NCI Definition: A neoplasm composed of spindle to ovoid cells which have morphologic and immunophenotypic characteristics of follicular dendritic cells. It affects lymph nodes and other sites including the tonsils, gastrointestinal tract, spleen, liver, soft tissues, skin, and oral cavity. It usually behaves as a low grade sarcoma. Treatment options include complete surgical removal of the tumor with or without adjuvant chemotherapy or radiotherapy. Recurrences have been reported in up to half of the cases. [1]

Follicular dendritic cell sarcomas most frequently harbor alterations in NFKBIA, CDKN2B, CDKN2A, BIRC3, and YAP1 [2].

Most Commonly Altered Genes in Follicular Dendritic Cell Sarcoma

CDKN2B Loss, CDKN2A Loss, YAP1 Loss, PIK3CA Mutation, and BIRC3 Loss are the most common alterations in follicular dendritic cell sarcoma [2].

Top Alterations in Follicular Dendritic Cell Sarcoma

Disease Details

Follicular Dendritic Cell Sarcoma/Tumor
Histiocytic and Dendritic Cell Neoplasm
OncoTree Name
Follicular Dendritic Cell Sarcoma
OncoTree Code


1. National Cancer Institute. NCI Thesaurus Version 18.11d. https://ncit.nci.nih.gov/ncitbrowser/ [2018-08-28]. [2018-09-21].

2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 8. This dataset does not represent the totality of the genetic landscape; see paper for more information.

3. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.