Orbital Tumours

a) What is cavernous haemangioma?

This is where abnormally dilated blood vessels behind the eye collect together and form a tumour. This is the most common benign orbital tumour in adults. It occurs more often in females and can develop anywhere in the orbit. More commonly, however, it develops beside the muscles that move the eye. Although this tumour is benign, it can grow slowly behind the eye and cause damage.

b) What are the symptoms of cavernous haemangioma?

There may be no symptoms. If the cavernous haemangioma continues to grow, however, it can cause the following:

• Proptosis (Eye sticking out)

• Double vision

• Blurring of vision

c) Will I need any tests?

We may take pictures of the eye and consider the following tests:

• CT scan

• MRI scan (picture above)

This can help us measure the size of tumour and where it is. This is useful if we are planning surgery or to check if the nerve is being squashed. Cavernous haemangioma on MRI scan.

d) What is the treatment of cavernous haemangioma?

If not causing problems we may just observe routinely in the clinic. If, however, it is growing and causing problems, surgery is the best treatment. Unfortunately bleeding, infection or damage to nerves and muscles of the eye can occur during surgery. The risk of this depends on the tumour location and size.

a) What is pleomorphic adenoma?

This is the most common benign tumour of the lacrimal gland. It is also known as benign mixed cell tumour. It is formed from the cells and ducts that produce tears in the lacrimal gland. They also commonly occur in the salivary glands. Although labelled as benign, very rarely pleomorphic adenoma can turn into malignant tumours.

b) What are the symptoms of lacrimal gland pleomorphic adenoma?

This causes a painless lump in the lacrimal gland and sometimes swelling to the top eyelid. This can slowly cause the eye to stick out (proptosis – picture above). It can also push the eye down and inwards. This may cause double vision and decreased the vision.

c) Will I need any tests?

Different types of scans can be used to help diagnose this benign tumour. It is also useful if surgery is being planned. Scans may include:

• CT scan

• MRI scan

• US scan

d) What is the treatment for lacrimal gland pleomorphic adenoma?

Removal with surgery may be required. We aim to remove the whole tumour without cutting into the tumour – this may cause it to spread and re-grow at a later stage. For this reason biopsy we do not take a biopsy before surgery.

a) What is lacrimal gland carcinoma?

This is a rare malignant tumour of the lacrimal gland. The commonest malignant lacrimal gland carcinoma is called adenoid cystic carcinoma. This tumour can grow quickly and spread to the bones and the nerves around the eye.

b) What are the symptoms of lacrimal gland carcinoma?

This tumour can grow quickly and cause swelling of the top eyelid. The eye may stick out (proptosis- picture above) or be pushed downwards and inwards. This can cause double vision and blurring. If the tumour spreads into the bone and the nerves this can cause pain or numbness over the face.

c) What are the risks of getting lacrimal gland carcinoma?

The following increase your risk of lacrimal gland carcinoma:

• Age – it is more common in your 30s.

• Previous radiation treatment to the face,

• Previous removal of a benign lacrimal gland pleomorphic adenoma.

d) Will I need any tests?

We may perform the following scans:

• CT scan

• MRI scan

• Ultrasound scan

This helps us measure the size of the tumour, position, and check if there is any spread into the bone. Biopsy of the tumour is sometimes performed to confirm the diagnosis in the laboratory.

e) How is lacrimal gland carcinoma treated?

Surgery and radiotherapy is usually required. Surgery may include removal of the tumour and tissue around the lacrimal gland. If the tumour has spread out of the lacrimal gland then exenteration may be required. Unfortunately treatment is rarely curative, however, our medical oncology team will guide us on the best treatment for you.

a) What is optic nerve glioma?

Optic nerve glioma is a slow growing tumour developing within the optic nerve. It is more common under the age of 20. Another name for this tumour is a pilocytic astrocytoma. Patients who get this tumour commonly have an under lying medical condition called neurofibromatosis type 1. This is a genetic condition that makes you more likely to grow tumours along your nerves.

b) What are the symptoms of optic nerve glioma?

Since this tumour is slow growing, it usually causes a gradual decrease in vision in the affected eye. The tumour can push the back of the eye causing the eye to stick out (proptosis- picture above). Rarely, the vision may quickly deteriorate. This may be due to bleeding into the tumour, causing it to squash the nerve or the blood vessels to the eye. Sometimes the tumour can spread backwards into the brain. If this happens then headaches (worse in the morning), nausea and vomiting may occur.

c) Will I need any tests?

If optic nerve glioma is suspected then scanning the orbit and the brain is likely to be required. This may include:

• MRI scan

• CT scan

• US scan

If neurofibromatosis is suspected then genetic testing may be performed. A full body scan may also be useful to look for other tumours else where in the body. If the diagnosis is uncertain we may decide to take biopsy.

d) What is the treatment for optic nerve glioma?

If not causing any problems then treatment may not be required. If, however, the tumour is growing and causing forward displacement of the eye or decreasing the vision, then surgery is recommended. Unfortunately, because the tumour is on the optic nerve, removing this may cause damage to the vision. Radiotherapy and chemotherapy may also be used if the cancer has spread to the brain. Our medical oncology team will guide us on the best treatment for your case.

e) How effective is the treatment?

Treatment is effective in slow growing optic nerve gliomas. These are more common in children. In adults, however, aggressive fast growing optic nerve gliomas are more common and are more likely to spread back into the brain and threaten life. These are more difficult to treat.

a) What is optic nerve sheath meningioma?

Wrapped around the optic nerve is a sheath. This is similar to the insulation layer of a wire. Along this sheath, a benign tumour called a meningioma can develop. This more commonly occurs between 30 and 60 years of life. It is 4 times more likely to occur in woman. This tumour can also develop in the protective layer of the brain and the spinal cord. Meningiomas may grow fast or slow.

b) What are the symptoms of optic nerve sheath meningioma?

The faster the tumour is growing then the quicker the symptoms will develop. This may include:

• Poor vision

• Proptosis (eye sticking out)

• Double vision .

c) Will I need any tests?

If meningioma is suspected, scanning of the orbit and the brain is usually carried out. Scans may include:

• MRI scan (picture above)

• CT scan

• US scan

Scans may be repeated at a later date to see if the meningioma is growing. If the diagnosis is uncertain we may decide to take a biopsy.

d) What is the treatment of optic nerve meningioma?

Treatment may not be required if the tumour is slow growing and not causing symptoms. This is more common in middle aged patients. In younger patients, however, the tumour is more likely to be aggressive and fast growing. If this is the case, surgery is required. Unfortunately surgery may damage the nerve and cause poor vision. Sometimes radiotherapy is given after surgery to reduce the risk of it coming back later in life. Unfortunately, radiotherapy may also damage the vision.

a) What is lymphoma?

In lymphoma, the white cells that usually fight infection are “out of control”, keep dividing, and do not die. These abnormal cells collect and grow in the lymph nodes. They can also develop elsewhere in the body, including behind the eye. There are two different types of lymphoma – hodgkins lymphoma (20% of cases) and non-hodgkins lymphoma (80% of cases). Hodgkins lymphoma is the easiest to treat, however, treatments for both types of lymphoma are very effective and can be cured in most cases.

b) What are the symptoms of orbital lymphoma?

This may cause:

• Proptosis (eye sticking out- picture above)

• Double vision

• Decreased vision

c) Will I need any tests?

A biopsy may be required to confirm the diagnosis. Scanning the eye, orbit, and rest of the body is important to make sure the lymphoma is not affecting anywhere else. This may include:

• CT scan

• MRI scan

• US scan

This helps us decide which treatment is needed.

d) What are the treatments for orbital lymphoma?

Treatments include:

• Radiotherapy

• Surgery

• Chemotherapy

Treatments vary patient to patient, however, discussion with our medical oncologist and pathologist will help us choose the best treatment for you.

a) What are orbital metastases?

Tumours starting elsewhere in the body can spread behind the eye in the orbit. This is rare, however, if a tumour is first found in the orbit then cancer elsewhere may have to be considered and looked for. Common places for cancer to grow first before spreading to the eye include the breasts, lungs, prostate, skin, bowel and kidneys.

b) What are the symptoms of orbital metastasis?

Orbital metastases may cause:

• Proptosis (eye sticking out- picture above)

• Double vision

• Decreased vision

In certain tumours, enophthalmos can occur; this is where the eye moves backwards into the orbit making the eye appear smaller. If cancer elsewhere is present then some of the following symptoms may be experienced:

• Shortness of breath

• Long standing cough

• Coughing up blood

• Breast lump

• Difficulty passing urine

• Blood in urine or stools

• Change in bowel habit

c) What tests will I get?

Scanning the eye and elsewhere in the body depending on symptoms experienced. Scans may include:

• CT scan

• MRI scan

• PET CT scan

• Chest X-ray

• Blood tests

If we are unsure of the diagnosis, an orbital biopsy may be carried out. This, however, will be discussed further in clinic.

d) What is the treatment for orbital metastasis?

Treatments may include:

• Radiotherapy

• Chemotherapy

• Surgery

The main goal is to preserve vision and keep the eye comfortable. Our medical oncologist will help us choose the best treatment for you.