Primary lymphoma of the orbit corresponds to one of the commonest orbital tumours and accounts for as much as half of all orbital malignancies. It is a B-cell non-Hodgkin lymphoma, and in most cases arises from mucosa-associated lymphoid tissue.
Orbital lymphomas account for only 2% of all lymphomas, but constitute 5-15% of all extranodal lymphomas and approximately 50% of all primary orbital malignancies in adults 1,6.
This possible infective aetiology may explain the probable increase in incidence of orbital lymphomas (see below) 3.
Typically patients are between 50 and 70 years of age, and no gender predilection is recognised 6.
Clinical presentation is variable as any part of the orbit can be involved. In 25% of patients the conjunctiva is involved in which case patients demonstrate a 'salmon red patch' of swollen conjunctiva 1.
The majority of patients who do not have conjunctival involvement (75% of cases) presentation is due to an orbital mass, usually in the superior lateral quadrant, in proximity to the lacrimal gland 1,6:
- palpable mass
- diplopia and abnormal ocular movement
Generally the mass is painless, however a subset of patients demonstrate inflammatory-like changes with pain, erythaema and swelling 1.
Primary orbital lymphoma (by definition) is Stage 1E disease (see: staging of non-Hodgkin lymphoma).
Orbital lymphoma is a B-cell non-Hodgkin lymphoma, and in most cases arises from mucosa-associated lymphoid tissue (i.e. orbital adnexal MALT lymphoma (OAML)): 50-78% of cases in Western nations, and up to 90% of cases from Japan and Korea 1. It is unclear however if MALT exists normally in the conjunctiva of the eye, or whether it represents the sequelae of inflammation (thus the potential link to chlamydial infection). The orbit is otherwise believed to be bereft of lymphatic tissue or lymphatic drainage 1.
Non OAML tumours are of a variety of histologies, with diffuse large B-cell lymphoma being relatively common 4.
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. Patients may go on to develop psittacosis, usually with respiratory tract manifestations 3.
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 gland.
Although the extraocular muscles may be surrounded or displaced by the mass, they can usually be identified as not being the origin of the tumour, helpful in distinguishing lymphomas from other orbital masses.
Invasion of the globe or optic nerve is rare.
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On non-contrast CT, the mass is usually homogeneous in density, either isodense or slightly hyperdense when compared to the extraocular muscles 1. Following administration of contrast, only mild to moderate enhancement is seen, similar again to the extraocular muscles and lacrimal gland.
Similar to intracranial lymphoma, the densely cellular nature of these tumours with high nucleus-to-cytoplasm ratio results in relatively specific appearances 1,5:
Signal characteristics include:
- T1: iso to hypointense to muscle
- T2: iso to hyperintense to muscle
- T1 C+ (Gd): homogeneous enhancement
- DWI: increased signal intensity-restricted diffusion
- ADC: reduced values-restricted diffusion
Treatment and prognosis
Orbital adnexal MALT lymphoma (OAML) have a better prognosis than other types of orbital lymphomas, and are not surprisingly more often conjunctival. The recent identification of Chlamydia psittaci as a likely important causative factor in the increasing incidence of orbital lymphomas, has lead to antibiotic therapy being used to not only reduce the size of the tumour, but in some cases result in remission 1-3.
Surgical biopsy/resection, radiotherapy and chemotherapy have all been used and currently no OAML treatment guidelines exist 1. Overall a 65% 5-year relapse-free rate is reported, with less than 5% of patients with OAML eventually dying from the disease 1. Systemic dissemination is only seen in 5-10% of cases 1.
In cases of non-MALT-lymphoma, then similarly a combination of two or more of surgical biopsy/resection, radiotherapy and chemotherapy are usually employed. Prognosis is less favourable.
General imaging differential considerations include:
- other malignancies
idiopathic orbital inflammatory pseudotumor
- may be difficult to distinguish from lymphoma
- if associated with fibrosis, will be of lower T2 signal 5
- thyroid associated orbitopathy
- overview of lymphoma
WHO classification of tumours of haematopoietic and lymphoid tissues
- Hodgkin lymphoma
mature B-cell lymphoma
- Burkitt lymphoma
- follicular lymphoma
- lymphoplasmacytic lymphoma (Waldenström's macroglobulinaemia)
- lymphomatoid granulomatosis
- mantle cell lymphoma
- mature T-cell and NK-cell lymphoma
- post-transplant lymphoproliferative/lymphoproliferation disorders
- mature B-cell lymphoma
- location-specific lymphomas
- central nervous system
- head and neck lymphoma
- thoracic lymphoma
- gastrointestinal lymphoma
- hepatobiliary lymphoma
- genitourinary lymphoma
- musculoskeletal lymphoma
- cutaneous lymphoma
- 1. 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): 835-46. doi:10.1093/annonc/mdm513 [pubmed citation]
- 2. Ferreri AJ, Guidoboni M, Ponzoni M et-al. Evidence for an association between Chlamydia psittaci and ocular adnexal lymphomas. J. Natl. Cancer Inst. 2004;96 (8): 586-94. doi:10.1093/jnci/djh102 [pubmed citation]
- 3. Moslehi R, Devesa SS, Schairer C et-al. Rapidly increasing incidence of ocular non-hodgkin lymphoma. J. Natl. Cancer Inst. 2006;98 (13): 936-9. doi:10.1093/jnci/djj248 [pubmed citation]
- 4. Tomohiko Mannami, Tadashi Yoshino, Koichi Oshima, Sumie Takase, Eisaku Kondo, Nobuya Ohara, Hideki Nakagawa, Hiroshi Ohtsuki, Mine Harada, Tadaatsu Akagi. Clinical, Histopathological, and Immunogenetic Analysis of Ocular Adnexal Lymphoproliferative Disorders: Characterization of MALT Lymphoma and Reactive Lymphoid Hyperplasia Modern Pathology. 14 (7): 641. doi:doi:10.1038/modpathol.3880366
- 5. 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): 921-30. doi:10.2214/AJR.07.2629 [pubmed citation]
- 6. Orbital tumors. Zeynel A. Karcioglu; with 482 illustrations, 276 in color. New York : Springer, c2005. ISBN:038721321X (find it at amazon.com)
Synonyms & Alternative Spellings
|Synonyms or Alternative Spelling||Include in Listings?|
|Lymphoma of the orbit||✓|
|Orbital adnexal MALT lymphoma||✓|
|Orbital adnexal MALT lymphoma (OAML)||✗|
|Primary lymphoma of the orbit||✓|
|Primary orbital lymphoma||✓|
|Orbital adnexal MALT-lymphoma||✗|
|Primary orbital lymphomas||✗|