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Chromosome-12 copy number alterations and MDM2, CDK4 and TP53 expression in soft tissue liposarcoma.

BACKGROUND: Liposarcoma is a heterogeneous group of soft tissue sarcomas in which definitive prognostic parameters need to be identified. MATERIALS AND METHODS: The series included 33 consecutive soft tissue (well-differentiated, WDLPS, n=19; and dedifferentiated, DDLPS, n=14) liposarcoma. Clinicopathological variables included age, gender, body location, degree of dedifferentiation and mitotic count. The rrolecular analysis included MDM2, CDK4 and TP53 expressions and chromosome-12 copy number alterations. RESULTS: Centrally located (retroperitoneal, abdominal cavity or groin region) WDLPS had more dedifferentiation (p=0.001). Patients with DDLPS and a high mitotic rate died (p=0.070) or experienced recurrencies (p=0.029) more frequently. Co-expression of MDM2/CDK4 (p=0.001) and TP53 accumulation (p=0.017) related to dedifferentiation but not to recurrence or death, both in WDLPS and DDLPS. DDLPS had higher centromeric chromosome-12 copy number than WDLPS (p=0.013), but this was unrelated to recurrence or death. CONCLUSION: Central location is a risk factor in WDLP. Co-expression of MDM2/CDK4/TP53 and chromosome-12 alterations characterize DDLPS suggesting a link with dedifferentiation.

Adult↗

Case of conjunctival liposarcoma.

A case of primary conjunctival liposarcoma was reported. The patient was a 62-year-old woman who complained of swelling of the left bulbar conjunctiva. As the swelling lesion did not respond to treatment and had gradually enlarged, surgical excision was performed 18 months after her first visit. The tumor was bean-sized and was located subconjunctivally without connecting orbital tissues. Histopathological examination of the specimen revealed numerous neoplastic cells containing stellate and hyperchromatic nucleus. The cytoplasm of these cells contained vacuoles resembling lipid droplets. Signet-ring type cells were also observed. These cells were considered to be lipoblasts. The stroma was myxomatous and thin-walled vessels were observed. The present tumor was confirmed by histopathological study as myxoid liposarcoma and considered to be of bulbar conjunctiva origin. The patient showed no sign of recurrence during 16 months of follow-up.

Conjunctival Neoplasms↗

[A rare case of a giant intra-abdominal mesocolic liposarcoma].

Intra-abdominal liposarcomas (IALS) represent a rare localization compared to other liposarcomatous (LS) sites such as the lower extremities and the retroperitoneum. The authors report their experience in a case of giant liposarcoma (weight: Kg 8.2) presenting a massive intra-abdominal extension. Diagnostic and therapeutic problems related to this type of neoplasm as well as a literature review are reported.

Aged↗

[Retroperitoneal myxoid liposarcoma of the kidney capsule as a cause of Budd-Chiari syndrome].

A retroperitoneal myxoid liposarcoma of the renal capsule must be differentiated from renal cell carcinomas, angiomyolipomas, fibrogenous lipomas, fibrolipomas and mixed tumours containing fat tissue. Myxoid liposarcomas can lead to intracaval tumour thromboses, which is often the case with renal cell carcinomas and revealed clinical with Budd-Chiari syndrome. Computed tomography and magnetic resonance imaging give additional information in the diagnosis of intracaval tumour thromboses and show the exact expansion of the topographic-anatomical structure.

Aged↗

[A case report of esophageal liposarcoma].

We report the first case of esophageal liposarcoma in Japan. A 46-year-old female was admitted to our hospital with chief complaint of protruding tumor out of the mouth. She had two episodes of protruding tumor after nausea in last 6 months. Barium swallow showed a large polypoid lesion of approximately 16cm in length with a stalk. By endoscopic examination, it was the polyp originated from the anterior wall of the cervical esophagus. It was covered with intact squamous epithelia, which had slight redness and erosion in the apex. Under general anesthesia the tumor was cut off at the base through the oral cavity. Specimen was 11 x 4 x 3cm in size and had yellowish cut surface. Histologically, the tumor composed of mature adipocytes and many atypical lipoblasts with spider web shaped cytoplasm. We diagnosed this tumor as well differentiated liposarcoma.

Esophageal Neoplasms↗

Dedifferentiated liposarcoma with features of rhabdomyosarcoma.

We describe a case of recurrent dedifferentiated retroperitoneal liposarcoma with light microscopy features of rhabdomyosarcoma, the first such reported case. The tumor contains a pleomorphic pattern consisting of plump fusiform cells, tadpole cells, malignant appearing lipoblasts, and other nondescript bizarre cells. Of particular interest is a number of tumor cells with plump, bizarre nuclei which contain cross-striations of skeletal muscle pattern. The literature on dedifferentiated liposarcoma is reviewed, and the significance of this case in terms of a current model of mesenchymal differentiation is discussed, with particular attention to the postulate of a common precursor for a variety of soft-tissue tumor histologies.

Cell Differentiation↗

[Conservative treatment of a recurring retroperitoneal liposarcoma. Apropos of a case followed over 27 years].

We report about the case of a patient who, over 27 years, presented with seven local recurrences of a retroperitoneal liposarcoma. This evolution seems to characterize differentiated (adult and myxoid) forms of liposarcoma, for which metastatic expansion is less frequent that in the undifferentiated, pleiomorphic or round-cell forms. The treatment of these tumors is mainly surgical. Adjuvant radiation therapy might reduce the frequency of local recurrence.

Combined Modality Therapy↗

[Clinico-diagnostic and therapeutic considerations on retroperitoneal liposarcoma].

Two patients with retroperitoneal liposarcoma are presented to determine the accuracy of diagnostic methods and the role of surgical treatment. In both the patients the large retroperitoneal liposarcoma recurred locally after surgery. Histological features, clinical presentation, diagnostic procedures, extent of surgical resection and adjuvant treatment are reviewed. These data suggest that an aggressive surgical approach followed by adjuvant postoperative irradiation is the treatment of choice in primary and recurrent neoplasms.

Aged↗

Primary liposarcoma of the pharynx.

We describe a rare case of primary pharyngeal liposarcoma in a 64-year-old man who had multiple recurrences. The available literature is reviewed and discussed. Although it is very difficult to assess the prognosis and treatment of pharyngeal liposarcomas, it appears that the histologic characteristics of the individual tumor and its location and resectability determine the outcome of treatment. However, because of its rare occurrence clinically, no single institution has been able to gain enough experience to formulate reliable long-term prognoses and therapeutic guidelines.

Humans↗

[Myxoid liposarcoma of the spermatic cord].

We report on a 50-year-old male patient who presented with a scrotal mass which he had noted two years earlier. Ultrasound revealed a well-defined, regular, paratesticular mass. Pathological examination of the surgical specimen revealed abundant lipoblasts and a stroma with myxoid changes, prompting a diagnosis of myxoid liposarcoma of the spermatic cord. Liposarcoma is a rare tumor of the spermatic cord which frequently metastasizes to the lumbar retroperitoneal lymph nodes. The foregoing characteristic and the histologic features of the tumor type influence treatment.

Genital Neoplasms, Male↗

[Retroperitoneal liposarcoma. Clinico-pathological analysis of 6 cases].

Six patients with retroperitoneal liposarcoma are reported. Their mean age was 58,66 years. There were four males and two females. The symptoms or signs at presentation were: abdominal mass (5 cases); abdominal pain (3 cases); sustained fever (2 cases), and lower limb edema (1 case). Abdominal computed tomography (CT) showed large masses with different density. Distant metastases were not documented. All patients underwent laparotomy, and the tumor was not resectable in 2 cases. In the histological study, liposarcoma was shown to be myxoid in 4 cases, pleomorphic in one and round cell type in the remaining case. In 3 patients local relapse was observed. The longest survival was 38 months and the shortest one 3 months. It was concluded that the leading cause for consultation in these patients was an abdominal mass. Remarkably, two patients had the uncommon feature of sustained tumoral fever. CT was shown to be the most useful diagnostic method. The good general condition of the patients with large tumoral masses was remarkable. The most common variety was myxoid, local relapse was frequent and the survival was poor.

Adult↗

Malignant myxoid liposarcoma: an immunohistochemical, electron-microscopical and cytogenetical analysis.

We discuss the morphological, immunohistochemical and electron-microscopical features of a malignant myxoid liposarcoma which appeared in the left thigh of a 52-year-old woman. The cytogenetic analysis following short-term tissue culture confirmed the existence of a nonrandom translocation t(12;16)(q13;p11). Furthermore, immunohistochemistry showed positivity for S-100 antigen and vimentin, both of which are considered to be markers for liposarcoma. The electron-microscopical study revealed a close contiguity between the tumoral cells and atypical pericytes.

Biomarkers, Tumor↗

[Giant liposarcoma of the spermatic cord: a case report].

A case of giant liposarcoma of the spermatic cord was reported. A 75-year-old man was admitted with a 25-year history of a slowly growing left scrotal solid mass without trans-illumination. The removed huge mass measuring 26.0 x 16.5 x 20.0 cm and weighing 4,500 g was confirmed to be a well-differentiated liposarcoma according to WHO classification. The patient died of acute pneumonia 5 months later, although the postoperative course was uneventful and he had no sign of recurrence.

Aged↗

Skeletal metastases in liposarcoma detectable only by magnetic resonance imaging.

Diffuse, asymptomatic skeletal metastases occurred in a 46-year-old patient with liposarcoma. These metastases were detected by magnetic resonance imaging (MRI) but not by roentgenograms or radionuclide scans. Pathologic confirmation from the MRI-detected area was obtained. This observation suggests a possible explanation for the present discrepancy between the incidence of skeletal involvement in antemortem and postmortem studies in metastatic liposarcoma. MRI may be an extremely sensitive tool for detection of bone and bone marrow metastasis.

Bone Marrow↗

[A case of retroperitoneal liposarcoma].

We reported a case of retroperitoneal well differentiated liposarcoma, which consisted of lipoma-like type and sclerosing type. The latter was represented as solid density tumor on CT, which was difficult to differentiate from undifferentiated liposarcoma and other soft tissue tumors. Angiographically, ureteral artery developed and became a main feeder.

Aged↗

Liposarcoma of the ileocecal valve: a case report.

Liposarcomas are common soft-tissue neoplasms but are rarely present in the gastrointestinal tract. We report a patient with a primary liposarcoma at the ileocecal valve, a previously unreported location. Wide local excision remains the treatment of choice, but local recurrences are common.

Female↗

[Primary liposarcoma of the left atrium surgically treated].

A case of myxoid liposarcoma of the left atrium in a 35-year old man is reported. The tumour, revealed by an atrial flutter, was excised. The patient died 13 months later, with multiple metastases. A review of the literature yielded only 7 cases of primary liposarcoma of the heart, including 4 which were surgically treated. This confirms the extreme rarity of a tumour which has a very poor prognosis due to recurrence in situ or metastatic spread.

Adult↗