Pancreatic adenocarcinosarcoma of monoclonal origin: a case report

World J Gastroenterol. 2014 Sep 21;20(35):12682-6. doi: 10.3748/wjg.v20.i35.12682.

Abstract

Adenocarcinosarcoma, a neoplasm containing both carcinomatous and sarcomatous components, is a rare form of a cancer and the pathophysiology is currently poorly understood. Moreover, definitive treatment guidelines for this disease have not yet been established. Pancreatic adenocarcinosarcoma is even more rare and the prognosis is fatal. Here, we report a case of a 77-year-old male with pancreatic adenocarcinosarcoma and metastasis to the liver. The patient presented at our hospital with uncontrolled glucose levels and diabetes mellitus. The patient's laboratory findings were unremarkable with the exception of elevated carbohydrate antigen 19-9 levels. Biopsies of the tumors in the pancreas and the liver revealed two types of tumors: pancreatic adenocarcinoma and a poorly differentiated sarcoma. To determine if KRAS mutations were present, we performed a peptide nucleic acid (PNA) clamp PCR-based assay. DNA sequencing by PNA clamp PCR identified a point mutation in codon 12 of exon 2 within KRAS from both tumor types. Because the KRAS mutation is observed in both tumor components, our findings support a monoclonal tumor origin followed by subsequent divergent differentiation into the sarcomatous and carcinomatous tumor populations. After we considered the patient's status and the late stage of tumor detection, gemcitabine chemotherapy was administered.

Keywords: Adenocarcinosarcoma; DNA sequencing; KRAS; Metastasis; Pancreas.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adenocarcinoma / blood
  • Adenocarcinoma / chemistry
  • Adenocarcinoma / drug therapy
  • Adenocarcinoma / genetics*
  • Adenocarcinoma / secondary
  • Aged
  • Antimetabolites, Antineoplastic / therapeutic use
  • Biopsy
  • CA-19-9 Antigen / blood
  • Cell Differentiation
  • DNA Mutational Analysis
  • Deoxycytidine / analogs & derivatives
  • Deoxycytidine / therapeutic use
  • Exons
  • Gemcitabine
  • Genetic Predisposition to Disease
  • Humans
  • Immunohistochemistry
  • Liver Neoplasms / blood
  • Liver Neoplasms / chemistry
  • Liver Neoplasms / drug therapy
  • Liver Neoplasms / genetics*
  • Liver Neoplasms / secondary
  • Magnetic Resonance Imaging
  • Male
  • Neoplasms, Complex and Mixed / blood
  • Neoplasms, Complex and Mixed / chemistry
  • Neoplasms, Complex and Mixed / drug therapy
  • Neoplasms, Complex and Mixed / genetics*
  • Neoplasms, Complex and Mixed / pathology
  • Pancreatic Neoplasms / blood
  • Pancreatic Neoplasms / chemistry
  • Pancreatic Neoplasms / drug therapy
  • Pancreatic Neoplasms / genetics*
  • Pancreatic Neoplasms / pathology
  • Phenotype
  • Point Mutation*
  • Proto-Oncogene Proteins / genetics*
  • Proto-Oncogene Proteins p21(ras)
  • Sarcoma / blood
  • Sarcoma / chemistry
  • Sarcoma / drug therapy
  • Sarcoma / genetics*
  • Sarcoma / secondary
  • Tomography, X-Ray Computed
  • Up-Regulation
  • ras Proteins / genetics*

Substances

  • Antimetabolites, Antineoplastic
  • CA-19-9 Antigen
  • KRAS protein, human
  • Proto-Oncogene Proteins
  • Deoxycytidine
  • Proto-Oncogene Proteins p21(ras)
  • ras Proteins
  • Gemcitabine