Extensive irregular destruction of the mastoid, petrous part of the temporal bone, medial cortex of the clivus, greater wing of the left sphenoid bone with associated mass extending into the left sphenoid sinus. The left otic capsule is partially eroded on its medial aspect.

Large complex signal lobulated T1 hypointense, T2 hyperintense with hemorrhagic foci and necrotic component involving the skull base/left temporal, petrous with extensive bony destruction with associated neurovascular involvement and mass effect on the brainstem and left cerebellar hemisphere

Differential diagnosis:

• Central skull base schwannoma
• Mass of haematogenous etiology (plasmacytoma/lymphoma)
• Chordoma
• HPE-CHORDOMA

• Uncommon malignant slow growing tumor that account for 1% of intracranial tumors and present due to mass effect on adjacent structures.
• Originate from embryonic remnants of the primitive notochord
• Age–usually seen in adults (30-70 years)
• Tumors in spheno-occipital region tumor 20-40 years age group
• Tumors in sacrococcygeal region-typically seen in a slight older age group (peak around 50 years)
• Location
  • sacrococcygeal: 30-50%
  • spheno-occipital: 30-35%
  • vertebral body: 15-30%

CT

• These are well-circumscribed, destructive lytic lesion. The associated expansile soft-tissue mass may have inhomogenous areas due to necrosis or haemorrhage; the soft-tissue mass is often disproportionately large relative to the bony destruction)
• Irregular intratumoral calcifications may be seen with moderate to marked enhancement

MRI

• These lesion show T1 intermediate to low signal intensity with small foci of hyperintensity (Intratumoral heamorrhage or a mucus pool) and appear T2 hyperintense. Hemorrhagic foci are noted with heterogeneous enhancement and honeycomb appearance corresponding to low T1 signal areas within the tumor

• Surgery
• Radiation therapy for recurrent
• Prognosis is typically poor, due to the locally aggressive nature of these tumors, with the 10-year survival approximately 40%