Category: Laparoscopic/ Robotic: Adrenal

VS17-1 - Laparoscopic adrenal-sparing surgery in pheochromocytoma associated with multiple endocrine neoplasia type 2

Sun, Sep 23
2:00 PM - 4:00 PM

Introduction & Objective : Multiple endocrine neoplasia type 2A (MEN 2A) is an autosomal dominant disorder for which RET is the responsible gene. Approximately 70% of cases of MEN 2A are associated with pheochromocytoma. Pheochromocytoma with MEN 2A is usually multiple and bilateral. Avoidance of Addisonian crisis is important after adrenalectomy and this requires corticosteroid supplementation for life. We report five cases of pheochromocytoma associated with MEN 2A that were treated with laparoscopic adrenal-sparing surgery to preserve adrenal function.


Methods : Between 2010 and 2017, five patients with MEN 2A were female with a median age of 26 years (range : 22-49) and their median BMI was 23.2 (range: 18.5-31.9). Two cases were contralateral pheochromocytoma after unilateral transperitoneal adrenalectomy for pheochromocytoma at 20 and 21 years ago, respectively. They underwent laparoscopic subtotal adrenalectomy. Three cases with synchronous bilateral pheochromocytoma were siblings. Two of them underwent laparoscopic bilateral adrenal-sparing surgery and another one underwent unilateral laparoscopic subtotal adrenalectomy after contralateral total adrenalectomy.


Results : In total, we performed laparoscopic adrenal-sparing surgery in 7 adrenal glands and their site of right and left were 3 and 4, respectively.  Four adrenal glands had  solitary tumor and other three had multiple tumors. The total numbers of extirpation tumors were 12 and the median size in diameter was 19 mm (range: 6-70). The median operating time was 195 min (range: 116-246) and the maximum estimated blood loss was 100 ml, none of the cases required transfusion.  There were no postoperative complications in all cases. The median follow-up time has been 13 months (range: 9-88). In all patients, the latest post operative catecholamine and metanephrine in a 24 hours stored urine were less than normal levels. And the latest plasma cortisol concentration in all cases were normal range without corticosteroid supplement therapy.


Conclusions : Laparoscopic adrenal-sparing surgery in pheochromocytoma associated with MEN2  is a minimally invasive and cortico-sparing treatment that avoids Addisonian crisis.

Yoshiyuki Miyaji

Associate Professor
Department of Urology, Kawasaki Medical School, Kurashiki, Japan
Kurashiki, Okayama, Japan

Masaichiro Fujita

Kurashiki, Okayama, Japan

Shohei Tsukimori

Kurashiki, Okayama, Japan

Seitetsu Sugiyama

Kurashiki, Okayama, Japan

Shin Ohira

Kurashiki, Okayama, Japan

Shinjiro Shimizu

Kurashiki, Okayama, Japan

Mikako Kaifu

Kurashiki, Okayama, Japan

Ryoei Hara

Kurashiki, Okayama, Japan

Tomohiro Fujii

Kurashiki, Okayama, Japan

Atsushi Nagai

Kurashiki, Okayama, Japan