Anaplastic haemangiopericytoma

Case contributed by Dr. Pablo Diluca

Presentation

20-year-old woman who presented with a 4-week history of blurred vision and headache.

Patient Data

Age: 20 years
Gender: Female

I would like to thank Dr. Hector Lambre, Jorge Chudyk, Pedro Lylyk, Ana Lia Taratuto and all Residents and Radiology Department staff of Clínica La Sagrada Familia.

Non-Contrast CT

Modality: CT

Enlarging extracranial lobulated and hyperdense mass in topography of the torcula and transverse sinuses.

3T MRI

Modality: MRI

MRI perfomed 6 month after CT scan.

The enlarging extracranial lobulated mass is again demonstrated. Now we can see that it grows deep intro the torcula, straight, transverse and superior longitudinal sinuses compressing the adjacent cerebellum and occipital lobes. 

The lesion has heterogeneous low and intermediate T2 signal and demonstrates a diffuse vivid enhancement. No areas of internal diffusion restriction.

At spectroscopy there is a high choline concentration, low NAA and a tiny lactate/lipid peak.

The main differential diagnosis were metastasis, meningioma, lymphoma and haemangiopericytoma.

DSA

Modality: DSA (angiography)

Hypervascular blush in posterior region dependent on pial and dural branches of the posterior circulation and branches of the external carotid arteries, preferably on the right side through the occipital arteries.

Histology

Modality: Pathology

MICROSCOPIC DESCRIPTION:  Celullar proliferation with nidus of small, hiperchromatic nuclei cells, and high mitosis. Some areas of necrosis are identified. 

Inmmunohistochemistry shows strong staining in endothelial cells and perinuclear for CD34.

Negative immunostaining for epithelial membrane antigen (EMA). KI 67 is positive.

DIAGNOSIS: Anaplastic Haemangiopericytoma (Solitary Fibrous Tumor/Haemangiopericytoma) WHO Grade III.

Case Discussion

This case demonstrates an extra-axial mass that expands and infiltrates the torcula, venous sinuses, leptomeningeal space and erodes the adjacent bone.

The main radiological differentials to be considered are metastasis, meningioma, haemangiopericytoma and lymphoma. The latter less likely due the inhomogeneous signal and enhancement in a big soft tissue mass. 

After surgery, histology study confirmed the diagnosis of anaplastic hemangiopericytoma

It is hard if not impossible to distinguish haemangiopericytoma from meningiomas on imaging. Meningiomas tend not to infiltrate leptomeningeal space and tend to thicken the adjacent bone.

Although invasion of the sinuses by a tumour, especially by a meningioma, can occur, mass lesions residing exclusively within the venous sinuses are rare. 

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Case Information

rID: 50719
Case created: 18th Jan 2017
Last edited: 15th Feb 2017
Inclusion in quiz mode: Included

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