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Primary intracranial sarcomas: radiological diagnosis with emphasis on arteriography.

The radiological manifestations of primary intracranial sarcomas may be non-specific and they vary widely according to their histological variations. Reticulum cell sarcomas, however, can be included in the differential diagnosis with a high degree of reliability when one observes a hypovascular mass with arterial encasement and deep medullary veins. Tumor vessels and tumor stains supplied by dilated feeding arteries suggest the possibility of a sarcoma of the brain or of the meninges in infants. Nine reticulum cell sarcomas, four undifferentiated sarcomas, and one fibrosarcoma are presented here with their radiological manifestations, especially on angiography.

Adolescent↗

Infantile and adult fibrosarcomas of the soft tissues.

Histologic sections of 68 soft-tissue sarcomas initially diagnosed as fibrosarcoma were reviewed, and 36 were excluded because of revised diagnosis. The tumors from the remaining 32 patients were analyzed clinicopathologically, and were classifed into two types; the adult type (22 cases) and the infantile type (10 cases). The adult type fibrosarcoma occurred in adults aged 25 to 67 years and consisted of spindle-shaped fibroblastic cells which formed interlacing bundles accompanied by variable amounts of collagen or reticulin fibers. The infantile fibrosarcoma affected children below the age of seven years in this series and was characterized by proliferation of immature fibroblasts forming indistinct bundles, frequently exhibiting areas of an angiosarcoma-like pattern and cavernous blood vessels. The authors expressed the view that infantile fibrosarcoma should be separated from adult fibrosarcoma, because between these two types of fibrosarcoma there were marked differences in the histologic feature as well as in the age, sex and anatomical distributions.

Adult↗

Separation of cells from mouse solid tumors by centrifugal elutriation.

Centrifugal elutriation was used to separate cells dissociated from two hypotetraploid mouse solid tumors, a fibrosarcoma and a sarcoma derived from L-P59 cells, based on their sedimentation rates. The separation was rapid, requiring less than 1 hr; yielded about 80 percent cell recovery; and resulted in little loss of cell viability. Aanlysis of DNA content by flow cytometry demonstrated the synchrony obtained with these tumor cells. The fractions with the lowest sedimentation rates contained predominantly normal cells, those with intermediate sedimentation rates contained predominantly tumor cells in the G1 phase of the cell cycle, and those with the highest sedimentation rates contained mostly tumor cells in S or G2. The clonogenicity of L-P59 cells, assayed in culture, markedly increased with increasing sedimentation rates. In contrast, the clonogencity of fibrosarcoma cells, assayed in vivo by a lung colony assay, was lower for the smaller cells, but was essentially constant among the larger cells. Autoradiography of cells labeled in vivo with tritiated thymidine demonstrated no differences in the proportions of cycling cells in various fractions. These results demonstrate that subpopulations differing in cell type, phase of the cell cycle, and clonogencity can be rapidly separated from solid tumors by centrifugal elutriation.

Animals↗

Radiation-associated sarcomas are characterized by complex karyotypes with frequent rearrangements of chromosome arm 3p.

Ionizing radiation is a well-known risk factor for sarcoma development. To investigate whether radiation-associated sarcomas are characterized by chromosome aberrations that distinguish them from de novo sarcomas, we identified those patients in our series of more than 500 cytogenetically abnormal sarcomas that fulfilled the following criteria: (1) each patient should have been irradiated for another malignancy at least 3 years prior to the sarcoma diagnosis, and (2) the sarcoma should have developed within the field of radiation. Ten patients fulfilling these criteria could be retrieved (median age at sarcoma diagnosis was 55 years, range 17-79; median latency period between primary tumor and radiation-associated sarcoma was 9 years, range 4-30). The diagnoses were typical for radiation-associated sarcomas: 2 each of malignant fibrous histiocytoma, leiomyosarcoma, and pleomorphic sarcoma, and 1 each of osteosarcoma, fibrosarcoma, myxofibrosarcoma, and spindle cell sarcoma. All 10 cases had relatively complex karyotypes with multiple, mostly unbalanced, structural rearrangements, similar to what has been reported in de novo sarcomas of the corresponding histologic subtypes. The only cytogenetic features that were unusually frequent among the radiation-associated sarcomas were the finding of unrelated clones in 3 cases, and loss of material from chromosome arm 3p, in particular 3p21-3pter, in 8 cases. Loss of the same chromosome segment has been described in 4 of the 8 previously published cases of radiation-associated sarcomas that have been analyzed after short-term culturing, which makes this imbalance significantly (P < 0.001) more frequent among radiation-associated sarcomas (12 of 18 cases) than among unselected cases of the corresponding histologic subtypes (74 of 282 cases). In contrast to the cytogenetic results, no 3p deletions were detected among the 6 cases of the present series that could be analyzed by comparative genomic hybridization (CGH). The most frequent imbalance detected by CGH was gain of 15cen-q15 (3 cases), followed by loss of chromosome 13 and gain of 5p, and 7cen-q22, each detected in 2 cases.

Adolescent↗

Place of resection in the management of primary bone tumours.

A primary malignant sarcoma of bone in an extremity must be removed so that it will not recur locally. Amputation will accomplish this in most cases but a prosthesis is required. In carefully selected cases of malignant giant cell tumour, low-grade chondrosarcoma, low-grade fibrosarcoma, parosteal osteogenic sarcoma and adam-antinoma, resection with insertion of an autograft, allograft or metallic implant has been successful. More recently implants have been used following resection of the more malignant tumours such as osteosarcoma, high-grade fibrosarcoma and high-grade chondrosarcoma, but further time is required to determine the problems secondary to chemotherapy, other complications, the length of time for rehabilitation, and the quality of life after this procedure as compared with the more conventional amputation with early prosthetic titting.

Adult↗

Treatment of bone and soft tissue malignant tumours of the extremities by radical resection. A preliminary report of 12 cases.

Radical local resection is undoubtedly the method of choice in treating malignant bone and soft tissue tumours in the extremities, provided there is no local recurrence after radical resection. This is even truer today when the chemotherapy of osteosarcoma has achieved encouraging advances in preventing lung metastasis. The diagnostic methods for the evaluation of the tumour infiltration are at a more complete stage, and operative reconstruction techniques have als made rapid progress. Radical local resection is especially suitable for sarcomata of the extremities which are at an early stage, with less infiltrative low-grade malignancy. Amputation is better when the tumour grows rapidly and is large, if the soft tissue is widely infiltrated, if the patient is from 20 to 30 years old, and especially if the tumour is located in the upper tibia where it is difficult to carry out local resection. In this article, we report and discuss the definition, necessity, and possibility of radical local resection as well as the method of surgical reconstruction and our results. The results and prognosis for radical resection of giant-cell sarcoma, chondrosarcoma, and fibrosarcoma are rather good. Three of five cases of osteosarcoma died of lung metastasis one year after surgery. Therefore, improving the results in local resection of osteosarcoma calls for further investigation.

Adolescent↗

Fine needle aspiration cytology of sarcomas metastatic to the lung.

A review was made of the morphologic features of cells aspirated from 17 sarcomas (5 malignant fibrous histiocytomas, 3 fibrosarcomas, 3 leiomyosarcomas, 3 endometrial stromal sarcomas, 1 osteosarcoma and 2 poorly differentiated sarcomas) metastatic to the lung, paying particular attention to the morphologic differences between the cells of sarcoma and carcinoma and between the cells of the different types of sarcoma. In general, sarcomas were characterized by loosely cohesive, rather flat, cellular aggregates and isolated cells. Three-dimensional cell balls or clusters were not present in any case. Cellular pleomorphism was a common, though not invariable, feature. Each type of sarcoma showed some morphologic distinctiveness; however, certain morphologic features were common to more than one type of sarcoma. By comparing the cytologic features of metastatic sarcomas in aspirates with the histologic features of the primary neoplasms, it should usually be possible to decide if a pulmonary lesion is a metastatic sarcoma.

Biopsy, Needle↗

The effect of 1, 25-dihydroxyvitamin D3 on the growth of soft-tissue sarcoma cells as mediated by the vitamin D receptor.

BACKGROUND: Soft-tissue sarcomas, malignant neoplasms originating from mesenchymal tissue, are rare but highly aggressive tumors. Present modes of therapy are associated with high rates of recurrence. 1, 25-Dihydroxyvitamin D3, the active metabolite of vitamin D, serves as a potent antiproliferative agent in human cancer cells. METHODS: In this study, six soft-tissue sarcoma cell lines were analyzed for vitamin D receptor (VDR) expression, which was then correlated with the degree of growth inhibition in response to 1, 25-dihydroxyvitamin D3. These cell lines included rhabdomyosarcoma (HS729, A204), fibrosarcoma (HS913t), synovial sarcoma (SW982), liposarcoma (SW872), and leiomyosarcoma (SKLMS-1). The level of VDR messenger RNA (mRNA) expression was determined using a ribonuclease protection assay, and functional receptor content was determined by using a ligand-binding assay. Growth studies, including [3H]thymidine uptake and growth curves, were performed on two of the six cell lines that expressed the highest and lowest receptor levels. RESULTS: Ribonuclease protection and ligand-binding assays demonstrated variable levels of VDR, with HS729 showing high expression and A204 showing no expression. In HS729, [3H]thymidine uptake was significantly decreased at 10(-7) M (33%) and 10(-6) M (40%) 1, 25-dihydroxyvitamin D3. Growth curve studies showed significant growth inhibition of 55% at 10(-6) M. A204 cells showed no growth inhibition upon treatment with 1, 25-dihydroxyvitamin D3. CONCLUSION: This study demonstrates the existence of VDR in soft-tissue sarcoma cells and suggests a correlation between the level of VDR in cells and the degree of growth inhibition caused by 1, 25-dihydroxyvitamin D3 which may potentially serve as an alternative form of therapy for soft-tissue sarcomas.

Calcitriol↗

Isolation and analysis of lectin-reactive sarcoma-associated membrane glycoproteins.

Sarcoma and normal tissue plasma membrane lectin-reactive glycoproteins were analyzed by sodium dodecyl sulfate polyacrylamide gel electrophoresis. Two peanut agglutinin-reactive N-acetylgalactosamine-containing glycoproteins of 1.05 x 10(6) and 1.25 x 10(5) Da and one lentil agglutinin-reactive mannose/N-acetylglucosamine(-fucose)/sialic acid-containing glycoprotein of 1.7 x 10(5) Da (Gp170) were detected in osteosarcoma and malignant fibrous histiocytoma (MFH), respectively. However, these glycoproteins were not detected in normal tissue plasma membranes. Concanavalin A, wheat germ and Ulex europaeus Type I agglutinins did not reveal any unique sarcoma-associated membrane glycoproteins. Preliminary studies on monoclonal antibodies (mAbs) generated against Gp170 (mAb 64-35-84) and against lentil-reactive glycoproteins from MFH (mAbs 67-34 and 67-117) revealed high specific binding to a number of membranes isolated from MFH and osteosarcoma tissues, with no crossreactivity to normal human tissues tested (liver, spleen and skin). Detailed analysis of mAb 67-102, which was generated against lentil-reactive glycoproteins isolated from MFH plasma membranes, exhibited significant binding to membranes isolated from osteosarcoma, liposarcoma and MFH; moderate binding to synovial sarcoma, aggressive fibromatosis and fibrosarcoma; and minimal to no binding to other soft tissue sarcoma plasma membranes. No binding was observed to twenty normal tissue specimens, with the exception of low positive binding to two of five fat and two of three colon specimens.

Adult↗

Metastatic alveolar soft part sarcoma presenting as a dural-based cerebral mass.

Sarcoma metastatic to the brain is uncommon and rarely occurs as the initial manifestation of tumor. Alveolar soft-part sarcoma, a rare but well studied subtype of a soft tissue sarcoma with a propensity for central nervous system invasion, presenting with brain metastases, has been reported only once previously. We report the case of a 28-year-old man who presented with partial seizures and who was found to have a homogeneously enhancing frontal lesion on a broad dural base disclosed by computed tomography. preoperatively, the lesion was thought to be a meningioma. The tumor was excised easily and had features typical of an alveolar soft-part sarcoma, which were revealed by light and electron microscopy as well as immunohistochemical analysis. Multiple lung nodules compatible with metastases were found on a chest film. Meningeal dissemination has been reported in a variety of sarcoma types, including rhabdomyosarcoma, fibrosarcoma, and leiomyosarcoma. We add alveolar soft-part sarcoma to this list and suggest that increased recognition of the propensity for these tumors to exhibit metastatic spread to the dura should eliminate diagnostic confusion and provide an earlier diagnosis of these rare lesions. The patterns of spread in metastatic sarcoma deserve further study.

Adult↗

[Introduction to x-ray diagnosis of primary malignant bone tumor (author's transl)].

Diagnosis of primary malignant bone tumors is based on a synopsis of X-ray findings, clinical symptoms, gross pathology and histology. Osteosarcoma, chondrosarcoma, fibrosarcoma and myelogenic sarcomas have a typical symptomatology and can be diagnosed in the radiograph by localisation, mode of extension, alterations of bone and periosteum.

Adolescent↗

Propagation in human cells of a filterable agent from the ST feline sarcoma.

Nineteen lines of human fibroblasts were inoculated with a filtrate prepared from the ST feline sarcoma. Seven lines showed morphological alteration and released focus-forming activity for feline cells, 2 lines showed morphological alteration but did not release focus-forming activity, and 11 lines showed no morphological alteration and released no focus-forming agent. Morphologically altered cells appeared enlarged, hyper-refractile, and intensely stained by hematoxylin. They neither assumed a crisscross pattern nor piled up to form a visible focus. Time-lapse cinegraph showed that the morphologically altered cells did not divide and were motile. The fluid from two human fibroblast cultures, inoculated 4 and 14 weeks previously with the ST sarcoma filtrate, induced fibrosarcoma in newborn kittens.

Animals↗

ST-feline fibrosarcoma virus: induction of tumors in marmoset monkeys.

Two newborn marmosets, inoculated with a cell-free extract of feline fibrosarcomas, developed multiple sarcomas and died within 46 days of inoculation, whereas two of these animals inoculated with a crude homogenate developed no tumors. This susceptibility to a mammalian RNA sarcoma virus suggests that marmosets may be particularly suitable for attempts to isolate infectious agents from man.

Animals↗

Leech salivary gland extract from Haementeria officinalis, a potent inhibitor of cyclophosphamide- and radiation-induced artificial metastasis enhancement.

Studies were designed to determine whether salivary gland extract (SGE) from the leech Haementeria officinalis could inhibit enhancement of lung tumor colonization induced by pretreatment of mice with cyclophosphamide (CY) or local thoracic irradiation (LTI). Tumor nodules in the lung were generated by i.v. injections of T241 sarcoma and FSA fibrosarcoma cells into syngeneic C57BL/6 and C3Hf/Kam mice, respectively. CY (200 mg/kg) was given i.p. 1 or 4 days prior to i.v. injection of tumor cells. In other mice, a single dose of 1000 rads of LTI was given 1 day before tumor cells. Three i.v. or i.p. injections of SGE at doses of 600 to 800 micrograms of protein per injection given at 2-hr intervals between 2 hr before and 4 hr after CY, LTI, or tumor cell injection strongly inhibited and, in some cases, abolished the artificial metastasis enhancing effect of CY and LTI. SGE was similarly effective in inhibiting the enhancement of lung colonization when given before or after cytotoxic agents. Using [125I]iododeoxyuridine-labeled tumor cells, it was observed that SGE did not affect the initial lodgement of tumor cells in the lung, but it greatly facilitated their subsequent release from the lung. In normal mice, the SGE was active when given on the day or 1 day before but not when given 4 days before tumor cells. The antimetastatic effect of SGE was ascribed to its anti-platelet-aggregating, anticoagulant, and antiproteolytic enzyme activities.

Animals↗

Ether à go-go potassium channel expression in soft tissue sarcoma patients.

BACKGROUND: The expression of the human Eag1 potassium channel (Kv10.1) is normally restricted to the adult brain, but it has been detected in both tumour cell lines and primary tumours. Our purpose was to determine the frequency of expression of Eag1 in soft tissue sarcoma and its potential clinical implications. RESULTS: We used specific monoclonal antibodies to determine the expression levels of Eag1 in soft tissue sarcomas from 210 patients by immunohistochemistry. Eag1 was expressed in 71% of all tumours, with frequencies ranging from 56% (liposarcoma) to 82% (rhabdomyosarcoma). We detected differences in expression levels depending on the histological type, but no association was seen between expression of this protein and sex, age, grade or tumour size. Four cell lines derived from relevant sarcoma histological types (fibrosarcoma and rhabdomyosarcoma) were tested for Eag1 expression by real-time RT-PCR. We found all four lines to be positive for Eag1. In these cell lines, blockage of Eag1 by RNA interference led to a decrease in proliferation. CONCLUSION: Eag1 is aberrantly expressed in over 70% sarcomas. In sarcoma cell lines, inhibition of Eag1 expression and/or function leads to reduced proliferation. The high frequency of expression of Eag1 in primary tumours and the restriction of normal expression of the channel to the brain, suggests the application of this protein for diagnostic or therapeutic purposes.

Adolescent↗

Circumscribed spontaneous heterotopic ossification in the soft tissues simulating sarcoma.

Eight cases are described of circumscribed heterotopic ossification in the soft tissues characterised by spontaneous onset, no history of trauma, and rapid course which have frequently led to a mistaken preoperative diagnosis of highly malignant tumour. The terminology differentiates it from myositis ossificans, which is a different entity. The authors emphasize the need for accurate evaluation of all the clinical and radiographic data in order to avoid an erroneous diagnosis of extraosseous osteogenic sarcoma or parosteal osteogenic sarcoma. In this regard, both radiography and histological examination demonstrate the so-called zoning pattern, namely a radiolucent central zone corresponding to the more immature area and consisting of fibrous tissue with active histiofibroblastic proliferations and a radiopaque peripheral area, where ossification is more mature the closer it gets to the periphery. Histological specimens obtained exclusively from the central area may lead even the most expert pathologists to a diagnosis of fibrosarcoma or osteogenic sarcoma. Finally, the pathogenetic aspects of the anomaly are discussed, as well as those concerned with the differential diagnosis from other types of heterotopic ossification.

Adolescent↗