Adrenocortical cancer
Adrenocortical cancer, also known as adrenal cortex carcinoma, is a rare but aggressive type of cancer that originates in the adrenal cortex, the outer layer of the adrenal glands located above each kidney. This malignancy can be either functioning, producing excess steroid hormones such as cortisol and aldosterone, or nonfunctioning, where it does not produce significant levels of hormones. While it typically affects adults between the ages of thirty and forty, it can also occur in young children, particularly under six years old. Risk factors include specific hereditary conditions like Li-Fraumeni syndrome and Beckwith-Wiedemann syndrome.
Symptoms can vary based on hormone production; functioning tumors may lead to weight gain, hypertension, and menstrual changes, while nonfunctioning tumors often remain asymptomatic. Diagnosis usually involves laboratory tests and imaging studies to assess the tumor's size and spread. Early-stage adrenocortical cancer may be successfully treated with surgery, but late-stage diagnoses often require chemotherapy and radiation. The prognosis varies significantly, with an average five-year survival rate of about 50%, impacted by factors such as tumor resectability and cancer stage at diagnosis.
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Adrenocortical cancer
ALSO KNOWN AS: Adrenal cortex carcinoma, adrenal cancer
RELATED CONDITIONS:Cushing syndrome, Conn syndrome, virilization
DEFINITION: Adrenocortical cancer is an aggressive malignancy that occurs when a tumor develops in the tissue of the adrenal cortex, the outer layer of tissues that surround the adrenal glands and produce steroid hormones. Adrenal glands are two small, triangular glands located on top of each kidney.
Risk factors: Certain hereditary diseases, including Li-Fraumeni syndrome, Beckwith-Wiedemann syndrome, and Carney complex, are the main known risk factors.
Etiology and the disease process: The cause of these tumors is unknown. An adrenocortical cancer tumor can be nonfunctioning, meaning it may produce certain hormones but without significant differentiation, or functioning, meaning it overproduces certain hormones that the adrenal cortex normally produces. These hormones include cortisol, aldosterone, testosterone, and estrogen. Adrenocortical cancer commonly becomes malignant, spreading to the lung, liver, bone, and peritoneum (lining of the abdominal cavity).
Incidence: Adrenocortical cancer is relatively rare, affecting only 1 out of 1 million every year. Although it generally occurs in adults between the ages of thirty and forty years, children can develop this cancer, usually under the age of six.
Symptoms: Nonfunctioning adrenocortical cancer tumors usually do not have symptoms. Functioning adrenocortical cancer tumors can generate many symptoms, owing to their overproduction of steroid hormones. Depending on the hormone, symptoms can include weight gain, high blood pressure, muscle weakness, acne, and changes in menstrual cycles.
Screening and diagnosis: Laboratory testing can be used to confirm changes in steroid hormone production. Abdominal radiological examination, such as computed tomography scanning and magnetic resonance imaging, can be used to determine the exact location and extent of the tumor. Adrenocortical cancer tumors are staged on a scale of I to IV, according to the size of the tumor and the degree of invasion, both locally and to distant sites.
Treatment and therapy: When discovered early, adrenocortical cancer can be cured through surgical removal of the tumor. However, because most tumors are not diagnosed until they are advanced, other treatment strategies such as radiation therapy and chemotherapy must be used. Most chemotherapy regimens to treat adrenocortical cancer include the drug mitotane, which is toxic to adrenal cortex cells, in addition to other chemotherapeutic agents such as cisplatin, doxorubicin, and etoposide.
Prognosis, prevention, and outcomes: The prognosis for patients diagnosed with adrenocortical cancer is generally poor but dependent on several factors. These include the ability of the tumor to be surgically removed, the stage of the cancer at diagnosis, the general health of the patient, and whether the tumor is recurrent. The average five-year survival for adrenocortical cancer is estimated to be 50 percent. The localized 5-year survival rate, meaning the cancer is within the gland only, is 73 percent. The regional 5-year survival rate, meaning the cancer has only spread to nearby lymph nodes or structures, is 53 percent. The distant 5-year survival rate, meaning the cancer has spread to further structures or organs, is 38 percent. The median survival for stage IV metastatic adrenocortical cancer is reported to be less than twelve months.
Bibliography
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Stefanidis, Dimitrios, et al. Guidelines for the Minimally Invasive Treatment of Adrenal Pathology. Los Angeles: Society of American Gastrointestinal Endoscopic Surgeons, 2013. PDF file.
Thomas, Paul. Endocrine Gland Development and Disease. Burlington: Elsevier Science, 2013. Print.
Valeri, Andrea. Surgery of the Adrenal Gland. Milan: Springer, 2012. Print.