Harlequin syndrome associated with ganglioneuroblastoma-induced Horner syndrome

Childs Nerv Syst. 2021 Aug;37(8):2683-2686. doi: 10.1007/s00381-020-04948-1. Epub 2020 Oct 30.

Abstract

A 1-year-old boy presented with a 4-month history of hypertension, ptosis of the right upper eyelid, left hemifacial sweating, and flushing. He was diagnosed with Harlequin syndrome associated with Horner syndrome. Computed tomography revealed a mass lesion in the right superior mediastinum. Therefore, the patient underwent total tumor resection. Histological examination demonstrated ganglioneuroblastoma. The MYCN oncogene was not amplified, and the mitosis-karyorrhexis index was low. Accordingly, radiation and chemotherapy were not performed. No recurrence was observed within 8 months after surgery, and the patient's blood pressure was normalized. However, the ptosis, hemifacial sweating, and flushing persisted.

Keywords: Ganglioneuroblastoma; Harlequin syndrome; Horner syndrome; Neuroblastoma.

Publication types

  • Case Reports

MeSH terms

  • Autonomic Nervous System Diseases
  • Flushing / etiology
  • Ganglioneuroblastoma* / complications
  • Ganglioneuroblastoma* / diagnostic imaging
  • Ganglioneuroblastoma* / surgery
  • Horner Syndrome* / etiology
  • Humans
  • Hypohidrosis
  • Infant
  • Male
  • Neoplasm Recurrence, Local

Supplementary concepts

  • Harlequin syndrome