Experience with surgical treatment for primary malignant adrenal tumors

Abstract

Background. Adrenal tumors occur in 3–10 % of the population and are mostly benign adrenal cortical tumors. Adrenocortical carcinoma is a very rare tumor and has an annual incidence of 1–2 cases per million people. The U.S. National Cancer Data Base registered 4275 patients with adrenocortical carcinoma in 1985 to 2007. It is extremely difficult to assess Russia’s epidemiological data, as reports on adrenocortical carcinoma are not presented separately. Materials and methods. A total of 133 patients (49 men and 84 women (1:1.7)) with adrenal tumors were operated on at the clinics of the Siberian State Medical University in the period December 1998 to March 2015. The patients’ mean age was 51.3 (16–80) years (median age 51.0 years). The right and left adrenal glands were affected in 49 (36.9 %) and 77 (57.9 %) patients, respectively; both adrenal glands were involved in 7 (5.3 %). A group of 21 (15.8 %) people with primary malignant adrenal tumors was identified among all the patients. The clinical manifestations of the disease were evaluated from the presence of hormonal activity, gastrointestinal symptoms, pain syndrome, and hypertension. All the patients were operated on under endotracheal anesthesia. The data were statistically processed using the program package Statistica 6.0. Survival rates were analyzed by the Kaplan–Meier method. The Gehan–Wilcoxon test was used to compare the groups. Results. The investigation analyzed treatment results in 21 (15.8 %) patients with primary malignant adrenal lesions (Group 1). The most common morphological form was adrenocortical carcinoma in 15 (11.3 %) patients (5 men and 10 women (1:2)); their mean age was 48.1 years. The right, left, and both adrenal glands were affected in 4, 9, and 2 cases, respectively. In Group 2, other malignant adrenal involvements were identified from 1 case of rare malignant adrenal tumors: malignant pheochromocytoma, sarcoma, melanoma, squamous cell carcinoma, Castleman’s disease, and oncocytic carcinoma. According to the tumor stage, the patients with adrenocortical carcinoma were divided as follows: Stage T1 (n = 3), Stage T2 (n = 3), Stage T3 (n = 4), and Stage T4 (n = 5). In the patients with adrenocortical carcinoma, the size of an adrenal tumor was 8.7 ± 4.9 cm; hypertension as a main clinical manifestation occurred in 5 patients; pain syndrome was observed in 10; hormonal activity was noted in 8, blood electrolyte disorders were seen in 3, and gastrointestinal manifestations were in 9 patients. In Group 1, 8 (38.1 %) patients were survivors and 13 (61.9 %) patients died. Moreover, the overall 5-year survival was 37.9 %. Five-year survival in patients with adrenocortical carcinoma was 42.4 % (6 (53.3 %) survivors and 9 (46.7 %)) dead persons); that in patients with other malignant adrenal tumors was 33.3 % (2 (33.3 %) survivors and 4 (66.7 %) dead persons). Conclusion . Surgical removal of an adrenocortical tumor is the only treatment option that can cure a patient or considerably prolong life particularly if the disease is detected at stage I or II.