Surgical management of multi-organ visceral tumors in patients with von Hippel-Lindau disease

A single stage approach

Jonathan J. Hwang, Edward M. Uchio, Christian Pavlovich, Stephen E. Pautler, Steven K. Libutti, W. Marston Linehan, McClellan M. Walther

Research output: Contribution to journalArticle

Abstract

Purpose: We assessed surgical feasibility of a 1-stage multi-organ approach for multiple visceral tumors in patients with von Hippel-Lindau disease. Materials and Methods: A total of 14 men and 15 women with von Hippel-Lindau disease underwent simultaneous multi-organ surgery for multiple adrenal, renal and pancreatic tumors at the National Cancer Institute between 1988 and 2001. Perioperative and followup data were analyzed retrospectively. The Mann-Whitney U test was used for statistical analysis. Results: Surgery involving 2 or more organs (mean 2.4 procedures per patient, range 2 to 4) was performed in all patients and concurrent pancreatic operations were performed in 12 (41%). Overall a combined 71 procedures, were performed including 4 cases (13%) treated laparoscopically. Mean ± SD operative time and estimated blood loss were 464 ± 142 minutes (range 206 to 830) and 2,798 ± 4,285 cc (300 to 20,000), respectively. In 16 patients (55%) blood transfusion was administered intraoperatively. At a median followup of 21 months (range 5 to 151) renal tumors recurred in 8 patients (28%), requiring further kidney operations, but no patient had pancreatic or adrenal recurrence. The overall complication rate was 38%, and there was no operative mortality. Conclusions: A single stage surgical approach for multi-organ visceral tumors is a viable option for patients with von Hippel-Lindau disease. With careful patient selection and surgical planning combined procedures can be safely performed in 1 operative setting.

Original languageEnglish (US)
Pages (from-to)895-898
Number of pages4
JournalJournal of Urology
Volume169
Issue number3
DOIs
StatePublished - Mar 1 2003

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von Hippel-Lindau Disease
Neoplasms
Kidney
National Cancer Institute (U.S.)
Operative Time
Nonparametric Statistics
Blood Transfusion
Patient Selection
Recurrence
Mortality

Keywords

  • Hippel-Lindau disease
  • Laparoscopy
  • Pheochromocytoma
  • Surgery

ASJC Scopus subject areas

  • Urology

Cite this

Surgical management of multi-organ visceral tumors in patients with von Hippel-Lindau disease : A single stage approach. / Hwang, Jonathan J.; Uchio, Edward M.; Pavlovich, Christian; Pautler, Stephen E.; Libutti, Steven K.; Marston Linehan, W.; Walther, McClellan M.

In: Journal of Urology, Vol. 169, No. 3, 01.03.2003, p. 895-898.

Research output: Contribution to journalArticle

Hwang, Jonathan J. ; Uchio, Edward M. ; Pavlovich, Christian ; Pautler, Stephen E. ; Libutti, Steven K. ; Marston Linehan, W. ; Walther, McClellan M. / Surgical management of multi-organ visceral tumors in patients with von Hippel-Lindau disease : A single stage approach. In: Journal of Urology. 2003 ; Vol. 169, No. 3. pp. 895-898.
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