Update on the Management of Head & Neck Paragangliomas: Papers from the First International Congress, Piacenza, Italy September 20–22, 2023

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

Deadline for manuscript submissions: closed (31 May 2024) | Viewed by 6317

Special Issue Editors


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Guest Editor
Casa di Cura Piacenza, Via Morigi 41, 29121 Piacenza, Italy
Interests: skull base surgery; neuro otology; facial nerve; lower cranial nerves; carotid artery; embolization; head & neck tumors
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Center for Advanced Studies and Technology, G. d’Annunzio University, Via Luigi Polacchi 11, 66100 Chieti, Italy
Interests: paraganglioma; gene-environment interactions; cancer genetics; oncogenesis; cancer pathology; cancer biomarkers

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Cancer Genomics Core-Lab, Istituto Oncologico Veneto IRCCS, Torre della Ricerca, corso Stati Uniti, 4, 35127 Padova, Italy
Interests: genetics; hereditary cancer; paraganglioma/pheochromocytoma

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Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
Interests: pheochromocytoma; paraganglioma; comprehensive genomic profiling; genomic alterations
Special Issues, Collections and Topics in MDPI journals

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Department of Otorhinolaryngology, Charles University, 3rd Faculty of Medicine, University Hospital Kralovske Vinohrady, 10034 Prague, Czech Republic
Interests: otorhinolaryngology; head and neck paraganglioma; SDH genes; germline mutations; genetic counselling; cranial nerve dysfunction

Special Issue Information

Dear Colleagues,

This Special Issue of Cancers will focus on papers from the "First International Congress Update on the Management of HEAD & NECK PARAGANGLIOMAS” (linked to https://paragangliomas.com/), due to take place in Piacenza, Italy on September 20–22, 2023.

Head & neck paragangliomas are hypervascular neural crest tumors that mainly originate from the parasympathetic paraganglia of the lower cranial nerves. These tumors are rare (<0.5% of all head-and-neck tumors), present at any age, peaking between the 4th and 7th decades, with female preference, and tend to grow slowly, but are invasive and radio/chemo-resistant. Depending on the site, they cause serious or even life-threatening complications, including hearing loss, dysphagia, cranial nerve palsy, bradycardia, hemorrhage, infiltration of the skull base and intracranial growth. Metastatic risk is low but never excludable, warranting lifelong follow-up. As with other forms of paraganglioma, head & neck paragangliomas occur in hereditary syndromes linked to predisposing mutations in diverse genes, many of which are implicated in the Krebs cycle and in the hypoxia response. Genetic predisposition cannot be ruled out for apparently sporadic tumors: in fact, at least 35–40% of all head & neck paraganglioma patients carry predisposing gene variants.

Much knowledge about the biological, genetic, pathological, neuroendocrinological and epidemiological characteristics of head & neck paragangliomas has been accumulated in the last two decades, together with significant improvements in diagnostic imaging. This knowledge opens new perspectives on disease etiology, molecular markers, and targeted therapies and directly impacts the management of head and neck paraganglioma patients and their families. However, surgery remains the only effective therapy. Surgical approaches and techniques have also evolved within the last 3 decades, and tumors once considered inoperable can now be safely removed, provided that special expertise is available. Presently, there is a need of improving the dissemination of specialized knowledge on state-of-the-art head & neck paraganglioma surgery, and it is also necessary that surgical and follow-up strategies consider biological, genetic, and pathological data predictive of aggressive disease, synchronous or metachronous tumors, and possible occurrence of familial clusters.

The aim of the Congress, reflected in this Special Issue, is to give an up-to-date overview of the specialized head & neck paraganglioma field. The Congress papers will integrate current scientific developments with surgical management and will provide guidelines for early diagnosis, treatment and follow-up of patients and at-risk relatives.

Prof. Dr. Mario Sanna
Prof. Dr. Renato Mariani-Costantini
Dr. Francesca Schiavi
Prof. Dr. Karel Pacak
Dr. Anasuya Guha
Guest Editors

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Published Papers (5 papers)

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Research

12 pages, 7098 KiB  
Article
Clinical Characteristics and Outcomes of Tympanomastoid Paragangliomas: A Report from Slovenia
by Manja Hribar, Iztok Fošnarič, Aleš Matos, Robert Šifrer, Aleš Grošelj, Maruša Debeljak, Nina Zidar, Primož Strojan and Klemen Jenko
Cancers 2024, 16(18), 3178; https://doi.org/10.3390/cancers16183178 - 17 Sep 2024
Viewed by 489
Abstract
(1) Background: Head and neck paragangliomas are neuroendocrine tumors that typically originate from the parasympathetic nervous system and are predominantly non-secretory. Their clinical manifestations result from their mass effect on the surrounding tissues. The approach to treating these tumors depends on factors such [...] Read more.
(1) Background: Head and neck paragangliomas are neuroendocrine tumors that typically originate from the parasympathetic nervous system and are predominantly non-secretory. Their clinical manifestations result from their mass effect on the surrounding tissues. The approach to treating these tumors depends on factors such as their location, size, impact on adjacent structures, and the patient’s overall health and preferences. (2) Methods: A retrospective analysis of the management of temporal bone paraganglioma classes A and B (according to the modified Fisch classification) was performed at the University Medical Centre, Ljubljana, between 2011 and 2023. (3) Results: We analyzed 23 cases, 19 of which underwent surgery; complete tumor removal was achieved in 18 of them. Four patients were irradiated due to tumor progression to class C. Three of these four patients initially refused surgery and were treated with radiotherapy (RT) 7, 13, and 18 years after diagnosis. In the fourth patient, complete surgical resection was not achieved and she was treated with RT four years after surgery, due to the growth of the tumor to class C. The average follow-up time from diagnosis was 8.9 years (median 6 years; range 1–26 years). (4) Conclusions: The surgical treatment of patients with class A and B paragangliomas is effective and safe. In cases where surgery is refused but the tumor continues to grow to class C, RT is an alternative and efficient method of controlling tumor growth. Full article
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11 pages, 1332 KiB  
Article
The Role of Internal Carotid Artery Stent in the Management of Skull Base Paragangliomas
by Riccardo Di Micco, Rolf Benedikt Salcher, Friedrich Götz, Omar Abu Fares and Thomas Lenarz
Cancers 2024, 16(13), 2461; https://doi.org/10.3390/cancers16132461 - 5 Jul 2024
Viewed by 1117
Abstract
Background: After two decades from its introduction in the lateral skull base paraganglioma surgery, the indications and results of preoperative internal carotid artery stenting should be critically assessed. Materials and Methods: Monocentric retrospective study on 26 patients affected by head and neck paragangliomas [...] Read more.
Background: After two decades from its introduction in the lateral skull base paraganglioma surgery, the indications and results of preoperative internal carotid artery stenting should be critically assessed. Materials and Methods: Monocentric retrospective study on 26 patients affected by head and neck paragangliomas (19 tympanojugular paragangliomas, 4 carotid body paragangliomas, 3 vagal paragangliomas) preoperatively treated with internal carotid artery stents between 2008 and 2023. The preoperative findings, the intraoperative complications and the final surgical results were analyzed. Results: The stent complication rate was less than 3.1%. Self-expanding highly flexible intracranial nitinol stents were applied. In all cases, it was possible to completely mobilize the internal carotid artery and perform a vascular dissection of the tumor. Gross total tumor resection was possible in 85% of cases. The median follow up was 7.83 y (SD +/− 3.93 y). No local recurrence was observed. Conclusions: The preoperative vascular stent facilitates tumor dissection from the internal carotid artery without risk of vascular damage, helping the surgeon to achieve surgical radicality. The vascular stent is indicated in the case of revision surgeries, circumferential involvement of the vessel and in cases with non-insufficient intracerebral crossflow. Procedural complications, temporary antiplatelet therapy and delay of surgery are the limitations of the procedure. Full article
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15 pages, 239 KiB  
Article
Head and Neck Paragangliomas: Overview of Institutional Experience
by Swar N. Vimawala, Alex Z. Graboyes, Bonita Bennett, Maria Bonanni, Aleena Abbasi, Tanaya Oliphant, Michelle Alonso-Basanta, Christopher Rassekh, Debbie Cohen, Jason A. Brant and Yonghong Huan
Cancers 2024, 16(8), 1523; https://doi.org/10.3390/cancers16081523 - 16 Apr 2024
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Abstract
Head and neck paragangliomas (HNPGLs) are rare and have high rates of genetic mutations. We conducted a retrospective review of 187 patients with 296 PGLs diagnosed between 1974 and 2023. The mean age of diagnosis was 48.8 years (range 10 to 82) with [...] Read more.
Head and neck paragangliomas (HNPGLs) are rare and have high rates of genetic mutations. We conducted a retrospective review of 187 patients with 296 PGLs diagnosed between 1974 and 2023. The mean age of diagnosis was 48.8 years (range 10 to 82) with 69.0% female and 26.5% patients with multiple PGLs. Among 119 patients undergoing genetic testing, 70 (58.8%) patients had mutations, with SDHB (30) and SDHD (26) being the most common. The rates of metastasis and recurrence were higher among patients with SDHB mutations or SDHD mutations associated with multiple PGLs. Metabolic evaluation showed elevated plasma dopamine levels were the most common derangements in HNPGL. MRI and CT were the most common anatomic imaging modalities and DOTATATE was the most common functional scan used in this cohort. Most patients (81.5%) received surgery as the primary definitive treatment, while 22.5% patients received radiation treatment, mostly as an adjuvant therapy or for surgically challenging or inoperable cases. Systemic treatment was rarely used in our cohort. Our single-center experience highlights the need for referral for genetic testing and metabolic evaluation and for a team-based approach to improve the clinical outcomes of patients with HNPGLs. Full article
14 pages, 9388 KiB  
Article
Long-Term Surgical Outcome of Class A and B Tympanomastoid Paragangliomas
by Melcol Hailu Yilala, Giuseppe Fancello, Virginia Fancello, Lorenzo Lauda and Mario Sanna
Cancers 2024, 16(8), 1466; https://doi.org/10.3390/cancers16081466 - 11 Apr 2024
Cited by 1 | Viewed by 879
Abstract
Objective: To analyze the long-term facial function as well as overall postoperative condition in surgically treated tympanomastoid PGL patients. Study Design: Retrospective study. Method: The medical records of patients with surgically managed class A and B tympanomastoid PGLs between 1983 and 2023 were [...] Read more.
Objective: To analyze the long-term facial function as well as overall postoperative condition in surgically treated tympanomastoid PGL patients. Study Design: Retrospective study. Method: The medical records of patients with surgically managed class A and B tympanomastoid PGLs between 1983 and 2023 were thoroughly evaluated. Result: Our center has treated a total of 213 cases of tympanomastoid PGL surgically. The mean age of patients was 54, and the male-to-female ratio was 1:6. The most common symptoms at presentation were hearing loss (80%), pulsatile tinnitus (77%), and vertigo (15%). According to the modified Fisch classification, 45% of the cases were classified as class A (A1 and A2), while 55% were classified as class B (B1, B2, and B3). All class A and most class B1 and B2 tumors were removed either with transcanal or retroauricular-transcanal approaches. However, more advanced class B3 lesions were removed with subtotal petrosectomy (SP) along with middle ear obliteration. Facial nerve outcome was excellent in all class A and B cases, while chances of postoperative paresis slightly increased with the size and extent of the tumor (p < 0.05). The hearing outcome is excellent for class A1, A2, B1, and B2 tumors, whereas more advanced class B3 cases have a loss of air conduction (AC) and increased bone conduction (BC) threshold (p < 0.05). Complete surgical removal was achieved in 97% of our cases. The most common late complication was permanent TM perforation (7%), and the recurrence rate was 3%. Conclusions: Tympanomastoid PGL represents the most common neoplasm of the middle ear space. The most frequent presenting symptoms include pulsatile tinnitus and hearing loss, whereas the presence of retrotympanic mass was evident in all cases at the time of initial otoscopic evaluation. Proper documentation of facial function and audiometric evaluation are crucial elements of preoperative workup. The most preferred preoperative radiologic examination is high-resolution computer tomography (HRCT), whereas magnetic resonance imaging (MRI) with or without gadolinium enhancement is reserved for cases with a dilemma of carotid artery or jugular bulb involvement. The main goal of tympanomastoid PGL treatment is complete disease removal with preservation of hearing and facial functions. Surgical treatment remains the preferred treatment modality with the benefits of complete disease removal, lower rate of recurrence and complication, and acceptable postoperative hearing level. Here, we present our 40 years of experience, which, to the very best of our knowledge, is the largest series of tympanomastoid PGL in the English literature. Full article
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9 pages, 344 KiB  
Article
The Additional Value of Somatostatin Receptor Positron Emission Computed Tomography ([68Ga]Ga-DOTATOC PET/CT) Compared with Magnetic Resonance Imaging of the Head and Neck Region in Paraganglioma Patients: A Pilot Study
by Carolijn J. M. de Bresser, Bart-Jeroen Petri, Arthur J. A. T. Braat, Bart de Keizer, Mark J. C. van Treijen, Jan Willem Dankbaar, Frank A. Pameijer, Marius G. J. Kok, Mischa de Ridder, Bernadette P. M. van Nesselrooij, Remco de Bree, Gert J. de Borst and Johannes A. Rijken
Cancers 2024, 16(5), 986; https://doi.org/10.3390/cancers16050986 - 28 Feb 2024
Cited by 1 | Viewed by 944
Abstract
The Dutch guideline for patients suspected of head and neck paragangliomas (HNPGLs) recommends magnetic resonance imaging (MRI) and/or computed tomography (CT) of the head and neck area. Additionally, it suggests considering additional nuclear imaging. The aim of this study was to evaluate the [...] Read more.
The Dutch guideline for patients suspected of head and neck paragangliomas (HNPGLs) recommends magnetic resonance imaging (MRI) and/or computed tomography (CT) of the head and neck area. Additionally, it suggests considering additional nuclear imaging. The aim of this study was to evaluate the outcomes of [68Ga]Ga-DOTATOC PET/CT compared to MRI in patients with suspected HNPGLs and carriers of genetic variations. Methods: In this single-center pilot study, retrospective data were obtained from consecutive patients between 2016 and 2023. Both MRI and [68Ga]Ga-DOTATOC PET/CT were performed within 12 months. The primary outcome was the location of HNPGLs. Results: A total of 25 consecutive patients were included, and 7 patients (28.0%, p = 0.5) showed differences between the imaging modalities, of whom 5 patients had unexpected localizations with additional uptake by somatostatin receptors (SSTR) on the [68Ga]Ga-DOTATOC PET/CT. Conclusions: The authors recommend performing baseline imaging with [68Ga]Ga-DOTATOC PET/CT (if available) in variant carriers and using MRI/CT for follow-up according to the regional protocol, thereby shifting the gold standard for baseline imaging from MRI/CT to [68Ga]Ga-DOTATOC PET/CT. Full article
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