Primary orbital lymphoma

Primary orbital lymphomas are a rare subset of tumors constituting 1-2% of non-Hodgkin lymphoma. They are mostly indolent B-cell lymphomas presenting with gradual progressive proptosis, decreased visual acuity, restricted ocular mobility, and diplopia. The role of surgery is mainly for obtaining a biopsy. Most of these tumors require multimodality treatment including chemotherapy, radiation, or both, which have major role.

Case description: We report one such case of marginal zone lymphoma of the orbit in a female with significant proptosis who was treated with multimodality treatment, including surgical excision as a major treatment modality. Decompression of symptomatic proptosis was followed by chemotherapy and radiation.

Conclusion: Primary orbital lymphoma is a rare clinical entity with diverse clinical outcomes. It can be successfully managed with surgical excision for decompression of mechanical proptosis followed by chemotherapy, radiation, or both 1).

Fourteen patients (8 females, 6 males - 13 primary orbital lymphomas, 1 systemic lymphoma). Mean age at diagnosis was 65.6 years (from 50 to 80 years). In 2/3 of the patients the lesion was localized in left eye, without a case with bilateral orbital involvement. Seven cases the diagnosis was made 6 months from the onset of symptoms, 5 cases - 1 year and 2 cases - more than one year. The most common clinical symptoms were exophthalmus (12 case), tumor or bulging mass of the eyelid - 8, diplopy - 6, decreased visual acuity - 5. Intraconal localization of the tumor was in 6 cases, extraconal - 2. intra-extraconal - 6. The diagnosis was made by neuro-ophthalmologist and precised with the aid of CT and MRI. All the patients were operated on with the following approaches - fronto-orbital - 9 cases, lateral - 3, anterior orbitotomy (without bone resection) - 2 cases. In six cases “'gross total” resection was achieved, in the rest of the cases the resection was partial. On histological examination, a high grade lymphoma was established in 2 cases with primary orbital lymphoma and one case with systemic lymphoma. All other cases were diagnosed as low-grade B-call small lymphocytic non-Hodgkin's lymphoma. Long-term survival (over 5 years) was observed in 4 cases with primary lymphoma, whereas the patient with systemic form of the disease died 10 months after the operation 2).

present a series of 3 patients with orbital lymphoma and 1 with GO treated with stereotactic intensity modulated radiation therapy (IMRT) to spare normal structures, including the lens. Three patients with orbital lymphomas and 1 with unilateral GO were treated with IMRT using a linac with stereotactic capabilities. Avoidance structures included the ipsilateral lens and globe, the contralateral lens and globe, the chiasm, and the brainstem. Two patients with orbital lymphoma were treated with 24 Gy in 12 fractions, and 1 patient was treated with 30.6 Gy in 17 fractions. The patient with GO was treated with 20 Gy in 10 fractions. The dosimetry was analyzed to determine the dose to normal tissues. Patient follow-up varies between 8 and 24 months. The mean minimal dose to the ipsilateral lens was 13.6% of the total dose, the mean maximal dose was 33.5%, and the mean median dose was 19.3%. The mean median dose to the contralateral eye was 1.1% of the total dose. The mean median dose to the chiasm was 14.9% of the total dose. The mean median dose to the brainstem was 1.9% of the total dose. No cataracts or other complications were noted in the 4 patients treated with this technique so far. IMRT gives a more conformal treatment to the orbital contents while sparing normal tissues such as the ipsilateral lens and adjacent critical structures. This should result in fewer complications such as cataracts 3).

Borkar AU, Jain K, Jain VK. A large primary orbital lymphoma with proptosis: A case report and review. Surg Neurol Int. 2018 Dec 4;9:249. doi: 10.4103/sni.sni_270_18. PMID: 30603233; PMCID: PMC6293602.
Tzekov Ch, Spiriev T, Hristova S, Chernikova S, Minkin K, Naydenov E, Bussarsky V, Romansky K, Marinov M, Kalev O, Cekov A, Laleva L, Kolarov D, Tanova R, Enchev V. [Orbital lymphoma]. Khirurgiia (Sofiia). 2009;(4-5):19-23. Bulgarian. PMID: 20506800.
Chino K, Tanyi JA, Stea B. Stereotactic radiotherapy for unilateral orbital lymphoma and orbital pseudo-tumors: a planning study. Med Dosim. 2009 Spring;34(1):57-62. doi: 10.1016/j.meddos.2007.12.004. Epub 2008 Mar 28. PMID: 19181257.
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