Based on the literature, it seems that FDCS is frequently associated with complex cytogenetic abnormalities, although consistent or specific aberration has not been established to date in this entity. This suggests another paraneoplastic manifestation of FDCS.6,15. ; Sailey, CJ. Some cases of antecedent Castleman disease demonstrate areas of follicular dendritic cell proliferation, and FDCS is hypothesized to arise in these areas.9  Additionally, a shared feature of Castleman disease and FDCS is expression of epidermal growth factor receptor (EGFR).10. Types of dendritic cells Langerhans cells.

Electron micrograph of follicular dendritic cell sarcoma shows well-developed, long interdigitating cytoplasmic processes and desmosome-like junctions; no Birbeck granules are seen (original magnification ×20 000).

; Myers, JL. ; McClure, SA. The Langerhans cells are the dendritic cells of the skin. Follicular dendritic cell sarcoma and associated myasthenia gravis: true, true, related? Recently, a multicenter study performed by Go et al26  examined the BRAF pathway and its contribution to the pathogenesis of histiocytic and dendritic cell neoplasms, including FDCS. [2], WHO 2001 classification of dendritic cell neoplasms:[4]. Unfavorable prognostic factors include intra-abdominal location, large tumor size (greater than 6 cm), coagulative necrosis, mitotic count greater than 5 mitoses per 10 high-power fields, and significant cellular atypia, with intra-abdominal location the single most important unfavorable prognostic factor.2,16,17,19,27  Although for many patients the disease responds to treatment, the relapse rate can be as high as 80%.21  The mortality rate is approximately 20%, usually after a protracted course. Follicular dendritic cell sarcoma, with highly cellular areas showing varied fascicular, whorled, and storiform architecture, low- and high-power (inset) views (hematoxylin-eosin, original magnifications ×40 and ×200 [inset]).

Yin, WH. ; Ord, RA. Annie Wu, Sheeja Pullarkat; Follicular Dendritic Cell Sarcoma. By continuing you agree to the use of cookies. Most cases of FDCS develop in the lymph nodes, but about 30% develop in extranodal sites. It is variably positive for epithelial membrane antigen, S100, and CD68, which can present diagnostic pitfalls.

"Follicular dendritic cell tumor: review of the entity.". Follicular dendritic cell sarcoma, abbreviated FDCS, is a very rare malignant tumour. Follicular dendritic cell sarcomas are characteristically well circumscribed or even encapsulated, bosselated masses that are solid with pink or white to tan-grey cut surfaces. Subject: Follicular Dendritic Cell Sarcoma, (Optional message may have a maximum of 1000 characters.).

They form dense three-dimensional meshwork patterns within benign follicles, which maintain the follicular architecture. In 1998 the largest study on the disease was a retrospective review with fifty-one patients. (Jun 2007). ; Olson, PR. Most cases are relatively bland and uniform but cytologic atypia may be seen in some cases with pleomorphic cells (Figure 3). Follicular dendritic cell sarcoma/tumour. 2 Follicular dendritic cells are normally present in both nodal and extranodal lymphoid follicles and serve as antigen-presenting cells to B-cell … ; Flati, VR. Through direct Sanger sequencing, as well as peptide nucleic acid clamp quantitative polymerase chain reaction, the authors found that the BRAFV600E mutation was positive in 18.5% (5 of 27) of FDCS cases, and in both conventional as well as inflammatory pseudotumor-like types. ; McAdams, HP. Oval or spindle-shaped cellular & nuclear morphology. "Follicular dendritic cell sarcoma of the mediastinum.".

Follicular dendritic cell sarcoma (FDCS) was first described in 1986 by Monda et al,1  in a series of 4 cases with initial presentation of unilateral cervical adenopathy, as a nonlymphomatous primary lymph node malignancy with features suggesting dendritic reticulum cell origin.

Perez-Ordoñez, B.; Rosai, J. This website is intended for pathologists and laboratory personnel but not for patients. "Folliculocentric B-cell-rich follicular dendritic cells sarcoma: a hitherto unreported morphological variant mimicking lymphoproliferative disorders.". (Apr 2009). ScienceDirect ® is a registered trademark of Elsevier B.V. ScienceDirect ® is a registered trademark of Elsevier B.V. Follicular dendritic cells: origin, function, and different disease-associated patterns, Nodular lymphocyte predominant Hodgkin lymphoma. Staining for CD1a, lysozyme, myeloperoxidase, CD34, CD3, CD79a, CD30, HMB-45, desmin, and high-molecular-weight cytokeratins is negative. Human herpesvirus 8 is not associated with FDCS.7  The inflammatory pseudotumor-like variant of FDCS is an uncommon tumor that is consistently associated with the Epstein-Barr virus. Diagnostic ultrastructural features of FDCS include long, slender, interdigitating cytoplasmic processes, sometimes producing a labyrinth-like pattern and desmosome-like junctions (Figure 5).18  The cytoplasm often contains few organelles. Also, unlike FDCS, inflammatory myofibroblastic tumor is positive for smooth muscle actin, may express desmin, and 50% are positive for ALK, with ALK gene arrangements.27. A helpful morphologic distinguishing feature is the presence of Hassall corpuscles in thymomas.

"Dendritic cell neoplasms: an overview.". While most follicular dendritic cell sarcomas arise from lymph nodes, at least one-third occur in extranodal sites. Interdigitating dendritic cell sarcomas have a paracortical distribution in lymph nodes and lack the storiform and whorled patterns of FDCS.27  Unlike FDCS, interdigitating dendritic cell sarcomas are diffusely positive for S100, negative for dendritic cell markers (CD21, CD35), and lack desmosomes on electron microscopy. Alexiev, BA. Search for other works by this author on: A primary lymph node malignancy with features suggestive of dendritic reticulum cell differentiation: a report of 4 cases, WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues, World Health Organization Classification of Tumours, Morphology, function and pathology of follicular dendritic cells, Histiocytic and dendritic cell neoplasms, Introduction, Human follicular dendritic cells: function, origin and development, Dendritic cell sarcoma: a pooled analysis including 462 cases with presentation of our case series, HHV-8 is not associated with follicular dendritic cell tumours, Inflammatory pseudotumor-like follicular dendritic cell tumor: a distinctive low-grade malignant intra-abdominal neoplasm with consistent Epstein-Barr virus association, Other histiocytic and dendritic cell neoplasms, Epidermal growth factor receptor expression in follicular dendritic cells: a shared feature of follicular dendritic cell sarcoma and Castleman's disease, Extranodal follicular dendritic cell sarcoma of the pharyngeal region: a potential diagnostic pitfall, with literature review, Follicular dendritic cell tumor: review of the entity, Paraneoplastic pemphigus associated with follicular dendritic cell sarcoma arising from Castleman's tumor, Paraneoplastic pemphigus associated with follicular dendritic cell sarcoma without Castleman's disease; treatment with rituximab. Follicular dendritic cell sarcomas generally demonstrate the immunophenotype of nonneoplastic follicular dendritic cells and are usually positive for dendritic cell markers CD21 (C3b complement receptor), CD23, and/or CD35 (C3d complement receptor), exhibiting cell membrane staining, although cytoplasmic staining can also be seen.9,18,21  Clusterin is a glycoprotein associated with many cellular functions, including apoptosis, and shows strong positivity in follicular dendritic cell tumors with weak to no expression in other dendritic cell tumors.22  Strong diffuse clusterin staining has high specificity and sensitivity for FDCS.23  Podoplanin (D2-40) is another marker shown to have high sensitivity for FDCS with strong membranous staining.24  γ-Synuclein is a relatively recent marker shown to strongly stain follicular dendritic cell meshwork and may also be considered helpful in identifying FDCS.25. Nodal and extranodal FDCSs have similar immunophenotypes. (May 1998). The location of the tumor is helpful in diagnosis. https://doi.org/10.1016/j.humpath.2012.10.005. While the role of radiation therapy and chemotherapy is not well defined, complete surgical resection appears to be the treatment of choice for both primary and recurrent lesions.6,16, For FDCSs with the BRAFV600E mutation, there is potential for vemurafenib, a BRAF enzyme inhibitor, as a targeted therapy as it was shown to exhibit dramatic effects in other dendritic and histiocytic neoplasms such as Erdheim-Chester disease and Langerhans cell histiocytosis.28  Another potential class of therapeutic agents is EGFR inhibitors, particularly in cases with moderate to strong expression of EGFR.10. Leipsic, JA. The authors note that BRAF mutations could potentially help differentiate inflammatory pseudotumor-like FDCS from inflammatory myofibroblastic tumor.26.

Notably, BRAFV600E was found in 40% (2 of 5) of inflammatory pseudotumor-like subtypes. Ki-67 labeling ranges from 1% to 25%.2,9. ; Shao, CK. Lorenzi, L.; Lonardi, S.; Petrilli, G.; Tanda, F.; Bella, M.; Laurino, L.; Rossi, G.; Facchetti, F. (Feb 2012). Scattered multinucleated cells, although in small numbers, can be seen and can resemble Warthin-Finkeldey giant cells.9  The cell borders are generally indistinct, imparting a syncytial appearance, and the cytoplasm is moderately abundant, lightly eosinophilic, and can have a fibrillary quality. In reactive conditions, FDC

Of these fifty-one patients, no conclusive pattern was found in regard to age, sex, race or presentation.

The inflammatory pseudotumor-like follicular dendritic cell tumor is a variant of FDCS with distinct histologic features. Interdigitating dendritic cell sarcoma/tumour.

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