Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:Q16637 (SMA)
 8,107 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report on a case of a sarcoma arising in the hyaline-vascular variant of Castleman disease (HVCD) of the skin and subcutis. The patient was a 38-year-old man who clinically presented with a subcutaneous non-fixed cyst-like mass on his right shoulder with an unremarkable prior medical history. Histologic sections showed a biphasic tumor with numerous atretic lymphoid follicles located in the deep dermis and subcutis and a spindle-cell neoplasm mainly situated in the deep subcutis and adjacent soft tissue. The atretic lymphoid component fulfilled the criteria for HVCD, whereas the spindle-cell lesion showed all the criteria for sarcoma including nuclear atypia and frequent mitotic figures. The sarcomatous component was diffusely positive for fascin, nerve growth factor receptor, and CD34 with focal weak reactivity for CD21 and CNA.42. Stains for CD23, CD31, CD35, CD99, ALK-1, SMA, ASMA, desmin, factor XIIIa, AE1-AE3, EMA, bcl-2, S-100, Melan-A, HMB-45, Cam 5.2, and factor VIII were negative in the neoplastic spindle cells. No monoclonal population of lymphocytes was detected and we could not identify EBV or HHV-8 virus by PCR. Electron microscopy of the sarcomatous component showed spindle cells with labyrinth-like invaginations of the nucleus and numerous long, slender, interwoven cytoplasmic processes. The sarcomatous component in this case is most consistent with a poorly differentiated follicular dendritic cell sarcoma based upon the morphologic and ultrastructural findings.
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PMID:Sarcoma arising in hyaline-vascular castleman disease of skin and subcutis. 1612 Oct 55

There is reliable information about how changes in spleen histology are influenced by the relationship among B and T lymphocytes, macrophages, dendritic cells and myofibroblasts. Moreover, if it can be applied in the day-by-day pathology laboratory. This work intends to elucidate morpho-functional aspects of relationships of these cells in the different spleen compartments, how they are influenced by pathological conditions and how basic immunohistochemical techniques could optimize the histopathological diagnosis. We analyzed the usefulness of the monoclonal antibodies CD45RO, CD20, CD21, CD35, CD68, caldesmon, the smooth muscle alpha-actin type 1 (SMA-1) in 91 specimens. CD21(+) CD35(+) follicular dendritic cells were organized into three patterns in agreement with the immune condition of the lymphoid follicle. Smooth muscle alpha-actin type 1(+)and caldesmon(+)myofibroblasts draw two double rings: marginal-perifollicular and germinal-marginal. The latter is closely related to T-cells. CD68(+)red pulp macrophages had clear and linear configuration. The interruption of this CD68(+) linear pattern in splenic marginal zone lymphoma cases could be a criterion to differentiate it from reactive hyperplasia. CD45RO, CD20, CD21, CD68 and SMA-1 provide a basic and quality immunohistochemical battery for a better comprehension of the human spleen and could improve its histopathological diagnosis.
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PMID:Immunohistochemical organization patterns of the follicular dendritic cells, myofibroblasts and macrophages in the human spleen--new considerations on the pathological diagnosis of splenectomy pieces. 2012 87

Follicular dendritic cell sarcoma (FDCS) can be divided into the conventional type, and the inflammatory pseudotumor (IPT)-like variant type. Epstein-Barr virus (EBV) infection is considered to be closely associated with the pathogenesis of IPT-like variant of FDCS. Hepatic FDCS has an exceedingly low incidence of only 29 cases reported, with most of these tumors being classified as the IPT-like type. We report a case of an IPT-like variant of FDCS of the liver in a 61-year old man who presented with no marked symptoms. The patient underwent laparoscopic surgery for the mass and was well during a 13-month follow-up periods. The postoperative pathological examination found a proliferation of spindle cells and a diffuse infiltration of inflammatory cells within the tumor. Immunohistochemistry revealed that neoplastic cells were positive for CD23, clusterin, fascin, and PD-L1, and weakly positive for CD35, SMA, and D2-40. The infiltrating lymphocytes were strongly positive for PD1, and IgG4-positive plasma cells were less than 10 cells/high-power field. In situ hybridization for Epstein-Barr virus encoded RNA (EBER) was negative. To our knowledge, the present case is the second case of hepatic IPT-like variant of FDCS without EBV involvement, indicating that EBV infection is not an absolute prerequisite for a diagnosis of the IPT-like variant of FDCS.
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PMID:Epstein-Barr virus-negative inflammatory pseudotumor-like variant of follicular dendritic cell sarcoma of the liver: A case report and literature review. 3254 Jan 41