Intraocular (intraorbital) lymphoma is a malignant tumor lesion of lymphoid tissue of eye fossa (orbit). The disease belongs to the group of non-Hodgkin lymphomas of extranodal type (that is, it is not the lymph nodes that are affected, but other internal organs, in this case orbital tissue).
Most often, eye choroid is affected due to its anatomical and physiological characteristics. This lymphoma is most often secondary (metastatic). In second place in terms of prevalence is retinal lymphoma, that is, the retina is affected. This neoplasm is considered a common primary lesion, usually accompanying primary malignant lymphoma of central nervous system.
Orbital lymphoma is a rare disease that progresses relatively slowly. In children, bilateral tumor localization may occur.
According to statistics, orbital tumors in children account for 25% of all eye cancers. Intraocular lymphomas account for only 1% of them. That is, orbital lymphoma in children is a very rare disease, and children with such a diagnosis should undergo treatment only in specialized modern oncology centers.
The staff at Pediatric Oncology Department at Tel Aviv Medical Clinic have unique experience in managing pediatric patients with orbital lymphomas, so if your child suspects such a diagnosis, do not hesitate! Seek help only from the best Israeli oncologists who work at TAMC.
In their practice, our clinic’s specialists are guided only by modern clinical recommendations and global treatment protocols, so there is no doubt about the quality of medical services provided and their compliance with current international standards.
The undeniable advantage of treating children in Pediatric Oncology Department of TAMC is the individual approach to each individual clinical case. Developing an examination and treatment plan, the doctor does not use one template for everyone, but adjusts the standards of treatment depending on individual characteristics of patient and tumor. This makes it possible to create the most effective personalized treatment regimen for each individual child, which in future only has a positive effect on the long-term prognosis.
Help for a child with ocular lymphoma is provided immediately by a whole team of specialized specialists – oncologists, neurologists, neurosurgeons, ophthalmologists, radiation therapists, chemotherapists, rehabilitation specialists, psychologists and other specialists if necessary. Thus, in one medical institution you can receive all the necessary procedures, starting from diagnostic stage, ending with complex treatment and necessary amount of rehabilitation measures.
Causes of orbital lymphoma in children
Lymphomas of this location are most often found among women, especially between the ages of 50 and 70 years. As already mentioned, such cases are very rare among children.
This type of tumor predominantly affects weakened patients with “poor” immunity (primary and secondary immunodeficiencies).
In addition to above-mentioned risk factors, unfortunately, the exact causes of orbital lymphoma are currently unknown.
There is some evidence that development of these tumors is associated with chronic infection with Chlamydophila psittaci (the bacterium that causes psittacosis, a pulmonary infection transmitted to humans from animals, mainly birds).
Symptoms of orbital lymphoma
The following symptoms may indicate the development of a tumor process in child’s orbit:
- general malaise;
- attacks of very intense headache;
- protrusion of one or two eyeballs from the orbit (exophthalmos);
- pain, pain in eyes, lacrimation, blurred vision and other eye symptoms; redness of eyeball.
The features of orbital lymphoma clinical manifestations completely depend on tumor location: choroid, retina, soft tissue of orbit, eyelids or conjunctiva.
Modern diagnostic methods
To correctly diagnose ocular lymphoma, first it is necessary to consult with a pediatric ophthalmologist. After a complete eyes examination – both view and internal structures, specialist can proceed to establish a preliminary diagnosis and further clarifying examinations. A feature of diagnostic process in TAMC is its speed and complexity, that is, in a short period of time (3-5 working days), patient receives a complete comprehensive examination in one place.
An examination by an ophthalmologist usually includes:
- Thorough external examination of eyeballs.
- Ophthalmoscopy – examination of eye and fundus structures using a special optical system (ophthalmoscope).
- Biomicroscopy – examination of eyeball structures using a powerful microscope.
- Diaphanoscopy is transillumination of eye structures with a light source passing through a dilated pupil. Allows to diagnose intraocular tumors.
- Ultrasound of eyeballs.
- Optical Coherence Tomography (OCT). With OCT, images of thin layers of retina and other structures of eye are obtained, which makes it possible to detect the slightest changes and diagnose tumors in initial stages. Photographic recording of fundus changes using a special RetСam camera.
- Intraocular pressure measuring.
- Electroretinography, which allows us to evaluate electrical activity of retina.
To determine tumor size, its boundaries and spread to neighboring anatomical structures, modern medical imaging methods are used. CT, MSCT, MRI, ultrasound of internal organs and tissues, bone scintigraphy, PET-CT, PET-MRI – these methods can identify distant metastases. Study is used to clarify the stage of the disease and plan a treatment program.
The final diagnosis of orbital lymphoma is established only after pathohistological examination. Usually, for such tumors, a biopsy is performed – taking a tissue sample of tumor conglomerate and then studying it under a microscope. After biopsy, we send all materials for revision to the best pathohistological laboratories in Israel, where they are reviewed by a qualified lymphoma specialist. Thanks to such checks, we are absolutely sure of diagnosis correctness and further chosen treatment tactics.
Also, after biopsy, some patients undergo innovative molecular genetic tests to identify tumor cells characteristics, which makes it possible to select the most effective treatment regimen, for example, innovative targeted drugs (immunobiological therapy).
One such test is Foundation One Comprehensive Genomic Tumor Profiling. This is a line of genetic tests for individual selection of targeted treatment for cancer. The Foundation One study is based on an in-depth analysis of tumor genome, which allows us to identify clinically significant mutations and potentially expand therapeutic options for each patient.
Prognosis for orbital lymphoma in children
The prognosis for eye orbital lymphoma in children depends entirely on stage at which the pathology is diagnosed. If diagnosis is made in initial stages, the disease can be completely cured in 70% of cases. If the tumor has progressed far (stages 3 and 4), long-term remission can be achieved in only 30% of cases.
Application of modern treatment protocols and innovative equipment can increase the chances of recovery. According to statistics, it is in Israel that the largest percentage of patients are completely cured of this disease. TAMC uses only advanced treatment methods, new equipment and modern instruments, allowing surgery to be performed with filigree precision. More than 90% of children permanently overcome intraocular lymphoma, while maintaining their vision.
Modern treatment of orbital lymphoma
Treatment of intraocular lymphoma in children requires a multidisciplinary and comprehensive approach. The choice of a combination of one or another therapy depends on tumor type, its location (only orbit or other parts of the body) and child’s general health. In each individual case, this is decided by a team of several specialists (oncologist, ophthalmologist and others).
Treatment is carried out with chemotherapy, radiation therapy, or a combination of both. If lymphoma has spread to structures of central nervous system, chemotherapy drugs can be injected directly into spinal canal (intrathecal).
For intraocular lymphoma, courses of radiation therapy are often carried out. In many cases, radiation is combined with systemic or intrathecal chemotherapy.
Systemic chemotherapy is given by infusion. In Pediatric Oncology Department, TAMC uses both proven drugs and experimental drugs developed by the largest pharmaceutical concerns in the world.
Also, for orbital lymphoma, a modern procedure is used – chemotherapy, injected directly into vitreous body of eye. Direct injection of chemotherapy drugs into vitreous body of eye in treatment of lymphoma showed excellent results: many patients completely recovered, and among those who recovered there were no relapses of the disease over following years.
Surgical treatment involves enucleation. Complete eyeball removal has been one of the common treatments for intraocular lymphoma in past. Today, it is used very rarely, in cases of ineffectiveness of other treatment methods and extensive damage to vision organ. Thanks to the development of microsurgical technology in ophthalmology, if it is necessary to perform surgery, in many cases it is possible to preserve the visual function and aesthetic appearance of patient.
Immunotherapy is also used for systemic treatment of ocular lymphoma. Its task is to destroy malignant cells and activate antitumor immunity, unmasking the tumor so that it becomes recognizable to immune system. In ophthalmology departments of leading medical centers in Israel, including TAMC, clinical phases of research into new immunotherapy drugs (targeted medications) are actively underway.
In each individual case, the decision on a combination of certain treatment methods is made jointly by a team of specialists. Thanks to this approach, we are able to achieve a high level of patient survival, even in the most difficult cases.
