Neoplasms of Histiocyte/Macrophage Lineage: Histiocytic Sarcoma and Similar Neoplasms
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Histiocytic sarcoma
CD68
Malignant histiocytosis
Neoplasm
CD163
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Histiocytic sarcoma
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Histiocytic sarcoma is characterized by a malignant invasive proliferation of atypical cells showing morphologic and immunophenotypic features similar to those of mature tissue histiocytes. We report a case of histiocytic sarcoma in 11-year-old male cynomolgus macaque fed with a high-fat diet for 6 years. Invasive proliferation of neoplastic cells was localized in systemic organs such as liver, spleen, aorta, kidney, lung, peritonea, and mesenterium. Diffuse cellular foci of invasive proliferation were mainly observed in the peri/intra vascular area, sinus and serous membrane frequently accompanying arteriosclerosis. The tumor cells showed a round to spindle or polygonal shape with abundant foamy to vacuolated cytoplasm. Hemophagocytosis was noted infrequently. In immunohistochemical examinations, some medium to large-sized histiocytic atypical cells were positive for histiocytic markers such as CD68 and Lysozyme. Our case raises the possibility that histiocytic proliferation might be related to hypercholesterolemia and atherosclerosis. This is the first case report of histiocytic sarcoma in non-human primates.
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This report describes a patient with cytophagic histiocytic panniculitis in which the aggressive use of chemotherapeutic agents resulted in an apparent remission. The histiocytic nature of the process was confirmed by immunohistochemical techniques. There are many similarities with malignant histiocytosis, and it is believed that this entity should be regarded as a variant of malignant histiocytosis.
Malignant histiocytosis
Histiocytic sarcoma
Panniculitis
Histiocytosis X
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Histiocytic sarcoma
Malignant histiocytosis
CD68
Red pulp
Langerhans Cell Histiocytosis
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Malignant histiocytosis
Histiocytic sarcoma
Multinucleate
Meninges
Infiltration (HVAC)
Histogenesis
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Proliferation of the mononuclear phagocyte system cells is the basis for histiocytic proliferative conditions (malignant histiocytosis, histiocytic "reticulosarcoma" histiocytosis X). Cells of the phagocytosing histiocyte type proliferate in malignant histiocytosis and histiocytic "reticulosarcoma), cells of the Langerhans type proliferate in histiocytosis X. According to Rappaport's classification of 1966, histiocytic "reticulosarcoma" is separated from the heterogeneous group of diffuse histiocytic lymphomas. Its morphological picture is non-distinguishable from that of malignant histiocytosis, particularly at the stage of generalization. The Letterer-Siwe disease due to its course, prognosis and special therapeutical approach should be separated from the group of conditions combined under the term "histiocytosis".
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Histiocytic sarcoma is very rare malignant neoplasm showing morphological and immunophenotypic characteristics of histiocyte. Morphologic diagnosis is challenging due to overlapping histologic features with diverse mimics. Histologic diagnosis is confirmed by expression of one or more histiocytic markers, including CD163, CD68, and lysozyme. We present a bona fida case of extranodal histiocytic sarcoma involving the small intestine in a 67-year-old male. The diagnosis is confirmed by diffuse immunoreactivity of CD163, which is believed to be more specific histiocytic marker than CD68.
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Clinicopathological analysis was performed in 19 patients diagnosed clinically with malignant histiocytosis. Ultimately, 9 patients died and 10 are still alive. All 19 had fever of unknown origin. Among the 10 surviving patients, 6 recovered with only supportive therapy such as antibiotic treatment. One recovered with steroid therapy and 2 with VP (vincristine and prednisolone) therapy. Complications due to immunodeficiency were detected in one surviving patient and 2 who died. All 9 patients who died had anemia, and 8 had thrombocytopenia. However, among survivors, only one had anemia and only 2 had thrombocytopenia. Chromosomal abnormality was detected in one patient who died. Histiocytic cells were classified morphologically into 3 types: immature, intermediate and mature. In 4 patients who died, histiocytic cells were immature, but in 4 others mature histiocytic cells were detected. In 5 of the 10 surviving patients, histiocytic cells were of the immature type. Immuno-histochemical analysis of the origin of histiocytic cells in 8 deceased patients showed T-zone histiocytes in one, T cells in one, monocyte phagocytic system (MPS) in 5, and histiocytes of unknown origin in one. Thus, malignant histiocytosis is a heterogenous entity including reactive histiocytic disorder, lymphocytic neoplasm and true histiocytic neoplasm. In histiocyte proliferative disorders, red blood cell counts and platelet counts are useful for assessing prognosis, while cytological findings only confuse this evaluation.
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Histiocytic sarcoma
Monocyte
Langerhans Cell Histiocytosis
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Histiocytic sarcoma is very rare malignant neoplasm showing morphological and immunophenotypic characteristics of histiocyte. Morphologic diagnosis is challenging due to overlapping histologic features with diverse mimics. Histologic diagnosis is confirmed by expression of one or more histiocytic markers, including CD163, CD68, and lysozyme. We present a bona fida case of extranodal histiocytic sarcoma involving the small intestine in a 67-year-old male. The diagnosis is confirmed by diffuse immunoreactivity of CD163, which is believed to be more specific histiocytic marker than CD68.
Histiocytic sarcoma
CD68
CD163
Neoplasm
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Canine histiocytic diseases are an emerging spectrum of diseases characterized by proliferations of histiocytic cells. Nonneoplastic histiocytic disease (reactive histiocytosis, comprising cutaneous and systemic histiocytosis) is uncommon. Neoplastic histiocytic diseases include cutaneous histiocytoma, which is a benign histiocytic tumor, and localized and disseminated histiocytic sarcoma (previously known as malignant histiocytosis), which are malignant diseases. The differentiation of histiocytic diseases can be challenging. This article outlines the characteristics of each disease entity and details the clinicopathologic, histologic, immunohistochemical, prognostic, and therapeutic differences among them.
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