Orbital lymphoproliferative lesions compromise a wide spectrum of diseases ranging from benign to malignant.
Patient's may present with a palpable mass or proptosis. Pain is an uncommon symptom unlike orbital pseudotumour, which manifests with acute pain.
These lesions represent a spectrum of disorders, including:
- lymphoid hyperplasia of the orbit (benign) 10-40% 1
- atypical lymphoid hyperplasia of the orbit
- ocular adnexal lymphoma (NHL) (malignant) most common 60-90% 1
Lesions may be a manifestation of systemic lymphoma or arise primarily from the orbit.
The major long-term risk is a development of systemic lymphoma (33-50%) in 10 years after the initial diagnosis of an orbital lymphoproliferative disorder. 1
- may be seen as a solid, homogeneously enhancing orbital tumour that moulds to and encases orbital structures
- It can occur anywhere in the orbit with predilection for anterior extraconal space (often in the superotemporal quadrant) and lacrimal gland (in 40% of cases) 2
- bilateral in 25% of patients 1
- iso-attenuating to slightly hyperattenuating (highly cellular).
- diffuse homogeneous post contrast enhancement
- bone destruction indicates aggressive histology
Modality of choice for evaluating location and extent of disease.1
- T1: mildly hyperintense to muscle; homogenous
- T2: mildly hyperintense to muscle
- T1 WI C+: moderate to marked homogeneous enhancement
On imaging, possible considerations include
- idiopathic orbital inflammatory pseudotumour
- orbital cellulitis
- orbital metastasis
- orbital sarcoidosis
- orbital Sjogren syndrome
- 1. Harnsberger HR, Glastonbury CM, Michel MA et-al. Diagnostic Imaging: Head and Neck. Lippincott Williams & Wilkins. (2010) ISBN:1931884781. Read it at Google Books - Find it at Amazon
- 2. Tailor TD, Gupta D, Dalley RW et-al. Orbital neoplasms in adults: clinical, radiologic, and pathologic review. Radiographics. 2013;33 (6): 1739-58. doi:10.1148/rg.336135502 - Pubmed citation
- 3. Haradome K, Haradome H, Usui Y et-al. Orbital lymphoproliferative disorders (OLPDs): value of MR imaging for differentiating orbital lymphoma from benign OPLDs. AJNR Am J Neuroradiol. 2014;35 (10): 1976-82. doi:10.3174/ajnr.A3986 - Pubmed citation
- 4. Valvassori GE, Sabnis SS, Mafee RF et-al. Imaging of orbital lymphoproliferative disorders. Radiol. Clin. North Am. 1999;37 (1): 135-50, x-xi. Pubmed citation