Primary hepatic neuroendocrine carcinoma in a child morphologic, immunocytochemical, and molecular biologic studies

Chuen Hsueh*, Xiaodi Tan, F. Gonzalez‐Crussi

*Corresponding author for this work

Research output: Contribution to journalArticle

44 Scopus citations

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 immuno‐cytochemical 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. Ultra‐structural 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.

Original languageEnglish (US)
Pages (from-to)2660-2665
Number of pages6
JournalCancer
Volume71
Issue number8
DOIs
StatePublished - Jan 1 1993

Keywords

  • carcinoid
  • child
  • hepatic tumor
  • liver
  • neuroendocrine carcinoma

ASJC Scopus subject areas

  • Oncology
  • Cancer Research

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