Adrenocorticol Carcinoma Treatment

Adrenocorticol Carcinoma – Treatment

Adrenocorticol carcinomas (ACC) are a rare and aggressive type of cancer that can cause Cushing’s syndrome, hyperaldosternonism, feminization or virilization.  ACC can produce hormonal symptoms that send a patient to the doctor for tests or it can produce no symptoms and be discovered incidentally. The rarity of this malignancy has inhibited progress in creating treatment plans.  Current clinical trials for recurrent and advanced ACC are attempting to discover better ways to treat this disease. Some of the trials are studying molecularly targeted therapy.

Treatment for a patient who is diagnosed with an adrenalcorticol carcinoma includes several options. The primary treatment for an adrenal carcinoma tumor is surgery. Total surgical resection or removal of the tumor is the best treatment option. Patients that are diagnosed at Stage I to Stage III and do not have any other conditions that would rule out surgery are the best candidates for resection.

A patient cannot be considered for surgery until a complete hormonal assessment indicates the secretory activity of the tumor. If the assessment shows that the tumor is producing cortisol, the patient will require glucocorticoid coverage to prevent postoperative adrenal insufficiency. To avoid incomplete resection, an experienced surgeon should perform the surgery. Removal of lymph nodes as a preemptive treatment has not been proved, so only suspicious lymph nodes should be removed. Completeness of the resection and tumor stage at diagnosis are the most important factors to determine the prognosis of the adrenal carcinoma. An improved prognosis is given to patients with low-grade tumors and no evidence of spread to local tissues or lymph nodes.

An oral antineoplastic drug, mitotane, is used in conjunction with surgery for patients at the highest risk for recurrence. Patients that have high-grade tumors, tumors that have invaded other local tissue, and/or some large tumors that are low grade but have vascular involvement are likely to have recurrent disease and should be placed on the drug within three months of surgery. The drug has also been used to limit recurrence in patients whose cancer is treated without surgery. Five years of mitotane treatment is recommended for patients with a high-risk diagnosis.  Low-risk patients typically receive two to three years of treatment.

Chemotherapy drugs are usually added to mitotane as part of a treatment plan. It is generally accepted that the combination therapy produces better outcomes than mitotane treatment alone, although research does not show a significant increase of being cured. Patients with extensive or rapidly progressive ACC will always take mitotane combined with chemotherapy. Radiation therapy may be added to a patient’s treatment plan if they have incomplete resected tumors, a stage III diagnosis, a tumor spread to local tissue at the time of surgery, or a high-grade diagnosis.

Adrenal function of patients with ACC should be closely monitored because they can experience adrenal insufficiency (caused by mitotane or resection surgery) or excess cortisol production (caused by tumor activity). Hypercortisolism can be present in patients not taking mitotane or those whose hypercortisolism is not controlled by mitotane. If this case, a more specific adrenal enzyme inhibitor is often added to the treatment plan.  Control of glucose, potassium and blood pressure is necessary.  Infection control is imperative because patients can die from infections related to the immunosuppression induced by hypercortisolism and chemotherapy.

Glucocorticoid replacement is essential after resection of cortisol-secreting ACC. It should also be given to patients taking mitotane, because the drug’s adrenolytic activity will produce adrenal insufficiency. Hydrocortisone is the treatment of choice for glucocorticoid replacement. Post-operative radiation therapy is indicated for all patients with incompletely resected ACC tumors, stage III or high-grade diagnosis or spillage of the tumor at the time of resection (the tumor’s protective covering was violated).

Post-operative treatment includes CT scans of the chest and abdomen every three months for two years, then every six months for five years. Percutaneous radiofrequency ablation may be included in the treatment plan for short-term local control for a tumor that was not resected at the time of surgery, especially those less than five centimeters.  Radiation therapy can provide palliative care for symptoms of advanced local ACC or metastatic tumors.

References:

Adrenocortical Carcinoma Treatment. Retrieved from https://www.cancer.gov/types/adrenocortical/hp/adrenocortical-treatment-…

Lacroix, André and Hammer, Gary D. (2016). Treatment of adrenocortical carcinoma. UpToDate. Retrieved from https://www.uptodate.com/contents/treatment-of-adrenocortical-carcinoma.