Adrenocortical carcinoma (ACC) is a rare and aggressive form of cancer that originates in the outer layer of the adrenal glands, known as the adrenal cortex. These glands, situated above each kidney, are responsible for producing hormones such as cortisol and aldosterone, which play roles in regulating various bodily functions. With an annual incidence of approximately one to two cases per million people, ACC is considered an “orphan” disease due to its infrequent occurrence. Its aggressive nature means tumors can grow rapidly and often spread to other parts of the body, such as the lungs or liver, even at the time of diagnosis.
Understanding Adrenocortical Carcinoma Survival Rates
A “survival rate” indicates the percentage of people with a specific type and stage of cancer who are still alive for a certain period after their diagnosis. These rates are often referred to as “5-year relative survival rates” and compare the survival of cancer patients to that of the general population.
These statistics are derived from large datasets. They are estimates and should not be seen as guarantees for any individual’s outcome.
Adrenocortical carcinoma has an overall 5-year relative survival rate of approximately 50% across all stages. For localized ACC, confined to the adrenal gland, the rate is around 73% to 80%. For regional disease, where cancer has spread to nearby tissues or lymph nodes, the rate typically ranges from 53% to 62%. In cases of distant metastasis, the 5-year relative survival rate is 0% to 39%.
Key Factors Influencing Survival
The stage of adrenocortical carcinoma at diagnosis is a significant determinant of survival outcomes. Early-stage detection, where the tumor is localized to the adrenal gland, correlates with a higher 5-year relative survival rate compared to more advanced stages. ACC is often diagnosed at later stages, including regional spread or distant metastases.
Tumor characteristics also play a role in prognosis. Larger tumor size is associated with a higher risk of malignancy and a less favorable outlook. The histological grade, which describes how aggressive the cancer cells appear under a microscope, indicates tumor behavior. Tumor hormone production (functional or non-functional) can influence symptoms and detection, though its direct impact on survival varies.
Patient-specific factors, such as age and overall health, affect outcomes. Younger patients and those in better general health often tolerate more aggressive treatments and may experience better recovery. Conversely, comorbidities or advanced age can limit treatment options or impact the body’s ability to withstand therapy.
The completeness of surgical resection is a major prognostic factor for ACC. Achieving a complete removal of the tumor (R0 resection), where no visible cancer remains, significantly improves long-term survival and reduces recurrence risk. If portions of the tumor are left behind, recurrence and poorer outcomes are more likely.
Treatment Strategies and Their Effect on Survival
Surgery is the primary treatment for adrenocortical carcinoma, especially for localized disease, offering the only potential for cure. The goal is to achieve a complete surgical resection, removing the entire tumor and any involved surrounding tissues or lymph nodes. This improves long-term survival and disease-free survival.
Following surgery, adjuvant therapy is considered to reduce cancer recurrence risk. Mitotane is a commonly used adrenolytic drug designed to suppress adrenal cortical cells and potentially eliminate microscopic residual disease. While its efficacy in improving survival after surgery has been debated, it is often prescribed to reduce recurrence. Patients receiving mitotane may experience side effects.
For advanced, metastatic, or recurrent ACC, systemic therapies like chemotherapy are used to manage disease and extend life. Regimens often combine different chemotherapy drugs, sometimes with mitotane, to target cancer cells throughout the body. While these treatments may not offer a cure for advanced disease, they can help control tumor growth, alleviate symptoms, and improve overall survival. However, response rates to chemotherapy for advanced ACC are modest.
Radiation therapy may be used in certain situations, such as for pain relief from bone metastases or to control local disease that cannot be surgically removed. It serves a palliative role in managing symptoms and improving comfort, rather than as a primary curative treatment for widespread ACC.
Navigating Prognosis and Support
While survival rates provide general insights, an individual’s prognosis for adrenocortical carcinoma is personal and varies significantly. These statistics are averages derived from large populations and do not account for every unique aspect of a patient’s health, tumor biology, or response to treatment. Individual outcomes are diverse, even among patients with similar disease stages.
A multidisciplinary approach to care is key to optimizing treatment and improving outcomes for ACC patients. This involves a team of specialists, including oncologists, endocrine surgeons, endocrinologists, and radiation oncologists, working together to develop a comprehensive and individualized treatment plan. This ensures all aspects of the disease are considered, from diagnosis to ongoing management and supportive care.
Patients are encouraged to be active participants in their care, seeking comprehensive information about their diagnosis and treatment options. Considering a second opinion from another specialized center can provide additional perspectives and confirm treatment strategies.
Navigating an ACC diagnosis also involves addressing the emotional and psychological impact. Engaging with support systems, such as patient advocacy groups, counseling services, or connecting with others who have faced similar challenges, can provide emotional support and practical advice. These resources can help patients and their families cope with the disease and its prognosis.