Extranodal Soft Tissue Rosai Dorfman Disease with Floret Cells Involving the Inguinal Region, Masquerading as Inflammatory Liposarcoma. Report of a Case Featuring Increased IgG4 Positive Plasma Cells
Rosai Dorfman disease (RDD) is a rare histiocytic proliferative disorder of unknown pathogenesis that had been originally described in lymph nodes, but was found to occur in different visceral organs and anatomic sites as well. We report herein a patient who presented with a mass in the inguinal region which was initially diagnosed on core biopsy as inflammatory pseudotumor, then as inflammatory liposarcoma on excision of the mass. The patient was referred to our hospital for further management. On review of the histopathological material, we made the diagnosis of extranodal soft tissue Rosai Dorfman disease (ENST-RDD) by the identification of the characteristic S100-positive histiocytes demonstrating emperipolesis. We noted the presence of floret cells, which probably prompted the earlier diagnosis of inflammatory liposarcoma. The presence of these cells had not been documented in earlier reports on ENST-RDD. In addition, there was heavy IgG4 positive plasma cell infiltration associated with storiform fibrosis in the same lesion, probably pointing to a link between ENST-RDD and IgG4-related disorders.
References
[1]
Rosai, J. and Dorfman, R.F. (1969) Sinus Histiocytosis with Massive Lymphadenopathy: A Newly Recognized Benign Clinicopathological Entity. Archives of Pathology and Laboratory Medicine, 87, 63-70.
[2]
Rosai, J. and Dorfman, R.F. (1972) Sinus Histiocytosis with Massive Lymphadenopathy: A Pseudolymphomatous Benign Disorder.analysis of 34 Cases. Cancer, 30, 1174-1188. https://doi.org/10.1002/1097-0142(197211)30:5<1174::aid-cncr2820300507>3.0.co;2-s
[3]
Foucar, E., Rosai, J. and Dorfman, R.F. (1990) Sinus Histiocytosis with Massive Lymphadenopathy (Rosai-Dorfman Disease): Review of the Entity. Seminars in Diagnostic Pathology, 7, 19-73.
[4]
Foucar, E., Rosai, J., Dorfman, R.F. and Eyman, J.M. (1984) Immunologic Abnormalities and Their Significance in Sinus Histiocytosis with Massive Lymphadenopathy. American Journal of Clinical Pathology, 82, 515-525. https://doi.org/10.1093/ajcp/82.5.515
[5]
Bonetti, F., Chilosi, M., Menestrina, F., Scarpa, A., Pelicci, P., Amorosi, E., et al. (1987) Immunohistological Analysis of Rosai-Dorfman Histiocytosis. Virchows Archiv A Pathological Anatomy and Histopathology, 411, 129-135. https://doi.org/10.1007/bf00712736
[6]
Montgomery, E.A., Meis, J.M. and Frizzera, G. (1992) Rosai-Dorfman Disease of Soft Tissue. The American Journal of Surgical Pathology, 16, 122-129. https://doi.org/10.1097/00000478-199202000-00004
[7]
Garcia, R.A. and DiCarlo, E.F. (2021) Rosai-Dorfman Disease of Bone and Soft Tissue. Archives of Pathology & Laboratory Medicine, 146, 40-46. https://doi.org/10.5858/arpa.2021-0116-ra
[8]
Abdulkader, M. (2014) Metachronous Multifocal Osseous Rosai-Dorfman Disease in a Pregnant Woman: Report of an Unusual Case and Brief Review of Pertinent Literature. Gynecology & Obstetrics, 4, Article ID: 1000198. https://doi.org/10.4172/2161-0932.1000198
[9]
Zhang, X., Hyjek, E. and Vardiman, J. (2013) A Subset of Rosai-Dorfman Disease Exhibits Features of Igg4-Related Disease. American Journal of Clinical Pathology, 139, 622-632. https://doi.org/10.1309/ajcparc3yq0klioa
[10]
Tataroglu, C., Ozgezmez, F.T. and Unsal, A. (2017) Soft Tissue Rosai-Dorfman Disease with Unusual Histopathologic Features: A Case Report. Journal of Clinical & Experimental Pathology, 7, Article ID: 1000330. https://doi.org/10.4172/2161-0681.1000330
[11]
Veinot, J.P., Eidus, L. and Jabi, M. (1998) Soft Tissue Rosai Dorfman Disease Mimicking Inflammatory Pseudotumor: A Diagnostic Pitfall. Pathology, 30, 14-16. https://doi.org/10.1080/00313029800169605
[12]
Al-Daraji, W., Anandan, A., Klassen-Fischer, M., Auerbach, A., Marwaha, J.S. and Fanburg-Smith, J.C. (2010) Soft Tissue Rosai-Dorfman Disease: 29 New Lesions in 18 Patients, with Detection of Polyomavirus Antigen in 3 Abdominal Cases. Annals of Diagnostic Pathology, 14, 309-316. https://doi.org/10.1016/j.anndiagpath.2010.05.006
[13]
Ghadially, F.N. (1997) Emperipolesis. In: Ghadially, F.N., Ed., Ultrastructural Pathology of the Cell and Matrix, Butterworth-Heinemann, 1224-1229.
[14]
Komaragiri, M., Sparber, L.S., Santos-Zabala, M.L., Dardik, M. and Chamberlain, R.S. (2013) Extranodal Rosai-Dorfman Disease: A Rare Soft Tissue Neoplasm Masquerading as a Sarcoma. World Journal of Surgical Oncology, 11, Article No. 63. https://doi.org/10.1186/1477-7819-11-63
[15]
Foucar, E., Rosai, J. and Dorfman, R.F. (1984) Sinus Histiocytosis with Massive Lymphadenopathy. An Analysis of 14 Deaths Occurring in a Patient Registry. Cancer, 54, 1834-1840. https://doi.org/10.1002/1097-0142(19841101)54:9<1834::aid-cncr2820540911>3.0.co;2-f
[16]
Guedes, A., Barreto, B., Barreto, L.G.S., Araújo, I.B.O., Athanazio, D.A. and Athanazio, P.R.F. (2008) Recurring Soft Tissue Rosai-Dorfman Disease. Jornal Brasileiro de Patologia e Medicina Laboratorial, 44, 283-286. https://doi.org/10.1590/s1676-24442008000400008
[17]
Hindermann, W. and Katenkamp, D. (2004) Extranodale Rosai-Dorfman-Erkrankung (Sinushistiozytose Mit Massiver Lymphadenopathie). Der Pathologe, 25, 222-228. https://doi.org/10.1007/s00292-003-0642-9
[18]
Betini, N., Munger, A.M., Rottmann, D., Haims, A., Costa, J. and Lindskog, D.M. (2022) Rare Presentation of Rosai-Dorfman Disease in Soft Tissue: Diagnostic Findings and Surgical Treatment. Case Reports in Surgery, 2022, Article ID: 8440836. https://doi.org/10.1155/2022/8440836
[19]
Levine, E.A. and Landry, M.M. (1994) Rosai Dorfman Disease of Soft Tissue. Surgery, 115, 650-652.
[20]
Londhe, S.B., Joshi, K.C. and Ambrish, P.I. (2021) Extranodal Rosai Dorfman Disease Masquerading as Metastatic Soft Tissue Sarcoma—A Case Report. Journal of Clinical Orthopaedics and Trauma, 20, Article ID: 101500. https://doi.org/10.1016/j.jcot.2021.101540
[21]
Mantilla, J.G., Goldberg-Stein, S. and Wang, Y. (2016) Extranodal Rosai-Dorfman Disease. Clinicopathologic Series of 10 Patients with Radiologic Correlation and Re-view of the Literature. American Journal of Clinical Pathology, 145, 211-221. https://doi.org/10.1093/ajcp/aqv029
[22]
Young, P.M., Kransdorf, M.J., Temple, H.T., Mousavi, F. and Robinson, P.G. (2005) Rosai-Dorfman Disease Presenting as Multiple Soft Tissue Masses. Skeletal Radiology, 34, 665-669. https://doi.org/10.1007/s00256-005-0906-y
[23]
Shmookler, B.M. and Enzinger, F.M. (1981) Pleomorphic Lipoma: A Benign Tumor Simulating Liposarcoma. A Clinicopathologic Analysis of 48 Cases. Cancer, 47, 126-133. https://doi.org/10.1002/1097-0142(19810101)47:1<126::aid-cncr2820470121>3.0.co;2-k
[24]
Azzopardi, J.G., Iocco, J. and Salm, R. (1983) Pleomorphic Lipoma: A Tumour Simulating Liposarcoma. Histopathology, 7, 511-523. https://doi.org/10.1111/j.1365-2559.1983.tb02264.x
[25]
Campbell, A.P. (1992) Multinucleated Stromal Giant Cells in Adolescent Gynaecomastia. Journal of Clinical Pathology, 45, 443-444. https://doi.org/10.1136/jcp.45.5.443
[26]
Magro, G., Amico, P., Vecchio, G.M., Caltabiano, R., Castaing, M., Kacerovska, D., et al. (2009) Multinucleated Floret-Like Giant Cells in Sporadic and NF1-Associated Neurofibromas: A Clinicopathologic Study of 94 Cases. Virchows Archiv, 456, 71-76. https://doi.org/10.1007/s00428-009-0859-y
[27]
Rudolph, P., Schubert, C., Harms, D. and Parwaresch, R. (1998) Giant Cell Collagenoma: A Benign Dermal Tumor with Distinctive Multinucleate Cells. The American Journal of Surgical Pathology, 22, 557-563. https://doi.org/10.1097/00000478-199805000-00006
[28]
FLETCHER, C.D.M. (1988) Giant Cell Fibroblastoma of Soft Tissue: A Clinicopathological and Immunohistochemical Study. Histopathology, 13, 499-508. https://doi.org/10.1111/j.1365-2559.1988.tb02074.x
[29]
Seregard, S., Calonje, E. and Chan, J.K.C. (1995) Giant Cell Angiofibroma. A Distinctive Orbital Tumor in Adults. The American Journal of Surgical Pathology, 19, 1286-1293. https://doi.org/10.1097/00000478-199511000-00009
[30]
Thomas, K.D., Delahoussaye, P., Schwartz, M.R., Ayala, A.G. and Ro, J.Y. (2021) Extranodal Rosai-Dorfman Disease Involving Soft Tissue Associated with Increased Igg4 Plasma Cells. Human Pathology: Case Reports, 24, Article ID: 200488. https://doi.org/10.1016/j.ehpc.2021.200488
[31]
Deshpande, V., Zen, Y., Chan, J.K., Yi, E.E., Sato, Y., Yoshino, T., et al. (2012) Consensus Statement on the Pathology of IgG4-Related Disease. Modern Pathology, 25, 1181-1192. https://doi.org/10.1038/modpathol.2012.72
[32]
Menon, M.P., Evbuomwan, M.O., Rosai, J., Jaffe, E.S. and Pittaluga, S. (2013) A Subset of Rosai-Dorfman Disease Cases Show Increased IgG4-Positive Plasma Cells: Another Red Herring or a True Association with IgG4-Related Disease? Histopathology, 64, 455-459. https://doi.org/10.1111/his.12274
[33]
Tauziede-Espariat, A., Polivka, M., Chabriat, H., Bouazza, S., Sene, D. and Adle-Biassette, H. (2015) A Case Report of Meningeal Rosai-Dorfman Disease Associated with IgG4-Related Disease. Clinical Neuropathology, 34, 343-349. https://doi.org/10.5414/np300871
[34]
Mudhar, H.S. and Duke, R. (2013) A Case of Orbital Rosai-Dorfman Disease with IgG4 Positive Plasma Cells. Orbit, 32, 315-317. https://doi.org/10.3109/01676830.2013.799705
[35]
Wimmer, D.B., Ro, J.Y., Lewis, A., Schwartz, M.R., Caplan, R., Schwarz, P., et al. (2013) Extranodal Rosai-Dorfman Disease Associated with Increased Numbers of Immunoglobulin G4 Plasma Cells Involving the Colon: Case Report with Literature Review. Archives of Pathology & Laboratory Medicine, 137, 999-1004. https://doi.org/10.5858/arpa.2011-0547-cr
[36]
Liu, L., Perry, A.M., Cao, W., Smith, L.M., Hsi, E.D., Liu, X., et al. (2013) Relationship between Rosai-Dorfman Disease and IgG4-Related Disease. American Journal of Clinical Pathology, 140, 395-402. https://doi.org/10.1309/ajcpfh0sj6yilxju
[37]
Wang, L., Li, W., Zhang, S., Peng, L., Shen, M., Song, S., et al. (2020) Rosai-Dorfman Disease Mimicking IgG4-Related Diseases: A Single-Center Experience in China. Orphanet Journal of Rare Diseases, 15, Article No. 285. https://doi.org/10.1186/s13023-020-01567-6
