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case_images: Pre-operative MRI of the spine: Sagittal T1 post-gadolinium contrast (A), sagittal T2 (B), axial T1 post-contrast (C), and axial DWI (D) demonstrating an intradural extramedullary mass (orange arrow) caudal and ventral to the conus medullaris (green arrow) at L1-L4 with T1 and T2 Iso-intensity, heterogeneous enhancement, and diffusion restriction (blue arrow). Note the largely distended bladder due to the mass effect and compression on the conus medullaris affecting bladder emptying (blue star).
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case_images: Subsequent total-spine MRI the following day revealed a large, intradural extramedullary mass caudal and ventral to the cauda equina spanning from L1-L4, measuring 13 x 17 x 53 mm3 (Figure 1A-D).
case_images: Intraoperative pictures. (A) Dorsal approach posterior L1-4 laminectomy with exposure of underlying dura (caudal is to the right). (B) Following dural opening, Penfield micro-dissectors are used to dissect the cauda equina and nerve roots ventral to the mass exposing a purple, friable mass. (C) Following exposure, the mass was centrally debulked with bipolar cautery and suction. The cauda equina nerve roots can be seen dorsally displaced. (D) After subtotal resection of spinal mass with residual mass ventral to the conus. (E) Expansile duraplasty was performed with Durerepair for dural closure. (F) The L1-L4 spinous processes and laminae were then reattached with KLS martin plates and screws to complete the laminoplasty.
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case_images: Following satisfactory hemostasis, primary dural closure was attempted but unsuccessful due to continued dorsal herniation of the cauda equina and nerve roots (Figure 2D-F).
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case_images: Intraoperative pictures. (A) Dorsal approach posterior L1-4 laminectomy with exposure of underlying dura (caudal is to the right). (B) Following dural opening, Penfield micro-dissectors are used to dissect the cauda equina and nerve roots ventral to the mass exposing a purple, friable mass. (C) Following exposure, the mass was centrally debulked with bipolar cautery and suction. The cauda equina nerve roots can be seen dorsally displaced. (D) After subtotal resection of spinal mass with residual mass ventral to the conus. (E) Expansile duraplasty was performed with Durerepair for dural closure. (F) The L1-L4 spinous processes and laminae were then reattached with KLS martin plates and screws to complete the laminoplasty.
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case_images: Following satisfactory hemostasis, primary dural closure was attempted but unsuccessful due to continued dorsal herniation of the cauda equina and nerve roots (Figure 2D-F).
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case_images: (A) H&E, 400x magnification: Intraoperative touch prep demonstrating dis-cohesive tumor cells with irregular nuclear contours, vesicular chromatin, and scant cytoplasm. (B) H&E, 400x magnification: Sheets of tumor cells with pleomorphic nuclei, prominent nucleoli, and scant cytoplasm. (C) H&E, 200x magnification: Small-round blue cell neoplasm with broad regions of necrosis. (D) INI1 immunohistochemical stain, 200x: Loss of nuclear expression of INI1 within tumor cells with preservation of expression within internal control (endothelial cells).
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case_images: Final histopathology of the tumor demonstrated poorly differentiated and discohesive cells with high nuclear to cytoplasmic ratios, vesicular chromatin, and prominent nucleoli (Figure 3A-D).
case_images: Post-operative MRI of the spine: Sagittal T1 post-gadolinium contrast (A), sagittal T2 (B), axial T1 post-contrast (C), and axial T2 (D) demonstrating persistent intradural extramedullary mass postoperatively (orange arrow) after incomplete resection with expected internal hemorrhagic changes and decreased mass effect on the conus medullaris (green arrow).
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case_images: She was noted to be ambulatory with moderate assistance. 6 weeks after surgery, post-operative MRI spine confirmed residual tumor along the conus with no new enhancing spinal lesions (Figure 4A-D).
case_images: Repeat MRI spine 5 months post-adjuvant chemoradiation: Sagittal T1 post-gadolinium contrast (A), sagittal T2 (B), axial DWI (C), and axial T2 (D) demonstrating reduced tumor size (orange arrow) without new enhancing lesions and decompressed conus medullaris (green arrow) and cauda equina (blue arrow).
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case_images: ACNS0333 guidelines over a 6-month period. At her most recent follow-up over 2 years after surgery, the patient's cancer remains in remission with age-appropriate developmental milestones (Figure 5).