Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:3.4.24.11 (CD10)
 9,792 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Perivascular epithelioid cell tumor (PEComa) of the laryngopharynx is extremely rare, and no such descriptions have been reported in the literature. This study describes the clinical, histological, and immunohistochemical features of three cases of laryngopharynx PEComa. The patients (two males and one female) were 38, 42, and 47 years old, respectively. Their tumors were well-demarcated, gray white on the cut surface with focal hemorrhage, and ranged from 3.0 to 5.0cm in diameter. Microscopically, the tumors were characterized by an epithelioid arrangement of tumor cells, which had abundant clear to pale eosinophilic granular cytoplasm, vesicular nuclei, and prominent nucleoli. The stroma was rich in capillaries, sinusoidal vasculature, and thick-walled blood vessels. Mitotic figures were rare. The tumors showed no cellular atypia, with two of them having moderate atypical nuclei focally. Immunohistochemically, the tumor cells were diffusely or focally positive for vimentin (3/3), HMB-45 (3/3), melan-A(3/3), alpha-smooth muscle actin (3/3), and S-100(1/3). They were uniformly negative for CK, Syn, CgA, CD34, CD10, and CD117. Two patients are well, with no evidence of disease at 13 and 30 months after surgery, one patient had recurrence at 15 months after operation. Laryngopharynx PEComas should be regarded as tumors with a malignant potential.
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PMID:Perivascular epithelioid cell tumors of the laryngopharynx: three case reports and literature review. 1940 23

A new myeloid leukemia cell line (CG-SH) with normal cytogenetics was established from a patient with acute myelogenous leukemia (AML) following myelodysplastic syndrome (MDS). The cells of CG-SH are immature blasts and have an immature myeloid phenotype (positive for myeloperoxidase, CD7, CD34, CD38, CD117, HLA-DR, negative for CD10, CD19, CD20, CD41, CD42). A partial expression of CD13, CD15, CD65 and a weak expression of CD33 and CD133 was noted. The cells are negative for EBER. By molecular analysis, a mutation of NRAS and heterozygous mutations of RUNX1 were detected. No mutations were detected in FLT3-ITD, MLL-PTD or NPM1. By real-time PCR, a series of 19 microRNAs was identified which are strongly expressed in CG-SH. In conclusion, a new cell line was established which will be useful for the study of AML with normal cytogenetics and mutations in NRAS and/or RUNX1.
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PMID:Characterization of a new myeloid leukemia cell line with normal cytogenetics (CG-SH). 1941 91

Presence of cytoplasmic granules in the blasts is a well known feature of myeloid leukemia. ALL presenting with the numerous cytoplasmic granules in blasts is a rarity and may be misdiagnosed as acute myeloid leukemia. We describe a rare case of hypergranular precursor B-cell acute lymphoblastic leukemia (ALL) in an adolescent male expressing CD10, CD19, CytoCD22, CD34, as well as CD13 and CD117. The blasts were cytochemically negative for myeloperoxidase (MPO), and acid phosphatase (ACP) but were positive for non-specific esterase (NSE). In centers where immunophenotypic panel is usually decided on the basis of morphology with limited antibodies may result in an erroneous typing of such rare diseases. Hence it is important to be aware of this rare entity and to confirm the lineage of acute leukemia by using a comprehensive panel of antibodies for immunophenotypic analysis.
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PMID:Hypergranular precursor B-cell acute lymphoblastic leukemia in a 16-year-old boy. 1967 81

Chromophobe renal cell carcinoma (ChRCC) is a subtype of renal cell carcinoma (RCC). ChRCC is diagnosed mainly in 6th decade of life. An incidence of ChRCC is similar in both men and woman. Eighty six percent of ChRCCs cases are diagnosed in stage 1 or 2. Prognosis of ChRCC is better than in other types of RCC. Five- and 10-year disease free survival (DFS) for ChRCC was 83.9% and 77.9%, respectively. Expression of immunohistological markers: cytokeratins (CK), vimentin, epithelial membrane antigen (EMA), CD10 could be potentially helpful in diagnosis of different subtypes of RCC. From all conventional RCC, CD 117 was detected (overexpression) in membrane of cells ChRCC.Overexpression of CD117 on cellular membranes of ChRCC could be a potential target for kinase inhibitors like: imatinib, dasatinib, nilotinib. The potential targets for other kinase inhibitors (sunitinib and sorafenib) in ChRCC seem to be VEGFR and PDGFR. On the basis for formulating research hypotheses which should be verified by prospective studies.
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PMID:Chromophobe renal cell cancer--review of the literature and potential methods of treating metastatic disease. 1981 59

The thymic type of precursor T-cell acute lymphoblastic lymphoma (pre-T ALL), an uncommon T-cell malignancy, typically presents as a thymic mass and expresses terminal deoxonucleotidyl transferase, CD7, and cytoplasmic CD3, with variable expression of other markers. Cutaneous presentation in pre-T ALL is highly unusual. We describe a case of pre-T ALL presenting as 2 papulonodular lesions on the face of an otherwise asymptomatic 27-year-old man. Microscopic examination of both lesions revealed a moderate to dense pandermal infiltrate of medium-sized lymphocytes with extensive "crush" artifact, whereas immunohistochemistry revealed positive staining of lesional cells for CD45, CD3, Bcl-2, Ki-67, CD5, CD7, and CD34 but negative staining for CD4, CD8, CD30, CD56, CD10, CD117, anaplastic lymphoma kinase protein, TdT, myeloperoxidase, CD79a, and CD20. Gene rearrangement studies performed on both biopsies identified a clonal population of T lymphocytes. A subsequent computed tomography scan revealed a 9-cm mediastinal mass encasing all major mediastinal vessels, whereas a bone marrow biopsy revealed blasts with an immunophenotype similar to that of the cutaneous lesions. Features unique to this case include the cutaneous presentation and the immunophenotype-absence of CD4, CD8, and TdT with expression of CD34-both highly unusual features for pre-T ALL.
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PMID:When dead cells tell tales-cutaneous involvement by precursor T-cell acute lymphoblastic lymphoma with an uncommon phenotype. 2001 Apr 5

Plasmablastic lymphoma (PBL) is an uncommon aggressive lymphoma arising most frequently in the oral cavity of HIV-infected patients. Rare cases of PBL have been reported in extraoral sites, particularly extranodal sites, as well as in immunocompetent patients. We report an unusual case of PBL in a 69-year-old, HIV-negative non-immunocompromised man presenting with generalized lymphadenopathy. To our knowledge, this is the first case of PBL presented as primarily generalized lymphadenopathy in HIV-negative patients. Histologic examinations of cervical, inguinal and axillary lymph nodes demonstrated a neoplastic proliferation of large cells with extensive necrosis. The neoplastic cells formed sheets with a relatively cohesive growth pattern interspersed by small lymphocytes and plasma cells. The large tumor cells expressed MUM1, OCT-2 and BOB.1, and were negative for CD138, CD38, AE1/AE3, melan A, PLAP, S100, vimentin, CD117, CD30, ALK-1, leukocyte common antigen (CD45), T-cell, B-cell and histolytic markers, CD56, CD10 and BCL-6. The proliferation index by Ki-67 immunohistochemistry was approaching 100%. In situ hybridization for Epstein-Barr Virus-encoded RNA (EBER) was positive in large malignant cells. A diagnosis of PBL was made. These findings indicate that PBL should be included in the differential diagnosis of an HIV-negative, immunocompetent patient with generalized lymphadenopathy. The adjacent plasma cells were positive for CD138 and CD38 and show kappa-light chain restriction, but without EBER expression, raising the possibility of a preexisting or concurrent plasmacytoma and that the PBL may be a high-grade transformation from a preexisting plasma cell neoplasm following Epstein-Barr virus infection. Electron microscopy showed numerous circumferential long slender peripheral cytoplasmic projections in the large tumor cells, suggesting that some of the previously reported large B-cell lymphoma with cytoplasmic projections may actually be PBL.
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PMID:Plasmablastic lymphoma may occur as a high-grade transformation from plasmacytoma. 2096 60

Solid-pseudopapillary neoplasms of the pancreas are tumors of low malignant potential whose histogenesis has been discussed controversially. In the series of 15 solid-pseudopapillary neoplasms presented here, we demonstrate that discovered on gastrointestinal stromal tumor 1 is expressed significantly by these tumors (diffuse expression in 8 cases, focal expression in 4 cases, and scarce expression in 3 cases). Similar to the high expression of CD117, this finding parallels the immunohistochemical findings in gastrointestinal stromal tumors. Using double immunohistochemistry and immunofluorescence, we furthermore show that centroacinar cells express discovered on gastrointestinal stromal tumors 1. Thus, our findings suggest that, similarly to CD10 or vimentin, the expression of discovered on gastrointestinal stromal tumors 1 may serve as a novel marker for centroacinar cells and for solid-pseudopapillary neoplasms, which is suggestive of a centroacinar origin of these neoplasms.
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PMID:Discovered on gastrointestinal stromal tumor 1 (DOG1) is expressed in pancreatic centroacinar cells and in solid-pseudopapillary neoplasms--novel evidence for a histogenetic relationship. 2129 18

The purpose of this study was to evaluate the use of a broad panel of antibodies used as diagnostic markers for abdominal mesenchymal tumors in uterine sarcomas. The expression of vimentin, AE1/AE3, smooth muscle actin (SMA), desmin , h-caldesmon, actin, Myf4, CD10, CD31, CD68, CD117, factor VIII, HMB-45, and S-100 protein was studied in 397 uterine sarcomas. SMA was positive in 90% of the ordinary leiomyosarcomas and when combined with desmin or h-caldesmon, a positivity of 96% and 92%, respectively, was achieved. Actin and Myf4 were positive in 4 of 5 rhabdomyosarcomas. Endometrial stromal sarcomas reacted positive with CD10 in 62 of 84 tumors and negative with h-caldesmon in 75 of 84 tumors. CD10 was the most frequent positive marker in adenosarcoma. Most tumor markers stained negative in undifferentiated uterine sarcoma, but 12 of 21 tumors reacted positive for SMA. A few focally HMB-45-positive cells were found within all tumor groups, except in rhabdomyosarcomas and giant cell tumors. Endothelial markers, S-100 protein, and CD117 do not seem to be of any diagnostic value in uterine sarcomas. In conclusion, when immunohistochemistry is used diagnostically in uterine sarcomas, a broad panel of markers provides better information than reliance on a single antibody.
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PMID:Diagnostic immunohistochemistry in uterine sarcomas: a study of 397 cases. 2146 30

This study was aimed to investigate the immunophenotyping characteristics of biphenotypic acute leukemia (BAL) and to analyze its curative efficacy. The four-color direct immunofluorescence staining and CD45/SSC gating analysis of flow cytometry were used to detect the cells of bone marrow and peripheral blood of 45 doubtful cases of BAL, and the immunophenotyping was performed according to the European Group for the Immunological Classification of Leukemia (EGIL) score criterion. The results showed that among 45 doubtful cases of BAL, the co-expression of cCD79a and cMPO was observed in 23 cases of My/B-ALL, with highest level of CD19 expression, followed by CD10; the co-expression of cCD3 and cMPO was observed in 18 cases of My/T-ALL, with highest level of CD7 expression, followed by CD5; the co-expression of cCD3 and cCD79a was found in 4 cases of T/B. Out of 45 cases of BAL, 30 cases showed cTdT expression, 25 cases showed CD34 expression, 31 cases showed CD117 expression. The results also indicated that the expression rate of CD33 was higher than that of CD13 in antigen expression of My/B-ALL and My/T-ALL patients. The complete remission (CR) rate of My/B-ALL with My/T-ALL CD34(+) was lower than that of My/B-ALL with CD34(-), the CR rate of My/B-ALL with CD34(+) was lower than that of My/T-ALL with CD34(-). It is concluded that the co-expression of My/B-ALL antigen has been found to be most common, then followed by My/T-All. cCD3, cCD79a, cMPO have important value for diagnosing and identifying of BAL. The flow cytometry with four-color direct immunofluorescence staining is most specific method for diagnosis of BAL. The clinical prognosis of BAL with low cell differentiation is poor.
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PMID:[Immunophenotyping characteristics of biphenotypic acute leukemia and analysis of curative effect]. 2151 79

We describe here a 62-year-old woman who presented with a perivascular epithelioid cell tumor arising in the sigmoid colon. Computed tomography revealed a 5-cm-sized intraluminal fungating mass. Histologically, the tumor consisted of plump, epithelioid cells with abundant clear-to-lightly eosinophilic cytoplasm and round nuclei, arranged in an alveolar or trabecular pattern. The tumor cells were strongly positive for HMB-45 and TFE3, but negative for vimentin, cytokeratin, smooth muscle actin, S100 protein, CD117, CD34, synaptophysin, chromogranin, CD10, hepatocyte antigen, CD1a, and desmin. The tumor cells had a high Ki-67 labeling index (up to 20%). Fluorescent in situ hybridization showed no evidence of the EWS rearrangement. Based on these histologic and immunohistochemical features, our patient was diagnosed with a perivascular epithelioid cell tumor of the sigmoid colon.
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PMID:Perivascular epithelioid cell tumor of the sigmoid colon with transcription factor E3 expression. 2154 94


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