Wagner H P, Bleher E A, Bürki K, Delaleu B, Grétillat P, Kühner U, Pedrinis E
Helv Paediatr Acta. 1977 Nov;32(4-5):331-42.
Of 47 children with an initial diagnosis of lymphosarcoma, reticulosarcoma or Non-Hodgkin's lymphoma (NHL), 13 had to be excluded at the histologic reevaluation: in 10 an undifferentiated sarcoma, in 2 Hodgkin lymphoma was found; in one patient no definite classification of the tumor was possible. Of the remaining 34 patients there were 26 boys and 8 girls. One patient had a nodular, 33 a diffuse NHL. Of the latter 16 had a Burkitt-type (LB-), 3 a lymphoblastic, convoluted (LC-), 8 a lymphoblastic, "other" (LO-) and 6 a histiocytoid (H-) NHL. Primary localization: abdomen: 13/34; "peripheral" lymph nodes: 9/34; mediastinum: 5/34; nasopharynx: 4/34; subcutis: 2/34; skeleton: 1/34. Twelve of 17 NHL with primary localization in the abdomen or nasopharynx were LB-NHL, 8/14 NHL with primary localization in "peripheral" nodes or mediastinum were LC- or LO-NHL. Only 2/17 NHL with abdominal or nasopharyngeal primary, but 9/14 NHL with "peripheral" nodal or mediastinal primary developed leukemic extension and/or CNS involvement. 6 of 34 patients are living without evidence of disease for 1 1/2+ to 13+ years; 5/34 died but lived for 85, 57, 37, 22 and 22 months; 9/34 lived 6--12 months; 14/34 died within less than 6 months. Patients with abdominal primary either died within 5 months or survived (for 165+, 63+ and 25+ months). Aggressive local therapy (surgery and radiotherapy with approximately 4000 R) may be adequate for strictly localized (stage I) disease, particularly if the primary localization is abdominal. In all other diffuse NHL of childhood an early, aggressive chemotherapy, later combined with radiotherapy to bulk disease and prophylactic CNS-treatment is essential for inducing long-term remissions and, possibly, cures. For prognosis the primary localization appeared to be more important than histology and stage. The most decisive factor, however, is therapy.
在最初诊断为淋巴肉瘤、网状细胞肉瘤或非霍奇金淋巴瘤（NHL）的47名儿童中，13名在组织学重新评估时被排除：10名诊断为未分化肉瘤，2名诊断为霍奇金淋巴瘤；1名患者的肿瘤无法明确分类。其余34名患者中，有26名男孩和8名女孩。1名患者为结节性NHL，33名为弥漫性NHL。在后者中，16名为伯基特型（LB-），3名为淋巴母细胞性、卷曲核型（LC-），8名为淋巴母细胞性、“其他”型（LO-），6名为组织细胞样型（H-）NHL。原发部位：腹部：13/34；“外周”淋巴结：9/34；纵隔：5/34；鼻咽部：4/34；皮下组织：2/34；骨骼：1/34。17名原发于腹部或鼻咽部的NHL患者中有12名是LB-NHL，14名原发于“外周”淋巴结或纵隔的NHL患者中有8名是LC-或LO-NHL。只有2/17原发于腹部或鼻咽部的NHL患者发生了白血病浸润和/或中枢神经系统受累，但14名原发于“外周”淋巴结或纵隔的NHL患者中有9名发生了这种情况。34名患者中有6名无疾病证据存活了1年半至13年以上；5/34死亡，但存活了85、57、37、22和22个月；9/34存活了6至12个月；14/34在不到6个月内死亡。原发于腹部的患者要么在5个月内死亡，要么存活（分别存活了165个月以上、63个月以上和25个月以上）。积极的局部治疗（手术和约4000伦琴的放疗）可能适用于严格局限（I期）的疾病，特别是当原发部位在腹部时。在所有其他儿童弥漫性NHL中，早期积极化疗，随后联合对大块病灶进行放疗和预防性中枢神经系统治疗对于诱导长期缓解并可能治愈至关重要。就预后而言，原发部位似乎比组织学和分期更重要。然而，最具决定性的因素是治疗。
