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Juxtacortical liposarcoma. A case report and review of the literature.

Juxtacortical liposarcoma is an uncommon entity. The purpose of this paper is to report the case of a 63-year-old man with juxtacortical liposarcoma. Liposarcomas arising as surface lesions of bone are extremely rare. Histologically, juxtacortical liposarcoma is composed of myxoid and round-cell areas. Because of the extent of compartmental involvement and the high-grade malignancy, limb-sparing surgery was not considered for the patient. A review of the English literature on diagnosis and treatment indicates a grave prognosis.

Femoral Neoplasms↗

[Therapeutic problems of intra-abdominal liposarcoma].

Intra-abdominal liposarcomas are very rare tumors. We present our personal experience with one case of mesenteric liposarcoma which had perforated into the peritoneal cavity, occurring in a seven-month pregnant female patient. Seven years later, the patient is doing well. The authors have carried out a review of the literature. In addition to liposarcomas of the mesentery and the mesocolon, they observed some cases of liposarcoma localized in the omentum and the stomach; other abdominal localizations occurred sporadically. Radical surgical excision was the choice treatment. Both the propensity for locoregional spread and the histological diversity set the conditions for prognosis; the latter seemed minimally influenced by either radio- or chemotherapy.

Abdominal Neoplasms↗

[Retroperitoneal liposarcoma: a report of 2 cases and review of 163 cases in Japan].

Two cases of retroperitoneal liposarcoma are reported. Case I was a 71-year-old-male presenting with a mass in the left abdomen. On X-ray examination, a large extrarenal tumor was found in the left retroperitoneal space. The tumor appeared to invade the psoas muscle and the aorta. Exploratory laparotomy was carried out. The histological diagnosis of the tumor was myxoid-type liposarcoma. He died of the disease. Case 2 was a 44-year-old-male who presented with a mass in the left abdomen. On X-ray examination, a large tumor was found in the left retroperitoneal space, and left kidney and the descending colon were markedly displaced by the tumor. Excisional surgery was carried out. The tumor was almost completely removed. The histological diagnosis was myxoid-type liposarcoma. A combination chemotherapy (actinomycin D, vincristine, cyclophosphamide) was started. but soon switched to radiation therapy because of liver dysfunction. Four months following the operation, there is no evidence of residual disease. One hundred sixty three cases of retroperitoneal liposarcoma reported in Japan are reviewed.

Adult↗

Electron microscopy of adipose tissue tumors: comparative features of hibernomas, myxoid and pleomorphic liposarcomas.

Two hibernomas, 16 myxoid, and eight pleomorphic liposarcomas were studied by transmission electron microscopy. Neoplastic cells in hibernomas and the type I cells--adipocytes--in myxoid liposarcomas consistently display abundant lipid droplet populations and prominent basal lamina deposition. Despite some variability in their distribution, these features clearly pertain to the mature and/or maturing characteristics of brown and white adipose cells. On the other hand, the more primitive type II cells of myxoid liposarcomas display highly variable, and occasionally absent, lipid droplet populations and basal lamina deposition. Moreover, the type II cells may also display other features such as prominent cytoplasmic filaments and rough endoplasmic reticulum that, while not characteristic of typical adipose cells, are still consistent with the more primitive mesenchymal cells from which they are assumed to derive. The cells of pleomorphic liposarcomas exhibit a variable lipid droplet population and apparently total absence of basal lamina deposition. Moreover, they often display atypical cell junctions that are neither characteristic for adipose cells nor for the "primitive reticular cells" from which adipocytes presumably derive. These observations would suggest that some "high grade" sarcomas may no be truly "undifferentiated"; rather, they may often display structural characteristics indicative of multiple as well as variable lines of differentiation. Therefore, classifications of such tumors based on quantitatively limited observations may not truly reflect their evident complexities.

Adipose Tissue↗

Liposarcoma. A clinicopathologic subtyping of 52 cases.

Fifty-two cases of liposarcoma were analyzed clinicopathologically, and were grouped into the following five types: myxoid (28 cases), well-differentiated (14 cases), pleomorphic (4 cases), round-cell (3 cases), and mixed (3 cases). Three of the 28 myxoid tumors showed a pleomorphic pattern resembling that of the myxoid variant of malignant fibrous histiocytoma, and one of the 3 round-cell tumors was considered to be a malignant counterpart of hibernoma. Ages ranged from 22 to 86 years with the average age of 49 years, the average patients with myxoid or round-cell liposarcoma being over 10 years younger than those with well-differentiated or pleomorphic liposarcoma. There was also a variation in the site of predilection of the cases: the great majority of myxoid type tumors occurred in the thigh, popliteal fossa and buttock (23 cases), while the majority of well-differentiated type tumors were seen in the retroperitoneum (10 cases). The rate of local recurrence of the tumor was 46%. Four of the 5 well-differentiated liposarcomas which recurred exhibited dedifferentiated pleomorphic components resembling those in malignant fibrous histiocytoma. The prognosis was more favorable in patients with ordinary myxoid tumors than in those with well-differentiated tumors. The overall relative five-year survival rate was 63%.

Adult↗

Myxoid liposarcoma of the anterior mediastinum. Diagnosis by fine needle aspiration biopsy.

The mediastinum is an uncommon primary site of liposarcoma, which constitutes < 1% of tumors at that site. We report a case of myxoid liposarcoma evaluated with fine needle aspiration biopsy in a 66-year-old male who presented with cardiac tamponade, superior vena cava syndrome and radiologic evidence of a large anterior mediastinal mass. Cytologically the neoplastic cells were strikingly dyscohesive, with ovoid or spindle-shaped, hyperchromatic nuclei. The cytoplasm was abundant and often finely vacuolated with lipid. They were associated with a prominent myxoid, intercellular substance and distinct, arborized capillary structures. Survival is influenced by the tumor cell type and differentiation. The overall prognosis seems to be poorer than that of liposarcomas occurring in other areas. To the best of our knowledge, this is the first reported case of primary mediastinal liposarcoma diagnosed by fine needle aspiration biopsy.

Aged↗

Long-term survival in a surgically treated non-encapsulated mediastinal primary liposarcoma. Diagnostic utility of core-needle biopsy for mediastinal tumors.

Primary liposarcomas of the mediastinum are uncommon tumors. Only six cases of mediastinal liposarcomas with survival after surgery longer than five years have been reported and only one of these was a non-encapsulated tumor. We report a new case of non-encapsulated primary liposarcoma of the mediastinum with long-term survival. The diagnosis was carried out using a CT-guided core-needle biopsy. We emphasize the utility of this technique to diagnose this kind of mediastinal tumors. A non-encapsulated primary liposarcoma of the mediastinum surgically treated with long-term survival is reported. We emphasize the diagnostic utility of CT-guided core-needle biopsy for the tumors.

Biopsy, Needle↗

Enhanced expression of catalytic subunits of protein phosphatase type 1 and high S-phase fraction in liposarcoma.

The expression of the three catalytic subunits of protein phosphatase (PP) type 1 and 2A, PP1 alpha, PP1 gamma 1, and PP2AC, was examined in 8 cases of lipoma as a benign tumor and 4 cases of liposarcoma as a malignant tumor using immunohistochemical analysis. Both types of of tumor cells stained positively with antisera against PP1 catalytic subunit isoforms PP1 alpha and PP1 gamma 1 were significantly higher in liposarcoma than in lipoma. Furthermore, liposarcoma showed a markedly high S-phase fraction in the cell cycle of tumor cells, as compared with lipoma. These results suggest that PP1 is involved in the accelerated growth of malignant cells in liposarcoma.

Adult↗

Chromosome 12 breakpoints are cytogenetically different in benign and malignant lipogenic tumors: localization of breakpoints in lipoma to 12q15 and in myxoid liposarcoma to 12q13.3.

Cytogenetic study of short-term cultures from 10 adipose tissue tumors (eight lipomas, one myxoid liposarcoma, and one mixed liposarcoma) have revealed clonal chromosome abnormalities in seven cases. In both malignant tumors, translocation (12;16) was the sole aberration, and in the mixed liposarcoma, the breakpoints could be sublocalized to bands 12q13.3 and 16p11.2, thus confirming findings of Eneroth et al., Cancer Genet. Cytogenet., 48: 101-107, 1990. Three lipomas displayed predominantly normal karyotypes; in a fourth case, the karyotype 44,XX,-6,der (7)t(6;7)(p21.3-22;p22)ins(7)(p22q11.2q22),-13 was found. Four remaining lipomas were characterized by structural rearrangements of chromosome 12. We were able to achieve high resolution banding patterns in two tumors with translocations (3;12)(q28;q15) and (1;2;12)(p36.;q13;q15). In both of these cases, the chromosome 12 breakpoint could be unequivocally assigned to band q15. Similarly, band 12q15 was also rearranged in two other lipomas with translocations (12;14)(q15;q32) and (12;20)(q15;q13.1). Our results support the hypothesis that the chromosome 12 breakpoint in lipomas is located more distally than the breakpoint in myxoid liposarcomas and some other soft-tissue malignant neoplasms and that it is cytogenetically identical with breakpoints detected in such benign tumors as uterine leiomyoma and pleomorphic adenoma of the salivary gland.

Adult↗

Liposarcoma.

Liposarcoma is second in frequency only to malignant fibrous histiocytoma among the soft-tissue sarcomas. It occurs almost exclusively in adults and is found most often in the thigh or retroperitoneum. It rarely arises from a lipoma and does not occur in the subcutaneous tissues. Liposarcomas are divided into histologic subtypes with different microscopic appearances and slightly different behavior characteristics. Surgical resection with a wide surgical margin is the treatment of choice. When amputation is required to obtain an adequate surgical margin, local irradiation can be used as an adjuvant and a limb-sparing operation can be done. Currently, no evidence exists that adjuvant chemotherapy is indicated for patients with liposarcoma, although numerous studies are being done to investigate its use. As is the case for all sarcomas, the lung is the most common site of metastasis; however, liposarcoma has an unusual propensity to metastasize to the retroperitoneum, mediastinum, and bone.

Adult↗

Mediastinal liposarcoma with local recurrence: a case report.

Liposarcoma is a rare mediastinal tumor. The treatment of choice is surgical resection. However, recurrence is not uncommon. In this report, recurrence of liposarcoma with local extension to chest wall is described in a 70-year-old Chinese male who had liposarcoma located in the anterior mediastinum and underwent tumor resection 5 years before. Long-term follow-up is indicated in patients with mediastinal liposarcoma because there may be an interval of several years between resection and recurrence.

Aged↗

Two cases of intrascrotal liposarcoma.

Liposarcomas in the scrotum are relatively rare and only 40 cases have previously been reported in Japan. The inside wall of the scrotum is an unusual origin and we report here two cases arising at this site that have been followed for 21 and 40 months, respectively, after surgical resection. Case 1: the patient was a 24-year-old male who noticed a left intrascrotal mass which was histologically demonstrated to be a myxoid type liposarcoma. Case 2: the patient was a 66-year-old male who had the complaint of a left intrascrotal swelling. He underwent radical orchiectomy and histologic examination of the resected tumor revealed a well-differentiated type of liposarcoma. These two cases are the 41st and 42nd intrascrotal liposarcomas reported in Japan.

Adult↗

[Dedifferentiated liposarcoma of the omentum simulating malignant peritoneal mesothelioma].

Liposarcoma of the omentum is a rare tumour which can simulate mesothelioma by naked eye and in histology as it was in the described case: A diffuse tumorous infiltration of parietal and visceral peritoneum was found in a male autopsy combined with two secondaries in the liver and one renal. The omentum was increased and quite infiltrated by the tumour. Single tumour foci consisted of structures reminding of a malignant neurogenous tumour, malignant fibrous histiocytoma and haemangiopericytoma. Some areas with a rather oval tumour cells with poor cohesivity reminded of a malignant lymphoma but were without any immunohistochemical positivity with markers proper for ML. A series of other tumours were to be eliminated in differential diagnostic consideration, i.e. sarcomatoid carcinoma, GIST, secondaries of a sarcomatoid variant of melanoma, malignant schwannoma, malignant fibrous histiocytoma, peritoneal leiomyomatosis. The prevalence of dedifferentiated structures can mostly veil the diagnosis of liposarcoma and immunohistochemical results are not always constant. A transition between well differentiated liposarcoma and its dedifferentiated structures are to be found in a single section for correct diagnosis. Prognosis of the dedifferentiated liposarcoma is presumed to be better than that of pleiomorphic sarcomas. The described patient was presented in terminal stage of disease and was monitored but for a month.

Aged↗

[Retrovesical liposarcoma: a case report].

A case of retrovesical liposarcoma is reported. The patient was a 41-year-old man who complained of left scrotal pain and of hip discomfort. Digital rectal examination revealed a hen's egg-sized mass with an elastic hard consistency at the left side of the normal prostate. Pelvic CT and MRI showed a heterogeneous tumor in the retrovesical region. Transperineal biopsy revealed liposarcoma. Pelvic exenteration, colostomy and ileal conduit were performed. The resected weight was 1,680 grams, and the histopathological diagnosis was myxoid type of liposarcoma, grade II. Postoperatively, a metastatic lesion to vertebrae thoracicae I developed causing radiculoneuropathy of the left superior limb. Spot radiation (50 Gy) was administered to the lesion, but there was no response. Currently, the patient is being treated with combination chemotherapy consisting of dacarbazine, vincristine, adriamycin, and cyclophosphamide. Cases of retrovesical liposarcomas reported in Japan are reviewed and discussed.

Adult↗

[Liposarcoma of the spermatic cord].

A case of well differentiated liposarcoma of the spermatic cord in a 80 year old man is presented. The preoperative diagnosis of spermatic cord liposarcoma is not easy; however, a careful comparison between clinical and ultrasonographic findings can lead to diagnostic suspicion. The treatment of choice is the excision of the liposarcoma associated with orchiectomy and high ligation of the spermatic cord to reduce the risk of recurrence. Postoperative radiotherapy may be indicated for poor differentiated liposarcomas.

Aged↗

Retroperitoneal liposarcomas. Study of 10 cases.

INTRODUCTION: Retroperitoneal liposarcomas are unusual tumors. Their biological and clinical features different from other retroperitoneal sarcomas. AIM: To analyze our series of retroperitoneal liposarcomas. PATIENTS AND METHODS: Retrospective study of 10 patients with retroperitoneal liposarcoma diagnosed in the last 16 years at our institution. Symptoms, diagnosis, treatment and prognosis were analyzed. RESULTS: Radical resection was done in all cases. Postoperative mortality was 10%. Complementary treatment was considered in each patient. Recurrent disease occurred in 8 patients (14 recurrences), during follow up. In 42% of them recurrences were resected. Actuarial 5-year survival rate was 44%. CONCLUSION: Poor prognosis of retroperitoneal liposarcoma is due to its high recurrence rate.

Adult↗

Primary myxoid liposarcoma with rhabdomyoblastic differentiation.

We report a unique case of primary myxoid liposarcoma of the floor of the mouth, in which well-differentiated rhabdomyoblasts were present. The tumor was incompletely excised. The patient is free of recurrence or metastasis following a course of radiotherapy. Heterologous components found in a liposarcoma include leiomyosarcoma, rhabdomyosarcoma, chondrosarcoma, osteosarcoma, and angiosarcoma. These elements have been described mainly in dedifferentiated liposarcomas situated in the retroperitoneum. To our knowledge, there has been only one report of a liposarcoma with rhabdomyosarcomatous differentiation occurring in the absence of a dedifferentiated component. The clinical implications of the presence of heterologous components without dedifferentiation are unclear.

Adult↗

Fine-needle aspiration in liposarcoma: cytohistologic correlative study including well-differentiated, myxoid, and pleomorphic variants.

We have reviewed cytopathology and the corresponding histopathology material of 86 liposarcomas (55 patients) seen at Institut Curie. The liposarcomas (LS) were well differentiated in 14 cases (9 pure, 2 dedifferentiated, 3 sclerosing), 64 myxoid, and 8 pleomorphic. Twenty-four tumors were primary, 34 recurrent, and 28 secondary. Smears in LS were composed in different proportions of round, spindle cells, lipoblasts, and myxoid and vascular arborizing structures. Pure well-differentiated LS were frequently composed of lipoblasts, and round or spindle cells were occasionally seen. Dedifferentiated and sclerosing liposarcomas were composed of spindle or round cells, but lipoblasts were also occasionally present. Myxoid or vascular arborizing structures were absent. Myxoid LS (including round and spindle cell LS) frequently showed a myxoid background and less frequently vascular arborizing structures. Tumor cells were round or spindle. Lipoblasts were also seen. Pleomorphic LS were composed of an admixture of all cellular and stromal elements. Well-differentiated LS should be distinguished from hibernoma and spindle cell lipoma, and myxoid LS from myxoma, myxoid chondrosarcoma, chordoma, myxoid leiomyosarcoma, and myxoid malignant fibrous histiocytoma. The demonstration of the specific translocation t(12;16)(q13;p11) of myxoid LS is very helpful to establish the diagnosis. Pleomorphic LS should be differentiated from other high-grade sarcomas, whenever possible.

Adolescent↗