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

Treatment of MEN2B in Metastatic Medullary Thyroid Carcinoma with Cushing's Syndrome Due to Ectopic ACTH or CRH Production

Clinical Scenario

This protocol addresses patients with Multiple endocrine neoplasia type 2B presenting with metastatic medullary thyroid carcinoma complicated by Cushing's syndrome arising from ectopic production of ACTH or CRH.

Patients in this setting are often markedly debilitated due to the combined burden of metastatic disease and ectopic ACTH- or CRH-driven hypercortisolism. Active management is indicated despite the poor prognosis.
Treatment Approach

Management in this scenario may include a surgical intervention directed at the source of excess cortisol production.

The complete structured regimen — including full decision criteria, sequencing, and management considerations — is available via the 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.

In cases refractory to medical treatment bilateral adrenalectomy is an option.

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