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Gamma Knife Radiosurgery in Intracranial Angiosarcoma

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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.

Intracranial Angiosarcoma
Intracranial Angiosarcoma

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:

Important Features_ Intracranial Angiosarcoma
Important Features_ Intracranial Angiosarcoma
  • 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

Clinical Presentations_ Intracranial Angiosarcoma
Clinical Presentations_ Intracranial Angiosarcoma

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

Diagnosis_ Intracranial Angiosarcoma
Diagnosis_ Intracranial Angiosarcoma
  1. 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.

  1. 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.

  1. 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.

  1. 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:

Gamma Knife benefits include_ Intracranial Angiosarcoma
Gamma Knife benefits include_ Intracranial Angiosarcoma
  • 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:

  1. https://pmc.ncbi.nlm.nih.gov/articles/PMC6895451/#sec7
  2. https://www.cancer.gov/pediatric-adult-rare-tumor/rare-tumors/rare-vascular-tumors/angiosarcoma#:~:text=You%20may%20have%20pain%20in,possible%20to%20feel%20the%20lump.
  3. https://www.pennmedicine.org/cancer/types-of-cancer/sarcoma/types-of-sarcoma/soft-tissue-sarcoma/angiosarcoma
  4. https://pubmed.ncbi.nlm.nih.gov/19467792/
  5. https://www.researchgate.net/publication/389465636_Gamma_Knife_Radiosurgery_in_Intracranial_Angiosarcoma
  6. https://www.sciencedirect.com/science/article/abs/pii/S0360301609002235
  7. https://diagnosticpathology.biomedcentral.com/articles/10.1186/s13000-023-01389-7
  8. https://my.clevelandclinic.org/health/diseases/22778-angiosarcoma
  9. https://www.researchgate.net/figure/A-proposed-flow-chart-for-the-treatment-of-intra-cranial-angiosarcoma_fig6_230713175
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You May Also Know Related to Gamma Knife
FAQ’s

Gamma Knife uses 192–201 precisely focused beams of gamma rays that converge at a single target point in the brain. This high dose of radiation damages the DNA of abnormal cells, stopping their growth or causing them to shrink, while minimizing exposure to nearby healthy tissue.

No, Gamma Knife is not a traditional surgery. It is a non-invasive outpatient procedure performed without any incision, stitches, or general anesthesia in most cases.

Gamma Knife is commonly used for:

Brain tumors (benign and malignant)
Metastatic brain lesions
Arteriovenous malformations (AVMs)
Trigeminal neuralgia
Acoustic neuromas (vestibular schwannomas)
Pituitary tumors
Certain functional disorders (like tremors)

The procedure is generally painless. Patients may feel mild pressure while the head frame (if used) is applied or may experience slight discomfort from anesthesia injections, but the treatment itself is not painful.

Depending on the condition and treatment plan, Gamma Knife may take 30 minutes to 3 hours. Most patients go home the same day.

Common side effects are usually mild and temporary, including:

Headache
Nausea
Fatigue
Mild scalp swelling or tenderness at the frame site
Rarely, some patients may experience delayed radiation effects such as brain swelling or radiation necrosis.

Most patients can return to their normal routine within 24–48 hours after the procedure.
You may feel mild fatigue for a few days.
Follow-up imaging is usually required after 1 year.

Gamma Knife has a high success rate and has been used worldwide for decades. Its effectiveness depends on the condition treated, size and location of the lesion, and overall patient health. In many cases, it offers results comparable to open surgery with fewer risks.

Yes, in some cases Gamma Knife treatment can be repeated if the disease recurs or if new lesions develop.

Yes. Gamma Knife is considered one of the safest and most precise forms of radiosurgery, with millions of patients treated globally and extensive clinical data supporting its use.

Eligibility depends on factors such as:

Size and location of the lesion
Overall health and age
Whether open surgery is too risky
A neurosurgeon and radiation oncologist will decide if Gamma Knife is the best option for you.

A detailed MRI or CT scan is performed to map the brain.
A lightweight head frame or mask is used for accuracy.
Your doctors plan the radiation dose and target areas using specialized software.

Local anesthesia is given if a head frame is used.
General anesthesia is usually not required, except for children or patients unable to remain still.

Costs vary depending on hospital, city, and condition treated. On average, Gamma Knife in India ranges from ₹1.5 lakh to ₹4.5 lakh. It is usually more affordable compared to treatment in Western countries. At AIIMS Delhi, it is much more affordable around ₹75,000 which makes it affordable for the patients in need.

AIIMS Delhi is one of the leading centres for Gamma Knife in India.

AIIMS uses the latest Gamma Knife Perfexion system.

It has successfully treated thousands of patients for brain tumors, blood vessel problems, and even eye cancers.

The treatment is done by expert neurosurgeons such as Dr. Deepak Agrawal and team.

Clinic timings for Gamma Knife OPD at AIIMS Delhi: Monday & Friday, 8:00 AM – 9:00 AM.

Cost is around ₹75,000 and is subsidized compared to private hospitals.

The whole procedure is usually done in one day:

  • Consultation & Planning – Doctors review your scans and decide the treatment.
  • Head Frame/Mask – A lightweight frame or mask is placed to keep your head still.
  • Imaging – MRI/CT scans are done to locate the exact target.
  • Treatment Planning – Specialists plan the dose and direction of radiation.
  • Gamma Knife Treatment – You lie on the machine; painless radiation beams treat the target (30 mins–3 hrs).
  • Recovery & Discharge – The frame is removed, and most patients go home the same day.
  • Follow-up – MRI after a few months to check results.

Before you get a treatment date, you need to complete a few steps:

  • OPD Registration – Visit the Neurosurgery OPD (Gamma Knife clinic at AIIMS, Mon & Fri 8–9 AM) and register.
  • Consultation with Doctor – Meet the neurosurgeon who will check your reports, MRI/CT scans, and medical history.
  • Investigations – Sometimes fresh MRI/CT or blood tests are needed for treatment planning.
  • Medical Fitness – Basic checks like blood pressure, sugar, heart condition, etc.

Financial/Insurance Approval –

  1. If using Ayushman Bharat or government schemes, you need approval papers.
  2. If self-paying, you will be given the estimated cost (~₹75,000 at AIIMS).
  • Consent & Admission Slip – Once doctors confirm you are fit and formalities are done, you sign the consent form.
  • Treatment Date Allotment – A date is given for your Gamma Knife procedure.

Once you receive your Gamma Knife date, you’ll need to do a few simple things before the procedure:

  • Admission Process – Report to AIIMS on the morning of your procedure and complete admission at the Neurosurgery ward/Daycare.
  • Paperwork – Carry your OPD slip, MRI/CT films, blood test reports, admission slip, and ID proof. If you are covered under Ayushman Bharat/insurance, keep those approval papers ready.
  • Consent Forms – You (or a family member) will sign consent for the procedure and anesthesia.
  • Medical Check-up – Doctors will check your BP, sugar, heart rate, and do a quick physical exam.
  • Fasting – Usually, you will be asked to not eat or drink for 6 hours before the procedure.
  • Pre-Procedure Prep – An IV line may be put, and medicines (sedation/antibiotics if needed) are given.
  • Treatment – You are then taken to the Gamma Knife room for frame/mask placement, imaging, and treatment.

After the procedure, you’ll be observed for a few hours and most patients are discharged the same day or next morning.

Urea & creatinine

No, You can have light breakfast.

Yes, you can take the medicine and then come for the treatment.

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