Evans A E, Chatten J, D'Angio G J, Gerson J M, Robinson J, Schnaufer L
Cancer. 1980 Mar 1;45(5):833-9. doi: 10.1002/1097-0142(19800301)45:5<833::aid-cncr2820450502>3.0.co;2-u.
The records of 207 neuroblastoma patients seen at the Children's Hospital of Philadelphia between 1944 and 1977 were reviewed to study some of the features associated with the unusually good prognosis found in patients with Stage IV-S neuroblastoma. Initially, 22 patients appeared to fit the criteria of small primary tumor and distant disease in liver, skin, and/or marrow without evidence of bone metastases; 5 patients were subsequently rejected as being incorrectly staged. The remaining 17 patients had abdominal primary tumors and hepatic disease; in 12 of the 17, an enlarged liver was the presenting sign. Six patients had skin lesions, 4 had disease in the marrow on routine smear, and additional sites of spread were pancreas and bowel serosa. The treatment given was not systematic, and it was not possible to correlate any specific form of therapy with a satisfactory outcome. Eleven of 17 patients survived; 6 of 11 survivors had spontaneous regression of all or part of their diseases, 5 of 6 who died received irradiation, chemotherapy, or both. Death usually occurred in the first month as a complication of the local disease; 1 patient succumbed to radiation nephritis. This study establishes that the special pattern of widespread neuroblastoma termed Stage IV-S does exist, and that is associated with a good prognosis. Careful consideration should be given before selecting treatment for the Stage IV-S child because spontaneous regression is likely to occur in most of them. In patients with rapidly enlarging livers, renal or pulmonary complications may develop because of liver bulk or coagulopathies. Treatment should be directed to the liver in these cases because distant metastases seldom supervene. Low-dose irradiation, mild chemotherapy, and possibly surgical release of intraabdominal pressure using a silastic patch have all been effective. Unfortunately, patients occasionally succumb to local disease in spite of these and more aggressive measures.
回顾了1944年至1977年间在费城儿童医院就诊的207例神经母细胞瘤患者的记录，以研究与IV-S期神经母细胞瘤患者异常良好预后相关的一些特征。最初，22例患者似乎符合原发性肿瘤小且有肝脏、皮肤和/或骨髓远处转移但无骨转移证据的标准；随后有5例患者因分期错误而被排除。其余17例患者有腹部原发性肿瘤和肝脏病变；17例中有12例以肝脏肿大为首发症状。6例患者有皮肤病变，4例在常规涂片检查中发现骨髓有病变，其他转移部位为胰腺和肠浆膜。所给予的治疗并不系统，无法将任何特定的治疗形式与满意的结果相关联。17例患者中有11例存活；11例存活者中有6例全部或部分疾病自发消退，死亡的6例中有5例接受了放疗、化疗或两者皆有。死亡通常发生在第一个月，是局部疾病的并发症；1例患者死于放射性肾炎。这项研究证实了被称为IV-S期的广泛神经母细胞瘤的特殊模式确实存在，且与良好预后相关。在为IV-S期儿童选择治疗方法之前应仔细考虑，因为大多数患者可能会自发消退。在肝脏迅速增大的患者中，可能会因肝脏肿大或凝血障碍而出现肾脏或肺部并发症。在这些情况下，治疗应针对肝脏，因为很少会出现远处转移。低剂量放疗、轻度化疗以及可能使用硅橡胶片进行腹腔内减压手术均已证明有效。不幸的是，尽管采取了这些及更积极的措施，患者偶尔仍会死于局部疾病。
