Pheochromocytoma: what kind of adrenal tumor is it

Hearing "adrenal tumor" from a doctor leaves you with a dozen questions. Pheochromocytoma is one such tumor: rare, hormone-producing, and far from always malignant. Below is a calm breakdown: what this mass is, how it manifests, which steps detect it, and when you must not delay.

What pheochromocytoma is

Pheochromocytoma is a rare tumor of adrenal tissue that releases too much adrenaline and noradrenaline. These hormones control heart rate, metabolism, and blood pressure, so their excess causes palpitations and spikes in blood pressure. By definition, it is a hormone-producing tumor of chromaffin cells. It mainly affects adults, more often in early and middle adulthood; the tumor also occurs in children.

Adrenal tumor: where and how it develops

The adrenal glands are two triangular glands, one above each kidney. Pheochromocytoma usually grows in the center of the gland, in the medulla, of one or both adrenal glands. In rare cases it arises outside the adrenal gland — as a rule, somewhere else in the abdomen. As the tumor grows, attacks become more frequent, longer, and more severe.

Is pheochromocytoma cancer or not

Most pheochromocytomas do not spread to other parts of the body, but about one in ten gives metastases — such tumors are called metastatic. Very few pheochromocytomas are cancerous. That is, the diagnosis of pheochromocytoma itself does not equal a diagnosis of "cancer," and the question of malignancy is decided by examination results, not by the name. The malignant variant — pheochromoblastoma — is rare, and it can cause weight loss and abdominal pain.

In many people the cause remains unclear, and the tumor does not recur in the family. Up to one case in three is associated with hereditary syndromes — von Hippel-Lindau and neurofibromatosis type 1. After diagnosis, the doctor may offer genetic testing; testing can also be useful for close relatives.

What signs and symptoms occur

Symptoms are unpredictable and occur in attacks — from a few minutes to an hour, sometimes longer. An attack can be triggered by emotional stress, physical exertion, palpation of the tumor, or a sudden change in body position. Most often during an attack the following appear:

• headache;
• heavy sweating;
• palpitations, a feeling of irregular heartbeat;
• increase in blood pressure;
• facial pallor, tremor, nausea and vomiting, a feeling of anxiety or panic.

Other manifestations also occur: abdominal or chest pain, irritability, weight loss, shortness of breath, sleep disturbances, seizures; an attack may end with profuse urination. Some people have no symptoms at all — the tumor is found incidentally, during examination for another reason, for example because of high blood pressure. In others, symptoms persist for years before diagnosis.

How pheochromocytoma is diagnosed

Recognizing it is not easy: the signs are general and occur in many more common conditions. First the doctor asks questions and examines the patient. Then come laboratory tests — catecholamines and metanephrines in blood and urine, glucose level. Next is imaging: CT or MRI of the abdomen, iodine-123 scintigraphy (MIBG), PET; if necessary, an adrenal biopsy is performed. At the appointment it makes sense to clarify exactly which tests have been ordered and why, whether genetic testing is needed, and what each examination will show.

Risks, complications, and when to see a doctor

Without treatment, the tumor seriously worsens the condition: cardiac arrhythmias, heart attack, stroke, and organ failure are possible. An attack may be complicated by retinal hemorrhage, cerebrovascular accident, or pulmonary edema. Most tumors are successfully removed surgically, and then symptoms usually resolve. In a small number of people the tumor returns, so regular check-ups are needed after surgery. See a doctor if headache, sweating, and palpitations appear, and also if you had a tumor in the past and symptoms have returned.

Pheochromocytoma by itself is not a reason to call an ambulance. Call 103 or 112 if during an attack your blood pressure has risen sharply and severe headache, chest pain, irregular heartbeat, marked shortness of breath, seizures, sudden weakness, or speech or movement impairment have appeared.

Support and an explanation of each next step can be obtained from your treating doctor — an endocrinologist or oncologist — and it is worth coming to the appointment with written questions. This material is for reference and does not replace a doctor's consultation.

This content is for reference and does not replace a doctor's consultation. Medical disclaimer