Tumour bleed manifesting as spontaneous extradural haematoma in posterior fossa

Abstract

We report a unique case of primary extradural angiosarcoma of posterior fossa manifesting as extradural haematoma in a 12-year-old boy who presented with acute onset headache, vomiting, nuchal rigidity and altered sensorium. The patient underwent a retromastoid suboccipital craniotomy on emergency basis, and the lesion was excised completely. Histopathology and immunohistochemistry revealed an angiosarcoma, following which radiation therapy was given. The patient showed complete clinical and neurological improvement. At a follow-up of 2 years he is in good health without any sign of regrowth.

Figure 1

(A) Plain axial CT of the brain showing an extra-axial well-defined rounded hyperdense lesion along the lateral surface of left cerebellar hemisphere with moderate perilesional oedema. There is effacement of basal cisterns and compression on the fourth ventricle. Postcontrast axial (B) and coronal (C) CT images showing no enhancement. Axial T2-weighted image (WI) (D), coronal fluid-attenuated inversion recovery (E) and sagittal T2-WI (F) showing a T2-heterogeneously hyperintense mass with a hypointense peripheral rim of dura. The lesion blooms on gradient echo image (G) while it is isointense on T1-WI. Postoperative axial T2-WI shows no residual tumour (I).

Figure 2

Photomicrographs show large pleomorphic tumour cells intricately admixed with blood-filled spaces and inflammatory cell infiltrate (A—H&E ×200; B—H&E ×400). The tumour showing frequent mitoses (as shown by the arrows in C—H&E ×400) and cytoplasmic membrane immunoreactivity for CD31 (D—immunohistochemistry ×400).