Purpose: Patients with von Hippel-Lindau disease are predisposed to multipl
e bilateral adrenal pheochromocytoma. In these patients partial adrenalecto
my may preserve adrenocortical function and avoid the morbidity associated
with medical adrenal replacement. We report our experience with such cases.
Materials and Methods: Laparoscopic partial adrenalectomy was performed in
patients with von Hippel-Lindau disease and pheochromocytoma when there was
evidence of normal adrenocortical tissue on preoperative imaging or intrao
perative examination. Suture ligature or a harmonic scalpel was used to exc
ise the tumors, leaving a 2 to 3 mm. margin of normal tissue.
Results: Two patients underwent laparoscopic partial adrenalectomy and 1 la
paroscopic bilateral partial adrenalectomy with preservation of normal adre
nocortical tissue. Seven pheochromocytomas were removed. Laparoscopic ultra
sound was essential for localizing 2 pheochromocytomas that were not visual
ized by the camera. Median operative time was 324 minutes, blood loss 100 c
c and parenteral narcotic requirement 22 mg. morphine equivalents. No patie
nt required hydrocortisone replacement. There has been no pheochromocytoma
recurrence during short-term followup.
Conclusions: Laparoscopic partial adrenalectomy is technically feasible in
patients with a hereditary form of pheochromocytoma, and may preserve adren
ocortical function. Laparoscopic ultrasound was necessary to identify 2 of
the 7 pheochromocytomas removed.