Sánchez-Romero Celeste, Pontes Hélder Antônio Rebelo, Pontes Flávia Sirotheau Corrêa, Rocha André Caroli, Carlos Román, Rendón Javier C, de Almeida Oslei Paes, Fonseca Felipe Paiva
Department of Oral Diagnosis, Piracicaba Dental School, University of Campinas, Piracicaba, Brazil.
Service of Oral Pathology, João de Barros Barreto University Hospital, Federal University of Pará, Belém, Brazil.
Oral Surg Oral Med Oral Pathol Oral Radiol. 2018 Aug;126(2):152-164. doi: 10.1016/j.oooo.2018.01.010. Epub 2018 Jan 31.
Our objective was to describe the clinicopathologic and immunohistochemical features of acute lymphoblastic leukemia/lymphoma (ALL/LBL) of the oral and maxillofacial region (OMF).
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.
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 afocal/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%.
OMF ALL/LBL is rare, but its microscopic features and immunohistochemical profiles CD3or CD79 a/CD10/CD34variable/CD99/TdT contribute to the correct diagnosis.
我们的目的是描述口腔颌面部急性淋巴细胞白血病/淋巴瘤（ALL/LBL）的临床病理和免疫组化特征。
从2005年至2017年巴西2个和危地马拉1个口腔病理科室的档案中检索诊断为口腔颌面部ALL/LBL的病例。对每个病例的显微镜和免疫组化特征进行回顾并详细描述，并从病理报告中检索临床数据。
在研究期间，共确定6例病例。男性患者受影响最多（4:2），平均年龄19岁。下颌骨受累2例，上颌骨2例，颊黏膜1例，腮腺1例。疼痛性肿胀是最常见的表现，3例患者还有全身症状。显微镜下，肿瘤显示中小等大小未成熟细胞的实性浸润。观察到“拼图样”和“满天星”模式，也发现了“单排”生长。免疫组化方面，2例诊断为T细胞ALL/LBL，具有白细胞共同抗原（LCA）/胞质CD3/CD79a阴性/CD20/PAX5/CD99/CD34/CD10/末端脱氧核苷酸转移酶（TdT）表型，4例为B细胞ALL/LBL，具有LCA/CD3/CD20/CD79a/CD10/CD34可变/TdT为主的表型。Ki67指数范围为80%至99%。
口腔颌面部ALL/LBL罕见，但其显微镜特征和免疫组化谱CD3或CD79a/CD10/CD34可变/CD99/TdT有助于正确诊断。
