Reticular angiomatoid "malignant" fibrous histiocytoma--a case report with cytogenetics and molecular genetic analyses

Hum Pathol. 2011 Sep;42(9):1359-63. doi: 10.1016/j.humpath.2010.12.003. Epub 2011 Mar 15.


Angiomatoid "malignant" fibrous histiocytoma is a rare sarcoma of low malignant potential that occurs most commonly in the extremities of children and young adults. Herein, we present a case of angiomatoid malignant fibrous histiocytoma with unusual histologic features arising in the mediastinum of an 80-year-old man. The tumor exhibited a reticular growth pattern and myxoid stroma. The tumor cells expressed epithelial membrane antigen and desmin. Cytogenetic analysis revealed the translocation t(2;22)(q33;q12). Molecular genetic analysis confirmed the rearrangement of the EWSR1 locus and the presence of the EWSR1/CREB1 fusion. This report expands the clinicopathologic spectrum of angiomatoid malignant fibrous histiocytoma and underscores the value of integrating morphologic, immunophenotypic, and molecular findings in the identification of its unusual morphologic variants.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged, 80 and over
  • Calmodulin-Binding Proteins / genetics
  • Chromosomes, Human, Pair 2 / genetics
  • Chromosomes, Human, Pair 22 / genetics
  • Cyclic AMP Response Element-Binding Protein / genetics
  • Cytogenetic Analysis
  • Histiocytoma, Malignant Fibrous / genetics
  • Histiocytoma, Malignant Fibrous / pathology*
  • Humans
  • Male
  • Mediastinal Neoplasms / genetics
  • Mediastinal Neoplasms / pathology*
  • Oncogene Proteins, Fusion / genetics
  • RNA-Binding Protein EWS
  • RNA-Binding Proteins / genetics
  • Translocation, Genetic


  • CREB1 protein, human
  • Calmodulin-Binding Proteins
  • Cyclic AMP Response Element-Binding Protein
  • EWSR1 protein, human
  • Oncogene Proteins, Fusion
  • RNA-Binding Protein EWS
  • RNA-Binding Proteins