GKRS is a safe and effective treatment modality for the management of ELSTs, particularly in residual or recurrent disease, offering excellent radiological control and symptom stabilization as both a primary and adjunctive treatment option.
Key Findings
Results
Gamma knife radiosurgery achieved stable disease in all five patients with endolymphatic sac tumors, with no increase in tumor size on follow-up MRI.
Five patients with radiologically diagnosed ELSTs were treated with GKRS between 2012 and 2023.
Minimum follow-up duration was 1 year.
Follow-up MRIs showed stable disease in all patients.
Both primary (upfront) and secondary (adjuvant) GKRS cases demonstrated radiological control.
Methods
Two of five patients received primary GKRS while three received secondary GKRS following prior surgical resection.
The cohort of five patients was split between upfront and adjuvant treatment approaches.
Secondary GKRS was used in cases of residual or recurrent disease after surgical resection.
This retrospective analysis spans approximately 11 years (2012–2023) at a single institution.
Treatment planning used frame-based MRI with the Leksell GammaPlan system.
Results
Marginal doses used for GKRS treatment of ELSTs ranged from 20 to 25 Gy across the five patients.
The marginal dose range of 20–25 Gy was applied across both primary and adjuvant GKRS cases.
Treatment was delivered using the Leksell GammaPlan system with frame-based MRI planning.
No procedure-related or radiation-induced complications, including radiation necrosis, were observed.
None of the patients required corticosteroid therapy.
Results
No patients experienced worsening or emergence of new neurological symptoms following GKRS.
Clinical outcomes were assessed through clinical review and follow-up imaging.
All five patients remained clinically stable at follow-up.
No radiation-induced complications were reported in any patient.
Corticosteroid therapy was not required by any patient in the cohort.
Background
ELSTs are rare, locally aggressive tumors often associated with von Hippel-Lindau disease, for which complete surgical excision is challenging due to their location and hypervascularity.
Recurrence following surgery is described as common.
The tumor's hypervascularity contributes to the difficulty of complete surgical excision.
GKRS has emerged as a potential noninvasive treatment modality particularly in recurrent or inoperable cases.
The rarity of ELSTs is reflected in the small case series of five patients over an 11-year single-institution period.
What This Means
This research suggests that a specialized form of radiation treatment called Gamma Knife radiosurgery (GKRS) can effectively control endolymphatic sac tumors (ELSTs), which are rare and aggressive tumors that grow near the inner ear and are often linked to a genetic condition called von Hippel-Lindau disease. Because of where these tumors are located and how blood-rich they are, surgeons often cannot remove them completely, and they tend to come back. This study followed five patients treated at a single hospital over about 11 years, some of whom received GKRS as their first treatment and others who received it after surgery had already been attempted.
This research suggests that GKRS stopped tumor growth in all five patients, with no tumors getting larger on follow-up MRI scans, and no patients developing new or worsening neurological problems. Importantly, no patients experienced complications from the radiation, such as radiation-induced tissue damage (necrosis), and none needed steroid medications to manage side effects. The radiation doses used ranged from 20 to 25 Gray units across patients.
This research suggests that GKRS represents a safe and viable option for managing these difficult tumors, whether used as the main treatment or as a follow-up after surgery. Because ELSTs are so rare, large clinical trials are very difficult to conduct, making this kind of single-institution case series valuable for understanding how patients respond to treatment. The findings support considering GKRS for patients with residual, recurrent, or inoperable ELSTs.
Mahajan V, Mishra S, Garg K, Garg A, Singh M, Chandra P. (2026). Gamma Knife Radiosurgery for Endolymphatic Sac Tumors: A Single-Institution Experience.. Neurology India. https://doi.org/10.4103/neurol-india.Neurol-India-D-25-00986