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)

Myofibroblastic differentiation occurs in 10-20% of all dermatofibromas, affecting < 25% of cells. We report on a series of 36 dermatofibromas collected from > 1,500 fibrohistiocytic lesions (2%), with more prominent (> 25%) myofibroblastic differentiation characterized by markedly slender and elongated spindle cells positive for smooth muscle markers. While most of the lesions did not otherwise differ from ordinary dermatofibromas, three cases (0.2%) from the neck-shoulder region of male adults showed extensive myofibroblastic features (> 90%). Clinically, these three lesions measured approximately 1 cm and had a firm consistency, with the differential diagnosis of some fibrohistiocytic tissue response. Histologically, densely packed cells and prominent, partially nodular, stromal sclerosis with focal palisading of nuclei indicate some overlap with other rare variants of fibrohistiocytic tissue response, such as cellular benign and palisading cutaneous fibrous histiocytoma. Yet, these features together with focal whorled nesting of more epithelioid cells (one case) also caused considerable diagnostic problems to exclude other myofibroblastic as well as (malignant) spindle cell lesions such as (palisaded) myofibroblastoma, dermatofibrosarcoma protuberans, and neurothekeoma. Immunohistochemically, all lesions were markedly (> 90%) labeled for smooth muscle markers (HHF35, anti-SMA) and with NK1C3 (CD57), while a broad panel for other spindle cell tumors, such as pan-keratin, S100 protein, EMA, desmin, CD34, CD31, and KiM1p, were negative. Electron microscopy of two cases revealed prominent endoplasmic reticulum and Golgi complex, numerous intermediate filaments, attachment plaques, and focal basal lamina formation. No recurrence was seen during a follow-up of 9 (two cases) and two years, respectively.
 ...
PMID:Prominent myofibroblastic differentiation. A pitfall in the diagnosis of dermatofibroma. 912 98

We examined 33 successive pulmonary biopsy specimens from nine patients who underwent pulmonary transplantation; they were compared to "normal" lung tissue selected from routine transbronchial biopsy material and also to surgical biopsy specimens from a previously reported study. We used as controls the three successive biopsy specimens from Patient 7, in whom rejection reaction did not develop, and the two specimens from Patient 6, who had pneumonia caused by cytomegalovirus. The material was embedded in paraffin, and the sections were stained by hematoxylin and eosin, periodic acid-Schiff, Masson's trichrome, and Gomori's silver stains. They were also immunostained for alpha-smooth muscle actin (alpha-SMA), desmin, transforming growth factor beta1, tumor necrosis factor alpha, keratin, CD3, CD20, CD68, and HLA-dr. In all of the lung tissue from patients in whom a rejection reaction developed, alveolar myofibroblasts (MFs) expressed alpha-SMA. Sometimes, alpha-SMA expression appeared before the classical manifestations of rejection reaction. MFs labeled by alpha-SMA antibody did not express desmin; alveolar septa containing alpha-SMA-positive cells were frequently lined by transforming growth factor beta1 and, occasionally, tumor necrosis factor alpha-laden Type II pneumocytes. In lung tissue (from successive biopsies) that did not show evidence of rejection reaction, alveolar MFs did not express alpha-SMA. Our findings demonstrate that modulation of alveolar MFs is one of the manifestations of rejection in transplanted lungs; furthermore, they suggest that alpha-SMA staining might be useful in predicting rejection reaction before the classical cellular events occur.
 ...
PMID:Phenotypic modulation of alveolar myofibroblasts in transplanted human lungs. 938 65

The purpose of this study was to elucidate the phenotypic conditions in the sella turcica/pituitary gland complex in human trisomy 18 fetuses. Fourteen human fetuses with gestational ages from 12 to 39 weeks were included in the study. Normal fetuses at corresponding ages were used as controls. Whole body and special radiographic examination was undertaken before the midsagittal cranial base block, including the pituitary gland, was excised and analyzed histologically and immunohistochemically (keratin wide spectrum [KWS], thyroid-stimulating hormone [TSH], and neurophysin [Nph]). In all trisomy 18 fetuses, TSH-positive adenopituitary tissue was present in the sella and in greater or lesser amounts pharyngeally. The neurohypophysis was Nph-positive and located normally in the sella turcica. The adenohypophyseal tissue reacted either KWS-faint or KWS-negative, whereas KWS-positive reaction occurs in normal fetuses. This circumstance might suggest an altered cytoskeletal structure of the surface ectoderm in the pituitary placode in trisomy 18. The sella turcica was malformed in all the fetuses. Very broad craniopharyngeal canals were observed in some of the fetuses. Because endocrine disorders occur in many congenital malformations, it is essential in future studies to chart the sella turcica/pituitary gland region systematically in different genotypes.
 ...
PMID:Pituitary gland and sella turcica in human trisomy 18 fetuses. 950 72

We describe an epithelioid leiomyosarcoma of bone located in the right knee of a 51-year-old woman. Plain radiograph and CT scan revealed a poorly defined lytic and destructive mass in the upper metaepiphyseal right tibia which involved surrounding soft tissues. The lesion was composed of proliferating monotonous round cells with a high mitotic activity with scanty intersecting spindle cell fascicles. Immunohistochemistry of both areas demonstrated a strong positivity for actin (HHF-35 and alpha-SMA) and vimentin, and negative reactions for desmin, keratin (AE1 AE3), epithelial membrane antigen, S-100 protein, factor VIII-related antigen, CD 31 and CD 34. Ultrastructural study confirmed a diagnosis of leiomyosarcoma. This is the first detailed description of the microscopic and radiological features of primary epithelioid leiomyosarcoma of bone.
 ...
PMID:Primary epithelioid leiomyosarcoma of bone. Case report and literature review. 1033 49

A 74-year-old woman complained of a small nodule in the outer lower quadrant of her left breast. On physical examination, a 0.9 x 0.8 cm, round-shaped and firmly elastic nodule was palpated. Excisional biopsy was performed. Histologically, the tumor was separated into, with a bicellular pattern, containing both numerous glandular structures and numerous spindle-shaped cells. Immunohistochemical staining for EMA and cytokeratin showed strongly positive immunoreactivity for epithelial cells. Staining for &aipha;-SMA showed strongly positive immunoreactivity for myoepithelial components. Staining for keratin and S-100 protein showed weakly positive immunoreactivity for myoepithelial cells. Microscopically, the tumor was diagnosed as adenomyoepithelioma of the breast. Immunohistochemical examination is needed to distinguish epithelial cell proliferation from myoepithelial cell proliferation. Immunohistochemical examination using antibodies against EMA, alpha-SMA, Keratin, cytokeratin and S-100 protein, is indispensable.
 ...
PMID:Adenomyoepithelioma of the Breast: A Case Reprot and a Review of Literature. 1109 56

A 45-year-old female with solitary fibrous tumor (SFT) of the pleura was reported. Chest X-ray and CT scan on admission showed a large tumor in the right thoracic cavity. A preoperative needle biopsy was performed. The microscopic appearance of biopsied specimen revealed many spindle cells. And an immunohistochemical study was positive for CD 34 and negative for keratin, epithelial membrane antigen, alpha-smooth muscle actin, S-100 protein. So SFT was strongly suspected and an operation was performed. A tumor arised from the visceral pleura of right middle lobe and was pedunculated. The tumor was 13 x 10 x 7.5 cm in size. An immunohistochemical study of the tumor was positive for CD 34, and negative for SMA, ki-67. From these immunohistochemical stainings and microscopic findings, the tumor was diagnosed as SFT. An immunohistochemical study of the tumor seems to be very useful for the diagnosis of SFT.
 ...
PMID:[A resected case of solitary fibrous tumor of the pleura]. 1184 60

Ectomesenchymal chondromyxoid tumour (ECT) of the anterior tongue was first described in 1995. To date, only 23 cases have been reported in the literature. Two new cases of ECT have been described, with immunohistochemical analysis including cytokeratin (CK) profile, GFAP, S-100 protein, SMA, CD-57, EMA, desmin and Ki67. Tumour cells showed intense and diffuse staining for GFAP and diffuse staining for S-100 protein. Pan-keratin, high- and low-molecular-weight CK, CK 7, 8, 18, 19 and 20 were negative. Tumour cells were also negative for desmin, SMA, CD-57 and EMA. Ki67 was positive in only scattered cells. The findings of the present study support the suggested ectomesenchymal origin for ECT, rather than myoepithelial salivary gland origin. The low Ki67 expression is in agreement with the low growth rate, small size and lack of mitotic activity in the present cases, as well as in those previously described.
 ...
PMID:Ectomesenchymal chondromyxoid tumour of the anterior tongue. 1514 46

The seminal article of Drs Franz Enzinger and Renyuan Zhang in 1988 defined plexiform fibrohistiocytic tumor (PFHT) as a distinctive entity. They described 65 cases (from 1965 to 1985) in children and young adults, with female and upper extremity predominance. These tumors were morphologically divided into 3 groups: fibroblastic, histiocytic (often with osteoclast-type giant cells), and mixed. Most tumors exhibited a plexiform and infiltrative arrangement of cells at the dermal/subcutaneous junction. Two fibroblastic PFHT had a metaplastic bone formation. Absence of cellular pleomorphism, low mitotic activity, dense hyalinization, hemorrhage, and chronic inflammation were observed. Vascular invasion was present in 1 recurrent, yet nonmetastatic, case. Tumors were negative for S100 protein, desmin, cytokeratin, factor VIIIrag, and lysozyme. Most patients were without disease up to 60 years after excision; 32 (37.5%) cases with follow-up recurred and 2 of those patients had regional lymph node metastasis at 9 and 36 months, respectively, yet there were no systemic metastases. In the interim, there have been additional studies on PFHT. We wanted to update the literature and add 66 new PFHT cases (1986-present) from the Armed Forces Institute of Pathology, since this seminal article, in honor of Dr Franz Enzinger. There were 37 men and 29 women; patient age ranged from 1 to 77 years (median, 20 years; 53% of patients were younger than 20 years). Twenty-eight cases occurred in the upper extremity (mostly forearm), 16 in lower extremity, 11 in trunk, 9 in head and neck, and 2 of unknown site. Although most cases were observed at the dermal/subcutaneous interface, 22 cases were predominantly dermal, and the rest predominantly subcutaneous, with 4 superficially involving skeletal muscle. Except for 12 predominantly dermal cases, most cases had an infiltrative growth pattern. Thirty-four cases were predominantly histiocytic, 16 predominantly fibroblastic, and the remaining 16 mixed. Two fibroblastic cases demonstrated the microfat cells (probably secondary to subcutis infiltration). All cases exhibited a plexiform growth pattern of small- to medium-sized nodules; 41 cases had giant cells, mainly osteoclast type, often the predominantly histiocytic type. The purely fibroblastic often had surrounding inflammation, 2 cases with marked inflammation. Perineural growth was observed in 5 cases, peri-Pacinian corpuscle growth in 2 cases, adnexal trapping in several, and, increased hyalinized collagen in 17 cases. Eight cases demonstrated focal myxoid change. Only 1 case, a histiocytic, had bone formation. Although increased cytologic atypia and mitotic activity were noted in a few cases, an atypical mitosis was only observed in 1 case. No cases demonstrated vascular or lymphatic invasion or necrosis. The tumors were generally positive for CD68 and SMA, occasionally for MSA, and negative for keratin, desmin, HMB45, S100 protein, and CD34. Overall, the findings were very similar to the original observations made by Dr Enzinger and his colleague, with the minor exceptions of roughly equal sex distribution (possibly due to timely referral bias), and additional morphologic features of myxoid change, adnexal sparing, increased inflammation, and microfat similar to recently described lipofibromatosis. The relationship between PFHT and cellular neurothekeoma is also explored.
 ...
PMID:An update on plexiform fibrohistiocytic tumor and addition of 66 new cases from the Armed Forces Institute of Pathology, in honor of Franz M. Enzinger, MD. 1787 15

We report 2 cases of Follicular Dendritic Cell Sarcomas of Oral Cavity in two elderly patients. The patients presented with oral cavity tumors. Initial tru-cut biopsies in both cases revealed spindle cell neoplasms. One of them was of low grade malignancy and showed positivity for of Vimentin. Few cells showed positivity for keratin and were negative for S-100 protein, CD 34, CD 68, EMA, SMA, HMB-45. A possible diagnosis of low grade fibrohistiocytic tumor was made. He underwent Lt total maxillectomy. Three years later he presented back with regional nodal metastasis. The regional lymph nodes showed features of follicular dendritic cell sarcoma. The second case revealed high grade spindle cell neoplasm and showed positivity for vimentin and S100 protein and was negative for EMA, keratin, CD-34, desmin, muscle actin and HMB-45. He was offered initial radio-therapy followed by hemifacial resection. The histology along with immuno histochemistry favoured a diagnosis of follicular dendritic cell sarcoma. He presented with local recurrence two months later. Both the cases are discussed in detail.
 ...
PMID:Follicular dendritic cell sarcoma of oral cavity: report of 2 cases. 1788 29

Sarcomas are neoplasms of mesenchymal origin, with a predominant cell population mimicking the organization of various soft tissues and/or bones. Previous categorizations also included the possibility of the presence of tissue macrophage-like (histiocytes) neoplasm cells, in a tumour described as malignant fibrous histiocytoma, but this group has been considered as a variety of undifferentiated pleomorphic sarcomas. Although this kind of malignancy is not rare in humans, only few cases have been reported in laboratory animals. We report an unusual single case of spontaneous tumour growth, detected by casual observation, in the left thoracic area of an 18-month-old male laboratory Wistar rat. Both this individual and his ancestors were not exposed to any known carcinogenic substance or radiation, thus suggesting the development of the neoplasm as a spontaneous event. The mass was extracted surgically under general anaesthesia, and slices were examined histologically and immunohistochemically, using photon microscopy. The pathologist reported the presence of a combination of fibroblasts and undifferentiated mesenchymal cells arranged in a storiform pattern. Immunohistochemistry was performed on the tissue using specific antibodies for several proliferation (Ki-67) and differentiation (S-100, CD-34, CD-68, pan-keratin, desmin and smooth muscle actin-SMA) markers. Positive reaction was observed for S-100, Ki-67, CD-68, desmin and SMA (limited) but not for CD-34 or cytokeratin.
 ...
PMID:Casual discovery of a thoracic tumour showing histological features of undifferentiated pleomorphic sarcoma in a male Wistar laboratory rat. 1802 53


1 2 3 4 Next >>