Irtan Sabine, Brisse Hervé J, Minard-Colin Véronique, Schleiermacher Gudrun, Galmiche-Rolland Louise, Le Cossec Chloé, Elie Caroline, Canale Sandra, Michon Jean, Valteau-Couanet Dominique, Sarnacki Sabine
Pediatric surgery department, Necker Enfants Malades Hospital, Paris Descartes University, Paris, France.
Radiology department, Curie institute, Paris, France.
Pediatr Blood Cancer. 2015 Sep;62(9):1543-9. doi: 10.1002/pbc.25511. Epub 2015 Mar 27.
Patients with neuroblastoma are now stratified at diagnosis according to the presence and number of image-defined risk factors (IDRFs). We examined the added value of IDRF assessment after neoadjuvant chemotherapy for predicting surgical resection.
From 2009-2012, 39 out of 91 patients operated on in our institution for neuroblastic tumors received neoadjuvant chemotherapy based on ongoing SIOPEN protocols or treatment guidelines. IDRFs were assessed both at diagnosis and preoperatively on CT and/or MRI.
Median age at diagnosis was 30 months [range 2-191]. The tumor locations were adrenal (n = 20), paravertebral (n = 13) and perivascular (n = 6). INRGSS stages were L2 (n = 13), M (n = 25) and Ms (n = 1). Eleven tumors (28%) were MYCN-amplified. Chemotherapy reduced the number of IDRFs in 54% of patients overall (21/39): 61.5% (16/26) of M and Ms patients, and 38.5% (5/13) of non metastatic patients (P < 0.001). The number of IDRFs lost after chemotherapy was proportional to the degree of tumor shrinkage (P = 0.002), independent of the primary tumor location (P = 0.73), although the number was higher in patients with left versus right adrenal locations (P = 0.004). Patients with neuroblastoma on post-surgical histology lost more IDRFs (median: 1[0-9]) than patients with ganglioneuroblastoma (median: 0[0-4]) (P < 0.001). The completeness of resection was related only to the number of preoperative IDRFs (P = 0.028).
IDRF assessment after neoadjuvant chemotherapy is useful for predicting completeness of resection of neurogenic tumors. A larger international study is needed to confirm these results and to explore a possible correlation between preoperative IDRF status and survival.
神经母细胞瘤患者目前在诊断时根据影像定义的风险因素（IDRFs）的存在情况和数量进行分层。我们研究了新辅助化疗后IDRF评估对预测手术切除的附加价值。
2009年至2012年期间，在我们机构接受神经母细胞瘤手术的91例患者中，有39例根据现行的SIOPEN方案或治疗指南接受了新辅助化疗。在诊断时以及术前通过CT和/或MRI对IDRFs进行评估。
诊断时的中位年龄为30个月[范围2 - 191个月]。肿瘤位置为肾上腺（n = 20）、椎旁（n = 13）和血管周围（n = 6）。国际神经母细胞瘤风险组分期（INRGSS）为L2期（n = 13）、M期（n = 25）和Ms期（n = 1）。11例肿瘤（28%）为MYCN扩增型。总体而言，化疗使54%（21/39）的患者IDRF数量减少：M期和Ms期患者中有61.5%（16/26），非转移性患者中有38.5%（5/13）（P < 0.001）。化疗后IDRF数量的减少与肿瘤缩小程度成正比（P = 0.002），与原发肿瘤位置无关（P = 0.73），尽管左侧肾上腺位置的患者IDRF数量高于右侧（P = 0.004）。神经母细胞瘤术后组织学诊断的患者比神经节神经母细胞瘤患者丢失更多的IDRF（中位数：1[0 - 9]比中位数：0[0 - 4]）（P < 0.001）。切除的完整性仅与术前IDRF的数量有关（P = 0.028）。
新辅助化疗后IDRF评估有助于预测神经源性肿瘤切除的完整性。需要开展更大规模的国际研究来证实这些结果，并探索术前IDRF状态与生存之间可能存在的相关性。
