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PubMed2003

Synergistic effects of adenosine A1 and P2Y receptor stimulation on calcium mobilization and PKC translocation in DDT1 MF-2 cells.

1. The effect of adenosine analogues and of nucleotides, alone or in combination, on intracellular calcium, accumulation of inositol (1,4,5) trisphosphate (InsP3), and on activation of protein kinase C (PKC) was studied in DDT1 MF2 cells derived from a Syrian hamster myosarcoma. These cells were found to express mRNA for A1 and some as yet unidentified P2Y receptor(s). 2. Activation of either receptor type stimulated the production of InsP3 and raised intracellular calcium in DDT1 MF2 cells. Similarly, the A1 selective agonist N6-cyclopentyladenosine (CPA) increased PKC-dependent phosphorylation of the substrate MBP(4-14) and induced a PKC translocation to the plasma membrane as determined using [3H]-phorbol dibutyrate (PDBu) binding in DDT1 MF-2 cells. However, neither adenosine nor CPA induced a significant translocation of transiently transfected gamma-PKC-GFP from the cytosol to the cell membrane. In contrast to adenosine analogues, ATP and UTP also caused a rapid but transient translocation of gamma-PKC-GFP and activation of PKC. 3. Doses of the A1 agonist CPA and of ATP or UTP per se caused barely detectable increases in intracellular Ca2+ but when combined, they caused an almost maximal stimulation. Similarly, adenosine (0.6 microM) and UTP (or ATP, 2.5 microM), which per se caused no detectable translocation of either gamma- or epsilon-PKC-GFP, caused when combined a very clear-cut translocation of both PKC subforms, albeit with different time courses. These results show that simultaneous activation of P2Y and adenosine A1 receptors synergistically increases Ca2+ transients and translocation of PKC in DDT1 MF-2 cells. Since adenosine is rapidly formed by breakdown of extracellular ATP, such interactions may be biologically important.

Adenosine↗
PubMed1989

Primary cardiac tumours--operative treatment of 20 patients.

This report describes a series of 20 patients operated on for a primary cardiac tumour. The majority of the tumours (16) were benign myxomas; 12 of them were located in the left atrium, two in the right atrium and two were biatrial. Two lipomas were found; one was epicardial and the other was located in the left atrium. The only intraventricular tumour was a malignant left ventricular myosarcoma. The propensity of intracardiac tumours to embolize was distinctive. Nine of the 16 myxomas presented with peripheral embolization, and in two patients surgery was complicated by fatal perioperative cerebral embolization of myxomatous tissue. Furthermore, in one patient embolization of a left atrial lipoma necessitated amputation of her left arm before cardiac surgery. Late postoperative recurrences were found in two patients with atrial myxomas. In one of them, reoperation showed that the tumour had grown at that site in the interatrial septum where the original pedicle had been excised. One patient developed severe mitral valve regurgitation and underwent replacement with a prosthetic valve at reoperation. Otherwise our late follow-up study showed that the results of surgery were usually excellent even though mild echocardiographic abnormalities were not uncommon. Our experience emphasizes the embolic potential of intracardiac myxomas and suggests, furthermore, that to avoid recurrences excisions with wide margins should be preferred. Echocardiography is an optimal method for the follow-up of these patients.

Echocardiography↗
PubMed1999

Aggressive congenital fibromatosis of the small intestine in the newborn. Report of a case and review of the literature.

A case of so-called congenital fibro(leio)myosarcoma of the small intestine in a 18-day-old female baby, treated only with surgical resection, was studied by immunohistochemistry and electron microscopy in order to investigate the proliferating cell type. The tumour cells showed positivity only for vimentin and CD 34 and were negative for smooth muscle actin, desmin, alpha-sarcomeric actin, factor VIIIR: Ag and S-100 protein. Ultrastructural findings showed oval nuclei with prominent nucleoli, rare intracytoplasmic mitochondria and well developed rough endoplasmic reticulum. According to histoimmunological and electron microscopy findings the proliferating cells were likely to be of fibroblastic origin. A 7-year follow-up showed a favourable clinical evolution thus confirming that surgical resection can be a sufficient therapeutic approach. The morphological findings and clinical behaviour suggest that more appropriate terminology for this tumour would be "aggressive congenital fibromatosis" which better highlights its local progressive invasion without metastases.

Female↗
PubMed2002

Prognostic factors and survival in late adolescent and adult patients with small round cell tumors.

The primary objective of this study is to review the clinical characteristics of 25 patients in the adult and late adolescent age group, diagnosed and treated with small round cell tumors involving soft tissues (extraosseous Ewing sarcoma, rhabdo-myosarcoma, primitive neuroectodermal tumor, and undiffer-entiated small round cell tumors). Additionally, survival and prognostic factors influencing the outcome with multimodality treatment are evaluated. There were 19 males (76%) and 6 females (24%). The median age was 26 years (range: 15-56 years). In 9 patients (36%), the tumor was located at an extremity, whereas 16 patients (64%) had central localizations. Tumor size was larger than 10 cm in 7 patients (29.2%). Six patients (24%) had metastatic disease. Twelve patients (48%) received radiation and 16 patients (64%) underwent surgery. Among the resected tumors, 2 were resected with contaminated margins (12.5%), whereas 2 were radically resected and 12 (75%) were resected with wide margins. All patients were given a median of 4 cycles of multiagent chemotherapy (1-14 cycles). With preoperative chemotherapy, complete regression (CR) of the tumor was achieved in 6 patients (24%). In 4 patients (16%), a partial response was obtained. After the completion of multimodality treatment, 12 patients (48%) had a CR. Progression-free (PFS) and overall survival (OS) for the entire group was 25.0 +/- 10.8% at 1 year and 30.5 +/- 15.5% at 3 years, respectively. Nonmetastatic disease, wide and radical resection, and presence of CR to multimodality treatment were associated with a significantly longer PFS and OS by univariate analysis. By multivariate analysis, CR to multimodality treat-ment was the only independent predictive factor for a longer OS (p: 0.0036, relative risk [RR]: 23.6, 95% CI: 2.8; 198.7) and metastatic presentation was the only independent factor predic-tive for a shorter PFS (p: 0.017, RR. 15, 95% CI: 1.6; 141.2). Large-scale, multicenter studies are required for a better eval-uation of the nonpediatric age group with small round cell tumors.

Adolescent↗
PubMed1995

Characterization of a novel human tumor necrosis factor-alpha mutant with increased cytotoxic activity.

Various novel recombinant human tumor necrosis factor-alpha (TNF) mutants were prepared using protein engineering techniques, and their cytotoxic activity was compared with that of the intact form of TNF (intact TNF). Mutant 471 (a TNF mutant molecule with the deletion of 7 amino acids at the amino-terminal and the substitution of Pro8Ser9Asp10 by ArgLysArg) had a 6-fold higher cytotoxic activity against murine L929 cells. The mutant TNF had an increased ability to bind to TNF receptor on murine L929 fibroblasts cells. A cross-linking study revealed that mutant 471 had an increased ability to form an active trimer. Mutant 471 also showed higher cytotoxic activity against human KYM myosarcoma cells and human MIA PaCa-2 pancreatic carcinoma cells. The possible cachectin activity of the mutant was almost the same as that of intact TNF. These results suggest that mutant 471 might be a more promising candidate as an anticancer agent than intact TNF.

Amino Acid Sequence↗
PubMed1979

Histopathological studies on experimentally induced pulmonary, pleural and peritoneal neoplasms in mice by intraperitoneal injection of chrysotile asbestos and N-methyl-N-nitrosourethane.

The cocarcinogenic effects of asbestos are presented. In lung carcinomas induced in mice, the number of carcinomas and the time of detection of the first carcinoma per tumor-bearing animals were greater and faster in the group with chrysotile plus MNU than either chrysotile or MNU alone. This suggested that chrysotile asbestos had a promoting or cocarcinogenic effect on some carcinogens in the respiratory tract. In the group treated with chryotile alone, a tumor was found in the right pleural cavity at 15 months. This tumor microscopically was similar to the biphasic form of the human diffuse mesothelioma. Microvilli, basement membrane, and junctional apparatus were seen by electron microscope, but other cytoplasmic organelles of the tumor cells were relatively scanty. Two peritoneal tumors developed in gastric and intestinal serosa at 11 and 12 months. Light and electron microscopic studies suggested that the tumors were probably myosarcomas or fibrosarcoms.

Adenocarcinoma↗
PubMed1982

Malignant mesenchymoma of the heart.

A case of a rare primary cardiac tumor in a 46-year-old woman is described. The tumor arose from the left atrium and was histologically composed of multiple mesenchymal elements of fibrosarcoma, rhabdomyosarcoma, chondrosarcoma, myosarcoma, and liposarcoma. Metastasis of this tumor occurred in the left femur, lung, and hilar lymph nodes after the second heart operation. Histogenesis of malignant mesenchymoma was considered with a survey of the literature.

Autopsy↗
PubMed1988

A case report of malignant mesenchymoma with discussion of musculoskeletal tumor staging: the Enneking system.

A 54-year-old black man presented with a soft-tissue sarcoma of the left anterior thigh. Surgical staging studies and initial biopsy results identified the lesion as a grade IIB pleomorphic liposarcoma. After radical hip disarticulation, follow up pathologic studies of the disarticulated limb showed the tumor to be confined to the anterior compartment of the left thigh without extracompartmental extension. The post-excisional surgical pathology report identified at least four different malignant mesenchymal elements: liposarcoma, myosarcoma, chondrosarcoma, and extraosseous osteogenic sarcoma. The sarcoma was therefore reclassified as a malignant mesenchymoma. The fact that the tumor was found to be intracompartmental at the time of surgery changed the staging of the tumor to stage IIA. A radical surgical margin, as recommended by Enneking, remained the treatment of choice. Three months postoperatively, the patient had chest pain and dyspnea. Chest films revealed multiple pulmonary nodules and the patient died of pneumonia 3 months later.

Femur↗
PubMed2000

Expression of MAGE-antigens in normal tissues and cancer.

The human MAGE gene family encodes products that can be recognized by autologous cytotoxic T cells. Because MAGE genes are silent in most normal tissues except testis but are activated in a variety of neoplastic lesions, MAGE antigens represent ideal targets for immunotherapy. Current knowledge of MAGE gene expression is based primarily on mRNA typing and relatively little is known about MAGE protein expression. Monoclonal antibody (MAb) 57B, originally thought to be specific for MAGE-3, but now known to be reactive with other MAGE components, was used in the present study to analyze MAGE expression in a panel of normal and malignant tissues. In tests with a wide range of normal tissues, only spermatogenic cells of testis were reactive with 57B. In tumor tissues, significant immunoreactivity was observed in malignant melanomas and carcinomas of the lung, head and neck as well as urinary bladder. No 57B reactivity was seen with colorectal, prostatic or renal cell carcinomas. Lipo- and myosarcomas, as well as malignant fibrous histiocytoma (MFH), were negative, but synovial sarcomas showed intense immunoreactivity. A subset of seminomas was also strongly reactive with 57B. Tumor specimens showed great variability in the number of tumor cells showing 57B reactivity, with some tumors showing only small isolated clusters of positive cells to other tumors with uniform staining throughout the tumor.

Antigens, Neoplasm↗
PubMed2000

[Primitive leiomyosarcoma of the right atrium presenting with a pulmonary embolism].

Primary Cardiac leiomyosarcoma is a very rare condition. The authors report the case of a 64 year old woman admitted to hospital for suspected pulmonary embolism, confirmed by thoracic angio-CT scan. Despite thrombolytic therapy, a persistent poor haemodynamic condition associated with a superior vena cava syndrome led to a transoesophageal echocardiographic examination which demonstrated an abnormal intra-right atrial mass obstructing the tricuspid valve. At surgery, a leio-myosarcoma was successfully resected under good conditions. During systematic three-monthly clinical and echocardiographic follow-up, transoesophageal echocardiography detected an asymptomatic local recurrence at 15 months, for which surgery was repeated with adjuvant chemotherapy.

Diagnosis, Differential↗
PubMed1976

Atypical fibroxanthoma of the skin. A clinico-pathological study of 57 cases.

A retrospective study of 57 patients with atypical fibroxanthoma of the skin is presented. The light microscopy is described and the differential diagnosis is discussed. Most of the atypical fibroxanthomas (32 out of 57 cases) were originally diagnosed as soft tissue sarcomas, e.g. fibrosarcoma, dermatofibrosarcoma, neurofibrosarcoma, myosarcoma and unspecified sarcoma. The tumour occurred chiefly in middle-aged and elderly patients; three patients were 15 years old or younger. The median age was 73 years in patients in whom the tumour occurred in the head and neck, and 34 years in patients in whom the tumour developed on the extremities and trunk. The sex ratio (male to female) was almost equal. Follow-up information about 43 patients was available. The follow-up period ranged from 1 year to 25 years with a median of 9 years. Eight patients died from intercurrent disease; all the other 35 patients are alive and well. The clinical course was benign in all but one patient in whom a recurrence developed and metastases to the regional lymph nodes appeared 7 years after the initial excision. The tumour in this case did not differ histologically from the other atypical fibroxanthomas with respect to cellularity, cellular and nuclear atypia or mitotic activity. It is suggested that the recurrence per se might be of prognostic importance.

Adolescent↗
PubMed1975

[Concentration of 99mTc-tin-phosphate complexes in soft tissues].

The concentration of 99mTc-pyrophosphate was determined in the lower extremities of rabbits (normal, abacterial and bacterial affected soft tissues), in osteoarthritis of the hip joint (capsule and muscle) as well as in knee joint effusions. Compared with the 85Sr-concentration, reflecting the calcification capacity, concentrations of 99mTc-pyrophosphate in soft tissues were found to be lower 2 hours p.i., but were up to elevenfold higher 24 hours p.i. These findings should be due to a fixation of 99mTc-pyrophosphate in collagen containing tissues as in the soft tissue tumors (myosarcoma, synvialioma, breast cancer) presented. A mechanism of delayed equilibration could explain augmented uptake in lymph-edema, ascites and effusions in florid osteoarthritis of the knee joint. The possible dependence of 99mTc-pyrophosphate concentration in bone and soft tissue on collagenous contents is discussed.

Animals↗
PubMed1975

[Ultrastructure of experimental virus-induced hemangiopericytomas].

Electron-microscopy studies of malignant low-differentiated hemangiopericytomas, induced by adenovirus of monkeys SA7(C8) and bovine adenovirus type 3 were, carried out. At earlier stages of the development of these tumours in tumourous cells located around capillaries there were observed the ultrastructural characteristics typical of pericytes and cells of human hemangiopericytis: the presence of the basal layer and fibrills as thick as 40 A. As differentiation proceeded, tumorous cells acquired an elongated form, and in some of them there appeared myofilaments typical of smooth-muscle cells, with local thickenings, as well as multitude of pinicytic vacules along the plasmatic membranes of cells. The foregoing confirms the assumption of hemangiopericytomas as a prestage of leio-(angioleio-) myosarcomas. Moreover, the tumour cells revealed the presence of cilia, fibrills as thick as 100A, 75A, annulate lamelae, and myelin-like formations.

Adenoviridae↗
PubMed1977

[Primary "muscle" tumors or tumors with a myosarcomatous component of the central nervous system. General review and attempt at classification based on 2 anatomo-clinical observations].

The authors report the first two French cases of primary rabdomyosarcoma of the central nervous system. They then review the literature on muscle tumours or primary myosarcomas of the nervous system of which 29 cases have now been published. From the point of view of morphology and classification, one should distinguish firstly, purely mesenchymatous tumours (rhabdomyosarcomas, leiosarcoma, mesenchymoma) and secondly, composite tumours of connective tissue or spino-epithelial tumours (medullomyoblastomas, neuromyoblastomas, gliomyosarcomas). Their histogenesis occurs from the ectomesenchyme of the neural crests.

Age Factors↗
PubMed1990

[Vascular tumors of the liver. Morphology, differential diagnosis, prognosis].

A group of 5 primary angiomas of the liver (4 angiosarcomas, 1 cavernous hemangioma) was studied. Classification, differential diagnosis and pathogenesis are discussed. Angiosarcomas may be caused by exposure to thorotrast (2 of our 4 tumors) or of polyvinylchloride. Highly cellular tumors with a herringbone-pattern must be differentiated from primarily extrahepatic fibrosarcomas or myosarcomas, and highly cellular solid or medullary tumors from metastasis from a carcinoma or lymphoma. Immunohistology--also in our tumors--permits the identification of angiomas by the demonstration of factor VIII and basement membrane protein, which are negative in other tumors. The binding of lectin Ulex europaeus can also be helpful. The common benign cavernous hemangioma is diagnosed very readily morphologically, but clinical and sonographic diagnosis can be difficult. Its biological significance is to be seen merely in possible rupture and hemorrhage.

Adult↗
PubMed1988

Application of monoclonal antibodies to intermediate filament proteins in surgical pathology of head and neck tumours. An overview.

Intermediate filament proteins are distributed in a tissue specific manner throughout human tissues. Using monoclonal or polyclonal antibodies to cytokeratins, vimentin, desmin, neurofilament proteins or the glial fibrillary acidic protein, epithelial, mesenchymal, myogenic, nervous and glial tissues, respectively, can be distinguished by immunohistochemical techniques. Since tumour cells generally retain the intermediate filament proteins typical for their cells of origin, such antibodies can also be used to discriminate between different types of neoplasma, i.e. carcinoma, lymphoma, myosarcoma, etc. Furthermore, monoclonal antibodies to individual cytokeratin proteins can be used to distinguish between several types of epithelial tissues and different types of carcinomas. The application of such antibodies in the histopathology of head and neck tumours can be of great help in the characterization of tumours that cannot be identified on the basis of routine histological techniques.

Antibodies, Monoclonal↗
PubMedDate not supplied

[Multiple, histologically varied malignancies in a case of Xeroderma pigmentosum with prolonged evolution by surgical interventions].

On the occasion of a case of Xeroderma pigmentosum followed for a period of 12 years the major data concerning this rare affection are presented. This is an autosomal genodermatosis characterized by hypersensitivity of the skin to certain ranges of the UV light spectrum, and the development of lesions especially on the face. The treatment is essentially preventive and consists in avoiding exposure to UV radiation, and also palliative, consisting in medical therapy, physiotherapy and surgery. The patient was followed and treated for a long period of time and developed in succession baso-cellular epitheliomas, spino-cellular epitheliomas, malignant melanoma, myosarcoma, and lymph-node metastases. Due to the extension of the lesions surgery was applied with good vital results, and also with satisfactory functional and esthetic results.

Adult↗