Radiosugery with CyberKnife in Primary Orbital MALT Lymphoma: A Case Report

Keywords

CyberKnife • Radiosurgery • ND4 • Mucosa-associated lymphoid tissue lymphoma • Ocular adnexal lymphoma of MALT-type

Introduction

The concept of Mucosa-Associated Lymphoid Tissue (MALT) lymphoma first described by Isaacson et al. is now recognized as distinct clinicalpathological and molecular entity [1]. According World Health Organization (WHO) classifications, Marginal Zone Lymphoma (MZL) consists of three subtypes: Splenic MZL; Nodal MZL; and Extra-nodal MZL, also called MALT lymphoma [2]. MALT lymphoma accounts for 5-8% of all B-cell lymphomas [3] and constitutes one half of all orbital malignancies [4]. Ocular Adnexal Lymphoma of MALT-type (OAML) can affect conjunctiva, the retrobulbar soft tissue, the lachrymal gland, the eyelid and the lachrymal sac [5]. Although radiation therapy is the primary modality of treatment many issues remain unresolved due to the various types of ocular involvement, radiation techniques and side effects.

Case Presentation

A 76-year-old Caucasian female patient presented with pain in the right eye. Two years before the patient had the same symptoms. The computed tomography at that time detected lesion in the right retrobulbar space. Exicisional biopsy was performed and the pathological report was consistent with lipoma. At present the physical exam showed exophthalmos of the right eye. No enlarged lymph nodes and no significant findings on systemic examination were found. Routine blood investigations were within normal limits. Computed tomography of the head revealed a lesion in the right retrobulbar space. Excisional biopsy was performed and the immunophenotyping of the material showed phenotype CD20+, BCL2+, BCL6-, CD5-, CD3-, CD10-, cyclin D1-, and ki-67 proliferation index of 15%. In context with the microscopic appearance the final pathology report revealed the diagnosis of extranodal Marginal Zone Lymphoma (MZL/MALT). Further staging with chest, abdominal and pelvic computed tomography and bone marrow biopsy were done. No enlarged lymph nodes and no bone marrow involvement were found, which was consistent with a clinical stage IE. After discussion of her medical case, a multidisciplinary team decision was to start treatment with 4 cycles of high dose Dexamethasone plus anti-chlamidial antibiotic therapy. From January 2019 till April 2019 the patient was treated with 4 cycles of high dose Dexamethasone. The control CT of the head showed stable disease.

Radiosurgery with CyberKnife

The multidisciplinary team decision was radiosurgery with CyberKnife as a second line therapy because this therapy option has the capacity to minimize irradiation of nearby critical structures. Before starting the treatment an orbital Magnetic Resonance Imaging (MRI) of the right retrobulbar mass was performed (Figure 1). Patient head was immobilized with a thermoplastic mask before CT/MRI imaging fusion. The nearby critical structures (eyes, lens, lacrimal glands, optic nerves and optic chiasm) were contoured. The dose was prescribed such that 96.8% of the PTV received 100% of the prescribed dose of 20 Gy. The prescribed dose was delivered in 4 fractions, 5 Gy daily. The radiation used for radiosurgery was 6 MeV photons. There was no significant acute toxicity registered. The months after the radiosurgery with CyberKnife no exophthalmos was detected, and the control CT showed very good response to the treatment (Figure 2).

Figure 1. Images of pre-treatment CT/MRI.

Figure 2. Images of post-treatment CT.

Conclusion

As the rates the Ocular Adnexal MALT Lymphoma (OAML) are increasing nowadays a new effective and save therapeutic options are more than needed. Conventional local radiation therapy is the preferred therapeutic option with proven efficacy in the treatment of OAML. According to previous published data and the result of our case, treatment using radiosurgery with CyberKnife is an effective therapeutic modality with an acceptable toxicity profile.

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