William R Johnston1, Rosa Hwang1, Peter Mattei2. 1. General, Thoracic, and Fetal Surgery, Department of Surgery, Wood 5, Children's Hospital of Philadelphia, 34th St. and Civic Center Blvd., Philadelphia, PA, 19104, USA. 2. General, Thoracic, and Fetal Surgery, Department of Surgery, Wood 5, Children's Hospital of Philadelphia, 34th St. and Civic Center Blvd., Philadelphia, PA, 19104, USA. mattei@chop.edu.
Abstract
INTRODUCTION: Neuroblastoma is a childhood cancer of neural crest cells occasionally associated with opsoclonus-myoclonus-ataxia syndrome (OMAS), a paraneoplastic process characterized by ataxia, rapid eye movements, and muscle twitching. OMAS treatment and outcomes are well studied, but prior reports do not detail how the presence of OMAS should impact surgical approach, particularly for tumors with image defined risk factors (IDRF). METHODS: We reviewed patients with neuroblastoma and OMAS at our institution from January 2009 to December 2020 and recorded tumor characteristics, operative details, OMAS therapies, and outcomes. RESULTS: We identified 14 patients with neuroblastoma and OMAS out of 212 patients referred for surgery. There were 11 gross total resections and three partial resections. Two patients with partial resections developed OMAS after initial resection. One patient with gross total resection developed tumor recurrence 10 years later with OMAS redevelopment signaling recurrence. Three patients were positive for IDRFs and the one receiving neoadjuvant therapy achieved a gross total resection. CONCLUSIONS: OMAS development after partial resection and OMAS recurrence following tumor recurrence indicates a correlation between tumor bulk and the paraneoplastic process. This justifies an aggressive resection even for low-risk tumors. Neoadjuvant therapy should be considered for potentially unresectable tumors with image defined risk factors. LEVEL OF EVIDENCE: IV.
INTRODUCTION: Neuroblastoma is a childhood cancer of neural crest cells occasionally associated with opsoclonus-myoclonus-ataxia syndrome (OMAS), a paraneoplastic process characterized by ataxia, rapid eye movements, and muscle twitching. OMAS treatment and outcomes are well studied, but prior reports do not detail how the presence of OMAS should impact surgical approach, particularly for tumors with image defined risk factors (IDRF). METHODS: We reviewed patients with neuroblastoma and OMAS at our institution from January 2009 to December 2020 and recorded tumor characteristics, operative details, OMAS therapies, and outcomes. RESULTS: We identified 14 patients with neuroblastoma and OMAS out of 212 patients referred for surgery. There were 11 gross total resections and three partial resections. Two patients with partial resections developed OMAS after initial resection. One patient with gross total resection developed tumor recurrence 10 years later with OMAS redevelopment signaling recurrence. Three patients were positive for IDRFs and the one receiving neoadjuvant therapy achieved a gross total resection. CONCLUSIONS: OMAS development after partial resection and OMAS recurrence following tumor recurrence indicates a correlation between tumor bulk and the paraneoplastic process. This justifies an aggressive resection even for low-risk tumors. Neoadjuvant therapy should be considered for potentially unresectable tumors with image defined risk factors. LEVEL OF EVIDENCE: IV.