Histiocytic Sarcoma: A Case Series of Extranodal and Nodal Presentations


Histiocytic sarcoma
Excision biopsy


How to Cite

Kasinathan G, Kori AN, Hassan N. Histiocytic Sarcoma: A Case Series of Extranodal and Nodal Presentations. Integr J Med Sci [Internet]. 2019 Mar. 8 [cited 2022 Aug. 13];6. Available from: https://www.mbmj.org/index.php/ijms/article/view/105


Introduction: Histiocytic sarcoma is an aggressive malignancy of mature histocytes which often carries a poor prognosis. Histiocytic sarcoma is defined in the World Health Organisation (WHO) classification of histiocytic and dendritic cell neoplasms.
Case Presentation: Case 1 depicts a 42 year old Malay gentleman with no premorbids presented to the haematology unit with a three month history of fever, night sweats, unintentional weight loss, left axillary and bilateral inguinal swellings which were progressively enlarging.  Physical examination revealed a medium built gentleman with left axilla, bilateral inguinal lymphadenopathies and hepatosplenomegaly. Excision biopsies of the left axillary and inguinal lymph nodes were compatible with histiocytic sarcoma. He did not have any bone marrow infiltration. He was treated with 6 cycles of CHOP (Cyclophosphamide, doxorubicin, vincristine, prednisolone) polychemotherapy but he subsequently succumbed to severe hemophagocytic syndrome shortly after his 6th CHOP chemotherapy. Case 2 describes a 55-year-old previously healthy Malay gentleman who presented with perianal swelling and weight loss for two months. Physical examination revealed a large perianal swelling measuring 10 cm with bilateral inguinal lymphadenopathies. Anorectal tissue histology was compatible with the diagnosis of histiocytic sarcoma. He underwent a transverse colostomy which was subsequently reversed post-chemotherapy. He completed 6 cycles of CHOP chemotherapy followed by upfront consolidation autologous stem cell transplant. He is currently 9 months in complete remission.
Conclusion: Histiocytic sarcoma remains a disease with poor treatment outcomes and high mortality. Understanding the pathogenesis and pathobiology of the disease will provide a future to the development of novel therapies.



Akiba J, Harada H, Kawahara A, Arakawa F, Mihashi H, Mihashi R, et al. Histiocytic sarcoma of the parotid gland region. Pathol Int. 2011 Jun; 61(6): 373-6. https://doi.org/10.1111/j.1440-1827.2011.02671.x

Werdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, et al. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. Geneva, Switzerland: WHO Press; 2017. [Accessed 03 Jul 2021]. Available From: http://apps.who.int/bookorders/anglais/detart1.jspcodlan=1&codcol=70&codcch=4002

Low SE, Stafford JS. Malignant histiocytosis: a case report of a rare tumour presenting with spontaneous splenic rupture. J Clin Pathol. 2006 Jul; 59(7): 770-772. https://doi.org/10.1136/jcp.2005.027870

Ansari J, Naqash AR, Munker R, El-Osta H, Master S, Cotelingam JD, et al. Histiocytic sarcoma as a secondary malignancy: pathobiology, diagnosis, and treatment. Eur J Haematol. 2016 Jul; 97(1): 9-16. https://doi.org/10.1111/ejh.12755

Takahashi E, Nakamura S. Histiocytic sarcoma: an updated literature review based on the 2008 WHO classification. J Clin Exp Hematop. 2013; 53(1) 1-8. https://doi.org/10.3960/jslrt.53.1

Hornick JL, Jaffe ES, Fletcher CDM. Extranodal histiocytic sarcoma: clinicopathologic analysis of 14 cases of a rare epithelioid malignancy. Am J Surg Pathol. 2004 Sep; 28 (9): 1133-44. https://doi.org/10.1097/01.pas.0000131541.95394.23

Facchetti F, Pileri SA, Lorenzi L, Tabanelli V, Rimsza L, Pittaluga S,et al. Histiocytic and dendritic cell neoplasms: what have we learnt by studying 67 cases. Virchows Arch. 2017 Oct; 471(4): 467– 489. https://doi.org/10.1007/s00428-017-2176-1

Yamamoto S, Tsukamoto T, Kanazawa A, Shimizu S, Morimura K, Toyokawa T, et al. Laparoscopic splenectomy for histiocytic sarcoma of the spleen. World J Gastrointest Surg. 2013 Apr 27; 5(4): 129-34. https://doi.org/10.4240/wjgs.v5.i4.129

Go H, Jeon YK, Huh J, Choi SJ, Choi YD, Cha JH, et al. Frequent detection of BRAF(V600E) mutations in histiocytic and dendritic cell neoplasms. Histopathology. 2014 Aug; 65(2): 261-72. https://doi.org/10.1111/his.12416

Carrasco DR, Fenton T, Sukhdeo K, Protopopova M, Enos M, You MJ, et al. The PTEN and INK4A/ARF tumor suppressors maintain myelolymphoid homeostasis and cooperate to constrain histiocytic sarcoma development in humans. Cancer Cell. 2006 May 9; (5)10:379-90. https://doi.org/10.1016/j.ccr.2006.03.028

Creative Commons License

This work is licensed under a Creative Commons Attribution 4.0 International License.

Copyright (c) 2019 Ganesh Kasinathan et al.


Download data is not yet available.