Acute lymphoblastic leukemia/lymphoma of the oral and maxillofacial region

Publication date: Available online 31 January 2018
Source:Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology
Author(s): Celeste Sánchez Romero, Hélder Antônio Rebelo Pontes, Flávia Sirotheau Corrêa Pontes, André Caroli Rocha, Román Carlos, Javier C. Rendón, Oslei Paes de Almeida, Felipe Paiva Fonseca
ObjectiveTo describe the clinicopathological and immunohistochemical features of acute lymphoblastic leukemia/lymphoma (ALL/LBL) of the oral and maxillofacial region (OMF).Study designCases diagnosed as ALL/LBL of the OMF region were retrieved from the files of two Brazilian and one Guatemalan oral pathology services from 2005 to 2017. Microscopic and immunohistochemical features of each case were reviewed and fully described, whereas clinical data were retrieved from the pathology reports.ResultsDuring the period considered, six cases were identified. Males were the most affected (4:2), with a mean age of 19 years old. The mandible was involved in two cases, the maxilla in two, the cheek mucosa in one and the parotid gland in one case. A painful swelling was the most common presentation, and three patients also had systemic complaints. Microscopically, tumors revealed solid infiltrations of small to medium sized immature cells. The "puzzle-like" and "starry-sky" patterns were observed, and "single lane" growth was also identified. Immunohistochemically, two cases were diagnosed as T-cell ALL/LBL with the LCA+/cCD3+/CD79a+focal/CD20-/PAX5-/CD99+/CD34-/CD10+/TdT+ phenotype and four as B-cell ALL/LBL with the LCA+/CD3-/CD20-/CD79a+/CD10+/CD34variable/TdT+ predominant phenotype. The Ki67 index ranged from 80 to 99%.ConclusionOMF ALL/LBL is rare, but its microscopic features and immunohistochemical profile CD3+orCD79a+/CD10+/CD34+variable/CD99+/TdT+ contribute to the correct diagnosis.



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