J. R. Jackson1, H. Tran2, J. Stein1, H. Shimada4, A. M. Patel3, A. Marachelian2, E. S. Kim1 3Children’s Hospital Los Angeles,Otolaryngology,Los Angeles, CA, USA 4Children’s Hospital Los Angeles,Pathology,Los Angeles, CA, USA 1Children’s Hospital Los Angeles,Pediatric Surgery,Los Angeles, CA, USA 2Children’s Hospital Los Angeles,Hematology/Oncology,Los Angeles, CA, USA
Introduction:
Neuroblastoma is a neural crest malignancy of childhood that arises from the sympathetic nerve chain from the neck to the pelvis. While studies have shown that extra-abdominal neuroblastoma (pelvic and thoracic) is associated with favorable biological and clinical characteristics, little has been published with regard to the management and outcomes of cervical neuroblastic tumors. Cervical neuroblastoma represents 2-4% of all neuroblastomas, and current practice is to resect as much tumor as possible without incurring injury to nearby vital structures. In this study we sought to determine the characteristics of these tumors and the effect that the extent of resection has on the overall survival and rate of complications in these patients.
Methods:
We performed a retrospective review of 325 children who were identified to have a neuroblastic tumor at Children’s Hospital Los Angeles over a 15-year period (1/1990–2/2015). Data collected from the medical record included location of tumor, age at diagnosis, age at resection, extent of resection, chemotherapy course, INSS stage, INPC histological classification, and MYCN amplification. Outcome variables included postoperative complications and overall survival.
Results:
Thirteen patients (13/325 – 4%) were identified to have cervical neuroblastic tumors (Table 1); 10 patients (77%) with neuroblastoma (NB), 1 patient with ganglioneuroblastoma (GNB)(7.7%), 2 patients with ganglioneuroma (GN)(15.4%). Median age at diagnosis was 5 months (range 1 mo-15 yrs). One 15 year old had high-risk stage 4 NB with unfavorable histology while the other 9 NB patients were infants <12 months of age. The remaining 3 patients (18-66 mos of age) had differentiating pathology (GN, GNB). All but one had favorable histology and none had MYCN amplification. Six of 10 NB patients underwent resection (5 gross total resection (GTR) and 1 partial resection) while the other 4 underwent biopsy followed by chemotherapy or observation. After GTR, 1 NB patient required prolonged intubation necessitating tracheostomy and another developed eyelid ptosis. The GNB patient, also post-GTR, developed Horner syndrome. The 2 GN patients underwent GTR as well, which resulted in permanent injury to 5 cranial nerves and eyelid ptosis. At latest follow-up, there has been 1 death secondary to relapsed disease.
Conclusion:
Cervical neuroblastic tumors represent favorable lesions with good outcomes similar to other extra-abdominal neuroblastic tumors. In our study, survival was excellent regardless of extent of tumor resection. Based on our data, with the high incidence of complications following GTR, we recommend a minimally agressive surgical approach in managing children with cervical neuroblastic tumors.
