Primary Tonsillar Epithelioid Follicular Dendritic Cell Sarcoma: Report of a Rare Case Mimicking Undifferentiated Carcinoma and a Brief Review of the Literature

Abstract

We present a 52 years old male with a left tonsillar follicular dendritic cell sarcoma with prominent epithelioid features that on light microscopical examination bore a striking resemblance to a lymphoepithelial or undifferentiated carcinoma. The tumor was immunohistochemically positive for CD21 and CD35 and negative for cytokeratins. Two distinct histopathological features (both present in our case) that may serve as clues to the correct diagnosis on light microscopical examination were formation of ectatic pseudovascular spaces lined by malignant cells and the presence of non-neoplastic multinucleated giant cells. Familiarity with the above-mentioned morphological clues, and awareness that this tumour may occur in anatomical sites outside the lymph node, are essential for accurate diagnosis.

This is a preview of subscription content, log in to check access.

Fig. 1
Fig. 2

References

  1. 1.

    Monda L, Warnke R, Rosai J. A primary lymph node malignancy with features suggestive of dendritic reticulum cell differentiation. A report of 4 cases. Am J Pathol. 1986;122(3):562–72.

    CAS  PubMed  PubMed Central  Google Scholar 

  2. 2.

    Saygin C, Uzunaslan D, Ozguroglu M, Senocak M, Tuzuner N. Dendritic cell sarcoma: a pooled analysis including 462 cases with presentation of our case series. Crit Rev Oncol/Hematol. 2013;88(2):253–71. https://doi.org/10.1016/j.critrevonc.2013.05.006.

    Article  Google Scholar 

  3. 3.

    Wu A, Pullarkat S. Follicular dendritic cell sarcoma. Arch Pathol Lab Med. 2016;140(2):186–90. https://doi.org/10.5858/arpa.2014-0374-RS.

    CAS  Article  PubMed  Google Scholar 

  4. 4.

    Biddle DA, Ro JY, Yoon GS, Yong YW, Ayala AG, Ordonez NG, et al. Extranodal follicular dendritic cell sarcoma of the head and neck region: three new cases, with a review of the literature. Mod Pathol. 2002;15(1):50–8. https://doi.org/10.1038/modpathol.3880489.

    Article  PubMed  Google Scholar 

  5. 5.

    Chan JK, Fletcher CD, Nayler SJ, Cooper K. Follicular dendritic cell sarcoma. Clinicopathologic analysis of 17 cases suggesting a malignant potential higher than currently recognized. Cancer. 1997;79(2):294–313.

    CAS  Article  Google Scholar 

  6. 6.

    Perez-Ordonez B, Erlandson RA, Rosai J. Follicular dendritic cell tumor: report of 13 additional cases of a distinctive entity. Am J Surg Pathol. 1996;20(8):944–55.

    CAS  Article  Google Scholar 

  7. 7.

    Chen T, Gopal MD. P. Follicular dendritic cell sarcoma. Arch Pathol Lab Med. 2017;141(4):596–9. https://doi.org/10.5858/arpa.2016-0126-RS.

    CAS  Article  PubMed  Google Scholar 

  8. 8.

    Lorenzi L, Doring C, Rausch T, Benes V, Lonardi S, Bugatti M, et al. Identification of novel follicular dendritic cell sarcoma markers, FDCSP and SRGN, by whole transcriptome sequencing. Oncotarget. 2017;8(10):16463–72. https://doi.org/10.18632/oncotarget.14864.

    Article  PubMed  PubMed Central  Google Scholar 

  9. 9.

    Singhi AD, Stelow EB, Mills SE, Westra WH. Lymphoepithelial-like carcinoma of the oropharynx: a morphologic variant of HPV-related head and neck carcinoma. Am J Surg Pathol. 2010;34(6):800–5. https://doi.org/10.1097/PAS.0b013e3181d9ba21.

    Article  PubMed  Google Scholar 

  10. 10.

    Wenig BM. Lymphoepithelial-like carcinomas of the head and neck. Semin Diagn Pathol. 2015;32(1):74–86. https://doi.org/10.1053/j.semdp.2014.12.004.

    Article  PubMed  Google Scholar 

  11. 11.

    Carpenter DH, El-Mofty SK, Lewis JS. Undifferentiated carcinoma of the oropharynx: a human papillomavirus-associated tumor with a favorable prognosis. Mod Pathol. 2011;24(10):1306–12. https://doi.org/10.1038/modpathol.2011.87.

    CAS  Article  PubMed  Google Scholar 

  12. 12.

    Pecorella I, Okello TR, Ciardi G, Ochola E, Ogwang MD. Follicular dendritic cell sarcoma of the head and neck. Literature review and report of the tonsil occurrence in a Ugandan patient. Pathologica. 2017;109(2):120–5.

    CAS  PubMed  Google Scholar 

  13. 13.

    Aydin E, Ozluoglu LN, Demirhan B, Arikan U. Follicular dendritic cell sarcoma of the tonsil: case report. European archives of oto-rhino-laryngology: official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology. Head Neck Surg. 2006;263(12):1155–7. https://doi.org/10.1007/s00405-006-0124-9.

    Article  Google Scholar 

  14. 14.

    Bothra R, Pai PS, Chaturvedi P, Majeed TA, Singh C, Gujral S, et al. Follicular dendritic cell tumour of tonsil—is it an under-diagnosed entity? Indian J Cancer. 2005;42(4):211–4.

    CAS  PubMed  Google Scholar 

  15. 15.

    Chan JK, Tsang WY, Ng CS, Tang SK, Yu HC, Lee AW. Follicular dendritic cell tumors of the oral cavity. Am J Surg Pathol. 1994;18(2):148–57.

    CAS  Article  Google Scholar 

  16. 16.

    Nayler SJ, Verhaart MJ, Cooper K. Follicular dendritic cell tumour of the tonsil. Histopathology. 1996;28(1):89–92.

    CAS  Article  Google Scholar 

  17. 17.

    Clement P, Saint-Blancard P, Minvielle F, Le Page P, Kossowski M. Follicular dendritic cell sarcoma of the tonsil: a case report. Am J Otolaryngol. 2006;27(3):207–10. https://doi.org/10.1016/j.amjoto.2005.09.003.

    Article  PubMed  Google Scholar 

  18. 18.

    Dominguez-Malagon H, Cano-Valdez AM, Mosqueda-Taylor A, Hes O. Follicular dendritic cell sarcoma of the pharyngeal region: histologic, cytologic, immunohistochemical, and ultrastructural study of three cases. Ann Diagn Pathol. 2004;8(6):325–32.

    Article  Google Scholar 

  19. 19.

    Duan G-j, Wu F, Zhu J, Guo D-y, Zhang R, Shen L-l, et al. Extranodal follicular dendritic cell sarcoma of the pharyngeal regiona potential diagnostic pitfall, with literature review. Am J Clin Pathol. 2010;133(1):49–58. https://doi.org/10.1309/AJCP7U8YISBUAVNW.

    Article  PubMed  Google Scholar 

  20. 20.

    Eun YG, Kim SW, Kwon KH. Follicular dendritic cell sarcoma of the tonsil. Yonsei Med J. 2010;51(4):602–4. https://doi.org/10.3349/ymj.2010.51.4.602.

    Article  PubMed  PubMed Central  Google Scholar 

  21. 21.

    Fan YS, Ng WK, Chan A, Chan GS, Tsang J, Chim CS, et al. Fine needle aspiration cytology in follicular dendritic cell sarcoma: a report of two cases. Acta Cytol. 2007;51(4):642–7. https://doi.org/10.1159/000325817.

    Article  PubMed  Google Scholar 

  22. 22.

    Grogg KL, Lae ME, Kurtin PJ, Macon WR. Clusterin expression distinguishes follicular dendritic cell tumors from other dendritic cell neoplasms: report of a novel follicular dendritic cell marker and clinicopathologic data on 12 additional follicular dendritic cell tumors and 6 additional interdigitating dendritic cell tumors. Am J Surg Pathol. 2004;28(8):988–98.

    Article  Google Scholar 

  23. 23.

    Hu T, Wang X, Yu C, Yan J, Zhang X, Li L, et al. Follicular dendritic cell sarcoma of the pharyngeal region. Oncol Lett. 2013;5(5):1467–76. https://doi.org/10.3892/ol.2013.1224.

    Article  PubMed  PubMed Central  Google Scholar 

  24. 24.

    Idrees MT, Brandwein-Gensler M, Strauchen JA, Gil J, Wang BY. Extranodal follicular dendritic cell tumor of the tonsil: report of a diagnostic pitfall and literature review. Arch Otolaryngol-Head Neck Surg. 2004;130(9):1109–13. https://doi.org/10.1001/archotol.130.9.1109.

    Article  PubMed  Google Scholar 

  25. 25.

    Kara T, Serinsoz E, Arpaci RB, Vayisoglu Y. Follicular dendritic cell sarcoma of the tonsil. BMJ Case Rep. 2013. https://doi.org/10.1136/bcr-2012-007440. 2013.

    Article  PubMed  PubMed Central  Google Scholar 

  26. 26.

    Kulkarni MP, Momin YA, Deshmukh BD, Sulhyan KR. Extranodal follicular dendritic cell sarcoma involving tonsil. Malays J Pathol. 2015;37(3):293–9.

    PubMed  Google Scholar 

  27. 27.

    Li L, Shi YH, Guo ZJ, Qiu T, Guo L, Yang HY, et al. Clinicopathological features and prognosis assessment of extranodal follicular dendritic cell sarcoma. World J Gastroenterol. 2010;16(20):2504–19.

    Article  Google Scholar 

  28. 28.

    Lu ZJ, Li J, Zhou SH, Dai LB, Yan SX, Wu TT, et al. Follicular dendritic cell sarcoma of the right tonsil: A case report and literature review. Oncol Lett. 2015;9(2):575–82. https://doi.org/10.3892/ol.2014.2726.

    Article  PubMed  Google Scholar 

  29. 29.

    McDuffie C, Lian TS, Thibodeaux J. Follicular dendritic cell sarcoma of the tonsil: a case report and literature review. Ear Nose Throat J. 2007;86(4):234–5.

    Article  Google Scholar 

  30. 30.

    Mondal SK, Bera H, Bhattacharya B, Dewan K. Follicular dendritic cell sarcoma of the tonsil. Natl J Maxillofacial Surg. 2012;3(1):62–4. https://doi.org/10.4103/0975-5950.102165.

    Article  Google Scholar 

  31. 31.

    Pang J, Mydlarz WK, Gooi Z, Waters KM, Bishop J, Sciubba JJ, et al. Follicular dendritic cell sarcoma of the head and neck: case report, literature review, and pooled analysis of 97 cases. Head Neck. 2016;38(Suppl 1):E2241-9. https://doi.org/10.1002/hed.24115.

    Article  PubMed  Google Scholar 

  32. 32.

    Shia J, Chen W, Tang LH, Carlson DL, Qin J, Guillem JG, et al. Extranodal follicular dendritic cell sarcoma: clinical, pathologic, and histogenetic characteristics of an underrecognized disease entity. Virchows Archiv. 2006;449(2):148–58. https://doi.org/10.1007/s00428-006-0231-4.

    Article  PubMed  Google Scholar 

  33. 33.

    Suchitha S, Sheeladevi CS, Sunila R, Manjunath GV. Extra nodal follicular dendritic cell tumor. Indian J Pathol Microbiol. 2010;53(1):175–7. https://doi.org/10.4103/0377-4929.59224.

    CAS  Article  PubMed  Google Scholar 

  34. 34.

    Suhail Z, Musani MA, Afaq S, Zafar A, Ahmed Ashrafi SK. Follicular dendritic cell sarcoma of tonsil. J Coll Physicians Surg–Pak. 2010;20(1):55–6. doi:01.2010/jcpsp.5556.

    PubMed  Google Scholar 

  35. 35.

    Tisch M, Hengstermann F, Kraft K, von Hinuber G, Maier H. Follicular dendritic cell sarcoma of the tonsil: report of a rare case. Ear Nose Throat J. 2003;82(7):507–9.

    Article  Google Scholar 

  36. 36.

    Vaideeswar P, George SM, Kane SV, Chaturvedi RA, Pandit SP. Extranodal follicular dendritic cell sarcoma of the tonsil—case report of an epithelioid cell variant with osteoclastic giant cells. Pathol Res Pract. 2009;205(2):149–53. https://doi.org/10.1016/j.prp.2008.07.006.

    Article  PubMed  Google Scholar 

  37. 37.

    Vargas H, Mouzakes J, Purdy SS, Cohn AS, Parnes SM. Follicular dendritic cell tumor: an aggressive head and neck tumor. Am J Otolaryngol. 2002;23(2):93–8.

    Article  Google Scholar 

  38. 38.

    Vorsprach M, Kalinski T, Vorwerk U. Follicular dendritic cell sarcoma of the tonsil. Pathol Res Pract. 2015;211(1):88–91. https://doi.org/10.1016/j.prp.2014.09.015.

    Article  PubMed  Google Scholar 

  39. 39.

    Perez-Ordonez B, Rosai J. Follicular dendritic cell tumor: review of the entity. Semin Diagn Pathol. 1998;15(2):144–54.

    CAS  PubMed  Google Scholar 

  40. 40.

    Gounder M, Desai V, Kuk D, Agaram N, Arcila M, Durham B, et al. Impact of surgery, radiation and systemic therapy on the outcomes of patients with dendritic cell and histiocytic sarcomas. Eur J Cancer. 2015;51(16):2413–22. https://doi.org/10.1016/j.ejca.2015.06.109.

    Article  PubMed  PubMed Central  Google Scholar 

  41. 41.

    Dalia S, Jaglal M, Chervenick P, Cualing H, Sokol L. Clinicopathologic characteristics and outcomes of histiocytic and dendritic cell neoplasms: the moffitt cancer center experience over the last twenty five years. Cancers. 2014;6(4):2275–95. https://doi.org/10.3390/cancers6042275.

    Article  PubMed  PubMed Central  Google Scholar 

  42. 42.

    Amiri-Kordestani L, Priebat D, Chia SH. Follicular dendritic cell sarcoma of the neck: case report and review of current diagnostic and management strategies. Ear Nose Throat J. 2010;89(7):E7–E14.

    Article  Google Scholar 

Download references

Funding

No funding was received.

Author information

Affiliations

Authors

Corresponding author

Correspondence to Fredrik Petersson.

Ethics declarations

Conflict of interest

The author has no conflict of interest.

Ethical Approval

It is our institution’s policy not to require formal ethical approval for reports on up to two patients.

Rights and permissions

Reprints and Permissions

About this article

Verify currency and authenticity via CrossMark

Cite this article

Wu, B., Lim, C.M. & Petersson, F. Primary Tonsillar Epithelioid Follicular Dendritic Cell Sarcoma: Report of a Rare Case Mimicking Undifferentiated Carcinoma and a Brief Review of the Literature. Head and Neck Pathol 13, 606–612 (2019). https://doi.org/10.1007/s12105-019-01015-3

Download citation

Keywords

  • Dendritic cell sarcoma
  • Follicular
  • Palatine tonsil
  • Carcinoma
  • Oropharynx