Intradural retroclival chordoma without bone involvement: no tumor regrowth 5 years after operation

Abstract

Chordomas are most commonly located in the extradural region. A 56-year-old man presented with a large chondroid chordoma located totally within the intradural retroclival region. The tumor was resected via the petrosal approach. Five years after subtotal removal, the residual tumor showed no sign of regrowth despite the fact that radiation therapy had not been used. The patient was free of symptoms except for moderate, conductive hearing loss in his right ear. The position of the intradural tumor could be preoperatively diagnosed by neuroimaging and, thus, the petrosal approach was selected. Primary intradural extraosseous chordomas are very rare and difficult to differentiate from ecchordoses physaliphorae on the basis of histological and radiological features; however, MIB-1 staining may be useful.

Skull base chordomas: overview of disease, management options, and outcome

“…Although rare, primarily intradural intracranial chordomas have been encountered. 70,107 Metastatic dissemination is possible and becomes clinically manifest in 10 to 20% of the patients. 54 At autopsy, however, foci of metastatic disease can be found in up to 40% of patients.…”

Intradural chordoma mimicking a lateral sphenoid wing meningioma: a

“…In our case, we encountered no difficulties in the dissection of the tumour from the arachnoid membrane and the dural attachment could be easily separated from the bone. However, even if the resection was incomplete and radiation therapy was not applied, long-term survival without disease progression is possible [3,17]. Therefore, the radiation therapy should not be applied directly after surgery to avoid overtreatment [19].…”

Intradural Retroclival Chordoma

“…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.…”

“…The differential diagnosis of ecchordosis physaliphora and chordoma based on histological and radiological features is likely to be difficult. The MIB-1 proliferating cell index may be useful in the histological differential diagnosis of chordomas, which are neoplastic tumors, and ecchordoses, which are not true tumor 13) . Intradural extraosseous chordoma has clearly different features from those of typical chordoma 17) .…”

“…This neoplasm is thought to be essentially extradural in nature and generally associated with extradural extension and bone destruction. However, if allowed to grow for long periods, it may invade the dura and extend intradurally as well as extradurally 13,16) . Intradural chordomas are very rare tumors that should be distinguished from neurinomas and classic chordomas, because of their different biological behaviors.…”