190 Multifocal Histiocytic Sarcoma of the Stomach
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Histiocytic sarcoma (HS) is a rare malignant proliferation of mature histiocytic cells accounting for less than 1% of all lympho-hematologic malignancies with a median age of 55 years at presentation and poor prognosis. HS generally presents in the lymph nodes, but the gastrointestinal tract is the most common extra-nodal site. Physical findings usually include lymphadenopathy, splenomegaly, hepatosplenomegaly or skin lesions and usually presents as a solitary mass. Here we present a case of a 76-year-old woman with past medical history significant for coronary artery disease who was admitted for symptomatic upper gastrointestinal bleeding. Endoscopy revealed multiple gastric polyps, and the patient subsequently underwent total gastrectomy for persistent bleeding. On gross examination, there were multiple, pedunculated polyps as well as an underlying thickened wall predominantly located in the fundus with sparing of the lesser curvature. Sectioning of the masses revealed uniform, fleshy cut surfaces with intact muscularis propria. Histological examination of the specimen showed mixed epithelioid and spindled cell morphology with increased mitotic rate and necrosis. Cytologically, the nuclei were uniformly enlarged with pale eosinophilic cytoplasm and prominent nucleolus. On immunohistochemistry, the tumor cells showed strong reactivity with histiocytic antigens PU.1/ CD163 and weakly reactive for CD68. In addition, vimentin was positive, but markers of epithelial, muscular, neural, lymphocytic, and melanocytic origin were all negative. Ki-67 showed a proliferative index of 30%. Follow-up of the patient shows persistent bleeding with local recurrence at the surgical site and metastasis to the left atrium. This case represents an unusual lesion in the differential diagnosis of gastric epithelioid and spindled neoplasms. Due to its aggressive nature, early recognition of this entity is required for appropriate management.Keywords:
Histiocytic sarcoma
CD68
Histiocytic sarcoma consisting of phagocyting histiocytes is a rare variant of histiocytic sarcoma. It is hard to detect because of its similarity with large cell lymphomas, in particular with anaplastic large cell lymphoma. Histological and immunohistochemical findings are presented. Differential diagnosis with tumours of hematopoietic and non-hematopoietic nature is described.
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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A 54-year-old male patient underwent upper gastrointestinal endoscopy, which revealed a 25-mm brown region in the angular section of the greater curvature of the stomach. The region was histologically determined to be gastric mucosa with an accumulation of histiocytes containing eosinophilic substances in the cytoplasm and chronic inflammatory cell infiltration. Histiocytes were immunohistologically positive for CD68, IgG, and κ. Based on these findings, the patient was diagnosed with gastric crystal-storing histiocytosis comprised of histiocytes phagocytosing IgG-κ-type immunoglobulin. This is a rare disease of which there have been no previous reports that included long-term follow-up. Here, we report the case with a literature review.
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Histiocytic sarcoma
CD68
Malignant histiocytosis
Neoplasm
CD163
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Tumours of histiocytes and accessory dendritic cells: an immunohistochemical approach to classification from the International Lymphoma Study Group based on 61 cases Neoplasms of histiocytes and dendritic cells are rare, and their phenotypic and biological definition is incomplete. Seeking to identify antigens detectable in paraffin‐embedded sections that might allow a more complete, rational immunophenotypic classification of histiocytic/dendritic cell neoplasms, the International Lymphoma Study Group (ILSG) stained 61 tumours of suspected histiocytic/dendritic cell type with a panel of 15 antibodies including those reactive with histiocytes (CD68, lysozyme (LYS)), Langerhans cells (CD1a), follicular dendritic cells (FDC: CD21, CD35) and S100 protein. This analysis revealed that 57 cases (93%) fit into four major immunophenotypic groups (one histiocytic and three dendritic cell types) utilizing six markers: CD68, LYS, CD1a, S100, CD21, and CD35. The four (7%) unclassified cases were further classifiable into the above four groups using additional morphological and ultrastructural features. The four groups then included: (i) histiocytic sarcoma ( n =18) with the following phenotype: CD68 (100%), LYS (94%), CD1a (0%), S100 (33%), CD21/35 (0%). The median age was 46 years. Presentation was predominantly extranodal (72%) with high mortality (58% dead of disease (DOD)). Three had systemic involvement consistent with `malignant histiocytosis'; (ii) Langerhans cell tumour (LCT) ( n =26) which expressed: CD68 (96%), LYS (42%), CD1a (100%), S100 (100%), CD21/35 (0%). There were two morphological variants: cytologically typical ( n =17) designated LCT; and cytologically malignant ( n =9) designated Langerhans cell sarcoma (LCS). The LCS were often not easily recognized morphologically as LC‐derived, but were diagnosed based on CD1a staining. LCT and LCS differed in median age (33 versus 41 years), male:female ratio (3.7:1 versus 1:2), and death rate (31% versus 50% DOD). Four LCT patients had systemic involvement typical of Letterer–Siwe disease; (iii) follicular dendritic cell tumour/sarcoma (FDCT) ( n =13) which expressed: CD68 (54%), LYS (8%), CD1a (0%), S100 (16%), FDC markers CD21/35 (100%), EMA (40%). These patients were adults (median age 65 years) with predominantly localized nodal disease (75%) and low mortality (9% DOD); (iv) interdigitating dendritic cell tumour/sarcoma (IDCT) ( n =4) which expressed: CD68 (50%), LYS (25%), CD1a (0%), S100 (100%), CD21/35 (0%). The patients were adults (median 71 years) with localized nodal disease (75%) without mortality (0% DOD). In conclusion, definitive immunophenotypic classification of histiocytic and accessory cell neoplasms into four categories was possible in 93% of the cases using six antigens detected in paraffin‐embedded sections. Exceptional cases (7%) were resolvable when added morphological and ultrastructural features were considered. We propose a classification combining immunophenotype and morphology with five categories, including Langerhans cell sarcoma. This simplified scheme is practical for everyday diagnostic use and should provide a framework for additional investigation of these unusual neoplasms.
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Canine histiocytic neoplasms include cutaneous histiocytoma, as well as localized and disseminated histiocytic sarcoma. These tumors have variable biologic behavior, although the malignant disorders often have a poor prognosis. Immunohistochemistry plays an essential role in differentiating histiocytic tumors from other neoplasias that may have similar histological appearances. This allows a definitive diagnosis to be established and provides a more accurate prediction of prognosis. This article reviews the biologic behavior, diagnosis, and treatment of histiocytic tumors in the dog.
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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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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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Malignant histiocytosis
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