J Korean Soc Radiol.  2015 Jul;73(1):40-44. 10.3348/jksr.2015.73.1.40.

Spinal Cerebrospinal Fluid Seeding of a Clival Chordoma: A Case Report

• Baek SH
• Yu IK
• Kim SM
• Park KS
• Son HJ

• KMID: 1823921
• DOI: http://doi.org/10.3348/jksr.2015.73.1.40

Abstract

Chordomas originate from remnants of the embryonic notochord and account for < 2% of all malignant bone tumors. Chordomas have a high rate of local recurrence. However, spinal cerebrospinal fluid (CSF) seeding of a chordoma is extremely rare. Here, we present a very rare case of clival chordoma with spinal seeding. Radiologists should consider spinal CSF seeding of a clival chordoma, particularly when accompanied by signs of dural perforation or caudal extension.

MeSH Terms

Cerebrospinal Fluid*
Chordoma*
Cranial Fossa, Posterior
Notochord
Recurrence

Figure

• 

  Fig. 1 18-year-old woman with clival chordoma. A-C. Sagittal brain MR images (T2-weighted, T1-weighted, fat-suppressed contrast-enhanced T1-weighted) shows high T2 signal intensity, contour-bulging heterogeneously enhancing mass (arrow), involving entire clivus, anterior perimedullary, lower prepontine cistern, with posterior compression to the brain stem. D. Micrograph shows physaliferous (having bubbles or vacuoles) cells with extensive myxoid stroma. The cells (arrow) are very large, with vacuolated cytoplasm, prominent vesicular nucleus and rare mitotic figures (hematoxylin and eosin, × 400).

• 

  Fig. 2 18-year-old woman with clival chordoma. A, B. Sagittal spine MR images (T2-weighted, fat-suppressed contrast-enhanced T1-weighted) show multifocal small nodular enhancing lesions (arrows) in the pial surfaces of distal conus, and lumbosacral spinal cord. C. Axial fat-suppressed contrast-enhanced T1-weighted images show multiple enhancing nodules (arrows) in the pial surfaces of spinal cord. D. On microscopic images, irregular clusters of neoplastic cells showing small bland nuclei and well-defined, homogeneous and eosinophilic cytoplasm are present in a mucoid matrix (hematoxylin and eosin, × 100). It is the typical histologic findings of chordoma.

Reference

1. Dahlin DC. Bone tumors: general aspects and data on 6221 cases. 3rd ed. Springfield: Charles C Thomas;1978. p. 329–343.

2. Bergh P, Kindblom LG, Gunterberg B, Remotti F, Ryd W, Meis-Kindblom JM. Prognostic factors in chordoma of the sacrum and mobile spine: a study of 39 patients. Cancer. 2000; 88:2122–2134.

3. Wang CC, James AE Jr. Chordoma: brief review of the literature and report of a case with widespread metastases. Cancer. 1968; 22:162–167.

4. McPherson CM, Suki D, McCutcheon IE, Gokaslan ZL, Rhines LD, Mendel E. Metastatic disease from spinal chordoma: a 10-year experience. J Neurosurg Spine. 2006; 5:277–280.

5. Zulch KJ. Brain tumors. Their biology and pathology. 3rd ed. Berlin Heidelberg: New York: Tokyo: Springer-Verlag;1986. p. 476–479.

6. Holzner H. [Unusual metastasis of chordoma]. Zentralbl Allg Pathol. 1954; 92:12–18.

7. Asano S, Kawahara N, Kirino T. Intradural spinal seeding of a clival chordoma. Acta Neurochir (Wien). 2003; 145:599–603. discussion 603

8. Krol G, Sze G, Arbit E, Marcove R, Sundaresan N. Intradural metastases of chordoma. AJNR Am J Neuroradiol. 1989; 10:193–195.

9. Stough DR, Hartzog JT, Fisher RG. Unusual intradural spinal metastasis of a cranial chordoma. Case report. J Neurosurg. 1971; 34:560–562.

10. Holton JL, Steel T, Luxsuwong M, Crockard HA, Revesz T. Skull base chordomas: correlation of tumour doubling time with age, mitosis and Ki67 proliferation index. Neuropathol Appl Neurobiol. 2000; 26:497–503.