09-06-2021-1005 - Spindle cell sarcoma Follicular dendritic cell sarcoma (FDCS) cancotics

Follicular dendritic cell sarcoma (FDCS) is a rare low-grade sarcoma that most commonly presents as a slowly growing, painless mass with the histologic appearance of spindle-shaped cells in a whorled pattern. Several hundred cases have been reported since it was first described in 1986 [1]. Occasional cases have an inflammatory presentation associated with fever and weight loss, and high-grade histologic features with cellular atypia.

FDCS is now recognized to be a low-grade sarcoma of mesenchymal dendritic cell origin [2]. Earlier classification schemes had erroneously categorized it as a histiocytic or dendritic cell neoplasm of myeloid origin.

 The term histiocytic neoplasm has historically been used to refer to tumors associated with monocyte/macrophage and dendritic cell phenotypes, including entities that are malignant (eg, FDCS, histiocytic sarcomas, interdigitating dendritic cell sarcoma, Langerhans cell sarcoma, indeterminate cell sarcomas) and benign (eg, Langerhans cell histiocytosis, Erdheim-Chester disease). FDCS is distinct because unlike the others, it is not of hematopoietic origin and its management more closely resembles that of other soft tissue sarcomas.

https://www.uptodate.com/contents/follicular-dendritic-cell-sarcoma

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