Adjuvant therapy in adrenocortical carcinoma: prognostic factors and treatment options

Abstract

Adrenocortical carcinoma (ACC) is a rare cancer with high recurrence rates and heterogeneous clinical behavior. The role of adjuvant therapy remains unclear because of the challenges in collecting high-quality data for a rare cancer. The current treatment recommendations and guidelines for adjuvant therapy are mostly derived retrospectively from national databases and the treatment outcomes of patients seen in referral centers. To better select patients for adjuvant therapy, multiple factors need to be considered including staging, markers of cellular proliferation (such as Ki67%), resection margins, hormonal function, and possibly genetic alterations of the tumor as well as patient-related factors such as age and performance status. Adjuvant mitotane remains the most commonly used adjuvant therapy in ACC based on clinical practice guidelines, though emerging data from ADIUVO trial (mitotane vs. observation in low-risk ACC) suggest that mitotane use in low-risk patients may not be needed. An ongoing clinical trial (ADIUVO-2) is evaluating the role of mitotane vs. mitotane combined with chemotherapy in high-risk ACC. The use of adjuvant therapy has been controversial but can be justified in select patients with positive resection margins or after the resection of localized recurrence. A prospective study is needed to study the role of adjuvant radiation in ACC as radiation is expected to help only with local control without impact on distant microscopic metastases. There are no recommendations or published data about using adjuvant immunotherapy in ACC, but this may be a future study after establishing the efficacy and safety profile of immunotherapy in metastatic ACC.

Survival nomogram and risk classification system for patients with adrenocortical carcinoma: a study based on the SEER database and external validation in China

“…In spite of the ongoing controversy surrounding the utilization of adjuvant therapy, the administration of chemotherapy can be justified in a subset of patients exhibiting positive resection margins or following the resection of localized recurrence ( 29 ). Furthermore, our research indicates that chemotherapy also plays a significant role in predicting prognosis, as displayed by our nomogram.…”

Adrenocortical Carcinoma with a Renal Vein Thrombus Extending to the Inferior Vena Cava Successfully Resected with the Left Kidney and Distal Pancreatectomy: A Case Report

“…Mitotane- or cisplatin-based chemotherapy is recommended as systemic treatment for ACC [ 10 ]. However, adjuvant therapy for ACC is controversial because of the limited number of randomized controlled trials owing to the low incidence of the disease [ 11 ]. Recently, the European Society of Endocrinology and ENSAT 2018 guidelines recommend adjuvant mitotane treatment in more than 10% of patients with ACC who are positive for Ki-67, but further case numbers and examination including use of cytotoxic agents are necessary over the world [ 12 ].…”

Early Detection of Recurrence and Progress Using Serum Steroid Profiling by LC–MS/MS in Patients with Adrenocortical Carcinoma

“…Currently, the management of patients with ACC includes surgery followed by adjuvant therapies (e.g., mitotane, radiotherapy or platin-based therapy) or active surveillance in a subset of low-risk patients with localized disease [ 1 , 7 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 ]. Treatment of recurrent disease is usually individualized [ 31 ] whereas in advanced disease, mitotane ± chemotherapy are the cornerstones of therapy [ 32 , 33 , 34 ].…”