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PubMedDate not supplied

Primary dedifferentiated liposarcoma of the retroperitoneum. Prognostic significance of computed tomography and magnetic resonance imaging features.

OBJECTIVES: To describe computed tomography (CT) and magnetic resonance (MR) imaging findings and to determine the prognostic significance of radiologic appearances in primary dedifferentiated liposarcoma of the retroperitoneum. METHODS: Initial CT and MR imaging studies of 20 pathologically confirmed cases of primary dedifferentiated liposarcoma of the retroperitoneum were retrospectively reviewed and assessed for correlations with the histopathologic features. CT and MR images were evaluated by 2 radiologists with agreement by consensus, and univariate analyses were conducted to evaluate survival with a mean clinical follow-up duration of 47 months (range, 5-114 months). RESULTS: Tumor invasion was more frequent in the anterior or posterior pararenal originating tumors than in pararenal tumors (P<0.05). Well-defined nonlipomatous masses juxtaposed with fatty tumors were identified in all cases. Calcification or ossification was seen in 6 patients (30%) on unenhanced CT. Imaging findings including attenuation, signal characteristics, and enhancement patterns of nonlipomatous masses were nonspecific regardless of histologic variances. Recurrent tumors (n=6) tended to invade surrounding organs. Univariate analysis revealed that calcification or ossification (P<0.05) and first recurrence with duration of a mean 13 months (P<0.05) identified by imaging studies had significant impacts on overall survival. CONCLUSIONS: Calcification or ossification and first recurrence identified by CT and MR imaging studies are significant adverse prognostic factors in primary dedifferentiated liposarcoma of the retroperitoneum.

Female↗
PubMed1995

Liposarcoma of the spermatic cord: report of 6 cases and review of the literature.

Liposarcoma of the spermatic cord is rare. We report 6 cases with followup ranging from 3 months to 23 years from presentation. Initially 4 liposarcomas were well differentiated and 2 had poorly differentiated components. All 6 patients underwent radical orchiectomy and 3 required additional surgical procedures. Although most liposarcomas of the spermatic cord are of low grade and stage, the propensity for local recurrence is high. The importance of long-term followup cannot be overemphasized.

Adult↗
PubMed2003

Comparative genomic hybridization study of paraffin-embedded dedifferentiated liposarcoma fixed with Holland Bouin's fluid.

Dedifferentiated and differentiated liposarcoma are characterized by 12q15 chromosomal amplification. Comparative genomic hybridization is a powerful tool able to detect DNA copy number changes in the genome. This technique has been widely used in frozen tumors and in some studies in paraffin-embedded tumors fixed with formalin. The purpose of this study was to demonstrate the ability of CGH to detect DNA copy number changes in the genome when the DNA was extracted from tissues fixed with Holland Bouin's fluid. Sixteen liposarcoma tumors both frozen and fixed in Holland Bouin's fluid were characterized by CGH. Eighty-one percent of the main chromosomal alterations detected in the frozen liposarcomas (amp 12q15, amp 6q23, amp 1p32, amp 16q22, +7, +8) were detected in the corresponding fixed tumors. The limitation of this technique when using Holland Bouin's fluid extracted DNA compared with formalin-extracted DNA was the yield of analyzable samples. Eighty-one percent of tumors fixed with Holland Bouin's fluid (13/16) were analyzable compared with 100% of formalin-fixed tumors (4/4). This study demonstrates that comparative genomic hybridization is a useful tool even if only fixed tissues (formalin and Holland Bouin's fluid tissues) are available, and that it allows more tumors to be analyzed in retrospective studies.

Acetic Acid↗
PubMed2004

Myxoid liposarcoma with adipocytic maturation: detection of TLS/CHOP fusion gene transcript.

This report describes a 49-year-old woman with a well-circumscribed nodule of liposarcoma. The patient noticed a soft, slowly growing mass at the right sural region. Both axial computed tomography and magnetic resonance imaging revealed a soft tissue tumor consisting of nonfatty lesion measuring 5 x 3 x 3 cm circumscribed by a 1-cm thickened fatty area. Histologically, the tumor was made of 2 distinct components: the inner component of the tumor was a classic myxoid liposarcoma with numerous lipoblasts; the outer component was a lipoma-like lesion consisting of mature adipocytes without atypical nuclei. Immunohistochemically, MDM2 overexpression was observed and p53 immunophenotype was negative in both components. Molecular analysis revealed that type 1 TLS/ CHOP fusion gene transcript, characteristic of myxoid/round cell liposarcoma, was detected in both areas.

Adipocytes↗
PubMed2005

Transperineal resection of a retroperitoneal liposarcoma presenting as a perineal mass.

BACKGROUND: Liposarcomas can present as giant liposarcomas in the retroperitoneal position. CASE: A healthy woman presented with a large recurrent proboscis-type mass at the left gluteus and underwent a transperineal resection of the mass. The patient tolerated the procedure well and was discharged after a short hospitalization. CONCLUSION: Once invasion into adjacent structures has been ruled out, a transperineal resection of giant retroperitoneal liposarcomas can be performed.

Biopsy, Needle↗
PubMed2006

Formation of the 12q14-q15 amplicon precedes the development of a well-differentiated liposarcoma arising from a nonchondroid pulmonary hamartoma.

Pulmonary hamartoma is a benign neoplasm that rarely recurs or undergoes malignant transformation. Herein, we report a 48-year-old woman with a history of an incomplete excised nonchondroid pulmonary hamartoma presenting as an indolent tumor recurrence. Excision of the tumor revealed a well-differentiated liposarcoma arising from the hamartomatous component. Fluorescence in situ hybridation analysis for HMGA2 and MDM2 was performed on both hamartomatous and liposarcomatous component. MDM2 and HMGA2 amplification were found in a subset of stromal cells in the hamartomatous component and in most cells of the well-differentiated liposarcoma. No rearrangement HMGA2 was found in the pulmonary hamartoma component. These findings suggest that the formation of the 12q14-q15 chromosome amplicon, the characteristic cytogenetic finding of well-differentiated liposarcomas and the structural genomic component of the supernumerary ring and giant rod chromosomes, occurred before the morphologic changes characteristic of these malignant adipose tissue tumors and likely represents a very early molecular event in their development.

Chromosomes, Human, Pair 12↗
PubMed2024

Extracellular Vesicle- MDM2 -DNA as a Potential Liquid Biopsy Biomarker for Disease Identification in Retroperitoneal Liposarcoma.

OBJECTIVE: We aimed to assess the levels of MDM2 -DNA within extracellular vesicles (EVs) isolated from the serum of retroperitoneal liposarcoma (RLS) patients versus healthy donors, as well as within the same patients at the time of surgery versus postoperative surveillance visits. To determine whether EV- MDM2 may serve as a possible first-ever biomarker of liposarcoma recurrence. BACKGROUND: A hallmark of well-differentiated and dedifferentiated (WD/DD) retroperitoneal liposarcoma is elevated MDM2 due to genome amplification, with recurrence rates of >50% even after complete resection. Imaging technologies frequently cannot resolve recurrent WD/DD-RLS versus postoperative scarring. Early detection of recurrent lesions, for which biomarkers are lacking, would guide surveillance and treatment decisions. METHODS: WD/DD-RLS serum samples were collected both at the time of surgery and during follow-up visits from 42 patients, along with sera from healthy donors (n=14). EVs were isolated, DNA purified, and MDM2 -DNA levels determined through q-PCR analysis. Nonparametric tests were employed to compare EV- MDM2 DNA levels from patients versus the control group, as well as the time of surgery versus postsurgery conditions. RESULTS: EV -MDM2 levels were significantly higher in WD/DD-RLS than controls ( P =0.00085). Moreover, EV- MDM2 levels were remarkably decreased in WD/DD-RLS patients after resection ( P =0.00036), reaching values comparable to control group ( P =0.124). During postoperative surveillance, significant increases of EV- MDM2 were observed in some patients, correlating with computed tomography scan evidence of recurrent or persistent postresection disease. CONCLUSIONS: Serum EV- MDM2 may serve as a potential biomarker of early recurrent or postoperatively persistent WD/DD-RLS, a disease currently lacking such determinants.

Humans↗
PubMed1983

Pleomorphic lipoma: a tumour simulating liposarcoma.

Pleomorphic lipoma or giant-cell lipoma is a recently recognized entity affecting predominantly elderly and middle-aged men. The neck, shoulder region and back are the sites of predilection. The clinical setting is similar to that seen with spindle-cell lipoma. The lesion is characterized by an intricate mixture of mature fibrous tissue, adipose tissue and myxoid tissue interspersed with cellular foci. Most characteristic of the latter are a variety of giant cells and especially the 'floret' giant-cells, so named because of the arrangement of their nuclei which is reminiscent of the petals of a flower. The pleomorphism of the lesion frequently leads to misdiagnosis as liposarcoma. Criteria for the differentiation from the various types of liposarcoma are discussed. The possible relationship of pleomorphic lipoma to some of the 'atypical lipomas' described in the recent literature is analysed. The pleomorphic lipoma is a benign lesion of subcutaneous tissue which must be sharply differentiated from sarcomas. Some of the liposarcomas alleged in the literature to have originated within a pre-existing lipoma possibly represented pleomorphic lipomas. Pleomorphic lipoma is an entity which must be added to the growing number of pseudosarcomatous lesions of soft tissue.

Adipose Tissue↗
PubMed2005

Soft-tissue angiomatosis in adulthood: a case in the forearm showing a prominent myxoid adipose tissue component mimicking liposarcoma.

Soft-tissue angiomatosis is a rare condition characterized by diffuse proliferation of benign vascular structures, accompanied by mature adipose tissue, which affect a large segment of the body in a contiguous fashion. Most patients present in childhood or adolescence. We report a case of large soft-tissue angiomatosis, located in the left forearm of a 65-year-old man, which showed extensive intramuscular and intermuscular infiltration, and embracement of the radius and the main vascular and neural bundles of the forearm. Due to the deep location, large size, ill-defined limits and non-homogeneous composition with areas suggestive of an adipose component in the radiological examination, the lesion raised the possibility clinically of a liposarcoma. The myxoid adipose tissue component of the lesion, a feature previously undescribed, and the predominance of small, capillary-sized vessels were considered concordant with the clinical diagnosis. This presentation is rare. The features of the lesion made complete surgical excision impossible and amputation of the extremity was considered the best treatment. The main differential diagnoses include infiltrating lipoma, myxolipoma, angiomyxolipoma, angiolipoma, intramuscular angioma, liposarcoma and low-grade myxofibrosarcoma. In conclusion, angiomatosis might simulate liposarcoma, particularly if the mass is large, heterogeneous, myxoid and appears later in life.

Adipose Tissue↗
PubMed2005

Giant retroperitoneal liposarcoma.

Liposarcomas are one of the most common soft tissue sarcomas of adulthood. Liposarcomas are remarkable because of their frequently large size. We report a case with an extremely large dedifferentiated liposarcoma that weighed 18 kg. Although it was capsulated and could be excised en bloc, the patient suffered a relapse 9 months later and died 1 year after the operation.

Abdominal Neoplasms↗
PubMed2004

Giant lipomas: a series of 12 consecutive cases and a giant liposarcoma of the thigh.

BACKGROUND: The clinical presentation and character of giant lipomas create diagnostic problems because of their close relationship with other mesenchymal benign and malign tumors. It is therefore of primary importance for the clinician to be familiar with epidemiologic and anatomic attributes of these lesions. OBJECTIVE: To evaluate and compare epidemiologic and clinical characteristics of giant lipomas operated in our clinic. METHODS: Twelve giant lipomas and one giant liposarcoma are included in the study. RESULTS: There were seven females and six males. The patients ranged in age between 22 and 75. The tumor duration was between 2 months and 40 years. The smallest lipoma size was 10x5x3 cm, and the largest was 19x9x5 cm. Two of the lipomas were located intermuscular. Mild hematoma formation was observed in two patients. Histopathologic evaluation was done for all specimens, and one myxoid liposarcoma was encountered. CONCLUSION: When dealing with giant lipomas, liposarcomas should always be included in the differential diagnosis.

Adult↗
PubMed1997

Fibrohistiocytic differentiation in subcutaneous fatty tumors. Study of spindle cell, pleomorphic, myxoid, and atypical lipoma and dedifferentiated liposarcoma cases composed in part of CD34+ fibroblasts and FXIIIa+ histiocytes.

Subsets of dendritic cells, fibroblasts which express the human progenitor cell antigen CD34 or histiocytes which express coagulation factor XIIIa (FXIIIa), are present in fat and in collagenous connective tissue. As components of the microvascular unit, these fibrohistiocytic cell subsets may interact during stromal remodeling, repair, and neoplasia. We studied white fat and subcutaneous fatty tumors to determine if CD34 and/or FXIIIa+ "fibrohistiocytic" dendritic cell subsets are involved in their morphogenesis. Three lipomas (L), 1 intramuscular lipoma (IL), 1 myxoid lipoma (ML), 2 pleomorphic lipomas (PL), 2 spindle cell lipomas (SCL), 8 angiolipomas (AN) in 4 patients, 1 atypical lipoma/well-differentiated liposarcoma (AL), 1 de novo dedifferentiated liposarcoma (DL), and 1 recurring atypical myxoid signet ring lipomatous tumor were examined for CD34, FXIIIa and in some cases for CD31, desmin, Ki 67, or S-100. Normal fat has scattered CD34+ dendritic cells and small FXIIIa+ dendritic histiocytes among variably S-100+ adipocytes. The CD34 and FXIIIa+ dendritic cells are more numerous near vessels and within fibrovascular septae. In L and IL, CD34 and FXIIIa+ dendritic cells are activated and some adipocytes express CD34. Mesenchymal areas of SCL, PL, ML, and AL and DL are composed of CD34+ dendritic cells with CD34+ but FXIIIa-negative floret cells in PL or atypical cells in AL and DL. FXIIIa+ dendritic cells are numerous in these lesions, comprising 30-40% of cells in SCL and PL, and 50% in ML, AL, and DL. AN have focal CD34+ interstitial cells and plump FXIIIa+ cells that in one case resembled multivacuolated lipoblasts. The myxoid signet ring lipomatous tumor was CD34 negative with few FXIIIa+ cells. We conclude that subsets of CD34+ and FXIIIa+ dendritic microvascular cells are present in normal fat and proliferate together in various types of lipomas and in at least some dedifferentiated liposarcomas.

Aged↗
PubMed1999

Mixed-type liposarcoma of the oral cavity: a case with unusual features and a long survival.

A case of mixed-type liposarcoma, which showed unusual dedifferentiation in the recurrence, is reported. The rapidly growing mass in the palate of a 60-year-old Japanese woman first revealed a combination of myxoid liposarcoma with features resembling storiform malignant fibrous histiocytoma. The recurrent neoplasm, showing an abrupt transition between myxoid and non-lipogenic parts, partially reverted to sclerosing well-differentiated liposarcoma. The patient died 10.1 years after the first operation.

Fatal Outcome↗
PubMed2004

Liposarcoma circumscriptum (lipoma-like) of the tongue: report of a case.

Liposarcoma, first described by Virchow in 1857, is the second most frequent sarcoma of soft tissues, although it is rare both in the head and neck and the oral cavity. Intra-orally, liposarcoma has been reported in the jawbones (particularly the maxilla) and the soft tissues -- mainly the cheek and floor of mouth, but it is rare in the tongue. A case of well-differentiated, superficial liposarcoma circumscriptum of the tongue is reported. The authors underline the difficulties in the clinical and histopathological diagnosis, as this tumour may be confused with lipoma. In view of the indolent behaviour of this tumour type, local recurrence or metastasis rarely occur; consequently, conservative surgical therapy is advised without adjuvant chemo-radio therapy.

Diagnosis, Differential↗
PubMed1993

Primary liposarcoma of the omentum.

This report is of a 54 year old female with omental liposarcoma. Diagnosis was made by ultrasonography, computed tomography, angiography and operation. The patient was treated by surgical excision. The histological examination revealed that the tumour was round cell liposarcoma. This is the first report to our knowledge of round cell liposarcoma of the omentum.

Angiography↗
PubMed1980

Radionuclide imaging of lipoma and liposarcoma.

Radionuclide imaging with technetium-99m phosphate compounds was performed in 18 patients with deep-seated soft-tissue masses. The histologically established diagnosis was intra- or intermuscular lipoma in 13 patients and liposarcoma in five. Radionuclide scans demonstrated disparate scintigraphic characteristics in these two often clinically similar lesions. Soft-tissue radionuclide accumulation occurred in two lipomas and corresponded to radiographically evident calcification. All liposarcomas were uncalcified and demonstrated intense radionuclide activity. This activity may be related to the angiographic and histologic hypervascularity of liposarcomas, which is also demonstrable on computed tomography as contrast enhancement; hence, these modalities offer the same nonspecific diagnostic information. It is concluded, however, that plain radiography and radionuclide scaning should suffice to establish the diagnosis of lipoma.

Adult↗
PubMed2006

Expression of the FUS-CHOP fusion protein in primary mesenchymal progenitor cells gives rise to a model of myxoid liposarcoma.

A subset of sarcomas is associated with specific chromosomal translocations that give rise to fusion genes believed to participate in transformation and oncogenesis. Identification of the primary cell environment that provides permissiveness for the oncogenic potential of these fusion genes is essential to understand sarcoma pathogenesis. We have recently shown that expression of the EWS-FLI-1 fusion protein in primary mesenchymal progenitor cells (MPCs) suffices to develop Ewing's sarcoma-like tumors in mice. Because most sarcomas bearing unique chromosomal translocations are believed to originate from common progenitor cells, and because MPCs populate most organs, we expressed the sarcoma-associated fusion proteins FUS/TLS-CHOP, EWS-ATF1, and SYT-SSX1 in MPCs and tested the tumorigenic potential of these cells in vivo. Whereas expression of EWS-ATF1 and SYT-SSX1 failed to transform MPCs, FUS-CHOP-expressing cells formed tumors resembling human myxoid liposarcoma. Transcription profile analysis of these tumors revealed induction of transcripts known to be associated with myxoid liposarcoma and novel candidate genes, including PDGFA, whose expression was confirmed in human tumor samples. MPC(FUS-CHOP) and the previously described MPC(EWS-FLI-1) tumors displayed distinct transcription profiles, consistent with the different target gene repertoires of their respective fusion proteins. Unexpectedly, a set of genes implicated in cell survival and adhesion displayed similar behavior in the two tumors, suggesting events that may be common to primary MPC transformation. Taken together, our observations suggest that expression of FUS-CHOP may be the initiating event in myxoid liposarcoma pathogenesis, and that MPCs may constitute one cell type from which these tumors originate.

Animals↗