Surgical considerations for neuroblastoma-associated opsoclonus–myoclonus–ataxia syndrome: a series of 14 patients from a single institution
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In brief
In brief
Single-institution review of 14 neuroblastoma patients with opsoclonus-myoclonus-ataxia syndrome demonstrates correlation between tumor burden and paraneoplastic symptoms, with OMAS developing after partial resection and recurring with tumor recurrence. Findings support aggressive surgical resection even for low-risk tumors and consideration of neoadjuvant therapy for cases with image defined risk factors.
Written by the GCMD Library team from the article.
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.