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PubMedDate not supplied

Amplification of the c-myc proto-oncogene in soft tissue sarcomas.

Soft tissue sarcoma fresh specimens from 23 patients were screened for genetic abnormalities of 3 cellular oncogenes, i.e., c-myc and c-myb and c-Ha-ras. Southern blot hybridization analysis demonstrated amplification of the c-myc gene (4- to 8-fold) in 7 cases, i.e., 3 malignant fibrous histiocytomas, 1 liposarcoma, 1 fibrosarcoma, 1 lymphoma of the soft tissues and 1 synovial sarcoma. Amplification of the c-myc gene was not associated to other genetic alterations such as gene rearrangement. Hybridizations with c-myb and c-Ha-ras probes showed no gene abnormalities in this series. Our results indicate that c-myc oncogene amplification may play an important role in the development of certain soft tissue sarcomas as it has been found for other human malignancies. The clinical significance of these features remains to be established.

Adult↗
PubMed2005

Understanding mesenchymal cancer: the liposarcoma-associated FUS-DDIT3 fusion gene as a model.

Chromosomal translocations entail the generation of gene fusions in mesenchymal tumors. Despite the successful identification of these specific and consistent genetic events, the nature of the intimate association between the gene fusion and the resulting phenotype still remains to be elucidated. Here these studies are reviewed, using FUS-DDIT3 as a model to illustrate how they have contributed to current understanding in unique and unexpected ways. FUS-DDIT3 is a chimeric oncogene generated by the most common chromosomal translocation t(12;16)(q13;p11) associated with liposarcomas. The application of transgenic methods to the study of this sarcoma-associated FUS-DDIT3 gene fusion has provided insights into their functions in vivo, and suggested mechanisms by which lineage selection may be achieved.

Animals↗
PubMed2002

Giant retroperitoneal sarcoma: a case report and review of the management of retroperitoneal sarcomas.

A 42-year-old man presented with lower abdominal pain and a vague abdominal mass. He underwent resection of a well-differentiated liposarcoma arising from his retroperitoneum measuring 50 cm and weighing 11.7 kg (25.8 lb). This is the second largest retroperitoneal soft-tissue sarcoma (RSTS) that has been reported. Over the last 15 years 1123 patients with RSTS in 25 series have been reported with a mean tumor size of 15.7 cm. RSTS represents 0.10 to 0.15 per cent of all malignancies but 45 per cent of all retroperitoneal tumors. Diagnosis and treatment of RSTS can be extremely challenging for a general surgeon. Symptoms are nonspecific and may occur only after the tumor is very large. Abdominal discomfort is the presenting complaint in 60 to 70 per cent of patients and palpable mass in 70 to 80 per cent. Treatment of RSTS remains surgical. Multiple trials of chemotherapy and radiation therapy show no survival benefit. The only successful treatment of this tumor is complete excision; 51.4 per cent of tumors can be completely excised, and 50.2 per cent of these excisions include adjacent organs. Long-term prognosis without complete excision is grim with average 5- and 10-year survival rates of 16.7 and 8.0 per cent. With aggressive surgical therapy survival is increased to 58.0 and 39.6 per cent.

Adult↗
PubMed1984

[Computed tomography in primary mesenteric tumors].

The value of CT in the investigation of mesenteric tumours was studied in 21 patients. It is possible to differentiate mesenteric cysts from sarcomas, and CT provides information concerning the size, localisation, structure and extent of the tumour and the degree of infiltration. Density measurements provide evidence concerning the structure of the lesion, whether it is liquid, lipomatous, myxoid or osseous. Only rarely is it possible to make a histological diagnosis. Localised infiltration of the gut cannot be recognised, but diffuse intra- and retro-peritoneal tumour spread can be seen by CT. Ascites, intra-peritoneal and haematogenous metastases are rare from mesenteric sarcomas.

Adolescent↗
PubMed1995

[Intraoperative radiotherapy within the treatment concept of retroperitoneal soft tissue sarcomas].

Sarcomas of the retroperitoneum are characterized by a high rate of local recurrence. External beam radiotherapy is known to improve local control after surgical therapy. To increase local dosage of radiotherapy without affection of sensitive structures we applied intraoperative radiotherapy (IORT) since 1991 in a dedicated operative unit. To compare morbidity and tumor control we used a partly historic control group of patients treated since 1988 for retroperitoneal soft tissue sarcoma. 25 patients with a mean age of 53 years were operated. Tumor histology was dominated by liposarcoma and leiomyosarcoma, UICC stage IIB (T2 G2 N0 M0) was present in 45% of cases. Distant metastasis were diagnosed in 19% at therapeutic intervention. Tumor free margins were achieved in 55% while 29% showed microscopic and 16% macroscopic tumor residues after surgical intervention. 11 patients received IORT with a mean of 18 Gy, eight of those patients were treated additionally with a mean of 40,4 Gy externally. There were no differences in distribution of known risk factors for recurrence in the group of patients treated with or without IORT. The analysis showed no difference for perioperative morbidity. Tolerance for IORT and additional external beam radiotherapy was good. Local tumor control tended to be improved by IORT (p = 0.082) while overall survival was not affected at a mean follow-up of 24 months.

Adolescent↗
PubMed2005

Dynamic contrast enhanced MRI in the differential diagnosis of soft tissue tumors.

PURPOSE: The value of the dynamic contrast enhanced-magnetic resonance imaging (DCE-MRI) in differentiating benign and malignant soft tissue tumors was investigated. MATERIALS AND METHODS: Turbo FLASH DCE-MRI was performed on 22 subjects (2-74 years) with soft tissue tumors. Enhancement in the first min (E(max/1)), second min (E(max/2)) and maximum peak enhancement (E(max)), and steepest slope were calculated. Discriminant analyses were performed to reveal parametric differences of benign and malignant lesions. RESULTS: Diagnosis of benign (N = 10) tumors were hemangioma (n = 3), neurogenic tumor (n = 3) lipoma (n = 2), giant cell tumor (n = 1) and desmoid (n = 1), whereas malignant lesions (N = 12) were classified as liposarcoma (n = 5), malignant fibrous histiocytoma (n = 5) and synovial sarcoma (n = 2). For malignant lesions E(max/1) was 65-198%, E(max/2) was 65-145%, E(max) was 78-198%, and steepest slope was 1.45-4.06. For benign lesions these values were 4-98%, 5-105%, 7-125% and 0.67-2.57, respectively. To determine the relation between the variables analysed, Pearson correlation coefficients were calculated. E(max) was found to be highly correlated with other variables (rxy > 0.86, P < 0.0001). Consequently, this variable was excluded from the discriminant analysis. In order to determine discrimination of malignant and benign tumors using E(max/1), E(max/2,) and steepest slope of the enhancement curve logistic regression was applied to the above mentioned data. When combined these parameters had a 95.5% of overall accuracy in classifying benign and malignant lesions (P = 0.004). CONCLUSION: DCE-MRI parameters that thought to be the surrogate markers of tumoral microcirculation and tissue perfusion provides a specific preoperative diagnosis. Dynamic imaging parameters are therefore advocated for monitoring the effect of chemotherapy in soft tissue tumors.

Adolescent↗
PubMed1996

Cytogenetics and experimental models.

Cytogenetic analysis plays a direct, potentially decisive role in the examination of benign and malignant bond and soft tissue tumors. Translocations, or the exchange of chromosomal material between two or more nonhomologous chromosomes, are frequently encountered as tumor-specific anomalies in mesenchymal neoplasms. The consequences of many of these translocations has been the formation of chimeric proteins with features characteristic of altered transcription factors. Recent studies have revealed new tumor-specific translocations (eg, cemento-ossifying fibroma and myxoid chondrosarcoma) and the association of established translocations, eg, t(11;22) and t(12;16), in rare histologic subtypes such as biphenotypic sarcomas with myogenic and with neural differentiation and round cell liposarcoma, respectively. In contrast to a single translocation, many mesenchymal neoplasms such as malignant fibrous histiocytoma and osteosarcoma are characterized by multiple, often complex, chromosomal abnormalities. Recent advances in molecular cytogenetics, eg, in situ hybridization and comparative genomic hybridization, have made the identification of many of these abnormalities possible and in some instances, have revealed chromosomal imbalances not detectable with traditional cytogenetic analysis.

Chromosome Aberrations↗
PubMed1975

[Ultrasound diagnosis of bone and soft tissue tumours].

The findings of ultrasonic examination in bone and soft tissue tumours are evaluated for their usefulness in preoperative tumour diagnosis, with particular reference to size, demarcation, structure, as well as the topographic situation of the tumour. Furthermore, the results of ultrasonic tomograms are compared with those of X-rays and angiography. It seems that this simple method, which is without risk to the patient, is a valuable additional investigation in the diagnosis and therapy of tumours of bone and soft tissue.

Bone Neoplasms↗