Histiocytic sarcoma: A study of five cases including the histiocyte marker CD163

Department of Hematopathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA.
Modern Pathology (Impact Factor: 6.19). 05/2005; 18(5):693-704. DOI: 10.1038/modpathol.3800346
Source: PubMed


Histiocytic sarcoma (HS) is a rare but controversial hematopoietic neoplasm. In the past, malignancies have been misclassified as histiocytic tumors due to overlapping histologic features and inadequate phenotypic data. CD163, a recently characterized hemoglobin scavenger receptor, appears to be a 'specific' marker of histiocytic lineage and a promising diagnostic tool for evaluating histiocytic neoplasms. Five cases of HS were studied to further elucidate the clinicopathologic features of these rare tumors and to demonstrate the diagnostic utility of CD163. Criteria for diagnosis included histologic and immunohistochemical evidence of histiocytic differentiation, CD45 positivity, and exclusion of lymphoid, epithelial, melanocytic and dendritic cell phenotype. Sites of disease included the colon (two cases), palate, inguinal lymph node, and testis. The clinical course was aggressive in 4/5 patients (survival=2-15 months). One patient with localized disease of the palate, survived 17 years after diagnosis. All patients with poor survival had tumors > or =3.5 cm. Histologically, all cases showed diffuse architecture with large, discohesive polygonal cells. Spindling of cells was focally noted. Hemophagocytosis was identified in 3/5 cases. A prominent inflammatory background was present in 4/5 tumors. All cases were immunoreactive for CD45, CD163, CD68, and lysozyme. S-100 was focally positive in 4/5 cases. Antibodies for melanocytic, epithelial, lymphoid, and dendritic cell markers were negative. Molecular studies showed monoclonal IgH gene rearrangements in three cases. Our findings suggest that HS is an uncommon neoplasm frequently extranodal in presentation and aggressive in behavior, with rare exceptions. Stage of disease and possibly tumor size are significant prognostic indicators. Molecular studies remain controversial in the diagnosis. The morphologic and phenotypic features are relatively uniform; however, the diagnosis requires exclusion of more common neoplasms by extensive immunophenotypic studies. CD163 appears to be a specific histiocytic marker and is important in establishing the diagnosis of HS.

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    • "case of a localized palatal disease successfully treated with surgery alone. The four other cases died within 15 months of diagnosis from progressive histiocytic sarcoma [19]. HS of the CNS has been treated with a variety of modalities in varying combinations. "
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    ABSTRACT: Histiocytic sarcoma (HS) is a rare, aggressive malignancy. Lesions previously called HS were typically non-Hodgkin lymphomas, not HS. As such, chemotherapy directed at lymphoid neoplasms was frequently successful, but it is unclear if these regimens are ideal for HS. We present a 33-year-old African gentleman who underwent sequential renal transplants for glomerulonephritis. He subsequently developed HS of the upper airway and multiple cutaneous sites. The patient received cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) followed by salvage ifosfamide, carboplatin, and etoposide (ICE) but had continuous progression of cutaneous involvement. Cladribine, high-dose cytarabine, G-CSF, and mitoxantrone (CLAG-M) yielded a partial response with near resolution of disease. Ultimately, the patient achieved a complete remission after myeloablative allogeneic hematopoietic stem cell transplant. HS occurring after solid organ transplant raises the possibility of HS as a potential posttransplant malignancy. The use of CLAG-M has not been reported in HS. In this case, histiocyte-directed chemotherapy with CLAG-M was superior to lymphoma-directed regimens.
    06/2015; 2015(Article ID: 728260):6. DOI:10.1155/2015/728260
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    • "In concordance with these studies, strong cytoplasmic immunoreactivity for CD163 was present in the case under discussion confirming histiocytic differentiation. Vos et al. [9] studied five cases of HS and concluded that CD163 appears to be a specific histiocytic marker and is important in establishing the diagnosis of HS. Most of the cases in the literature are reported to be a CD68 positive. "
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    ABSTRACT: Histiocytic sarcoma is a malignant proliferation of cells showing morphological and immunophenotypic features of mature tissue histiocytes. Most of the cases in the literature have reported CD68 positivity. We report a case of histiocytic sarcoma whose presentation mimicked a metastatic breast cancer. A 40-year-female patient presented with a 13 × 11 cm left axillary mass in close proximity to the left breast. Tru-cut biopsy from the lesion suggested the diagnosis of a lymphoid neoplasm. Complete excision of the axillary mass was done. On simple microscopy, numerous mature small lymphocytes were seen dispersed in the follicles. Immunohistochemistry revealed CD31- and CD163-positive cells, which stained negative for CD68, CD1a, cytokeratin and S100; thus, confirming the diagnosis of histiocytic sarcoma.
    07/2014; 2014(7). DOI:10.1093/jscr/rju071
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    • "Some cells present with phagocytosis phenomena. The diagnosis is based on immunohistochemistry markers of histiocytic cells such as positive CD68, lysozyme, α1-antitrypsin and CD163, which is regarded as a specific HS marker [11]. However, HS presents negative markers of epithelial (epithelial membrane antigen or cytokeratin), melanocytic (CD34 or myeloperoxidase) and lymphoid (CD3 or CD20) neoplasms as in our case. "
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    ABSTRACT: Introduction Histiocytic sarcoma is a rare neoplasm with few cases reported in the literature of which some were diagnosed in animals. This neoplasm arises from abnormal reticuloendothelial system cell proliferation of histiocytes and has an aggressive behavior especially if located in the central nervous system. We present the first case of a patient with histiocytic sarcoma that involved the meninges and had a good course after multidisciplinary treatment. Case presentation Our patient was a 41-year-old Caucasian woman with no previous history of disease who started with systemic symptoms such as headache and chills. Magnetic resonance imaging with gadolinium contrast of the brain suggested a mass 1.5×2cm in diameter in the temporal lobe with a non-uniform vasogenic edema. This lesion was implanted in the meninges and surgery was the first treatment. The histological findings revealed a histiocytic sarcoma. The patient received concomitant chemoradiotherapy after surgery with good tolerance and currently lives without disease. Conclusion Although histiocytic sarcomas in the brain present an unusual location and have a poorer prognosis, we have identified the first primary leptomeningeal histiocytic sarcoma with a disease-free survival greater than 3 years following multidisciplinary treatment with surgery and chemotherapy and radiotherapy.
    Journal of Medical Case Reports 05/2013; 7(1):127. DOI:10.1186/1752-1947-7-127
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