Intracranial solitary fibrous tumours/Hemangiopericytomas

Want to submit your case? Click HERE

Solitary fibrous tumours/Hemangiopericytomas are rare perivascular mesenchymal malignancies that develop at the level of soft tissues and blood vessels. 
This neoplasm, belonging to the group of differentiated vascular sarcomas, can arise anywhere in the body where there are capillaries, in particular:

• Head
• Limbs
• Pelvic area
• Neck

In the field of vascular structure tumours, hemangiopericytomas are among the most aggressive soft tissue sarcomas.

Causes of intracranial solitary fibrous tumours/hemangiopericytomas

The causes as well as the risk factors for this type of tumour of the meninges are still unknown. 

However, a valid hypothesis affirms that hemangiopericytomas originate derives from the uncontrolled proliferation of pericytes.

In addition, it has been found that vascular tumours develop more frequently in subjects who have previously undergone radiation therapies, for example, to treat previous tumour forms.

Symptoms of intracranial solitary fibrous tumours/hemangiopericytomas

Symptoms are usually generic, just like for other sarcomas. As a consequence, this type of neoplasm is usually diagnosed late.

Some of the symptoms of hemangiopericytomas are:

• Seizures
• Persistent headaches
• Nausea and vomiting
• Drowsiness
• Personality changes
• Visual field limitations
• Worsening eyesight
• Diplopia
• Loss of strength and sensitivity
• Slowing of motor functions
• Language difficulties
• Loss of smell or taste
• Problem swallowing

In some cases, hemangiopericytomas may arise as a painless lump.

Diagnosis of intracranial solitary fibrous tumours/hemangiopericytomas

In case of suspected intracranial solitary fibrous tumours/hemangiopericytomas, a neurological examination is required so that the specialist can determine a correct patient's medical history. 

Subsequently, diagnostic imaging techniques must be used:

• Computed tomography (CT)
• Nuclear magnetic resonance (NMR)
• Cerebral angiography (MR-Angiography)

For an accurate diagnosis, a histological analysis that includes immunohistochemical and electron microscopy methods is required for a differential diagnosis to determine angiomatous meningiomas from any other mesenchymal neoplasms of the meninges.

Given the aggressive nature of this tumour of the meninges, analysing other areas of the body that may be subject to metastases, such as lungs and bones, should be also appropriate.

Treatment of intracranial solitary fibrous tumours/hemangiopericytomas

The treatment to be used is assessed based on the location and size of the tumour mass, and the patient's medical condition. Surgical resection is usually the treatment of choice, combined with post-operative radiotherapy in order to reduce the risk of local recurrence.

In cases of highly invasive tumours, incomplete resections or inoperable patients,a treatment with adjuvant radiotherapy is required.