Intradural Prepontine Chordoma in a Child: Case Report and Review of the Pediatric Skull Base Literature

Introduction: Pediatric chordomas are rare, malignant tumors arising from primitive notochord remnants with less than 300 cases reported to date. Further, since the notochord develops into the osseous vertebral column, intracranial intradural chordomas without bony attachments are extremely rare.

Objective: To present a rare case of a pediatric prepontine intradural chordoma and review the literature with consideration for alternative benign notochordal cell tumors

Methods: A retrospective patient chart review was performed in conjunction with a comprehensive PUBMED/MEDLINE literature search to identify all reports of pediatric intracranial intradural chordomas.

Results:

Case Report: A 10-year-old female presented with headaches and gait instability. MRI demonstrated a large, diffusion restricting, minimally enhancing, T1 hypointense, T2 hyperintense mass centered in the prepontine cistern causing mass effect and obstructive hydrocephalus strongly resembling an epidermoid cyst ([Fig. 1]). A right retrosigmoid craniotomy was performed with near total resection ([Fig 2]). She developed a House-Brackmann grade III facial palsy, which thereafter resolved. Pathology demonstrated epithelioid cells with physaliphorous cytoplasm with a rich chondromyxoid matrix. Immunohistochemistry was positive for pan-cytokeratin, EMA, S100, Brachyury and INI-1 ([Fig. 3]) further supporting the diagnosis of chordoma.

Literature Search: Five articles met criteria including five pediatric patients with intradural chordomas without an extraosseous component ([Table 1]). The most common presentation was headaches and the most common location was the prepontine cistern. Three patients (60%) had a single lesion, all of which underwent surgical resection without recurrence. Two patients presented with disseminated disease, one which underwent biopsy and another who underwent subtotal resection and radiation.

Conclusion: Pediatric chordomas without extraosseous components are an anomaly. Despite the aggressive nature of chordomas, in pediatric patients without disseminated disease, it may be reasonable to treat with surgical resection without adjuvant radiation and follow for recurrence. Further research in differentiating benign versus malignant notochordal cell tumors, from a molecular and pathologic standpoint, is needed to guide postoperative treatment.

Fig. 1

Fig. 2

Fig. 3

Table 1 Literature review for all articles depicting intracranial chordomas with a pure intradural component in the pediatric population

Publication History

Article published online:
05 February 2024