Interdigitating Dendritic Cell Sarcoma

Abstract

Interdigitating Dendritic Cell Sarcoma (IDCS) is a very uncommon neoplasm embedded in the group of histiocytic and dendritic cell neoplasms. IDCS is a diagnosis of exclusion, being mandatory the preclusion of follicular dendritic cell sarcoma, histiocytic sarcoma, melanoma, and metastases, among another neoplasm. The clinical course is aggressive. BRAF V600E has been reported in IDCS.

A Case Presentation of a Rare Pelvic Interdigitating Dendritic Cell Sarcoma

“…IDCS is a rare neoplasm with approximately 100 cases reported in the English literature [1,4]. There have been nodal cases described, however, no pelvic nodal cases like this case have been reported [1][2][3][4]. This case is a rare presentation of a pelvic nodal IDCS.…”

“…IDCS was first reported in 1981 by Feltkamp and coworkers where a patient was found to have a mass in the mediastinum. There is a slight male predominance (1.38:1), and the median age of diagnosis is 56.5 years [1][2][3][4]. The clinical presentation will vary depending on the site of pathology.…”

“…The clinical presentation will vary depending on the site of pathology. The most common pathology is an isolated lymph node [1][2][3][4]. Other sites of involvement include extranodal lesions including the liver, gastrointestinal tract, lung, spleen, skin, nasopharynx, kidney, bladder, ovary, and testis [1].…”

“…The etiology of IDCS remains unclear. There are a few postulated theories with hematopoietic stem cells, migration of Langerhans cells, and infectious origins such as Epstein-Barr virus or human herpesvirus [3][4][5].…”

“…This is done by presenting antigens for B-cells and stimulating B-cell proliferation and differentiation. Interdigitating dendritic cells are found in the T-cell areas of secondary lymphoid tissues [1][2][3][4].…”