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| Orbital lymphoma | |
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| Specialty | Oncology  |
Orbital lymphoma is a common type of non-Hodgkin lymphoma that occurs near or on the eye. Common symptoms include decreased vision and uveitis. Orbital lymphoma can be diagnosed via a biopsy of the eye and is usually treated with radiotherapy or in combination with chemotherapy.
There are two main types of intraocular lymphomas: primary central nervous system involvement (PCNSL) and primary central nervous system with ocular involvement (PCNSLO). The difference between PCNSL and PCNSLO is that PNSCL involves the central nervous system, while PCNSLO does not. 56-86% of orbital lymphomas are classified PCNSL and 15-25% are classified PCNSLO. [1] [2] [3] [4]
PCNSLO is common in people who are severely immunosuppressed.
Symptoms of this form of ocular lymphoma include painless decreased vision, sensitivity to light, a red eye, and floaters. Diagnosis is difficult due to its gradual onset and the fact that the symptoms are the same as other diseases.
PCNSLO is usually bilateral, but sometimes grows unevenly. Like other metastatic tumors of the eye, it is usually confined to the choroid. [5]
Primary visible signs of ocular lymphoma include proptosis and a visible mass in the eye. Symptoms are due to mass effect.
Recent studies[ by whom? ] have detected the presence of viral DNA in ocular lymphoma cells. This implies that pathogens play a role in ocular lymphoma. Other studies have found that the aging population, the increasing number of immunosuppressive drugs, and the AIDS epidemic have also contributed to the increased incidence of Non-Hodgkin lymphomas.
Ocular MALT lymphomas may also be associated with Chlamydia psittaci , [6] [7] although whether or not this is the case is still debated. [6]
Follicular lymphoma, diffuse large B cell lymphoma, mantle cell lymphoma, B-cell chronic lymphocytic leukemia, peripheral T-cell lymphoma, and natural killer cell lymphoma have also been reported to affect the orbit.[ citation needed ]
There are two types of ocular lymphomas: intraocular lymphomas and adnexal lymphomas. An intraocular lymphoma occurs within the eye, while an adnexal lymphoma occurs outside, but adjoined to the eye.
Radiotherapy is the most effective treatment for local disease either as the sole treatment for low-grade lymphoma or in combination with chemotherapy for intermediate- and high-grade lymphoma. [8] [9] Radiotherapy dose in range of 30-45 Gy administered in fractions are advised in treating the local orbital lymphomas. [10]
Orbital lymphoma accounts for 55% of malignant orbital tumors in adults. [11] In one study, this was 10% of patients presenting with orbital tumors or similar lesions. [12] Orbital lymphoma is more prevalent in Asia and Europe than in the United States. [13] [14] [15] [16]
Although intraocular lymphoma is rare, the number of cases per year is rising, affecting mainly people in their seventies [17] [18] and immunocompromised patients. [19] [20] A recent study has shown that ocular lymphoma is more prevalent in women than men. [21]
The survival rate is approximately 60% after 5 years.[ citation needed ]
Retinoblastoma (Rb) is a rare form of cancer that rapidly develops from the immature cells of a retina, the light-detecting tissue of the eye. It is the most common primary malignant intraocular cancer in children, and it is almost exclusively found in young children.
Latanoprost, sold under the brand name Xalatan among others, is a medication used to treat increased pressure inside the eye. This includes ocular hypertension and open angle glaucoma. It is applied as eye drops to the eyes. Onset of effects is usually within four hours, and they last for up to a day.
Optic nerve sheath meningiomas (ONSM) are rare benign tumors of the optic nerve. 60–70% of cases occur in middle age females, and is more common in older adults. It is also seen in children, but this is rare. The tumors grow from cells that surround the optic nerve, and as the tumor grows, it compresses the optic nerve. This causes loss of vision in the affected eye. Rarely, it may affect both eyes at the same time.
Intravitreal is a route of administration of a drug, or other substance, in which the substance is delivered into the vitreous humor of the eye. "Intravitreal" literally means "inside an eye". Intravitreal injections were first introduced in 1911 when Ohm gave an injection of air into the vitreous humor to repair a detached retina. In the mid-1940s, intravitreal injections became a standard way to administer drugs to treat endophthalmitis and cytomegalovirus retinitis.
Primary central nervous system lymphoma (PCNSL), also termed primary diffuse large B-cell lymphoma of the central nervous system (DLBCL-CNS), is a primary intracranial tumor appearing mostly in patients with severe immunodeficiency. It is a subtype and one of the most aggressive of the diffuse large B-cell lymphomas. PCNSLs represent around 20% of all cases of lymphomas in HIV infections.. Primary CNS lymphoma is highly associated with Epstein-Barr virus (EBV) infection in immunodeficient patients, and does not have a predilection for any particular age group. Mean CD4+ count at time of diagnosis is ~50/uL. In immunocompromised patients, prognosis is usually poor. In immunocompetent patients, there is rarely an association with EBV infection or other DNA viruses. In the immunocompetent population, PCNSLs typically appear in older patients in their 50s and 60s. Importantly, the incidence of PCNSL in the immunocompetent population has been reported to have increased more than 10-fold from 2.5 cases to 30 cases per 10 million population. The cause for the increase in incidence of this disease in the immunocompetent population is unknown.
Graves’ ophthalmopathy, also known as thyroid eye disease (TED), is an autoimmune inflammatory disorder of the orbit and periorbital tissues, characterized by upper eyelid retraction, lid lag, swelling, redness (erythema), conjunctivitis, and bulging eyes (exophthalmos). It occurs most commonly in individuals with Graves' disease, and less commonly in individuals with Hashimoto's thyroiditis, or in those who are euthyroid.
Orbital cellulitis is inflammation of eye tissues behind the orbital septum. It is most commonly caused by an acute spread of infection into the eye socket from either the adjacent sinuses or through the blood. It may also occur after trauma. When it affects the rear of the eye, it is known as retro-orbital cellulitis.
Iridodialysis is a localized separation or tearing away of the iris from its attachment to the ciliary body.
Eye neoplasms can affect all parts of the eye, and can be a benign tumor or a malignant tumor (cancer). Eye cancers can be primary or metastatic cancer. The two most common cancers that spread to the eye from another organ are breast cancer and lung cancer. Other less common sites of origin include the prostate, kidney, thyroid, skin, colon and blood or bone marrow.
Presumed ocular histoplasmosis syndrome (POHS) is a syndrome affecting the eye, which is characterized by peripheral atrophic chorioretinal scars, atrophy or scarring adjacent to the optic disc and maculopathy.
Intraocular lymphoma is a rare malignant form of eye cancer. Intraocular lymphoma may affect the eye secondarily from a metastasis from a non-ocular tumor or may arise within the eye primarily. PIOL is a subset of primary central nervous system lymphoma (PCNSL). PCNSL are most commonly a diffuse large B-cell immunohistologic subtype of non-Hodgkin's lymphoma according to the World Health Organization (WHO) classification of lymphomas. The most common symptoms of PIOL include blurred or decreased vision due to tumor cells in the vitreous. Most cases of PIOL eventuate to central nervous system involvement (PCNSL) while only 20% of PCNSL lead to intraocular (PIOL) involvement. PIOL and PCNSL remain enigmas because both structures are immunologically privileged sites and so do not normally have immune cells trafficking through these structures. What is more, while the vast majority of PCNSL in patients with acquired immune deficiency syndrome (AIDS) is related to the Epstein-Barr virus (EBV), the development of PCNSL and PIOL in immunocompetent patients is unknown and shows no general relation to infectious DNAs.
Optic disc drusen (ODD) are globules of mucoproteins and mucopolysaccharides that progressively calcify in the optic disc. They are thought to be the remnants of the axonal transport system of degenerated retinal ganglion cells. ODD have also been referred to as congenitally elevated or anomalous discs, pseudopapilledema, pseudoneuritis, buried disc drusen, and disc hyaline bodies.
Marginal zone B-cell lymphomas, also known as marginal zone lymphomas (MZLs), are a heterogeneous group of lymphomas that derive from the malignant transformation of marginal zone B-cells. Marginal zone B cells are innate lymphoid cells that normally function by rapidly mounting IgM antibody immune responses to antigens such as those presented by infectious agents and damaged tissues. They are lymphocytes of the B-cell line that originate and mature in secondary lymphoid follicles and then move to the marginal zones of mucosa-associated lymphoid tissue, the spleen, or lymph nodes. Mucosa-associated lymphoid tissue is a diffuse system of small concentrations of lymphoid tissue found in various submucosal membrane sites of the body such as the gastrointestinal tract, mouth, nasal cavity, pharynx, thyroid gland, breast, lung, salivary glands, eye, skin and the human spleen.
Diktyoma, or ciliary body medulloepithelioma, or teratoneuroma, is a rare tumor arising from primitive medullary epithelium in the ciliary body of the eye. Almost all diktyomas arise in the ciliary body, although, rarely, they may arise from the optic nerve head or retina.
Panophthalmitis is the inflammation of all coats of the animal eye including intraocular structures. It can be caused by infection, particularly from Pseudomonas species, such as Pseudomonas aeruginosa, Clostridium species, Whipple's disease, and also fungi. It can also be cause by other stress.
Conjunctival squamous cell carcinoma and corneal intraepithelial neoplasia comprise what are called ocular surface squamous cell neoplasias. SCC is the most common malignancy of the conjunctiva in the US, with a yearly incidence of 1-2.8 per 100,000. Risk factors for the disease are exposure to sun, exposure to UVB, and light-colored skin. Other risk factors include radiation, smoking, HPV, arsenic, and exposure to polycyclic hydrocarbons.
Santosh Gajanan Honavar is an Indian ophthalmologist and is currently the editor of the Indian Journal of Ophthalmology and Indian Journal of Ophthalmology - Case Reports, the official journals of the All India Ophthalmological Society; Director, Medical Services ; Director, Department of Ocular Oncology and Oculoplasty at Centre for Sight, Hyderabad; and Director, National Retinoblastoma Foundation.
Sarah Coupland is an Australian-born pathologist and professor who is the George Holt Chair in Pathology at the University of Liverpool. Coupland is an active clinical scientist whose research focuses on the molecular genetics of cancers, with particular interests in uveal melanoma, conjunctival melanoma, intraocular and ocular adnexal lymphomas and CNS lymphoma. Coupland is also an NHS Honorary Consultant Histopathologist at the Royal Liverpool University Hospital. Since 2006, Coupland has been head of the Liverpool Ocular Oncology Research Group; from which she runs a multidisciplinary oncology research group focussing on Uveal melanoma, based in the Department of Molecular and Clinical Cancer Medicine at the University of Liverpool. Her research laboratory is currently located in the Institute of Translational Medicine From April 2014 to December 2019, Coupland was also Director of the North West Cancer Research Centre, @UoL. In both 2019 and 2020, Coupland was included on the 'Pathology Powerlist' on The Pathologist website.
Robert M. Schertzer is a Canadian ophthalmologist with a subspecialty in glaucoma treatment. He currently serves as the Clinical Associate Professor of Ophthalmology and Visual Sciences at the University of British Columbia.
Orbital emphysema is a medical condition that refers to the trapping of air within the loose subcutaneous around the orbit that is generally characterized by sudden onset swelling and bruising at the impacted eye, with or without deterioration of vision, which the severity depends on the density of air trapped under the orbital soft tissue spaces.