The Role of the Adrenalectomy in the Management of Pheochromocytoma: the Experience of a Portuguese Referral Center

Overview

Journal Endocrine

Specialty Endocrinology

Date 2024 Jun 7

PMID 38849646

Authors

Ines Costa Carvalho

Miguel V B Machado

Joao P Morais

Filipa Carvalho

Elisabete Barbosa

Jose Barbosa

Affiliations

Soon will be listed here.

Abstract

Purpose: Pheochromocytoma is a rare neuroendocrine tumor. Despite the low incidence, these tumors are of indisputable importance. This study aimed to analyze the management of pheochromocytoma in a referral center, with an emphasis on the minimally invasive adrenalectomy, which is the preferred therapeutic approach.

Methods: A retrospective analysis was performed on a cohort of patients diagnosed with pheochromocytoma who underwent adrenalectomy between January 2013 and December 2022. Clinical data including demographics, timelines, symptomatology, comorbidities, biochemical markers, genetic testing, surgical details, and follow-up outcomes, were collected and analyzed.

Results: The cohort included 44 patients, predominantly women (52.27%), with a median age of 53.39 years (range 13-83). Most of patients exhibited paroxysmal symptoms suggesting catecholamine excess. Documented hypertension was the most frequent (86.36%), along with glucose anomalies (40.01%) and anxiety disorder (31.82%). Genetic testing was performed in 36 (81.81%) patients and 14 (38.88%) revealed a positive result, predominantly RET pathogenic variant. Laparoscopic surgery was performed in 34 (79.07%) patients, showing significantly shorter operative time (2.5 h vs. 4.25 h, t-test p < 0,001) and fewer complications (23.53% vs 77.78%, p = 0.008). Postoperative complications occurred in 36.36% of the patients, mostly mild (grade I, 56.25%), with no mortality. SDHB pathogenic variant correlated with both recurrent and metastatic disease (p = 0.006). One-year follow-up reported 9.09% recurrence and 6.82% metastasis.

Conclusions: Adrenalectomy demonstrated a high safety and effectiveness. This study exhibited a higher rate of genetic testing referral than other studies. Despite past advances, there is still a need for further studies to establish protocols and evaluate new techniques.

References

Colton M, Tompkins K, ODonnell E, Aisner D, Lieu C, Konnick E . Case of Metastatic Pheochromocytoma and Meningiomas in a Patient With Lynch Syndrome. JCO Precis Oncol. 2022; 6:e2100251. PMC: 9848592. DOI: 10.1200/PO.21.00251. View

Soares N, Pacheco M, de Sousa M, Matos M, Ferreira S . Pheochromocytoma: a retrospective study from a single center. Endocr Regul. 2021; 55(1):16-21. DOI: 10.2478/enr-2021-0003. View

Lima Jr J, Scalissi N, de Oliveira K, Lindsey S, Olivati C, Ferreira E . Germline genetic variants in pheochromocytoma/paraganglioma: single-center experience. Endocr Oncol. 2023; 3(1):e220091. PMC: 10388674. DOI: 10.1530/EO-22-0091. View

Eisenhofer G, Prejbisz A, Peitzsch M, Pamporaki C, Masjkur J, Rogowski-Lehmann N . Biochemical Diagnosis of Chromaffin Cell Tumors in Patients at High and Low Risk of Disease: Plasma versus Urinary Free or Deconjugated -Methylated Catecholamine Metabolites. Clin Chem. 2018; 64(11):1646-1656. DOI: 10.1373/clinchem.2018.291369. View

Mineur Y, Cahuzac E, Mose T, Bentham M, Plantenga M, Thompson D . Interaction between noradrenergic and cholinergic signaling in amygdala regulates anxiety- and depression-related behaviors in mice. Neuropsychopharmacology. 2018; 43(10):2118-2125. PMC: 6098039. DOI: 10.1038/s41386-018-0024-x. View

Amar L, Lussey-Lepoutre C, Lenders J, Djadi-Prat J, Plouin P, Steichen O . MANAGEMENT OF ENDOCRINE DISEASE: Recurrence or new tumors after complete resection of pheochromocytomas and paragangliomas: a systematic review and meta-analysis. Eur J Endocrinol. 2016; 175(4):R135-45. DOI: 10.1530/EJE-16-0189. View

Parasiliti-Caprino M, Lucatello B, Lopez C, Burrello J, Maletta F, Mistrangelo M . Predictors of recurrence of pheochromocytoma and paraganglioma: a multicenter study in Piedmont, Italy. Hypertens Res. 2019; 43(6):500-510. DOI: 10.1038/s41440-019-0339-y. View

Sbardella E, Cranston T, Isidori A, Shine B, Pal A, Jafar-Mohammadi B . Routine genetic screening with a multi-gene panel in patients with pheochromocytomas. Endocrine. 2017; 59(1):175-182. DOI: 10.1007/s12020-017-1310-9. View

Farrugia F, Martikos G, Tzanetis P, Charalampopoulos A, Misiakos E, Zavras N . Pheochromocytoma, diagnosis and treatment: Review of the literature. Endocr Regul. 2017; 51(3):168-181. DOI: 10.1515/enr-2017-0018. View

10.

Taieb D, Nolting S, Perrier N, Fassnacht M, Carrasquillo J, Grossman A . Management of phaeochromocytoma and paraganglioma in patients with germline SDHB pathogenic variants: an international expert Consensus statement. Nat Rev Endocrinol. 2023; 20(3):168-184. DOI: 10.1038/s41574-023-00926-0. View

11.

Ando Y, Ono Y, Sano A, Fujita N, Ono S, Tanaka Y . Clinical characteristics and outcomes of pheochromocytoma crisis: a literature review of 200 cases. J Endocrinol Invest. 2022; 45(12):2313-2328. DOI: 10.1007/s40618-022-01868-6. View

12.

Neumann H, Young Jr W, Eng C . Pheochromocytoma and Paraganglioma. N Engl J Med. 2019; 381(6):552-565. DOI: 10.1056/NEJMra1806651. View

13.

Araujo-Castro M, Minguez Ojeda C, Garcia Centeno R, Lopez-Garcia M, Lamas C, Hanzu F . Glycemic disorders in patients with pheochromocytomas and sympathetic paragangliomas. Endocr Relat Cancer. 2022; 29(12):645-655. DOI: 10.1530/ERC-22-0218. View

14.

Ebbehoj A, Stochholm K, Jacobsen S, Trolle C, Jepsen P, Robaczyk M . Incidence and Clinical Presentation of Pheochromocytoma and Sympathetic Paraganglioma: A Population-based Study. J Clin Endocrinol Metab. 2021; 106(5):e2251-e2261. DOI: 10.1210/clinem/dgaa965. View

15.

Nolting S, Bechmann N, Taieb D, Beuschlein F, Fassnacht M, Kroiss M . Personalized Management of Pheochromocytoma and Paraganglioma. Endocr Rev. 2021; 43(2):199-239. PMC: 8905338. DOI: 10.1210/endrev/bnab019. View

16.

Su T, Yang Y, Jiang L, Xie J, Zhong X, Wu L . SDHB immunohistochemistry for prognosis of pheochromocytoma and paraganglioma: A retrospective and prospective analysis. Front Endocrinol (Lausanne). 2023; 14:1121397. PMC: 10061060. DOI: 10.3389/fendo.2023.1121397. View

17.

Rodriguez K, Harris K, Singla N . Adrenal pheochromocytoma in a patient with Lynch Syndrome. Urol Case Rep. 2022; 42:102015. PMC: 9073213. DOI: 10.1016/j.eucr.2022.102015. View

18.

Lenders J, Kerstens M, Amar L, Prejbisz A, Robledo M, Taieb D . Genetics, diagnosis, management and future directions of research of phaeochromocytoma and paraganglioma: a position statement and consensus of the Working Group on Endocrine Hypertension of the European Society of Hypertension. J Hypertens. 2020; 38(8):1443-1456. PMC: 7486815. DOI: 10.1097/HJH.0000000000002438. View

19.

Iglesias P, Santacruz E, Garcia-Sancho P, Marengo A, Guerrero-Perez F, Pian H . Pheochromocytoma: A three-decade clinical experience in a multicenter study. Rev Clin Esp (Barc). 2021; 221(1):18-25. DOI: 10.1016/j.rceng.2019.12.011. View

20.

Lorenz K, Langer P, Niederle B, Alesina P, Holzer K, Nies C . Surgical therapy of adrenal tumors: guidelines from the German Association of Endocrine Surgeons (CAEK). Langenbecks Arch Surg. 2019; 404(4):385-401. DOI: 10.1007/s00423-019-01768-z. View