Retrospective Study
Copyright ©The Author(s) 2024. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Jun 6, 2024; 12(16): 2729-2737
Published online Jun 6, 2024. doi: 10.12998/wjcc.v12.i16.2729
Treatment for paraganglioma with stereotactic radiotherapy
Antonio Pontoriero, Paola Critelli, Marco Zeppieri, Filippo Flavio Angileri, Tamara Ius
Antonio Pontoriero, Paola Critelli, Radiation Oncology Unit, Department of Biomedical, Dental Science and Morphological and Functional Images, University of Messina, Messina 98125, Italy
Marco Zeppieri, Department of Ophthalmology, University Hospital of Udine, Udine 33100, Italy
Filippo Flavio Angileri, Neurosurgery Unit, Department of Biomedical, Dental Science and Morphological and Functional Images, University of Messina, Messina 98125, Italy
Tamara Ius, Neurosurgery Unit, Head-Neck and NeuroScience Department, University Hospital of Udine, Udine 33100, Italy
Author contributions: Pontoriero A and Critelli P wrote the outline, did the research and wrote the paper; Angileri FF and Ius T assisted in the writing, editing, and making critical revisions and visualization; Zeppieri M assisted in the conception and design of the study, writing, outline, and completed the English and scientific editing (a native English speaker); and all authors approved the final version of the manuscript.
Institutional review board statement: The surgical techniques are standard and the data collection was retrospective, thus special IRB approval was not required. This study was performed according to the ethical standards of our Institutional Review Board and in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments.
Informed consent statement: Patients were not required to give informed consent to the study because the analysis used anonymous clinical data that were obtained after each patient agreed to treatment by written consent.
Conflict-of-interest statement: The authors declare no conflict of interest.
Data sharing statement: Data is available upon written request.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Marco Zeppieri, MD, PhD, BSc, Department of Ophthalmology, University Hospital of Udine, p.le S. Maria della Misericordia 15, Udine 33100, Italy. markzeppieri@hotmail.com
Received: January 7, 2024
Revised: April 8, 2024
Accepted: April 19, 2024
Published online: June 6, 2024
Processing time: 143 Days and 1.3 Hours
Abstract
BACKGROUND

Paragangliomas (PG) are rare neoplasms of neuroendocrine origin that tend to be highly vascularized, slow-growing, and usually sporadic. To date, common treatment options are surgical resection (SR), with or without radiation therapy (RT), and a watch-and-wait approach.

AIM

To evaluate the local control and effectiveness of exclusive fractionated stereotactic RT (FSRT) treatment in unresectable PG (uPG).

METHODS

We retrospectively evaluated patients with uPG (medically inoperable or refused SR) treated with FSRT with a Cyberknife System (Accuray Incorporated, Sunnyvale, California). Toxicity and initial efficacy were evaluated.

RESULTS

From May 2009 to January 2023, 6 patients with a median age of 68 (range 20-84) were treated with FSRT. The median delivered dose was 21 Gy (range 20-30 Gy) at a median isodose line of 75.5% (range 70%-76%) in 4 fractions (range 3-5 fractions). The median volume was 13.6 mL (range 12.4-65.24 mL). The median cumulative biological effective dose and equivalent dose in 2-Gy fractions were 70 Gy and 37.10 Gy respectively. Site of origin involved were the timpa-nojugular glomus (4/6), temporal bone, and cervical spine. In 1 of the 6 patients, the follow-up was insufficient; 5 of 6 patients showed a 5-year overall survival and 5-year progression-free survival of 100%. We observed negligible toxicities during and after RT. The majority of patients showed stable symptoms during follow-up. Only 1 patient developed spine metastases.

CONCLUSION

Our preliminary results on this small cohort of patients suggest that FSRT could be an effective and safe alternative to SR.

Keywords: Unresectable paraganglioma, Fractionated stereotactic radiation therapy, Cyberknife, Neurosurgery, Metastasis

Core Tip: Paragangliomas (PGs) are rare tumors with neuroendocrine origin. These lesions tend to be highly vascular and embryologically arise from the extra-adrenal autonomic nervous system, located in the thoracic, abdominal, and head-neck regions. PGs are usually sporadic, except in a few cases that are genetically determined by gene mutations. The clinical signs and symptoms include pulsatile tinnitus, headache, hearing loss, vocal fold paresis, vertigo, lower cranial nerve palsies, and tachycardias. Radiographic studies are pathognomonic in diagnosis. Treatment with fractionated stereotactic radiation therapy can be an effective option for these lesions, especially in reserving facial nerve function.