This protocol addresses Cushing's syndrome arising in the context of adrenocortical carcinoma (ACC). Patients with ACC carry a poor prognosis, and definitive management of the underlying tumour is central to care.
Hypercortisolism in this setting is associated with increased mortality, making cortisol control an essential concurrent objective.
Target: eucortisolismThe cornerstone of this first-line protocol is complete surgical resection of the adrenocortical tumour. Adjunctive steroidogenesis inhibitors are incorporated as part of the approach to reduce cortisol levels and achieve eucortisolism.
The complete protocol specifies surgical strategy, selection and application of adjunctive therapy, and the criteria defining treatment success — all available via the link below.
DOI: 10.1210/jc.2015-1818
Patients with ACC have a poor prognosis, and clinicians should attempt definitive treatment via complete resection.
Surgeons increasingly perform transperitoneal laparoscopic adrenalectomy, particularly for small tumors, but this is controversial because many recommend open surgery to assess stage and achieve en bloc resection.
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 adrenocortical carcinoma (ACC).
Because hypercortisolism is associated with increased mortality, medical treatment may be needed to achieve eucortisolism.
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