157 - Incidence of Horner’s Syndrome in Pediatric Neuroblastoma
Saturday, April 29, 2023
3:30 PM – 6:00 PM ET
Poster Number: 157 Publication Number: 157.22
Nicholas J. Kelly, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, United States; Rosa Hwang, Childrens Hospital of Philadelphia, Philadelphia, PA, United States; Peter Mattei, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, United States
Medical Student Perelman School of Medicine at the University of Pennsylvania Philadelphia, Pennsylvania, United States
Background: Neuroblastoma (NBL) is the most common extracranial solid organ tumor in children and adolescents, with an incidence of 1.4 per 100,000 patients under 19 years of age. As the tumor arises from neural crest-derived tissue, it can occur anywhere along the sympathetic chain and manifests clinically in a variety of ways. Horner syndrome (HS), the result of a disruption of the three-neuron oculosympathetic pathway, is characterized by ptosis, miosis, and anhidrosis. Although rare, HS can be the first sign of occult NBL, even in the absence of other signs or symptoms. Despite this, the incidence of HS in pediatric NBL patients is understudied, and sparse guidelines exist to direct the oncologic workup of children with this syndrome. Objective: To characterize the incidence of Horner syndrome in patients with neuroblastoma. Design/Methods: We reviewed patients with NBL and HS at our institution from August 2002 to December 2020 and recorded tumor characteristics, operative details, and outcomes. Results: We identified 16 patients (9 males, 7 females, mean age 4.4y, IQR 0.64-8.97y) with both NBL and HS out of 227 patients referred for surgery. Of these, six had Horner syndrome at initial presentation. Ten subsequently developed HS as a long-term sequela of treatment. Four of the six patients who presented with Horner syndrome did so in the absence of other signs or symptoms suggestive of NBL. Primary tumor location among patients with HS was widely distributed, including four in the adrenal medulla, four in the paraspinal sympathetic ganglia, six in the chest, one in the neck, and one in the thorax. Mean tumor size was 4.3 cm. Five of 16 relapsed, and four patients died, three of whom had ganglioneuroblastoma-nodular type with an unfavorable histology.
Conclusion(s): We identified 16 patients with NBL and HS. Four of the six with HS at presentation had no other symptoms. These findings suggest that, in the absence of known causes of HS (birth trauma, iatrogenic injury), children who present with Horner syndrome should undergo a diagnostic workup for malignancy.
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