Edit concept Question Editor Create issue ticket

Orbital Lymphoma

Primary Orbit Lymphoma

Orbital lymphoma, most commonly arising as a non-Hodgkin, mucosal-associated lymphoid tissue (MALT) lymphoma, is primarily seen in the elderly population. The clinical presentation is comprised of nonspecific signs - a painless palpable mass accompanied by swelling, ptosis, excessive tearing, and irritation of the eye. The diagnosis is made through the use of imaging studies, such as computed tomography and magnetic resonance imaging, whereas a surgical biopsy is necessary to confirm the exact subtype.


Presentation

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].

Soft Tissue Mass
  • A 72-year-old woman presented with a diffuse infiltrating soft tissue mass involving the entire right orbit. She had a pre-existing phthisis of the right eye secondary to retinal detachment, and had developed painless proptosis on the same side.[ncbi.nlm.nih.gov]
  • Orbital lymphoma usually appears as a soft tissue mass, either involving the conjunctiva (especially in the case of orbital adnexal MALT lymphoma (OAML)) or elsewhere in the orbit, frequently in the upper outer quadrant, closely associated with the lacrimal[radiopaedia.org]
  • Cystic retrobulbar lesions (such as dermoid and epidermoid cyst or mucocele) have different density (on CT) and intensity (on MRI) from soft tissue mass.[link.springer.com]
Euthyroid
  • Subsequent thyroid testing revealed euthyroid status and computed tomography scan revealed orbital lymphoma.[ncbi.nlm.nih.gov]
Pallor
  • There was no evidence of chorioretinal folds, optic nerve oedema, or pallor. Hertel exophthalmometry readings revealed a 5-mm protrusion of the right eye, but no restriction of eyeball movement or binocular diplopia.[bmcophthalmol.biomedcentral.com]
Generalized Lymphadenopathy
  • A 44-year-old man (Patient #1) and a 50-year-old man (Patient #2) presented with generalized lymphadenopathy due to grade 1 follicular lymphoma proven on lymph node biopsy.[ncbi.nlm.nih.gov]
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]
  • Discussion Orbital Lymphoma This patient presented with acute awareness of a long-standing problem: fullness of upper lid, binocular diplopia, and injection OS.[webeye.ophth.uiowa.edu]
Unilateral Proptosis
  • proptosis and diffuse involvement of the cra nial vault and brain parenchyma is extremely rare.[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]
Bilateral Proptosis
  • The most frequent cause of bilateral proptosis is Graves' ophthalmopathy, and when it is associated with weight loss in an elderly patient, the initial diagnostic consideration is thyrotoxic Graves' disease.[ncbi.nlm.nih.gov]
  • Physical examination ( Fig. 1, upper panel ) showed marked, bilateral proptosis, soft tissue inflammation, and mild impairment of ocular movement ( 1 ). There was no upper lid retraction.[academic.oup.com]
Unilateral Proptosis
  • proptosis and diffuse involvement of the cra nial vault and brain parenchyma is extremely rare.[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]
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]
Responsiveness Decreasing
  • The following degrees of response were considered in the first check-up (after 6-12 months): complete response (disappearance of lesion), partial response (decrease of the size of the tumour of more than 50 % of the longest diameter), stable disease ([link.springer.com]

Workup

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].

Lymphocytopenia
  • This is the first case, to the authors' knowledge, of orbital lymphoma with idiopathic CD4 lymphocytopenia.[ncbi.nlm.nih.gov]

Treatment

  • In some cases, combining such systemic therapy with local radiation treatment is beneficial.[ncbi.nlm.nih.gov]

Prognosis

  • 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]

Etiology

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

Epidemiology

  • 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[jnci.oxfordjournals.org]
  • Epidemiology: Orbital lymphoma is more prevalent in Europe and Asia than in the United States. Incidence of Orbital lymphoma increases as the age advances.[oculoplastic-eyelid-orbit-surgery.com]
  • Diagnosis: Orbital Lymphoma EPIDEMIOLOGY Incidence: in Florida, 2 cases per million Age: all ages Among the most common orbital tumors seen in the adults: cavernous hemangioma, lymphoid tumors, and meningiomas SIGNS Salmon patch lesion on globe CT scan[webeye.ophth.uiowa.edu]
Sex distribution
Age distribution

Pathophysiology

  • 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]

Prevention

  • 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]
  • 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]
  • 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]
  • 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]

References

Article

  1. Eckardt AM, Lemound J, Rana M, Gellrich N-C. Orbital lymphoma: diagnostic approach and treatment outcome. World J Surg Oncol. 2013;11:73.
  2. Demirci H, Shields CL, Shields JA, et al. Orbital tumors in the older adult population. Ophthalmology. 2002;109:243–248.
  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.
  5. Tsang RW, Gospodarowicz MK, Pintilie M, et al. Localized mucosa- associated lymphoid tissue lymphoma treated with radiation therapy has excellent clinical outcome. J Clin Oncol. 2003;21:4157–4164.
  6. Maksimovic O, Bethge WA, Pintoffl JP, et al. Marginal zone B-cell non-Hodgkin's lymphoma of mucosa-associated lymphoid tissue type: imaging findings. AJR Am J Roentgenol. 2008;191(3):921-930.
  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.

Ask Question

5000 Characters left Format the text using: # Heading, **bold**, _italic_. HTML code is not allowed.
By publishing this question you agree to the TOS and Privacy policy.
• Use a precise title for your question.
• Ask a specific question and provide age, sex, symptoms, type and duration of treatment.
• Respect your own and other people's privacy, never post full names or contact information.
• Inappropriate questions will be deleted.
• In urgent cases contact a physician, visit a hospital or call an emergency service!
Last updated: 2019-07-11 21:41