Adrenocortical cancer: diagnostic and curative peculiarities

Abstract

Adrenocortical carcinoma (ACC) is a rare endocrine malignant tumor with an average incidence of two cases per one million population per year. Approximately 4% of  adrenal incidentalomas are an ACC. This tumor is quite aggressive and is a cause of cancer-related deaths in 0.2% of cases, but their prevalence among other human malignancies represents only 0.02%. This is one of the reasons for a poor five-year survival, varying from 32% to 45%. A complete surgical resection is the sole  potentially effective therapy for ACC. The possibility of a complete removal of the tumor and regional metastases is an essential predicting factor of the disease. But in  carefully selected patients a local recurrence or isolated distant metastases are also subject to surgical resection. That is why the main goal of radical surgery is  removal of all tumor tissue. Patients who underwent incomplete resection of ACC (less than total resection of the primary tumor or total resection of primary tumor in  leaving inoperable metastases) have an extremely poor prognosis with a median survival of less than one year. Mitotane therapy is an adequate method of  pharmacological influence on ACC, which is to be combined with surgery. Mitotane is also used as a symptomatic treatment for inoperable patients. Chemotherapy  may be used for the spreading process to influence remote metastases or for residual tumor with mitotane in combination with mitotane in ACC without hormonal  hypersecretion or in cases of mitotane resistance. Chemotherapy does not replace surgery nor mitotane therapy and may be recommended only in the presence of  individual indications. Use of chemotherapy after radical primary surgery with no signs of remote metastases is not appropriate. X-ray therapy has a limited curative  significance and may be used as a symptomatic means of influence on painful bone metastases. In inoperable cases we have used X-ray therapy in combination with other therapeutic methods to prolong patient’s survival and alleviate patient’s suffering. The recurrence is the main cause of ACC patients’ mortality. The most  common site of recurrence is regional lymph nodes. Systematic lymph node dissection is to be recommended as a mandatory surgical procedure after primary tumor  resection to patients with ACC. Combination of complete tumor resection (adrenalectomy) with lymphatic dissection — even in the absence of signs of regional lymph  node involvement — gives the best survival rate compared with traditional potentially radical surgery without prophylactic retroperitoneal dissection. In our opinion,  taking into account a similar frequency of complications, lymphatic dissection is to be recommended as a routine procedure to all patients with adrenocortical  carcinoma even in the absence of macroscopic signs of lymph node involvement.