متلازمة ACTH الهاجرة: عندما يفشل استئصال الورم في تحقيق الهدأة

References

• We recommend steroidogenesis inhibitors under the following conditions: as second-line treatment after TSS in patients with CD, either with or without RT/radiosurgery; as primary treatment of EAS in patients with occult or metastatic EAS; and as adjunctive treatment to reduce cortisol levels in ACC.
• We suggest bilateral adrenalectomy for occult or metastatic EAS or as a life-preserving emergency treatment in patients with very severe ACTH-dependent disease who cannot be promptly controlled by medical therapy.
• We suggest targeted therapies to treat ectopic ACTH syndrome.
• Several case reports show that octreotide, a potent SST2 agonist, may control ACTH and cortisol secretion for a short- to midterm period in patients with recurrent or unresectable ectopic ACTH-secreting tumors.
• In three reports, the tyrosine kinase inhibitors vandetanib and sorafenib rapidly and fully controlled hypercortisolism caused by ACTH secretion from metastatic medullary thyroid carcinomas.
• The goal is clinical normalization using cortisol levels as a proxy end-point (except for mifepristone, see below).
• With no cross-reactivity issues, the treatment target is either UFC in the normal range or mean serum cortisol levels that are between 5.4 and 10.8 µg/dL (150–300 nmol/L) throughout the day.