adrenal cancer

Cancer of the adrenal gland is an uncommon cancer. The cancer may arise from the cortex (outer portion) of the adrenal gland or from the adrenal medulla (the inner portion of the adrenal gland).

Adrenal cancers may secrete hormones and present with a Cushing’s syndrome or a pheochromocytoma.

Adrenal Cortical cancer

Adrenal cortical cancer arises from the cortex of the adrenal gland. This tumor can occur in children and in adults. It occurs in children younger than 6 years and has a higher incidence in girls than in boys. In children, virilisation (development of male sexual characteristics in female children) is a common presenting feature. Approximately 65% of the children with adrenal cancer can be cured by complete removal of the tumor. A pediatric surgical oncologist should evaluate children with adrenal cancer.

In the adult the tumor commonly presents between the ages of 40 and 50 years. Up to 70% of patients with adrenal cortical cancer present with excess secretion of adrenal hormones and commonly present with a Cushing’s syndrome. These tumors can often grow to a very large size before it is diagnosed. It has been estimated that more than 70% of patients with adrenal cortical cancer present with stage 3 or stage 4 (advanced stages) disease. The majority of these tumors secrete steroid hormones and patients present with signs and symptoms of a Cushing’s syndrome.

Treatment of adrenocortical cancer:

All patients with adrenocortical cancer should have a CT scan of the chest and a very detailed CT scan of the abdomen to evaluate whether surrounding structures that are found close to the adrenal gland such as the liver, large and important blood vessels such as the inferior vena cava, kidney and diaphragm are involved by the tumor. Many patients require removal of the kidney on the side of the cancer and therefore a careful evaluation of the opposite kidney should be performed to ensure adequate kidney function after the surgery.

Aggressive surgical removal of the cancer is the preferred treatment for adrenocortical cancer. This operation should be performed by an experienced surgeon in adrenal surgery. Since the cancer can reach a very large size, attachment to surrounding organs by the cancer often occurs. For best surgical results any organ that is attached or invaded by the cancer such as the diaphragm, part of the liver, and the kidney that are associated with the right adrenal gland and diaphragm, spleen and pancreas and kidney that are assoxciated with the left adrenal gland should be removed with the cancer.

Complete removal of small cancers can be curative and even for larger stage three cancers, prolonged survival can be accomplished by complete radical surgical removal of the tumor. In some patients where the complete cancer cannot be removed due to the extent of invasion of surrounding organs, tumor debulking where as much as of the tumor as possible is removed may allow improvement of symptoms from steroid hormone oversecretion from the tumor and the pain from the tumor.

Life long monitoring is necessary after removal of adrenal cancer. If a patient develops a recurrence of the adrenal cancer then complete removal of the recurrent tumor is associated with an improved survival compared to patients in whom the recurrences are not removed.

In highly selected patients, metastases of the tumor outside of the adrenal such as in the liver, lung, brain may provide improvements in survival. Since chemotherapy options are limited for patients with adrenal cancer aggressive surgical treatment if possible is the best option for prolonged survival.

Cancer of the Adrenal Medulla

The adrenal medulla secretes catecholamines. Tumors of the adrenal medulla produce excessive amounts of catecholamines and present with flushing, high blood pressure and palpitations. This is called a pheochromocytoma. 80% of pheochromocytomas are benign are and 20% of these tumors are malignant. The distinction between benign and malignant pheochromocytoma can be difficult even after removal of the tumor and a close follow up of the patient after the surgery is necessary.

The exact incidence of cancer in pheochromocytoma is controversial. While old studies suggested that only 10% of pheochromocytomas were cancerous, more recent information suggest that up to 30% to 50% of these tumors can be cancerous. Close long term surveillance after removal of pheochromocytomas is therefore necessary to detect any recurrences from cancerous pheochromocytomas early.

Two tests are available for detection of recurrences from a previously removed pheochromocytoma:

  • MIBG scan: in this study a substance utilized in the synthesis of catecholamines by the adrenal medulla is given to the patient. The adrenal tumor will selectively concentrate the substance. The substance is labeled with a radioactive label and therefore the hotspot created by the tumor is detected on nuclear medicine imaging.
  • Octreotide scan.
It is recommended that these studies are done at least once every two years and preferably on a yearly basis. Metastatic disease may be widespread and may be found in the abdomen, lungs, bones or brain.

Treatment of recurrent pheochromocytoma

If the recurrent tumor is localized and surgically removable then aggressive surgical removal of the tumor is a treatment of choice.

Treatment of metastatic pheochromocytoma

treatment options are limited in patients in whom the tumor is found to be unresectable (surgically unremovable) or who has widespread metastatic disease. Chemotherapy has not found to be useful in this group of patients.

If the MIBG scan shows that the tumor takes up the substance MIBG, then this property of the tumor has been utilized to treat it with MIBG compound labeled with radioactive iodine labeled with the MIBG substance. The tumor takes up the radioactive iodine labeled MIBG and the tumor is then destroyed by the high dose of local radiation in the tumor.

This targeted radiotherapy of the tumor with radioactive iodine labeled MIBG is presently the treatment of choice for metastatic pheochromocytoma. MIBG treatment may sometimes be combined with chemotherapy.

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