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Medical Edge Newspaper Column from Mayo Clinic

TUMOR SURGERY LIKELY TO RELIEVE CHILD’S SUFFERING

DEAR MAYO CLINIC: I have a 1-year-old niece who has been in the hospital for 16 days in intensive care. Doctors suspect she has pheochromocytoma.

She has had extreme difficulty with hypertension. She has not gained weight in months. We suspect she has had hypertension her whole life. She has had flushing/sweating, inability to sleep, at times not napping for days and up all night. She has crying periods and is extremely inconsolable. She startles very easily. She has had two episodes of pulmonary hemorrhage. She has been tested for everything they can think of and this is the one blood test that has come back positive.

Can you please give me some perspective on what to do? I am searching for clues to help this baby who is experiencing a lot of pain. Thank you so much for your help. — Seattle

ANSWER: We understand your concern, and feel for the whole family as you all deal with your niece’s medical challenges.

Pheochromocytoma (fee-o-krom-o-si-TOM-a) is a tumor that produces hormones called catecholamines (kat-a-KOL-a-meens). These are chemicals such as adrenaline that influence blood pressure, how fast the body uses energy, and agitation. These tumors are usually located in the adrenal glands near the kidneys, but they can also hide in the nerve chains running along the sides of the backbone. Pheochromocytomas can occur in children and adults, but they are fairly unusual at this young age.

Diagnosis: The patient’s blood and urine tests show abnormally high levels of catecholamines and catecholamine breakdown products. Imaging by ultrasound, CT and MRI can usually identify the location of the tumor or tumors. Special scans such as the MIBG (metaiodobenzyl guanine) can help localize hidden pheochromocytomas that don’t show up on other imaging tests.

Certainly, other medical conditions can cause poor weight gain and high blood pressure. That’s why your niece’s doctors have been careful to look for lots of possible causes.

Treatment: Surgical removal of the tumor or tumors usually solves the problem. Surgery, however, must be done by a team that includes surgeons and anesthesiologists who are familiar with the medical management of pheochromocytoma in children.

The hormone shifts that occur with tumor removal can cause dangerous swings in blood pressure, and the doctors will want to provide good, intensive care for your niece around the time of the operation. And your niece’s doctors will want to be sure they don’t miss any tumors. Sometimes, there are multiple pheochromocytomas in the body.

If your niece does have a pheochromocytoma, other family members might consider testing as well. These tumors can run in families, sometimes in association with other hormone-related tumors. Early detection helps, since it is much better to remove the tumors before they begin to cause symptoms.

Phil Fischer, M.D., Pediatric and Adolescent Medicine
Aida Lteif, M.D., Pediatric Endocrinology and Metabolism
Mayo Clinic; Rochester, Minn.

Additional Resources:
Treatment of Pheochromocytoma
Appointments Information
More information on Pheochromocytoma

DEAR MAYO CLINIC: Is there a way to apply radiation to inoperable cancers that’s not so hard on the body? — Memphis, Tenn.

ANSWER: Yes, a new form of cancer-destroying radiation called intraoperative electron radiation is available to treat various forms of cancer. IOERT is the delivery of radiation at the time of an operation, as a supplement to a course of external radiation. This allows the surgeon to move healthy organs away from the radiation beam, while the radiation oncology team precisely targets an inoperable tumor.

This new technology allows doctors to maximize the dose of radiation to the tumor and minimize exposure of normal tissues — a great improvement from the days when the dose of external radiation needed to control the cancer would exceed the body’s ability to handle it.

The results: The use of IOERT — in conjunction with surgery, external beam therapy and/or chemotherapy — has been shown to improve the outcome of patients with various types of locally advanced or recurrent cancers.

Leonard Gunderson, M.D., Radiation Oncology
Mayo Clinic, Scottsdale, Ariz.

Additional Resources:
Intraoperative Electron Radiation

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Medical Edge from Mayo Clinic is an educational resource and doesn’t replace regular medical care. To e-mail a question, go to www.mayoclinic.org, or write: Medical Edge from Mayo Clinic, c/o TMS, 2225 Kenmore Ave., Suite 114, Buffalo, N.Y., 14207. For health information, visit www.mayoclinic.com.