Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: EC:3.2.1.17 (lysozyme)
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Reticulohistiocytoma and multicentric reticulohistiocytosis are designations for uncommon, incompletely characterized histiocytic proliferations of the skin or soft tissues. In this study, we analyzed a uniform group of 44 lesions composed of epithelioid histiocytes, comprising a subset of lesions originally designated as reticulohistiocytoma, and propose designating them as "solitary epithelioid histiocytoma" (SEH), in line with the recently published classification proposal for histiocytic disorders. There were 26 males and 18 females with a median age of 35 years (range, 2.5-74 years). All patients had a superficial, circumscribed, mildly elevated, solitary lesion (size range, 1.5-11 mm; median, 4 mm), located in the trunk wall (n = 16), lower extremity (n = 12), head and neck (n = 8, including 2 in the oral cavity), upper extremity (n = 6), penis (n = 1), and an unspecified site (n = 1). Histologically, the lesions typically involved upper and mid-dermis and were not ulcerated. They were composed of large epithelioid histiocytes with a varying number of lymphocytes and neutrophils. The histiocytes had abundant, typically densely eosinophilic, cytoplasm and mostly mild, if any, nuclear atypia. Multinucleated forms with randomly oriented nuclei were also present. The histiocytes had low mitotic activity (range, 0-4 mitoses per 10 wide HPFs; median, 1 mitosis per 10 HPFs). The lesions contained varying numbers of CD3-positive T cells, whereas B lymphocytes, plasma cells, eosinophils, and mast cells were scant, if present at all. Immunohistochemically, the epithelioid histiocytes were positive for CD163, CD68, lysozyme (variably), and vimentin. They often had focal nuclear immunoreactivity for microphthalmia transcription factor, and they sometimes had focal reactivity for Factor XIIIa and S-100 protein. Membrane positivity for CD31, CD43, and CD45 was variable. The epithelioid histiocytes were consistently negative for CD3, CD20, CD30, HMB45, and keratins. All 12 patients with follow-up information had an uneventful clinical course with no recurrences (median, 13 years). SEH is a benign, probably reactive, histiocytic proliferation of unknown etiology. It needs to be distinguished from Rosai-Dorfman disease, juvenile xanthogranuloma, a variety of granulomatous conditions, and some malignant neoplasms, including histiocytic sarcoma, melanoma, and epithelioid sarcoma.
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PMID:Reticulohistiocytoma (solitary epithelioid histiocytoma): a clinicopathologic and immunohistochemical study of 44 cases. 1662

Myeloid sarcoma can involve any anatomic site, but involvement of the gynecologic tract is uncommon. We describe 11 women, 17 to 60 years old, with myeloid sarcoma involving the gynecologic tract, including 5 patients in whom myeloid sarcoma presented as an isolated mass. The uterus was the most frequently involved anatomic site, in 8 patients (5 corpus, 3 cervix). Each neoplasm diffusely infiltrated normal structures, and, cytologically 7 tumors were immature, 3 were differentiated, and 1 was blastic. In 9 cases assessed, immunohistochemical stains showed that all neoplasms were positive for myeloperoxidase and lysozyme; CD117 was positive in 7 of 8 cases, and cytochemical staining for naphthol AS-D chloroacetate was positive in all 6 neoplasms analyzed. Following chemotherapy, complete remission and long-term survival were achieved in a subset of patients, as was particularly true for 2 patients (cases 8 and 10), with complete remission 12.5 and 31 years after diagnosis, respectively.
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PMID:Myeloid sarcoma involving the gynecologic tract: a report of 11 cases and review of the literature. 1670 83

We report a rare case of myeloid sarcoma (MS) of the extrahepatic bile ducts presenting as obstructive jaundice in a patient without leukemia at time of diagnosis. A 75-year-old female presented with a one-month history of abdominal pain and jaundice. Computerized tomography scan of the abdomen showed stenosis of the extrahepatic bile ducts. Endoscopic retrograde cholangiography disclosed an irregular narrowing of the common biliary duct, suggestive of a cholangiocarcinoma, and resection was performed. Histologic examination showed diffuse transmural infiltration of malignant cells. These cells exhibited medium-sized round nuclei with central nucleoli and eosinophilic cytoplasm, and were strongly positive for myeloperoxidase, CD68, lysozyme, CD45, CD117 (c-kit protein) and CD43. Eight months following surgery the patient presented with multiple cutaneous nodules and bone marrow trephine biopsy showed acute myelomonocytic leukemia. A literature search identified two previously reported cases of MS of the extrahepatic biliary duct. MS should be taken into consideration in the differential diagnosis of a patient with obstructive jaundice. Immunohistochemistry is essential for a correct diagnosis.
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PMID:Myeloid sarcoma of the extrahepatic bile ducts presenting as obstructive jaundice. 1694 23

A 12-year-old Persian cat was examined for a firm swelling of the right tarsal region and enlargement of the corresponding right popliteal lymph node. Cytologic evaluation demonstrated a population of malignant cells consistent with large cell lymphoma. Necropsy revealed a multi-lobulated subcutaneous mass involving the tarsus with some extension into adjacent deep muscular tissue. Histologically, the mass was composed of round cells with eosinophilic cytoplasm and pleomorphic anisokaryotic nuclei. Evidence of articular and nodal infiltration by these cells was observed. Differential diagnoses included synovial sarcoma and histiocytic sarcoma. Neoplastic cells were negative for cytokeratin, CD79a, and CD3 and positive for CD18, vimentin, lysozyme, and alpha-1-antitrypsin, most consistent with a diagnosis of histiocytic sarcoma. This is the first report of a histiocytic sarcoma involving a joint of a cat. The final diagnosis was based on the light microscopic appearance in combination with the immunohistochemical stains.
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PMID:Histiocytic sarcoma in the tarsus of a cat. 1709 63

Thirty-two cases of granulocytic sarcoma (GS) are reported in this paper. Age range was from 16 - 70 years. GS was accompanied by AML in 13 cases, ALL (My+) in one case, CML in 11 cases and MDS in two cases. GS was diagnosed simultaneously with leukemia in five cases and preceded the leukemia in eight. Lymph node and soft tissue were the most commonly detected localizations. Seven cases had first been diagnosed as NHL. Histopathologically blastic, immature and mature variants were found in 11, nine and 11 cases respectively and overall survival was shortest in the blastic type. Myeloperoxidase and lysozyme were found to be positive in 30 and 24 cases respectively. Therapy was radiation in five cases and surgery in three. Systemic chemotherapy was given to the cases. The clinical outcome of the patients after the diagnosis of GS was poor. GS is a unique entity; prognosis is poor but it is important to detect the signaling pathways associated with migration of myeloid cells to the extra-medullary tissues. The critical factors for detecting this interesting tumor are to be aware of this disease, cooperation between clinician and pathologist and the application of special stains to detect the myeloid origin.
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PMID:Granulocytic sarcoma: 32 cases and review of the literature. 1716 97

Seven novel cell lines from canine histiocytic sarcoma (HS), three of which were disseminated cutaneous HS and four of which were synovial HS, were established. All of the established cell lines had the same morphological (by light and electron microscopic findings), cytochemical (alpha-naphthyl butyrate esterase-positive), and immunohistochemical (vimentin- and lysozyme-positive, and cyto-keratin-negative) characteristics as the original HS tumor cells. All of the established cell lines injected into nude mice subcutaneously produced solid tumors. Because the established cell lines also showed phagocytic and processing activities, the HS tumor cells appear to originate from the mononuclear phagocytic system cells, despite their differences in locations or organs.
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PMID:Establishment and biological characterization of canine histiocytic sarcoma cell lines. 1721 6

Histiocytic sarcoma is an uncommon neoplasm of mature histiocytes with a poor clinical outcome. We report a case of a true histiocytic sarcoma with prominent and evenly distributed multinucleated giant cells that mimics a giant cell tumor of soft tissue. The tumor was located between the appendix, right ovary, and the terminal ileum with severe adhesion. The liver and spleen were not enlarged. Grossly, the tumor appeared grayish white, solid, and soft. Microscopically, polygonal mononuclear tumor cells aggregated to form somewhat epithelioid nests, which occasionally showed coagulative necrosis. Prominent and evenly scattered giant cells were present in all sections. In addition, tumor cell infiltration was noted in regional lymph nodes. The tumor cells were positive for lysozyme, CD68, CD163, and negative for T- and B-cell lineage markers, follicular dendritic cell, megakaryocytic, epithelial, muscular, and melanocytic markers, CD1a and CD30. This case posed great difficulty in clinical and pathological diagnoses. Gross pictures, microscopic findings, and extensive immunostains are important for the differential diagnosis.
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PMID:Histiocytic sarcoma - a case with evenly distributed multinucleated giant cells. 1767 73

A 4-year-old neutered male Golden Retriever was presented to the oncology service of the North Carolina State University Veterinary Teaching Hospital for staging of a histiocytic sarcoma of the left forelimb, diagnosed on the basis of biopsies submitted by the referring veterinarian. Cytologic assessment of aspirates of 2 splenic nodules identified on ultrasonographic examination of the abdomen revealed a highly phagocytic population of neoplastic round cells morphologically suggestive of plasma cells. Histologic assessment of the forelimb mass after amputation of the limb revealed a neoplastic round cell population demonstrating extensive cytophagia and erythrophagia. Immunohistochemical analysis of the tumor population revealed it to be negative for BLA.36 with sporadic positivity for lysozyme and CD79a. Immunofluorescent evaluation revealed weak tumor cell positivity for immunoglobulin (Ig) A and IgM, but extensive strong positivity for IgG, confirming the plasma cell origin of the tumor. Although extensive phagocytic activity may strongly suggest histiocytic origin, plasma cell origin must also be considered among the differential diagnoses for phagocytic round cell tumors.
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PMID:Phagocytic plasmacytoma in a dog. 1780 81

Histiocytic sarcoma is the most frequent hematopoietic tumor in rats. We report here a histiocytic sarcoma infiltrating the liver, the spleen and the pancreas from a Wistar rat. In the liver, the tumor was associated with oval cell and bile duct hyperplasia. The cells looked like neoplastic histocytic cells described in this species but with some particularities (e.g. lack of multinucleated giant cells). At immunohistochemistry, neoplastic cells in the liver were vimentine positive but lysozyme and CD68 negative. In the kidney, lysozyme-positive cytoplasmic droplets were observed. We describe here an atypical case of histiocytic sarcoma in the rat and we compare the nature of these neoplastic cells to other species.
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PMID:An atypical case of histiocytic sarcoma in a Wistar rat (Rattus norvegicus). 1822 80

We describe a 56-year-old woman with histiocytic sarcoma involving the bone marrow. The neoplastic cells proliferated diffusely and showed occasional erythrophagocytosis. Immunohistochemically, the neoplastic cells were positive for CD68, lysozyme, CD4 and CD163, but negative for B- and T-cell markers, S100 protein and epithelial markers. The patient received multi-agent chemotherapy and is living at 22 months after diagnosis without recurrence. Histiocytic sarcoma is an exceedingly rare hematopoietic neoplasm and the prognosis is poor due to its rapid progression, widespread disease and poor response to therapy. It is important to recognize this rare neoplasm and to confirm the diagnosis using specific immunohistochemical markers.
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PMID:Histiocytic sarcoma: identification of its histiocytic origin using immunohistochemistry. 1823 26


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