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New Insights into Pheochromocytoma and Paraganglioma
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New Insights into Pheochromocytoma and Paraganglioma

A special issue of Cancers (ISSN 2072-6694). This special issue belongs to the section "Clinical Research of Cancer".

Deadline for manuscript submissions: closed (31 January 2026) | Viewed by 3629

Special Issue Editor

Dr. Wolfgang Raber

E-Mail Website
Guest Editor
Department of Medicine III, Medical University of Vienna, Vienna, Austria
Interests: endocrinology; adrenal; pheochromocytoma; paraganglioma; clinical research; diagnosis; long-term follow-up; genetics

Special Issue Information

Dear Colleagues,

The clinical care of patients with pheochromocytoma and paraganglioma, known together as PPGL, has been constantly improving in recent years, based on sophisticated biochemical diagnosis, functional imaging, or genetic analyses to detect germline pathogenic variants. All of these methods, however, impose significant economic burdens and may not be universally available.

Given the low annual incidence of PPGL (0.04-0.66 per 100.000), centers have to serve a population of 1.5 million to diagnose, treat, and follow 10 new patients per year at best. Available retrospective studies from single centers often suffer from a small number and/or incomplete characterization of patients or insufficient follow-up; multicenter studies follow non-uniform protocols as to the selection, clinical, biochemical, radiological, and genetic characterization or follow-up of patients.

The scope of this Special Issue of Cancers aims to include studies on biochemical (not necessarily chromatography/mass spectrometry), genetic (including neuroendocrine panels), or functional imaging procedures for diagnosis and follow-up, methods preferably approved by national bodies or international regulations, on genotype–phenotype interactions of hereditary compared to sporadic PPGL or research concerning long-term prognostic data based on pertinent guidelines (1). Multicenter cooperations are most welcome, but should include desirable standards (2). Authors are encouraged to submit their work in accordance with guidelines enhancing the quality and transparency of health research (https://www.equator-network.org/reporting-guidelines/stard/) in order to avoid potential statistical errors.

Dr. Wolfgang Raber
Guest Editor

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as communications are invited. For planned papers, a title and short abstract (about 250 words) can be sent to the Editorial Office for assessment.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Cancers is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2900 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • pheochromocytoma
  • paraganglioma
  • diagnosis
  • genetics
  • imaging
  • long-term follow-up
  • standards

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Published Papers (4 papers)

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12 pages, 1158 KB  
Article
Symptomatic Pheochromocytoma: A Risk Model
by María Consuelo Muñoz, Beatriz Febrero, Miriam Abellán and José Manuel Rodríguez
Cancers 2026, 18(3), 528; https://doi.org/10.3390/cancers18030528 - 6 Feb 2026
Viewed by 513
Abstract
Background/Objectives: Pheochromocytoma (PHEO) is increasingly detected incidentally or through genetic screening; however, predictors of symptomatic presentation and its perioperative impact remain unclear. We aimed to quantify the prevalence of symptomatic PHEO, identify associated factors, compare perioperative outcomes, and develop a predictive nomogram. [...] Read more.
Background/Objectives: Pheochromocytoma (PHEO) is increasingly detected incidentally or through genetic screening; however, predictors of symptomatic presentation and its perioperative impact remain unclear. We aimed to quantify the prevalence of symptomatic PHEO, identify associated factors, compare perioperative outcomes, and develop a predictive nomogram. Methods: We retrospectively analyzed patients diagnosed and/or operated on for PHEO at a tertiary referral center between 1984 and 2021. Associations with symptomatic presentation were assessed using univariate and multivariable logistic regression analyses. A nomogram was constructed based on independent predictors and evaluated using receiver operating characteristic (ROC) analysis and the area under the curve (AUC). Results: Among 173 patients (mean age 44.4 ± 15.8 years; 53.2% women), 67.1% were symptomatic. In multivariable analysis, male sex (odds ratio [OR] 0.33; p = 0.023) and the presence of a germline mutation (OR 0.15; p = 0.004) were associated with a lower likelihood of symptoms, whereas a noradrenergic secretion profile was associated with a higher likelihood (OR 12.73; p = 0.02). Symptomatic patients had higher rates of intraoperative (OR 2.60; p = 0.032) and postoperative complications (OR 3.09; p = 0.04). The nomogram incorporating sex, genetic status, and noradrenergic profile demonstrated moderate discrimination (AUC 0.799; 95% confidence interval 0.722–0.877; p < 0.001). Conclusions: Symptomatic PHEO is associated with sex, genetic status, and noradrenergic secretion profile and is linked to increased perioperative morbidity. A simple nomogram based on readily available variables may help estimate individual risk and support perioperative management. Full article
(This article belongs to the Special Issue New Insights into Pheochromocytoma and Paraganglioma)
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13 pages, 466 KB  
Article
Organizational Resources in Rare Cancer Outcomes: Survival Analysis After Surgery for Pheochromocytoma and Paraganglioma
by Kelvin Memeh, Sara Abou Azar, Nicholas R. Suss and Tanaz M. Vaghaiwalla
Cancers 2025, 17(23), 3884; https://doi.org/10.3390/cancers17233884 - 4 Dec 2025
Viewed by 742
Abstract
Background: For very-rare cancers such as pheochromocytoma and paraganglioma (PPGL), center-level case volume is uniformly low, rendering the traditional volume–outcome paradigm uninformative. This study examines whether cancer programs’ institutional resources, after adjusting for tumor-specific case volume, impact overall survival (OS) after surgery. [...] Read more.
Background: For very-rare cancers such as pheochromocytoma and paraganglioma (PPGL), center-level case volume is uniformly low, rendering the traditional volume–outcome paradigm uninformative. This study examines whether cancer programs’ institutional resources, after adjusting for tumor-specific case volume, impact overall survival (OS) after surgery. Methods: The 2004–2021 National Cancer Database was queried for patients with a diagnosis of PPGL with malignant potential. Demographics, clinicopathologic characteristics, socioeconomic status, and treatment and survival variables—together with program resource tier (high resource = Academic/Research + Comprehensive Community Cancer Programs; low resource = Community Cancer + Integrated Network Programs), were extracted. IPW-Cox proportional hazard model and survival analysis were performed. Results: 1306 patients were identified, of whom 1066 (81.6%) were treated at high-resource programs. Mean age was 59.0 years and 55.1% were female (n = 719). Median follow-up was 61.7 months (maximum 207 months). Mortality was 28.3% (n = 278). Age, race, median income, tumor size, and surgical approach did not differ by resource tier. Patients treated at high- vs. low-resource programs differed by Charlson– Deyo score (p = 0.008), gender (p = 0.033), insurance status (p = 0.004), and distance traveled to facility (p < 0.001). On adjusted survival analysis, treatment at a high-resource program was associated with improved OS (HR = 0.64, p = 0.043) and a mean survival advantage of 23 months (p = 0.009) vs. a low-resource program. Age (HR = 1.03), tumor size >10 cm (HR = 4.18), and metastasis (HR = 4.17) independently predicted worse OS. Conclusions: Despite uniformly low PPGL case volumes nationally, treatment at high-resource cancer programs was associated with a 23-month longer mean survival and a 36% lower risk of death compared with low-resource cancer programs. Further studies are needed to identify the specific institutional factors that drive this survival advantage in rare cancers. Full article
(This article belongs to the Special Issue New Insights into Pheochromocytoma and Paraganglioma)
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16 pages, 630 KB  
Article
Metastatic Pheochromocytoma/Paraganglioma: Diagnostic Performance of Functional Imaging (18F-Fluoro-L-DOPA-, 68Ga-DOTA- and 18F-Fluoro-Deoxyglucose-Based PET/CT) and of 123I-MIBG Scintigraphy in 57 Patients and 527 Controls During Long-Term Follow-Up
by Andreas Scheuba, Oana Cristina Kulterer, Reinhard Lehner, Mateja Rybiczka-Tešulov, Harald Esterbauer and Wolfgang Raber
Cancers 2025, 17(23), 3855; https://doi.org/10.3390/cancers17233855 - 30 Nov 2025
Cited by 1 | Viewed by 1132
Abstract
Background: Large-scale data on the diagnostic performance of functional imaging in metastatic pheochromocytoma/paraganglioma (PPGL) are scarce. Objective: To analyze the diagnostic accuracy of functional imaging for the assessment of metastases during long-term follow-up (FU). Design: Retrospective cohort study, 1991–2025. Setting [...] Read more.
Background: Large-scale data on the diagnostic performance of functional imaging in metastatic pheochromocytoma/paraganglioma (PPGL) are scarce. Objective: To analyze the diagnostic accuracy of functional imaging for the assessment of metastases during long-term follow-up (FU). Design: Retrospective cohort study, 1991–2025. Setting: Referral center. Outcomes: Sensitivity and specificity of 123MIBG-, 18F-DOPA-, 68GaDOTA-based and 18FDG PET/CT. Patients: Patients with metastatic PPGL and without PPGL, ≥1 functional imaging prior to first diagnosis and/or during FU and FU ≥ 3 months. Results: 59 123MIBG-, 101 18F-DOPA-, 11 18FDG- and 74 68GaDOTA-based PET/CT were performed in 57 patients with metastatic PPGL and 37 123MIBG-, 323 18F-DOPA-, 259 18FDG- and 641 68GaDOTA-based imaging in 527 patients without PPGL. FU was 11.6 ± 11.4 and 5.1 ± 4.7 years, respectively. Sensitivity for the detection of all metastases (total cohort) by patient-based analysis was comparable between 18F-DOPA (77%), both 68GaDOTA-based tracers combined (67%) and 123MIBG (72%); lesion-based analysis was better for 18F-DOPA (94%) than for 68GaDOTA (85%) and 123MIBG (67%). Specificity (patient- and lesion-based) of 18F-DOPA vs. 68GaDOTA (96–99%) was comparable and better than 123MIBG (73%) and 18FDG (75%). Conclusions: Sensitivity of 18F-DOPA was superior to 68GaDOTA for the detection of all and bone metastases in the total cohort and in patients with PCC, that of 68GaDOTA was better for bone metastases with PGL, specificity was comparable and for both was better than for 18FDG and 123MIBG. Given the beneficial pharmaceutical properties and favorable diagnostic performance, 18F-DOPA may be a good alternative to 68GaDOTA-based tracers for the functional imaging of metastatic PPGL. Full article
(This article belongs to the Special Issue New Insights into Pheochromocytoma and Paraganglioma)
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20 pages, 1460 KB  
Systematic Review
Surgical Approaches and Perioperative Outcomes in Mediastinal Paragangliomas: A 20-Year Comprehensive Systematic Review
by Nicola Rotolo, Giorgia Cerretani, Sabrina Casagrande, Elisa Nardecchia, Elena Asteggiano, Alberto Colombo, Luca Filipponi, Filippo Piacentino, Schiorlin Ilaria and Federico Fontana
Cancers 2026, 18(3), 486; https://doi.org/10.3390/cancers18030486 - 1 Feb 2026
Viewed by 781
Abstract
Background: Mediastinal paragangliomas (MPs) are rare, highly vascular neuroendocrine tumors whose surgical resection is the gold standard but carries a high risk of perioperative complications due to the MPs’ proximity to major cardiovascular structures with potential life-threatening hemorrhage. Due to their rarity, the [...] Read more.
Background: Mediastinal paragangliomas (MPs) are rare, highly vascular neuroendocrine tumors whose surgical resection is the gold standard but carries a high risk of perioperative complications due to the MPs’ proximity to major cardiovascular structures with potential life-threatening hemorrhage. Due to their rarity, the literature consists primarily of case reports. Our systematic review aims to synthesize the evidence from the last two decades to propose a standardized, multidisciplinary approach to the diagnosis and surgical management of MPs. Methods: A systematic literature review was conducted from 2005 to 2025. Studies reporting on surgically resected adult mediastinal paragangliomas were included. Patient demographic data, diagnostic workup, surgical approaches, and outcomes were extracted and analyzed descriptively. Results: Analysis of 79 patients from 75 papers revealed a median age of 50 years (female predominance of 62%). Most tumors were in the middle mediastinum (51.9%). Sternotomy was the most common surgical approach (44.3%), with cardiopulmonary bypass utilized in 27.8% of cases. Postoperative complications occurred in 28% of patients, with left vocal cord palsy (12.7%) being most frequent. The median follow-up was 12 months. All percentages refer to the number of patients. Conclusions: Surgical removal is the standard of care for curative treatment of MPs. However, surgical treatment requires meticulous planning within a multidisciplinary team to reduce the risk of perioperative complications. The choice of surgical approach—open, minimally invasive, or with circulatory support—depends on tumor site, size, and vascular involvement. This review consolidates existing evidence of MPs’ surgical management, aiming to mitigate the significant risks associated with surgery. Lifelong follow-up is essential due to the potential for recurrence. Full article
(This article belongs to the Special Issue New Insights into Pheochromocytoma and Paraganglioma)
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