-- Prof. Hartmut P. H. Neumann, M.D., Albert-Ludwigs University, Freiburg, Germany, interviewing Prof. Günter Janetschek, Dept of Urology, University of Innsbruck, Austria

with Editorial Note from McClellan Walther, M.D., National Cancer Institute

In the summer of 1996, Prof. Georg Bartsch, Chairman of the Department of Urology of the University of Innsbruck, Austria, introduced me to Prof. Günter Janetschek, a urologist in his department at Innsbruck. Prof. Janetschek is one of the most experienced surgeons for laparoscopic surgery of the retroperitoneum, a minimally invasive surgery of the abdominal cavity.1 The following interview is intended to inform the VHL community about this new option for surgery for pheochromocytoma.2

Prof. Neumann: Could you give us a brief overview of the principles of laparoscopic surgery?

Prof. Janetschek: Laparoscopic surgery avoids the incision required for open surgery. Instead, a video endoscope3 is introduced into the abdomen through a 10 mm. (0.4 in.) incision, and in unilateral4 procedures the surgeon operates through 2-3 additional 10 mm. incisions. For the patient this minimally invasive technique has several advantages, namely minimal postoperative pain, few postoperative adhesions, minimized blood loss, low risk of surgical complications, short hospital stay, and early return to normal activities. Furthermore, several studies have shown that in experienced hands the complication rate of laparoscopy is also very low.

Prof. Neumann: Does laparoscopic surgery for pheochromocytoma require special skills and experience on the part of the surgeon?

Prof. Janetschek: Laparoscopic surgery on the adrenal gland is a highly specific procedure which requires not only mastery of laparoscopic surgery in general but also broad experience with open adrenalectomy. Surgery for pheochromocytoma carries the risk of a life-threatening elevation of blood pressure during the operation, which, however, can be prevented by gentle handling of the adrenal tissue and by tying off the adrenal vein early in the procedure. Therefore this type of surgery should only be performed by laparoscopic surgeons who already have extensive experience with paraoscopic adrenalectomy.

Prof. Neumann: How much time does it take for a pheochromocytoma to be removed laparoscopically? Are there special risks associated with long operating times?

Prof. Janetschek: The mean operating time in the nine unilateral adrenalectomies performed for pheochromocytomas at our department so far was 180 minutes [3 hours], which is within the range of open surgery. Of course, the operating time is substantially longer in partial adrenalectomy5 or when additional extra-adrenal6 pheos have to be removed as well. However, a study presented by Fornara at the German Urological Association in 1996 demonstrated that long operating times do not result in an increase in danger to the patient.

Prof. Neumann: Is organ-sparing surgery, that is, enucleation7 of the tumor instead of adrenalectomy, technically feasible by means of laparoscopy?

Prof. Janetschek: To my knowledge, we were the first and are still the only group performing laparoscopic adrenal-sparing surgery. The first laparoscopic partial adrenalectomy was done for an aldosterone-producing adenoma;8 the case report will soon be published in the Journal of Endocrinology. As a next step, we performed partial adrenalectomy for pheochromocytoma, which is technically more demanding than the corresponding procedure for Conn's adenoma,8 since pheochromocytomas commonly originate in the center of the adrenal gland. Nevertheless, all five procedures we have performed so far in three patients with bilateral disease could be completed successfully. Above all, patients with bilateral pheochromocytoma benefit a lot because they can be spared lifelong steroid replacement therapy.9 In this connection, adrenal-sparing surgery may also be of advantage in unilateral disease, since if the patient should later develop a large pheo on the other side, an enucleation might not be technically feasible on that second tumor.

Prof. Neumann: In Freiburg, Germany, we have developed a specific program for preoperative patient preparation including magnetic resonance imaging (MRI) in the frontal, coronal, and transverse planes,10 metaiodobenzylguanidine scintigraphy (MIBG), and computed tomography (CT) scanning. In addition, alpha- and beta-blockers11 are given followed by 24-hour blood pressure monitoring. Is this essential for the procedure, and could you discuss it in more detail?

Prof. Janetschek: Careful preoperative work-up including all imaging techniques you just mentioned is mandatory, since pheochromocytomas do not arise exclusively from the adrenals but also from the so-called Zuckerkandl bodies,12 which includes all tissue of sympathetic origin. It is essential to identify and evaluate not only adrenal but also all extra-adrenal pheochromocyomas, since they have to be removed as well. All four patients treated for familial pheochromocytoma (MEN2 and VHL)13 at our department had extra-adrenal pheos.

Prof. Neumann: Are there surgeons in America, England, France and Japan who have extensive experience with laparoscopic retroperitoneal surgery, particularly on the adrenal glands?

Prof. Janetschek: Laparoscopic adrenalectomy was first described by Gagner, a general surgeon from Canada, in 1992. Reports on large series including up to 50 patients were published by urologists from Japan. Guazzoni, an Italian urologic surgeon, was the first to show that laparoscopy is superior to open surgery in terms of reducing the complication rate. As already mentioned, laparoscopic adrenal-sparing surgery for pheochromocytoma and laparoscopic excision of extra-adrenal pheos are new therapeutic concepts which we have recently introduced at Innsbruck University Hospital.

Notes:

1. (REH-tro-pear-ih-to-NEE-um) The retroperitoneum is a space inside the abdominal cavity, and in front of the spinal column and the lumbar muscles, which contains the kidneys and adrenal glands. [Return to text]

2. (FEE-oh-kro-mo-sigh-TOE-mah) A pheochromocytoma is a tumor of the adrenal gland which can occur in VHL. For more information, please see the VHL Handbook, available from the VHL Family Alliance. Pheochromocytoma is often abbreviated to "pheo". [Return to text]

3. (EN-doe-skope) A video endoscope is a tiny video camera mounted on a very thin tube, which displays closed-circuit television pictures on a monitor in the operating room. This camera is inserted through the incision so that the surgeon can see and guide the surgical tools beneath the skin. [Return to text]

4. In a unilateral [one sided] operation, surgery is performed on only one adrenal gland. In a bilateral [two sides] operation, there is surgery on both adrenal glands in one sitting. [Return to text]

5. (a-DREE-nall-EK-toe-mee) An adrenalectomy is an operation to remove an adrenal gland. In a partial adrenalectomy, only part of the adrenal gland is removed. [Return to text]

6. (EKS-trah a-DREE-nall) Extra-adrenal pheos are pheochromocytomas which occur outside of [in Latin, extra] the adrenal glands. See also note 12 below. [Return to text]

7. (ee-NU-klee-AY-shun) Enucleation is the removal of the tumor from the surrounding tissue, like removing the yolk from a boiled egg. In this case, removing the tumor while leaving as much of the remaining adrenal gland in place as possible. [Return to text]

8. Conn's adenoma, an aldosterone-producing adenoma, is a benign type of tumor of the adrenal gland that does not occur in people with VHL, and that produces a different hormone than a pheo, but presents similar challenges to the patient and the surgeon. [Return to text]

9. If a person has no adrenal glands, then it is necessary to take by mouth the hormones which would have been produced by the adrenal glands. If enough adrenal tissue can be left in place, this replacement therapy can be avoided. Depending on the size of the tumor, this may not be possible, no matter what the surgical procedure. [Return to text]

10. MRI pictures showing views of the tumor, as if the camera were mounted in front of the patient, looking toward his stomach [frontal]; a view that looks like they sliced the tumor in half from top to bottom and took a view of the cut surface [coronal]; and another view that looks like they sliced the tumor in half crosswise and took a picture of the cut surface [transverse]. [Return to text]

11. In addition to the body's natural and appropriate stress reaction, the pheo is generating adrenaline, causing the blood pressure to rise. These drugs are given to suppress the natural generation of stress hormones which would tend to occur under the anxiety and stress of the operation itself, to keep the blood pressure from rising to a dangerous level during surgery. After the drugs have been administered the test ensures that the drugs are doing their job. [Return to text]

12. Zuckerkandl bodies, nodes of the sympathetic nervous system lying along the aorta. Pheos grow most commonly inside the adrenal glands, but may also grow in a number of other places, roughly along a line from the groin to the collar-bone on each side, but very rarely in the chest. [Return to note 6] [Return to text]

13. There are two conditions where pheos are caused by an inherited genetic alteration: VHL and multi-endocrine neoplasia, type 2 (MEN2). With better imaging studies and the MIBG test, pheos can now be found which have previously gone undetected. [Return to text]

Editorial note: Nine reviews of cases of laparoscopic adrenalectomy for pheochromocytoma have been published since 1994, and our own paper will appear shortly in the journal Urology. A number of surgeons are working with this procedure, but the incidence of adrenal tumors is low, so expertise with this technique is still not widespread. Patients interested in finding a qualified surgeon should consult first with their local doctors, or with a large teaching hospital in their local area. At the National Institutes of Health we have performed 26 such surgeries on tumors of the adrenal gland. The only partial adrenalectomies we have done laparoscopically have been of quite small tumors, and we do not usually remove small inactive pheochromocytomas. Therefore partial adrenalectomies for larger pheos are more commonly done with open surgery. -- Dr. McClellan Walther, Urologic Surgery, National Cancer Institute. [Return to top]

As published in the VHL Family Forum 5:1, March 1997. For permission to reprint, please contact VHL Family Alliance, editor@vhl.org. Further information is available from the VHL Family Alliance, info@vhl.org.