Genomic analysis of follicular dendritic cell sarcoma by molecular inversion probe array reveals tumor suppressor-driven biology

Mod Pathol. 2017 Sep;30(9):1321-1334. doi: 10.1038/modpathol.2017.34. Epub 2017 Jun 16.


Follicular dendritic cell sarcoma is a rare malignant neoplasm of dendritic cell origin that is currently poorly characterized by genetic studies. To investigate whether recurrent genomic alterations may underlie the biology of follicular dendritic cell sarcoma and to identify potential contributory regions and genes, molecular inversion probe array analysis was performed on 14 independent formalin-fixed, paraffin-embedded samples. Abnormal genomic profiles were observed in 11 out of 14 (79%) cases. The majority showed extensive genomic complexity that was predominantly represented by hemizygous losses affecting multiple chromosomes. Alterations of chromosomal regions 1p (55%), 2p (55%), 3p (82%), 3q (45%), 6q (55%), 7q (73%), 8p (45%), 9p (64%), 11q (64%), 13q (91%), 14q (82%), 15q (64%), 17p (55%), 18q (64%), and 22q (55%) were recurrent across the 11 samples showing abnormal genomic profiles. Many recurrent genomic alterations in follicular dendritic cell sarcoma overlap deletions that are frequently observed across human cancers, suggesting selection, or an active role for these alterations in follicular dendritic cell sarcoma pathogenesis. In support of a tumor suppressor-driven biology, homozygous deletions involving tumor suppressor genes CDKN2A, RB1, BIRC3, and CYLD were also observed. Neither recurrent gains nor amplifications were observed. This genomic characterization provides new information regarding follicular dendritic cell sarcoma biology that may improve understanding about the underlying pathophysiology, provide better prognostication, and identify potential therapeutic markers for this rare disease.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Biomarkers, Tumor / genetics*
  • Chromosomes, Human*
  • Dendritic Cell Sarcoma, Follicular / genetics*
  • Dendritic Cell Sarcoma, Follicular / pathology
  • Female
  • Gene Deletion
  • Gene Expression Profiling*
  • Gene Expression Regulation, Neoplastic
  • Genes, Tumor Suppressor*
  • Genetic Predisposition to Disease
  • Genomics / methods*
  • Homozygote
  • Humans
  • Loss of Heterozygosity
  • Male
  • Middle Aged
  • Oligonucleotide Array Sequence Analysis*
  • Phenotype
  • Young Adult


  • Biomarkers, Tumor