Eosinophilic esophagitis as paraneoplastic syndrome in a patient with ganglioneuroblastoma

S Prader1, J Spalinger2, J Caduff3

  • 1Immunology, University Children's Hospital Zurich, Zurich, Switzerland.

Klinische Padiatrie
|May 19, 2015
PubMed

Insights

Eosinophilic esophagitis in a child resolved after removing a rare nerve tumor, suggesting a paraneoplastic syndrome. This case highlights a potential link between ganglioneuroblastoma and gastrointestinal symptoms.

Area of Science:

  • Pediatric Gastroenterology
  • Pediatric Oncology
  • Clinical Medicine

Background:

  • Eosinophilic esophagitis (EoE) is an immune-mediated esophageal disease.
  • Paraneoplastic syndromes can manifest with diverse symptoms, including gastrointestinal issues.
  • Ganglioneuroblastoma is a rare tumor typically affecting the sympathetic nervous system.

Observation:

  • A 16-month-old boy presented with failure to thrive, hypersalivation, abdominal pain, chronic diarrhea, and blepharitis.
  • Initial diagnosis of eosinophilic esophagitis (EoE) did not improve with standard treatments.
  • Further investigation revealed an intrathoracic, paraspinal ganglioneuroblastoma.

Findings:

  • Surgical resection of the ganglioneuroblastoma led to the complete resolution of all presenting symptoms.
  • Esophageal biopsies confirmed the absence of eosinophilic esophagitis post-tumor removal.
  • This case represents the first reported instance of EoE as a paraneoplastic syndrome associated with a non-carcinomatous malignancy.

Implications:

  • Suggests a potential paraneoplastic etiology for eosinophilic esophagitis in pediatric cases, particularly with non-carcinomatous tumors.
  • Highlights the importance of a comprehensive diagnostic work-up for refractory pediatric gastrointestinal symptoms.
  • Underscores the need to consider rare tumors in the differential diagnosis of complex pediatric presentations.