Cauda equina neuroendocrine tumors: an 11-year retrospective report on clinicopathological characteristics and...

Cauda equina neuroendocrine tumors (CENETs), formerly referred to as cauda equina paragangliomas, are uncommon neuroendocrine neoplasms with distinct biological and genetic features, located in the cauda equina or filum terminale region. This study examined the clinicoradiological characteristics, immunohistochemical profile, and management follow-up of ten CENET cases.

All cases identified as spinal paraganglioma or CENET were retrieved from the dataset of spinal tumors diagnosed between April 2014 and April 2025 in the Department of Pathology at our hospital and reviewed.

Were included 10 cases of CENETs, accounting for 2.2% of all spinal tumors. Mean age at diagnosis was 64.7 years, with a sex ratio of 1. All patients had low back pain and varying degrees of radicular impairment. Magnetic Resonance Imaging and intraoperative appearance indicated vascular, well-circumscribed intradural (mean diameter 22.9 mm), extramedullary tumors of the cauda equina (80%) or conus terminalis (20%), suggestive of either schwannoma or ependymoma. Intraoperatively, a serpentine dilated vessel was typically found at the cephalad part of the tumor. Gross total resection of the tumor was achieved in all cases. All cases were positive for synaptophysin, chromogranin A, cytokeratin AE1/AE3, and NSE, while negative for GFAP, GATA3, Olig2, and EMA. All exhibited positivity for S100 in sustentacular cells and intense positivity in the gangliocytic component (30%). All tested cases retained SDH-B cytoplasmic staining. No recurrence was observed.

CENETS are rare and often misdiagnosed. Serpentine dilated vessel identification at the tumor's cephalad part during surgery may be indicative, and gross total resection be curative.