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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: 31 December 2025 | Viewed by 513

Special Issue Editor


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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

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Keywords

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

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Published Papers (2 papers)

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Research

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 94
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
Viewed by 181
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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