First reported case of intradural extramedullary epithelioid hemangioma of the cauda equina: resection of an extremely rare vascular neoplasm. Illustrative case
- 1Department of Neurosurgery, Medical College of Georgia at Augusta University, Augusta.
- 2Department of Pathology, Medical College of Georgia at Augusta University, Augusta, Georgia.
Abstract
Background
Intradural extramedullary (IDEM) tumors of the cauda equina are most often meningiomas, nerve sheath tumors, and myxopapillary ependymomas; purely vascular IDEM lesions are exceptionally rare. Fewer than 30 IDEM hemangiomas have been reported, mostly cavernous or capillary. Epithelioid hemangioma (EH), a benign vascular neoplasm arising in soft tissue and bone, has not previously been reported at the cauda equina.Observations
A 63-year-old male with prior cutaneous melanoma presented with 3 months of progressive left L5-distribution radicular pain. MRI showed a 1.0 × 2.0-cm well-circumscribed IDEM mass at L4-5 with left L5-S1 foraminal stenosis. Under intraoperative ultrasound localization and continuous multimodal neurophysiological monitoring, en bloc gross-total resection was achieved through L4-5 laminectomy. Histopathological analysis revealed a well-circumscribed vascular neoplasm with lobular endothelial architecture and no cytological atypia; immunohistochemical analysis (ERG, CD31, and CD34 positivity; HHV8, inhibin, and melanocytic marker negativity) established the diagnosis of EH. At the 7-week follow-up, gross-total resection was confirmed on postoperative MRI.Lessons
To the authors' knowledge, this is the first reported intradural EH at any spinal level. Recognition of epithelioid endothelial morphology combined with a comprehensive vascular and melanocytic immunohistochemistry panel is essential for diagnosis and separates EH from more common intradural vascular differentials. https://thejns.org/doi/abs/10.3171/CASE26857.
Related
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- Spinal subependymoma mimicking an intradural extramedullary tumor: illustrative case.
- A Rare Case of Distant Intradural Extramedullary Thoracic Metastasis from an Intracranial Solitary Fibrous Tumor
- High-grade thoracic intradural extramedullary solitary fibrous tumor: A case report and literature review.
- Anterior mediastinal epithelioid hemangioendothelioma associated with myelolipoma and extramedullary hematopoiesis mimicking a mature teratoma: A radiologic-pathologic correlation.
BibTeX
@article{baker2026first,
title = {{First reported case of intradural extramedullary epithelioid hemangioma of the cauda equina: resection of an extremely rare vascular neoplasm. Illustrative case}},
author = {Al Baker and Sam Granelli and David Baker and William Woodall and Matthew Lee and Bryan Morales-Vargas and Hyunwoo Do},
journal = {Journal of neurosurgery. Case lessons},
year = {2026},
month = nov,
doi = {10.3171/case26857},
url = {https://europepmc.org/article/PMC/PMC13726438},
note = {CC BY-NC-ND},
}
RIS
TY - JOUR
TI - First reported case of intradural extramedullary epithelioid hemangioma of the cauda equina: resection of an extremely rare vascular neoplasm. Illustrative case
AU - Al Baker
AU - Sam Granelli
AU - David Baker
AU - William Woodall
AU - Matthew Lee
AU - Bryan Morales-Vargas
AU - Hyunwoo Do
PY - 2026
DA - 2026/11/02
JO - Journal of neurosurgery. Case lessons
DO - 10.3171/case26857
UR - https://europepmc.org/article/PMC/PMC13726438
AB - Background Intradural extramedullary (IDEM) tumors of the cauda equina are most often meningiomas, nerve sheath tumors, and myxopapillary ependymomas; purely vascular IDEM lesions are exceptionally rare. Fewer than 30 IDEM hemangiomas have been reported, mostly cavernous or capillary. Epithelioid hemangioma (EH), a benign vascular neoplasm arising in soft tissue and bone, has not previously been reported at the cauda equina.Observations A 63-year-old male with prior cutaneous melanoma presented with 3 months of progressive left L5-distribution radicular pain. MRI showed a 1.0 × 2.0-cm well-circumscribed IDEM mass at L4-5 with left L5-S1 foraminal stenosis. Under intraoperative ultrasound localization and continuous multimodal neurophysiological monitoring, en bloc gross-total resection was achieved through L4-5 laminectomy. Histopathological analysis revealed a well-circumscribed vascular neoplasm with lobular endothelial architecture and no cytological atypia; immunohistochemical analysis (ERG, CD31, and CD34 positivity; HHV8, inhibin, and melanocytic marker negativity) established the diagnosis of EH. At the 7-week follow-up, gross-total resection was confirmed on postoperative MRI.Lessons To the authors' knowledge, this is the first reported intradural EH at any spinal level. Recognition of epithelioid endothelial morphology combined with a comprehensive vascular and melanocytic immunohistochemistry panel is essential for diagnosis and separates EH from more common intradural vascular differentials. https://thejns.org/doi/abs/10.3171/CASE26857.
SN - 2694-1902
ER -