Acute lymphoblastic leukemia/lymphoma of the oral and maxillofacial region

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Abstract

Objective.

Our objective was to describe the clinicopathologic and immunohistochemical features of acute lymphoblastic leukemia/lymphoma (ALL/LBL) of the oral and maxillofacial region (OMF).

Study Design.

Cases diagnosed as ALL/LBL of the OMF region were retrieved from the files of 2 Brazilian and 1 Guatemalan oral pathology services from 2005 to 2017. Microscopic and immunohistochemical features of each case were reviewed and fully described, and clinical data were retrieved from the pathology reports.

Results.

During the period considered, 6 cases were identified. Male patients were the most affected (4:2), with a mean age of 19 years old. The mandible was involved in 2 cases, the maxilla in 2, the cheek mucosa in 1, and the parotid gland in 1. Painful swelling was the most common presentation, and 3 patients also had systemic complaints. Microscopically, tumors revealed solid infiltrations of small to medium-sized immature cells. “Puzzle-like” and “starry-sky” patterns were observed, and “single lane” growth was also identified. Immunohistochemically, 2 cases were diagnosed as T-cell ALL/LBL with the leukocyte common antigen (LCA)+/cCD3+/CD79 a+focal/CD20−/PAX5−/CD99+/CD34−/CD10+/terminal deoxynucleotidyl transferase (TdT)+ phenotype and 4 as B-cell ALL/LBL with the LCA+/CD3−/CD20−/CD79 a+/CD10+/CD34 variable/TdT+ predominant phenotype. The Ki67 index ranged from 80% to 99%.

Conclusion.

OMF ALL/LBL is rare, but its microscopic features and immunohistochemical profiles CD3+or CD79 a+/CD10+/CD34+variable/CD99+/TdT+ contribute to the correct diagnosis.

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