Neuroblastoma Presentation With Multiple Cranial Nerve Involvement

Abstract

Objective NA. Background We report a case of neuroblastoma, a pediatric neuroendocrine tumor of the sympathetic nervous system, in a 3-year-old female with multiple cranial nerve involvement. Design/Methods A 3-year-old afebrile, lethargic female presented with bilateral eyelid droop, right head tilt, slurred speech, gagging, abnormal walking and no bowel movement. Neurological examination noted bilateral ptosis, dysarthria, left tongue deviation, proximal weakness in upper and lower extremities, areflexia in biceps and patellar tendons, dysmetria, and wide-based gait. MRI of the brain showed heterogeneous appearance of the clivus and MRI of the spine showed right adrenal mass and heterogeneous enhancement of multiple vertebrae, suggesting possible metastatic disease. Serum and cerebrospinal studies were unremarkable. Patient was treated with intravenous methylprednisolone and plasmapheresis for suspected paraneoplastic syndrome; however, she continued to clinically progress. Adrenal mass biopsy results and elevated urine VMA and HVA levels were consistent with the diagnosis of neuroblastoma. Nuclear imaging and meta-iodobenzylguanidine scan were negative. Paraneoplastic panels and Lambert Eaton panel were negative for autoantibodies. Total resection of the abdominal mass and right adrenal gland with continued steroid taper, resulted in reported near total symptom resolution. Results Our patient presented with antibody negative paraneoplastic polyneuropathy due to neuroblastoma. Cranial nerve involvement in neuroblastoma can result from tumor involvement of the sympathetic chain, paraneoplastic syndromes, or metastasis to the skull. Our patient's initial imaging suggested potential metastatic or inflammatory involvement of the clivus, suspected of causing her cranial nerve symptoms. However, nuclear studies were negative for metastatic disease and patient's symptoms resolved after resection of the main mass suggesting a paraneoplastic etiology. Although her paraneoplastic panel and Lambert-Eaton panel were negative for common associated antibodies, it is likely an unmeasured autoantibody, cytokine, hormones, or peptide was at play. Conclusions Neuroblastoma should be considered as a differential for a neurological presentation involving multiple cranial nerves in a child.