One of the most commons types of lymphoma to affect the human body is orbital lymphoma. Generally, orbital lymphoma can grow in the lacrimal gland, the eye muscles, conjunctiva or the tissues of the eyelids. Orbital lymphoma can be categorized according to the site where they are detected so they can be either orbital and adnexal or intraocular lymphoma. Most orbital lymphomas are extraconal but they may extend intraconally also.
For intraocular lymphoma the diagnosis is done by an intraocular biopsy and the most common symptom is progressive deterioration in vision. A combination of radiation and chemotherapy is the most effective however significant amount of ocular morbidity is associated with this treatment. Intraocular lymphoma generally affects very old adults, mostly older than 60 years of age. Predominantly, women tend to suffer from intraocular lymphoma more as compared to men.
Orbital and Adnexal lymphoma generally do not present symptoms for a long time. Generally, this type of lymphoma causes pressure on the surrounding structure, double vision, and proptosis and in some cases decrease in vision.
Very rarely intraocular and orbital lymphomas occur together.
Broadly, orbital lymphomas are divided into two categories depending upon their origin. Primary orbital lymphomas are the lymphomas that grown in the ocular region and secondary orbital lymphoma are the ones which grow in some other body part but spread and invade the orbit. Orbital lymphomas account for more than half of the lymphoma cases reported annually in India.Orbital lymphomas can affect anyone. Orbital lymphoma can be unilateral or bilateral which means that either they can affect one eye or may affect both the orbits.
Causes of orbital lymphoma:-
One of the most important reasons that have been attributed to orbital lymphoma is weak immunity. Diseases which affect immunity like AIDS, old age and medication which adversely affect the immunity are believed to trigger orbital lymphoma.
Symptoms of Orbital Lymphoma:-
The most common symptom of orbital lymphoma is bulging globe of the eyes which is caused by the pressure that is built up by the orbital lymphoma. Fluid retention in the eyelids and tender orbital mass can indicate orbital lymphoma. Drooping of the eyelid or ptosis is also a common symptom of orbital lymphoma. Double vision is also a symptom of orbital lymphoma.
Grading and stages of orbital lymphomas:-
The aggressiveness of the orbital lymphoma is indicated by a grading system and how far the orbital lymphoma has metastasized is indicated by the stages of the orbital lymphoma. According to the grade of orbital lymphoma, a low grade orbital lymphoma is a slow spreading orbital lymphoma. An intermediate grade orbital lymphoma spreads at a moderate rate. A high grade orbital lymphoma is a very aggressive orbital lymphoma and spreads at a very high rate.
A stage I orbital lymphoma is only confined to the orbital area. The stage II orbital lymphoma invades the nearby anatomy such as the nose. Stage III and stage IV orbital lymphomas extend to other body parts sometimes including the liver and the bones.
Diagnosis of orbital lymphoma:-
Following diagnostic tools are used to confirm the presence of orbital lymphomas:-
- A complete look into the history of the patient.
- Before the treatment and the surgery, the orbital and the oral cavity are checked for the grade of orbital lymphoma.
- Thorough blood work is done on the patient.
- Various imaging tests like the CT scan and the MRI are done on the orbit, abdomen and other suspected body parts for any trace of orbital lymphoma. For intraocular lymphomas, a B-scan ultrasonography is performed which gives more precise results as compared to MRI and CT scan. For orbital and adnexal lymphomas, a strong conclusive diagnosis can be made with the help of MRI and CT scan.
Orbital lymphomas appear as distinctive homogenous masses which have well defined boundaries, on the CT scan. They generally take shape of the structure where they grow without disintegrating the bone. In case the orbital lymphomas are high- grade lymphomas they cause erosion of bones and are heterogeneous in appearance.
- A minor surgery or a biopsy is done to check for the cancerous tissues. A vitreous biopsy is performed for testing for lymphoma which has ocular involvement. A retinal biopsy may also be performed after a vitreous biopsy through an intraocular or transscleral approach. Biopsy of the lesion is also performed for diagnosis and grading of lymphoma. For lesions of the eyelid and conjunctiva a direct approach can be used for biopsy. An orbitotomy is performed if the lesion is present posteriorly in the orbit or the lacrimal gland. In case both the eyes are affected, biopsy is generally performed on one eye only.
- If the patient is showing the signs of CNS lymphoma then CSF may also be tested by cytology.
- For ruling out the possibility of retroperitoneal lymphoma, CT scans of the chest and abdomen are also done.
Treatment of orbital lymphoma in India:-
If the orbital lymphoma is present in the orbital cavity, the treatment can be done by four methods which include surgery as one of the options. The other three treatment methods for orbital lymphoma in India include immunotherapy, radio therapy and chemotherapy which can be done in addition to surgery. The prognosis in the case of orbital lymphoma depends upon the nature of the orbital lymphoma i.e. its grade and stage. One of the top most important things to know about the treatment of orbital lymphoma with surgery is that if the treatment is only done by surgery there are very high chances of relapsing of orbital lymphoma. The top reason behind this is that the location of the orbital lymphoma makes it nearly impossible for the surgeon to completely excise the orbital lymphoma by surgery while ensuring that the vision is not compromised. Radiotherapy is utilized more in India for the best treatment of orbital lymphoma because orbital lymphomas may extend beyond the orbital cavity. The best approach which should be taken while using radiotherapy is that the entire orbit should be treated with radiation; this reduces the chance of the relapse after the surgery. Combination of therapies along with surgery is most commonly done in India. After the surgery and treatment, patient needs to go for lifelong follow-up to ensure that there is no recurrence of orbital lymphomas. Over the follow up, the patient is put under a series of imaging tests. Sometimes, in the imaging tests residual fibrosis may also appear to be similar to ocular lymphoma. With the help of various imaging tests, the surgeon can decide whether to perform a biopsy or not. After the first surgery, a secondary surgery may be required for complete resection.
India is fast gaining notoriety for being the country with one of the highest rates of orbital cancers. Every year, almost 11000 cases are being diagnosed for several types of orbital cancers. One of the top problems with medical care in India is the lack of ophthalmologists who can perform the best treatment and surgery of orbital lymphomas. Surgery remains the best option for treatment in most cases in India but there is a dearth of affordable treatment and surgery in most of India. This leads to patients either delaying the treatment or having to come to cities like Mumbai for surgery. Cities like Mumbai and Delhi have the top facilities for surgery however; there are inadequate cost- effective surgery options. There has been a significant increase in the number of people who are coming to Mumbai for best orbital cancer treatment and surgery. One of the top reasons why orbital cancers are neglected in India is lack of awareness. Dr. Debraj Shome, who is one of the best oculoplastic and facial plastic surgeons in India and regularly performs surgery on people in Mumbai for orbital lymphoma, says that it is astonishing that most of his patients in Mumbai are coming from cities in India which have no cancer surgery options. He says that status of health care and cancer surgery in cities except metros like Mumbai is appalling. The focus of government should be now areas other than metros like Mumbai, Chennai etc. which are in dire need of best healthcare services including surgery. As a consultant to some of the best hospitals in Mumbai, Dr. Shome feels that affordable and top quality healthcare services including surgery is everyone’s right. In Mumbai, and other cities he conducts conferences and seminars to spread awareness about this life- changing condition. Because of his endeavors Mumbai is now the epicenter of cancer surgery and top quality cancer treatment.