Purpose: To present the case of an 8-month-old girl with undiagnosed pre-B-cell acute lymphoblastic leukemia (ALL)-presenting as an orbital mass. Design: Observational case report and literature review. Methods: Review of clinical history,radiologic findings,histology of cervical lymph node and orbital biopsies,and follow-up. Results: An 8-month-old girl presented with a 2-week history of right-sided tearing,lid swelling,proptosis,and rhinorrhea. A computed tomographic scan of the orbits revealed a homogeneous,retrobulbar orbital mass eroding into the ethmoid sinuses and nasal cavity,as well as cervical lymphadenopathy. Biopsy of the lesion and enlarged lymph node disclosed medium-sized,uniform,cytologically atypical lymphocytes in a starry sky pattern. Immunohistochemical analysis showed reactivity to antibodies against CD-10,CD-19,CD-34,and terminal deoxynucleotidyl transferase. Cytogenetic analysis of the tumor also revealed a gene rearrangementon chromosome11q23. Based on these findings,the diagnosis of pre-B-cell acute lymphoblastic leukemia was made,and systemic as well as intrathecal chemotherapy was instituted,which resulted in rapid remission of the leukemia. Conclusions: The initial presentation of ALL as an orbitalmass is exceedingly rare. To the best of the authors’ knowledge,this is the earliest reported case of pre-B-cell ALL presenting as an orbital lesion.
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