Multiple endocrine neoplasia type 2A
ICD-10 E31.8 · ICD-11 2F7A.0.2

Treatment of MEN2A with Cushing's Syndrome in Metastatic Medullary Thyroid Carcinoma

This protocol addresses a distinct and serious presentation of Multiple endocrine neoplasia type 2A: metastatic medullary thyroid carcinoma accompanied by Cushing's syndrome arising from ectopic ACTH or CRH production.

Clinical Scenario

Metastatic medullary thyroid carcinoma with concurrent Cushing's syndrome due to ectopic ACTH or CRH secretion — a combination that significantly compromises patient condition and shapes the management strategy.

Patients presenting with this combination are often markedly debilitated. Treatment is nonetheless indicated despite the poor prognosis, reflecting the clinical importance of addressing the cortisol excess in this context.

Treatment Approach

In this scenario, management may include a surgical intervention directed at controlling cortisol excess. The complete approach — including indications, sequencing, and all relevant steps — is detailed in the full protocol below.

References

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.

DOI: 10.1089/thy.2014.0335 View source ↗