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Follicular dendritic cell sarcoma

Authors
Eric Jacobsen, MD
Mrinal M Gounder, MD
Section Editor
Robert Maki, MD, PhD
Deputy Editors
Diane MF Savarese, MD
Alan G Rosmarin, MD

INTRODUCTION

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.

The epidemiology, clinical manifestations, pathologic features, diagnosis, and management of FDCS will be described here. The diagnosis and management of other low-grade sarcomas, histiocytic sarcomas, and nonmalignant histiocytic neoplasms are addressed separately. (See "Clinical presentation, histopathology, diagnostic evaluation, and staging of soft tissue sarcoma" and "Histiocytic sarcoma" and "Clinical manifestations, pathologic features, and diagnosis of Langerhans cell histiocytosis" and "Erdheim-Chester disease".)

EPIDEMIOLOGY

Follicular dendritic cell sarcoma (FDCS) is a rare disorder, but the precise incidence is unknown. Only a few hundred cases have been reported in the medical literature. FDCS constitutes <0.4 percent of soft tissue sarcomas [3]. In a pooled analysis that included 462 cases of FDCS, the median age was in the fifth decade, although cases have been reported in children [4]. Men and women are affected equally, but the inflammatory variant is more common in women [5]. (See 'Pathologic features' below.)

                       
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Literature review current through: Oct 2017. | This topic last updated: Aug 17, 2017.
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