Hachitanda Y, Hata J
Division of Pathology, Clinical Laboratory, National Children's Hospital, Tokyo, Japan.
Hum Pathol. 1996 Nov;27(11):1135-8. doi: 10.1016/s0046-8177(96)90304-4.
This study reports clinicopathologic and biological analysis of 45 stage IVS neuroblastomas (IVS-NBs). All IVS-NB cases had small or undetectable primary tumors associated with disease involving the liver, skin, or bone marrow. Their ages at diagnosis ranged from 1 day to 12 months, with a median age of 4 months. The primary sites were adrenal in 34 cases, retroperitoneum in two, mediastinum in one, and unknown in eight; the liver was involved in 24 of 31 cases, the bone marrow in 11 of 27, and the skin in 4 of 29. According to the histopathological evaluation (Shimada classification), 43 IVS-NB tumors had a favorable histology, and 2 cases showed an unfavorable histology. Three patients died of progressive disease and six of indirect tumor death. Among these three deceased cases with tumor progression, two cases showed unfavorable histology, and one case had amplified N-myc (20 copies). These data suggest that most IVS-NB can be described as a tumor presenting characteristic dissemination pattern in the younger age group, having a favorable histology and showing an excellent outcome of the patients. However, poor prognosis group in stage IVS does exist, and this group could be detected histopathologically and biologically.
本研究报告了45例IV-S期神经母细胞瘤（IVS-NBs）的临床病理及生物学分析。所有IVS-NB病例均有小的或无法检测到的原发肿瘤，伴有肝脏、皮肤或骨髓受累。其诊断时年龄范围为1天至12个月，中位年龄为4个月。原发部位：肾上腺34例，腹膜后2例，纵隔1例，8例不明；31例中有24例肝脏受累，27例中有11例骨髓受累，29例中有4例皮肤受累。根据组织病理学评估（岛田分类），43例IVS-NB肿瘤组织学良好，2例组织学不良。3例患者死于疾病进展，6例死于间接肿瘤死亡。在这3例肿瘤进展死亡病例中，2例组织学不良，1例N-myc扩增（20拷贝）。这些数据表明，大多数IVS-NB可描述为在较年轻年龄组中呈现特征性播散模式的肿瘤，组织学良好，患者预后极佳。然而，IV-S期确实存在预后不良组，该组可通过组织病理学和生物学方法检测出来。
