Category: Laparoscopic/ Robotic: Adrenal
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
Department of Urology, Kawasaki Medical School, Kurashiki, Japan
Kurashiki, Okayama, Japan