Extranodal Peripheral T-Cell Lymphoma with Oro-Nasal Erosion - A Diagnostic Dilemma: A Case Report with Literature...

Extranodal peripheral T-cell lymphomas (PTCLs) are rare and commonly affect patients with immunosuppression or systemic autoimmune disorders. The involvement of oral cavity is extremely rare, and the chances of missing the initial diagnosis are high. A case of 38-year-old, previously healthy male presented with whitish ulcerative lesion (3 × 1.5 cm) over the soft palate crossing the midline with uvula involvement and nasal stuffiness for the last 4- and 8-months, respectively. Initial investigations were unremarkable, and the case was discharged following conservative management. However, 1-month later, the case returned with difficulty in swallowing and a destructive ulcer over the soft palate causing palatal perforation (1 × 3 cm), resulting in the formation of an oro-nasal fistula and destruction of uvula. Various investigations, including CECT brain and thorax, serology, and inflammatory markers, as well as those for autoimmune disorder were unremarkable. CECT face revealed soft palate erosion with complex deviated nasal septum. Nasal mucosal biopsy revealed atypical lymphoid cells with high anaplasia, and angiocentric spread in certain tumor cells suggesting lymphoproliferative disorder. Immunohistochemistry was positive for CD3, CD5, and CD7. Thus, diagnosis of extranodal aggressive PTCL was reached, and chemotherapy with CHOP regimen was initiated. However, following second cycle, the patient succumbed.