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

Treatment of MEN 2B in Metastatic Medullary Thyroid Carcinoma with Cushing's Syndrome due to Ectopic ACTH or CRH Production

This protocol covers the management of Multiple endocrine neoplasia type 2B (MEN 2B) in patients with metastatic medullary thyroid carcinoma who develop Cushing's syndrome arising from ectopic ACTH or CRH production — a particularly challenging clinical presentation.

Patients with metastatic MTC and Cushing's syndrome due to ectopic production of ACTH or CRH are often markedly debilitated and should be treated despite their poor prognosis.

Treatment Approach

Medical therapy is the primary strategy for addressing Cushing's syndrome in this setting. The specific agents, selection criteria, and clinical sequencing are detailed in the full protocol.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1089/thy.2014.0335

Patients with metastatic MTC and Cushing's syndrome due to ectopic production of ACTH or CRH are often markedly debilitated and should be treated despite their poor prognosis.

Treatment options include medical therapy with ketoconazole, mifepristone, aminoglutethimide, metyrapone, or mitotane.

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