Burkitt lymphoma is an aggressive mature B-cell lymphoma defined as a monomorphic, medium-sized B-cell lymphoma with basophilic cytoplasm, multiple small nucleoli, a germinal-center B-cell phenotype, a high proliferation index, and an IG::MYC rearrangement. It is epidemiologically classified as endemic/African, sporadic/American or associated with immunosuppression subtypes. All variants demonstrate the same microscopic features, but with differing clinical presentations and different association rates with Epstein-Barr virus (EBV). Burkitt lymphoma exhibits a male predominance and predominantly occurs in young patients, accounting for approximately 30% to 50% of all infantile lymphomas. In the oral cavity, Burkitt lymphoma manifests as a painful swelling more frequently in the posterior region of the mandible. It often demonstrates a rapid growth rate, usually extending to surrounding soft tissues of the palate, gingiva and buccal mucosa, with the presence of ulceration and areas of tissue necrosis. This neoplasm shows a diffuse growth pattern of monomorphic, medium-sized neoplastic cells with the typical starry-sky pattern. Tumor cells are positive for CD20 and germinal center markers, with very high Ki67 expression. Although Burkitt lymphoma is a highly aggressive neoplasm, it is considered a curable disease with available chemotherapeutic regimens.

错误:搜索内容不能为空,请输入英文关键词
错误:关键词超出字数限制,请精简
高级检索

Burkitt Lymphoma

  • Chané Smit,
  • Júlio Cesar Tanos de Lacerda,
  • Javier Rendon Henao,
  • Hélder Antônio Rebelo Pontes,
  • Felipe Paiva Fonseca

摘要

Burkitt lymphoma is an aggressive mature B-cell lymphoma defined as a monomorphic, medium-sized B-cell lymphoma with basophilic cytoplasm, multiple small nucleoli, a germinal-center B-cell phenotype, a high proliferation index, and an IG::MYC rearrangement. It is epidemiologically classified as endemic/African, sporadic/American or associated with immunosuppression subtypes. All variants demonstrate the same microscopic features, but with differing clinical presentations and different association rates with Epstein-Barr virus (EBV). Burkitt lymphoma exhibits a male predominance and predominantly occurs in young patients, accounting for approximately 30% to 50% of all infantile lymphomas. In the oral cavity, Burkitt lymphoma manifests as a painful swelling more frequently in the posterior region of the mandible. It often demonstrates a rapid growth rate, usually extending to surrounding soft tissues of the palate, gingiva and buccal mucosa, with the presence of ulceration and areas of tissue necrosis. This neoplasm shows a diffuse growth pattern of monomorphic, medium-sized neoplastic cells with the typical starry-sky pattern. Tumor cells are positive for CD20 and germinal center markers, with very high Ki67 expression. Although Burkitt lymphoma is a highly aggressive neoplasm, it is considered a curable disease with available chemotherapeutic regimens.