Atypical Presentation of Orbital Natural Killer cell Lymphoma

Natural killer (NK)/T-cell lymphoma is a rare malignancy accounting for less than 1 percent of all lymphomas in North America and Europe. Ocular manifestations frequently presented as orbital cellulites that does not improve with adequate antibiotics. We report a case of NK/T cell lymphoma with rare ocular manifestation that was initially confused with orbital cellulites and chronic sinusitis due to multiple negative biopsies. 92-year-old female presented with right eye vision loss, and ipsi lateral orbital swelling for four weeks. MRI demonstrated right intra-orbital extension to the orbital apex. Workups for infection and vasculitis were negative. Multiple biopsies with histopathology and flow cytology were unrevealing for malignancy. Patient failed to improve on antibiotics and steroids. Five months after the initial presentation, patient presented with the same complaint; however, the orbital mass had grown in size and involved the maxillary sinus and contra lateral side. Re-biopsy revealed positive CD 56 for NK lymphoma. 1/3 Copyright © All rights are reserved by Brandon Menke. Volume 1 Issue 4 Med Surg Ophthal Res Copyright © Brandon Menke 2/3 How to cite this article: Brandon Menke. Atypical Presentation of Orbital Natural Killer cell Lymphoma. Med Surg Ophthal Res. 1(4). MSOR.000518. 2018. Volume 1 Issue 4 orbitotomy. Endoscopy observed semi solid and granular whitish material in ethmoid sinuses, extensive mucus in the sphenoid sinus, and no bony destruction. Several biopsies were taken of the sinus demonstrating inflamed stoma with mixed serous and mucinous glands, dense inflammatory infiltrate of acute and chronic inflammatory cells with areas of necrosis, rare eosinophils and benign respiratory epithelium. Cultures and special stains for bacteria and fungi were positive only for Propionibacterium acne. The leading differential diagnosis at this point was chronic sinusitis with necrosis. Patient was discharged on rapid prednisone taper and 4 weeks of vancomycin /ceftriaxone /metronidazole. Figure 1 : Abduction deficit, partial ptosis, and proptosis of the right eye (top: straight gaze, middle: left gaze, bottom: right gaze) The patient was readmitted 2 weeks later for lower extremity deep vein thrombosis in the setting of a thrombocytopenia of 65000 and a persistent medial orbital mass. A new antibiotic regimen of doxycycline/metronidazole resulted in resolution of the thrombocytopenia. Antibiotics were later discontinued all together due to lack of ocular improvement. The patient was taken back to the operating room for a repeat biopsy due to a CT scan showing an enhancing mass involving the extraconal soft tissue paralleling the posterior ethmoid air cells, concerning for a possible neoplasm. Intra operatively, the medial rectus appeared to be grayish in color concerning for muscle infarction. Final pathology noted a partial fibro vascular and fibro muscular necrosis containing only a few crushed lymphocytes and scattered acute inflammatory cells. Cultures continued to be negative. Flow cytometry was negative for lymphoma. No diagnostic evidence of malignancy or features of lymph proliferative disorder were indicated on histology. CT chest/ abdomen/pelvis to rule out metastatic malignancy was only notable for a 1 cm pulmonary nodule in left lower lobe. Idiopathic orbital inflammatory syndrome labs showed mildly elevated erythrocyte sedimentation rate and CRP; negative RPR, ANA, c-ANCA and p-ANCA. Given these findings, the diagnosis of chronic sinusitis with orbital extension was still the leading diagnosis. The patient was discharged again on a steroid taper (Figures 2 & 3). Figure 2 : Retrobulbar extraconal mass along the medial orbit near the apex impinging on the right optic nerve, and consolidation in the ethmoid air cells and the sphenoid sinus Figure 3 : Persistent opacification of sphenoid sinuses, right ethmoidal air cells with extension from medial orbit into the orbial apex (arrow). New involvement of maxiallary and possible frontal sinuses month later, the patient presented for the third time after sustaining a fall with accompanying fever. On examination, complete ophthalmoplegia and complete ptosis of the right side with V1-V2 anaesthesia consistent with orbital apex syndrome was identified. CT sinus showed that the orbital apex mass had increased in size and now involved the maxillary sinus and the contra lateral side. The patient also endorsed intermittent fever, night sweats, and 6 pounds of weight loss over the past month. She was transferred to a tertiary care facility for further workup. There, chest X-ray and CT head were again negative. She initially did well with medial wall orbital decompression and biopsy and was started on antifungal