A rare case of precursor B-cell lymphoblastic lymphoma of the orbit in a child

A 5-year-old boy presented with gradually progressive painful proptosis of the left eye for 5 months. He had an incidental testicular swelling for which he underwent high inguinal orchidectomy and histopathology showed atypical lymphoid cells. His vision was 6/6, N6 in the right eye, and no perception of light in the left eye. Left eye examination revealed an inferior dystopia and total ophthalmoplegia with corneal exposure changes. A computerized tomography scan showed an isodense mass molding around the globe. An incision biopsy from the subconjunctival mass showed atypical large lymphocytes on histopathology which on immunohistochemistry confirmed a diagnosis of precursor B-cell lymphoblastic lymphoma (B-LBL). Bone marrow aspiration and biopsy were normal. The patient was started on oral steroids and high-risk acute LBL chemotherapy protocol to which he showed excellent response. Precursor B-LBL is a rare neoplasm of the pediatric and adolescent age group. A total of 22 cases involving the orbit have been reported to date of which 17 belonged to the pediatric age group. Early detection and prompt initiation of multiagent chemotherapy have shown good survival rates in these patients.