Hemangioblastoma - Causes and Symptoms

Introduction

A hemangioma is an overgrowth or proliferation of blood vessels on the surface or under the surface of the skin and other internal organs. The hemangiomas present as red or purple lumps or swellings on the skin. There are two most common types of hemangiomas, namely capillary hemangiomas which develop on the superficial or outer layers of skin, and cavernous hemangiomas, which develop on the deeper layers of skin.

What Is Hemangioblastoma?

A hemangioblastoma is defined as a benign and slow-growing tumor that arises from the cells that form the lining of the blood vessels. The tumors form on the blood vessels of the brain, the spinal cord, and the retina of the eye. It is a harmless overgrowth of vascular tissue. These hemangioblastomas are associated with the central nervous system. They do not cause any serious health problems, but the overgrowth may cause pressure on other surrounding tissues, and this may lead to health issues.

What Is the Cause of Hemangioblastoma?

The exact cause of the occurrence of hemangioblastoma is unknown. But they occur as a part of a genetic condition called von Hippel-Lindau syndrome. They develop sporadically; that is, they occur sometimes but not regularly. Von Hippel-Lindau(VHL) syndrome is a rare inherited disease developing multiple cysts and tumors in different parts of the body like the brain, spinal cord, inner ear, eyes, adrenal glands, pancreas, kidneys, and reproductive extract. This syndrome increases the risk of kidney and pancreatic cancer in the affected individual. This is caused due to a genetic mutation in the VHL gene located on chromosome 3.

What Is the Incidence of Hemangioblastoma?

Hemangioblastomas are uncommon tumors and account for about two percent of all primary brain tumors. About 10 percent of affected individuals have von Hippel-Lindau syndrome. It is slightly more common in males than in females.

What Are the Signs and Symptoms of Hemangioblastoma?

The symptoms usually occur during adulthood. The symptoms vary slightly depending on its size and location. The symptoms are caused mainly due to the compression of surrounding tissue by the tumor and interruption in the movement of cerebrospinal fluid, also due to the compression.

Hemangioblastomas of the Spinal Cord Cause:

Numbness or tingling sensation(pins and needles in the arms and legs).

• Clumsiness of hands.
• Sensory loss.
• Weakness of muscles.
• Difficulty in walking and with gait.
• Bowel incontinence (inability to control bowel movements leading to involuntary soiling).
• Constipation.
• Urinary incontinence (inability to control bladder movement leading to urinary leakage).
• Urinary retention (Incomplete emptying of the bladder).

Hemangioblastomas of the Brain Causes:

• Nausea and vomiting.
• Headaches.
• Difficulty in balancing and coordination.
• Changes in the behavior.
• Changes in mental function.
• Hydrocephalus (collection of CSF in the brain).
• Polycythemia (increased red blood cell production).

Hemangioblastomas in the Retina of the Eye Causes:

• Pain and swelling in the eye.
• Loss of vision.
• Retinal detachment.

How Is Hemangioblastoma Diagnosed?

• Computed Tomography (CT): It is a specialized imaging technique that uses a special machine to produce cross-sectional images of internal organs, bones, soft and hard tissues, and blood vessels.

• Magnetic Resonance Imaging (MRI): It is also a medical imaging technique that uses strong magnetic and radio waves to generate detailed pictures of internal organs.

• Digital Subtraction Angiography (DSA) And X-Ray: It is a fluoroscopy method used as a part of interventional radiology where a dye is injected into the tumor to visualize them more clearly in a radiographic image.

• Clinical Examination: A complete physical and clinical examination of the tumor can help diagnose the condition. A family history of any case of von Hippel-Lindau syndrome is to be enquired about.

• Microscopic Examination: A sample of the excised tumor will present with the following features under a microscope.

  • Presence of neoplastic stromal cells with many small blood vessels.

  • Non Infiltrative, compact, and well-demarcated growth.

  • Foamy and clear cytoplasm.

  • Thin-walled blood vessels surrounded by connective tissue.

What Is the Treatment of Hemangioblastoma?

• Surgical Excision: The tumor mass is removed completely without causing damage to the surrounding healthy tissue while the patient is under general anesthesia. The large lesions are challenging to remove.

• Preoperative Embolization: It is a procedure done before the surgical resection of large tumors. A clotting material is injected into an artery which will block the blood supply to the tumor. This procedure is done a few days before the surgery. It will reduce the blood loss during the surgery and will greatly decrease the time taken for the completion of surgery and the need for blood transfusion. Since the supply of oxygen and nutrients to the tumor is cut, its size reduces, and it becomes easier to remove it.

• Radiation Therapy: Ionizing radiation is used to reduce the size of the tumors by killing the rapidly dividing cells.

• Monitoring the Disease: If the tumor size and location are unfavorable for surgical removal or if the tumors are growing slowly and are unstable, only observation and regular monitoring of the tumors is advised with scans and X-rays.

What Is the Prognosis of Hemangioblastoma?

The prognosis of hemangioblastoma is good if the surgeons are successful in removing the tumor completely and the tumor has not caused any long-term serious brain damage or vision damage. Hemangioblastomas grow back rarely but are seen in 25 percent of people. But regular check-ups with a proper investigation are recommended to detect any recurrence of the tumor and will also help in early diagnosis.

What Are the Differential Diagnosis of Hemangioblastoma?

• Brain Metastasis: It is the spread of cancer cells from their origin to the brain.

• Astrocytoma: A malignant tumor that develops from star-shaped cells in the cerebrum called astrocytes.

• Ependymoma: A malignant tumor that develops from ependymal cells lining the pathways through which cerebrospinal fluid flows.

• Arteriovenous Malformation: An abnormal connection between arteries and veins.

• Cavernoma: It is a cluster of abnormal and thin-walled blood vessels in the brain and the spinal cord.

• Medulloblastoma: It is the most common type of malignant brain tumor in children developing in the cerebellum.

Conclusion

The mutual teamwork of the department of oncology and neurology is essential to successfully treat a hemangioblastoma. It requires an experienced panel of specialists like neurosurgeons, neurologists, neuro-oncologists, radiation oncologists, medical oncologists, geneticists, and pathologists.