8 Hemangiopericytoma

Burak Sade and Joung H. Lee

Fig. 8.1 (A) T1-weighted postcontrast axial and (B) coronal magnetic resonance images showing a diffusely enhancing 8-cm perisellar mass originating from the anterior clinoid process. Note the significant midline shift and ventricular dilatation.

Clinical Presentation

A 17-year-old right-handed boy presents with a few months’ history of behavioral changes and worsening school performance.

His neurologic evaluation is significant for severe visual deterioration at the level of light perception and central-type facial weakness on the left side.

Magnetic resonance imaging (MRI) scan of the brain is presented in Fig. 8.1.

Questions

What are the significant findings in the MRI?

What is your differential diagnosis?

What would be your initial management?
The patient was operated through a right frontotemporal craniotomy. The surgery had to be staged because of extensive bleeding. Gross total resection was achieved after the second stage (Fig. 8.2). The tumor histology confirmed the diagnosis of hemangiopericytoma.
How is hemangiopericytoma classified in the most recent World Health Organization (WHO) classification in 2007, and how does it differ from meningioma in this classification?
Is there any role for adjuvant treatment?
Following surgery, the patient received intensity modulated radiotherapy (IMRT).
Seven years after his initial surgery and radiotherapy, he had a recurrence to the left ventral petrous region, which was treated with surgery and IMRT. Nine years after his initial surgery, he developed recurrence to his original tumor bed, which was resected.
At the time of his second recurrence, a metastasis was detected in his pancreas, which was treated with radiotherapy. Currently, he is 11 years out of his first surgery, with no intracranial detectable disease and stable pancreatic metastasis.
What is the prognosis for hemangiopericytoma?