Presentation
Left leg pain and numbness with associated saddle numbness.
Patient Data
An 18 x 27 x 19mm intradural mass is seen at the level of the L2 vertebral body, the inferior margin of the lesion at the level of the L2/3 disc. The lesion has heterogeneous internal signal with some cystic change. There is vivid in enhancement, other than in the area of cystic change.
MACROSCOPIC DESCRIPTION: An ovoid fragment of dark brown tissue 27x18x18mm, with a narrow cyclindrical stalk 15x2x2mm, extending to the cranial end, marked with a suture.
MICROSCOPIC DESCRIPTION: The sections show a moderately cellular tumor comprising packets and clusters of cells showing mild nulcear plemorphism and surrounded by moderate amount of eosinophilic granular cytoplasm. There is a conspicuous vascular network surrounding tumor clusters. No mitotic figures are identified. There is no necrosis. The tumor is completely excised.
The tumor cells show strong and diffuse immunostaining for neuroendocrine markers synaptophysin and chromogranin. Most of the tumor clusters show S-100 positive sustentacular cells at the periphery. The tumor cells are negative for EMA. The features are of paraganglioma.
FINAL DIAGNOSIS: Paraganglioma.
Case Discussion
Cauda equina neuroendocrine tumors (aka paragangliomas), although rare, do occur in the cauda equina region and can mimic schwannomas.
Note: These tumors were previously known as spinal paragangliomas however they are molecularly and genetically distinct from paragangliomas seen elsewhere in the body and as such the term spinal (or cauda equina) neuroendocrine tumor is preferred in the 5th edition (2021) WHO classification of CNS tumors.
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