Safety and curative effect analysis of bilateral simultaneous adrenalectomy for the treatment of the bilateral adrenal pheochromocytoma associated with multiple endocrine neoplasia type 2A

Abstract

Objective To study the clinical features of the bilateral adrenal pheochromocytomas (PHEO) in patients associated with multiple endocrine neoplasia type 2A (MEN2A) and to evaluate the safety and feasibility of applying simultaneous bilateral adrenalectomy (SBA).Methods From November 2003 to April 2011,clinical data of 8 patients from 4 unrelated MEN2A families treated with SBA were analyzed.There were 6 males and 2 females.The mean age was 44 years (range,35 to 56 years).4 patients were asymptomatic and 4 had clinical features related to PHEO.The diagnosis of bilateral PHEO was made after the diagnosis of MTC in 4 cases,previous hypertension in 1 case,and family investigation found 3 MTC and all were found with hyperparathyroidism.Plasma catecholamines were substantially elevated in 4 patients.Ultrasound and computerized tomography (CT) examination showed bilateral adrenal masses,of which 6 were bilateral and multiple tumors,2 were single tumor in one side and multiple tumors in the other side.On contrast CT,the tumors displayed heterogeneous contrast enhancement and low density area in the center of the tumors observed in 3 cases;renal,splenic vessels displacement and deformation were observed;local lymph node enlargement was not found.The mean maximum diameter of PHEO was 4.4 cm (range,2.7 to 7.0 cm).RET screening showed a heterozygous nucleotide substitution on exon 11,c.G1901A (p.C634Y) in all patients.After adequate adrenergic blockade,the SBA was performed by a single surgeon,PHEO were extirpated by laparoscopic radical adrenalectomy in 2 patie.nts,while in other 6 patients,PHEO were removed by partial adrenalectomy or adrenal tumorectomy with at least one normal appearing adrenal tissue preserved (4 by laparoscopic approach and 2 by open approach).Results The procedures were successful carried out on 8 cases with bilateral PHEO.There were totally 43 pieces of tumor obtained.The average time of operation was 181 min (range,150 to 240 min).Mean blood loss was 171ml (range,100 to 300 ml).Blood pressure fluctuations happened in 2 cases during operation (maximum:230/110 vs minimum:70/40 mm Hg).Histopathology showed PHEO in all patients.During the follow-up of 35 months (range,3 to 90),local and/or distant metastatic recurrence was not found in all cases,while two treated by total bilateral adrenalectomy and one by adrenal-sparing surgery required corticosteroid replacement.Conclusions SBA for bilateral PHEO in MEN2A is technically safe and feasible.Transperitoneal laparoscopic partial adrenalectomy for the treatment of bilateral PHEO can be considered as preferential choice. Key words: Multiple endocrine neoplasia type 2A; Pheochromocytoma; Bilateral simultaneous adrenalectomy; RET gene; Laparoscopy