M. J. Zobel1, A. K. Zamora1, L. Wang1,2, J. E. Stein1,2, A. Marachelian1,2, E. S. Kim1,2 1Children’s Hospital Los Angeles,Los Angeles, CA, USA 2Keck School of Medicine, University Of Southern California,Los Angeles, CA, USA
Introduction:
Pelvic neuroblastomas are rare and often present in children as massive tumors whose surgical resection following chemotherapy can be associated with significant morbidity given involvement of sacral nerve roots and close proximity to pelvic vascular structures. We sought to examine the characteristics of pelvic neuroblastoma at our institution and the effect of extent of surgical resection on survival and surgical outcomes.
Methods:
After IRB approval, a retrospective chart review of 554 neuroblastoma patients was performed at Children’s Hospital Los Angeles over an 18-year period (2000-2018). Collected data included tumor location, size, histology, stage and risk classification, MYCN oncogene amplification, use of pre-operative chemotherapy and/or radiation, and extent of surgical resection. Outcome variables included postoperative complications and survival.
Results:
Ten patients (7 females: 3 males) with primary pelvic neuroblastoma tumors were identified. The median age at diagnosis was 4.2 years (5 months to 11 years). Five patients presented with a localized pelvic tumor; four with stage 1 and one with stage 2. Five patients presented with advanced disease; two patients with stage 3 and three patients with metastatic stage 4 disease. The mean post-operative follow-up was 3.9 years with overall survival of 90%. One patient died from extensive metastatic disease for which no resection was attempted. Of the remaining 9 patients, surgical resection was attempted. Five patients with localized tumors (stage 1 or 2) underwent gross total resection, with one complication of a small bowel obstruction seven years post-operatively. The other 4 patients with advanced disease (stage 3 or stage 4) received neoadjuvant chemotherapy, followed by partial resection (30-90% debulked). Of these, one patient, whose tumor encased the left internal iliac vein, which resulted in ligation, subsequently experienced lower extremity hypotonia following a gross total resection attempt.
Conclusion:
Our data shows that patients with pelvic neuroblastoma have an excellent overall survival following surgery. Patients with localized pelvic neuroblastomas who undergo gross total resection and those with advanced disease who undergo neoadjuvant therapy followed by partial resection have good outcomes with minimal morbidity. Based on these findings, we recommend that small localized pelvic neuroblastoma undergo gross total resection or even be observed, while large unresectable tumors undergo neoadjuvant chemotherapy followed by a partial debulking resection to avoid neurovascular morbidity.