Abstract
Objective
To evaluate the recurrence and functional outcomes in a primarily hereditary cohort
of patients undergoing partial adrenalectomy for pheochromocytoma.
Methods
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.
Results
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.
Conclusion
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 accessOne-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:
Subscribe to UrologyAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Pheochromocytoma and extra-adrenal paraganglioma: updates.Arch Pathol Lab Med. 2008; 132: 1272-1284
- Comprehensive molecular characterization of pheochromocytoma and paraganglioma.Cancer Cell. 2017; 31: 181-193
- Germ-line mutations in nonsyndromic pheochromocytoma.N Engl J Med. 2002; 346: 1459-1466
- Genetics and clinical characteristics of hereditary pheochromocytomas and paragangliomas.Endocr Relat Cancer. 2011; 18: R253-R276
- 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
- Adrenal insufficiency.Lancet. 2014; 383: 2152-2167
- Management of hereditary pheochromocytoma in Von Hippel-Lindau Kindreds with partial adrenalectomy.J Urol. 1999; 161: 395-398
- Partial adrenalectomy—why should it be considered?.Urol Pract. 2015; 2: 359-366
- Long-term outcomes of surgical treatment for hereditary pheochromocytoma.J Am Coll Surg. 2013; 216: 280-289
- Recurrence and functional outcomes of partial adrenalectomy: a systematic review and meta-analysis.Int J Surg (London, England). 2015; 16: 7-13
- 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
- Robot-assisted laparoscopic partial adrenalectomy for pheochromocytoma: the National Cancer Institute technique.Eur Urol. 2011; 60 ([Epub ahead of print]): 118-124
- Surgical management of pheochromocytoma with the use of metyrosine.Ann Surg. 1990; 212: 621-628
- 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
- Characteristics of pediatric vs adult pheochromocytomas and paragangliomas.J Clin Endocrinol Metab. 2017; 102: 1122-1132
- Malignant pheochromocytoma and paraganglioma: 272 patients over 55 Years.J Clin Endocrinol Metab. 2017; 102: 3296-3305
- Outcomes of patients with metastatic phaeochromocytoma and paraganglioma: a systematic review and meta-analysis.Clin Endocrinol. 2017; 87: 440-450
- Treatment for patients with malignant pheochromocytomas and paragangliomas: a perspective from the Hallmarks of cancer.Front Endocrinol. 2018; 9 (277-277)
- Is preservation of the adrenal vein mandatory in laparoscopic adrenal-sparing surgery?.JSLS. 2007; 11: 215-218
- Laparoscopic partial or cortical-sparing adrenalectomy by dividing the adrenal central vein.Surg Endosc. 2001; 15: 747-750
- Critical size of residual adrenal tissue and recovery from impaired early postoperative adrenocortical function after subtotal bilateral adrenalectomy.Surgery. 2003; 134: 1020-1027
- Laparoscopic partial adrenalectomy using near-infrared imaging: the initial experience.Minim Invasive Ther Allied Technol. 2019; : 1-7
- 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)
- Adrenal-sparing surgery for phaeochromocytoma.Br J Surg. 1999; 86: 94-97
- Laparoscopic and retroperitoneoscopic treatment of pheochromocytomas and retroperitoneal paragangliomas: results of 161 tumors in 126 patients.World J Surg. 2006; 30: 899-908
- Minimally invasive surgery (MIS) in children and adolescents with pheochromocytomas and retroperitoneal paragangliomas: experiences in 42 patients.World J Surg. 2018; 42: 1024-1030
- Laparoscopic adrenalectomy in Cushing's syndrome and pheochromocytoma.N Engl J Med. 1992; 327: 1033
- Robot-assisted laparoscopic partial adrenalectomy: initial experience.Urology. 2011; 77: 775-780
- 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
- The role of robotics for adrenal pathology.Curr Opin Urol. 2009; 19: 89-96
Article info
Publication history
Published online: February 25, 2020
Accepted:
February 17,
2020
Received:
November 5,
2019
Footnotes
Funding Support: This research was supported by the Intramural Research Program of the NIH, National Cancer Institute, Center for Cancer Research grant (ZIA BC011038).
Identification
Copyright
Published by Elsevier Inc.
