Unit of Lymphoid Malignancies, Department of Onco-Haematology, San Raffaele Scientific Institute, Milan, Italy.
Genesiscare and Bond University, Inland Dr., Tugun, QLD, Australia.
Cancer Treat Rev. 2015 Mar;41(3):235-46. doi: 10.1016/j.ctrv.2015.02.001. Epub 2015 Feb 7.
Recent studies have contributed to the enhancement of clinical and molecular knowledge on bone lymphomas, a group of rare malignancies with particular characteristics. Nevertheless, several questions remain unanswered and the level of evidence supporting some diagnostic and therapeutic decisions remains low. Currently, three different forms of bone lymphomas can be distinguished: the primary bone lymphoma, consisting of a single bone lesion with or without regional lymphadenopathies; the polyostotic lymphoma, consisting of multifocal disease exclusively involving the skeleton; and the disseminated lymphoma with secondary infiltration of the skeleton. The first two forms exhibit a good prognosis, requiring treatments similar to those commonly used for nodal lymphomas of the same category, but several issues regarding the role of surgery and local control of the disease, the sequence of treatment, radiation volumes and doses, management of pathological fractures and prevention of late sequelae deserve particular attention. Due to its rarity, prospective trials exclusively focused on bone lymphomas appear unrealistic, thus, critical revision of our own experience and analyses of large cumulative series as well as molecular studies on archival cases remain valid alternatives to improve our knowledge on this obscure lymphoproliferative malignancy. The present review is based on the analysis of the largest available database of bone lymphomas established under the sponsorship of the International Extranodal Lymphoma Study Group (IELSG) as well as on the critical revision of related literature. We provide recommendations for diagnosis, staging, treatment, and response assessment of these patients in everyday practice as well as for the management of special conditions like pathological fractures, indolent forms and central nervous system prophylaxis.
最近的研究有助于提高对骨淋巴瘤的临床和分子认识，骨淋巴瘤是一组具有特殊特征的罕见恶性肿瘤。然而，仍有几个问题尚未得到解答，一些诊断和治疗决策的证据水平仍然较低。目前，可以区分三种不同形式的骨淋巴瘤：原发性骨淋巴瘤，由单个骨病变伴有或不伴有区域性淋巴结病组成；多灶性淋巴瘤，仅由骨骼多灶性疾病组成；和弥漫性淋巴瘤，骨骼继发浸润。前两种形式预后良好，需要的治疗与同一类别的淋巴结淋巴瘤相同，但关于手术和疾病局部控制、治疗顺序、放射剂量和体积、病理性骨折的处理和预防晚期后遗症的作用等几个问题值得特别关注。由于其罕见性，专门针对骨淋巴瘤的前瞻性试验似乎不太现实，因此，批判性地审查我们自己的经验和对大型累积系列的分析以及对存档病例的分子研究仍然是提高我们对这种模糊性淋巴增生性恶性肿瘤的认识的有效替代方法。本综述基于对国际结外淋巴瘤研究组（IELSG）赞助下建立的最大骨淋巴瘤可用数据库的分析以及对相关文献的批判性审查。我们为这些患者的日常诊断、分期、治疗和反应评估以及病理性骨折、惰性形式和中枢神经系统预防等特殊情况的管理提供建议。
