Intradural extraosseous chordoma in the foramen magnum region

Abstract

Chordomas that are entirely extraosseous and intradural are extremely rare. The tumors described in the literature were observed mostly in the prepontine region. This is the first case reported of an intradural extraosseous chordoma occurring in the foramen magnum region. The tumor was totally excised. The distinction between an intradural extraosseous chordoma and a classic chordoma is important from a clinical viewpoint because of the potential for complete surgical excision and a more benign growth pattern.

Giant ecchordosis physaliphora in an adolescent girl

“…Intradural chordomas almost never have an associated stalk, and, unlike EP, they usually enhance with Gd administration. 5,10,15,16,20,22,24,38 However, there are cases of isolated intradural EP without a stalk, and in some rare cases, an intradural chordoma may not demonstrate enhancement. Another important consideration is the clinical presentation.…”

“…Just like EP, intradural chordomas may have a low MIB-1 index, making it difficult to discern the two based on immunohistochemical means as well. 15,21,25,36 The MRI and CT characteristics may be helpful in distinguishing between the two. Intradural chordomas almost never have an associated stalk, and, unlike EP, they usually enhance with Gd administration.…”

Intradural Retroclival Chordoma

“…Classic chordomas in bone frequently have ill-defined margins, and complete resection is usually not feasible even with extensive surgery because a few tumor cells are often left behind in the bone or dura. In contrast, the intradural type of tumor characteristically has a slower growth pattern, sharply circumscribed margins that allow total excision, and it never metastasizes 6,11,20) . Most patients with skull base chordomas have been treated with a combination of surgery and radiotherapy, although the efficacy of radiosurgery is controversial 21) .…”

“…Including the this case, only 18 previous cases of primary (Table 1) 1, 4,6,8,13) . It can be seen that many are from the pre-MRI era, and many do not provide histological or immuno-cytochemical evidence, raising the possibility that some may not be entirely extraosseous and some may be not actually be chordoma.…”

Intradural Retroclival Chordoma Without Bone Involvement-Case Report-

“…The tumor tissue was characterized histologically by lobules composed of typical physaliphorous cells with abundantly vacuolated cytoplasm. Histological examination of the speci- 3) 57, M prepontine autopsy none Mathews and Wilson (1974) 11) 41, M intrasellar surgical findings subtotal Stam and Kamphorst (1982) 14) 75, M prepontine autopsy none Mapstone et al (1983) 10) 26, M prepontine MR imaging/surgery subtotal Vaquero et al (1983) 16) 39, F suprasellar surgery total Yuhi et al (1986) 20) 28, M prepontine CT cisternogram ND Katayama et al (1991) 8) 30, F foramen magnum MR imaging/surgery total Warnick et al (1991) 18) 58, M tentorium CT/surgery subtotal Hardie (1992) 7) 28, F prepontine MR imaging/surgery ND Tashiro et al (1994) 15) 56, F prepontine CT/MR imaging/surgery ND Wanibuchi et al (1994) 17) 54, M suprasellar CT/surgery total Nishigaya et al (1998) 12) 56, M prepontine MR imaging/surgery subtotal Korinth et al (1999) 9) 48, F ponto-cerebellar MR imaging/surgery subtotal Danilewicz et al (2000) 5) 19, F prepontine CT/surgery total Dow et al (2003) 6) 9, F cerebellar MR imaging/surgery total Present study 63, M prepontine CT/MR imaging/surgery total CT: computed tomography, MR: magnetic resonance, ND: not described.…”