Start Over

Acute lymphoblastic leukemia/lymphoma of the oral and maxillofacial region.

Authors :: Sánchez-Romero C
Pontes HAR
Pontes FSC
Rocha AC
Carlos R
Rendón JC
de Almeida OP
Fonseca FP
Source :: Oral surgery, oral medicine, oral pathology and oral radiology [Oral Surg Oral Med Oral Pathol Oral Radiol] 2018 Aug; Vol. 126 (2), pp. 152-164. Date of Electronic Publication: 2018 Jan 31.
Publication Year :: 2018
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).<br />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.<br />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) <superscript>+</superscript> /cCD3 <superscript>+</superscript> /CD79 a <superscript>+</superscript> focal/CD20 <superscript>-</superscript> /PAX5 <superscript>-</superscript> /CD99 <superscript>+</superscript> /CD34 <superscript>-</superscript> /CD10 <superscript>+</superscript> /terminal deoxynucleotidyl transferase (TdT) <superscript>+</superscript> phenotype and 4 as B-cell ALL/LBL with the LCA <superscript>+</superscript> /CD3 <superscript>-</superscript> /CD20 <superscript>-</superscript> /CD79 a <superscript>+</superscript> /CD10 <superscript>+</superscript> /CD34 variable/TdT <superscript>+</superscript> predominant phenotype. The Ki67 index ranged from 80% to 99%.<br />Conclusion: OMF ALL/LBL is rare, but its microscopic features and immunohistochemical profiles CD3 <superscript>+</superscript> or CD79 a <superscript>+</superscript> /CD10 <superscript>+</superscript> /CD34 <superscript>+</superscript> variable/CD99 <superscript>+</superscript> /TdT <superscript>+</superscript> contribute to the correct diagnosis.<br /> (Copyright © 2018 Elsevier Inc. All rights reserved.)