Super-Duper Rare Thyroid Cancers — ASN Events

Super-Duper Rare Thyroid Cancers (#8)

John Watkinson 1
  1. University Hospitals Birmingham, Birmingham, United Kingdom

Super-duper Thyroid Cancers arise in a number of situations. Firstly, the primary tumours such as the rarer types of Differentiated Thyroid Cancer (DTC), i.e. Tall Cell and Insular Variants, also included are Thyroid Lymphoma, Anaplastic Carcinoma, Thyroid Cancer in Children and Ectopic Thyroid Tissue. Within all groups rare situations arise when disease involves either the airway or the neck. Another important sub-group of rare cancers includes Medullary Thyroid Cancer (MTC) which can be both congenital and acquired. A third are genetically positive and can be associated with syndromes such as MEN -1, MEN-2a and MEN-2b. Personalised medicine now means an increased focus on genetic testing in RET negative families. Epidemiological studies indicate the heritable component of thyroid cancer is relatively uncommon unless there are >2 affected relatives. There are however several distinctive autosomal dominant DTC syndromes that may present with characteristic phenotypes, tumour associations and early onset thyroid cancer. Cowden syndrome (a PTEN hamartoma tumour syndrome) has been reported with a high frequency of benign thyroid disease and DTC (as early as 6 years), learning difficulties, macrocephaly as well as breast and endometrial cancer in adulthood. Childhood DTC is also seen in the DICER1 related syndrome, most commonly presenting with congenital pleuro-pulmonary-blastoma and both benign and malignant tumours before the age of 10. In familial adenomatous polyposis approximately 12% of female gene carriers develop follicular thyroid cancer in adulthood. Patients are best dealt with in regional multidisciplinary clinics who understand these disorders and take a three generation family history when required. Thyroid recurrence following treatment is rare, and stratified approaches are required in specific situations. In addition rarely the Thyroid can be the source of isolated secondary deposits from the breast, kidney & melanoma and examples of these will be discussed.

#COSA2015