Adrenalectomy for familial pheochromocytoma in the laparoscopic era.
OBJECTIVE
To report the results of treatment of patients with familial pheochromocytomas in the laparoscopic era.


BACKGROUND
The optimal surgical management of pheochromocytomas that arise in familial neoplasia syndromes may be complicated by bilateral involvement and associated endocrinopathies.


METHODS
Twenty-one patients with familial pheochromocytomas (15 with multiple endocrine neoplasia [MEN] 2A, 4 with MEN 2B, 1 each with von Hippel-Lindau and neurofibromatosis type 1) underwent adrenalectomy between December 1993 and July 2001. Clinical, biochemical, and pathologic data were obtained by retrospective review of perioperative medical records, postoperative biochemical testing, and patient questionnaire.


RESULTS
Mean age at diagnosis was 37 +/- 11 years. Twenty of the 21 patients had elevated urine catecholamines, and all had radiographic evidence of an adrenal tumor or tumors. Pheochromocytoma-related symptoms were present in 11 patients (52%). One patient with MEN 2B underwent open adrenalectomy due to previous adrenal surgery and megacolon. Laparoscopic adrenalectomy was attempted in the remaining 20 patients (9 right, 11 left, 2 bilateral). Two patients (9.1%) were converted to open adrenalectomy. Intraoperative hypertensive episodes occurred in 15 patients (71%) and were easily controlled medically. Mean operative time was 216 +/- 57 minutes, mean postoperative length of stay was 3.1 +/- 1.3 days, and mean tumor size was 3.1 +/- 1.0 cm. Minor complications occurred in three patients (14.3%) and major complications in two patients (9.5%). During a mean follow-up of 57 months, a contralateral pheochromocytoma developed in four patients with MEN 2 (33%); three of them underwent adrenalectomy. There have been no long-term complications related to hypertension or adrenalectomy.


CONCLUSIONS
This study is the largest series of patients with familial pheochromocytoma undergoing adrenalectomy during the laparoscopic era. The results suggest that the laparoscopic approach is safe and effective for managing unilateral or bilateral adrenal medullary disease in this population.
Brunt
L Michael
LM
Department of Surgery, Section of Endocrine and Oncologic Surgery, Washington University School of Medicine, St. Louis, Missouri 63110, USA. bruntm@msnotes.wustl.edu
Lairmore
Terry C
TC
Doherty
Gerard M
GM
Quasebarth
Mary A
MA
DeBenedetti
Mary
M
Moley
Jeffrey F
JF
D000310:Adrenal Gland Neoplasms / Q000235:genetics* / Q000601:surgery*; D000315:Adrenalectomy; D000328:Adult; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D010535:Laparoscopy; D007902:Length of Stay / Q000706:statistics & numerical data; D008297:Male; D018813:Multiple Endocrine Neoplasia Type 2a / Q000235:genetics; D018814:Multiple Endocrine Neoplasia Type 2b / Q000235:genetics; D010673:Pheochromocytoma / Q000235:genetics* / Q000601:surgery*; D011183:Postoperative Complications / Q000453:epidemiology; D013997:Time Factors