0022-5347/99/16 12-0395$03.00/0 zyxwvutsrqpon THE JOVRNAL OF UROLOGY Copyright zyxwvutsrqponm 0 1999 zyxwvutsrqponml by AMERICAN UROLOCICAL ASSOCIATION, INC. Vol. zyxw 161.39S398. February 1999 zy Printed zyx in USA. MANAGEMENT OF HEREDITARY PHEOCHROMOCYTOMA IN VON HIPPEL-LINDAU KINDREDS WITH PARTIAL ADRENALECTOMY McCLELLAN M. WALTHER, HARRY R. WISER, PETER L. CHOYKE, WALTER RAYFORD, J. CHRIS LYNE AND W. MARSTON LINEHAN From the Urologic Oncology Branch, National Cancer Institute, National Heart, zyxwvu Lung and Blood Institute, and Diagnostic Radiology Department, Warren G. Magnuson Clinical Center, National Institutes of Health, Bethesda, Maryland ABSTRACT Purpose: In patients with von Hippel-Lindau disease multiple bilateral adrenal pheochromo- cytoma can develop, which has traditionally been treated with adrenalectomy. Partial adrenal- ectomy can preserve normal adrenal function and avoid the morbidity associated with medical adrenal replacement. We demonstrate whether adrenal function could be preserved by partial adrenalectomy in patients with von Hippel-Lindau disease. Materials and Methods: From 1995 to 1998,13 consecutive von Hippel-Lindau disease patients with pheochromocytoma underwent 14 partial and 6 complete unilateral adrenalectomies. Func- tion of residual normal adrenal and recurrence of adrenal pheochromocytoma were determined at followup. Results: Of the patients 2 had undergone unilateral adrenalectomy and 1 had undergone complete and partial adrenalectomy previously. Following surgery residual normal adrenal tissue consisted of 1 partial adrenal in 3 patients, bilateral partial adrenal in 5, partial and complete adrenal gland in 1, 1 complete adrenal gland in 3 and no adrenal tissue in 1. Three patients with residual adrenal tissue were placed on medical adrenal replacement until adreno- corticotropic hormone stimulation testing demonstrated adrenocortical function. In 2 patients 1 adrenal and 2 extra-adrenal new pheochromocytomas developed 11 and 152 months, respec- tively, after partial adrenalectomy. No morbidity related to pheochromocytoma was observed during followup. Conclusions: Partial adrenalectomy can preserve adrenal function in patients with a heredi- tary form of pheochromocytoma. KEY WORDS: pheochromocytoma, Hippel-Lindau disease, treatment outcome, adrenalectomy The risk of development of pheochromocytoma in patients with von Hippel-Lindau disease is related to the type of mutation found in the von Hippel-Lindau disease The rate of pheochromocytoma development varies in von Hippel-Lindau disease from 0 to 57% of affected patients from different kindreds.2.3 Among those who have pheochro- mocytoma bilateral adrenal tumors develop in zyxwvut 47%.2 Pheo- chromocytoma is traditionally treated with adrenalectomy. Given the high risk of bilateral disease, many patients face the possibility of losing all adrenal function following bilat- eral adrenalectomy. Medical replacement therapy is associ- ated with decreased quality of life.4.5 Preservation of normal adrenocortical tissue is important in patients with a solitary adrenal gland or hereditary tumor syndromes that involve both adrenal glands. Partial adrenalectomy can preserve ad- renal function, avoiding the morbidity of medical adrenal replacement. We present the outcome of von Hippel-Lindau disease patients with pheochromocytoma who underwent ad- renal sparing surgery when possible. Patients were followed to evaluate function of residual adrenocortical tissue and recurrence of pheochromocytoma. MATERIALS AND METHODS From September 1995 to January 1998,13 consecutive von Hippel-Lindau disease patients with pheochromocytoma were treated at the National Institutes of Health (table 1). Medical records were reviewed to identify previous treatment for pheochromocytoma. Median followup from the first diag- nosis of pheochromocytoma was 23 months (range 3 to 408). Accepted for publication August 7, 1998. Pheochromocytoma evaluation has been previously de- scribed.2 Patients underwent abdominal computerized to- mography and 24-hour urine collection for catecholamine excretion. Tumor volume was calculated by the formula 0.523 x (diameterL3 Metaiodobenzylguanidine, magnetic resonance imaging and physiological testing were performed as indicated.2 Followup consisted of abdominal computerized tomography 3 to 6 months after surgery and every 1 to 2 years thereafter for all patients. Patient 10 who had under- gone prior partial adrenalectomy was excluded from compar- ison with the partial adrenalectomy group. Contiguous pheo- chromocytomas were scored together. Partial adrenalectomy was performed through an open surgical approach in 12 of 13 patients as a portion of normal adrenal tissue with its blood supply could be preserved. Tu- mors in the middle of the adrenal gland were resected with a rim of normal adrenal. Interrupted 3-zero silk horizontal mattress suture ligatures were placed to prevent bleeding from normal adrenal tissue. Tumors in the head or tail of the adrenal were removed by amputating the tumorous portion of the gland. The amputation approach was readily transfer- able to a laparoscopic technique, which was performed in 1 patient.6 Dissection of the normal adrenal tissue and its pedicle was avoided to preserve the vascular supply. Com- plete adrenalectomy was performed through a laparoscopic approach.6 Patients were administered replacement doses of hydrocor- tisone when there was concern about residual adrenocortical function after surgery. Adrenocorticotropic hormone (ACTHI stimulation testing was performed 1 week to 6 months after 395