Table 1 Demographic and clinical characteristics in 18 patients with neurolymphomatosis in the orbit associated with lymphoma
Patient # | Age,y/ sex | Lymphoma subtype | Prior disease sites | Time to recurrence | Symptoms | Treatment |
|---|---|---|---|---|---|---|
1 | 64/M | DLBCL | NA | NA | maxillary sinus mass with orbital extension, periorbital edema and epiphora | CHOP/Bleo followed by interferon |
2 | 72/F | DLBCL | Skull base | 10 years | left cheek mass with cranial neuropathies and diplopia | R-ESHAP, R-CHOP |
3 | 70/F | DLBCL | Uterus | 1.5 years | facial palsy and trigeminal neuralgia | R-CHOP radiation (30 Gy) |
4 | 73/M | DLBCL | NA | NA | maxillary sinus/cheek mass with infraorbital nerve PET/CT activity | R-CHOP, stem cell transplant |
5 | 51/M | DLBCL | NA | NA | lacrimal gland/orbital mass with optic disc edema and optic neuropathy | R-DHAP |
6 | 59/M | DLBCL | NA | NA | diplopia/maxillary sinus mass with secondary orbital involvement | R-CHOP |
7 | 60/M | DLBCL | NA | NA | presented with nasopharyngeal mass with secondary orbital involvement; also diplopia due to multiple cranial neuropathies including 3rd, 4th and 6th nerves | R-CHOP |
8 | 51/M | DLBCL | Eyebrow | 6 months | presented with an eyebrow mass (which was lymphoma on biopsy) as well as a maxillary sinus mass with V1 infiltration on MRI | R-CHOP, methotrexate |
9 | 75/M | DLBCL | Head & neck nodes | 2 years | presented with cheek mass with V2 and pterygopalatine involvement | R-EPOCH, methotrexate |
10 | 58/F | Marginal zone | Chest | 11 years | proptosis | Rituxan-hyper CVAD |
11 | 62/M | Marginal zone | Chest, abdomen | 1 year | presented with an asymptomatic orbital/pterygoplatine infiltrative mass that was lymphoma; otherwise asymptomatic | ultra low dose XRT (4 Gy) |
12 | 24/M | Marginal zone | NA | NA | Long standing visual loss and optic neuropathy also with orbital and pterygopalatine mass with intracranial extension biopsy proven to be lymphoma | R-EPOCH, methotrexate |
13 | 54/M | Low-grade B-cell | NA | NA | presented with a posterior orbital mass; no specific ocular or periocular symptoms | Ibrutinib, Rituxan and Temodar |
14 | 72/F | Low-grade B-cell | NA | NA | proptosis/orbital mass | SMILE with IT chemotherapy |
15 | 69/M | Mantle cell | NA | NA | presented with upper eyelid and periorbital lesions; MRI showed bilateral orbital masses | R-CHOP |
16 | 48/F | Mantle cell | Ovary | 9 months | facial and jaw pain and parasthesias on the cheek | R CHOP |
17 | 78/M | CLL/SLL | Abdomen | 10 years | third nerve palsy /diplopia | R-ICE |
18 | 66/M | NK/T-cell | NA | NA | presented with a nasal cavity mass with involvement of the nasolacrimal duct and secondary epiphora (tearing) | R-HyperCVAD/ R-MTX/ R-MA |