Implication of Image-Defined Risk Factors for the Extent of Surgical Resection and Clinical Outcome in Patients with Pelvic Neuroblastoma.

 Pelvic neuroblastoma (NB) is a rare entity and occurs in 2 to 5% of all NBs. Surgery in the pelvic area is-even for the experienced oncological surgeon-technically challenging, as injuries of bladder and/or rectal innervation may carry lifelong consequences for the patient. Several studies have proven the impact of image-defined risk factors (IDRFs) for outcome, complications and extent of resection in NB; however, the specific role of IDRF in pelvic NB has not been investigated yet.  Patient charts were retrospectively evaluated for International Staging System stage, IDRF status, MYCN amplification, and outcome parameters.  Between 2003 and 2019, 277 NBs were surgically resected in the department of pediatric surgery of Dr. von Hauner Children's Hospital. Out of these, 11 patients (3.9%) had pelvic NB. Evaluation of the preoperative imaging showed two patients without IDRF (stage L1) and eight patients in stage L2. One patient had stage M according to distant metastasis. Patients without IDRF underwent complete macroscopical resections, whereas complete tumor removal was not possible without mutilation in patients with IDRF. At time point of diagnosis, only patients with IDRF had functional neurological problems. Three patients developed perioperative complications; all of them had at least one IDRF. Three patients developed local recurrence during the course of the disease, all of them had at least one IDRF.  Our results indicate on a preliminary level the importance of IDRF as a prognostic tool for surgical removal of pelvic NB.

Froeba-Pohl A ，von Schweinitz D ，Muehling J ，Paolini M ，Hubertus J ... -  《-》

Image-defined Risk Factors Correlate with Surgical Radicality and Local Recurrence in Patients with Neuroblastoma.

Neuroblastoma is the second most common solid pediatric tumor and the most common cancer to be detected in children younger than 12 months of age. To date, 2 different staging systems describe the extent of the disease: the International Neuroblastoma Staging System (INSS) and the International Neuroblastoma Risk Group Staging System (INRGSS). The INRGSS-system is characterized by the presence or absence of so called image-defined risk factors (IDRFs), which are described as surgical risk factors. We hypothesized that IDRFs correlate with surgical complications, surgical radicality, local recurrence and overall survival (OS). Between 2003 and 2010, 102 patients had neuroblastoma surgery performed in our department. We analyzed medical records for IDRF-status and above named data. 16 patients were IDRF-negative, whereas 86 patients showed one or more IDRF. Intra- or postoperative complications have been reported in 21 patients (21%). 19 of them showed one or more IDRF and 2 patients were IDRF-negative (p=n.s.). Patients who suffered from intra- or postoperative complications demonstrated a decreased OS (p=0.011). Statistical analysis revealed an inverse correlation between the extent of macroscopical removal and IDRF-status (p=0.001). Furthermore, the number of IDRFs were associated with a decreased likelihood of radical tumor resection (p<0.001). 19 patients had local recurrence; all of them were IDRF-positive (p=0.037). Pediatric surgeons should consider IDRFs as a useful tool for risk assessment and therefore planning for neuroblastoma surgery.

Pohl A ，Erichsen M ，Stehr M ，Hubertus J ，Bergmann F ，Kammer B ，von Schweinitz D ... -  《-》

Image-defined risk factors in localized thoracic neuroblastoma and ganglioneuroma.

The pretreatment International Neuroblastoma Risk Group Staging System (INRGSS) discriminates localized tumors L1/L2 depending on the absence/presence of image-defined risk factors (IDRFs) at diagnosis. Referring to this new staging system, we assessed initial imaging of localized thoracic neuroblastoma (NB) and ganglioneuroma (GN) and the extent of initial tumor resection. Patients with localized thoracic NB/GN from the German clinical trials NB97 and NB2004 were included. Imaging at diagnosis and operative reports were reviewed retrospectively. IDRFs were assessed centrally and correlated to International Neuroblastoma Staging System (INSS) stage and extent of tumor resection. Additionally, we analyzed data on surgery-related complications. Imaging series of 88 patients were available for central review. In 18 children, no IDRF was present, 28 exhibited one IDRF, 42 two or more IDRFs, resulting in 70 patients with L2 disease. The most frequently observed IDRF was encasement of any vessel (n = 38). Initial surgical resection was aimed for in 45 patients (L1: n = 11; L2: n = 34). Complete and gross total resection rates were higher children with L2 NB (n = 8/25 L1, n = 17/25 L2 vs. n = 2/15 L1, n = 13/15 L2, respectively). The proportion of surgical complications was very similar between INRGSS L1 and L2 (n = 4/11 vs. n = 17/34). All complications were manageable, and no surgery-related deaths were observed. In this retrospective cohort, the extent of resection and the rate of surgical complications did not differ substantially between patients classified as L1/L2, indicating that INRGSS L2 does not equate unresectability. It appeared that individual IDRFs differ in value. Larger studies are needed to assess the significance and therapeutic/prognostic impact of such findings.

Butzer SK ，Krug B ，Reisberg M ，Fuchs J ，Warmann SW ，Hubertus J ，Körber F ，Berthold F ，Simon T ，Hero B ... -  《-》

Validity of image-defined risk factors in localized neuroblastoma: A report from two centers in Western Japan.

Japanese Neuroblastoma Study Group (JNBSG) has been employing image-defined risk factors (IDRFs) since 2010. However, the report from INRG in 2011 supplemented description stating that isolated contact is considered to be IDRF-positive only in renal vessels. The aim of this study was to evaluate the validity of IDRFs by comparing the previous (PG) and new guidelines (NG). IDRFs of patients with localized neuroblastoma treated at two centers in Western Japan from 2002 to 2013 were retrospectively reviewed by radiologists. 47 neuroblastomas (abdomen 38, pelvis 2, mediastinum 7) were evaluated. For abdominal neuroblastomas, IDRFs were present in 15/38 (39.5%) using PG and in 31/38 (81.6%) using NG. Moreover, the IDRF-positive rate increased from 26.7% (4/15) to 80.0% (12/15) in 15 cases diagnosed during mass screening. Of the IDRF-positive cases, complete primary resection was achieved in 2/15 (13.3%) using PG and 17/31 patients (54.8%) using NG. There were two major surgical renal complications in the IDRF-positive cases based on the use of either guidelines, and the specificity decreased from 64% to 19%. According to NG, the IDRF-positive rate increased, and the resection rate decreased. NG may overestimate surgical risks, leading to unnecessary chemotherapy and a prolonged hospital stay.

Fumino S ，Kimura K ，Iehara T ，Nishimura M ，Nakamura S ，Souzaki R ，Nishie A ，Taguchi T ，Hosoi H ，Tajiri T ... -  《-》

Can Image-Defined Risk Factors Predict Surgical Complications in Localized Neuroblastoma?

Image-defined risk factors (IDRFs) have been propounded for predicting the surgical risks associated with localized neuroblastoma (NB) since 2009. In 2011, a new guideline (NG) for assessing IDRFs was published. According to the NG, the situation in which "the tumor is only in contact with renal vessels," should be considered to be "IDRF-present." Previously, this situation was diagnosed as "IDRF absent." In this study, we evaluated the IDRFs in localized NB patients to clarify the predictive capability of IDRFs for surgical complications, as well as the usefulness of the NG. Materials and A total of 107 localized patients with NB were included in this study. The enhanced computed tomography and magnetic resonance images from the time of their diagnoses were evaluated by a single radiologist. We also analyzed the association of clinical factors, including the IDRFs (before and after applying the NG), with surgical complications. Of the 107 patients, 33 and 74 patients were diagnosed as IDRF-present (OP group), and IDRF-absent (ON group) before the NG, respectively. According to the NG, there were 76 and 31 patients who were classified as IDRF-present (NP group) and IDRF absent (NN group), respectively. Thus, 43 (40%) patients in the ON group were reassigned to the NP group after the NG. Surgical complications were observed in 17 of 82 patients who underwent surgical resection. Of the patients who underwent secondary operations, surgical complication rates were 55% in the OP group and 44% in the NP group. According to a univariate analysis, non-INSS 1, IDRFs before and after the NG and secondary operations were significantly associated with surgical complications. In a multivariate analysis, non-INSS 1 status and IDRFs after the NG were significantly associated with surgical complications.

Yoneda A ，Nishikawa M ，Uehara S ，Oue T ，Usui N ，Inoue M ，Fukuzawa M ，Okuyama H ... -  《-》