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Open Access 08-06-2024 | Original Article

The role of the adrenalectomy in the management of pheochromocytoma: the experience of a Portuguese referral center

Authors: Inês Costa Carvalho, Miguel V. B. Machado, João P. Morais, Filipa Carvalho, Elisabete Barbosa, José Barbosa

Published in: Endocrine

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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.
Literature
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go back to reference L. Amar, C. Lussey-Lepoutre, J.W. Lenders, J. Djadi-Prat, P.F. Plouin, O. Steichen, Management of endocrine disease: Recurrence or new tumors after complete resection of pheochromocytomas and paragangliomas: A systematic review and meta-analysis. Eur. J. Endocrinol. 175, 135–45 (2016). https://doi.org/10.1530/EJE-16-0189CrossRef L. Amar, C. Lussey-Lepoutre, J.W. Lenders, J. Djadi-Prat, P.F. Plouin, O. Steichen, Management of endocrine disease: Recurrence or new tumors after complete resection of pheochromocytomas and paragangliomas: A systematic review and meta-analysis. Eur. J. Endocrinol. 175, 135–45 (2016). https://​doi.​org/​10.​1530/​EJE-16-0189CrossRef
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Metadata
Title
The role of the adrenalectomy in the management of pheochromocytoma: the experience of a Portuguese referral center
Authors
Inês Costa Carvalho
Miguel V. B. Machado
João P. Morais
Filipa Carvalho
Elisabete Barbosa
José Barbosa
Publication date
08-06-2024
Publisher
Springer US
Published in
Endocrine
Print ISSN: 1355-008X
Electronic ISSN: 1559-0100
DOI
https://doi.org/10.1007/s12020-024-03916-y
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