Adrenocortical carcinoma

Adrenocortical carcinoma (ACC) is a rare and highly invasive malignant endocrine tumor. 1 ~ 4 years old and 40 ~ 50 years old are more common, and the prevalence rate of women is higher.

The prognosis of the disease is poor, and the 5-year overall survival rate is only 15% ~ 44%. Tumor stage is the key prognostic factor of acc. the expected 5-year survival rate of stage I patients is 80%, while that of stage IV patients is 13%. About 2 / 3 of patients with local diseases relapse and need comprehensive treatment based on systemic chemotherapy.

Insulin like growth factor-2 (IGF-2) overexpression and Wnt are the most common in patients with acc/ β- The catenin pathway is continuously activated. IGF-2 overexpression is associated with allele replication after modification of epigenetic imprinting on 11p15. β- The activation of catenin is related to the decline of overall survival rate in patients with acc. Steroid factor (SF1) can promote the proliferation of adrenocortical cells, which is related to the poor prognosis of ACC patients.

Patients with ACC involve the globular zone, fascicular zone and reticular zone of the adrenal cortex, and the larger the tumor will also squeeze the adrenal medulla. The main clinical manifestations are obesity (91.4%), Cushings syndrome (87.1%), fatigue (81.7%), abnormal menstruation in women (84.3%), purple lines on the skin of lower abdomen and thigh root (69.9%), and osteoporosis (57%).

Patients with suspected ACC should be carefully evaluated, including medical history, clinical signs and signs of autonomic excess of adrenocortical hormone. In addition, genetic testing and imaging evaluation are required.

Complete surgical resection is one of the methods to cure acc. For potentially resectable stage I ~ III tumors, complete surgical resection was taken as the initial treatment according to the ensat staging standard. For T1 stage tumors, laparoscopic resection can be considered; Open surgery is recommended for stage T2 and above.

The use of mitotan adjuvant therapy is based on three main prognostic factors: tumor stage, integrity of resection and proliferation rate.

The most important clinical factors determining the prognosis of ACC are disease stage and the complete degree of resection. In addition to the stage and the complete degree of resection, pathological / morphological factors also affect the biological behavior of ACC. The histological manifestations of ACC can range from mild atypia to highly anaplastic tumors composed of deformed giant cells. At present, the widely used Weiss multifactor scoring system is based on 9 histopathological features (nuclear grade, mitotic rate, atypical mitosis, clear cell composition, diffuse structure, tumor necrosis, venous structure, sinus structure or tumor envelope invasion). Tumors that meet only 2 or less criteria are usually considered benign. These criteria were confirmed as prognostic factors. 

 

Cindy