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Primitive neuroectodermal tumor arising in the pancreas

D B Danner1, R H Hruban, H A Pitt

  • 1Department of Pathology, Johns Hopkins Medical Institutions, Baltimore, Maryland.

Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc
|February 1, 1994
PubMed
Summary

Peripheral primitive neuroectodermal tumors (PNETs) are rare pancreatic cancers. This case report details a PNET in a 17-year-old male, emphasizing diagnostic challenges in visceral organs.

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Area of Science:

  • Oncology
  • Pathology
  • Genetics

Background:

  • Peripheral primitive neuroectodermal tumors (PNETs) are rare small round blue cell tumors with neuroectodermal differentiation.
  • Typically found in soft tissue or bone, PNETs predominantly affect children and young adults.

Observation:

  • A 17-year-old male presented with a pancreatic mass.
  • Histological examination revealed small round cells positive for cytokeratin, neuron-specific enolase, and 12E7.
  • Immunohistochemistry excluded neuroendocrine and myogenic markers.

Findings:

  • The pancreatic neoplasm was diagnosed as a PNET based on characteristic histology and immunohistochemistry.
  • Cytogenetic analysis confirmed the diagnosis by identifying the PNET-specific chromosomal translocation t(11;12)(q24;q12).

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Implications:

  • This case underscores the diagnostic difficulties of PNETs in unusual visceral locations.
  • Distinguishing PNETs from more common pancreatic neoplasms like neuroendocrine tumors and adenocarcinomas is crucial.