Multiple endocrine neoplasia type 2B
ICD-10 E31.8 · ICD-11 2F7A.0.3

First-line treatment for Multiple endocrine neoplasia type 2B with advanced or metastatic medullary thyroid carcinoma

Clinical scenario

This protocol applies to patients with Multiple endocrine neoplasia type 2B presenting with advanced, locally advanced, or metastatic medullary thyroid carcinoma accompanied by significant tumor burden and symptomatic or progressive disease as assessed by RECIST criteria.

Treatment approach

First-line systemic therapy centres on a single-agent tyrosine kinase inhibitor with dual activity against RET and VEGFR kinases. Full agent selection, sequencing, and management details are contained in the structured protocol.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1089/thy.2014.0335

In patients with significant tumor burden and symptomatic or progressive metastatic disease according to RECIST treatment with TKIs targeting both RET and VEGFR tyrosine kinases should be considered as systemic therapy.

The TKIs vandetanib or cabozantinib can be used as single-agent first-line systemic therapy in patients with advanced progressive MTC.

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