De Bernardi Bruno, Gambini Claudio, Haupt Riccardo, Granata Claudio, Rizzo Antonino, Conte Massimo, Tonini Gian Paolo, Bianchi Maurizio, Giuliano Maria, Luksch Roberto, Prete Arcangelo, Viscardi Elisabetta, Garaventa Alberto, Sementa Angela Rita, Bruzzi Paolo, Angelini Paola
Department of Pediatric Hematology-Oncology, Giannina Gaslini Children Hospital, Largo Gerolamo Gaslini 5, 16148 Genova, Italy.
J Clin Oncol. 2008 Apr 1;26(10):1710-6. doi: 10.1200/JCO.2006.08.8799.
To review a historical cohort of childhood ganglioneuroma (GN), the benign representative of the peripheral neuroblastic tumor (PNT) family.
Of 2,286 PNTs enrolled between 1979 and 2005, 146 (6.4%) were registered as GN. Histological revision was carried out on 76 tumors. Diagnosis was confirmed in 45, while 27 were reclassified as ganglioneuroblastoma intermixed (GNBI) and four were reclassified as other PNT subtypes.
GNs differed from other PNTs for sex, age, tumor site, stage, tumor markers, and scintigraphic results. Characteristics of 76 reviewed and 70 nonreviewed patients were comparable. Reviewed GN and GNBI patients were comparable except for homovanillic acid excretion, metaiodobenzylguanidine scintigraphy, and DNA content. Seven patients were only biopsied and 139 underwent surgery. Twenty-two patients suffered surgery-related complications, of which two were fatal and seven were severe. Radical tumor resection and surgery-related complication rates were comparable for GN, GNBI, and nonreviewed instances. Six patients developed tumor progression but survived. Two patients developed a late malignancy but survived. None of the 146 patients received chemotherapy. Of 146 patients, two died of surgery-related complications and 144 survived.
Diagnosis was changed to GNBI for approximately one third of 76 reviewed tumors. Patients with confirmed GN, reclassified as GNBI, and nonreviewed histology presented with comparable clinical, biochemical, and biologic features. Surgical results, complication rate, number of progressions, and outcome were similar for the three groups. Surgery was associated with significant risk of complications. Survival was not influenced by extent of tumor resection. Aggressive surgical approach should not be recommended for childhood GN and GNBI.
回顾一组儿童神经节神经瘤（GN）的历史队列，其为外周神经母细胞瘤（PNT）家族的良性代表。
在1979年至2005年间登记的2286例PNT中，146例（6.4%）被登记为GN。对76例肿瘤进行了组织学复查。45例确诊为GN，27例重新分类为混合型神经节神经母细胞瘤（GNBI），4例重新分类为其他PNT亚型。
GN在性别、年龄、肿瘤部位、分期、肿瘤标志物和闪烁扫描结果方面与其他PNT不同。76例复查患者和70例未复查患者的特征具有可比性。除高香草酸排泄、间碘苄胍闪烁扫描和DNA含量外，复查的GN和GNBI患者具有可比性。7例患者仅接受了活检，139例接受了手术。22例患者出现手术相关并发症，其中2例死亡，7例严重。GN、GNBI和未复查病例的根治性肿瘤切除率和手术相关并发症发生率具有可比性。6例患者出现肿瘤进展但存活。2例患者发生晚期恶性肿瘤但存活。146例患者均未接受化疗。146例患者中，2例死于手术相关并发症，144例存活。
76例复查肿瘤中约三分之一被重新诊断为GNBI。确诊为GN、重新分类为GNBI和未复查组织学的患者在临床、生化和生物学特征方面具有可比性。三组的手术结果、并发症发生率、进展数和结局相似。手术伴有显著的并发症风险。生存不受肿瘤切除范围的影响。对于儿童GN和GNBI，不应推荐积极的手术方法。
