From the Guidelines
Post-operative management of adrenocortical carcinoma (ACC) should prioritize complete en bloc resection of all adrenal tumors suspected to be ACC by an experienced surgeon, followed by adjuvant mitotane therapy in patients at high risk of recurrence. The management and monitoring of ACC post-operatively involve a multidisciplinary approach, considering the patient's overall health, tumor characteristics, and risk of recurrence.
- Key considerations include:
- Complete en bloc resection of the tumor, with locoregional lymphadenectomy, as the mainstay of treatment 1
- Perioperative hydrocortisone replacement in patients with autonomous cortisol secretion 1
- Adjuvant mitotane therapy in patients at high risk of recurrence, such as those with stage III disease, R1-RX resection, or Ki-67 index >10% 1
- Radiological imaging every 3 months for 2 years, then every 3-6 months for at least another 3 years, is recommended for monitoring 1. This approach allows for early detection of recurrence and prompt intervention.
- Adjuvant therapy, including mitotane and radiation therapy, should be discussed on an individual basis, considering the patient's risk of recurrence and overall health 1.
- Laboratory monitoring, including serum cortisol, aldosterone, androgens, and 24-hour urinary steroid profiles, should be performed regularly to assess for recurrence and monitor the effects of adjuvant therapy.
- A multidisciplinary team, including endocrinologists, oncologists, and surgeons, should be involved in the management of ACC patients to ensure comprehensive care and optimal outcomes.
From the Research
Management of Adrenocortical Carcinoma Post-Op
- The management of adrenocortical carcinoma (ACC) post-op involves a multidisciplinary approach, including surgery, adjuvant therapy, and monitoring for recurrence 2, 3.
- Adjuvant mitotane treatment is suggested for patients after radical surgery who have a perceived high risk of recurrence, such as those with ENSAT stage III, R1 resection, or Ki67 >10% 2.
- For advanced ACC not amenable to complete surgical resection, local therapeutic measures such as radiation therapy, radiofrequency ablation, and chemoembolization may be of value 2, 4.
- The combination of etoposide, doxorubicin, cisplatin, and mitotane (EDP-M) is a treatment option for metastatic ACC, with a reported median progression-free survival of 5.0 months and overall survival of 14.8 months 4, 5.
Monitoring for Recurrence
- Patients with ACC should be monitored regularly for recurrence, including clinical assessment, endocrine work-up, and imaging studies 2, 3.
- The frequency and modality of monitoring should be individualized based on the patient's risk of recurrence and overall health status 3.
- In patients with recurrent disease, surgery or other local therapies may be considered if a complete resection or ablation seems feasible 2.
Adjuvant Therapy
- Adjuvant therapy with mitotane may improve recurrence-free and overall survival in patients with ACC 4.
- The use of adjuvant therapy should be individualized based on the patient's risk of recurrence and overall health status 2, 3.
- Other adjuvant therapies, such as radiation therapy, may also be considered in selected patients 4.