Ask the Experts:

Question: “I was recently told that I would probably not be able to have a partial adrenalectomy for my pheo - even though it’s very small. The doctor said that the entire adrenal is overproducing and a partial adrenalectomy would only result in future pheos on the same side.” -- Angela S., Florida

Answer: We used to hear this in the early 90’s...when we first started doing partial adrenalectomies.

Our rationale is that we believe strongly in organ sparing management in VHL patients, whenever possible. When it comes to the adrenal, our goal in developing the minimally invasive, adrenal sparing partial adrenalectomies is because pheochromocytoma in VHL patients can be bilateral. We always work to preserve the adrenals in these individuals, whenever possible. We do not like to see both adrenals removed from a VHL patient (or any patient) whenever possible.

We know it is true that a pheochromocytoma can recur. However, in most instances, we feel that it is better to leave the functioning adrenal tissue. These patients are watched closely in any event, as pheos can occur even without adrenals. So, if a pheochromocytoma does re-occur (on either side), it can be managed (most often by minimally invasive laparoscopic removal).

At the NCI we rarely remove an entire adrenal gland in a VHL patient. When we do, the tumor is very, very large.

-- W. Marston Linehan, M.D., Chief, Urologic Oncology Branch, U.S. National Cancer Institute, Bethesda, Maryland

Diner,E.K., Franks,M.E., Behari,A., Linehan,W.M. and Walther,M.M. Partial adrenalectomy: the National Cancer Institute experience, Urol., 66: 19-23, 2005. PMID: 15961144

As printed in the VHL Family Forum 14:2, August/September 2006. For permission to reprint, please contact VHL Family Alliance, editor@vhl.org. Further information is available from the VHL Family Alliance, info@vhl.org