Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
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.
 ...
PMID:Sarcoma arising in hyaline-vascular castleman disease of skin and subcutis. 1612 Oct 55

Vascular benign lesions are the most common non-hematological splenic primary tumors. Although rare they may sometimes pose problems in differential diagnosis, because of their morphologic heterogeneity. We present vascular lesions of the spleen, which were found in archive of the Chair of Pathomorphology. Immunohistochemistry including CD34, CD31, factor VIII, CD8, CD21, CD68, lysosyme, GLUT-1, D2-40, VEGFR3, SMA, Ki67 were performed. 8 benign vascular lesions were identified, including two hamartomas, two lesions representing sclerosing angiomatoid nodular transformation (SANT) and four hemangiomas. We present briefly the spectrum of vascular lesions occurring in the spleen and discuss differential diagnosis and nosological status of selected lesions.
 ...
PMID:From hamartoma to splenic hemangioma. 1865 69

Sclerosing angiomatoid nodular transformation (SANT) of the spleen is a rare inflammatory tumor-like lesion composed of vascular nodules and non-neoplastic stroma including spindle cells and inflammatory cells. The focus of our study was on the stromal proliferating process in SANT. Nine cases of SANT were examined. All cases showed alpha-smooth muscle actin (alpha-SMA) and vimentin on the spindle cells but not CD21, CD31, CD34, CD68, desmin, S100, human herpes virus-8, or anaplastic lymphoma kinase-1. In one case, 20-30% of the myofibroblasts in Epstein-Barr-virus (EBV)-positive spindle cells were detected using double-labeling immunohistochemistry for alpha-SMA and EBV-encoded small RNA in situ hybridization. A quantitative analysis of IgG and IgG4-positive plasma cells (pPCs) in SANT was performed. The median densities of IgG-pPCs and IgG4-pPCs in SANT were approximately four-fold and 13-fold higher than those in the normal spleens, respectively. In addition, there was a statistically significant increase of IgG4/IgG-pPCs ratio in SANT in comparison to the control specimens. In conclusion, the fibrogenesis in a subset of SANT may be associated with EBV-infected myofibroblasts in an overlapping immune reaction indicated by the presence of infiltrating IgG4-pPCs. Further investigation is needed to elucidate the association between SANT and IgG4-related sclerosing disease.
 ...
PMID:Detection of Epstein-Barr virus-encoded small RNA-expressed myofibroblasts and IgG4-producing plasma cells in sclerosing angiomatoid nodular transformation of the spleen. 1869 8

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.
 ...
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

In this study, we investigate the hypothesis that IgG4-related autoimmune reaction is involved in the formation of inflammatory aortic aneurysms (IAA). We obtained 23 cases of IAA and 11 cases of atherosclerotic aortic aneurysms (AAA) as control group. We evaluated the expression of IgG4 in both IAA study cases and AAA control cases. In addition, immunohistochemical expression of C-Kit, CD21, CD34, S-100 protein, SMA, vimentin, p53, beta-catenin, and ALK-1, and EBV-LMP1 expression by in situ hybridization were performed only in IAA cases. Of the 23 patients, 20 were males and 3 were females (M: F ratio 6.7:1); age ranged from 43 to 81 years (average 64.3 years). Histologically, all 23 cases of IAA formed a mass that displayed inflammatory myofibroblastic tumor-like features. All lesions stained strongly and diffusely for vimentin and SMA (100%); 17 stained strongly and focally for CD34 (74%); and all were negative for C-Kit, CD21, S-100 protein, p53, beta-catenin, EBV-LMP1, and ALK-1. The numbers of infiltrating IgG4-positive plasma cells in IAA cases exceed that of AAA cases. Score 3 (>50 plasma cells/one 40X field) of IgG4-positive plasma cells was only seen in IAA cases (13/23, 57%), whereas none of the 11 cases of AAA showed score 3 IgG4-positive plasma cells (P=0.0018, Fischer's exact test). Our findings suggest that IAA may be an aortic manifestation of the IgG4-related sclerosing disease. The high number of positive plasma cells, >50 plasma cells/one 40X field is more specific for the IAA than for AAA; however, lesser number can be seen in both IAA and AAA patients.
 ...
PMID:Inflammatory aortic aneurysm: possible manifestation of IgG4-related sclerosing disease. 2341 50

We present the case of a 62-year-old male patient with a three-month history of pain in the left shoulder. Magnetic resonance imaging of the left scapula showed an osteo-destructive lesion. H and E stained sections revealed a Langerhans cell sarcoma, and immunohistochemistry was performed additionally; CD68, CD163, CD14, fascin, HLA-DR, lysozyme, S100 CD1a and langerin showed a positive reaction, while CD20, CD30, CD34, CD31, pan-cytokeratin, AE/1AE3, SMA, desmin, EMA, ERG, INI-1, CD21, CD4, PLAP, MPO and CD117c were negative. We suggested palliative treatment with chemotherapy and radiation. The patient refused any treatment and died 2 weeks later.
...
PMID:Langerhans cell sarcoma: a case report and review of the literature. 2754 73