Follicular Dendritic Cell Sarcoma
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. 
Follicular dendritic cell sarcomas most frequently harbor alterations in NFKBIA, CDKN2B, CDKN2A, BIRC3, and YAP1 .
CDKN2B Loss, CDKN2A Loss, YAP1 Loss, PIK3CA Mutation, and BIRC3 Loss are the most common alterations in follicular dendritic cell sarcoma .
There is 1 clinical trial for follicular dendritic cell sarcoma, of which 1 is open and 0 are completed or closed. Of the trial that contains follicular dendritic cell sarcoma as an inclusion criterion, 1 is phase 2 (1 open).
Pembrolizumab is the most common intervention in follicular dendritic cell sarcoma clinical trials.
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.