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PubMed1997

[Photomicrographs of a pleural biopsy specimen, showing malignant mesothelioma and necrosis--malignant pleural mesothelioma in a patient with sarcoidosis].

A 45-year-old woman was admitted to Nara Medical University Hospital because of right-sided chest pain. Both a chest X-ray film and a chest CT scan revealed bilateral hilar and mediastinal lymphadenopathy and right pleurisy. She underwent fiberoptic bronchoscopy and mediastinoscopy. Biopsy specimens of the lung and mediastinal lymph nodes showed non-caseous epithelioid cell granulomas. After on ophthalmologic examination, she was given a diagnosis of sarcoidosis with a pleural lesion and uveal involvement. The pleurisy become less severe and the lymph nodes shrank without any treatment. About one year later, she complained of right-sided chest pain again and a chest X-ray film revealed diffuse thickening of the pleural on the right side. Examination of pleural biopsy specimens revealed malignant mesothelioma. We know of no previous report from Japan of malignant pleural mesothelioma in a patient with sarcoidosis.

Biopsy↗
PubMed1998

Malignant lymphoma in Thailand: changes in the frequency of malignant lymphoma determined from a histopathologic and immunophenotypic analysis of 425 cases at Siriraj Hospital.

BACKGROUND: Analysis of malignant lymphoma in a single institution at different periods of time can determine the changing status of the disease in the region. METHODS: To compare with the large series of 1095 lymphoma cases reported between 1957-1971 at Siriraj Hospital, the largest hospital in Thailand, a similar study was performed through histopathologic evaluation of 425 lymphoma cases diagnosed consecutively at the same institution between August 1993 and October 1995. Phenotypic analysis was performed by paraffin section-immunoperoxidase studies. RESULTS: A striking increase in lymphoma cases was noted from 73 cases/year in the first series to 189 cases/year in the second series (an increase of 158.9%). Lymphoma occurred in all age groups, with a peak incidence at the seventh decade of life. The male to female ratio decreased from 2:1 in 1957-1971 to 1.3:1 in the more recent series. The incidence of Hodgkin's disease (HD) was found to have decreased from 28.9% to 8.5%. There were 36 cases (8.5%) of HD and 389 cases (91.5%) of non-Hodgkin's lymphoma (NHL) reported in the second series. The subtypes of HD included 16 cases of mixed cellularity, 13 cases of nodular sclerosis, 6 cases of lymphocyte depletion, and 1 case of lymphocyte predominance. According to the Working Formulation, the 389 NHL cases included low grade (14.1%), intermediate grade (57.3%), high grade (11.3%), and miscellaneous groups (17.2%). They were classified as small lymphocytic (9.5%), follicular (11.1%), diffuse (50.9%), immunoblastic (4.1%), small noncleaved (4.4%), lymphoblastic (2.8%), anaplastic large cell (9.0%), mycosis fungoides (1.8%), hairy cell leukemia (0.3%), true histiocytic (0.5%), and extramedullary plasmacytoma (1.0%). The immunophenotypes of the 359 NHL cases available for paraffin section-immunoperoxidase studies were B-cell (71.0%), T-cell (24.5%), histiocyte (0.6%), and undetermined phenotypes (3.9%). CONCLUSIONS: The incidence of malignant lymphoma is increasing in Thailand, with a high frequency of intermediate to high grade NHL of B-cell phenotype reported.

Adolescent↗
PubMedDate not supplied

Malignant catarrhal fever in cattle experimentally inoculated with a herpesvirus isolated from a case of malignant catarrhal fever in Minnesota USA.

A malignant catarrhal fever (MCF)-like syndrome was experimentally induced in three steers, which were under immunization trials with a herpesvirus previously isolated from a case of MCF in a cow in Minnesota USA. The clinical signs observed in the three steers, and the pathological and histological lesions observed in two of these steers which succumbed to the disease syndrome were indistinguishable from those described for MCF. Although seroconversion was readily demonstrated in the three animals, virus was not re-isolated from the blood leucocytes, secretions and tissues obtained from the two animals which succumbed to the syndrome during the course of the disease and after death. However, a herpesvirus which showed cell rounding cytopathic effects (cpe) in bovine thyroid cells (Bth), was re-isolated from the one steer which survived the disease.

Animals↗
PubMed2003

Neuroleptic malignant syndrome-like, or--dopaminergic malignant syndrome--due to levodopa therapy withdrawal. Clinical features in 11 patients.

A clinical picture named neuroleptic malignant-like syndrome has been described in patients with Parkinson's disease (PD) who suddenly stop their L-dopa treatment. The sudden withdrawal of the drug is deemed to lead to an acute deficiency stage in a patient who has an iatrogenic increase of dopaminergic transmission. We present a series of 11 patients with PD with an average age of 72.09 years, a mean disease duration of 9.45 years who developed this problem after a 92.72 h latency period. If patients with PD develop severe rigidity, stupor and hyperthermia, L-dopa withdrawal should be suspected and the dopaminergic drug restarted as soon as possible.

Adult↗
PubMed2003

Prevention and treatment of malignant syndrome in Parkinson's disease: a consensus statement of the malignant syndrome research group.

We report a consensus statement of the collaborative research group on the prevention and treatment of malignant syndrome (MS) in Parkinson's disease. The syndrome is quite similar to neuroleptic MS. Although sudden withdrawal of levodopa was the most frequent cause, many other precipitating events were found such as intercurrent infections, dehydration, hot weather, discontinuation of other anti-parkinsonian drugs, and "wearing off" phenomenon. Awareness of this syndrome is most important for its early detection and the prompt commencement of treatment. MS should be suspected whenever the body temperature rises above 38 degrees C without an apparent cause. Treatment consists of ample intravenous fluid, cooling the body, anti-parkinsonian drugs (particularly levodopa and bromocriptine), dantrolene sodium, and antibiotics if infection is present. Rhabdomyolysis, disseminated intravascular coagulation, and acute renal failure constitute serious complications.

Antiparkinson Agents↗
PubMed2005

Echocardiographic changes in patients with malignant phase hypertension: the West Birmingham Malignant Hypertension Register.

In order to study the echocardiographic abnormalities in consecutive patients with malignant phase hypertension (MHT), we reviewed echocardiograms of 31 patients (23 male; mean age 52+/-14 years) with MHT who were admitted to our unit. Trans-thoracic echocardiography was carried out in all patients, and echocardiographic measurements were compared with those of 39 patients (30 male; mean age 54+/-10 years) with controlled nonmalignant essential hypertension, and 32 (19 male; mean age 51+/-10 years) healthy normotensive volunteers. Patients with MHT had a significantly higher mean systolic and diastolic blood pressure (P<0.001) compared to the other two groups. MHT patients had significantly greater mean left atrial dimensions (P=0.002), as well as aortic root dimensions (P=0.01) and left ventricular (LV) dimensions (with the exception of the diastolic internal diameter) (P<0.001). MHT patients also had a mean larger LV mass and LV mass index (both P<0.001) when compared to the other two groups. The mean ejection fraction was also lower in the MHT group (P<0.001). In conclusion, patients with MHT have significant cardiac hypertrophy, in association with systolic dysfunction and dilated left atria, irrespective of the duration of known hypertension. These abnormalities may predispose MHT patients to cardiovascular complications including heart failure and cardiac arrhythmias, such as atrial fibrillation.

Adult↗
PubMed2002

Malignant germ cell tumors: clinical characteristics, treatment, and outcome. A report from the study group for Pediatric Solid Malignant Tumors in the Kyushu Area, Japan.

PURPOSE: This study aims to assess the prognostic factors and optimal treatments for malignant germ cell tumors (MGCT) in childhood. METHODS: Among 117 MGCT, the clinical features were analyzed. Regarding the histology, there were 89 embryonal carcinomas, 13 dysgerminomas, 4 choriocarcinomas, and 11 others. The prognostic factors and treatments were assessed based on the 5-year survival rate. RESULTS: (1) Stage: 100% for stage I (n = 54), 75.0% for stage II (n = 4), 67.3% for stage III (n = 14), and 54.8% for stage IV (n = 33); Unknown: n = 12. (2) Primary site: 93.4% for the testis (n = 52), 86.7% for the ovary (n = 31), 56.9% for the sacrococcygeal (n = 21), and 60.6% for others (n = 12); unknown: n = 1. (3) Surgical intervention for primary tumor: 100% for stage I with a complete resection (n = 53), 78.4% for stage III, IV with a complete resection (n = 26), and 33.3% for stage III, IV with an incomplete resection (n = 21). (4) Type of chemotherapy for the stage III and IV: 83.9% for the PVB (cisplatin, vinblastin, bleomycin; n = 13), 66.7% for the VAC (vincristine, actinomycin D, cyclophosphamide; n = 6), and 47.1% for other regimens (n = 25). CONCLUSIONS: An early stage, a diagnosis under 1 year of age and a primary site in the gonads were favorable prognosis factors, whereas histologic findings of choriocarcinoma and liver or lung metastasis were unfavorable. Radical complete resection alone is a sufficient treatment for localized MGCT. The PVB regimen is optimal chemotherapy for advanced MGCT; however, high-risk cases still may require more aggressive treatment.

Adolescent↗
PubMed2002

Presence of two different genetic traits in malignant hyperthermia families: implication for genetic analysis, diagnosis, and incidence of malignant hyperthermia susceptibility.

BACKGROUND: Malignant hyperthermia susceptibility (MHS), an uncommon syndrome often inherited as an autosomal dominant trait, is characterized by a genetic and clinical heterogeneity. In this article, the authors described six pedigrees in which both parents of MHS patients were diagnosed with MHS by an diagnostic test. Haplotype and mutation analysis revealed that more than one MHS genetic trait was present in these families. METHODS: A panel of 104 MHS families were investigated with a caffeine halothane contracture test on muscle biopsy specimens. When possible, blood creatine kinase concentrations of MHS patients were measured. Haplotyping studies were conducted with chromosome 19q13.2 polymorphic markers and mutations were searched for in patients' DNA. RESULTS: In six families, the diagnostic test and genetic studies demonstrated that both, apparently unrelated, parents of MHS patients were MHS. In three families, homozygous or compound heterozygous individuals for RYR1 mutations were characterized at a molecular level. In one family, a compound heterozygous patient harboring a RYR1 mutation and a CACNA1S mutation was identified. While patients with two mutated alleles did not show differences in their muscle response to halothane or caffeine, their creatinine kinase concentrations were significantly elevated compared with the heterozygous patients. CONCLUSIONS: Based on genetic and diagnostic test data, more than one MHS allele associated with the MHS phenotype was evidenced in four families. These data should be considered in view of the use of genetics for the diagnosis of MHS and when reaching conclusions of genetic heterogeneity in MHS families. Taking into account the usual dominant mode of transmission of MHS and the size of the investigated population, the authors propose an evaluation of the incidence of the MHS in the general population based on genetic data.

Adolescent↗
PubMed1997

In vitro contracture test for diagnosis of malignant hyperthermia following the protocol of the European MH Group: results of testing patients surviving fulminant MH and unrelated low-risk subjects. The European Malignant Hyperthermia Group.

BACKGROUND: Determination of sensitivity and specificity of the in vitro contracture test (IVCT) for malignant hyperthermia (MH) susceptibility using the European MH Group (EMHG) protocol has been performed in some laboratories but only on a small sample from the combined EMHG. Thus, the purpose of the present study was to determine combined EMHG sensitivity and specificity of the test. METHODS: Results of IVCT of patients with previous fulminant MH and normal, low-risk subjects (controls) were collected from 22 centres of the EMHG. IVCT was performed according to the EMHG protocol. Patients were included in the study if the clinical crisis had a score of at least 50 points with the Clinical Grading Scale. Low-risk subjects were included provided they did not belong to a family with known MH susceptibility, they had not developed any signs of MH at previous anaesthetics, and they did not suffer from any neuromuscular disease. For inclusion of both MH patients and low-risk subjects, at least 1 muscle bundle in the IVCT should have twitches of 10 mN (1 g) or more. For evaluation of individual tests, only muscle bundles with twitch heights of 10 mN (1 g) or more were used. RESULTS: A total of 1502 probands had undergone IVCT because of a previous anaesthesia with symptoms and signs suggestive of MH. Of these, 119 had clinical scores of 50 and above. From these 119 MH-suspected patients and from 202 low-risk subjects, IVCT data were collected. Subsequently, 14 MH-suspected patients were excluded from further analysis for the following reasons: In 3 patients, the suspected MH episode could be fully explained by diseases other than MH; in 11 MHS patients, IVCT was incomplete (n = 1), data were lost (n = 3), or none of the muscle bundles fulfilled twitch criteria (n = 7). Of the remaining 105 MH-suspected patients, 89 were MHS, 10 MHEh, 5 MHEc, and one MHN. Thus, we observed a diagnostic sensitivity of the IVCT of 99.0% if the MHE group is considered susceptible (95% confidence interval 94.8-100.0%). Of the 202 low-risk subjects, 3 were MHS, 5 MHEh, 5 MHEc, and 189 MHN. This gives a specificity of the IVCT of 93.6% (95% confidence interval 89.2-96.5%). CONCLUSION: The IVCT for diagnosis of MH susceptibility in Europe has a high sensitivity and a satisfactory specificity.

Adolescent↗
PubMed2004

4-chloro-m-cresol cannot detect malignant hyperthermia equivocal cells in an alternative minimally invasive diagnostic test of malignant hyperthermia susceptibility.

Malignant hyperthermia (MH) is an inherited skeletal muscle disorder triggered by commonly used anesthetics. Mutated ryanodine receptors have been identified as molecular targets. The sensitivity of myotubes from individuals classified by the in vitro contracture test as MH susceptible (MHS), normal (MHN), and equivocal (MHEH) was assessed for the Ca2+-releasing activity of 4-chloro-m-cresol (4-CmC) and caffeine. In this study, we sought to determine whether 4-CmC can differentiate the MH status of an individual on the basis of the release of intracellular Ca2+, particularly in regard to MHEH diagnosis. Intracellular Ca2+ concentration was determined photometrically with Fura2. Regions of the ryanodine receptor 1 harboring most of the described MH mutations were sequenced from MHS and MHEH cells. One MH mutation (Gly2434Arg) was found in one MHS individual. Results of the caffeine-induced Ca2+ release in MHS and MHN cells correlated well with the in vitro contracture test results. MHS cells showed a higher sensitivity against caffeine and, to a lesser extent, against 4-CmC. Cells of MHEH individuals showed low sensitivities against both caffeine and 4-CmC, comparable to those of the MHN group. Therefore, with myotubes, caffeine was able to discriminate between MHS and MHN cells, but both caffeine and 4-CmC failed to detect MHEH cells.

Caffeine↗
PubMed1996

Human papilloma viruses and p53 mutations in normal pre-malignant and malignant oral epithelia.

HPV infections have been previously observed in oral cancers, and inactivation of the p53 gene has been shown to be one of the most common genetic alterations in human tumors. We examined 179 oral specimens from 70 individuals with histologic findings of either normal mucosa (n = 6) or oral disease that ranged from mild dysplasia to invasive squamous-cell carcinoma (n = 64) to determine the occurrence of both HPV infection and p53 mutations and their relationship with several clinical factors. HPV infection was detected by PCR amplification of viral DNA, and the presence of p53 mutations was assayed using the single-strand conformation polymorphism (SSCP)-PCR technique. HPV infection was found in 31% of individuals with oral disease and was not seen in healthy individuals. Mutations in exons 5, 6, 7 or 8 of the p53 gene were detected in 37.5% of patients with oral lesions and in a biopsy from 1 healthy individual who was a heavy smoker. Approximately one-third of lesions classified as pre-malignant (dysplasia and carcinoma in situ) and 42% of invasive carcinomas contained p53 mutations. The majority of these mutations were G:T transversions located within exons 7 and 8. Tumor tissues from 6 patients with oral lesions were found both to be HPV-16-positive and to contain p53 mutations; of these, 4 were poorly differentiated carcinomas that were diagnosed as late-stage disease. In this study, p53 mutations were detected in the early stages of cancer development.

Aged↗
PubMed1999

Computer-assisted cytogenetic analysis of 51 malignant peripheral-nerve-sheath tumors: sporadic vs. neurofibromatosis-type-1-associated malignant schwannomas.

Cytogenetic studies in small groups of patients with malignant peripheral-nerve-sheath tumors (MPNST) revealed complex karyotypes with no consistent changes. A computer-assisted cytogenetic analysis using a cytogenetic database was performed to determine recurrent cytogenetic alterations in 51 MPNSTs (44 from the literature and 7 new cases) and to allow direct cytogenetic comparison between NF-1-associated and sporadic MPNSTs. Significant loss (p < 0.05) was observed in the chromosomal regions 9p2, 11p1, 11q2 and 18p1. Also, loss in 1p3, 9p1, 11q1, 12q2, 17p1, 18q1-q2, 19p1, 22q1, X and Y was detected. Gain of chromosomal material was found in chromosome 7, especially 7q1 (p < 0.05). Most involved breakpoints were: 1p13, 1q21, 7p22, 9p11, 17p11, 17q11, 22q11. Cytogenetic differences between NF-1-associated and sporadic MPNSTs included a relative loss of chromosomal material in NF-1-associated MPNSTs in 1p3, 4p1 and 21p1-q2 and a relative gain in 15p1-q1. Differences in breakpoints between the NF-1 associated and the sporadic MPNST group were observed in 1p21-22 (28% of NF-1 vs. 0% of sporadic MPNSTs), 1p32-34 (17% vs. 0%), 8p11-12 (7% vs. 27%) and 17q10-12 (24% vs. 7%). This approach, in which the cytogenetic results of various reports are combined, shows that losses in 9p2 and gains in 7q1 could be of oncogenetic importance in MPNSTs. Loss of 17q1, on which the NF-1 gene has been located (17q11.2), is not a common cytogenetic finding in NF-1-associated MPNSTs. The observed differences between NF-1-associated and sporadic MPNSTs might reflect different oncogenetic pathways.

Adolescent↗
PubMed1975

Malignant soft tissue tumors of probable histiocytic origin (malignant fibrous histiocytomas): general considerations and electron microscopic and tissue culture studies.

Correlated light and electron microscopic study of four fibrous histiocytomas of proven malignancy has confirmed the presence of histiocyte-like and fibroblast-like cells in this tumor. In addition, an undifferentiated cell type, giant cells, xanthomatous cells, and rare cells with morphological characteristics intermediate between those of histiocytes and fibroblasts were seen. "Nuclear body" type inclusions were commonly present in both principal cell types in all four cases, as were the somewhat less common cytoplasmic inclusion bodies. Electron microscopic study of a tissue culture of one of these tumors demonstrated only fibroblast-like, histiocyte-like, and xanthomatous cells. The possibility is suggested that both principal cell types in this tumor may derive from the same undifferentiated stem cell.

Adult↗
PubMed1978

Malignant fibrous histiocytoma (malignant fibrous xanthoma: xanthosarcoma) of bone.

Seven cases of malignant fibrous histiocytoma of bone are documented. In addition to typical histologic features such as the presence of a storiform pattern, histiocytic and fibrocytic cell types and severe cellular atypism, in five cases there were areas in which osteoclast-type giant cells were prominent and the lesion resembled a giant cell tumor. Three of the seven patients developed pulmonary metastases within two years of amputation, one developed extensive local recurrence including soft tissue and vascular invasion, and the other three are without evidence of disease but have been followed for only short periods.

Adolescent↗
PubMed1980

Body composition in cachexia resulting from malignant and non-malignant diseases.

The body composition of 23 patients with cachexia resulting from malignant or benign inflammatory disease was studied. Skinfold thickness and radioisotope tracer measurements enabled us to estimate total body fat, lean body mass, the ratio of fat loss to lean body loss, erythrocyte and plasma volumes, total body water, extra-, and intracellular water volumes, total body potassium, and intracellular potassium concentration. The total body fat and lean body mass information suggests that the body composition in cancerous cachexia differs from that in cachexia caused by benign inflammatory disease, in that lean tissue appears to be better conserved in the former. The radioactive tracer studies showed that the cachectic patients were anemic, potassium-deficient, and overhydrated; however, fluid partitioning into extra- and intracellular compartments was normal.

Adolescent↗
PubMed1980

Activity and isoenzymes of acid phosphatase in human B-cell lymphomas of low-grade malignancy: a novel aid in the classification of malignant lymphoma.

Activity and isoelectric focusing (IEF) pattern of lysosomal acid phosphatase (E.C.3.1.3.2.) were investigated in 55 cases of low-grade malignant B-cell lymphoma, classified as chronic B-lymphocytic leukemia (B-CLL), centroblastic/centrocytic follicular lymphoma (CB/CC), lymphoplasmacytic/lymphoplasmacytoid lymphoma (Immunocytoma, IC), and plasmacytoma (PC), applying the criteria of the Kiel classification. The results show (1) that the four lymphoma types present a characteristic range of enzyme activity in an increasing order: B-CLL, BC/CC, IC, and PC. B lymphocytes, germinal center cells, and plasmacytes are the main constituents of these lymphomas. This sequence might reflect one possible mode of B-cell transformation into plasmacytes traversing an amplification stage in germinal centers under normal conditions. (2) All cases showed the basic IEF pattern of normal B lymphocytes with 12 bands localized in three regions between pH 6.1 and 3.9. This finding supports the B-cell origin and the close phenotypical relationship among the investigated lymphomas. (3) The IEF patterns of B-CLL and CB/CC did not differ from that of normal B lymphocytes, whereas two additional isoenzymes were encountered in cases of IC and seven in PC; this suggests that the higher enzyme activity of IC and PC is at least partly due to the appearance of "new" isoenzymes. The results support the validity of the underlying classification and indicate the individually, B-cell origin, and close relationship among the four lymphoma entities investigated.

Acid Phosphatase↗
PubMed1982

Experimental studies on malignant fibrous histiocytomas in rats. I. Production of malignant fibrous histiocytomas by 4-hydroxyaminoquinoline 1-oxide in bone of Fischer 344 strain rats.

Malignant fibrous histocytomas (MFHs) were induced by a single injection of 4-hydroxyaminoquinoline 1-oxide (4-HAQO) into periosseous tissue of the tibia or by inserting solid 4-HAQO into the bone marrow of the tibia of male Fischer 344 rats. Periosseous MFHs were induced by doses of 2 mg and 4 mg of 4-HAQO per rat in three of 13 (23%) rats and nine of 13 (69%) rats, respectively, at 18 to 27 weeks after treatment. Bone MFHs were induced by doses of 2 mg, 4 mg, and 8 mg of 4-HAQO per rat in one of 15 (7%) rats, 11 of 18 (61%) rats, and 12 of 14 (86%) rats, respectively, after 18 to 29 weeks. Radiologic examination of bone MFH revealed bone destruction with or without a periosteal reaction, pathologic fracture, and tumor invasion into periosseous soft tissue. The serum alkaline phosphatase levels were elevated in rats with bone MFHs. Histologically, these MFHs were divided into fibrous, giant cell, myxoid, and inflammatory types. The incidence of fibrous MFHs was highest. Lung and inguinal and/or retroperitoneal lymph node metastases were observed in some rats, and these were of the fibrous type. These MFHs were quite similar to those in humans histologically and electron microscopically. This work establishes an animal model for studying the pathogenesis of bone MFH.

4-Hydroxyaminoquinoline-1-oxide↗