open access
Laparoscopic cortical-sparing adrenal surgery in pheochromocytomas associated with hereditary neoplasia syndromes
, 1, 1, 2, 1, 1, 1, 3, 3, 2, 1, 1, 1
Affiliations- Department of Nuclear Medicine and Endocrine Oncology, Maria Sklodowska-Curie National Research Institute of Oncology, Gliwice Branch, Poland
- The Oncologic and Reconstructive Surgery Clinic, Maria Sklodowska-Curie National Research Institute of Oncology, Gliwice Branch, Poland
- Department of Genetic and Molecular Diagnostics of Cancer, M. Sklodowska-Curie National Research Institute of Oncology, Gliwice Branch, Poland
open access
Abstract
Introduction: Pheochromocytomas in hereditary syndromes tend to grow multifocal with adrenal involvement on both sides. Surgical treatment with bilateral adrenalectomy inevitably leads to life-long hormonal dependence, which significantly affects quality of life. The development of minimally invasive adrenal surgery has created a chance to preserve adrenal cortex function in these patients. The aim of the present study was to evaluate the safety of laparoscopic cortical-sparing adrenal surgeries and their efficacy in the prevention of postoperative adrenal insufficiency in patients with hereditary pheochromocytomas.
Material and methods: We retrospectively analysed the medical histories of 10 patients, who underwent 10 laparoscopic cortical sparing adrenal surgeries from January 2015 to January 2019 in our centre. The decision to perform sparing surgery was based on preoperative diagnosis of hereditary syndrome in line with the result of DNA analysis or its diagnosis based on the clinical appearance. All surgeries were performed laparoscopically from transperitoneal access in the lateral decubitus position, with preserving 1/3–1/4 adrenal tissue. The sufficiency of remnant adrenal tissue was assessed in all patients. The median time of follow-up was three years (ranged 0.5–4 years).
Results: No intraoperative complications were observed. One case of acute heart failure was the only early postoperative adverse event. There were no late postoperative complications and no local recurrences observed. In one out of three patients undergoing sparing surgery as a second procedure after former total adrenalectomy, adrenal cortex failure occurred. In all patients after unilateral surgery or after bilateral surgery performed simultaneously (total adrenalectomy at one side and sparing surgery contralaterally), function of remnant adrenal tissue was preserved.
Conclusions: In hereditary pheochromocytomas, with minimal risk of malignant process, laparoscopic cortical sparing adrenal surgeries are the safe approach and provide the chance to preserve adrenal cortex function.
Abstract
Introduction: Pheochromocytomas in hereditary syndromes tend to grow multifocal with adrenal involvement on both sides. Surgical treatment with bilateral adrenalectomy inevitably leads to life-long hormonal dependence, which significantly affects quality of life. The development of minimally invasive adrenal surgery has created a chance to preserve adrenal cortex function in these patients. The aim of the present study was to evaluate the safety of laparoscopic cortical-sparing adrenal surgeries and their efficacy in the prevention of postoperative adrenal insufficiency in patients with hereditary pheochromocytomas.
Material and methods: We retrospectively analysed the medical histories of 10 patients, who underwent 10 laparoscopic cortical sparing adrenal surgeries from January 2015 to January 2019 in our centre. The decision to perform sparing surgery was based on preoperative diagnosis of hereditary syndrome in line with the result of DNA analysis or its diagnosis based on the clinical appearance. All surgeries were performed laparoscopically from transperitoneal access in the lateral decubitus position, with preserving 1/3–1/4 adrenal tissue. The sufficiency of remnant adrenal tissue was assessed in all patients. The median time of follow-up was three years (ranged 0.5–4 years).
Results: No intraoperative complications were observed. One case of acute heart failure was the only early postoperative adverse event. There were no late postoperative complications and no local recurrences observed. In one out of three patients undergoing sparing surgery as a second procedure after former total adrenalectomy, adrenal cortex failure occurred. In all patients after unilateral surgery or after bilateral surgery performed simultaneously (total adrenalectomy at one side and sparing surgery contralaterally), function of remnant adrenal tissue was preserved.
Conclusions: In hereditary pheochromocytomas, with minimal risk of malignant process, laparoscopic cortical sparing adrenal surgeries are the safe approach and provide the chance to preserve adrenal cortex function.
Keywords
pheochromocytoma; adrenal sparing surgery; adrenal insufficiency
About this articleTitle
Laparoscopic cortical-sparing adrenal surgery in pheochromocytomas associated with hereditary neoplasia syndromes
Journal
Issue
Article type
Original paper
Pages
518-523
Published online
2020-10-28
Page views
1003
Article views/downloads
812
DOI
Pubmed
Bibliographic record
Endokrynol Pol 2020;71(6):518-523.
Keywords
pheochromocytoma
adrenal sparing surgery
adrenal insufficiency
Authors
Kornelia Hasse-Lazar
Marcin Zeman
Agnieszka Kotecka-Blicharz
Marta Legutko
Agnieszka Czarniecka
Barbara Michalik
Aleksandra Król
Piotr Szymański
Agnieszka Pawlaczek
Małgorzata Oczko-Wojciechowska
Maciej Grajek
Tomasz Gawlik
Daria Handkiewicz-Junak
Barbara Jarząb
References (21)- Rossitti HM, Söderkvist P, Gimm O. Extent of surgery for phaeochromocytomas in the genomic era. Br J Surg. 2018; 105(2): e84–e98.
- Favier J, Amar L, Gimenez-Roqueplo AP. Paraganglioma and phaeochromocytoma: from genetics to personalized medicine. Nat Rev Endocrinol. 2015; 11(2): 101–111.
- Dahia PLM. Pheochromocytoma and paraganglioma pathogenesis: learning from genetic heterogeneity. Nat Rev Cancer. 2014; 14(2): 108–119.
- Bausch B, Schiavi F, Ni Y, et al. European-American-Asian Pheochromocytoma-Paraganglioma Registry Study Group. Clinical Characterization of the Pheochromocytoma and Paraganglioma Susceptibility Genes SDHA, TMEM127, MAX, and SDHAF2 for Gene-Informed Prevention. JAMA Oncol. 2017; 3(9): 1204–1212.
- Neumann HPH, Tsoy U, Bancos I, et al. International Bilateral-Pheochromocytoma-Registry Group. Outcomes of adrenal-sparing surgery or total adrenalectomy in phaeochromocytoma associated with multiple endocrine neoplasia type 2: an international retrospective population-based study. Lancet Oncol. 2014; 15(6): 648–655.
- Kotecka-Blicharz A, Hasse-Lazar K, Jurecka-Lubieniecka B, et al. Occurrence of phaeochromocytoma tumours in RET mutation carriers - a single-centre study. Endokrynol Pol. 2016; 67(1): 54–58.
- Pogorzelski R, Toutounchi S, Ambroziak U, et al. Effectiveness of unilateral laparoscopic adrenalectomy in ACTH-independent hypercortisolaemia and subclinical Cushing's syndrome - a retrospective study on a large cohort. Endokrynol Pol. 2018; 69(4): 411–415.
- Kozłowski T, Rydzewska-Rosolowska A, Myśliwiec J, et al. The impact of laparoscopic adrenalectomy on renal function. Results of a prospective randomised clinical trial. Endokrynol Pol. 2019; 70(5): 409–416.
- Bednarczuk T, Bolanowski M, Sworczak K, et al. Adrenal incidentaloma in adults — management recommendations by the Polish Society of Endocrinology. Endokrynol Pol. 2016; 67(2): 234–258.
- Lenders JWM, Duh QY, Eisenhofer G, et al. Endocrine Society. Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline. J Clin Endocrinol Metab. 2014; 99(6): 1915–1942.
- Castinetti F, Taieb D, Henry JF, et al. Managment of endocrine Disease:Outcome of adrenal sparing surgery in heritable pheochromocytoma. Eur J Endocrinol. 2016; 174(1): R9–18.
- Volkin D, Yerram N, Ahmed F, et al. Partial adrenalectomy minimizes the need for long-term hormone replacement in pediatric patients with pheochromocytoma and von Hippel-Lindau syndrome. J Pediatr Surg. 2012; 47(11): 2077–2082.
- Otto M, Dzwonkowski J. Adrenal-preserving surgery of adrenal tumours. Endokrynol Pol. 2015; 66(1): 80–96.
- Biteman BR, Randall JA, Brody F. Laparoscopic bilateral cortical-sparing adrenalectomy for pheochromocytoma. Surg Endosc. 2016; 30(12): 5622–5623.
- Nockel P, El Lakis M, Gaitanidis A, et al. Preoperative genetic testing in pheochromocytomas and paragangliomas influences the surgical approach and the extent of adrenal surgery. Surgery. 2018; 163(1): 191–196.
- Machens A, Dralle H. Advances in risk-oriented surgery for multiple endocrine neoplasia type 2. Endocr Relat Cancer. 2018; 25(2): T41–T52.
- Cavallaro G, Letizia C, Polistena A, et al. Laparoscopic adrenal-sparing surgery: personal experience, review on technical aspects. Updates Surg. 2011; 63(1): 35–38.
- Toutounchi S, Pogorzelski R, Wołoszko T, et al. Adrenal-sparing surgery for a hormonally active tumor — a single-center experience. Endokrynol Pol. 2020; Ahead of print.
- Lee JE, Curley SA, Gagel RF, et al. Cortical-sparing adrenalectomy for patients with bilateral pheochromocytoma. Surgery. 1996; 120(6): 1064–70; discussion 1070.
- Neumann HPH, Tsoy U, Bancos I, et al. International Bilateral-Pheochromocytoma-Registry Group. Comparison of Pheochromocytoma-Specific Morbidity and Mortality Among Adults With Bilateral Pheochromocytomas Undergoing Total Adrenalectomy vs Cortical-Sparing Adrenalectomy. JAMA Netw Open. 2019; 2(8): e198898.
- Fallon SC, Feig D, Lopez ME, et al. The utility of cortical-sparing adrenalectomy in pheochromocytomas associated with genetic syndromes. J Pediatr Surg. 2013; 48(6): 1422–1425.
