Opsoclonus-myoclonus ataxia syndrome is primarily associated with which type of tumor in children?

Prepare for USMLE Step 1 Pathology Exam with comprehensive quizzes, flashcards, and detailed explanations. Enhance your understanding and be exam-ready!

Opsoclonus-myoclonus ataxia syndrome (OMAS), also known as "dancing eyes-dancing feet syndrome," is primarily associated with neuroblastoma in children. This paraneoplastic syndrome occurs due to an autoimmune response triggered by the tumor, leading to the characteristic movement disorders such as opsoclonus (rapid, uncontrolled eye movements), myoclonus (sudden muscle jerks), and ataxia (loss of coordination).

Neuroblastoma is the most common extracranial solid tumor in children and often presents with a variety of paraneoplastic syndromes, one of which is OMAS. The syndrome is thought to result from antibodies that target neuronal tissue due to the presence of the neuroblastoma. This association highlights the importance of recognizing the links between specific tumors and their corresponding paraneoplastic syndromes, which can aid in timely diagnosis and management.

While other tumors like ovarian teratoma and thymoma can also cause paraneoplastic syndromes, they are less commonly associated with opsoclonus-myoclonus ataxia syndrome specifically in the pediatric population, making neuroblastoma the clear answer. Understanding these associations is crucial for clinical practice, particularly in pediatrics, where early recognition

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