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Biomedicines
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Advances in Pheochromocytoma and Paraganglioma
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Advances in Pheochromocytoma and Paraganglioma

A special issue of Biomedicines (ISSN 2227-9059). This special issue belongs to the section "Neurobiology and Clinical Neuroscience".

Deadline for manuscript submissions: closed (31 October 2022) | Viewed by 10882

Special Issue Editor

Dr. Ivana Jochmanová

E-Mail Website
Guest Editor
1st Department of Internal Medicine, Faculty of Medicine, Pavol Jozef Šafárik University in Košice and Teaching Hospital of Louis Pasteur, Košice, Slovakia
Interests: pheochromocytoma; paraganglioma; signaling pathways; genetics; metabolomics; targeted therapies
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Pheochromocytoma and Paraganglioma (PPGL) are rare neuroendocrine characterized by catecholamine secretion which are responsible for alterations in basically all organ systems. These tumors also bear, so far unpredictable, potential to metastasize, even years after the primary tumor was found and removed. Undiagnosed and untreated PPGLs are associated with high morbidity and mortality, mainly due to cardiovascular complications. Recent years lead to significant progress in the genetics, pathogenesis, and metabolomics of PPGLs, which, in turn, allows for the development of novel diagnostic and therapeutic methods. However, the only curative treatment available is the complete surgical resection, which is not an option for metastatic disease and non-resectable tumors. Therefore, the diagnosis and treatment of metastatic PPGL represents a major health concern and is the main focus of researchers.

The journal Biomedicines is launching the Special Issue on Advances in Pheochromocytoma and Paraganglioma. Reviews or original articles dealing with the genetic, biochemical, and molecular aspects associated with PPGL, pathogenesis in experimental models and humans, as well as articles providing an up-to-date overview of the use of circulating/tissue biomarkers in early diagnosis and management of PPGL and metastatic disease are particularly welcome. In addition, we warmly invite you to submit articles reporting on evidence and expectations from innovative and personalized treatments.

Dr. Ivana Jochmanová
Guest Editor

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Keywords

  • pheochromcytoma
  • paraganglioma
  • metastatic disease
  • genetics
  • pathogenesis
  • diagnosis
  • biomarkers
  • targeted therapy
  • personalized medicine

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Related Special Issue

Published Papers (5 papers)

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Research

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13 pages, 887 KiB  
Article
Hypermetabolism and Substrate Utilization Rates in Pheochromocytoma and Functional Paraganglioma
by Ondřej Petrák, Denisa Haluzíková, Judita Klímová, Matěj Zítek, Robert Holaj, David Michalský, Květoslav Novák, Radka Petráková-Doležalová, Jan Kvasnička, Thi Minh Phuong Nikrýnová Nguyen, Zuzana Krátká, Martin Matoulek, Jiří Widimský, Jr. and Tomáš Zelinka
Biomedicines 2022, 10(8), 1980; https://doi.org/10.3390/biomedicines10081980 - 16 Aug 2022
Cited by 3 | Viewed by 1631
Abstract
The overproduction of catecholamines in pheochromocytoma/paraganglioma (PPGL) induces a hypermetabolic state. The aim of this study was to evaluate the incidence of a hypermetabolic state and differences in substrate metabolism in consecutive PPGL patients divided by catecholamine phenotype. Resting energy expenditure (REE) and [...] Read more.
The overproduction of catecholamines in pheochromocytoma/paraganglioma (PPGL) induces a hypermetabolic state. The aim of this study was to evaluate the incidence of a hypermetabolic state and differences in substrate metabolism in consecutive PPGL patients divided by catecholamine phenotype. Resting energy expenditure (REE) and respiratory quotient (RQ) were measured in 108 consecutive PPGL patients and 70 controls by indirect calorimetry. Hypermetabolic state was defined according to the Mifflin St. Jeor Equation as a ratio above 110%. Hypermetabolic state was confirmed in 70% of PPGL patients, regardless of phenotype. Older age, prevalence of diabetes mellitus and arterial hypertension were correlated with hypermetabolic PPGL as compared to normometabolic form. Analysis according to overproduced catecholamine showed differences in VCO2 (p < 0.05) and RQ (p < 0.01) and thus different substate metabolism between phenotypes in hypermetabolic form of PPGL. Lipid utilization was higher in the adrenergic phenotype (p = 0.001) and positively associated with the percentage of REE ratio (R = 0.48, p < 0.001), whereas the noradrenergic phenotype preferentially oxidizes carbohydrates (P = 0.001) and is correlated with the percentage of REE ratio (R = 0.60, p < 0.001). Hypermetabolic state in PPGL is a common finding in both catecholamine phenotypes. Hypermetabolic PPGL patients are older and suffer more from diabetes mellitus and arterial hypertension. Under basal conditions, the noradrenergic type preferentially metabolizes carbohydrates, whereas the adrenergic phenotype preferentially metabolizes lipids. Full article
(This article belongs to the Special Issue Advances in Pheochromocytoma and Paraganglioma)
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10 pages, 844 KiB  
Article
From SGAP-Model to SGAP-Score: A Simplified Predictive Tool for Post-Surgical Recurrence of Pheochromocytoma
by Mirko Parasiliti-Caprino, Fabio Bioletto, Chiara Lopez, Martina Bollati, Francesca Maletta, Marina Caputo, Valentina Gasco, Antonio La Grotta, Paolo Limone, Giorgio Borretta, Marco Volante, Mauro Papotti, Anna Pia, Massimo Terzolo, Mario Morino, Barbara Pasini, Franco Veglio, Ezio Ghigo, Emanuela Arvat and Mauro Maccario
Biomedicines 2022, 10(6), 1310; https://doi.org/10.3390/biomedicines10061310 - 3 Jun 2022
Cited by 3 | Viewed by 1748
Abstract
A reliable prediction of the recurrence risk of pheochromocytoma after radical surgery would be a key element for the tailoring/personalization of post-surgical follow-up. Recently, our group developed a multivariable continuous model that quantifies this risk based on genetic, histopathological, and clinical data. The [...] Read more.
A reliable prediction of the recurrence risk of pheochromocytoma after radical surgery would be a key element for the tailoring/personalization of post-surgical follow-up. Recently, our group developed a multivariable continuous model that quantifies this risk based on genetic, histopathological, and clinical data. The aim of the present study was to simplify this tool to a discrete score for easier clinical use. Data from our previous study were retrieved, which encompassed 177 radically operated pheochromocytoma patients; supervised regression and machine-learning techniques were used for score development. After Cox regression, the variables independently associated with recurrence were tumor size, positive genetic testing, age, and PASS. In order to derive a simpler scoring system, continuous variables were dichotomized, using > 50 mm for tumor size, ≤ 35 years for age, and ≥ 3 for PASS as cut-points. A novel prognostic score was created on an 8-point scale by assigning 1 point for tumor size > 50 mm, 3 points for positive genetic testing, 1 point for age ≤ 35 years, and 3 points for PASS ≥ 3; its predictive performance, as assessed using Somers’ D, was equal to 0.577 and was significantly higher than the performance of any of the four dichotomized predictors alone. In conclusion, this simple scoring system may be of value as an easy-to-use tool to stratify recurrence risk and tailor post-surgical follow-up in radically operated pheochromocytoma patients. Full article
(This article belongs to the Special Issue Advances in Pheochromocytoma and Paraganglioma)
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15 pages, 662 KiB  
Article
Influence of Receptor Polymorphisms on the Response to α-Adrenergic Receptor Blockers in Pheochromocytoma Patients
by Annika M. A. Berends, Mathieu S. Bolhuis, Ilja M. Nolte, Edward Buitenwerf, Thera P. Links, Henri J. L. M. Timmers, Richard A. Feelders, Elisabeth M. W. Eekhoff, Eleonora P. M. Corssmit, Peter H. Bisschop, Harm R. Haak, Ron H. N. van Schaik, Samira el Bouazzaoui, Bob Wilffert and Michiel N. Kerstens
Biomedicines 2022, 10(4), 896; https://doi.org/10.3390/biomedicines10040896 - 13 Apr 2022
Cited by 1 | Viewed by 2321
Abstract
Background: Presurgical treatment with an α-adrenergic receptor blocker is recommended to antagonize the catecholamine-induced α-adrenergic receptor mediated vasoconstriction in patients with pheochromocytoma or sympathetic paraganglioma (PPGL). There is, however, a considerable interindividual variation in the dose-response relationship regarding the magnitude of blood pressure [...] Read more.
Background: Presurgical treatment with an α-adrenergic receptor blocker is recommended to antagonize the catecholamine-induced α-adrenergic receptor mediated vasoconstriction in patients with pheochromocytoma or sympathetic paraganglioma (PPGL). There is, however, a considerable interindividual variation in the dose-response relationship regarding the magnitude of blood pressure reduction or the occurrence of side effects. We hypothesized that genetically determined differences in α-adrenergic receptor activity contribute to this variability in dose-response relationship. Methods: Thirty-one single-nucleotide polymorphisms (SNPs) of the α1A, α1B, α1D adrenoreceptor (ADRA1A, ADRA1B, ADRA1D) and α2A, α2B adrenoreceptor (ADRA2A, ADRA2B) genes were genotyped in a group of 116 participants of the PRESCRIPT study. Haplotypes were constructed after determining linkage disequilibrium blocks. Results: The ADRA1B SNP rs10515807 and the ADRA2A SNPs rs553668/rs521674 were associated with higher dosages of α-adrenergic receptor blocker (p < 0.05) and with a higher occurrence of side effects (rs10515807) (p = 0.005). Similar associations were found for haplotype block 6, which is predominantly defined by rs10515807. Conclusions: This study suggests that genetic variability of α-adrenergic receptor genes might be associated with the clinically observed variation in beneficial and adverse therapeutic drug responses to α-adrenergic receptor blockers. Further studies in larger cohorts are needed to confirm our observations. Full article
(This article belongs to the Special Issue Advances in Pheochromocytoma and Paraganglioma)
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12 pages, 393 KiB  
Article
Gene Profile of Adipose Tissue of Patients with Pheochromocytoma/Paraganglioma
by Judita Klímová, Miloš Mráz, Helena Kratochvílová, Zdeňka Lacinová, Květoslav Novák, David Michalský, Jan Kvasnička, Robert Holaj, Denisa Haluzíková, Radka Petráková Doležalová, Matěj Zítek, Zuzana Krátká, Veronika Todorovová, Jiří Widimský, Jr., Martin Haluzík, Tomáš Zelinka and Ondřej Petrák
Biomedicines 2022, 10(3), 586; https://doi.org/10.3390/biomedicines10030586 - 2 Mar 2022
Cited by 3 | Viewed by 2290
Abstract
Background: Brown adipose tissue (BAT) is a therapeutic target to combat obesity and related disorders. Pheochromocytoma and functional paraganglioma (PPGL) are associated with activated BAT due to catecholamine excess. Our aim was to evaluate BAT activity by gene profile and assess its relation [...] Read more.
Background: Brown adipose tissue (BAT) is a therapeutic target to combat obesity and related disorders. Pheochromocytoma and functional paraganglioma (PPGL) are associated with activated BAT due to catecholamine excess. Our aim was to evaluate BAT activity by gene profile and assess its relation to clinical characteristics and overproduced catecholamine. Methods: mRNA expression of 15 genes in subcutaneous adipose tissue (SAT) and visceral adipose tissue (VAT) was measured via RT-PCR in 25 patients with PPGL and 14 controls undergoing cholecystectomy. Results: We found in VAT of PPGL higher expression of UCP1 (p < 0.001), CEBPB, PPARGC1A (both p < 0.001), PRDM16 (p = 0.069) and DIO2 (p = 0.005). UCP1 expression correlated only with norepinephrine levels and its metabolite. UCP1 expression, among others, correlated negatively with BMI, age and positively with HDLc levels. Dominance of BAT or BeAT markers was not assessed in PPGL. In SAT of PPGL, we found higher expression of ADRB3, CIDEA (both p < 0.05), and PPARGC1A (p = 0.001), but not UCP1. Conclusion: We demonstrate signs of UCP1-dependent norepinephrine-induced thermogenesis connected with higher expression of DIO2, PPARGC1A, CEBPB and PRDM16 in retroperitoneal VAT of PPGL and its relations to circulating HDLc and triglycerides levels. However, no direct relationship with increased basal energy metabolism measured by calorimetry was found. Full article
(This article belongs to the Special Issue Advances in Pheochromocytoma and Paraganglioma)
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Review

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13 pages, 478 KiB  
Review
Clinical and Pathological Tools for Predicting Recurrence and/or Metastasis in Patients with Pheochromocytoma and Paraganglioma
by Chiara Bima, Fabio Bioletto, Chiara Lopez, Martina Bollati, Stefano Arata, Matteo Procopio, Iacopo Gesmundo, Ezio Ghigo, Mauro Maccario and Mirko Parasiliti-Caprino
Biomedicines 2022, 10(8), 1813; https://doi.org/10.3390/biomedicines10081813 - 28 Jul 2022
Cited by 4 | Viewed by 2115
Abstract
Pheochromocytomas and paragangliomas are endocrine tumors belonging to the family of neural crest cell-derived neoplasms. They have an extremely variable clinical course, characterized by a non-negligible percentage of relapse and/or metastasis after radical surgery. To date, there are no reliable methods to predict [...] Read more.
Pheochromocytomas and paragangliomas are endocrine tumors belonging to the family of neural crest cell-derived neoplasms. They have an extremely variable clinical course, characterized by a non-negligible percentage of relapse and/or metastasis after radical surgery. To date, there are no reliable methods to predict the metastatic potential of these neoplasms, despite several clinical, molecular, and histopathological factors that have been extensively studied in the literature as predictors of the recurrence and/or metastasis in these neoplasms with different performances and results. In this review, we aimed to discuss and analyze the most important clinical and histopathological tools for predicting recurrence risk in patients affected by pheochromocytomas or paragangliomas. Thus, we compared the main available predictive models, exploring their applications in stratifying patients’ risks. In conclusion, we underlined the importance of simple and validated tools to better define disease aggressiveness and establish tailored patients’ treatments and follow-ups. Full article
(This article belongs to the Special Issue Advances in Pheochromocytoma and Paraganglioma)
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