Bone Cancer Research Today is a free monthly online journal that collates and summarizes the latest research about Bone Cancer, including details on symptoms, types, treatment.

Pre-B-cell acute lymphoblastic leukemia presenting as an orbital mass in an 8-month-old.

Thakker MM, Rubin PA, Chang E

Department of Ophthalmology, University of Washington, Seattle, Washington 98195, USA. mthakker@u.washington.edu

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 rearrangement on chromosome 11q23. 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 orbital mass 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.

Published 6 February 2006 in Ophthalmology, 113(2): 343-6.
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