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

Abstract

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.