Prognosis of Ocular Adnexal Region Involvement in MCL Remains Poor


Ocular adnexal region involvement remains rare, presenting in only between 1% to 5% of mantle cell lymphoma cases.

Compared with systemic mantle cell lymphoma (MCL), patients with orbital adnexal region (OAR) involvement do not differ significantly in terms of epidemiological, pathological, and prognostic characteristics, and better prognostic models are needed for better characterization of patients with purported high-risk disease, according to a literature review and case study published in Hematology/Oncology and Stem Cell Therapy.

Previous studies have reported that patients with OAR involvement had a poor prognosis, even in the era of chemoimmunotherapy. An estimated 8% of all extra nodal lymphomas arise in the OAR region, with secondary involvement of the region standing at 5%. OAR involvement is present in 1% to 5% of MCL cases, most commonly among men in their sixth decade of life and often results in a poor prognosis.

One case study looked at a 61-year-old man presenting with redness and watery discharge from both eyes. The patient’s symptoms lasted for 5 months, during which he was evaluated by several ophthalmologists and prescribed multiple courses of antibiotics for presumed conjunctivitis. As the symptoms persisted, the patient developed a periocular edema around the corner of his eyes. A CT scan found bilateral enhancing soft tissue masses in the superior lateral anterior orbit.

An excisional biopsy of the lacrimal gland found ductal epithelium with diffuse atypical lymphocytic infiltrate consisting of predominantly monotonous small lymphocytes with round nuclei, mature chromatin inconspicuous nucleoli, and a minimal amount of cytoplasm. CD5 and CD20 were strongly expressed, but the patient was negative for CD3, CD10, or CD23. The proliferative rate by Ki67 was 10%. A bone marrow biopsy confirmed MCL involvement.

An MRI of the brain ruled out any abnormalities while a PET/CT scan showed hyper metabolism in bilateral upper outer orbital masses. Stage IV MCL was confirmed. The patient reported no changes in his visual acuity, but said he had mild difficulty in breathing and a feeling of pressure on the throat. The patient’s initial white blood cell count was 9.4 × 109/L; hemoglobin was slightly low at 12.9 g/dL with a normal platelet count of 204 × 109/L. His lactate dehydrogenase was at 122 U/L (Normal range: 122–222 U/L) with an otherwise normal chemistry. MCL international prognostic index (MIPI) score was calculated at 5.57, classifying him as low risk.

The patient was admitted to the hospital for treatment over concerns of airway compromise. He was put on the Nordic regimen, which consists of alternating cycles of rituximab (Rituxan), cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) and high-dose cytarabine (Cytosar-U). After 3 cycles of the Nordic regimen, a post-treatment staging PET/CT showed interval resolution of hypermetabolic activity in periorbital masses and adenopathy in the neck, mediastinum and inguinal area. However, only mild residual metabolic activity was seen in the retrotracheal soft tissue. After the scan, treatment was switched to R-CHOP alone for 3 cycles. Additionally, the patient completed 6 cycles of high-dose chemotherapy with camustine, etoposide, cytarabine, and melphalan followed by an autologous hematopoietic cell transplant. Ninety-one days post-transplant, the patient remains in complete metabolic response.

A literature review of 9 previously-reported cases of OAR involvement of MCL found that over one-third of the patients with MCL presented with bilateral OAR involvement. Additionally, 67% of the 9 patients had stage IV disease upon initial diagnosis, with conjunctiva being the most common lymphomatous involvement location. All reported patients who presented with relapsed MCR with OAR involvement had evidence of intra-ocular spread of disease. 

Iqbal M, Castano Y, Shet T, et al. Intraocular involvement of mantle cell lymphoma: A case report and literature review. Hematol./ Oncol. Stem Cell Ther. 2021;14(2):147-152. doi:10.1016/j.hemonc.2019.03.002

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