Indian Journal of Pathology and Microbiology
Home About us Instructions Submission Subscribe Advertise Contact e-Alerts Ahead Of Print Login 
Users Online: 1221
Print this page  Email this page Bookmark this page Small font sizeDefault font sizeIncrease font size

  Table of Contents    
Year : 2012  |  Volume : 55  |  Issue : 2  |  Page : 233-235
Multifocal histiocytic sarcoma of the gastrointestinal tract

1 Department of Pathology, Cancer Institute (W.I.A.), Chennai, India
2 Department of Surgical Oncology, Cancer Institute (W.I.A.), Chennai, India
3 Department of Medical Oncology, Cancer Institute (W.I.A.), Chennai, India

Click here for correspondence address and email

Date of Web Publication3-Jul-2012


We report a rare case of extranodal histiocytic sarcoma with multifocal gastrointestinal tract involvement, which has not been documented in the literature so far. A diagnosis of interdigitating dendritic cell/ histiocytic sarcoma was made on the preoperative gastric biopsy. Computed tomography scan revealed multifocal, circumferential gastrointestinal wall thickening involving the stomach and jejunal loops. Patient underwent distal gastrectomy with extended D1 dissection and proximal jejunal resection with gastrojejunostomy. Immunohistochemistry profile of both the gastric and jejunal tumors was similar to the preoperative gastric biopsy. The histiocytic origin of the tumor was confirmed by positive reaction of the tumor cells for CD 163. She received four cycles of CHOP chemotherapy, and is free of disease three years, following surgery.

Keywords: Extranodal, gastrointestinal tract, histiocytic sarcoma

How to cite this article:
Sundersingh S, Majhi U, Seshadhri RA, Tenali GS. Multifocal histiocytic sarcoma of the gastrointestinal tract. Indian J Pathol Microbiol 2012;55:233-5

How to cite this URL:
Sundersingh S, Majhi U, Seshadhri RA, Tenali GS. Multifocal histiocytic sarcoma of the gastrointestinal tract. Indian J Pathol Microbiol [serial online] 2012 [cited 2021 Dec 1];55:233-5. Available from: https://www.ijpmonline.org/text.asp?2012/55/2/233/97891

   Introduction Top

Histiocytic tumors are rare comprising less than 1% of all nodal or extranodal lymphoid tissue tumors. [1],[2] The term histiocytic sarcoma was introduced in 1970 by Mathe et al[3] for tumors with histologic similarities to macrophages, which were previously known as reticulum cell sarcoma and later histiocytic lymphoma. Recently, World Health Organization (WHO) has defined histiocytic sarcoma as malignant proliferation of cells showing morphologic and immunophenotypic features of mature tissue histiocytes. [4] The diagnosis of histiocytic sarcoma relies predominantly on the confirmation of its histiocytic lineage and the exclusion of other poorly differentiated tumors like lymphoma, carcinoma, sarcoma, and melanoma. Cases arising primarily at extranodal sites may go unrecognized. [5]

   Case Report Top

A 28-year-old female presented to us with complaints of pain in the abdomen since six months and melena since three months. On examination, two hard lobulated and irregular masses were palpable in the right hypochondrium and right iliac fossa measuring 5x7 cm and 7x7 cm, respectively. There was no hepatosplenomegaly. Computed tomography scan revealed multifocal, circumferential gastrointestinal wall thickening involving the stomach and jejunal loops. No evidence of bowel obstruction was noted. Rest of the bowel loops and mesentery appeared normal. Oesophago-gastro-duodenoscopy (OGD) showed Borrmann type V lesion starting from incisura extending to pyloric antrum. Colonoscopy was done and visualized portions of bowel up to cecum were normal. Per vaginal and per rectal examinations were normal. OGD biopsy was done.

Histopathological examination showed gastric mucosa infiltrated by diffuse sheets of round oval to fusiform cells with abundant eosinophilic cytoplasm and irregular vesicular nuclei. Many of these cells contained red cells in their cytoplasm (erythrophagocytosis); and mitosis was increased [Figure 1]. Admixed lymphocytes and plasma cells were also seen. An extensive panel of immunohistochemistry was done, which showed positive reaction of the tumor cells for vimentin, CD 45, CD 45 RO, S100 protein, CD 68 and negative reaction for CD 20, CD 79 α, CD 3, CD 43, CD 30, Alk 1, MPO, CD 1a, CD 21, CD 23, CD 35, SMA, Desmin, Myogenin, c-Kit, EMA, keratin, HMB 45 and Melan A. The ki-67 index was 60-70%. A diagnosis of interdigitating dendritic cell/ histiocytic sarcoma was made.
Figure 1: Diffuse proliferation of tumor cells exhibiting erythrophagocytosis and increased mitoses (hematoxylin and eosin, ×400)

Click here to view

The patient underwent distal gastrectomy with extended D1 dissection and proximal jejunal resection with gastrojejunostomy. Gross examination of the gastrectomy specimen showed a large ulceroproliferative growth measuring 9x9x2.5 cm involving the body and pyloric antrum, whereas examination of the jejunal specimen showed three discrete ulceroproliferative/polypoidal growths measuring 10x9x3.5, 7x6x3.5, and 2.5x2.2x1.5 cm, respectively. Histopathological examination of both gastric and jejunal growths showed features as described earlier in the biopsy material [Figure 2] and [Figure 3]. Splindling of tumor cells imparting a storiform pattern were seen at foci. Mitotic figures of more than 20/10 high power fields, areas of necrosis, and lymphatic tumor emboli were also noted. Tumor was seen infiltrating the entire thickness of gastric and jejunal wall extending upto the inked serosa. Immunohistochemistry profile of both the gastric and jejunal tumors was similar to the preoperative gastric biopsy. The histiocytic origin of the tumor was confirmed by positive cytoplasmic reaction of the tumor cells for CD 163 [Figure 4]. Five lymph nodes around the stomach and three lymph nodes around the jejunum were involved by metastatic tumor.
Figure 2: Gastric mucosa infiltrated by tumor showing lymphatic tumor emboli (hematoxylin and eosin, ×200)

Click here to view
Figure 3: Jejunum infiltrated by tumor (hematoxylin and eosin, ×200)

Click here to view
Figure 4: Positive reaction of tumor cells for CD 163 (DAB, ×400)

Click here to view

   Discussion Top

Histiocytic sarcoma is rare, with only limited numbers of bona fide cases reported. Gastrointestinal involvement manifests in the form of abdominal mass, pain abdomen, intestinal obstruction, lower gastrointestinal bleeding or weight loss. Histopathological examination shows diffuse proliferation of large round to oval cells with abundant eosinophilic cytoplasm and round to oval irregularly folded vesicular often eccentrically placed nuclei with moderate to marked atypia and one or more nucleoli. Focal sarcomatoid areas characterized by spindling of tumor cells and hemophagocytosis may be present. A variable number of reactive lymphocytes, plasma cells, neutrophils, and eosinophils are seen in the background. Tumors arising in the gastrointestinal tract often also involve regional lymph nodes.

Extranodal histiocytic sarcoma may cause a diagnostic dilemma to pathologists. Immunohistochemistry plays a major role in the confirmation of its histiocytic lineage. Expression of one or more histiocytic markers like CD 163, CD 68 (KP1 and PGM1) and lysozyme are seen. CD 163, a hemoglobin scavenger receptor that identifies histiocytic cells with a high degree of specificity has become a promising marker for the diagnosis of histiocytic malignancies. [6],[7] CD45, CD 45RO (T and macrophage/histiocytic marker) and HLA-DR are usually positive. There may be expression of S-100 protein, but there is no positivity for specific B and T-cell markers. The Ki67 index is variable.

Histiocytic sarcoma should be differentiated from other histiocytic and dendritic cell tumors. [8] A panel of markers like CD 68, lysozyme, CD 1a, S100 protein, CD 21, CD 23 and CD 35 has been recommended to aid in the differential diagnosis. Langerhan cell sarcoma consistently expresses S100 protein and CD1a. Interdigitating dendritic cell sarcoma expresses S100 protein and is weakly positive for CD 68, lysozyme and CD 45 however, it lacks the specific histiocytic marker CD 163. [4] Follicular dendritic cell sarcomas are strongly positive for one or more of the follicular dendritic cell markers including CD 21, CD 23 and CD 35. Diffuse large B-cell lymphoma (DLBCL) or an anaplastic large cell lymphoma is distinguished by the expression of pan-B markers such as CD19, CD 20, CD 22, and CD 79α in the former and CD 30 and Alk 1 in the latter. Other tumors that form the differential diagnosis are carcinomas, melanomas, and soft tissue sarcoma, which can be excluded by lack of reactivity for keratin, HMB 45, Melan A, CD 99, c-Kit, SMA, desmin, and myogenin.

Treatment options for histiocytic sarcoma include surgery, chemotherapy and radiotherapy. Surgical resection with wide margins is the gold standard of treatment. Radiotherapy may play an important role in cases, where a complete R0 resection is not possible. Chemotherapy regimens remain controversial. Although most cases of histiocytic sarcoma have been treated with a chemotherapeutic regimen similar to that used for DLBCL, prognosis was dismal due to poor response to therapy, rapid progression and the presence of widespread disease. Addition of etoposide to CHOP chemotherapy has shown promising results with prolonged clinical response and event free survival. [9] The biologic behavior of histiocytic sarcoma is aggressive with a poor response to therapy. Tumor size and stage are believed to be important factors in the prognosis. [4] Our patient received four cycles of CHOP chemotherapy, is on regular follow-up and free of disease three years following surgery.

   Acknowledgment Top

The authors wish to thank Dr. S. Gujral, Professor, Department of Pathology, Tata Memorial Hospital, Mumbai, India, for his kind help in performing CD 163 and CD 68 (KP1) immunohistochemistry.

   References Top

1.Favara BE, Feller AC, Pauli M, Jaffe ES, Weiss LM, Arico M, et al. Contemporary classification of histiocytic disorders. The WHO Committee On Histiocytic/Reticulum Cell Proliferations. Reclassification Working Group of the Histiocyte Society. Med Pediatr Oncol 1997;29:157-66.   Back to cited text no. 1
2.Jaffe ES, Harris NL, Stein H, Vardiman JW, editors. Pathology and Genetics of Tumours of the Haematopoeitic and Lymphoid Tissues. Lyon, France: IARC Press; 2001. p. 273-90.  Back to cited text no. 2
3.Mathé G, Gerard-Marchant R, Texier JL, Schlumberger JR, Berumen L, Paintrand M. The two varieties of lymphoid tissue "reticulosarcomas", histiocytic and histioblastic types. Br J Cancer 1970;24:687-95.  Back to cited text no. 3
4.Grogan TM, Pileri SA, Chan JK, Weiss LM, Fletcher CD. Histiocytic sarcoma. In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, et al, editors. WHO Classification of Tumours of Haematopoeitic and Lymphoid Tissues. Lyon, France: IARC; 2008. p. 356.  Back to cited text no. 4
5.Hornick JL, Jaffe ES, Fletcher CD. Extranodal histiocytic sarcoma: Clinicopathologic analysis of 14 cases of a rare epithelioid malignancy. Am J Surg Pathol 2004;28:1133-44.  Back to cited text no. 5
6.Vos JA, Abbondanzo SL, Barekman CL, Andriko JW, Miettinen M, Aguilera NS. Histiocytic sarcoma: A study of five cases including the histiocyte marker CD163. Mod Pathol 2005;18:693-704.  Back to cited text no. 6
7.Lau SK, Chu PG, Weiss LM. CD163: A specific marker of macrophages in paraffin-embedded tissue samples. Am J Clin Pathol 2004;122:794-801.  Back to cited text no. 7
8.Pileri SA, Grogan TM, Harris NL, Banks P, Campo E, Chan JK, et al. Tumours of histiocytes and accessory dendritic cells: An immunohistochemical approach to classification from the International Lymphoma Study Group based on 61 cases. Histopathology 2002;41:1-29.  Back to cited text no. 8
9.Yoshida C, Takeuchi M. Histiocytic sarcoma: Identification of its histiocytic origin using immunohistochemistry. Intern Med 2008;47:165-9.  Back to cited text no. 9

Correspondence Address:
Shirley Sundersingh
New No.23, Jambulingam Street, Nungambakkam, Chennai - 600 034
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.97891

Rights and Permissions


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

This article has been cited by
1 An advanced case of gastric histiocytic sarcoma treated with chemotherapy and gastrectomy: a case report and review of literature
Ryu Matsunaga, Yoshikazu Kanazawa, Kunihiko Matsuno, Daisuke Kakinuma, Taichiro Tokura, Atsushi Marumo, Shunsuke Yui, Fumihiko Ando, Yuka Masuda, Nobutoshi Hagiwara, Tsutomu Nomura, Shunji Kato, Toshiro Yoshiyuki, Yukako Domoto, Takashi Sakatani, Ryuji Ohashi, Koiti Inokuchi, Hiroshi Yoshida
Clinical Journal of Gastroenterology. 2021; 14(4): 1053
[Pubmed] | [DOI]
2 Overview of Gastrointestinal Lymphoproliferative disorders?
Aaron Auerbach, Nadine S. Aguilera
Seminars in Diagnostic Pathology. 2021; 38(4): 1
[Pubmed] | [DOI]
3 Leukemic Phase of Histiocytic Sarcoma of the Digestive System: A Rare Manifestation of a Rare Disease
Liron Hofstetter, David Aranovich, Hanna Bernstine, Lucille Hayman, Yael Shahal-Zimra, Esther Rabizadeh, Amos Cohen, Meir Lahav, Pia Raanani, Ofir Wolach
Acta Haematologica. 2021; 144(2): 229
[Pubmed] | [DOI]
4 Histiocytic Sarcoma of the Bile Duct
Katsuyuki Miyabe,Ayako Masaki,Takahiro Nakazawa,Tomoaki Ando,Kazuki Hayashi,Itaru Naitoh,Fumihiro Okumura,Shuya Shimizu,Hiromu Kondo,Michihiro Yoshida,Hiroaki Yamashita,Shuichiro Umemura,Yasuki Hori,Yan Ge,Hirotaka Ohara,Takashi Joh,Hiroshi Inagaki
Internal Medicine. 2014; 53(7): 707
[Pubmed] | [DOI]
5 Primary Gastric Histiocytic Sarcoma Reminiscent of Inflammatory Pseudotumor: A Case Report with Review of the Literature
Dakeun Lee,Young-Bae Kim,Sook Hee Chung,Sang-Ryung Lee,Cheul Su Byun,Sang-Uk Han,Jae Ho Han
Korean Journal of Pathology. 2014; 48(3): 258
[Pubmed] | [DOI]
6 A Rare Case of Follicular Dendritic Cell Sarcoma with Pseudochylous Effusion and Review of Literature From India
Kamal Kant Sahu,Gaurav Prakash,Sandeep Rao,Amanjit Bal,Pankaj Malhotra,Jasmina Ahluwalia,Rakesh K. Vashistha
Indian Journal of Hematology and Blood Transfusion. 2014;
[Pubmed] | [DOI]
7 Histiocytic sarcoma of the stomach
Dolzhikov, A.A., Khizhnyakov, K.O., Mukhina, T.S., Lugovskol, S.L.
Arkhiv Patologii. 2013; 75(1): 34-36
8 Primary histiocytic sarcoma of the stomach: A case report with imaging findings
Shen, X.-Z., Liu, F., Ni, R.-J., Wang, B.-Y.
World Journal of Gastroenterology. 2013; 19(3): 422-425


    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Email Alert *
    Add to My List *
* Registration required (free)  

   Case Report
    Article Figures

 Article Access Statistics
    PDF Downloaded116    
    Comments [Add]    
    Cited by others 8    

Recommend this journal