Cargando…
Changes in Clinical Presentation and Perioperative Management of Pheochromocytomas and Paragangliomas: A Four-Decade Experience in a Academic Center
Objective: Latin American reports on pheochromocytomas and paragangliomas (PPGL) are scarce. Recent studies have shown changes in both clinical presentation and management of these patients. We aimed to assess the main characteristics of PPGL patients in a single academic center over the last four d...
Autores principales: | , , , , , , , , , , , , , , , , , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Oxford University Press
2021
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8090006/ http://dx.doi.org/10.1210/jendso/bvab048.158 |
_version_ | 1783687177555673088 |
---|---|
author | Uslar, Thomas Francisco, Ignacio San Olmos, Roberto Ignacio Macchiavello, Stefano Pietro Zuñiga, Alvaro Rojas, Pablo Garrido, Marcelo Huete, Alvaro Medez, Gonzalo Cifuentes, Joaquin Castro, Fernando Zemelman, Jose Tomas Olivari, Daniela Fardella, Carlos E Arteaga, Eugenio Ruiz-Tagle, Jose Miguel Dominguez Valdes, Gloria Tagle, Rodrigo Baudrand, Rene |
author_facet | Uslar, Thomas Francisco, Ignacio San Olmos, Roberto Ignacio Macchiavello, Stefano Pietro Zuñiga, Alvaro Rojas, Pablo Garrido, Marcelo Huete, Alvaro Medez, Gonzalo Cifuentes, Joaquin Castro, Fernando Zemelman, Jose Tomas Olivari, Daniela Fardella, Carlos E Arteaga, Eugenio Ruiz-Tagle, Jose Miguel Dominguez Valdes, Gloria Tagle, Rodrigo Baudrand, Rene |
author_sort | Uslar, Thomas |
collection | PubMed |
description | Objective: Latin American reports on pheochromocytomas and paragangliomas (PPGL) are scarce. Recent studies have shown changes in both clinical presentation and management of these patients. We aimed to assess the main characteristics of PPGL patients in a single academic center over the last four decades. Experimental design: Cohort study. Patients and methods: Demographic, clinical, biochemical, genetic and perioperative data from 105 PPGL patients were retrospectively and prospectively collected over the 1980–2019 period. Patients were categorized into four groups (14 patients in the 1st, 25 patients in the 2nd, 27 patients in the 3th and 39 patients in the 4th decade) according to the date of diagnosis. Results: The mean age at diagnosis was 46±19 years, and the tumor size was 5.3±2.2 cm, female gender was 63%, bilateral tumor of 15%, paragangliomas 9% and metastatic disease in 15%. The aforementioned parameters remained stable across the four decades. During the study period we observed significant increases in doxazosin dosing (2.7±2.6 mg vs. 8.0±4.5 p<0.003) and laparoscopic procedures (28% vs. 84% p<0.001) along with a decrease in the length of hospital stay (10.0±8.9 vs. 3.8±1.7 days p=0.007). Among the 24 genetic tests performed, we identified 59% germline mutations. The most frequent mutations were RET (18%) and SDHX (18%), followed by VHL (14%), MAX (5%) and NF1 (4%). Notably, in the last decade we observed a dramatic increase in the proportion of incidental PPGL diagnosis (0% vs. 53% p<0.001) and genetic testing analyses (0 vs. 19 p<0.001). When comparing incidental diagnosis (n=25) versus clinically suspicious cases(n=50), incidentalomas had fewer adrenergic symptoms (38 vs. 62%; p<0.001), and lower rates of hypertension (64 vs. 80%; p=0.01), hypertension crises (28 vs. 44%; p=0.02), functionality (79 vs. 100%; p=0.01) and total catecholamines and/or metanephrine levels (8.4 vs. 12.5 fold above the upper normal limit; p=0.04). Conclusions: The implementation of a multidisciplinary program increased diagnosis and genetic testing and also optimized anesthesia and surgical procedure, translating into a notorious improvement in perioperative outcomes. In addition, we observed a change in the clinical presentation of PPGL in recent decades with a marked increase in incidental cases, which highlights the importance of early diagnosis and treatment. |
format | Online Article Text |
id | pubmed-8090006 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | Oxford University Press |
record_format | MEDLINE/PubMed |
spelling | pubmed-80900062021-05-06 Changes in Clinical Presentation and Perioperative Management of Pheochromocytomas and Paragangliomas: A Four-Decade Experience in a Academic Center Uslar, Thomas Francisco, Ignacio San Olmos, Roberto Ignacio Macchiavello, Stefano Pietro Zuñiga, Alvaro Rojas, Pablo Garrido, Marcelo Huete, Alvaro Medez, Gonzalo Cifuentes, Joaquin Castro, Fernando Zemelman, Jose Tomas Olivari, Daniela Fardella, Carlos E Arteaga, Eugenio Ruiz-Tagle, Jose Miguel Dominguez Valdes, Gloria Tagle, Rodrigo Baudrand, Rene J Endocr Soc Adrenal Objective: Latin American reports on pheochromocytomas and paragangliomas (PPGL) are scarce. Recent studies have shown changes in both clinical presentation and management of these patients. We aimed to assess the main characteristics of PPGL patients in a single academic center over the last four decades. Experimental design: Cohort study. Patients and methods: Demographic, clinical, biochemical, genetic and perioperative data from 105 PPGL patients were retrospectively and prospectively collected over the 1980–2019 period. Patients were categorized into four groups (14 patients in the 1st, 25 patients in the 2nd, 27 patients in the 3th and 39 patients in the 4th decade) according to the date of diagnosis. Results: The mean age at diagnosis was 46±19 years, and the tumor size was 5.3±2.2 cm, female gender was 63%, bilateral tumor of 15%, paragangliomas 9% and metastatic disease in 15%. The aforementioned parameters remained stable across the four decades. During the study period we observed significant increases in doxazosin dosing (2.7±2.6 mg vs. 8.0±4.5 p<0.003) and laparoscopic procedures (28% vs. 84% p<0.001) along with a decrease in the length of hospital stay (10.0±8.9 vs. 3.8±1.7 days p=0.007). Among the 24 genetic tests performed, we identified 59% germline mutations. The most frequent mutations were RET (18%) and SDHX (18%), followed by VHL (14%), MAX (5%) and NF1 (4%). Notably, in the last decade we observed a dramatic increase in the proportion of incidental PPGL diagnosis (0% vs. 53% p<0.001) and genetic testing analyses (0 vs. 19 p<0.001). When comparing incidental diagnosis (n=25) versus clinically suspicious cases(n=50), incidentalomas had fewer adrenergic symptoms (38 vs. 62%; p<0.001), and lower rates of hypertension (64 vs. 80%; p=0.01), hypertension crises (28 vs. 44%; p=0.02), functionality (79 vs. 100%; p=0.01) and total catecholamines and/or metanephrine levels (8.4 vs. 12.5 fold above the upper normal limit; p=0.04). Conclusions: The implementation of a multidisciplinary program increased diagnosis and genetic testing and also optimized anesthesia and surgical procedure, translating into a notorious improvement in perioperative outcomes. In addition, we observed a change in the clinical presentation of PPGL in recent decades with a marked increase in incidental cases, which highlights the importance of early diagnosis and treatment. Oxford University Press 2021-05-03 /pmc/articles/PMC8090006/ http://dx.doi.org/10.1210/jendso/bvab048.158 Text en © The Author(s) 2021. Published by Oxford University Press on behalf of the Endocrine Society. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs licence (http://creativecommons.org/licenses/by-nc-nd/4.0/ (https://creativecommons.org/licenses/by-nc-nd/4.0/) ), which permits non-commercial reproduction and distribution of the work, in any medium, provided the original work is not altered or transformed in any way, and that the work is properly cited. For commercial re-use, please contact journals.permissions@oup.com |
spellingShingle | Adrenal Uslar, Thomas Francisco, Ignacio San Olmos, Roberto Ignacio Macchiavello, Stefano Pietro Zuñiga, Alvaro Rojas, Pablo Garrido, Marcelo Huete, Alvaro Medez, Gonzalo Cifuentes, Joaquin Castro, Fernando Zemelman, Jose Tomas Olivari, Daniela Fardella, Carlos E Arteaga, Eugenio Ruiz-Tagle, Jose Miguel Dominguez Valdes, Gloria Tagle, Rodrigo Baudrand, Rene Changes in Clinical Presentation and Perioperative Management of Pheochromocytomas and Paragangliomas: A Four-Decade Experience in a Academic Center |
title | Changes in Clinical Presentation and Perioperative Management of Pheochromocytomas and Paragangliomas: A Four-Decade Experience in a Academic Center |
title_full | Changes in Clinical Presentation and Perioperative Management of Pheochromocytomas and Paragangliomas: A Four-Decade Experience in a Academic Center |
title_fullStr | Changes in Clinical Presentation and Perioperative Management of Pheochromocytomas and Paragangliomas: A Four-Decade Experience in a Academic Center |
title_full_unstemmed | Changes in Clinical Presentation and Perioperative Management of Pheochromocytomas and Paragangliomas: A Four-Decade Experience in a Academic Center |
title_short | Changes in Clinical Presentation and Perioperative Management of Pheochromocytomas and Paragangliomas: A Four-Decade Experience in a Academic Center |
title_sort | changes in clinical presentation and perioperative management of pheochromocytomas and paragangliomas: a four-decade experience in a academic center |
topic | Adrenal |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8090006/ http://dx.doi.org/10.1210/jendso/bvab048.158 |
work_keys_str_mv | AT uslarthomas changesinclinicalpresentationandperioperativemanagementofpheochromocytomasandparagangliomasafourdecadeexperienceinaacademiccenter AT franciscoignaciosan changesinclinicalpresentationandperioperativemanagementofpheochromocytomasandparagangliomasafourdecadeexperienceinaacademiccenter AT olmosrobertoignacio changesinclinicalpresentationandperioperativemanagementofpheochromocytomasandparagangliomasafourdecadeexperienceinaacademiccenter AT macchiavellostefanopietro changesinclinicalpresentationandperioperativemanagementofpheochromocytomasandparagangliomasafourdecadeexperienceinaacademiccenter AT zunigaalvaro changesinclinicalpresentationandperioperativemanagementofpheochromocytomasandparagangliomasafourdecadeexperienceinaacademiccenter AT rojaspablo changesinclinicalpresentationandperioperativemanagementofpheochromocytomasandparagangliomasafourdecadeexperienceinaacademiccenter AT garridomarcelo changesinclinicalpresentationandperioperativemanagementofpheochromocytomasandparagangliomasafourdecadeexperienceinaacademiccenter AT huetealvaro changesinclinicalpresentationandperioperativemanagementofpheochromocytomasandparagangliomasafourdecadeexperienceinaacademiccenter AT medezgonzalo changesinclinicalpresentationandperioperativemanagementofpheochromocytomasandparagangliomasafourdecadeexperienceinaacademiccenter AT cifuentesjoaquin changesinclinicalpresentationandperioperativemanagementofpheochromocytomasandparagangliomasafourdecadeexperienceinaacademiccenter AT castrofernando changesinclinicalpresentationandperioperativemanagementofpheochromocytomasandparagangliomasafourdecadeexperienceinaacademiccenter AT zemelmanjosetomas changesinclinicalpresentationandperioperativemanagementofpheochromocytomasandparagangliomasafourdecadeexperienceinaacademiccenter AT olivaridaniela changesinclinicalpresentationandperioperativemanagementofpheochromocytomasandparagangliomasafourdecadeexperienceinaacademiccenter AT fardellacarlose changesinclinicalpresentationandperioperativemanagementofpheochromocytomasandparagangliomasafourdecadeexperienceinaacademiccenter AT arteagaeugenio changesinclinicalpresentationandperioperativemanagementofpheochromocytomasandparagangliomasafourdecadeexperienceinaacademiccenter AT ruiztaglejosemigueldominguez changesinclinicalpresentationandperioperativemanagementofpheochromocytomasandparagangliomasafourdecadeexperienceinaacademiccenter AT valdesgloria changesinclinicalpresentationandperioperativemanagementofpheochromocytomasandparagangliomasafourdecadeexperienceinaacademiccenter AT taglerodrigo changesinclinicalpresentationandperioperativemanagementofpheochromocytomasandparagangliomasafourdecadeexperienceinaacademiccenter AT baudrandrene changesinclinicalpresentationandperioperativemanagementofpheochromocytomasandparagangliomasafourdecadeexperienceinaacademiccenter |