Intracranial Angiosarcoma

An extremely uncommon and violent malignant tumour that develops from the endothelial cells lining the brain’s blood arteries is called an intracranial angiosarcoma. It presents considerable difficulties in diagnosis, therapy, and long-term management because of its rarity and highly vascular character. Even though this condition is rare, it must be understood in order to improve patient outcomes and act immediately.

What is it? (Intracranial Angiosarcoma)

One kind of soft tissue sarcoma that develops from vascular or lymphatic endothelial cells is called angiosarcoma. The term “intracerebral angiosarcoma” describes the tumor’s appearance inside the cranial cavity, while the majority of angiosarcomas develop in the skin, soft tissue, liver, heart, or breast. It could affect the dura mater, intracranial vascular, or brain parenchyma.

Important Features:

Extremely hostile and prone to quick growth.
High risk of bleeding due to a rich vascular supply.
Either primary (originating in the cranium) or secondary (originating elsewhere).
Primarily impacts middle-aged individuals, while incidences have also been documented in older and paediatric populations.
Has radiologic similarities to other vascular tumours or hemorrhagic lesions, making diagnosis difficult.

Clinical Presentations

The size, location, and development rate of the tumour all affect the symptoms of cerebral angiosarcoma, which are frequently non-specific. Typical signs and symptoms include:

Chronic headaches
Seizures
Deficits in the nervous system (e.g., weakness, sensory abnormalities)
Papilledema and vomiting are symptoms of elevated intracranial pressure.
Changes in mental state
Abrupt neurological deterioration brought on by tumour bleeding

Its aggressive nature causes these symptoms to deteriorate quickly over time.

Diagnosis

Imaging:

The main modality is MRI with contrast, which usually shows a heterogeneous, enhancing mass with necrotic and bleeding regions. Nevertheless, the imaging results are not definitive and frequently resemble other intracranial tumours including glioblastomas, hemangioblastomas, or metastases.

The study of histopathology

Histopathological analysis after biopsy or surgical removal is necessary for a conclusive diagnosis. Important conclusions include:

Uneven vascular channels formed by malignant endothelial cells
Elevated mitotic activity
Positive immunohistochemistry results for FLI-1, ERG, CD31, and CD34

Strategies for Treatment

The treatment of cerebral angiosarcoma is multimodal and patient-specific due to its aggressive nature.

Resection via Surgery

When possible, surgical excision continues to be the primary form of care. Because of the vascularity of the tumour, intraoperative bleeding frequently complicates surgery. Complete excision might not always be feasible, particularly in deep-seated or eloquent brain regions.

Radiation therapy

To lower the chance of a local recurrence, post-operative radiation is frequently used. Depending on the size and location of the tumour, either stereotactic radiosurgery or conventional fractionated radiation may be utilised.

The use of chemotherapy

Based on procedures for soft tissue sarcomas, chemotherapy medicines such paclitaxel, doxorubicin, or ifosfamide have been utilised, although the exact role of chemotherapy is still unknown due to a lack of data. Immunotherapy and targeted therapeutics are being investigated.

Intracranial Angiosarcoma and Gamma Knife Radiosurgery

A type of stereotactic radiosurgery called Gamma Knife Radiosurgery (GKRS) spares the surrounding healthy brain tissue while precisely delivering high-dose radiation beams to intracranial lesions. It works especially well for vascular tumours and tumours in difficult-to-operate places.

Function in Angiosarcoma Intracranial:

Although it is not a first-line treatment, Gamma Knife radiosurgery is crucial in adjuvant or palliative settings. It is useful for:

For recurring or persistent tumour tissue after surgery.
Is the main treatment for patients who are not suitable for surgery.
In the treatment of brain-metastasized angiosarcomas.
To reduce the risk of bleeding in highly vascularised tumors.

Gamma Knife benefits include:

Outpatient, non-invasive procedure
Accurate targeting reduces harm to nearby structures.
Less chance of bleeding than with open surgery
In certain cases, local tumour management is effective.

Restrictions:

Appropriate for tumours with a considerable volume
Systemic therapy may still be necessary.
Due to its rarity, long-term results in cases unique to angiosarcoma are not well reported.

The outlook

For patients with intracranial angiosarcoma, the prognosis is still uncertain. Because of the tumor’s aggressive nature and high recurrence rates, survival prospects are typically poor. Although early detection, total surgical resection, and adjuvant therapy like Gamma Knife Radiosurgery may improve results in some individuals, the median survival is frequently less than a year.

In conclusion

An uncommon but dangerous brain tumour, intracranial angiosarcoma is known for its high recurrence rate, rapid growth, and risk of bleeding. For the best care, an integrated group of neurosurgeons, oncologists, radiation therapists, and pathologists is essential. A useful tool in the neurosurgical toolbox, innovations like Gamma Knife Radiosurgery provide targeted therapy with fewer problems, particularly for tumours in surgically difficult sites.

To choose the best and most individualised course of therapy if you or a loved one has been diagnosed with intracranial angiosarcoma, it is essential to speak with a skilled neurosurgery team.

Source: