Adrenal gland cancers
Adrenal gland cancers are rare malignancies that originate in the adrenal glands, which are located above the kidneys and produce essential hormones. There are several types of adrenal cancers, including adrenocortical cancer, pheochromocytoma, and neuroblastoma. Adrenocortical cancer arises from the adrenal cortex and is associated with hormone production that can lead to symptoms such as high blood pressure and weight gain. Pheochromocytoma originates in the adrenal medulla and can cause severe hypertension and anxiety. Neuroblastoma predominantly affects young children and may manifest as abdominal lumps or persistent pain.
Adrenal tumors are classified as functioning or nonfunctioning based on their hormone production capabilities. While the exact causes of adrenal cancers remain unknown, certain genetic syndromes and mutations have been linked to increased risk. Diagnosis often involves imaging tests and hormone level assessments, with treatment typically centered on surgical removal of the affected gland. The prognosis varies significantly depending on the tumor type and stage, with five-year survival rates ranging from approximately 50% for adrenocortical cancer to 91% for low-risk neuroblastoma. There are currently no known preventive measures for adrenal gland cancers.
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Adrenal gland cancers
ALSO KNOWN AS: Pheochromocytoma, adrenal cortical cancer, adrenocortical cancer, neuroblastoma
RELATED CONDITIONS: Cushing syndrome, Conn syndrome, adrenogenital syndrome
DEFINITION: The adrenal glands are hormone-producing endocrine glands located directly above the kidneys. The outer layer of each gland is called the adrenal cortex. Hormones produced in the adrenal cortex include cortisol (aids in regulating blood sugar level), aldosterone (aids in regulating fluid and electrolyte balance and blood pressure), and testosterone and estrogen (male and female sex hormones). A primary malignant tumor in the outer layer of the adrenal gland is called adrenocortical cancer. The inner area of each gland is called the adrenal medulla. Hormones produced in the medulla include epinephrine and norepinephrine, which help regulate the body’s response to physical and emotional stress. A primary malignant tumor that originates in the medulla is called a pheochromocytoma. Another form of cancer that originates in the medulla is neuroblastoma, which is typically found in infants and children younger than age ten.
Adrenal tumors are commonly classified by their effect on hormone production in the affected area. Adrenal tumors that increase hormone production are called functioning tumors; those that do not produce hormones are called nonfunctioning tumors.
Risk factors: Although specific risk factors for adrenal cancers have not been identified, they have been associated with genetically linked familial syndromes, such as Li-Fraumeni syndrome, Beckwith-Wiedemann syndrome, Carney complex, and multiple endocrine neoplasia, type 1 (MEN 1). Mutations in the TP53 or IGF2 genes are also associated with adrenal cancer.
Etiology and the disease process: The cause of adrenal cancer is unknown. Functioning tumors that secrete hormones produce changes in the body systems affected by those hormones. Nonfunctioning tumors typically grow large enough to produce pressure on other organs, causing discomfort or pain in the abdomen or flank.
Incidence: Primary adrenal gland cancers are rare, affecting only one or two persons per 1 million people. Approximately 60 to 80 percent of adrenal cancers are functional, secreting large amounts of adrenal hormones. Adrenocortical cancer is most common in men between the ages of forty and fifty; neuroblastoma most commonly affects children younger than age five; and pheochromocytoma is most common between ages thirty and forty.
Symptoms: A functioning adrenocortical cancer tumor typically produces excess amounts of cortisol and aldosterone, which can cause elevated blood pressure, weight gain, thirst, urinary frequency, facial swelling, cramping of the muscles in the arms and legs, and increased hair growth on the face, arms, and back. If the tumor produces sex hormones, men may develop a loss of sex drive, impotence, and enlarged breasts, whereas women may develop a deeper voice, menstrual irregularities, and increased facial hair, and children may experience early puberty. A neuroblastoma can cause an unusual abdominal lump or fullness, weight loss, extreme tiredness, persistent diarrhea, flushing, sweating, elevated blood pressure, and behavior indicating the presence of abdominal pain. A pheochromocytoma can cause elevated blood pressure that is resistant to treatment, excessive sweating, severe headaches, tremors, rapid pulse, palpitations, and extreme anxiety.
A nonfunctioning adrenal cancer may not produce symptoms until the cancer is advanced or has metastasized. Typical symptoms are caused by the tumor pressing on other abdominal organs, causing pain or an abnormal feeling of fullness. Tiredness, fever, and weight loss may also be present.
Screening and diagnosis: Diagnosis begins with a thorough patient and family medical history and physical examination. If adrenal cancer is suspected, the patient’s blood and urine are tested for elevated levels of adrenal hormones, and computed tomography (CT) scanning, magnetic resonance imaging (MRI), or positron emission tomography (PET) scans are performed to identify tumors. The metaiodobenzylguanidine (MIBG) scan, during which images are taken after the injection of small amounts of radioactive materials, is used to detect the presence and location of a pheochromocytoma. Staging of adrenal cancer depends on the specific type of tumor.
Treatment and therapy: Treatment for adrenal cancer usually includes surgery to remove the affected gland. If the cancer has spread, doctors may remove additional structures or develop alternative treatment plans for the additional cancer sites. Mitotane may be used in patients with advanced adrenocortical carcinoma. Mitotane blocks hormone production and destroys adrenal cancer cells and may be combined with cisplatin, doxorubicin, and etoposide.
Following surgery, neuroblastoma may be treated with chemotherapy using doxorubicin, cyclophosphamide, and etoposide.
Surgery to remove a pheochromocytoma is typically preceded by the administration of medication to block the secretion of adrenalin, which can cause very high blood pressure and serious surgical complications. MIBG radiotherapy may be used after surgery or to treat metastatic pheochromocytoma.
Prognosis, prevention, and outcomes: There is no known prevention for adrenal cancer. The prognosis for adrenal cancer depends on the type of tumor and the stage of the disease. The five-year survival rate after surgery for adrenocortical cancer is approximately 50 percent; for localized pheochromocytomas it is about 83 percent. The prognosis for neuroblastoma varies widely depending on the child’s risk grouping. The five-year survival rate ranges from about 91 percent for a low-risk child to about 50 percent for a high-risk child. Metastatic tumors of all types have a poor prognosis.
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