title: Primary hepatic neuroendocrine carcinoma in a child. Morphologic, immunocytochemical, and molecular biologic studies.
abstract: BACKGROUND
  Primary neuroendocrine tumor of the liver is uncommon, and virtually all reported patients with the tumor have been adults. Most of the tumors were carcinoids. The authors report an 8-year-old girl with primary hepatic neuroendocrine carcinoma.
  
  
  METHODS
  Histologic, ultrastructural, and immunocytochemical studies and Southern blot analysis of tumor N-myc DNA were performed in the patient.
  
  
  RESULTS
  Histologically, the tumor revealed a characteristic organoid pattern with a spectrum of differentiation varying from well-differentiated carcinoid-like areas to poorly differentiated pleomorphic areas. Ultrastructural features included neurosecretory granules and interdigitating cytoplasmic extensions. The tumor cells showed immunoreactivity to neuron-specific enolase (NSE), S-100 protein, chromogranin, and synaptophysin. No evidence of amplification of tumor N-myc DNA was present. However, the molecular weight of the tumor N-myc DNA (1.8 kb) was significantly lower than the normal control (from normal liver tissue) (2.0 kb).
  
  
  CONCLUSIONS
  This report documents the occurrence of primary hepatic neuroendocrine carcinoma in a child. Thorough studies and complete clinical evaluation are essential to the establishment of diagnosis. The result of N-myc DNA analysis probably is attributable to deletion of part of the tumor N-myc gene. The clinical implication of this finding is unknown, and additional investigation is warranted.
authors.affiliation: Department of Pathology, Children's Memorial Hospital, Northwestern University Medical School, Chicago, Illinois 60614.
authors.forename: C
authors.initials: C
authors.lastname: Hsueh
authors.forename: X D
authors.initials: XD
authors.lastname: Tan
authors.forename: F
authors.initials: F
authors.lastname: Gonzalez-Crussi
mesh_terms: D002648:Child; D005260:Female; D006801:Humans; D008113:Liver Neoplasms / Q000473:pathology* / Q000648:ultrastructure; D009361:Neoplasm Invasiveness; D009490:Neurosecretory Systems / Q000473:pathology* / Q000648:ultrastructure