Acute orbital presentation in an activated B-cell subtype, diffuse large B-cell lymphoma: clinicoradiological, histopathological and immunohistochemical correlation

Abstract

<br>Non-Hodgkin's lymphoma is the most common ocular adnexal neoplasm in adults. Involvement of the ocular adnexae can be primary in origin or secondary resulting from systemic disease. We report a 66-year-old male patient with an acute onset of orbital lymphoma with several atypical clinicoradiological features in the absence of any clinical signs of systemic disease at presentation. A histopathological diagnosis of diffuse large B-cell lymphoma was made and immunophenotyping revealed it to be an activated B-cell (ABC) subtype with a “non-double-expressor” phenotype. Rapid progression of disease was noted and resulted in a poor outcome. Clinicoradiological, histopathological, and immunohistochemical correlation is critical for accurate diagnosis in such situations. Additional immunophenotyping to delineate the subtype of DLBCL may have an important role in decision-making regarding the choice of treatment, and may contribute to the prognostication of disease outcome.<br>