<p>Primary mediastinal large B-cell lymphoma (PMBCL) is an aggressive B-cell lymphoma, which has been considered to be a separate subtype different from diffuse large B-cell lymphoma (DLBCL). PMBCL has unique molecular signatures characterized by the activation of JAK-STAT and NF-κB pathways, which are more similar to classical Hodgkin’s lymphoma (cHL). Moreover, the immune-privileged phenotype is predominant, which is achieved by a series of genetic alterations. Most patients have a superior response to initial therapies, but there are still some patients with relapsed/refractory (R/R) disease. Novel therapies targeting potential therapeutic targets underlying the molecular pathogenesis may improve the outcomes of R/R patients. Additionally, abundant research indicated that PET-CT radiomics and some clinical features were significantly correlated with PMBCL prognosis. The role of consolidative radiotherapy remains controversial. More accurate prognostic-models based on PET radiomics and related clinical features are required to provide precise risk-stratification and guide subsequent treatment choices for PMBCL patients.</p>

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Revealing the promising therapeutic targets and prognostic markers of primary mediastinal large B-cell lymphoma

  • Yuge Liu,
  • Shuai Li,
  • Chen Zhang,
  • Jingyao Zhang,
  • Yongheng Li

摘要

Primary mediastinal large B-cell lymphoma (PMBCL) is an aggressive B-cell lymphoma, which has been considered to be a separate subtype different from diffuse large B-cell lymphoma (DLBCL). PMBCL has unique molecular signatures characterized by the activation of JAK-STAT and NF-κB pathways, which are more similar to classical Hodgkin’s lymphoma (cHL). Moreover, the immune-privileged phenotype is predominant, which is achieved by a series of genetic alterations. Most patients have a superior response to initial therapies, but there are still some patients with relapsed/refractory (R/R) disease. Novel therapies targeting potential therapeutic targets underlying the molecular pathogenesis may improve the outcomes of R/R patients. Additionally, abundant research indicated that PET-CT radiomics and some clinical features were significantly correlated with PMBCL prognosis. The role of consolidative radiotherapy remains controversial. More accurate prognostic-models based on PET radiomics and related clinical features are required to provide precise risk-stratification and guide subsequent treatment choices for PMBCL patients.