Complications of Advanced Kadish Stage Esthesioneuroblastoma: Single Institution Experience and Literature Review

INTRODUCTION: In esthesioneuroblastoma, greater disease extent and Kadish staging correlate with greater recurrence, complications, and mortality. These advanced stage malignancies require extensive resections and aggressive adjuvant therapy. This increases the risk of complications such as cerebrospinal fluid leak, neurologic deficits, and osteomyelitis. We present our case series and then analyze the literature to ascertain whether advanced stage tumors corresponds to greater rates of complications. METHODS: A retrospective review of consecutive patients with histologically-proven esthesioneuroblastoma who were aggressively managed at our institution was performed. This was followed by an extensive literature search of published original data, in large series from 2006-2016, where both surgery and adjuvant therapy were used for the treatment of esthesioneuroblastoma. RESULTS : Single institution review revealed eight patients with esthesioneuroblastoma, half with advanced Kadish staging. All Kadish A patients ( Kadish A: confined to nasal cavity) underwent endoscopic approaches alone, while Kadish C patients (Kadish C: extends beyond nasal cavity and paranasal sinuses) and D patients (Kadish D: lymph node or distant metastases) underwent craniofacial approaches, while all patients received post-operative adjuvant therapies. Complications such as cerebrospinal fluid (CSF) leak, seizures, meningitis, and abscess only occurred in high Kadish stage patients. Literature review demonstrated a higher proportion of advanced Kadish stage cases correlated with increasing rates of pneumocephalus, infection, and recurrence. A higher proportion of Kadish C and D tumors was inversely correlated with CSF leak rate and overall survival. DISCUSSION: Advanced stage tumors are often associated with a higher incidence of adverse events up to 33%, both due to disease burden and treatment effect. There is increasing use of endoscopy and neoadjuvant therapy, which have the potential to decrease complication rates. CONCLUSION : Advanced Kadish stage esthesioneuroblastoma necessitates meticulous surgical resection and aggressive adjuvant therapies, together, these increase the likelihood of adverse events, including CSF leak, neurologic deficits, and infections, and may represent the real morbidity cost of radically treating these tumors to achieve an improvement in overall survival. In selected patients, less-invasive approaches or neo-adjuvant therapies can be used without compromising on a curative resection.