Surgical management of multi-organ visceral tumors in patients with von Hippel-Lindau disease: a single stage approach.
OBJECTIVE
We assessed surgical feasibility of a 1-stage multi-organ approach for multiple visceral tumors in patients with von Hippel-Lindau disease.


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.
Hwang
Jonathan J
JJ
Urological Oncology Branch, National Cancer Institute, National Institute of Health, Bethesda, Maryland, USA.
Uchio
Edward M
EM
Pavlovich
Christian P
CP
Pautler
Stephen E
SE
Libutti
Steven K
SK
Linehan
W Marston
WM
Walther
McClellan M
MM
D000293:Adolescent; D000310:Adrenal Gland Neoplasms / Q000150:complications / Q000601:surgery*; D000328:Adult; D002292:Carcinoma, Renal Cell / Q000150:complications / Q000601:surgery; D005260:Female; D006801:Humans; D007680:Kidney Neoplasms / Q000150:complications / Q000601:surgery*; D008297:Male; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary / Q000601:surgery; D018358:Neuroendocrine Tumors / Q000150:complications / Q000601:surgery; D010190:Pancreatic Neoplasms / Q000150:complications / Q000601:surgery*; D010673:Pheochromocytoma / Q000150:complications / Q000601:surgery; D011183:Postoperative Complications; D012189:Retrospective Studies; D006623:von Hippel-Lindau Disease / Q000150:complications*