12
Treatment
Î In patients with significant tumor burden and symptomatic or
progressive metastatic disease according to Response Evaluation
Criteria in Solid Tumors (RECIST), treatment with tyrosine kinase
inhibitors (TKIs), targeting both RET and vascular endothelial growth
factor receptor, should be considered as systemic therapy. (A)
Note: The TKIs vandetanib or cabozantinib can be used as single agent first
line systemic therapy in patients with advanced progressive MTC.
Î Patients with advanced MTC and diarrhea should be treated initially with
anti-motility agents. Alternative therapies include somatostatin analogues
and local therapies such as surgery or chemoembolization. (C)
Î Patients with metastatic MTC and Cushing's syndrome due to ectopic
production of adrenocorticotropic hormone or corticotropin-releasing
hormone are often markedly debilitated and should be treated despite
their poor prognosis. (C)
Note: Treatment options include medical therapy with ketoconazole,
mifepristone, aminoglutethimide, metyrapone, or mitotane. In cases refractory
to medical treatment bilateral adrenalectomy is an option.
