Diagnostic Challenge of Extranodal Marginal Zone Lymphoma of Extraocular Muscles: A Case Report

<b>Background and clinical significance:</b> The current report examines an unusual case of extranodal marginal zone lymphoma (EMZL) affecting only the extraocular muscles. The diagnostic challenges connected to such atypical manifestations are highlighted. The resemblance to inflammatory or infiltrative processes may lead to diagnostic delays; therefore, therapy administration may be withheld. <b>Case presentation:</b> A 77-year-old male was admitted to our hospital with periorbital edema accompanied with vision abnormalities affecting his left eye. The complete diagnostic workup and surgical treatment is presented using a navigation system. Adequate biopsy samples from the delicate orbital tissue can be obtained by utilizing contemporary visualization techniques, particularly navigation systems. The pathology report proved indispensable, with ample raw muscle tissue providing sufficient material from whicha definitive diagnosis was made. The indicated therapy was administered without delay. <b>Conclusions:</b> The uncommon, isolated affection of extraocular muscles by extranodal marginal zone lymphoma is exemplified in this case, highlighting the need for early clinical suspicion in order to differentiate this condition from other more prevalent inflammatory pathologies. The implementation of advanced technologies, such as image-guided navigation systems, alongside a highly skilled multidisciplinary medical team, ensures optimal patient results.