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. 2025 Sep 29:16:1687570.
doi: 10.3389/fendo.2025.1687570. eCollection 2025.

Diagnosis and treatment of paragangliomas and pheochromocytomas: a survey from the Italian Association for Neuroendocrine Tumors

Erica Pietroluongo #  1 , Antonella Lucia Marretta #  2 , Mauro Cives  3 , Elisa Andrini  4 , Leonardo Della Sala  5 , Elisabetta Dell'Unto  6 , Alice Carli  7 , Nevena Mikovic  8 , Ilaria Mascagni  9 , Francesco Panzuto  6 , Salvatore Tafuto  10
Affiliations

Diagnosis and treatment of paragangliomas and pheochromocytomas: a survey from the Italian Association for Neuroendocrine Tumors

Erica Pietroluongo et al. Front Endocrinol (Lausanne). .

Abstract

Background: Pheochromocytomas (PCCs) and paragangliomas (PPGLs) are rare neuroendocrine neoplasms (NENs) with heterogeneous clinical presentations. Given the rarity of PCCs/PPGLs and the paucity of high-level evidence, therapeutic decisions and treatment sequences vary across institutions. This survey explored current diagnostic practices and treatment patterns among Italian healthcare professionals (HCPs) dedicated to NENs.

Methods: An online survey was conducted among Italian HCPs, members of the Italian Association for Neuroendocrine Tumors (ITANET). The survey included 33 questions covering diagnosis, genetic counseling, imaging, and treatment approaches. Responses were collected from December 15, 2023, to May 30, 2024, and analyzed using descriptive statistics to identify trends in clinical practice.

Results: We recorded 80/355 responses from invited HCPs (response rate: 23%). Most HCPs (90%) referred all PCC/PPGL patients for genetic counseling, with 71% adopting gene panels for syndromes like VHL, MEN2, and familial PPGLs. Functional imaging preferences included 68Ga-DOTA-peptide PET/CT (38%), 18F-DOPA PET/CT (26%), and 123I-MIBG scintigraphy (19%). First-line systemic treatments favored somatostatin analogs (39%), clinical trial enrollment (19%), and CVD chemotherapy (15%). Radioligand therapy (RLT) emerged as the preferred second-line treatment (49%). Overall, RLT was perceived as the most effective treatment for achieving objective responses, durable responses, and improving health-related quality of life.

Conclusions: Clinical wisdom rather than formal evidence and guidelines recommendations appears to guide the management of PCC/PPGLs among Italian HCPs. International, multi-institutional clinical trials designed to take into account the rarity of PCCs/PPGLs are needed to generate high-level evidence and provide guidance for standard clinical practice.

Keywords: genetic counseling; imaging; neuroendocrine neoplasms; paragangliomas; pheochromocytomas; treatment.

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Conflict of interest statement

MC has performed consultation for Advanz, Esteve, Harbor BioMed, Harpoon Therapeutics, and has received speaker’s fee from AAA - Novartis and Ipsen. Dr. MC is co-inventor in patent applications filed by Moffitt Cancer Center Tampa, FL, USA, involving adoptive immunotherapy products. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The author(s) declared that they were an editorial board member of Frontiers, at the time of submission. This had no impact on the peer review process and the final decision.

Figures

Figure 1
Figure 1
Demographic and professional characteristics of survey respondents including gender distribution (A), age distribution (B), medical specialties (C) and number of PCC/PGL cases diagnosed in the last 10 years (D).
Figure 2
Figure 2
Preferred first- (A) and second-line therapeutic options (B) for patients with advanced PCCs/PPGLs according to survey respondents.
Figure 3
Figure 3
Reported number of patients with PCCs/PPGLs treated with somatostatin analogs, chemotherapy, radioligand therapy (RLT) and tyrosine-kinase inhibitors (TKIs) in the first- (A) and second-line treatment setting (B).
Figure 4
Figure 4
Perceived safety and efficacy of radioligand therapy (RLT), somatostatin analogs, chemotherapy and other treatments (irrespective of treatment line) according to key domains (possibility to induce tumor shrinkage, duration of response, health-related quality of life (HR QoL).
See this image and copyright information in PMC

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