Simultaneous bilateral laparoscopic cortical-sparing adrenalectomy for bilateral pheochromocytomas in multiple endocrine neoplasia type 2

Xiao-Ping Qi; Bi-Jun Lian; Xu-Dong Fang; Fang Dong; Feng Li; Hang-Yang Jin; Ke Zhang; Kang-Er Wang; Yi Zhang

doi:10.3389/fsurg.2022.1057821

Simultaneous bilateral laparoscopic cortical-sparing adrenalectomy for bilateral pheochromocytomas in multiple endocrine neoplasia type 2

Front Surg. 2023 Jan 10:9:1057821. doi: 10.3389/fsurg.2022.1057821. eCollection 2022.

Authors

Xiao-Ping Qi¹, Bi-Jun Lian¹, Xu-Dong Fang¹, Fang Dong¹, Feng Li¹, Hang-Yang Jin¹, Ke Zhang², Kang-Er Wang³, Yi Zhang⁴

Affiliations

¹ Department of Oncologic and Urologic Surgery, The 903rd PLA Hospital, Hangzhou Medical College, Hangzhou, China.
² Center for Radiation Oncology, Affiliated Hangzhou Cancer Hospital, Zhejiang University School of Medicine, Hangzhou, China.
³ Department of Urology, Affiliated Hospital of Hangzhou Normal University, Hangzhou, China.
⁴ Department of Urology, The Affiliated People's Hospital of Ningbo University, Ningbo, China.

Abstract

Purpose: This study aimed to assess the feasibility of synchronous bilateral laparoscopic or open cortical-sparing adrenalectomy (SB-LCSA or SB-OCSA) for bilateral pheochromocytomas (bPHEOs) in multiple endocrine neoplasia type 2 (MEN2).

Methods: Altogether, 31 patients (54.8% were women) were diagnosed with MEN2-related bPHEOs, and 29 of them underwent varying specific adrenalectomies. We systematically analyzed and evaluated their clinical profiles, mutation types, tumor histopathological features, and follow-up records.

Results: All 31 patients with bPHEOs presented with RET-C634 (90.3%) and RET-M918T (9.7%) mutations, and the median age at initial presentation was 38 years (range, 23-78). bPHEOs were synchronous in 27 patients and metachronous in 4 (12.9%) patients. In total, 29 patients underwent initial cortical-sparing adrenalectomy (CSA) including 23 (79.3%) undergoing synchronous bilateral CSA (18 SB-LCSA and 5 SB-OCSA) and 6 (20.7%) undergoing metachronous CSA. SB-LCSA and synchronous surgery were associated with less bleeding volume and shorter length of hospital stay than SB-OCSA and metachronous surgery (all P's < 0.05). Corticosteroid replacement treatment was necessary for 14 patients (45.2%) after bilateral CSA. During a median follow-up period of 7 years (range, 1.8-23), three of these patients (10.3%) had a recurrent disease that required reoperation.

Conclusion: SB-LCSA is feasible for treating synchronous bPHEOs and should be recommended as a prioritized surgical approach.

Keywords: RET proto-oncogene; laparoscopy; multiple endocrine neoplasia type 2; pheochromocytoma; simultaneous bilateral adrenal-sparing adrenalectomy.

Grants and funding

This work was supported by the National Natural Science Foundation of China (81472861); the Key Project of Zhejiang Province Science and Technology Plan, China (2014C03048-1); and the Hangzhou Municipal Commission of Health and Family Planning Science and Technology Program (OO20190253, B20210355).