Authors' objectives: Thyroid cancer is the most common malignant endocrine tumour, but represents only about 1% of all malignancies.1 According to Cancer Research UK, 2,791 new cases of thyroid cancer were reported in England in 2013.2 The disease is more common in females than males: the age-standardised incidence rate is reported to be 7.7 per 100,000 persons in women and 3.1 per 100,000 persons in men.2 There are four main types of thyroid cancer: papillary, follicular, medullary and anaplastic. Medullary thyroid carcinoma (MTC), the disease type which will be considered within this appraisal, is a rare type of cancer that presents as a mass of tumours in the thyroid gland of the neck. MTC occurs in the parafollicular cells (also known as C-cells). Symptoms relating to pressure effects may include dysphagia (difficulty or discomfort in swallowing) and dysphonia (difficulty in speaking). There are four types of MTC: sporadic, multiple endocrine neoplasia (MEN) 2A and 2B and familial medullary thyroid carcinoma; approximately 75% of cases of MTC are sporadic in nature. MTC is rare and accounts for approximately 5% of all thyroid cancers 3% (adult) to 10% (paediatric) of all thyroid cancers.1 Treatment options for MTC include surgery, chemotherapy and radiotherapy. Recent guidelines from the British Thyroid Association (BTA)1 note that the use of surgery is common; surgery aims to remove some or all of the thyroid gland, and sometimes the lymph nodes. The BTA guidelines highlight that re-operative surgery in the neck and mediastinum provides long-term disease eradication in at least one third of patients and should be considered even when there are known distant metastases in order to minimise the risk of large volume disease compromising the airway, oesophagus or laryngeal nerves.1 In addition, palliative radiotherapy may serve a valuable role in the management of unresectable masses and painful bone metastases. Chemotherapy is rarely used and doxorubicin produces symptomatic response in fewer than 30 per cent of cases; most of these responses are partial and of short duration. Targeted therapies (vandetanib and cabozantinib) are the modality of choice for inoperable progressive and symptomatic MTC. Decisions concerning the use of these therapies in the first-line setting are guided by licensed indications and toxicity

Project Status: Completed

URL for project: https://www.journalslibrary.nihr.ac.uk/programmes/hta/165119/#/

Year Published: 2019

URL for published report: https://www.journalslibrary.nihr.ac.uk/programmes/hta/165119/#/

English language abstract: An English language summary is available

Publication Type: Not Assigned

Country: England, United Kingdom

Organisation Name: NIHR Health Technology Assessment programme

Contact Address: NIHR Journals Library, National Institute for Health and Care Research, Evaluation, Trials and Studies Coordinating Centre, Alpha House, University of Southampton Science Park, Southampton SO16 7NS, UK

Contact Name: journals.library@nihr.ac.uk

Contact Email: journals.library@nihr.ac.uk

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