Presentation
Progressive bilateral upper and lower limb weakness and loss of sensation for 6 weeks. Associated with urinary retention and bowel fallout. No history of trauma or fever.
Patient Data
Expansile Intra-medullary lesion extending from C3 to C7 level with peri-tumoral cysts and hemosiderin staining on T2 WI. Associated long segment cervical and thoracic loculated syringohydromyelia. No drop metastasis or leptomeningeal enhancement. Massively distended urinary bladder. Incidental T7 vertebral body hemangioma and adnexal cyst.
Normal imaged cervical spine with no degenerative changes or destructive bone lesion. No prevertebral soft tissue thickening. Imaged airways and skull base are normal.
The patient had surgery with histology:
Macroscopy:
The specimen consists of multiple friable grey-brown tissues measuring 14 mm x 6 mm x 4 mm.
Microscopy:
The sections show multiple fragments of lesional tissue demonstrating a cellular ependymal tumor with moderate cytologic atypia, abundant perivascular rosettes, and numerous true ependymal rosettes. Non-palisading necrosis is present. 3 mitoses per 10 high fields are seen. No evidence of microvascular proliferation, palisading necrosis, severe nuclear pleomorphism, or atypical mitotic figures.
Immunohistochemistry:
EMA: Widespread dot-like positivity in tumor cells.
D240: isolated dot-like positivity in tumor cells.
GFAP: weak cytoplasmic positivity
S100: diffuse cytoplasmic activity within the tumor.
Ki-2%.
Pathological diagnosis-ependymoma WHO grade 2.
Case Discussion
The case illustrates an intramedullary lesion. The differential on imaging includes spinal astrocytoma, hemangioblastoma, and paraganglioma. Pathology confirmed an ependymoma of the cord.
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