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Updated: Aug 16, 2026
Surgical Treatment of an Endolymphatic Sac Tumor
Published on: May 26, 2023
[The jugulotympanic paragangliomas: 41 cases report]
D Q Nguyen1, E Boulat, J Troussier
1CHU Grenoble, Service ORL, F-38043 Grenoble cedex 09, France. DQNGuyen@chu-grenoble.fr
Insights
Radical surgery offers high success rates for jugulotympanic paragangliomas (JTP) with low recurrence. Radiotherapy stabilizes tumors but carries risks, making surgery preferable when feasible for these vascular tumors.
Area of Science:
- Otolaryngology
- Neurosurgery
- Oncology
Background:
- Jugulotympanic paragangliomas (JTP) are the most common middle ear and temporal bone tumors after acoustic neurinoma.
- The optimal management strategy for these vascular tumors remains unclear.
- This study reports institutional experience with JTP management.
Purpose of the Study:
- To evaluate the effectiveness and outcomes of different therapeutic approaches for jugulotympanic paragangliomas.
- To compare surgical, radiotherapeutic, and combined treatment modalities for JTP.
- To inform clinical decision-making for JTP management.
Main Methods:
- Retrospective analysis of 41 patients with JTP treated between 1973 and 1996.
- Classification of tumors based on Fisch staging, with 49% exhibiting intracranial extension.
- Patients were grouped by primary treatment: surgery, radiation therapy, or surgery followed by radiation therapy.
Main Results:
- Surgical tumor removal achieved >95% success with no recurrence at 6-year follow-up, but significant morbidity (cranial nerve injury) was noted.
- Radiotherapy in 75% of cases led to tumor stabilization (5-year follow-up) but posed a risk of radionecrosis.
- One death occurred due to laryngospasm post-surgery.
Conclusions:
- Radical surgery is encouraged for JTP when feasible due to high success rates.
- Radiotherapy is indicated for elderly patients, those at surgical risk, or with extensive tumors, aiming for stabilization.
- Multidisciplinary management and genetic screening for familial cases are crucial for optimal JTP care.
Objective:
The jugulotympanic paragangliomas (JTP) represents the most frequent tumour of the middle ear but also of the temporal bone, after the acoustic neurinoma. The management of these vascular tumours remains uncleared. The purpose of this study was to report our experience about JTP in the CHU of Grenoble.
Materials And Methods:
Retrospective study of 41 patients, between 1973 and 1996. Six stages A, 8 stages B and 27 stages C are reported in whom 20 cases (49%) presented an intracranial extension (classification of Fisch). There were 2 familial cases with multiple localisations, in particular carotid. All the patients were divided in 3 groups: surgery or radiation therapy in first intention, surgery followed by radiation therapy.
Results:
A total tumor removal without recurrence was achieved by surgery in more than 95% of the cases with 6 years follow-up but was associated with significant morbidity (major cranial nerve injury). We noticed one death by laryngospasme (C2Di2 tumour operated by infratemporal A approach). A stabilization of the tumour was obtained with radiotherapy in first intention in 75% of the cases (5 years follow-up) but with a risk of radionecrosis. A revision surgery was necessary in 3 cases.
Conclusion:
The comparaison of our different therapeutic management, surgery (23), radiation therapy (16) or combined (2), encourage us to perform a radical surgery whenever possible. Because of the slow rate of growth, the radiotherapy is indicated for older patients, at risk for surgery or extensive tumors. The objectives of the radiation therapy are to obtain a tumoral stabilization with improvement of the symptoms and low morbidity. The management of this rare pathology must be multidisciplinary. The recent discoveries on genes encoding three succinate dehydrogenase subunits (SDHD, SDHB et SDHC) will allow a genetic detection of asymptomatic case and will define the procedures for their management, coordinated by a national network PGL.NET. A retrospective study could also study the real incidence of familial paragangliomas.