Brugières Laurence, Minard Véronique, Patte Catherine
Département de Cancérologie de l'enfant et de l'adolescent, Institut Gustave-Roussy, 94805 Villejuif.
Rev Prat. 2012 Apr;62(4):453-8.
Non Hodgkin lymphomas in the pediatric population are a heterogeneous group of tumors with distinct pathologic, immunologic and clinical characteristics. Over the past three decades, significant advancements have been made in the molecular characterization of these disorders. More than 90% of pediatric childhood non Hodgkin lymphomas are high grade lymphomas belonging to four major histologic subtypes, Burkitt lymphoma, diffuse large B-cell lymphoma, lymphoblastic lymphoma and anaplastic large cell lymphoma. With the use of intensive multiagent chemotherapy, most patients can now be cured and long-term event free survival is above 80% in most histologic subtypes. The identification of molecular events associated with carcinogenesis leads to the identification of novel targeted therapy which should allow a further improvement of treatment including a reduction of treatment burden in early stages and improvement of survival in advanced stages.
儿童非霍奇金淋巴瘤是一组具有不同病理、免疫和临床特征的异质性肿瘤。在过去三十年中，这些疾病的分子特征有了显著进展。超过90%的儿童非霍奇金淋巴瘤是高级别淋巴瘤，属于四种主要组织学亚型，即伯基特淋巴瘤、弥漫性大B细胞淋巴瘤、淋巴母细胞淋巴瘤和间变性大细胞淋巴瘤。通过使用强化多药化疗，现在大多数患者可以治愈，大多数组织学亚型的长期无事件生存率超过80%。与致癌作用相关的分子事件的识别导致了新型靶向治疗的识别，这应该能够进一步改善治疗，包括减轻早期治疗负担和提高晚期生存率。
