Clinical Characteristics and Outcomes of Tympanomastoid Paragangliomas: A Report from Slovenia

TL;DR

This paper summarizes the treatment and outcomes of a rare type of tumor called tympanomastoid paraganglioma, emphasizing the effectiveness of surgery and radiotherapy.

Contribution

The study provides a detailed clinical overview and treatment approach for paragangliomas in Slovenia, focusing on surgical and radiotherapy outcomes.

Findings

Surgery achieved complete tumor removal in 18 out of 19 operated patients with class A and B paragangliomas.

Radiotherapy was effective in controlling tumor growth in patients who refused surgery or had incomplete resection.

The average follow-up time was 8.9 years, with no mention of tumor recurrence in surgically treated patients.

Abstract

Temporal bone paragangliomas are rare neuroendocrine tumors that typically arise from the parasympathetic nervous system. Since they are not a common pathology in the daily practice of an otorhinolaryngologist, we would like to provide an overview of current treatment strategies for patients with tympanomastoid paragangliomas. In addition, we present our approach for patients with class A and B tympanomastoid paragangliomas and reiterate that surgical therapy is an effective and safe treatment modality. In cases where surgery is refused and the tumor has grown to class C or more, radiotherapy is an alternative method to control tumor growth.

(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.

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