Surgical treatment of a clival-C2 atypical teratoid/rhabdoid tumor

Abstract

The authors present the case of en bloc resection of a clival-C2 atypical teratoid/rhabdoid tumor. These aggressive lesions of early childhood generally occur in the cerebellum or cerebral hemispheres. This 7-year-old boy presented with pain on turning his head and was found to have a clival-C2 mass. A metastatic workup was negative for disseminated disease. A transoral biopsy procedure revealed an atypical teratoid/rhabdoid tumor on histological examination. The tumor was resected via a transoral approach, and the patient's spine was stabilized with posterior instrumented fusion from the occiput to C-5. Postoperatively, the patient underwent 16 months of chemotherapy along with 6 weeks of overlapping radiation therapy. Twenty-seven months after the initial surgery he presented with leg pain and was found to have a solitary metastatic lesion at the conus medullaris. There was no local recurrence at the clivus. The conus tumor was resected and found to be consistent with the primary tumor. Several months later the patient presented with disseminated intrathecal disease and ultimately died 42 months after the initial resection.

FIG. 1

Preoperative sagittal precontrast (A) and postcontrast (B) MR images demonstrating an enhancing clival-based tumor with mass effect on the brainstem. Postoperative sagittal MR image (C) revealing resection of the mass. Preoperative CT scan (D) demonstrating tumor and bony erosion of the clivus and dens, and postoperative CT scan (E) showing removal of the tumor, dens, most of the body of C-2 and the inferior half of the clivus.

FIG. 2

Photomicrographs showing spindle and epithelioid tumor cells adjacent to bone (A), tumor cells with strong expression of cytokeratins (AE1.3, B), epithelial membrane antigen focally (C), and diffuse loss of nuclear INI1 expression in large neoplastic cells but retained expression in intratumor vasculature and lymphocytes (D). H & E (A), original magnification × 100.

FIG. 3

Intraoperative and postoperative images showing the transoral approach to the clival mass (A), the mass itself (B), the anterior dura and ligaments (C), the resected tumor specimen (D), post–posterior fusion and alignment in the halo (E), and the anterior surgical incision 6 months after surgery (F).

FIG. 4

Preoperative sagittal MR images, precontrast (A) and postcontrast (B), demonstrating an enhancing mass at L-1. Postoperative sagittal MR image (C) confirming resection of the mass.