Neuroblastoma/Ganglioneuroblastoma

Neuroblastoma/Ganglioneuroblastoma
Key Facts
Terminology
  • Neuroblastoma (NB), ganglioneuroblastoma (GNB)
  • Differentiating neuroblastoma and immature ganglioneuroma both synonyms for GNB
  • Malignant neoplasm composed of immature neural elements (NB) that may be admixed with ganglioneuromatous component (GNB)
Etiology/Pathogenesis
  • GNB is believed to represent a transitional tumor of sympathetic cell orgin
  • NB is a tumor believed to be of neural crest orgin
Clinical Issues
  • Tumor is more common in younger individuals
  • Usually in patients < 15 years of age
  • NB and GNB are usually in posterior mediastinum
  • Prognosis
    • Depends on clinical stage at time of diagnosis
    • Depends on degree of differentiation of tumor
    • Children appear to have more favorable outcome
Top Differential Diagnoses
  • Neuroblastoma
  • Ganglioneuroma (GN)
  • Rhabdomyosarcoma
  • Ewing sarcoma/PNET
Diagnostic Checklist
  • Small round blue cell tumor
  • Areas of neuropil
  • Presence of ganglion cells or precursors
Low-power view shows a ganglioneuroblastoma with predominant ganglioneuromatous component image. Note the presence of the small cluster of cells representing the neuroblastomatous component image.
High-power view shows the neuroblastomatous image component in a GNB. Note that in the periphery there are still identifiable ganglion cells image.
TERMINOLOGY
Abbreviations
  • Neuroblastoma (NB), ganglioneuroblastoma (GNB)
Synonyms
  • Differentiating neuroblastoma and immature ganglioneuroma both synonyms for GNB
Definitions
  • Malignant neoplasm composed of immature neural elements (NB) that may be admixed with ganglioneuromatous component (GNB)
ETIOLOGY/PATHOGENESIS
Etiology
  • GNB is believed to represent a transitional tumor of sympathetic cell orgin
  • NB is a tumor believed to be of neural crest orgin
CLINICAL ISSUES
Epidemiology
  • Incidence
    • Unusual tumors in mediastinal compartment
  • Age
    • Tumor more common in younger individuals
    • Usually in patients < 15 years old
  • Gender
    • No gender predilection
    • NB may be more commonly seen in male patients
Site
  • NB and GNB are usually in posterior mediastinum
  • Unusual cases may be in anterior mediastinum
Presentation
  • Chest pain
  • Paraplegia
  • Horner syndrome
  • Chronic diarrhea
  • Various congenital anomalies
  • Asymptomatic
Tags:
Jul 9, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Neuroblastoma/Ganglioneuroblastoma

Full access? Get Clinical Tree
Get Clinical Tree app for offline access