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Journal of Gastrointestinal Cancer

, Volume 50, Issue 4, pp 913–918 | Cite as

Follicular Dendritic Cell Sarcoma of Gastrointestinal Tract: an Uncommon Lesion, Commonly Missed

  • Usman HassanEmail author
  • Iftikhar Ali Rana
  • Sajid Mushtaq
  • Muhammad Azam
  • Noreen Akhter
Original Research

Abstract

Introduction

Follicular dendritic cell sarcoma (FDCS) is a rare neoplasm, accounting for only 0.4% of soft-tissue sarcomas. It shows both nodal and extranodal involvement. Considering the rarity and difficulties in diagnosing this tumor, we consider it very important to share our experience of diagnosing FDCS. Its correct diagnosis cannot be overemphasized as the treatment and prognosis of FDCS are very much different from tumors which come in its differential diagnosis.

Material and Methods

We are presenting eight cases of extranodal FDCS in gastrointestinal tract diagnosed at our center in a period of 3 years (Feb 2015 to Feb 2018). Presenting complaints, demographic details, gross description, histologic features, immunostain results, and clinical follow-up were evaluated.

Results

Four patients were females and four were males. Tumor ranged in size from 5.5 to 35 cm. In five cases, tumor cells were arranged in storiform and whorling pattern. Lymphocytes were seen sprinkled in between these cells. In one case, lymphocytic infiltrate was extensive. Giant cells and frequent mitoses were noted in two cases. One case showed extensive necrosis. Tumor cells were strongly and diffusely positive for CD21 and CD35. Mean follow up of 11.8 months (range 01 to 24 months) was noted.

Conclusion

FDCS is a rare tumor having distinct morphology and phenotype which if known can be correctly diagnosed. Therefore, knowledge of its varied location, morphology, and phenotype is very important to correctly diagnose this tumor and to prevent misdiagnosis and mistreatment.

Keywords

Follicular dendritic cell sarcoma Gastrointestinal tract 

Notes

Compliance with Ethical Standards

Conflict of Interest

The authors declare that there is no conflict of interest.

References

  1. 1.
    Meyer RL. Malignant lymphomas other than Hodgkin’s disease: histology, cytology, ultrastructure, immunology. JAMA. 1979;242(2):193.CrossRefGoogle Scholar
  2. 2.
    Monda LA, Warnke RO, Rosai JU. 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.PubMedPubMedCentralGoogle Scholar
  3. 3.
    Hollowood K, Stamp G, Zouvani L, Fletcher CD. Extranodal follicular dendritic cell sarcoma of the gastrointestinal tract: morphologic, immunohistochemical and uhrastructural analysis of two cases. Am J Clin Pathol. 1995;103(1):90–7.PubMedCrossRefGoogle Scholar
  4. 4.
    Li J, Geng ZJ, Xie CM, Zhang XK, Chen RY, Cai PQ, et al. Computer tomography imaging findings of abdominal follicular dendritic cell sarcoma: a report of 5 cases. Medicine. 2016;95(1):e2404.PubMedPubMedCentralCrossRefGoogle Scholar
  5. 5.
    Tew JG, Wu J, Qin D, Helm S, Burton GF, Szakal AK. Follicular dendritic cells and presentation of antigen and costimulatory signals to B cells. Immunol Rev. 1997;156(1):39–52.PubMedCrossRefGoogle Scholar
  6. 6.
    Chan JK, Fletcher CD, Nayler SJ, Cooper K. Follicular dendritic cell sarcoma. Cancer. 1997;79(2):294–313.PubMedCrossRefGoogle Scholar
  7. 7.
    Su Z, Liu G, Liu J, Fang T, Zeng Y, Zhang H, et al. Paraneoplastic pemphigus associated with follicular dendritic cell sarcoma: report of a case and review of literature. Int J Clin Exp Pathol. 2015;8(10):11983.PubMedPubMedCentralGoogle Scholar
  8. 8.
    Chow SC, Yeung EC, Ng CS, Wong RH, Fai To K, Wan IY. Mediastinal follicular dendritic cell sarcoma with paraneoplastic pemphigus. Asian Cardiovasc Thorac Ann. 2015;23(6):732–4.PubMedCrossRefGoogle Scholar
  9. 9.
    Cakir E, Aydin NE, Samdanci E, Karadag N, Sayin S, Kizilay A. Follicular dendritic cell sarcoma associated with hyaline-vascular Castleman’s disease. J Pak Med Assoc. 2013;63(3):393–5.Google Scholar
  10. 10.
    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.PubMedPubMedCentralCrossRefGoogle Scholar
  11. 11.
    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: WJG. 2010;16(20):2504.PubMedCrossRefGoogle Scholar
  12. 12.
    Chen T, Gopal P. Follicular dendritic cell sarcoma. Arch Pathol Lab Med. 2017;141(4):596–9.PubMedCrossRefGoogle Scholar
  13. 13.
    Chan JK, Tsang WY, Ng CS. Follicular dendritic cell tumor and vascular neoplasm complicating hyaline-vascular Castleman’s disease. Am J Surg Pathol. 1994;18(5):517–25.PubMedCrossRefGoogle Scholar
  14. 14.
    Biddle DA, Ro JY, Yoon GS, Yong YW, Ayala AG, Ordonez NG. 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.PubMedCrossRefGoogle Scholar
  15. 15.
    Soriano AO, Thompson MA, Admirand JH, Fayad LE, Rodriguez AM, Romaguera JE, et al. Follicular dendritic cell sarcoma: a report of 14 cases and a review of the literature. Am J Hematol. 2007;82(8):725–8.PubMedCrossRefGoogle Scholar
  16. 16.
    Kröber SM, Marx A, Aebert H, Dohmen BM, Kaiserling E. Sarcoma of follicular dendritic cells in the dorsal mediastinum. Hum Pathol. 2004;35(2):259–63.PubMedCrossRefGoogle Scholar
  17. 17.
    Khalid T, Folman R. Symptoms in cancer patients and an unusual tumor: case 3. Follicular dendritic cell sarcoma. J Clin Oncol. 2005;23(36):9425–6.PubMedCrossRefGoogle Scholar
  18. 18.
    Galati LT, Barnes EL, Myers EN. Dendritic cell sarcoma of the thyroid. Head Neck. 1999;21(3):273–5.PubMedCrossRefGoogle Scholar
  19. 19.
    Romero-Guadarrama MB, Reyes-Posada O, Hernández-González MM, Durán-Padilla MA. Follicular dendritic cell sarcoma/tumor: 2 cases of a rare tumor of difficult clinical and pathological diagnosis. Ann Diagn Pathol. 2009;13(4):257–62.PubMedCrossRefGoogle Scholar
  20. 20.
    Geerts A, Lagae E, Dhaene K, Peeters M, Waeytens A, Demetter P, et al. Metastatic follicular dendritic cell sarcoma of the stomach: a case report and review of the literature. Acta Gastro-Enterol Belg. 2004;67(2):223–7.Google Scholar
  21. 21.
    Granados R, Aramburu JA, Rodríguez JM, Nieto MA. Cytopathology of a primary follicular dendritic cell sarcoma of the liver of the inflammatory pseudotumor-like type. Diagn Cytopathol. 2008;36(1):42–6.PubMedCrossRefGoogle Scholar
  22. 22.
    Urun Y, Kankaya D, Koral L, Yalcin B, Karabork A, Ceyhan K, et al. Intraabdominal follicular dendritic cell sarcoma: a report of three cases and review of the literature. Tumori. 2013;99(2):e65–9.PubMedCrossRefGoogle Scholar
  23. 23.
    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.PubMedCrossRefGoogle Scholar
  24. 24.
    Grogg KL, Macon WR, Kurtin PJ. Nascimento AG. A survey of clusterin and fascin expression in sarcomas and spindle cell neoplasms: strong clusterin immunostaining is highly specific for follicular dendritic cell tumor. Mod Pathol. 2005;18(2):260.PubMedCrossRefGoogle Scholar
  25. 25.
    Yu H, Gibson JA, Pinkus GS, Hornick JL. Podoplanin (D2-40) is a novel marker for follicular dendritic cell tumors. Am J Clin Pathol. 2007;128(5):776–82.PubMedCrossRefGoogle Scholar
  26. 26.
    Lima FE, Jorge IF, Kauffman J, Gerson G, Beco MD, Cavalcante JM, et al. Follicular dendritic cell sarcoma: report of two cases and literature review. Jornal Brasileiro de Patologia e Medicina Laboratorial. 2015;51(4):258–64.CrossRefGoogle Scholar
  27. 27.
    Fonseca R, Tefferi A, Strickler JG. Follicular dendritic cell sarcoma mimicking diffuse large cell lymphoma: a case report. Am J Hematol. 1997;55(3):148–55.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Shaukat Khanum Memorial Cancer Hospital and Research CentreLahorePakistan
  2. 2.Department of PathologyShaukat Khanum Memorial Cancer Hospital and Research CentreLahorePakistan

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