Abstract

Background:

The treatment strategies for ocular adnexa lymphomas (OALs), including conjunctiva, orbital, and lacrimal gland lymphomas, remain controversial. In this study, we assessed the efficacies and outcomes of different modality treatment, including chemotherapy, radiotherapy, and surgery for OALs.

Methods and Materials:

Between Jan. 1990 to Dec. 2013, patients receiving first-line chemotherapy (oral alkylating agents, rituximab-based treatment, or CHOP [cyclophosphamide, doxorubicin, vincristine, and steroid]-like regimens), radiotherapy (30 to 50 Gy, in 1.8- to 2.0- Gy per daily fractions), and surgery for newly diagnosed OAL were included in this study. The clinicopathologic features, including patients' characteristics, primary lymphoma locations, pathology subtypes, treatment modality, event-free survival (EFS) following first-line therapy, and OS (OS) were analyzed.

Results:

There were 56 men and 37 women with median age of 58 years were included. Of them, 79 patients (85%) were diagnosed with low-grade mucosa-associated lymphoid tissue (MALT) lymphoma, 5 (5%) with high-grade transformed MALT lymphoma, and 9 (10%) with diffuse large B cell lymphoma. Orbit was the most common involved location (45 patients, 48%), followed by conjunctiva (35 patients, 38%), and lacrimal gland (13 patients, 14%). Of 79 patients with stage I-IIE1 disease, 22 received chemotherapy, 34 with radiotherapy, and 23 patients with other modality treatments (18 with surgery, 4 with combined radiotherapy and chemotherapy, and 1 with intra-lesional injection of rituximab). The 5-year EFS for chemotherapy, radiotherapy, and other modality treatment was 89.2%, 89.7%, and 83.1%, respectively, whereas the 5-year OS for chemotherapy, radiotherapy, and other modality treatment was 100%, 90.4%, and 87.5%, respectively. Of 14 patients with stage IIE2 to IV disease, the 5-year EFS for chemotherapy alone (n=9) and combined radiotherapy and chemotherapy (n=5) were 68.6%, 80%, respectively, whereas the 5-year OS for both group was 80.0%. Among 42 patients receiving radiotherapy, 7 (16.7%) patients developed cataract, 4 (9.5%) patients with keratitis, and 2 (4.8%) patients with maculopathy. In multivariate analysis, high-grade transformation (P=0.049) was the significant factor for shorter EFS of 1st -line treatment. Old age (> 60 years) (p = 0.014), advanced stage (stages III and IV) (p = 0.03), and high-grade transformation (p = 0.018) were the prognostic factors of poor OS.

Conclusions:

In addition to radiotherapy, our data indicate that chemotherapy as first-line therapy provides good disease control and less radiotherapy-associated side effects for patients with stage I to IIE1 OALs. Old age, advanced stage, and high-grade histology subtype remain poor prognostic factors for OALs, and new treatment strategies for these patients are warranted.

Disclosures

No relevant conflicts of interest to declare.

Author notes

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Asterisk with author names denotes non-ASH members.