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[Difficult diagnosis of fever of unknown origin related to an infraclinic liposarcoma: "small tumor and fever of unknown origin"].

INTRODUCTION: Palpation of soft tissues constitutes part of the physical examination when faced with a patient with fever of unknown origin. Our case highlights the difficulty of diagnosis when the tumor still remains impalpable ten months after onset of fever and severe biological inflammatory process. EXEGESIS: A 49-year-old woman was admitted for fever of unknown origin associated with important biological inflammatory process. In view of her past medical history of breast carcinoma, a search for a relapse was performed and remained negative. Despite multiple investigations and repeated physical examinations, no diagnosis was obtained. Finally, [18F] fluorodoexyglucose positron emission tomography led to a diagnosis showing a high fixation located in the right thigh, related to a myxoid liposarcoma after surgical resection. CONCLUSION: Dramatic regression of fever and biological inflammatory process after surgical treatment illustrates the concept of "small tumor and fever of unknown origin", an exceptional entity but which physicians should be aware of. The role of new technical imaging with [18F] fluorodoexyglucose positron emission tomography has however to be clarified in the evaluation of fever of unknown origin.

Breast Neoplasms↗

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↗

Hand-assisted laparoscopic removal of retroperitoneal liposarcoma.

Liposarcoma is known to be the most common soft-tissue sarcoma, and the treatment of retroperitoneal disease is surgical excision. We present a case of complete surgical removal with concomitant nephrectomy using a hand-assisted laparoscopic approach and provide a review of the published studies.

Aged↗

[Neck liposarcoma. Presentation of one case and revision of the literature].

Neck liposarcoma is an infrequent tumor in the head and neck area. A case in a 70 years male is reported with a presentation the in inferior third of the lateral neck region. Two previous surgical procedures and Radiotherapy (50 Gy) were carried out. A modified radical dissection was performed. No metastatic nodes were found. A revision of the literature is done with special remarks on prognostic factors; histology and surgical treatment with wide margins are favourable factors.

Aged↗

[Retroperitoneal liposarcoma. Case report].

We report one case of retroperitoneal liposarcoma treated by surgery and radiotherapy. With the literature, we discuss the pathologic and therapeutic aspect of this lesion.

Diagnosis, Differential↗

Chest wall implantation of a mediastinal liposarcoma after thoracoscopy.

We report a case of mediastinal liposarcoma resected by thoracoscopy. Despite the precautionary measures, chest wall implantations occurred rapidly at the port's sites in the chest wall and led to death within 24 months. We conclude that thoracoscopy is not a good approach for resection of anterior mediastinal masses in view of their possible malignant character.

Adult↗

Case report: recurrent liposarcoma of the oesophagus.

A case of recurrent liposarcoma of the oesophagus is presented. The clinical and radiological findings are discussed. Our case displays the characteristic features and behaviour of a common sarcoma arising in a rare site.

Esophageal Neoplasms↗

Renal liposarcoma: case report.

A case of renal liposarcoma is described. The clinical presentation, angiographic findings and treatment are discussed.

Cyclophosphamide↗

Malignant soft tissue tumors (malignant fibrous histiocytoma, pleomorphic liposarcoma, and pleomorphic rhabdomyosarcoma): an electron microscopic study.

A comparative ultrastructural analysis of malignant soft tissue tumors (malignant fibrous histiocytoma, pleomorphic liposarcoma, and pleomorphic rhabdomyosarcoma) revealed similar ultrastructural features in this group of tumors. However, by electron microscopy these tumors can be differentiated on the basis of cytoplasmic and extracytoplasmic features (myosin filaments, lipid droplets, and perinuclear intermediate filaments, for example). This is even true of less well differentiated tumors and tumor cells. These findings support and amplify the concept of a common histogenesis for tumors of mesenchymal origin. Paradoxical features observed by light microscopy warrant further study by electron microscopy if the correct diagnosis is to be made in atypical cases, such as apparent malignant fibrous histiocytoma with cross striations.

Adult↗

A well-differentiated liposarcoma with a new type of chromosome 12-derived markers.

Well-differentiated liposarcomas (WDLPS) are cytogenetically characterized by the presence of supernumerary ring or giant rod marker chromosomes. These supernumerary chromosomes are composed of amplified sequences from chromosome 12 (12q14 approximately 15) in association with amplified segments from various other chromosomes, and contain alterations of the alpha satellite sequences. We report a case of WDLPS of the lipoma-like and sclerosing subtype that contains a novel type of supernumerary marker chromosome. Instead of rings or giant rods, these cells had three apparently identical copies of a subtelocentric supernumerary marker with a size and shape similar to C-group chromosomes. Fluorescence in situ hybridization analysis revealed that the markers were composed of amplified material from 12q14 approximately 15, including the genes MDM2 and CDK4. Similar to the rings and giant rods observed in other WDLPS cases, these unusual markers had no alpha satellite repeats at the primary constriction site, but centromeric activity could be demonstrated by using anti-centromere protein C antibodies. These findings show that the supernumerary markers of WDLPS may be variable in size and shape, but consistently share the same genomic structure, specifically 12q amplified sequences together with centromere alterations, and underline the importance of molecular methods in the diagnosis of adipose tissue tumors.

Aged↗

[Calcified retroperitoneal liposarcoma].

Case report of a retroperitoneal liposarcoma pleomorfico with calcification in diagnosed in our hospital. We comment on its clinical characteristics, diagnostic methods and treatment. We likewise comment the possible differential diagnoses.

Aged↗

Liposarcoma of the cheek in an infant.

A case of liposarcoma of the cheek in a 6-month-old infant has been presented. This tumor, one of the most common malignant tumors of soft tissue in the body generally, is exceedingly rare in the head and neck regions, having been reported in this location on only a few occasions. A review of the literature reveals that this patient was one of the youngest so affected. The postoperative course was uneventful, and after three years no evidence of recurrence or metastasis has been observed.

Cheek↗

A histochemical and biochemical study of a liposarcoma with several aspects on the development of fat synthesis.

The report describes a case of lipocytic and lipoblastic liposarcoma, morphologically to be classified as well-differentiated. A considerable number of liopoblastic areas with the mass of the tumour proper which contain large amounts of glycogen are interspersed. These immature lipocytes reproduce the histogenesis of normal white fat. The quantity of glycogen-storing lipoblastic cells nearly equals that of the fat-storing lipocytic cells. Acid mucopolysaccharides are seen in only small areas of tumour. Histochemical staining as well as biochemical investigations, show that the majority of tumour fat consists of triglycerides, followed by cholesterol esters. There are also small amounts of free cholesterol, phospholipids, free fatty acids and a further fraction, which seems to be a glycerol ether. There exist differences in the quantitative content of cholesterol, phospholipids and triglycerides of tumour fat compared to normal fat. No significant difference exists between the fatty acids in tumour fat and those in normal fat outside of the tumour.

Cell Differentiation↗

Benign recurring lipoblastoma in an adult versus well differentiated subcutaneous myxoid liposarcoma: clinicopathologic, immunohistochemical and molecular analysis of a unique case.

Subcutaneous myxoid liposarcoma (ML) is exceedingly rare, with only two or three cases having been reported. Lipoblastoma (LB), a rare fatty tumor that arises in infants and children, is unknown after adolescence. In contrast to ML, LB is often superficial. The two tumors can be difficult to distinguish due to many histologic similarities. We examined a 0.9 cm superficial subcutaneous nodule from the dorsal neck of a 48 year old man that had been growing slowly. Three and one half years later, a 0.4 cm palpable recurrent nodule was excised from the scar. The patient is now free of disease at 7.5 years. Because of these unusual features, we performed clinicopathologic, immunohistochemical and molecular analysis of this unusual tumor to decide if this represented a rare cutaneous ML or an unprecedented example of LB in an adult. The primary featured a thick fibrous pseudocapsule with foci of lymphocytes and infiltrating nests of semi-mature fetal-appearing adipocytic tissue. This surrounded a more immature cellular-but-cytologically-bland myxoid tissue featuring stellate cells and signet lipoblasts. There were fibrous sep at the periphery and the vasculature was rather inconspicuous. The 0.4 cm diameter recurrence was distinctly lobular and had minute satellite nodules. It was composed of uniform fetal-appearing bland myxoid lipoblastic tissue featuring signet ring lipoblasts surrounded by a few spindle cells. In both tumors, lipoblasts expressed S-100 protein. In the primary, 5% of the lesional cells were FXIIIa+ dendritic stromal histiocytes while in the recurrence, 15% of the lesional cells were FXIIIa+ dendritic cells. CD34 stained only scattered small capillaries. The Ki67 proliferation index was 1% in the primary and 3% in the recurrence. RT-PCR assay for TLS/FUS-CHOP fusion transcripts was negative despite three repeat tests performed on paraffin sections of the primary tumor in the presence of good m-RNA internal controls. We reviewed the clinicopathologic and cytogenetic features of ML and LB. Based on this review and on the growth pattern, anatomic features and molecular data from the present case, we conclude that this tumor may represent the first reported case of adult LB.

Antigens, CD34↗

An unusual case of spinal metastasis from a liposarcoma.

A 58-year-old man presented with spinal cord compression due to a metastatic liposarcoma of the thoracic spine. There was no evidence of vertebral bone involvement radiographically. This rare case is presented and its clinical features and diagnosis are discussed.

Epidural Neoplasms↗

Metastatic cardiac liposarcoma: diagnosis by transesophageal echocardiography and magnetic resonance imaging.

The most superior portion of the right atrium is not well visualized by transthoracic echocardiography. This limits the ability of the technique to detect intracardiac disease in this area. We describe a 41-year-old man with a history of liposarcoma in whom transthoracic echocardiography was unable to elucidate a right atrial metastasis. Transesophageal echocardiography demonstrated the morphology and extent of the large right atrial mass. These findings were well correlated with both magnetic resonance imaging and surgery.

Adult↗

Myxoid liposarcoma of the buccal vestibule. A case report.

A case of a myxoid liposarcoma in the buccal vestibule of the mandible is presented. The patient, an 86-year-old women, had 2 local recurrences after initial excision and died several months later. The literature is briefly reviewed.

Aged↗

Sternal resection for primary presternal and retrosternal mediastinal liposarcoma.

In a 34-year-old patient, sternal resection was necessary for complete removal of a primary mediastinal myxoid liposarcoma grade I, which had grown around the right sternal border. Reconstruction was by the methylmethacrylate sandwich technique. Five months postoperatively part of the device had to be removed due to persistent inflammation. Two years after the initial operation there is no evidence of local recurrence or distant metastases.

Adult↗