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Liposarcoma of the stomach.

We present a case of gastric liposarcoma, a very uncommon pathology; only nine cases have been reported in the literature. We describe the radiologic findings of this neoplasm and emphasize the correlation between computed tomography (CT) and the macroscopic morphology of the tumor, which is conditioned by its histology. In our case, CT demonstration of fatty areas within the gastric mass facilitated the diagnosis. This finding has not been described for liposarcomas of the stomach.

Adipose Tissue↗

Well-differentiated liposarcoma of the retroperitoneum with a fat-fluid level: US, CT, and MR appearance.

We report a case of retroperitoneal liposarcoma with a fat-fluid level that has not been previously described. A 36-year-old man presented with abdominal distension. Ultrasonography, CT, and MR imaging showed a tumor with a fat-fluid level; nondependent fluid was characteristic of fat. Liposarcoma should be included in the differential diagnosis of tumors exhibiting a fat-fluid level.

Adipose Tissue↗

Dedifferentiated liposarcoma with rhabdomyoblastic differentiation.

Dedifferentiated areas of dedifferentiated liposarcoma (DDL) usually show malignant fibrous histiocytoma (MFH)- or fibrosarcoma-like features and lack any histologic signs of specific differentiation. However, some reports have demonstrated specific differentiation in these areas, with histologic features resembling those of rhabdomyosarcoma, leiomyosarcoma, and osteosarcoma. We report here a pathologic and genetic analysis of three cases of DDLs with rhabdomyosarcomatous areas. MFH- or fibrosarcoma-like areas of one primary DDL and two recurrent DDLs contained various amounts of rhabdomyoblasts, which were immunoreactive for desmin, myoglobin, muscle actin (HHF-35), and myogenin. An ultrastructural examination demonstrated rhabdomyoblasts with abundant cytoplasm containing thin and thick filaments and Z-bands. By real-time PCR, amplification of mdm2 and cdk4 was confirmed in both well-differentiated and dedifferentiated areas with rhabdomyoblasts of all cases. To our knowledge, only seven cases of DDLs with rhabdomyosarcomatous components have been reported, and furthermore, the genetic profiles of the rhabdomyosarcomatous components in DDLs have not been investigated. This study demonstrates that DDLs with rhabdomyosarcomatous areas have genetic alterations that are common to well-differentiated/dedifferentiated liposarcomas.

Biomarkers, Tumor↗

Adhesion proteins, cellular morphology and fibrous components around the cell/extracellular-matrix interface in myxoid liposarcomas.

We examined the cell/extracellular-matrix interface in 13 myxoid liposarcomas by determining the distribution of collagen and reticular fibers in the myxoid matrix, the presence of adhesion proteins and the morphological features of the cytoplasmic border. Adhesion proteins (fibronectin, integrin alpha3) and the cytoplasmic border were examined by immunofluorescence and a differential interference-contrast image analysis respectively. A network of reticular fibers and collagen fibers was present in the myxoid matrix of 11 cases (85%) and 3 cases (23%) respectively. Tumor cells with dendritic cytoplasmic processes were observed in 8 cases (62%). Adhesion proteins were sparsely present in tumor cells and there was no correlation between those proteins and collagen fibers, reticular fibers or cytoplasmic processes. Collagen fibers, dense reticular fibers or well-developed cytoplasmic processes were more frequently observed in the cases of long-term-surviving patients than those with recently developed tumors or patients who died. All 3 cases positive for collagen fibers also contained both dense reticular fibers and cytoplasmic processes. Our findings suggest that the fibrous components in the myxoid matrix and the well-developed cytoplasmic processes may limit the invasiveness of malignant cells. This peculiar architecture may also explain the slowly progressive nature of myxoid liposarcomas.

Adult↗

Pancreas-sparing duodenectomy for a recurrent retroperitoneal liposarcoma: report of a case.

Pancreas-sparing duodenectomy (PSD), which allows preservation of the pancreas in its entirety, is a promising procedure for low-grade malignancies of the duodenum, the periampullary region, and the neighboring retroperitoneum. We report a case of recurrent retroperitoneal liposarcoma involving the second and third parts of the duodenum, which was extirpated using PSD, after a right hemicolectomy for tumor invasion of part of the colonic hepatic flexura. The Roux-en-Y jejunal limb was sutured to the duodenal bulb in an end-to-end fashion, and the biliary and pancreatic duct systems were reconstructed with end-to-side anastomoses, placing the jejunal limb distal to the bulbo-jejunostomy after a septoplasty to repair the adjacent pancreatic and biliary ducts. Because retroperitoneal liposarcoma has a low incidence of lymph node metastasis, pancreaticoduodenectomy may be inappropriate, especially if minimally extensive surgery can ensure organ preservation. Pancreas-sparing duodenectomy could be the most appropriate procedure for nonepithelial malignant tumors located around the duodenum distal to the pylorus, which have no involvement with the pancreatic parenchyma or periduodenal lymph nodes.

Aged↗

Retroperitoneal and scrotal giant liposarcoma: report of a case.

The case of a 63 year-old man with a giant scrotal and retroperitoneal tumor is herein reported. The initial symptoms began in the scrotum and subsequent abdominal distention resulted in discomfort 2 years later. The intraabdominal organs were under pressure because of the bulky mass, and the patient had dyspnea. Ultrasonograpy, computed tomography, and fine needle aspiration biopsy investigations all revealed a retroperitoneal tumor suspected to be liposarcoma. At operation, a tumor weighing 42 kg was excised. Respiratory support was provided in the early postoperative period. The histopathological diagnosis was myxoid liposarcoma. The patient was discharged from the hospital 14 days after the operation and was scheduled to undergo radiotherapy.

Genital Neoplasms, Male↗

Successful resection of a primary liposarcoma in the anterior mediastinum in a child: report of a case.

Primary liposarcomas of the mediastinum are very rare. We report on a 13-year-old girl who presented with a huge mediastinal tumor. The tumor was extirpated by a median sternotomy with a right thoracotomy. The tumor included the superior vena cava in the anterior mediastinum. It therefore probably originated from the anterior mediastinal fat tissue, possibly from the thymus. A pathological examination revealed myxoid liposarcoma. At 35 months postoperatively, the patient has not shown any recurrence.

Adipose Tissue↗

A myxoid liposarcoma in the lower leg, with a large intra-abdominal metastasis.

We report a patient with a large intra-abdominal metastasis of myxoid liposarcoma. The patient first noticed an asymptomatic mass in her left leg in 1985, when she was 20 years old. The mass was left untouched until she realized its rapid growth and consulted a local doctor in 1994. After needle biopsy, she was histologically diagnosed as having a myxoid liposarcoma. She disagreed with the recommendation for an amputation below the knee, made at another hospital. A marginal resection was performed as an alternative treatment. She subsequently underwent three more marginal resections and four intra-lesional resections for repeated local recurrences. In 1997, an abdominal computed tomography scan revealed the presence of multiple intra-abdominal metastases, and the lesions were judged to be inoperable. Ileus and respiratory distress, caused by compression by the abdominal mass, gradually worsened, and she died in 1999, at the age of 34. The girth of her abdomen was 135 cm at the time of death.

Abdominal Neoplasms↗

Myxoid liposarcoma--the frequency and the natural history of nonpulmonary soft tissue metastases.

BACKGROUND: Myxoid liposarcomas (ML) make up the major subset of liposarcomas, which in most series represent the second or third most common type of soft tissue sarcoma. The tendency for ML to metastasize to other soft tissues (STM) in preference to lung parenchyma has been previously described; however, the natural history of this tumor's behavior is poorly documented. Our intent was to analyze the natural history of ML and further quantify the incidence of STM, concentrating on their significance in terms of survival. METHODS: We reviewed the experience at the Royal Marsden Hospital over a 10-year period, documenting the clinicopathological behavior of ML, including the frequency of STM. RESULTS: There were 50 patients, with a median follow-up of 43 months. The actuarial 5-year soft tissue metastasis rate was 31%, and the most common sites of STM were the retroperitoneum, abdominal wall, and abdominal cavity. In those 12 patients who had STM there was a median interval of 23 months after original diagnosis to the time the first metastasis became apparent (range, 0-142 months). Median survival following first metastasis was 35 months; 6 of the 12 patients died between 6 and 50 months. Four patients who had STM remain disease free at 15 to 59 months after their first STM. Any round cell component of the ML was associated with a significantly greater chance of metastatic disease (P = .02). In this series, the overall 5-year and 7-year survival rates were 85% and 68%. Patients with STM had an 11 times greater chance of dying than those who did not. CONCLUSIONS: ML usually is an indolent disease, but there is a subset of patients who develop STM and have a significantly worse prognosis. STM can occur years after the initial diagnosis and can be associated with medium-long-term survival after they occur. STM should be managed aggressively because of this.

Abdominal Neoplasms↗

Liposarcoma presenting on the vulva.

A myxoid liposarcoma of the vulvar perineum in a 15-year-old girl is described. The tumor recurred as a poorly differentiated round cell liposarcoma and resulted in the patient's death. Clinicians and pathologists should be aware that this tumor may rarely present on the vulva.

Adolescent↗

Liposarcoma of the leg.

Forty-eight patients with liposarcomas of the leg were reviewed. Patients were treated by three approaches: wide local excision, amputation, and regional hyperthermic perfusion. Deaths from distant metastases occurred at a similar rate in all groups. The local recurrence rate was 43 percent in the wide excision group and 29 percent in the amputation group. No perfused patient had a local recurrence. This study demonstrates that hyperthermic regional perfusion and excision are superior to wide excision or amputation in preventing local recurrence for a liposarcoma of the leg and allow salvage of a functional limb.

Adult↗

Mediastinal involvement by myxoid liposarcoma.

Metastatic involvement of the mediastinum by liposarcoma is uncommon, and clinical experience in its management is limited. A patient with liposarcoma of the lower extremity having a concomitant anterior mediastinal mass as the only manifestation of distant metastatic involvement is described.

Adult↗

Liposarcoma of the esophagus.

Liposarcoma is one of the most common soft tissue sarcomas found in adults, yet it rarely develops in the alimentary tract. This report describes the pathological features and management of a liposarcoma of the esophagus.

Aged↗

Liposarcoma involving the scapula.

An unusual case of a soft tissue liposarcoma arising in the upper limb was studied by computed tomography. Computed tomography suggested the diagnosis of myxoid liposarcoma using the criteria of textural inhomogeneity and bony erosion and scalloping of the scapula.

Aged↗

Cytogenetic studies of adipose tissue tumors. II. Recurrent reciprocal translocation t(12;16)(q13;p11) in myxoid liposarcomas.

Detailed chromosome studies, briefly reported previously, from short-term cultures of tumor cells from myxoid liposarcomas are reported. A common reciprocal translocation, t(12;16)(q13;p11), was found in three cases and a complex t(1;12;16)(p11;q13;p11) in the fourth one. This nonrandom primary change, not described before in solid tumors, could characterize the myxoid form of liposarcoma. The involvement of a closely located breakpoint on chromosome #12 in a reciprocal t(3;12)(q28;q14) described in a lipoma in the previous article of this series, suggests a common basis in the biological process of proliferation of tumors sharing a common histogenesis.

Adult↗

Liposarcoma: report of a case and review of the literature.

Liposarcoma of the head and neck region is extremely rare. An additional case of pleomorphic liposarcoma of the buccal mucosa is reported, representing an even rarer combination. The importance of combined radiation and radical surgical therapy as a means of improving survival is emphasized.

Aged↗

Primary mediastinal liposarcoma. A report of a case and review of the literature.

A case of primary liposarcoma of the mediastinum with a review of the literature is presented. Liposarcomas may attain a large mass and exhibit various kinds of clinical symptoms according to their localisation. Histological typing is very important in determining the type and extent of therapy. Surgical excision of the tumour is the therapy of choice in all cases. Radiotherapy may be of value for the palliation of unresectable cases.

Aged↗