Abstract
Fibroblastic reticulum cell tumor (FRCT) is a rare dendritic neoplasm arising from fibroblastic reticulum cells (FBRCs) and exhibiting peculiar cytokeratin expression. FRCTs usually involve the lymph nodes, although they can also be encountered in the spleen and soft tissues. FRCTs are composed of mildly atypical spindle or ovoid cells, arranged in loose whorls, which express almost invariably low-weight cytokeratins, smooth muscle actin, and CD68. An admixed lymphoplasmacytic infiltrate is also frequently present in solid organ sites. The clinical picture may vary from very indolent to aggressive disease exhibiting features of malignancy, such as cytological pleomorphism, necrosis, or high mitotic rate and metastatic potential. FRCT is a challenging diagnosis, due to its rarity and deceptive cytokeratin expression. Hereafter, we revise the most recent literature regarding such condition and report the case of an extremely indolent splenic FRCT, with no features of malignancy.
References
Alaggio R, Amador C, Anagnostopoulos I et al (2022) The 5th edition of the World Health Organization Classification of haematolymphoid tumours: lymphoid neoplasms. Leukemia https://doi.org/10.1038/s41375-022-01620-2
Swerdlow SH, Campo E, Harris NL et al (2017) WHO classification of tumours of haematopoietic and lymphoid tissues. IARC Press, Lyon
Franke WW, Moll R (1987) Cytoskeletal components of lymphoid organs. Differentiation 36:145–163. https://doi.org/10.1111/j.1432-0436.1987.tb00189.x
Lucioni M, Boveri E, Rosso R et al (2003) Lymph node reticulum cell neoplasm with progression into cytokeratin-positive interstitial reticulum cell (CIRC) sarcoma: a case study. Histopathology 43:583–591. https://doi.org/10.1111/j.1365-2559.2003.01725.x
Gould VE, Bloom KJ, Franke WW et al (1995) Increased numbers of cytokeratin-positive interstitial reticulum cells (CIRC) in reactive, inflammatory and neoplastic lymphadenopathies: hyperplasia or induced expression? Virchows Arch 425:617–629. https://doi.org/10.1007/BF00199352
Banchereau J, Briere F, Caux C et al (2000) Immunobiology of dendritic cells. Annu Rev Immunol 18:767–811. https://doi.org/10.1146/annurev.immunol.18.1.767
Mellman I (2013) Dendritic cells: master regulators of the immune response. Cancer Immunol Res 1:145–149. https://doi.org/10.1158/2326-6066.CIR-13-0102
Schuerfeld K, Lazzi S, De Santi MM et al (2003) Cytokeratin-positive interstitial cell neoplasm: a case report and classification issues. Histopathology 43:491–494. https://doi.org/10.1046/j.1365-2559.2003.01738.x
Kaji S, Hiruta N, Sasai D et al (2020) Cytokeratin-positive interstitial reticulum cell (CIRC) tumor in the lymph node: a case report of the transformation from the epithelioid cell type to the spindle cell type. Diagn Pathol 15:121. https://doi.org/10.1186/s13000-020-01032-9
Eberhard N, Cordier D (2020) An unusual bump on the head: intracranial and extracranial cytokeratin-positive interstitial reticular cell tumor. World Neurosurg 139:60–62. https://doi.org/10.1016/j.wneu.2020.03.173
Ying-Chun D, Bo WU, Zhen S et al (2008) Cytokeratin-positive interstitial reticulum cell tumors of lymph nodes: a case report and review of literature. Chin Med J (Engl) 121:658–663. https://doi.org/10.5555/cmj.0366-6999.121.07.p658.01
Li H, Shen P, Liang Y, Zhang F (2016) Fibroblastic reticular cell tumor of the breast: a case report and review of the literature. Exp Ther Med 11:561–564. https://doi.org/10.3892/etm.2015.2922
Bösmüller H, Klenske J, Bonzheim I et al (2016) Cytokeratin-positive interstitial reticulum cell tumor: recognition of a potential “in situ” pattern. Hum Pathol 49:15–21. https://doi.org/10.1016/j.humpath.2015.10.001
Doglioni C, Dell’Orto P, Zanetti G et al (1990) Cytokeratin-immunoreactive cells of human lymph nodes and spleen in normal and pathological conditions. Virchows Archiv A 416:479–490. https://doi.org/10.1007/BF01600298
Roy S, Das I, Saha K et al (2015) A rare case of cytokeratin-positive interstitial reticulum cell sarcoma and review of the entity. Indian J Surg Oncol 6:271–275. https://doi.org/10.1007/s13193-015-0392-1
Chan AC, Serrano-Olmo J, Erlandson RA, Rosai J (2000) Cytokeratin-positive malignant tumors with reticulum cell morphology: a subtype of fibroblastic reticulum cell neoplasm? Am J Surg Pathol 24(1):107–116. https://doi.org/10.1097/00000478-200001000-00013
Lee HJ, Jeon HJ, Park SG, Park CY (2015) Castleman’s disease of the spleen. World J Gastroenterol 21:1675–1679. https://doi.org/10.3748/wjg.v21.i5.1675
Lopez LV, Marker DF, Bailey N et al (2019) Soft tissue fibroblastic reticular cell tumor with whole-exome sequencing findings: an unexpected presentation of lynch syndrome. AJSP Rev Rep 24:288–291. https://doi.org/10.1097/pcr.0000000000000337
Saigo C, Kito Y, Hasegawa M et al (2021) Incidental cytokeratin-positive interstitial reticulum cell tumor of the lymph node accompanied by breast cancer: status of YAP/TAZ expression in tumor cells. Human Pathology: Case Reports 25:200526. https://doi.org/10.1016/j.ehpc.2021.200526
Sundersingh S, Majhi U, Krishnamurthy A, Velusami SD (2013) Cytokeratin-positive interstitial reticulum cell sarcoma: extranodal presentations mimicking carcinoma. Indian J Pathol Microbiol 56:172–5
Cheuk W, Hill RW, Bacchi C, Dias MA, Chan JK (2000) Hypocellular anaplastic large cell lymphoma mimicking inflammatory lesions of lymph nodes. Am J Surg Pathol 24(11):1537–1543. https://doi.org/10.1097/00000478-200011000-00010
McMahon G, Rady K, Prince HM (2015) Inflammatory pseudotumor of the spleen. Hematol Rep 7:35–37. https://doi.org/10.4081/hr.2015.5905
Kutok JL, Pinkus GS, Dorfman DM, Fletcher CDM (2001) Inflammatory pseudotumor of lymph node and spleen: an entity biologically distinct from inflammatory myofibroblastic tumor. Hum Pathol 32:1382–1387. https://doi.org/10.1053/hupa.2001.29679
Karim Z, Saravana R, Shenjere P, Reid F (2013) Fibroblastic reticulum cell tumor of spleen: a case report. Int J Surg Pathol 22:447–450. https://doi.org/10.1177/1066896913509009
Shinomiya A, Ye J, Miyake K et al (2021) Necropsy-confirmed case of cytokeratin-positive interstitial reticulum cell tumor in the skull bone. Pathol Int 71:856–859. https://doi.org/10.1111/pin.13167
Behera G, Purkait S, Patra S et al (2020) Extra-nodal cytokeratin-positive interstitial reticulum cell sarcoma presenting as a colonic polyp: report of a rare case with review of literature. J Gastrointest Cancer 51:1034–1038. https://doi.org/10.1007/s12029-020-00380-5
Kwon JE, Yang W-I, Kim HK et al (2009) Cytokeratin-positive interstitial reticulum cell sarcoma: a case report with cytological, immunohistochemical, and ultrastructural findings. Cytopathology 20:202–205. https://doi.org/10.1111/j.1365-2303.2008.00634.x
Goto N, Tsurumi H, Takami T et al (2015) Cytokeratin-positive fibroblastic reticular cell tumor with follicular dendritic cell features: a case report and review of the literature. Am J Surg Pathol 39
Martel M, Sarli D, Colecchia M et al (2003) Fibroblastic reticular cell tumor of the spleen: report of a case and review of the entity. Hum Pathol 34:954–957. https://doi.org/10.1016/S0046-8177(03)00399-X
Suárez D, Izquierdo FM, Méndez JR, Escobar J, Cabeza A, Junco P (2011) Tumor of fibroblastic reticular cells of lymph node coincidental with an undifferentiated endometrial stromal sarcoma. Report of a case with distinctive immunophenotype and Kikuchi-like necro-inflammatory response. APMIS 119(3):216–220. https://doi.org/10.1111/j.1600-0463.2010.02712.x
Acknowledgements
We would like to thank Ms. Elisa De Nanni for her kind contribution to the language editing of the manuscript.
Author information
Authors and Affiliations
Contributions
Conceptualization, G.A.C., C.P., G.L..; data curation, G.L., C.P..; formal analysis, C.P., G.L., G.A.C.; investigation, G.A.C., C.P.; methodology, G.L., C.P.; supervision, G.A.C., U.G.; writing—original draft, C.P., and G.L.; writing—review and editing, C.P., G.L., G.A.C., U.G. All authors have read and agreed to the published version of the manuscript.
Corresponding author
Ethics declarations
Ethics approval and consent to participate
The study was conducted according to the guidelines of the Declaration of Helsinki and informed consent was obtained from the patient.
Conflict of interest
U.G. received fees from Novartis Farma S.p.A. and Jazz Pharma as a consultant speaker and advisory board. All other authors declare no conflict of interest.
Additional information
Publisher's note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
About this article
Cite this article
Pescia, C., Lopez, G., Gianelli, U. et al. Fibroblastic/cytokeratin-positive interstitial reticular cell tumor of the spleen with indolent behavior: a case report with review of the literature. Virchows Arch 482, 1069–1077 (2023). https://doi.org/10.1007/s00428-022-03463-9
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00428-022-03463-9