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Fibroblastic Reticular Cell Tumor

Written2019-04Luis Miguel Juárez Salcedo, Diego Conde Royo, Samir Dalia
Gregorio Marañón University General Hospital, Madrid, Spain (LMJS); Principe de Asturias University Hospital, Madrid, Spain (DCR); Oncology and Hematology, Mercy Clinic Joplin, Joplin, MO, USA (SD).

Abstract Review on Fibroblastic reticular cell tumor, with data on clinics, and the genes involved.

Keywords Fibroblastic Reticular Cell Tumor; Cytokeratin-positive interstitial reticulum cell tumor; Fibroblastic dendritic cell tumor; dendritic cell sarcoma

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ICD-Topo C96.7
ICD-Morpho 9759/3 Intermediate dendritic cell tumour
Atlas_Id 1836
Other namesCytokeratin-positive interstitial reticulum cell tumor
Fibroblastic dendritic cell tumor

Clinics and Pathology

Disease Fibroblastic reticular cell tumor (FRCT) is the rarest subgroup of dendritic cell sarcoma (DCS) with few cases published in the literature (Kwon JE et al.,2009; Dalia S et al., 2013). Fibroblastic reticular cells are stromal support cells located in the parafollicular are and deep cortex of lymph nodes and in the extrafollicular areas of the spleen and tonsils. (Andriko JW et al., 1998). In general, FRCT are presents in the lymph nodes, but also in the spleen, lung, liver, and soft tissue (Martel M et al., 2003). Most of the patients presented with a localized disease, and stage have an important effect in the overall survivor of patients.
Phenotype / cell stem origin Dentritic/reticular cells are structural and functional accessory cells components of immune system. They have been subdivided on the bases of their location and phenotype into 3 major subsets: follicular dendritic cells (FDCs), interdigitating dendritic cells (IDCs), and fibroblastic reticular cells (FRCs). These cells are stromal cells located in the parafollicular area and deep cortex of lymph nodes and also in the extrafollicular areas of the spleen and tonsils (Doglioni C, et al., 1990). These cells are crucial to the interaction between IDCs and T cells in the primary immune response (Andriko JW et al., 1998). This pathology was also known as cytokeratin-positive interstitial reticulum cell tumor.
Epidemiology Median age for FRCT was 61 years old (range 13-80 years) with slight male predilection.
Clinics Information about FRCT generally comes from series of isolated patients mainly reported in the manuscripts by Saygin C et al and Dalia S et al. Both papers reported that isolated nodal disease is more common (84.2%) when compared to other pathological entities. Cervical and mediastinal lymph nodes were the ones most commonly involved during disease course. Extranodal sites included the liver, lung, kidney, adrenal, bone, and soft tissue. Higher-stage disease has a significantly shorter survival rate. Patients with local disease had a 2-year survival rate of 85.7%; median survival was not reached. Patients with distant disease died in 2 years and had a median survival rate of one year (Saygin C et al.,2013). Most patients present with a newly diagnosed asymptomatic mass that was surgically removed (Saygin C et al., 2013, Martel M et at., 2003).
Diagnosis The value of CT scans, bone marrow biopsy, and other staging work in single nodal disease is unknown, but it becomes important in patients with multiple enlarged lymph nodes. Excisional biopsy of the lymph node is the gold-standard diagnostic method for FRCT.

Pathology Morphologically this disorder is described as spindle to ovoid cells with whorls in the paracortical areas associated with abundant reticulin staining fibers. Immunohistochemistry is positive for vimentin, desmin factor XIIIa, and smooth muscle actin. CD45RB, CD21, CD35, S100, CD65, and CD1a are negative (Saygin C et al., 2013). Ultraestructural evaluation reveals peripherally located fusiform densities, long cytoplasmic extension, and desmosomal-like intercellular attachments (Dalia S et al., 2013). A subset of fibroblastic reticulum cells that express cytokeratine 8 and 18 and various other epithelial markers has been identified. (Golud VE et al., 1995).
Treatment Surgery is the treatment of choice for patients with localized disease. Limited data exist about the role of the radiotherapy. In localized disease, chemotherapy is not indicated. Patient should be included in clinical trials or referred to specialized care centers.


Reticulum cell neoplasms of lymph nodes: a clinicopathologic study of 11 cases with recognition of a new subtype derived from fibroblastic reticular cells.
Andriko JW, Kaldjian EP, Tsokos M, Abbondanzo SL, Jaffe ES.
Am J Surg Pathol. 1998 Sep;22(9):1048-58.
PMID 9737236
Dendritic cell and histiocytic neoplasms: biology, diagnosis, and treatment.
Dalia S, Shao H, Sagatys E, Cualing H, Sokol L.
Cancer Control. 2014 Oct;21(4):290-300. Review.
PMID 25310210
Cytokeratin-immunoreactive cells of human lymph nodes and spleen in normal and pathological conditions. An immunocytochemical study.
Doglioni C, Dell'Orto P, Zanetti G, Iuzzolino P, Coggi G, Viale G.
Virchows Arch A Pathol Anat Histopathol. 1990;416(6):479-90.
PMID 1692434
Increased numbers of cytokeratin-positive interstitial reticulum cells (CIRC) in reactive, inflammatory and neoplastic lymphadenopathies: hyperplasia or induced expression?
Gould VE, Bloom KJ, Franke WW, Warren WH, Moll R.
Virchows Arch. 1995;425(6):617-29.
PMID 7535166
Cytokeratin-positive interstitial reticulum cell sarcoma: a case report with cytological, immunohistochemical, and ultrastructural findings.
Kwon JE, Yang WI, Kim HK, Kwon KW, Kwon TJ, Choi
Cytopathology. 2009 Jun;20(3):202-5
PMID 19207304
Fibroblastic reticular cell tumor of the spleen: report of a case and review of the entity.
Martel M, Sarli D, Colecchia M, Coppa J, Romito R, Schiavo M, Mazzaferro V, Rosai J.
Hum Pathol. 2003 Sep;34(9):954-7.
PMID 14562295
Dendritic cell sarcoma: a pooled analysis including 462 cases with presentation of our case series.
Saygin C, Uzunaslan D, Ozguroglu M, Senocak M, Tuzuner N.
Crit Rev Oncol Hematol. 2013 Nov;88(2):253-71.
PMID 23755890


This paper should be referenced as such :
Juárez-Salcedo LM, Conde Royo D, Dalia S
Fibroblastic Reticular Cell Tumor;
Atlas Genet Cytogenet Oncol Haematol. in press
On line version :

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