Adrenal Cancer (Adrenocortical Carcinoma)
Rare CancerCategory: 内分泌ICD-10: C74
Overview
Adrenocortical carcinoma (ACC) is an extremely rare malignancy of the adrenal cortex, with an annual incidence of about 1-2 per million. It most commonly occurs in the 40-50 age group. Approximately 60% of tumors are hormone-producing.
Symptoms
Hormone-producing tumors may cause Cushing syndrome (moon face, central obesity), virilization, or feminization. Non-functional tumors present with abdominal mass, pain, or incidental discovery on imaging.
Diagnosis
CT or MRI characterizes the adrenal mass size and features. Hormonal evaluation (cortisol, DHEA-S, aldosterone) determines functionality. Adrenal masses larger than 4 cm warrant surgical consideration.
Treatments
Complete surgical resection is the only curative treatment, performed via laparoscopic or open approach. Adjuvant mitotane therapy is used for high-risk resected cases. Unresectable disease is treated with EDP-M (etoposide, doxorubicin, cisplatin plus mitotane).
Latest Research & Approaches
Immune checkpoint inhibitors are being evaluated in clinical trials for adrenocortical carcinoma. Genomic profiling-based molecular classification and personalized treatment approaches are under active investigation.
Sources & References
NCI - Adrenocortical Carcinoma Treatment
American Cancer Society - Adrenal Cancer
NCCN Guidelines: Neuroendocrine and Adrenal Tumors
American Cancer Society - Adrenal Cancer
NCCN Guidelines: Neuroendocrine and Adrenal Tumors