Adrenocortical carcinoma (ACC) is an aggressive cancer that begins in the adrenal cortex (the tissue that produces steroid hormones). ACC is known for its ability to produce a variety of endocrine symptoms, such as Cushing's syndrome, Conner's syndrome, virilization and feminization, which are mostly seen in patients with functional tumors. ACC usually has already invaded nearby tissues or metastasized to distant organs at the time of diagnosis, and its five-year overall survival rate is only about 50%. Although this tumor is relatively rare, with an annual incidence of only one to two per million people, its diagnosis is still very important because early detection will greatly improve the effectiveness of treatment.
The annual incidence of adrenal cancer is relatively low, but it is often advanced at the time of diagnosis.
The presentation of adrenal cancer varies with age. In children, most tumors are functional and the most common symptom is virilization, followed by Cushing's syndrome and precocious puberty. In adults, Cushing's syndrome is the most common presenting symptom, followed by concurrent Cushing's disease and virilization. In rare cases, such as Conn's syndrome, high blood pressure and low blood potassium levels are the main symptoms, often accompanied by headaches and muscle weakness. In addition, feminization symptoms are more pronounced in men and include breast enlargement, decreased libido, and erectile dysfunction.
PathophysiologyThe primary cause of adrenal cancer is unknown, however families with Li-Fraumeni syndrome (caused by inherited inactivating mutations in the TP53 gene) are at increased risk. Many genes were found to have possible repeated mutations, including TP53, CTNNB1, MEN1, etc. Tumor-related genes are expressed abnormally, causing a series of growth and development problems. These genes play an important role in the development of adrenal cancer and have indicator value for early detection and diagnosis.
Diagnosing the endocrine symptoms of adrenal cancer requires appropriate laboratory tests. The hallmark manifestations of Cushing's disease are elevated serum glucose and urinary cortisol, while adrenal virilization is evidenced by excess serum androstenedione and dehydroepiandrosterone. Laboratory tests of Cushing's disease reveal low serum potassium, low antidiuretic activity, and high aldosterone levels.
Imaging examinations such as CT and MRI play a key role in localizing adrenal tumors, differentiating adenomas, and evaluating the extent of tumor invasion. CT images typically show a heterogeneous appearance with peripheral enhancement after contrast injection. MRI images show low signal with strong heterogeneous contrast enhancement.
Targeted imaging examinations of adrenal cancer can effectively confirm the presence and characteristics of the tumor.
Adrenal tumors are usually diagnosed through pathological examination after surgery. Surgical specimens typically show large, yellowish tumors with hemorrhage and necrosis. Examination under a microscope can reveal abnormal proliferation of tumor cells, which is the key to determining whether the tumor is cancer.
Currently the only cure for adrenal cancer is complete removal of the tumor. Still, not all patients are candidates for surgery. In addition to surgery, radiotherapy and radiofrequency ablation can also be used for patients who are not suitable for surgery. Chemotherapy regimens often include the use of drugs that inhibit steroid production, such as mitotane, which improves survival in some patients.
The five-year survival rate after successful surgery is approximately 50-60%, while the five-year disease-free survival rate for patients with resected stage I-III cancer is approximately 30%.
Overall, the prognosis of adrenal cancer remains challenging. Factors that affect prognosis include patient age and tumor stage. In addition, tumor density, invasiveness and other biomarkers are also important parameters for prognostic assessment. According to research, younger patients and those whose tumors are discovered early have higher survival rates, which also highlights the importance of early diagnosis.
Although adrenal cancer is rare, it is important to understand its signs, diagnosis, and treatment. People should think about whether there are more effective ways to ensure that this type of cancer can be identified and treated in the early stages to improve patients' survival rate and quality of life?