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The hidden face of adrenal cancer: Why is this cancer so mysterious?
Adrenocortical carcinoma (ACC) is an aggressive cancer that originates in the adrenal cortex, the tissue that normally produces steroid hormones. Although adrenal cancer is relatively rare among cancers overall, with an annual incidence of only 1 to 2 cases per million people worldwide, its cryptic nature and variable clinical manifestations make this disease full of mystery. According to the latest data, the five-year survival rate for adrenal cancer is about 50%. Most patients often have invaded nearby tissues or have distant metastases at the time of diagnosis, which complicates treatment and prognosis.
The most common manifestations in patients with adrenal cancer include Cushing's syndrome, Connell's syndrome, and male or female transgender manifestations.
Symptoms and signs
Symptoms of adrenal cancer vary by age. In children, the most common manifestation of functional tumors is degeneration, followed by Cushing's syndrome and precocious puberty. In adults, Cushing's syndrome alone is the most common, followed by combined Cushing's syndrome and degeneration.
When patients overproduce steroid hormones, it can lead to symptoms including weight gain, muscle wasting, thinning of the skin and high blood pressure.
In addition to hormonal syndromes, patients with non-functional adrenal cancer often present with unusual symptoms such as abdominal or flank pain, varicose veins, and renal vein thrombosis, and may even be discovered incidentally without symptoms. Because of these diverse and nonspecific symptoms, adrenal cancer is often misdiagnosed or delayed.
Pathophysiology
The main cause of adrenal cancer is currently unknown. However, some families, such as Li-Fraumeni syndrome, carry mutations in the TP53 gene and are at higher risk. Some genes, such as TP53, CTNNB1 and MEN1, are often mutated. In addition, amplification of the TERT gene and loss of CDKN2A are also commonly seen in adrenal cancer cases.
The evaluation of adrenal tumors relies mainly on pathological examination, in which macroscopic examination usually reveals features of tumor enlargement, hemorrhage, and necrosis.
Diagnosis
The diagnosis of adrenal cancer usually requires a combination of clinical symptoms, laboratory tests, and imaging studies. A battery of hormonal tests can confirm the presence of symptoms of Cushing's syndrome, degeneration, or Conn's syndrome, which involves measuring serum levels of cortisol, DHEA, and other hormones.
Imaging examinations, such as CT and MRI, can help determine the location of the tumor, differentiate between benign and malignant tumors, and evaluate the invasion of the tumor into surrounding tissues. Abnormal imaging findings, such as uneven appearance and peripheral enhancement, may indicate highly active and aggressive tumors.
Adrenal cancer may present with complex features on imaging and requires further confirmation by pathological examination.
Treatment methods
The only curative treatment for adrenal cancer is complete removal of the tumor. Surgery can be performed even when the tumor infiltrates large blood vessels. However, many patients are inoperable by the time of diagnosis. Research shows that minimally invasive surgery for adrenal tumors has advantages in recovery time over traditional surgery, but long-term results remain uncertain.
For patients who cannot undergo surgery, radiation therapy and radiofrequency ablation offer the possibility of symptom relief. Chemotherapy is mainly based on mitotane, which is toxic to adrenocortical cells and is used in conjunction with standard chemotherapy drugs. Clinical trials have shown that mitotane combined with surgery can improve patient survival rates.
Prognosis
The prognosis of adrenal cancer depends on many factors, of which patient age and tumor stage are the most important indicators. Although the typical five-year survival rate for adrenal cancer is about 50%, survival rates in early-stage cases can be significantly improved if complete removal is possible. However, high proliferative activity and venous invasion may indicate a poor prognosis.
After adrenal cancer is diagnosed, early intervention in treatment is crucial because the earlier surgery is performed, the better the patient's overall survival prognosis.
Adrenal cancer is a relatively rare and challenging disease among adrenal tumors. Due to its potential diversity and uncertainty, how can we improve the understanding and detection capabilities of this disease to promote early diagnosis and treatment? What are the possibilities?
