Orbital lymphomas, although regarded as a rare entity (encompassing only 1% of all non-Hodgkin lymphomas), are one of the most important tumors arising from the orbit, encompassing up to 25% of all tumors in certain studies [1] [2] [3]. The main locations of lymphoma within the orbit are the conjunctiva, the lacrimal apparatus, the eyelid, and the extraocular muscles [4] [5]. This malignancy is principally diagnosed in the elderly population (> 60 years of age), and a strong predilection toward female gender (male-to-female ratio of 1:2.9) was observed [1] [2] [3]. The most common type of lymphoma in the orbit and the adnexa is MALT lymphoma, but various types, both low-grade and high-grade, have been described [1] [2] [6] [7]. The clinical presentation of orbital lymphoma consists of a progressively enlarging and painless orbital mass, followed by ptosis, proptosis, eye irritation, excessive tearing, and visual disturbances [4]. An insidious course is rather common, which is the primary reason why the diagnosis is often delayed for a significant period of time (weeks, months, or even years may pass from the appearance of symptoms) [1] [8]. The importance of early recognition further lies in the fact that survival rates are estimated at 64% for 5 years and 42% for 10 years, with up to a third of patients suffering from disease recurrence [2].

• Swelling

  We present the case of a 72-year-old woman who consulted us with a prominent unattractive swelling of both lower eyelids. [ncbi.nlm.nih.gov]

• Short Stature

  stature-facial dysmorphism-hypergonadotropic hypogonadism syndrome Moyamoya disease Moyamoya disease-short stature-facial dysmorphism-hypergonadotropic hypogonadism Moyamoya disease with early-onset achalasia Moynahan syndrome MPA MPAN MPD MPD1 MPD3 [orpha.net]

• Diplopia

  An 82-year-old man with hypothyroidism had vertical diplopia and swelling around his left eye. Visual acuity was 20/20 OD and 20/50 OS. There was moderate blepharoptosis and edema of the left eyelids and superior scleral show of the right eye. [ncbi.nlm.nih.gov]

  Three months after the initiation of chemotherapy, strabismus and diplopia had resolved, and proptosis markedly decreased, although there was no change in her BCVA. [bmcophthalmol.biomedcentral.com]

  Ptosis and decreased levator function may indicate superior orbital and levator muscle involvement, and motility should also be measured if the patient complains of diplopia. [eyewiki.aao.org]

• Unilateral Proptosis

  proptosis and diverse features due to diffuse involvement of cranial vault and brain parenchyma. [3] Our present case had unilateral proptosis and diffuse involvement of cranial vault, but because of lack of MRI facilities it was not possible to demonstrate [cancerjournal.net]

  proptosis in adults) References Hasegawa M, Kojima M, Shioya M, Tamaki Y, Saitoh J, Sakurai H, Kitamoto Y, Suzuki Y, Niibe H, Nakano T. [webeye.ophth.uiowa.edu]

  Strianese D, Piscopo R, Elefante A, Napoli M, Comune C, Baronissi I, Liuzzi R,Ferrara M, D'alessandro A, Ruggiero P, Napolitano P, Grassi P, Iuliano A, RussoC, Brunetti A, Bonavolontà G: Unilateral proptosis in thyroid eye disease with subsequent contralateral [karger.com]

• Visual Impairment

  Search for other works by this author on: Claudio Marcocci Published: 01 September 2009 A 70-yr-old woman 1 was referred because of orbital pain, bilateral proptosis, eyelid swelling, inflammatory symptoms, and visual impairment. [academic.oup.com]

  Restricted ocular motility and visual impairment are the features elicited by the ophthalmologist. [ijo.in]

• Visual Impairment

  Search for other works by this author on: Claudio Marcocci Published: 01 September 2009 A 70-yr-old woman 1 was referred because of orbital pain, bilateral proptosis, eyelid swelling, inflammatory symptoms, and visual impairment. [academic.oup.com]

  Restricted ocular motility and visual impairment are the features elicited by the ophthalmologist. [ijo.in]

• Strabismus

  Three months after the initiation of chemotherapy, strabismus and diplopia had resolved, and proptosis markedly decreased, although there was no change in her BCVA. [bmcophthalmol.biomedcentral.com]

  Optic nerve gliomas may be asymptomatic and discovered incidentally in patients with NF-1, or patients may present with a visual field deficit, vision loss strabismus, or relative afferent pupillary defect. [pubs.rsna.org]

• Chorea

  […] syndrome Morava-Mehes syndrome Morgagni-Stewart-Morel syndrome MORM syndrome Morning glory disc anomaly Morning glory syndrome Morquio disease Morquio disease type A Morquio disease type B Morris syndrome Morse-Rawnsley-Sargent syndrome Morvan's fibrillary chorea [orpha.net]

To diagnose orbital lymphoma in its early stages, and thus significantly improve patient outcomes, the role of the physician in recognizing clinical symptoms through a detailed patient history and a meticulous physical examination is essential. The presence of ocular complaints and an orbital mass in elderly patients must prompt the physician to conduct additional tests, including a full laboratory workup (a complete blood count, liver transaminases, kidney function tests, and a peripheral blood smear) [4], but more importantly, imaging studies. Computed tomography (CT) and magnetic resonance imaging (MRI) are both recommended for the evaluation of orbital lymphomas [4] [6] [8]. This tumor appears as a well-defined homogeneous lesion (sometimes having a hyperdense appearance compared to extraocular muscles is observed) without the involvement of bone on both CT and MRI [4] [6] [8]. Additional MRI findings include a high signal intensity on T2-weighted images and low signal intensity on T1-weighed images, as well as high diffusion-weighted imaging (DWI) signal and low apparent diffusion coefficient (ADC) values [6] [8]. In addition to CT and MRI, orbital ultrasonography has also been described as a potential diagnostic tool [8]. A definite diagnosis and determination of tumor stage are achieved after performing a surgical biopsy and subsequent histopathological examination, together with previously mentioned imaging procedures [1] [4] [8].

• Chlamydia

  A multiplex touchdown enzyme time-release PCR assay designed to simultaneously detect Chlamydia trachomatis, Chlamydia pneumoniae, and C psittaci was performed. Authenticity of the PCR-amplified products was verified by Southern blot hybridization. [ncbi.nlm.nih.gov]

  Recently an association between Chlamydia psittaci infection and orbital adnexal MALT lymphoma has been described 1-2. Infection by Chlamydia psittaci is usually the result of exposure to infected birds and household pets. [radiopaedia.org]

  Nadine Radomski, Rebekka Einenkel, Anne Müller and Michael R Knittler, Chlamydia–host cell interaction not only from a bird's eye view: some lessons from Chlamydia psittaci, FEBS Letters, 590, 21, (3920-3940), (2016). [doi.org]

  A single case associating Chlamydia trachomatis –induced adult inclusion conjunctivitis with conjunctival EMZL was reported. 37 Recently, Ferreri et al 22 examined whether Chlamydia psittaci, trachomatis, and pneumoniae infections may be associated with [bloodjournal.hematologylibrary.org]

• Polyps

  […] double-sided deafness in children and adults Surgery of the oral cavity and oropharynx Tonsillectomy, surgery to remove benign and malignant tumors Larynx and pharynx surgery Endoscopic surgery (microlaryngoscopy) of benign lesions on the local cords (polyps [bookinghealth.com]

  […] osteitis of the clavicle Median arcuate ligament syndrome Median cleft face syndrome Median cleft lip/mandibule Median cleft lower facial stage Median cleft of the upper lip and maxilla Median cleft of the upper lip-corpus callosum lipoma-cutaneous polyps [orpha.net]

Most patients received treatment with 250 kVP or 60Co radiation, using either an en face anterior field or wedged anterior and lateral fields. Median treatment dose was 25 Gy. Lens shielding was performed if possible. [ncbi.nlm.nih.gov]

OBJECTIVE: To explore the relationship between IgH gene rearrangement and orbital MALT (mucose-associated lymphoid tissue) lymphoma removal operation prognosis, and to quantify the effect of IgH gene rearrangement on primary orbital MALT lymphoma prognosis [ncbi.nlm.nih.gov]

and to quantify the effect of IgH gene rearrangement on primary orbital MALT lymphoma prognosis. [europeanreview.org]

Jakobiec FA, Iwamoto T, Patell M, et al: Ocular adnexal monoclonal lymphoid tumors with a favorable prognosis. Ophthalmology 1986; 93:1547-1557. 3. [healio.com]

Although tracheal MALT lymphoma is extremely rare, it is associated with favorable prognosis and long term survival. [archbronconeumol.org]

These different microorganisms may play similar roles in the etiology of orbital MALT lymphomas from different geographic regions. Antibiotic therapy could be considered for orbital MALT lymphomas associated with positive infection. [ncbi.nlm.nih.gov]

Discussion Primary OAL is a distinct clinical entity in which natural history, etiology, and pathogenesis are not well characterized. [bloodjournal.hematologylibrary.org]

The possible infective etiology may explain the probable increase in the incidence of orbital lymphomas (see below) 3. Typically patients are between 50 and 70 years of age, with no recognized gender predilection 6. [radiopaedia.org]

Etiology Tumors arise from germinal center cells (follicular lymphoma), mantel cells (mantle cell lymphoma) or memory B cells (extranodal marginal zone lymphoma) all of which have undergone antigen exposure. [eyewiki.aao.org]

Article Navigation Affiliation of authors: Division of Cancer Epidemiology and Genetics, National Cancer Institute, NIH, DHHS, Bethesda, MD Correspondence to: Roxana Moslehi, PhD, Division of Cancer Epidemiology and Genetics, National Cancer Institute [doi.org]

They also present the epidemiological, clinical, radiological, macroscopic and microscopic features of this entity. 2 Citations References SHOWING 1-10 OF 21 REFERENCES [semanticscholar.org]

Pathophysiology Immunosuppression due to any cause—including AIDS, immunosuppressive drugs, or increasing age—has long been established as the main factor contributing to the pathophysiology of orbital lymphoma. [aao.org]

Pathophysiology and Natural History Conjunctival lymphoma can appear clinically identical to reactive lymphoid hyperplasia. Both conditions are examples of lymphoproliferative neoplasms and must be carefully distinguished. [webeye.ophth.uiowa.edu]

Pathophysiology of neurofibromatosis type 1. Ann Intern Med 2006 ;144(11):842–849. Google Scholar 40 Aydin MD, Yildirim U, Gundogdu C, Dursun O, Uysal HH, Ozdikici M. [pubs.rsna.org]

The lenses are blocked to prevent cataract formation. However, blocking of the lens by traditional methods can be difficult for tumors located anteriorly and extending into the retrobulbar space. [ncbi.nlm.nih.gov]

While differential diagnoses in this case are extensive, early diagnosis may prevent or disclose further systemic spread. [pmj.bmj.com]

How can MALT Lymphoma of Orbit be Prevented? Currently, it is not possible to prevent MALT Lymphoma of Orbit. However, controlling certain factors my help lower one’s risk for the condition. [dovemed.com]

Lid infiltration prevented right fundus examination, but a B-scan revealed a serous retinal detachment and an intraocular mass. Figure 1 Mass on the temporal area of superior right lid. [bjo.bmj.com]

OS no difference Conclusion: IFRT able to prevent local relapse MSKCC, 2005 - PMID 15936555 — "Radiation treatment planning techniques for lymphoma of the stomach." Della Biancia C et al. Int J Radiat Oncol Biol Phys. 2005 Jul 1;62(3):745-51. [en.wikibooks.org]

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3. Ahmed S, Shahid RK, Sison CP, Fuchs A, Mehrotra B. Orbital lymphomas: a clinicopathologic study of a rare disease. Am J Med Sci. 2006;331:79–83.
4. Yadav BS, Sharma SC. Orbital lymphoma: Role of radiation. Indian Journal of Ophthalmology. 2009;57(2):91-97.
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7. Decaudin D, de Cremoux P, Vincent-Salomon A, Dendale R, Rouic LL. Ocular adnexal lymphoma: a review of clinicopathologic features and treatment options. Blood. 2006;108:1451–1460.
8. Ferreri AJ, Dolcetti R, Du MQ, et al. Ocular adnexal MALT lymphoma: an intriguing model for antigen-driven lymphomagenesis and microbial-targeted therapy. Ann Oncol. 2008;19(5):835-846.