Mora J, Wollner N
Department of Pediatrics, Memorial Sloan-Kettering Cancer Center, New York, New York 10021, USA.
Med Pediatr Oncol. 1999 Feb;32(2):102-5. doi: 10.1002/(sici)1096-911x(199902)32:2<102::aid-mpo6>3.0.co;2-y.
Primary epidural non-Hodgkin lymphoma (NHL) is a rare but devastating event in pediatric oncology. Spinal cord compression due to an epidural mass from NHL, although it presents as a localized problem, in fact, is a systemic disease. Over the last 3 decades, aggressive systemic treatments with central nervous system prophylaxis have been designed with improving results.
We reviewed the 20-year experience of the treatment of NHL with the LSA2-L2 protocol in our institution. Among 256 patients treated, five presented with primary epidural mass. Clinical features, treatment, and outcome of those five patients are presented.
Three patients out of five responded favorably, and after a median follow-up of 10 years, they are cured. After presenting with paraplegia, functional and neurological recoveries were excellent. Of the patients who failed treatment, relapse occurred early, either during the treatment or right after completion of therapy. The relapses were within the central nervous system as well as systemic.
Patients with primary epidural NHL presented with long histories of persistent back pain. Plain films of the spine were useless. All patients were diagnosed when neurologic damage had already been established. However, when the epidural NHL is treated aggressively with systemic chemotherapy and vigorous rehabilitation, the prognosis for complete neurological recovery and cure of the lymphoma is encouraging.
原发性硬膜外非霍奇金淋巴瘤（NHL）在儿童肿瘤学中是一种罕见但极具破坏性的疾病。由NHL硬膜外肿块导致的脊髓压迫，尽管表现为局部问题，但实际上是一种全身性疾病。在过去30年中，已设计出采用中枢神经系统预防措施的积极全身治疗方法，且效果不断改善。
我们回顾了本机构采用LSA2-L2方案治疗NHL的20年经验。在接受治疗的256例患者中，有5例表现为原发性硬膜外肿块。现介绍这5例患者的临床特征、治疗及结果。
5例患者中有3例反应良好，中位随访10年后，他们已治愈。出现截瘫后，功能和神经恢复情况极佳。治疗失败的患者复发较早，要么在治疗期间，要么在治疗结束后不久。复发部位在中枢神经系统以及全身。
原发性硬膜外NHL患者有长期持续性背痛病史。脊柱X线平片无用。所有患者在神经损伤已确立时才被诊断出来。然而，当硬膜外NHL采用全身化疗和积极康复治疗时，淋巴瘤完全神经恢复和治愈的预后令人鼓舞。
