Autopsy and Case Reports
Autopsy and Case Reports
Article / Clinical Case Report

Pediatric multifocal histiocytic sarcoma- a fatal diagnosis not to miss!

Darilin Shangpliang; Pakesh Baishya; Vandana Raphael; Biswajit Dey; Vikas Kantilal Jagtap

Downloads: 3
Views: 456


Histiocytic sarcoma (HS) is a rare hematolymphoid malignant neoplasm with an aggressive clinical course. It can arise de novo or from low-grade B-cell lymphoma. We describe the case of a 16-year-old boy referred to our hospital with generalized lymphadenopathy, weight loss, and decreased appetite for one month. The patient died undiagnosed on the 7th day of hospitalization. Lymph node and bone marrow biopsies were performed one day before the patient died. The lymph node biopsy revealed an architectural effacement with a diffuse proliferation of large pleomorphic neoplastic cells containing large, multilobulated nuclei, coarse vesicular chromatin, prominent nucleoli, and a moderate amount of eosinophilic cytoplasm. The bone marrow aspiration smears and biopsy also showed evidence of infiltration by these above-mentioned cells. Based on the morphology, along with the exclusion of many differential diagnoses by an extensive panel of immunohistochemical markers, a diagnosis of HS was made. This case report aims at evaluating all the clinical and immunophenotypic features of a case of HS with multifocal presentation and an aggressive clinical course in order to give a correct and definite diagnosis at the proper time.


Histiocytic Sarcoma, Lymphoma, Immunophenotyping


Machado ES, Miranda AC, Escopelli T, Caron R, Escopelli AC. Histiocytic sarcoma.

Rev Bras Hematol Hemoter. 2011;33(2):155-7. []. [PMID:23284265]

Skala SL, Lucas DR, Dewar R. Histiocytic sarcoma: review, discussion of transformation from B-Cell lymphoma, and differential diagnosis.

Arch Pathol Lab Med. 2018;142(11):1322-9. []. [PMID:30407858]

Zhao J, Niu X, Wang Z, Lu H, Lin X, Lu Q. Histiocytic sarcoma combined with acute monocytic leukemia: a case report.

Diagn Pathol. 2015;10(1):110. []. [PMID:26187047]

Jang YH, Park CJ, Huh JR, Jang S, Chi HS. A case of histiocytic sarcoma diagnosed by bone marrow biopsy in a patient suffering from fever for 8 months.

Korean J Lab Med. 2009;29(4):282-5. []. [PMID:19726888]

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. []. [PMID:23801128]

Sundersingh S, Majhi U, Seshadhri RA, Tenali GS. Multifocal histiocytic sarcoma of the gastrointestinal tract.

Indian J Pathol Microbiol. 2012;55(2):233-5. []. [PMID:22771652]

Venkataraman G, McClain KL, Pittaluga S, Rao VK, Jaffe ES. Development of disseminated histiocytic sarcoma in a patient with autoimmune lymphoproliferative syndrome and associated Rosai-Dorfman disease.

Am J Surg Pathol. 2010;34(4):589-94. []. [PMID:20216376]

Park MI, Song KS, Kang DY. Histiocytic sarcoma of rectum - a case report.

Korean J Pathol. 2006;40:156-9.

Kim JM, Oh YS, Lee DW. A case of histiocytic sarcoma arising in head and neck region with rhabdoid differentiation.

Korean J Otorhinolaryngol-Head Neck Surg. 2016;59(9):672-5. [].

Swerdlow SH, Campo E, Pileri SA, et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms.

Blood. 2016;127(20):2375-90. []. [PMID:26980727]

Flego V, Popper H, Volaric D, Bulat-Kardum L. Pulmonary histiocytic sarcoma: a case report and literature review.

AJIM. 2017;5(5):91-4. [].

Hornick J, Jaffe E, Fletcher C. Extranodal histiocytic sarcoma: clinicopathologic analysis of 14 cases of a rare epithelioid malignancy.

Am J Surg Pathol. 2004;28(9):1133-44. []. [PMID:15316312]



















Publication date:

5eb2abdc0e8825c24ad76ee1 autopsy Articles
Links & Downloads

Autops Case Rep

Share this page
Page Sections