Journal of Cytology
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Year : 2014  |  Volume : 31  |  Issue : 4  |  Page : 205-206
Fine needle aspiration of Histiocytic sarcoma

National Institute of Pathology, ICMR, Safdurjang Hospital, New Delhi, India

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Date of Web Publication10-Feb-2015


Histiocytic sarcoma is derived from histiocytes and is among the rarest of the tumors affecting the lymphoid tissue. We report a case of a histiocytic sarcoma arising from the lymph node in a 35-year-old male who came for a fine needle aspiration cytology and a subsequent biopsy was conducted and the tissue subjected to hematoxylin and eosin and immunohistochemistry staining.

Keywords: Diffuse large B cell lymphoma; histiocytic sarcoma; immunohistochemistry

How to cite this article:
Mallya V, Bansal A, Kapoor S. Fine needle aspiration of Histiocytic sarcoma. J Cytol 2014;31:205-6

How to cite this URL:
Mallya V, Bansal A, Kapoor S. Fine needle aspiration of Histiocytic sarcoma. J Cytol [serial online] 2014 [cited 2022 Jul 4];31:205-6. Available from:

   Introduction Top

Histiocytic sarcomas are very rare tumors that affect the hematopoietic and lymphoid tissue. [1],[2] Defined by the WHO as a malignancy with morphological and immunophenotypic features resembling mature tissue histiocytes, these tumors occur at any age and can involve multiple sites in the body. [3] Most of these neoplasms arise from the lymph nodes, the other common sites being the skin and gastrointestinal tract. [2] Involvement of multiple sites is also known. The clinical features would range from the mass, fever, weight loss, skin lesions to hepatosplenomegaly. We hereby report a case of histiocytic sarcoma in a 35-year-old male who was referred for a fine needle aspiration cytology (FNAC).

   Case Report Top

A 35-year-old male with a swelling on the left side of the neck was referred for an FNAC. On examination the left cervical lymph nodes were enlarged and measured 3 cm × 2 cm, were discrete and mobile. There were no other swellings or any systemic symptoms. A fine needle aspiration (FNA) was performed, and large pleomorphic cells with increased nuclear cytoplasmic ratio and coarse chromatin were seen. They were present in the background of mixed inflammatory infiltrate [[Figure 1]a]. Emperipolesis was also seen. A presumptive diagnosis of poorly differentiated carcinoma/lymphoma was made, and a biopsy advised. The biopsy tissue was examined. The architecture of the lymph node was completely effaced and replaced by diffuse non-cohesive proliferation of large atypical cells having abundant eosinophilic cytoplasm, large nucleus, coarse chromatin and prominent nucleoli. Some multinucleated forms were also seen. Mitosis was brisk. Furthermore, admixed were lymphocytes and few eosinophils [[Figure 1]b]. Extensive spindling of the tumor cells was also appreciated. On immunohistochemistry (IHC) these atypical cells showed granular cytoplasmic staining for CD68 [[Figure 2]a]. The cells were focally positive for S100 and CD45Ro [[Figure 2]b]. Weak expression of CD4 was seen. The cells were negative for CD3, CD20, CD33, CD30 and vimentin. Based on the histopathological and IHC findings a diagnosis of histiocytic sarcoma was made.
Figure 1: ( a) Large pleomorphic cells with increased nuclear cytoplasmic ratio and coarse chromatin seen in a background of mixed infl ammatory infi ltrate (Giemsa, ×400). (b) Large atypical cells having abundant eosinophilic cytoplasm, large nucleus, coarse chromatin and prominent nucleoli (H and E, ×400)

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Figure 2: (a) Granular cytoplasmic staining of atypical cells with CD68 (IHC, ×100) (b) focal posi�� vity of atypical cells with CD45 (IHC, ×100)

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   Discussion Top

Histiocytic sarcoma designated in the past as true histiocytic lymphoma is a histiocytic cell neoplasm. Very few cases have been reported in the cytology literature. Most of the previously diagnosed cases are now recognized as diffuse large B cell lymphomas (DLBCL). The term "histiocytic sarcoma" was coined in 1970 by Mathι et al. [4] One-third of the cases present in the lymph node, another one-third in the skin and the rest at extranodal sites such as intestine, bone marrow, liver, spleen, and the central nervous system. [3] Fever and weight loss is common. The age of presentation is wide with a male predilection. [1],[3] A few cases have been described with prior mediastinal germ cell tumor and malignant teratomas. [3],[5] The architecture of the normal organ is effaced with polymorphic tumor cells with the cells being large with increased nuclear cytoplasmic ratio, large nucleus and irregular nuclear outlines. [1],[3],[6] The tumor cells are positive for CD68, CD163, lysozyme, CD11c and CD14 and are negative for myeloperoxidase, CD33 and CD34. [1],[3],[6]

Our case was a 35-year-old male who had the sole complaint of cervical lymphadenopathy of 3 months duration. On FNA, large atypical cells with dense large nuclei were seen prompting a presumptive diagnosis of poorly differentiated carcinoma/anaplastic lymphoma. The histopathological slides of the biopsy showed effacement of lymph node architecture with large cells having pleomorphic nuclei with dense chromatin. Mitosis was brisk. A characteristic feature we noticed was spindling of tumor cells. Histiocytic sarcoma needs to be distinguished from other neoplasms like interdigitating dendritic cell tumor and DLBCL. [5],[6],[7] In our case, the cells were strongly positive for CD68 and focally positive for S100, CD4 and CD45Ro. IHC for CD3, CD20, CD33, CD30 and vimentin was negative. A diagnosis of interdigitating dendritic cell sarcoma was excluded as the tumor cells were not strongly positive for S100. [1],[6] Absence of vimentin, focal S100 and strongCD68 staining also helped to exclude a diagnosis of follicular dendritic cell sarcoma. [1],[6] Also as the tumor cells were negative for CD20 and CD30 on IHC, DLBCL could be ruled out. [1],[6] Negativity of cytokeratin ruled out the possibility of poorly differentiated carcinoma.

Pleomorphic tumor cells with multinucleation and an inflammatory background should suggest the possibility of histiocytic sarcoma as a differential diagnosis for more common large cell non-Hodgkin's lymphomas (DLBCL and anaplastic large cell lymphoma), metastatic melanoma and metastatic undifferentiated large cell carcinoma in cytology.

As this neoplasm is rare, it requires a high degree of suspicion and it poses a diagnostic challenge and employs the use of immunohistochemical studies to establish the true histiocytic lineage to exclude other neoplasms of the lymphoid tissue. [1],[2],[5],[6]

   References Top

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. 1
Miliauskas JR. Fine-needle aspiration cytology: True histiocytic lymphoma/histiocytic sarcoma. Diagn Cytopathol 2003;29:233-5.  Back to cited text no. 2
Jaffe ES, Harris NL, Stein H, Vardiman JW. Pathology and Genetics of Tumours of Hematopoetic and Lymphoid Tissues. 2 nd ed. Lyon: IARC Press; 2001. p. 273-90.  Back to cited text no. 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. 4
Shinoda H, Yoshida A, Teruya-Feldstein J. Malignant histiocytoses/disseminated histiocytic sarcoma with hemophagocytic syndrome in a patient with mediastinal germ cell tumor. Appl Immunohistochem Mol Morphol 2009;17:338-44.  Back to cited text no. 5
Alexiev BA, Sailey CJ, McClure SA, Ord RA, Zhao XF, Papadimitriou JC. Primary histiocytic sarcoma arising in the head and neck with predominant spindle cell component. Diagn Pathol 2007;2:7.  Back to cited text no. 6
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. 7

Correspondence Address:
Varuna Mallya
Research Associate, Department of Pathology, National Institute of Pathology, ICMR, Safdurjang Hospital Campus, New Delhi
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0970-9371.151133

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