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[Castleman's disease. X-ray computed tomographic aspect. Apropos of 2 mediastinal forms and a multicentric form].

A multicentric form of Castleman's disease is described, this being a rare affection for which CT image characteristics have not, to our knowledge, been reported. Two cases of localized and one case of multicentric Castleman's disease are reported, and differential characters of these two forms with common histology: angio-follicular lymphoid hyperplasia, outlined. The localized form is found mainly in the mediastinum in young patients and follows a favorable course with recovery after exeresis. The diffuse form develops in later life and presents with severe systemic signs and a marked biological inflammatory syndrome Glands and viscera are affected and its course is grave, with mostly fatal relapses. Angiography suggests diagnosis when images show hypervascular lesions in lymph glands. CT scan imaging with contrast is very suggestive when glandular lesions are present that take up contrast strongly. This appearance should always raise the possibility of Castleman's disease.

Adult↗

Molecular genetic and immunohistochemical analyses of a case of multicentric Castleman's disease.

Whether Castleman's disease is primarily hyperplastic or neoplastic in nature is a matter of controversy; however, the classical localized form has been thought clinically benign in itself, because it can be cured by local therapy. Recently, a multicentric form of the disease has been reported and has received attention because of its aggressive clinical course and high incidence of developing malignant lymphoma. Here, we describe a case of multicentric Castleman's disease which occurred in a 24-year-old Japanese man. To ascertain whether a monoclonal population was present or not, we performed a molecular genetic analysis of the genes encoding the T-cell receptor and the immunoglobulin, as well as a conventional immunohistochemical analysis. Using these sensitive methods, no monoclonal population could be found. The findings suggest that our case represents a reactive rather than a neoplastic disorder.

Adult↗

Multicentric reticulohistiocytosis. Report of three cases with the evaluation of tissue proteinase activity.

Three cases of multicentric reticulohistiocytosis showing typical clinical, histologic, and ultrastructural findings are reported. In one, gastric cancer occurred; in the other two cases, severe polyarthritis was the only detectable internal involvement. The serine proteinases, urokinase and tissue-type plasminogen activator, were evaluated both with the autohistographic technique and spectrophotometric assay in lesional skin and synovia. Urokinase levels appeared grossly increased in the lesional synovia and moderately increased in the lesional skin. We suggest that urokinase, presumably released by the activated proliferating histiocytes, may play a major role in the extracellular matrix degradation leading to erosion of cartilage and adjacent bone in multicentric reticulohistiocytosis.

Adult↗

Multicentric reticulohistiocytosis.

This is a short report on the first case of a multicentric reticulohistiocytosis diagnosed at Middle Road Hospital in 1987. He presented with multiple reddish brown papules and nodules but without arthritis. Systemic review did not show any associated malignancies. Treatment with methotrexate has not been useful. Multicentric Reticulohistiocytosis is a proliferative histiocytic disorder that is benign. It is characterised by a dermatoarthritis, extensive red nodule and a rheumatoid-like arthritis. Histologically, multinucleated giant cells in ground glass cytoplasma and mononuclear histiocytes form the granulomatous infiltrate that invades the dermis, mucosa, synovium and others--bone, plasma, pericardium etc.

Histiocytes↗

[Multicentric carcinoma of the esophagus and double primary malignant neoplasm of the upper alimentary tract--report on 44 patients].

During the past fifteen years, 44 patients with multicentric carcinoma of the esophagus or double primary malignant neoplasm of the upper alimentary tract, collected from 5316 patients with cancer of esophagus or gastric cardia in the same period, were treated in our hospital. The incidence was 0.83%. All were proven by pathology. Exploratory thoracotomy was done in 32 patients and 26 lesions were resected with a resection rate of 81.3%. All patients with cancer resected survived over 1 year, 6 out of 9 survived over 3 years and 2 out of 3, over 5 years. In the 18 patients who received chemotherapy alone, 12 were followed and all died within 1 year. It is shown that multicentric carcinoma of the esophagus or double malignant neoplasm of the upper alimentary tract should be given energetic treatment. Finally, the incidence, diagnosis criteria, diagnosis method, etiology and management are discussed with a review of literature.

Adenocarcinoma↗

[Multicentric cancer of the breast].

Multicentric breast carcinoma was identified in 3.7% of 119 patients operated on at the Center Clinics. A slow growth, small size of tumors and infrequent lesions in the regional lymph nodes were registered. A correlation between the degree of malignancy and the number of tumor nodes in breast tissues was established. The best end results--a survival rate of 16.8% at 10 years--were obtained after radical mastectomies. A classification of multicentric breast cancers is suggested.

Adult↗

Multicentric fibromatosis of familial inheritance.

This is an unusual case presentation of a young woman with a clinical history and course simulating juvenile hyaline fibromatosis, but without hyalinization in the stroma. Because of the age of onset, the apparent familial inheritance, the nonaggressive and nonregressive tumor traits, the disease cannot be classified as multicentric fibromatosis. Therefore, we are referring to this case as multicentric fibromatosis with familial inheritance, a previously unreported entity to our knowledge.

Adult↗

Multicentric ovarian and extraovarian cystadenofibroma.

A case of multicentric ovarian and diffuse pelvic cystadenofibroma is described. Despite multicentricity, the histologic findings are completely benign. Electron microscopy was performed and possible mesothelial origin is discussed.

Adenofibroma↗

Multicentric giant lymph node hyperplasia, Kaposi's sarcoma, and lymphoma.

We studied two cases of a recently recognized systemic lymphoproliferative disease with morphological features of Castleman's disease: multicentric giant lymph node hyperplasia. Both patients developed Kaposi's sarcoma and had laboratory evidence of immune abnormalities, including reversed T4 to T8 ratios. One patient's disease had a subacute course with fevers of unknown origin, mucocutaneous candidiasis, and progressive thrombocytopenia, while the other patient's disease had a fulminant course with anemia, thrombocytopenia, and splenic lymphoma. Both patients were elderly, and both died of complications of multicentric giant lymph node hyperplasia.

Acquired Immunodeficiency Syndrome↗

Chronic nephropathy in idiopathic multicentric osteolysis.

A 19-year-old girl having a sporadic form of idiopathic multicentric osteolysis with nephropathy is described. She was first diagnosed as having sporadic carpotarsal osteolysis at the age of 6. Her proteinuria was first detected at 15 years of age and renal biopsy specimens obtained at the same age were compatible with those of focal segmental glomerulosclerosis with interstitial fibrosis and tubular atrophy. Immunofluorescence microscopy revealed negative deposits of Ig G, Ig A and C3 but a trace amount of mesangial Ig M in unsclerosed segments of the glomeruli. Electronmicroscopy showed no specific changes. Renal vascular changes such as proliferation of vascular intima and medial hypertrophy were not demonstrated in the present case. It is our conclusion that focal segmental glomerulosclerosis is part of the spectrum of nephropathy in the sporadic form of idiopathic multicentric osteolysis.

Adult↗

Pirenzepine in duodenal ulcer. A multicentre double-blind controlled clinical trial. Second of two parts.

Ninety-six patients with endoscopically proved active duodenal ulcers were admitted to a multicentre double-blind trial with either pirenzepine (100 mg/day, 25 mg in the morning and midday and 50 mg at bed time) or placebo for 4 weeks. Ninety-two patients (46 in each group) completed the trial. After 4 weeks, complete healing had been achieved in 70% of the pirenzepine-treated patients and in 32% of the placebo-treated ones (P less than 0.01). No important side-effects and no abnormal changes in blood values or urinalysis were observed during treatment. The difference in the ulcer healing rates observed in the two parts of this multicentre trial is briefly discussed.

Adult↗

Relationship between multicentric reticulohistiocytosis and tuberculosis.

Some cases presently classified as multicentric reticulohistiocytosis may represent unusual manifestations of tuberculosis. Antituberculous chemotherapy may be justified in patients who have clinical and histopathologic evidence of multicentric reticulohistiocytosis in association with a positive tuberculin skin test.

Arthritis↗

[Multicentricity of urothelial tumors of the efferent urinary tracts].

On the basis of 8 cases with primary urothelial tumours of the upper efferent urinary tract with simultaneously only partly preoperatively diagnosed secondary tumours of the urothelium the problems of diagnostics and therapy are discussed. A conservative surgery may be regarded as sufficiently radical only then when a multicentric genesis may be excluded with certainty. In our patients with tumours of the upper efferent urinary tract we found a multicentricity in 18.4%.

Aged↗

[Bilateral adrenal neuroblastoma of multicentric origin].

Bilateral neuroblastoma is extremely rare. In the literature few cases are reported which are mostly considered to represent metastases rather than multicentric tumoral occurrence. A case of bilateral adrenal neuroblastoma is reported; given its various aspects, and especially the prognosis features, this tumor is, in our opinion, of a multicentric origin.

Adrenal Gland Neoplasms↗

Multicentric nature of vulvar carcinoma in situ.

A detailed histologic and DNA-microspectrophotometric study is presented of 31 lesions selected from 12 of 65 patients with vulvar carcinoma in situ whom the authors have seen. In 6 patients the lesions were multicentric and in 6 they were confluent. The purpose of the study was to determine whether or not there was a measurable biologic difference between these 2 distinctly different clinical lesions that would justify separating them into different categories. A hypothesis as to the clonal evolution of multicentric and confluent vulvar carcinoma is presented based on the authors' findings.

Carcinoma in Situ↗

Identification of multiple breast cancers of multicentric origin by histological observations and distribution of allele loss on chromosome 16q.

Breast cancer is often detected as multiple lesions clinically and/or histopathologically. To examine if the origin of such lesions can be identified objectively by comparison of their loss of heterozygosity (LOH) patterns, LOH on chromosome 16q was analyzed in a total of 60 cases of multiple breast cancer by Southern blot analysis. Based on continuity among tumors and satellite nodule features, 30 cases of unilateral multiple cancer were classified morphologically into 3 groups: A, multicentric origin (11 cases); B, multifocal invasion of one intraductal carcinoma (15 cases); and C, intramammary metastases (4 cases). As controls, group D, synchronously bilateral breast cancers (11 cases), and group E, sets of a primary tumor and a lymph node metastasis (19 cases), were also examined. On a highly probable assumption that LOH on 16q occurs randomly in 50% of breast cancer cases at an early stage, the number of cases showing a concordant LOH pattern on 16q among tumors was compared between observed data, and the value was estimated from a normal distribution model in each group. In groups A and D, the allele pattern on 16q among tumors was concordant in 5 of 11 cases each, thus supporting their independent occurrence and multicentric origin, whereas the LOH pattern among tumors was identical in all of the cases in groups B, C, and E, thus supporting their monocentric origin. This comparison of the LOH pattern in multiple breast cancer was shown to yield results compatible with the morphological classification and was suggested to be of diagnostic value.

Breast Neoplasms↗

Expression and hypomethylation of alpha-fetoprotein gene in unicentric and multicentric human hepatocellular carcinomas.

The messenger RNA and DNA methylation of the alpha-fetoprotein gene were studied in 101 resected primary hepatocellular carcinomas, of which 93 were unicentric and 8 were multicentric. Fifty-five were 5 cm or less in diameter (small) and 46 were more than 5 cm in diameter (large). In 48.5% of the cases, we detected alpha-fetoprotein messenger RNA in hepatocellular carcinomas, more frequently in large (60.9%) than in small (38.2%; p < 0.00001) but not in any of the nontumorous livers. The alpha-fetoprotein messenger RNA was detected in 83%, 70% and 6.8% of patients with serum alpha-fetoprotein levels of 320 ng/ml or more, 100 to 319 ng/ml and less than 100 ng/ml, respectively. This finding suggests that alpha-fetoprotein gene expression in hepatocellular carcinoma contributes to the serum alpha-fetoprotein elevation in patients with hepatocellular carcinoma. alpha-Fetoprotein messenger RNA appeared as a major band of 2.4 kb, with two minor species of about 6.5 and 3.6 kb in the hepatocellular carcinoma and the fetal liver. Hypomethylation of the 5' end of the alpha-fetoprotein gene was detected in 78.3% of hepatocellular carcinomas expressing alpha-fetoprotein messenger RNA but infrequently (16.7%) in hepatocellular carcinomas with no detectable alpha-fetoprotein messenger RNA (p < 0.0003). This finding suggests that hypomethylation at the 5' region of the gene is associated with alpha-fetoprotein gene reexpression in hepatocellular carcinoma. The alpha-fetoprotein gene expression helped to differentiate unicentric from multicentric hepatocellular carcinomas and to identify other hidden alpha-fetoprotein-secreting hepatocellular carcinomas.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Viral implications in the formation of multicentric inverting papilloma.

There is growing information to support a viral etiology in inverting papilloma formation. Evidence for this hypothesis comes from clinical findings of multicentricity, high recurrence rates, identification of human papilloma virus (HPV), and the presence of viral-like particles within the tumour tissue. Four cases of multicentric inverting papilloma are presented. In one patient, a squamous papilloma (wart) of the nasal vestibule recurred as an inverting papilloma. Another patient also had a cutaneous wart on the right index finger. These cases and others from the literature are discussed with respect to a viral mode of transmission.

Adolescent↗