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S.R. Krawitz, M.M. Thakker; Pre–B–Cell Acute Lymphoblastic Leukemia Presenting as an Orbital Mass in an 8 Month–Old . Invest. Ophthalmol. Vis. Sci. 2004;45(13):4698.
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© ARVO (1962-2015); The Authors (2016-present)
Purpose: To report the case of a patient with previously undiagnosed pre–B–cell acute lymphoblastic leukemia presenting as an orbital mass. Methods: Review of clinical history, laboratory findings, histology, immunocytochemistry and genetic analysis of orbital tumor. Results: An 8 month–old female presented with a two week history of rhinorrhea and right–sided tearing, lid edema, and proptosis. A CT scan of the orbits revealed a retro–orbital mass extending 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 a "starry sky" pattern. Immunohistochemical analysis showed reacitvity to antibodies against CD–10, CD–19, CD–34, and terminal deoxynucleotidyl transferase (Tdt). Cytogenetic analysis of the tumor also revealed a gene rearrangement on chromosome 11q23. Based on these finding, the diagnosis of pre–B–cell acute lymphoblastic leukemia was made. Treament included systemic and intrathecal chemotherapy. This treatment resulted in rapid remission. Conclusions: This is an extremely rare case of pre–B–cell acute lymphoblastic leukemia that presented as an orbital mass in an 8 month–old female. The disease was successfully treated with systemic and intrathecal chemotherapy. This case emphasizes the importance of tissue biopsy, immunocytochemistry and cytogenetics in establishing the diagnosis and directing treatment.
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