Adrenocortical carcinoma (ACC) is an aggressive cancer originating from the adrenal cortex, which is primarily responsible for the production of steroid hormones. This cancer presents different clinical features in different age groups, especially between children and adults. Studies have pointed out that the incidence is higher in children younger than five years old and in adults aged 30 to 40 years, but the overall incidence of this disease is still very low, with an annual incidence rate of one to two cases per million people.
Adrenocortical cancer is closely associated with various hormonal syndromes, including Cushing's disease, Conn syndrome, and possible male or female symptoms.
In children, most adrenal cancers are functional tumors, and the most common symptom is virilization, followed by Cushing's disease and precocious puberty. The presence of functional tumors leads to abnormal secretion of hormones in the body, which drives the onset of these symptoms. For example, symptoms of virilization may include excessive facial and body hair, skin addiction, and enlargement of the genitals. These symptoms are particularly pronounced in boys, and doctors will be highly alert to these conditions.
Compared with children, Cushing's disease is the most common symptom of adrenal cancer in adults, with symptoms including weight gain, muscle atrophy, purple streaks on the abdomen, and moony face. The second is virilization symptoms, which are symptoms of excessive male hormones that affect female patients. For male patients, feminizing symptoms such as breast enlargement and loss of libido may occur. Although Conn syndrome (excess mineralocorticoids) is relatively rare in adults, the occurrence of symptoms of high blood pressure and hypokalemia still requires the attention of doctors.
All patients with suspected adrenocortical cancer should be thoroughly evaluated for symptoms of associated hormonal syndromes.
For the diagnosis of adrenocortical cancer, hormonal abnormalities should first be confirmed through laboratory tests. For example, laboratory findings of Cushing's disease include increased blood sugar and urinary cortisol, and Virilism is diagnosed by excess serum androstenedione and dehydroepiandrosterone. Imaging studies are also critical, often with a CT scan or MRI scan to determine the location of the tumor and assess its extent of invasion into surrounding tissue.
If surgery is performed, the diagnosis of adrenocortical cancer is usually confirmed during pathological examination. These tumors generally appear larger in appearance, with a yellowish color on the surface of the mass, accompanied by bleeding and necrosis. Microscopic examination shows that tumor cells often exhibit atypia and may invade surrounding tissue.
Currently, the only treatment that may offer a chance of cure is complete tumor resection. In cases where the tumor has invaded large blood vessels, surgery is still a viable option. Despite this, many patients are unable to undergo surgery due to health conditions that do not qualify for surgery. If surgery is not possible, radiation therapy or other palliative treatments may be an option. For chemotherapy, a regimen that includes Mitotane, a drug that inhibits steroid synthesis, is often chosen.
The prognosis for adrenocortical cancer usually depends on the age of the patient and the stage of the tumor. But even so, the overall five-year survival rate is about 50%, and the five-year disease-free survival rate for patients with complete resection is about 30%.
Although adrenocortical cancer is a relatively rare but aggressive cancer, symptoms and progression of the disease vary across age groups. Is it possible for us to improve the efficiency of diagnosis and treatment to better meet the challenges of this disease?