References 467

I will not cut y but will leave this to be done by men who are practitioners of this work y

Extract from the Hippocratic Oath, fourth century BCE

Medullary thyroid cancer (MTC) is uncommon and with the exception of those who work in tertiary referral centres will rarely be diagnosed or treated by individual surgeons. MTC is unique in that it occurs as genetically determined disease in 25 per cent of cases; it occurs in the paediatric and adult setting and is associated with other endocrine tumours. MTC merits a more aggressive surgical approach than differentiated thyroid cancer. Patients with MTC require care from an experienced multidisciplinary team that includes specialist surgeons, an endocrinologist, oncologist and pathologist who meet and discuss cases regularly with a radiologist, nuclear medicine physician and biochemist, all interested in the management of thyroid cancer. Close links and involvement with personnel from a Clinical Genetics Service are essential. National thyroid cancer guidelines in the UK recommend that patients with MTC should be referred for treatment to a cancer centre.