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Fusion of the EWS and CHOP genes in myxoid liposarcoma.

The translocation t(12;16)(q13;p11), which cytogenetically characterizes myxoid liposarcomas (MLS), results in a fusion of the CHOP gene in 12q13 and the FUS gene in 16p11, creating a chimeric FUS/CHOP gene. We have identified two cases of MLS with translocations giving rise to recombination between 12q13 and 22q12. The result was a fusion of the N-terminal part of the EWS gene in 22q12, involved in a number of mesenchymal tumor types, with the CHOP gene and the creation of an EWS/CHOP chimeric gene. The presence of the EWS/CHOP chimeric gene in MLS shows that (i) the N-terminal part of FUS may be replaced by the N-terminal part of EWS in a CHOP fusion oncoprotein (ii) the two N-terminal parts, when fused to certain transcription factors, have a common or very similar oncogenic potential and (iii) the tumorigenic process in MLS and the morphogenetically distinctly different EWS-associated tumor types may be related.

Amino Acid Sequence↗

[Primary liposarcoma of the left ventricle. Apropos of a case and review of the literature].

Primary malignant myocardial tumours are rare and essentially sarcomas. The authors report a case of primary left ventricular liposarcoma which is the 18th reported case. The presenting signs were of cardiac failure. Metastases are common by the time of diagnosis. Surgical ablation, though rarely complete because of its myocardial localisation, is justified for precise histological diagnosis. The prognosis of these lesions is poor. Complementary treatment is rarely used. However, it should be proposed as surgery alone has been shown to have limited curative applications.

Adult↗

Human liposarcoma cell line, SW872, secretes cholesteryl ester transfer protein in response to cholesterol.

Cholesteryl ester transfer protein (CETP) mediates the exchange of phospholipids and neutral lipids between the plasma lipoproteins, and plays an important role in high density lipoprotein (HDL) metabolism. While there are reports of low-level CETP secretion from cultured cells, the lack of a good model cell line has hampered the detailed study of CETP regulation and secretion. In this study, we have found that the human liposarcoma cell line, SW872, secretes cholesteryl ester transfer protein at levels substantially higher than observed from other cell lines. The secretion of CETP from this adipose-derived cell was up-regulated by 25-OH cholesterol and by low density lipoprotein (LDL) cholesterol in a concentration-dependent manner. Analysis of both full length and exon 9-deleted CETP mRNA demonstrated increases in response to LDL and 25-OH cholesterol, providing evidence for regulation at the message level. Our results suggest that the CETP-producing SW872 cell line may provide a model in which to study the regulation of this important modulator of lipoprotein metabolism.

Adipocytes↗

[Should lipoma be removed by liposuction? Apropos of a case of grade 1 myxoid liposarcoma of the shoulder].

Liposuction of a big lipoma--mostly on the chest and close to the upper limbs--is a fairly neat solution for such areas, which usually do not heal very well. The author reminds us of the fact that some liposarcomas may have the same features as a benign lipoma on inspection and palpation, and histology ("lipoma-like"). He recommends systematic biopsies and possibility grozen-section examination.

Aged↗

Primary liposarcoma of the mediastinum. Report of a case and review of the literature.

Primary liposarcomas of the mediastinum are very rare tumors. We record herein the fiftieth documented case, and the sixth in which there was evidence of superior vena caval obstruction. All previously reported cases have been studied; their salient clinical and pathological features have been tabulated. Review of these case reports reveals that the majority of patients have complaints of respiratory distress or chest pain. Other common presenting complaints include cough and weight loss. These tumors grow to an enormous size, and symptoms are referable to compression of contiguous intrathoracic structures. Radiotherapy or chemotherapy or both are ineffective theraputic modalities. The treatment of choice is surgical in all cases. Such an approach serves to establish a tissue diagnosis, to relieve the patient's symptoms, and may at times result in a cure.

Adolescent↗

[Retroperitoneal liposarcoma with lung, skin and pancreatic metastases].

Liposarcomas are soft-tissue tumors usually located in the lower extremities and the retroperitoneum. We describe the case of a woman with this neoplasm and a prior familial history of lipoma. Skin, lung and pancreatic metastasis and systemic signs developed quickly. Clinical aspects and the main tools for diagnosing these neoplasms are described.

Aged↗

[Intratumor and retroperitoneal massive hemorrhage caused by retroperitoneal liposarcoma].

Case report of a giant retroperitoneal liposarcoma which presented with a picture of acute abdomen resulting from a massive tumoral haemorrhage. Diagnosis was achieved by ECO and CT, and was confirmed by Pathological Anatomy. Treatment was surgical, performing tumour removal plus polar nephrectomy of the affected side. The patient evolution was favourable. The standard methods of diagnosis and prognostic factors are commented.

Hemorrhage↗

[A case of mediastinal liposarcoma surgically removed by bilateral thoracotomy].

A rare case of mediastinal liposarcoma was experienced at our institute. A 64-year-old male patient was accidentally found by chest X-ray photo. The tumor was so gigantic and seemed to involve the descending aorta, therefore, it was removed through bilateral thoracotomy. It was well encapsulated and its malignancy resides in that of low grade to be completely removed. The patient is expected to acquire long term survival.

Humans↗

[A case of liposarcoma associated with chronic tuberculous empyema].

A case was 77-year-old male with left back pain who had received artificial pneumothorax therapy 40 years ago due to lung tuberculosis. He had a spindle shaped mass in his left chest wall which had connection to the chronic tuberculous cavity. Because malignant cell was not detected by biopsy, it was therapied as tuberculous abscess. Then the mass grew rapidly into 20 cm in diameter. Although surgical operation was carried out, it was impossible to remove it all. After the surgery, the pathological findings was liposarcoma. In spite of the radiotherapy, the patient died 4 months after the surgery. We should always keep in mind the early diagnosis of malignant tumor associated with chronic tuberculous empyema.

Aged↗

[Results of surgical therapy of primary and recurrent retroperitoneal liposarcoma].

Between 1978 and 1994, 27 patients with a retroperitoneal liposarcoma underwent a total of 64 operations at our institution with a resectability rate of 96% of the primary and 100% of recurrent tumors. Operative morbidity (7.8%) and mortality (3.1%) were low, although multivisceral reactions were necessary in the majority of cases (73% of all primary tumors). The single most important prognostic factor was a potentially curative resection at first operation (5-year survival: RO: 75%, R1/R2: 34%), but also at first operation for tumor recurrence.

Adult↗

Giant pedunculated liposarcomas of the esophagus: literature review and case report.

Although liposarcoma is the second most common soft-tissue sarcoma in adults, its incidence within the gastrointestinal tract is distinctly low. Esophageal involvement is exceedingly rare and only four cases have been described so far. A fifth case is presented here along with a thorough review of the literature of polypoid lipomatous tumors of the esophagus. Diagnostic and therapeutical strategies of these tumors are discussed in detail.

Diagnosis, Differential↗

[Myxoid liposarcoma of the larynx].

The paper presents a rare case of tumor--liposarcoma myxoides of untypical location--in the area of subglottic larynx. Diagnostic problems that resulted in erroneous recognition of the case, leading to improper treatment, are discussed.

Adult↗

[Retroperitoneal liposarcoma. Apropos of 2 cases].

We report two cases of retroperitoneal liposarcoma that required multiple surgical excisions for locoregional relapse diagnosed by surveillance based on CT-scan. One patient has had adjuvant radiotherapy. With the literature review, we discuss the pathologic and therapeutic aspects of these lesions.

Female↗

Liposarcoma of the tongue.

Liposarcoma is a rare tumor in the head and neck. No previous report of its occurrence in the tongue is found at the time of this writing and this prompts its presentation. Significant in its management is wide surgical excision. Irradiation may be effective in some metastases. Prognosis is best in well differentiated forms.

Adult↗

[Clinical considerations on retroperitoneal liposarcoma. Report of 2 cases].

Liposarcoma is one of the more common tumours of the soft retroperitoneal tissues. It usually occurs in aged subjects and displays a marked contrast between its malignant histological picture and its protracted, poorly symptomatic clinical picture. Eventually, compression or invasion of nearby organs appears, whereas the patient's general condition is not affected. Two personal cases are described.

Aged↗

[Bilateral breast carcinoma after recurrent myxoid liposarcoma of the breast].

The case is presented of a 26-year-old woman with myxoid liposarcoma of the right breast who developed a relapse with anaplastic spindle-cell mesenchymal tumor 7 months after primary therapy. Additionally, 13 months later a bilateral adeno-carcinoma of differing histological grade occurred in the breasts. To the best of our knowledge, no comparable sarcoma-carcinoma sequence in the mammary gland has been reported. For this reason this case is discussed, with special emphasis on possible causative factors and the relationship to the Li-Fraumeni syndrome.

Adenocarcinoma↗

Characterization and expression of the human gene encoding two translocation liposarcoma protein-associated serine-arginine (TASR) proteins.

Translocation liposarcoma protein (TLS)-associated serine-arginine (TASR)-1 and -2 are two newly identified serine-arginine splicing factors. Our recent studies suggest that disruption of TASR-mediated pre-mRNA splicing is involved in the pathogenesis of human leukemia and sarcomas. The mRNA transcripts for TASR-1 and -2 share an identical sequence at the 5' untranslated region (5' UTR) and in part of the coding region; however the other regions of the transcripts diverge from each other and it was not clear whether the differences resulted from alternative splicing or transcription from two distinct genes. Here we describe the assignment of both TASR cDNAs to the same 16 kb DNA segment located on chromosome 1. Despite the presence of at least three retroposed products of TASR-1 mRNA in the human genome, only the 16 kb structural TASR gene on chromosome 1 is actively transcribed. In addition, multiple polyadenylation sites and a rare U12-type intron were found within the TASR gene. Transcription initiation site of the TASR gene was determined by primer extension; analysis of the TASR promoter revealed that it lacks the TATA box but contains a GC-rich sequence. When cloned into a luciferase reporter and transfected into human cells, the TASR promoter construct generated luciferase activity that was at least 2000 fold greater than the promoterless plasmid. Northern blot analysis showed that at least five different TASR-1 and -2 transcripts are expressed in a broad range of human tissues.

Alternative Splicing↗