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Functional and Oncologic Outcomes of Partial Adrenalectomy for Pheochromocytoma in Patients With VHL Syndrome
TAKE-HOME MESSAGE
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In this study, the authors reviewed their single-institution functional outcomes and recurrence rates after partial adrenalectomy in patients with VHL. A total of 36 partial adrenalectomies for pheochromocytoma were performed in 26 patients (23 open and 13 laparoscopic). After a median follow-up of 9.25 years (range, 5–46), no one developed metastatic disease. There were 5 local recurrences in 3 patients (11%). All recurrences were asymptomatic and detected by radiographic imaging on follow-up. In addition, 3 of 26 patients (11%) subsequently required partial adrenalectomy for pheochromocytoma on the contralateral adrenal gland. Only 3 patients (11%) became steroid-dependent.
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Partial adrenalectomy for pheochromocytoma in patients with VHL is feasible and recommended, which may obviate need for steroid replacement in this patient population.
– Juan Chipollini, MD
abstract
This abstract is available on the publisher's site.
Access this abstract nowPURPOSE
Although the safety and feasibility of partial adrenalectomy in patients with von Hippel-Lindau syndrome have been established, long-term outcomes have not been examined. In this study we evaluate the recurrence and functional outcomes in a von Hippel-Lindau syndrome cohort treated for pheochromocytoma with partial adrenalectomy with a followup of at least 5 years.
MATERIALS AND METHODS
We reviewed the records of patients with von Hippel-Lindau syndrome treated with partial adrenalectomy for pheochromocytoma at the National Cancer Institute. Demographic, germline mutation status, surgical indication, oncologic and functional outcome data were collected. Local recurrence was defined as radiographic evidence of recurrent tumor on the ipsilateral side of partial adrenalectomy. Patients were considered steroid dependent if they required steroids at most recent followup.
RESULTS
A total of 36 partial adrenalectomies for pheochromocytoma were performed in 26 patients with von Hippel-Lindau syndrome between September 1995 and December 2003. Of these cases 23 were performed open and 13 were performed laparoscopically. Prior surgical history was obtained for all patients. At a median followup of 9.25 years (range 5 to 46) metastatic pheochromocytoma had not developed in any patients. In 3 patients (11%) there were 5 local recurrences treated with surgical extirpation or active surveillance. All recurrences were asymptomatic and detected by radiographic imaging on followup. In addition, 3 of 26 patients (11%) subsequently required partial adrenalectomy for pheochromocytoma on the contralateral adrenal gland. In the entire cohort only 3 patients became steroid dependent (11%).
CONCLUSIONS
Outcomes of partial adrenalectomy in patients with von Hippel-Lindau syndrome with pheochromocytoma are encouraging at long-term followup and should be recommended as a primary surgical approach whenever possible. Adrenal sparing surgery can obviate the need for steroid replacement in the majority of patients. Local recurrence rates appear to be infrequent and can be managed successfully with subsequent observation or intervention.
Additional Info
Disclosure statements are available on the authors' profiles:
Functional and Oncologic Outcomes of Partial Adrenalectomy for Pheochromocytoma in Patients With von Hippel-Lindau Syndrome After at Least 5 Years of Followup
J Urol 2010 Nov 01;184(5)1855-1859, JN Benhammou, RS Boris, K Pacak, PA Pinto, WM Linehan, G BratslavskyFrom MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.
Benhammou and colleagues report on outcomes of partial adrenalectomy for pheochromocytoma in patients with VHL disease having had at least 5 years of follow-up. Of 36 partial adrenalectomies in 26 patients with a median follow-up of 9.25 years, 11% (3 patients with 5 recurrences) had a local recurrence, and 11% (3 patients) had a contralateral recurrence. All recurrences were asymptomatic and detected by radiographic surveillance. Only 3 patients (11%) became steroid-dependent. No patient developed metastatic disease. This study provides one of the largest experiences and longest follow-up for partial adrenalectomy for pheochromocytoma, assuring an increasingly important role for partial adrenalectomy in the management of VHL-related pheochromocytoma.
Several issues require attention, however. If metabolic surveillance such as with serum fractionated metanephrines were done, they were not reported in this study, and it does not appear that these were part of the surveillance strategy. It is possible that results of these may have altered results. Additionally, patients who were subclinically Addisonian, and who may have atypical and nonspecific symptoms, may not have been identified, as steroid dependence frequently relied on outside physician reporting.
It should also be noted that the group from the NIH is one of the most experienced with VHL-related tumors. These cases, particularly in the reoperative setting, require technical excellence and proficiency with nuances such as not disrupting the delicate stellate arterial system of the adrenal, non-division of the adrenal vein, and delicate dissection around the tumor pseudocapsule without breaching the tumor or causing tumor rupture. The results from the study by Benhammou and colleagues represent the outcomes from this center of excellence and may not translate similarly in inexperienced hands. On a final note of caution, these results should not be extrapolated to metastatic disease to the adrenal glands, for which partial adrenalectomy is unproven and likely inferior.
The results of this study provide strong reassurance for surgeons offering partial adrenalectomy to patients with VHL-related pheochromocytoma, and provide hope for these patients who are hoping to avoid lifelong steroid replacement.