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)

Little is known about the frequency, type and pathogenesis of fibrotic changes that may occur in the pancreas of persons without any clinically apparent or macroscopically visible pancreatic disease. We screened pancreas specimens for the presence and pattern of fibrosis, determined the relationship between fibrosis, age, and duct lesions, and studied the fibrogenic mechanisms. In 89 postmortem specimens from persons without any known pancreatic disease (age range 20-86 years), fibrosis was recorded and graded and the patients were divided into two age classes (younger or older than 60 years). In addition, we analyzed the association between ductal papillary hyperplasia [i.e., pancreatic intraepithelial neoplasia type 1B (PanIN-1B)] and fibrotic foci in the pancreatic tissue to determine the potential impact of obliterating duct lesions on pancreatic fibrosis. Finally, we studied the occurrence in the pancreas of myofibroblasts, identified on the basis of their alpha-SMA and desmin positivity, and determined their relationship to the fibrotic foci. Thirty-eight (44%) of 89 pancreata showed scattered foci of lobular fibrosis affecting peripheral lobuli. Fibrotic changes were significantly more common in individuals older than 60 years. Fibrotic foci were commonly associated (p<0.05) with ductal papillary hyperplasia in ducts draining fibrotic lobuli. Myofibroblasts were detected in the fibrotic foci. The "normal" pancreas develops a specific type of focally accentuated fibrosis that is highly age related. This patchy lobular fibrosis in the elderly (PLFE) was closely associated with PanIN-1B lesions in the ducts, suggesting that the narrowing of a duct due to papillary hyperplasia of the epithelium may hamper secretion and cause fibrosis of the drained lobule. The presence of myofibroblasts in association with the fibrotic foci indicates an ongoing fibrogenic process.
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PMID:Pancreatic fibrosis associated with age and ductal papillary hyperplasia. 1602 8

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

In this study, we aimed to investigate the effect of vitamin A on the transformation of the Ito cells to fibrogenic form and suppression of the development of fibrosis. Carbon tetrachloride intoxication was performed on rats for 2, 8, 12 or 20 weeks and 5x10(4) IU vitamin A (as retinol palmitate) was injected subcutaneously once every 4 weeks. Ito cells were detected by gold chloride impregnation, as well as desmin and alpha-smooth muscle actin (alpha-SMA) immunohistochemistry. Additionally, all groups were examined ultrastructurally. The number of Ito cells that were labelled positively with gold impregnation decreased in the fibrotic groups; however, alpha-SMA and desmin immunopositive Ito cells increased. The samples from animals that were treated with vitamin A showed an increase in labelling with gold impregnation but a decrease in alpha-SMA immunopositivity. The data showed that vitamin A can prevent hepatic injury, by suppressing the transformation of Ito cells to fibrogenic form. We conclude that vitamin A has potential for the treatment of hepatic fibrotic diseases. Alpha-SMA immunohistochemistry was found to be more informative than desmin immunohistochemistry for monitoring liver fibrosis.
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PMID:The effect of vitamin A on CCl4-induced hepatic injuries in rats: a histochemical, immunohistochemical and ultrastructural study. 1625 75

Histologic and immunohistochemical studies (use of antibodies for viruses of herpes simplex type 1 and 2, desmin, vimentin, SMA as well as polymerase chain reaction to DNA of viruses of herpes simplex) were made on the material of the valves taken from 1326 patients with valvular heart disease, the ascending aorta of 30 patients with aneurysm, valves of 35 deceased patients without cardiovascular pathology. As a result, expression of viruses of herpes simplex type 1 and/or 2 was found in all cases with mesenchymal dysplasia and cystic medianecrosis in endotheliocytes, fibroblasts, smooth muscle cells of valves and aorta. This indicates the role of these viruses in the pathogenesis of these diseases and their common etiology.
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PMID:[Mesenchymal dysplasia of heart valves, cystic medianecrosis of the aorta and herpetic infection]. 1632 74

Myofibroblasts play an important role in chronic renal interstitial fibrosis. However, the origin and developmental mechanisms remain to be elucidated. The myofibroblasts may express various cytoskeletons during the development. Immunoexpressions of vimentin, desmin and alpha-smooth muscle actin (alpha-SMA) were analyzed using experimentally (cisplatin and unilateral ureteral obstruction) induced rat and spontaneous canine fibrotic kidneys or kidney-related cell lines incubated with transforming growth factor-beta1 (TGF-beta1), platelet-derived growth factor-BB (PDGF-BB) or their combination at various concentrations. In rat fibrotic kidneys, both renal epithelia and interstitial cells showed positive reactions to alpha-SMA and vimentin, supporting epithelial-mesenchymal transition (EMT) theory; however, renal epithelia did not react to desmin, though interstitial cells were reactive. Renal epithelia in canine fibrotic kidneys did not show a positive reaction to alpha-SMA, whereas interstitial cells reacted strongly to alpha-SMA; conversely, renal epithelia reacted strongly to desmin, but interstitial cells did not; vimentin expression was infrequently seen in renal epithelia and interstitial cells of canine kidneys. Exposure of TGF-beta1 to porcine renal epithelial cells (LLC-PK1), rat renal interstitial cells (NRK-49F), and rat immature mesenchymal cells (MT-9) dose-dependently increased selectively alpha-SMA-positive cell numbers. Moreover, PDGF-BB exhibited an additive effect on TGF-beta1-induced alpha-SMA expression in these cell lines when simultaneously added. alpha-SMA was the most plastic cytoskeleton under fibrogenic stimuli. This study shows that there are interspecies differences in cytoskeletal immunoexpressions of renal epithelia or interstitial cells between rat and canine fibrotic kidneys, and that the derivation of renal myofibroblasts may be heterogeneous, such as renal epithelia, interstitial cells or immature mesenchymal cells.
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PMID:Differential immunoexpressions of cytoskeletons in renal epithelial and interstitial cells in rat and canine fibrotic kidneys, and in kidney-related cell lines under fibrogenic stimuli. 1632 24

An 83-year-old woman was admitted to our hospital because of colicky pain under the right costal arch suggesting cholecystitis. Physical examination confirmed by ultrasound scan indicated a palpable tumor in that location. Laparotomy was performed and the tumor was removed. Histopathological examination revealed malignant peripheral nerve sheath tumor (MPNST) originating in neurofibroma of the mesentery. Macroscopic, histological and cytological features were typical for MPNST. High nuclear pleomorphism, hyperchromasia were observed but on average only two mitotic figures per high power field were seen. The growth fraction determined by Ki-67 immunoreactivity was about 30%. Immunohistochemical stains revealed positivity of individual cells for NK-1(CD57), S-100 protein and NSE. It was lack of positivity for cytokeratin, EMA, vimentin, desmin, SMA, CD34. We report a well documented case of MPNST originating in preexisting neurofibroma of the mesentery. To our knowledge, is the first case in the Polish literature.
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PMID:Malignant peripheral nerve sheath tumor originating in neurofibroma of the mesentery. Case report. 1633 83

Canine livers with congenital portosystemic shunt were investigated histopathologically and immunohistochemically before and 8-272 days after partial ligation of the shunt. Lesions included hypoplasia of portal veins, arteriolar and ductular proliferation, lymphangiectasis, mild to moderate fibrosis, fatty cysts, and mostly mild hepatocellular damage with frequent atrophy and steatosis, regardless of the location of the shunting vessel. Perisinusoidal hepatic stellate cells (HSCs) in normal canine liver expressed alpha-smooth muscle actin (alpha-SMA), but no desmin. In altered livers, however, raised expression of alpha-SMA was detected, together with expression of desmin, in varying numbers of HSCs. This was interpreted as a sign of cellular proliferation and transformation to myofibroblast-like cells. Additionally, there was an obvious perisinusoidal increase of several extracellular matrix components. Postoperative biopsy samples showed basically the same lesions as those of pre-operative samples, except that signs of resolution of hepatic changes were apparent.
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PMID:Histopathological and immunohistochemical investigations of hepatic lesions associated with congenital portosystemic shunt in dogs. 1642 74

Nodular fasciitis is a benign myofibroblastic proliferation with a predilection for the subcutaneous tissues of the upper extremities, trunk, and head and neck of young adults. Nodular fasciitis is not generally recognized to arise within joints. In this study, the clinicopathologic and immunohistochemical features of 10 cases of intraarticular nodular fasciitis are described. Six patients were female and 4 were male, with a median age of 33 years (range, 9-50 years). Lesional size ranged from 2 to 4 cm (median, 2.6 cm). Seven tumors arose in the knee, 2 in the hand, and 1 in the ankle. Most patients complained of joint pain; 4 presented with a palpable mass. Only 1 patient reported antecedent trauma. The duration of symptoms prior to surgery ranged from 2 months to 1 year (median, 6 months). The clinical differential diagnoses included giant cell tumor of tendon sheath, pigmented villonodular synovitis, synovial chondromatosis, inflammatory arthritis, and lymphoma. Grossly, the lesions were solid, nodular, rubbery, or firm masses. Histologically, all tumors were circumscribed but unencapsulated and showed typical features of nodular fasciitis, being composed of cytologically bland plump spindle cells arranged in short, intersecting bundles within a variably loose myxoid to collagenous stroma, containing extravasated red blood cells and scattered lymphocytes. Five lesions showed prominent stromal hyalinization, in 2 cases keloidal in appearance. In 4 cases, the tissue at the periphery of the lesion showed hemosiderin deposition. By immunohistochemistry, all tumors examined were positive for SMA, 1 was positive for desmin, and all were negative for caldesmon and S-100 protein; none showed nuclear staining for beta-catenin. Clinical follow-up information was available for 5 patients, ranging from 2 to 86 months. No lesion recurred. In summary, intraarticular nodular fasciitis occurs most commonly in the knees of young adults, and often appears to have a somewhat longer preoperative duration than typical subcutaneous or intramuscular nodular fasciitis. Intraarticular lesions show morphologic features similar to other cases of nodular fasciitis, with the exception that stromal hyalinization and adjacent hemosiderin deposition are common, likely attributable to frictional trauma in this location.
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PMID:Intraarticular nodular fasciitis--a rare lesion: clinicopathologic analysis of a series. 1643 99

Mucinous cystic neoplasms (MCN) of the pancreas are mucin-producing cystic tumors with an ovarian-like stroma (OLS). In the present study MCN were obtained from 27 patients. These MCN were derived from 22 pancreas, three livers, spleen, and mesentery. MCN in various organs have common clinicopathological profiles, being unilocular or multilocular cystic tumors, with a fibrous capsule and lined by mucin-secreting epithelium associated with an underlying subepithelial OLS. The OLS showed strong positivity for alpha-smooth muscle actin (alpha-SMA) and vimentin and weak, focal positivity for desmin. Both estrogen receptors and progesterone receptors were expressed in the nuclei of OLS cells. In addition, 20 ovarian MCN and 13 normal ovaries were studied with particular attention to the stroma. The stroma of ovarian MCN was strongly immunopositive for alpha-SMA and vimentin and focally positive for desmin, whereas normal ovarian stroma was immunonegative for both alpha-SMA and desmin. The OLS of MCN mentioned here was similar to the septa of ovarian MCN but not to ovarian stroma. In conclusion, MCN in various organs should be lumped together as 'extra ovarian' MCN. The OLS was identified on the basis of myofibroblastic proliferation both in response to neoplastic development and dependent on hormones.
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PMID:Pancreatic, hepatic, splenic, and mesenteric mucinous cystic neoplasms (MCN) are lumped together as extra ovarian MCN. 1644 18

We present an extensive immunohistochemical analysis of 7 mammary sarcomas that did not fit into any specific soft tissue sarcoma category. Histologically, they were composed of spindle cells with highly pleomorphic nuclei and abundant mitoses. Our immunohistochemical antibody panel included pan-cytokeratin (CK), basal cell type CKs (34betaE12, CK5/6, CK14, CK17) and vimentin antibodies, antibodies to established (SMA, CD10, p63, S-100, maspin, calponin, GFAP, SM-myosin), and novel (CD29, 14-3-3sigma) myoepithelial markers, as well as antibodies to CD34, desmin, h-caldesmon, steroid receptors (estrogen, progesterone, androgen), and EGFR (Her-1). Whereas CKs, CD34, desmin, and h-caldesmon were not expressed, all tumors were positive for CD10 and vimentin. CD29 and SMA were observed in 3 cases each (43%), and p63 and calponin in 2 cases each (29%). Other myoepithelial markers and steroid receptors were absent, except androgen receptors, which were expressed in one sarcoma. Five sarcomas showed positivity for EGFR. The distinction of specific, histogenetically defined sarcoma entities (such as leiomyosarcoma, angiosarcoma, liposarcoma) from NOS-type sarcoma with CD10 expression is usually clear-cut because the former exhibit a characteristic histomorphology and immunoprofile. Phyllodes tumors with stromal overgrowth or recurrent phyllodes tumors lacking epithelial structures as well as periductal stromal sarcomas can be ruled out by their frequent expression of CD34 and negativity for myoepithelial markers. The most important differential diagnosis is sarcomatoid metaplastic carcinoma because its treatment includes axillary lymphadenectomy. Since some NOS-type sarcomas with CD10 expression and most metaplastic carcinomas show positivity for CD29, SMA, and p63, differential diagnosis can be extremely difficult and requires extensive immunohistochemical evaluation for CKs and additional myoepithelial markers such as S-100, 14-3-3sigma, and maspin. The immunophenotype of NOS-type sarcomas with CD10 expression suggests that these neoplasms represent a mammary sarcoma variant with myoepithelial features.
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PMID:Mammary NOS-type sarcoma with CD10 expression: a rare entity with features of myoepithelial differentiation. 1662 90


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