SFE2004 Oncology Strand Neuroendocrine Tumours (4 abstracts)
Royal Hallamshire Hospital, Sheffield, UK.
Adult and paediatric patients with Medullary Thyroid Cancer (MTC) may present in a variety of clinical scenarios. These include patients with a neck mass and 'apparently' sporadic disease or the index case of genetically determined disease (MEN2A/2B or FMTC), individuals from known MTC kindred who may be symptomatic or screen detected (either gene positive with an elevated basal / stimulated calcitonin, or gene positive and normocalcitonaemic) and patients with recurrent disease. Each group may require a specific and tailored surgical approach.
Best practice in established MTC at initial presentation includes whenever possible a preoperative diagnosis, exclusion of phaeochromocytoma prior to neck surgery and an appropriate surgical intervention, a minimum of total thyroidectomy and central neck lymph node dissection. All patients diagnosed with MTC should undergo RET mutation analysis to exclude familial disease. Follow up will require a multidisciplinary approach and close liaison with clinical chemistry and diagnostic imaging services.
Although operation on patients who present with symptomatic disease may not achieve biochemical or clinical cure, long-term survival even with established visceral metastases is common. The role of the surgeon is to minimise the risk of and / or effectively treat loco regional relapse in the neck and mediastinum to prevent airway, oesophageal or neurological complications that diminish quality of life and result in early death.