Abstract Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT type) is an indolent lymphoma that may arise in various extranodal sites; however, craniofacial involvement remains rare and may present a diagnostic challenge due to non-specific clinical manifestations. We report two patients with extranodal marginal zone B-cell lymphoma occurring in uncommon craniofacial locations. A 66-year-old woman presented with a slowly enlarging frontal mass of unclear origin and underwent complete surgical excision, which established the diagnosis. Subsequent staging revealed disseminated disease, and systemic immunochemotherapy with rituximab and bendamustine was initiated with a favorable response. A 37-year-old man presented with bilateral orbital involvement manifested by progressive eyelid swelling. Diagnosis was established using image-guided core needle biopsy to minimize surgical morbidity. He was treated with rituximab–bendamustine-based therapy, resulting in marked clinical and radiological regression. Craniofacial extranodal marginal zone B-cell lymphoma represents a rare entity that may mimic other benign or malignant conditions. These cases highlight the importance of appropriate diagnostic strategy selection, including the role of minimally invasive biopsy techniques, and emphasize the importance of multidisciplinary management in achieving optimal outcomes.
Kalmanova et al. (Sat,) studied this question.
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