Long-term Functional and Oncologic Outcomes of Partial Adrenalectomy for Pheochromocytoma

Published:February 25, 2020DOI:



      To evaluate the recurrence and functional outcomes in a primarily hereditary cohort of patients undergoing partial adrenalectomy for pheochromocytoma.


      A retrospective review from a prospectively managed database of patients undergoing partial adrenalectomy from 1995 to 2018 at the National Cancer Institute was performed. Local recurrence was defined as imaging evidence of a recurrent or de novo lesion on the operative side. Steroid dependency was defined as requiring daily steroid replacement at time of last follow-up.


      One hundred and twenty-four partial adrenalectomies, removing 162 tumors, were performed in 107 patients. Most patients had a known hereditary predisposition to develop bilateral, multifocal, and recurrent pheochromocytoma. Median tumor size was 2 cm (interquartile range (IQR) 1.5-2.8). Median follow-up was 60 months (IQR 13-131). Local recurrence occurred in 17 patients (15.8%) and were managed with active surveillance or surgery. A single patient (1/106, 0.9%) developed metastatic spread of pheochromocytoma approximately 14 years after his first of 2 partial adrenalectomies and remains alive under active surveillance. Median time to recurrence was 71 months (IQR 26-127) with 10 patients (9.3%) requiring daily steroid replacement at time of last follow-up.


      Partial adrenalectomy offers excellent oncologic and functional outcomes, sparing most patients from lifelong steroid replacement therapy. Recurrences can be easily managed with repeat surgery or active surveillance via functional work-up and imaging. Partial adrenalectomy remains the recommended surgical management for patients pre-disposed to development of bilateral, multifocal and recurrent pheochromocytoma.
      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


      Subscribe to Urology
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


        • Tischler AS
        Pheochromocytoma and extra-adrenal paraganglioma: updates.
        Arch Pathol Lab Med. 2008; 132: 1272-1284
        • Fishbein L
        • Leshchiner I
        • Walter V
        • et al.
        Comprehensive molecular characterization of pheochromocytoma and paraganglioma.
        Cancer Cell. 2017; 31: 181-193
        • Neumann HPH
        • Bausch B
        • McWhinney SR
        • et al.
        Germ-line mutations in nonsyndromic pheochromocytoma.
        N Engl J Med. 2002; 346: 1459-1466
        • Jenny W
        • Peter S
        • Oliver G
        Genetics and clinical characteristics of hereditary pheochromocytomas and paragangliomas.
        Endocr Relat Cancer. 2011; 18: R253-R276
        • Walther MM
        • Reiter R
        • Keiser HR
        • et al.
        Clincial and genetic characterization of pheocrhomocytoma in Von Hippel-Lindau Familys: comparison with sporadic pheochromocytoma gives insited into natorual history of pheochromocytoma.
        J Urol. 1999; 162: 659-664
        • Charmandari E
        • Nicolaides NC
        • Chrousos GP
        Adrenal insufficiency.
        Lancet. 2014; 383: 2152-2167
        • Walther MM
        • Keiser HR
        • Choyke PL
        • Rayford W
        • Lyne JC
        • Linehan WM
        Management of hereditary pheochromocytoma in Von Hippel-Lindau Kindreds with partial adrenalectomy.
        J Urol. 1999; 161: 395-398
        • Madala A
        • Daugherty M
        • Bratslavsky G
        Partial adrenalectomy—why should it be considered?.
        Urol Pract. 2015; 2: 359-366
        • Grubbs EG
        • Rich TA
        • Ng C
        • et al.
        Long-term outcomes of surgical treatment for hereditary pheochromocytoma.
        J Am Coll Surg. 2013; 216: 280-289
        • Nagaraja V
        • Eslick GD
        • Edirimanne S
        Recurrence and functional outcomes of partial adrenalectomy: a systematic review and meta-analysis.
        Int J Surg (London, England). 2015; 16: 7-13
        • Benhammou JN
        • Boris RS
        • Pacak K
        • Pinto PA
        • Linehan WM
        • Bratslavsky G
        Functional and oncologic outcomes of partial adrenalectomy for pheochromocytoma in patients with von Hippel-Lindau syndrome after at least 5 years of followup.
        J Urol. 2010; 184: 1855-1859
        • Asher KP
        • Gupta GN
        • Boris RS
        • Pinto PA
        • Linehan WM
        • Bratslavsky G
        Robot-assisted laparoscopic partial adrenalectomy for pheochromocytoma: the National Cancer Institute technique.
        Eur Urol. 2011; 60 ([Epub ahead of print]): 118-124
        • Perry RR
        • Keiser HR
        • Norton JA
        • et al.
        Surgical management of pheochromocytoma with the use of metyrosine.
        Ann Surg. 1990; 212: 621-628
        • Amar L
        • Servais A
        • Gimenez-Roqueplo AP
        • Zinzindohoue F
        • Chatellier G
        • Plouin PF
        Year of diagnosis, features at presentation, and risk of recurrence in patients with pheochromocytoma or secreting paraganglioma.
        J Clin Endocrinol Metab. 2005; 90: 2110-2116
        • Pamporaki C
        • Eisenhofer G
        • Fliedner S
        • et al.
        Characteristics of pediatric vs adult pheochromocytomas and paragangliomas.
        J Clin Endocrinol Metab. 2017; 102: 1122-1132
        • Hamidi O
        • Young JWF
        • Iñiguez-Ariza NM
        • et al.
        Malignant pheochromocytoma and paraganglioma: 272 patients over 55 Years.
        J Clin Endocrinol Metab. 2017; 102: 3296-3305
        • Hamidi O
        • Young WF
        • Gruber L
        • et al.
        Outcomes of patients with metastatic phaeochromocytoma and paraganglioma: a systematic review and meta-analysis.
        Clin Endocrinol. 2017; 87: 440-450
        • Jimenez C
        Treatment for patients with malignant pheochromocytomas and paragangliomas: a perspective from the Hallmarks of cancer.
        Front Endocrinol. 2018; 9 (277-277)
        • Roukounakis N
        • Dimas S
        • Kafetzis I
        • et al.
        Is preservation of the adrenal vein mandatory in laparoscopic adrenal-sparing surgery?.
        JSLS. 2007; 11: 215-218
        • Ikeda Y
        • Takami H
        • Niimi M
        • Kan S
        • Sasaki Y
        • Takayama J
        Laparoscopic partial or cortical-sparing adrenalectomy by dividing the adrenal central vein.
        Surg Endosc. 2001; 15: 747-750
        • Brauckhoff M
        • Gimm O
        • Thanh PN
        • et al.
        Critical size of residual adrenal tissue and recovery from impaired early postoperative adrenocortical function after subtotal bilateral adrenalectomy.
        Surgery. 2003; 134: 1020-1027
        • Tuncel A
        • Balci M
        • Aykanat C
        • Aslan Y
        • Berker D
        • Guzel O
        Laparoscopic partial adrenalectomy using near-infrared imaging: the initial experience.
        Minim Invasive Ther Allied Technol. 2019; : 1-7
        • Neumann HPH
        • Tsoy U
        • Bancos I
        • et al.
        Comparison of pheochromocytoma-specific morbidity and mortality among adults with bilateral pheochromocytomas undergoing total adrenalectomy vs cortical-sparing adrenalectomy.
        JAMA Netw Open. 2019; 2 (e198898-e198898)
        • Neumann HP
        • Bender BU
        • Reincke M
        • Eggstein S
        • Laubenberger J
        • Kirste G
        Adrenal-sparing surgery for phaeochromocytoma.
        Br J Surg. 1999; 86: 94-97
        • Walz MK
        • Alesina PF
        • Wenger FA
        • et al.
        Laparoscopic and retroperitoneoscopic treatment of pheochromocytomas and retroperitoneal paragangliomas: results of 161 tumors in 126 patients.
        World J Surg. 2006; 30: 899-908
        • Walz MK
        • Iova LD
        • Deimel J
        • et al.
        Minimally invasive surgery (MIS) in children and adolescents with pheochromocytomas and retroperitoneal paragangliomas: experiences in 42 patients.
        World J Surg. 2018; 42: 1024-1030
        • Gagner M
        • Lacroix A
        • Bolte E
        Laparoscopic adrenalectomy in Cushing's syndrome and pheochromocytoma.
        N Engl J Med. 1992; 327: 1033
        • Boris RS
        • Gupta G
        • Linehan WM
        • Pinto PA
        • Bratslavsky G
        Robot-assisted laparoscopic partial adrenalectomy: initial experience.
        Urology. 2011; 77: 775-780
        • Ball MW
        • Hemal AK
        • Allaf ME
        International consultation on urological diseases and European Association of Urology International Consultation on Minimally Invasive Surgery in Urology: laparoscopic and robotic adrenalectomy.
        BJU Int. 2017; 119: 13-21
        • Hyams ES
        • Stifelman MD
        The role of robotics for adrenal pathology.
        Curr Opin Urol. 2009; 19: 89-96