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PubMed2000

Liposarcoma: new entities and evolving concepts.

Liposarcoma is the most common soft tissue sarcoma and accounts for approximately 20% of all mesenchymal malignancies. In the last decade, the results of several studies have led to the delineation of new variants as well as to the introduction of new concepts, mainly as a result of the fruitful interactions between genetics and pathology. Spindle cell liposarcoma represents an uncommon variant of well-differentiated liposarcoma. It tends to occur in adults and often involves the subcutaneous soft tissue. However, from the observation of a larger number of cases, the anatomic distribution of spindle cell liposarcoma seems to be comparable to that of the other well-differentiated liposarcoma subtypes. Spindle cell liposarcoma tends to recur locally and may dedifferentiate. Morphologically it is composed of a fairly bland neural-like spindle cell proliferation set in a fibrous and/or myxoid background and is associated with an atypical lipomatous component. Great debate has been generated by the introduction of the term atypical lipoma to emphasize the fact that well-differentiated liposarcoma shows risk of local recurrence but no potential for metastasis. In our opinion well-differentiated liposarcoma and atypical lipoma should be considered synonyms that describe lesions identical both morphologically and kayotypically. Dedifferentiated liposarcoma is a distinct type of liposarcoma in which transition from low-grade to high-grade nonlipogenic morphology within a well-differentiated liposarcoma is observed. The transition usually occurs in an abrupt fashion; however, in rare cases it can be more gradual. Recently, it also has been proposed that dedifferentiated liposarcoma should be further classified into low and high grade. Dedifferentiated liposarcoma rarely exhibits heterologous (most often myoid) differentiation. A peculiar "neural-like whorling pattern" of dedifferentiation also has been described recently. Surprisingly, the clinical outcome of dedifferentiated liposarcoma is less aggressive that in other high-grade pleomorphic sarcomas but genetic as well as molecular data exist that may partiallyjustify such a discrepancy. Myxoid and round cell liposarcoma, even if still classified by the World Health Organization as two distinct subtypes, share both clinical and morphologic features. Lesions combining both patterns are very frequent and wide agreement exists in considering round cell liposarcoma as the high-grade counterpart of myxoid liposarcoma. Furthermore, myxoid and round cell liposarcoma share the same characteristic chromosome change. Albeit rare, it has been recently shown that liposarcoma indeed can occur as a primary skin lesion. It often presents clinically as a dome-shaped or polypoid lesion that, histologically, most frequently shows high-grade morphologic features but carries a comparatively good prognosis. Considering currently available data, the most logical classification of liposarcoma is into three main groups: (1) well-differentiated liposarcoma (including adipocytic, sclerosing, inflammatory, spindle-cell, and dedifferentiated variants), characterized by ring or long markers chromosomes derived from the long arm of chromosome 12; (2) myxoid and round cell (poorly differentiated myxoid) liposarcoma, characterized in most cases by a reciprocal translocation t(12;16)(q13;p11); and (3) pleomorphic liposarcoma, characterized by complex karyotypes.

Adult↗

PubMed2002

Soft tissue liposarcoma: histological subtypes, MRI and CT findings.

PURPOSE: Liposarcoma is the second most common malignancy of soft tissues. The commonly accepted classification of liposarcoma includes five basic histological categories: well-differentiated, myxoid, round cell, dedifferentiated and pleomorphic liposarcoma. The clinical behaviour of liposarcoma closely reflects its histological appearance, so that to identify the histological subtypes is very important for both prognosis and therapy. The aim of this study was to ascertain whether the histological features of liposarcoma subtypes can be correlated with MRI (Magnetic Resonance Imaging) and CT (Computed Tomography) findings by retrospectively evaluating nineteen cases of histologically-proved liposarcoma. MATERIALS AND METHODS: The MRI examinations performed over the past eight years on nineteen patients affected by liposarcoma were retrospectively reviewed. All patients underwent ultrasound and MRI examination; T2 and T1-weighted sequences were available in all cases and fat-saturated sequences in 4 cases; all patients were administered paramagnetic contrast material. CT scans were obtained in twelve patients. All patients had a biopsy, surgical resection and histology. RESULTS: The study group had 7 well-differentiated, 8 myxoid, 3 pleomorphic and 1 round cell liposarcoma. Well-differentiated liposarcomas had largely lipomatous appearance on both CT and MRI, typically with septa and areas showing high signal intensity on T2w MR images and low signal intensity on T1w images, and slightly hypodense compared to the muscle in CT, representing the sarcomatous areas. Myxoid liposarcomas were mildly heterogeneous with typical high signal intensity on T2w images and isointense to the muscle in T1w images, with lacy or linear septa of fatty tissue in six cases. The pleomorphic and round-cell subtypes demonstrated marked heterogeneity on MR images, with areas of necrosis and heterogeneous contrast enhancement, indistinguishable from other high-grade sarcomas. DISCUSSION AND CONCLUSIONS: Well-differentiated liposarcoma may be distinguished from other types of liposarcoma by its largely lipomatous appearance. Myxoid liposarcoma may be distinguished on the basis of its homogeneous or mildly heterogeneous structure due to the large amounts of the extracellular myxoid material that give it its typical MR appearance. Both well-differentiated and myxoid liposarcomas, the most common types accounting for about 50% of all liposarcomas, have a more favourable clinical behaviour than the other histological types. Differentiation of these from the other histological types of liposarcoma therefore has a high significance for prognosis and therapeutical approach. On the basis of our experience and of the literature, we believe that diagnostic imaging and in particular the MR examination may lead to a correct diagnosis.

Adult↗

PubMed2001

Cytogenetic and molecular genetic analyses of liposarcoma and its soft tissue simulators: recognition of new variants and differential diagnosis.

Liposarcoma is one of the most common sarcomas of adults. Its differential diagnosis and accurate subclassification are often problematic; the latter is also important with regard to appropriate treatment and prognosis. We studied a series of 23 liposarcomas that had unusual or previously undescribed features and 10 liposarcoma simulators and correlated the morphologic, cytogenetic, and molecular genetic findings. We found that use of cytogenetic-molecular genetic techniques aids in the distinction between myxoid-round cell liposarcoma and their simulators, chondroid lipoma, myxoid spindle cell-pleomorphic lipoma, cellular intramuscular myxoma, and myxofibrosarcoma. Poorly differentiated forms of round cell liposarcoma lacking morphologic evidence of lipogenesis can also be diagnosed using these techniques; however, the techniques do not aid in distinguishing low-grade myxoid from high-grade round cell liposarcomas. This study also shows that retroperitoneal liposarcomas with myxoid liposarcoma-like zones are part of the morphologic spectrum of well-differentiated-dedifferentiated liposarcoma rather than true myxoid liposarcomas. Perhaps most importantly, our results provide the first molecular genetic evidence that true mixed liposarcomas (mixed well-differentiated and myxoid liposarcoma) do indeed exist. They also unequivocally demonstrate the existence of small, round cell variants of pleomorphic liposarcoma that closely simulate myxoid-round cell liposarcoma.

Adult↗

PubMed2002

[Relationship between expression of c-myc and p53 in liposarcoma].

BACKGROUND & OBJECTIVE: The relationship between liposarcoma and gene c-myc and p53 is not clear. There are also different reports on p53 mutation in liposarcoma. This study was designed to investigate the relationship between the expression of c-myc and p53 genes and liopsarcomas. We hope to better understand the role of the c-myc and p53 genes in molecular biology. METHODS: Immunohistochemical labelled streptavidin-biotin (LSAB) method, single strand conformation polymorphism analysis of polymerase chain reaction products (PCR-SSCP), and DNA sequencing were used. RESULTS: 38.09% (16/42) liposarcoma samples detected were immunohistochemically c-myc protein positive. p53 protein was detected in 52 liposarcomas with the positive staining rate of 48.08% (25/52). In different subtypes of liposarcomas, the positive staining rates of c-myc and p53 gene proteins were much lower in well-differentiated liposarcomas than in the poorly differentiated liposarcomas. There was a positive correlation between c-myc and p53 expression in liposarcoma. There was no statistical significance for the different positive staining rate of c-myc and p53 protein in the primary and the recurrent liposarcoma. Abnomality in the single-stranded DNA pattern was determined by PCR-SSCP analysis in 2 samples (pleomophic liposarcomas). Missense mutation in exon 8 of codon 268 of p53 gene (AAC-->ATC) were detected by DNA sequencing. Another heterozygotic cosense mutation may exist at exon 6 of codon 221 of p53 gene (GAG-->GAA). CONCLUSIONS: The c-myc and p53 protein are associated with the development, differentiation, and malignancy of liposarcoma, but not with the recurrence of liposarcoma. Detecting the level of c-myc and p53 protein expression may be valuable in evaluating the level of differentiation and malignancy of liposarcoma. c-myc and p53 play synergic roles in the cooperation of development of liposarcoma. There appear the point mutation on exon 8, 6 of p53 gene.

DNA↗

PubMed1989

A clinicopathologic comparison of malignant fibrous histiocytoma and liposarcoma.

The diagnosis of MFH depends on the demonstration of histiocytic and fibroblastic functions. MFHs may phagocytose fat; therefore, lipid stains are useless. By electron microscopy, cytoplasmic lipid is membrane-bound. Immunohistochemical staining for vimentin and histiocytic markers may be helpful. Liposarcoma is diagnosed only when there is convincing evidence of synthesis and storage of fat by the tumor cells. By electron microscopy, cytoplasmic lipid is nonmembrane-bound. Both MFH and liposarcoma have subtypes. In MFH, the pleomorphic forms are the most common. Myxoid MFH is less common; all other types are rare. In liposarcoma, the myxoid types are by far the most common. The myxoid types of both MFH and liposarcoma may contain other elements that vary in degree and geographic distribution and that can raise the histologic grade. About 50% of liposarcomas are low-grade tumors; these are almost always purely myxoid. Low-grade myxoid liposarcoma has a much better prognosis than other types. Myxoid liposarcoma has a better prognosis than myxoid MFH. The peak incidence of MFH is in the seventh decade of life whereas that of liposarcoma is in the fifth decade. A substantial number (roughly 25% to 30%) of MFHs occur in the subcutaneous tissue. Clinically they are almost invariably mistaken for ganglion cysts. Liposarcoma, however, is likely to occur in or below the buttocks. Most are in the anterior thigh. Subcutaneous liposarcoma is extremely rare. A tumor in this area is likely to be either a more malignant myxoid MFH or one of the benign atypical lipomatous tumors. In both MFH and liposarcoma, the development of distant lesions is related to the tumor's histologic grade and size and to local recurrence. Favored metastatic sites of MFH are lung and lymph nodes. Favored sites of distant lesions in the myxoid/round cell types of liposarcoma are intra-abdominal, retroperitoneal, other soft-tissue areas (especially in the neck), and bone. Lymph node involvement is very rare. Because myxoid/round cell liposarcomas have a marked propensity to involve intra-abdominal sites, abdominal computed tomography and bone scan are recommended in the initial evaluation and follow-up of high-risk patients (those with high-grade tumors larger than 15 cm and those with local recurrence of intermediate- or high-grade tumors of any size).

Diagnosis, Differential↗

PubMed1999

Classification of human liposarcoma and lipoma using ex vivo proton NMR spectroscopy.

Prognostication in patients with liposarcoma is a complex and controversial subject based on recognition of lipoblasts, adipocyte nuclear atypia, and qualitative estimations of cellularity and cell size. We show here that for 30 patients with liposarcoma and 5 patients with lipoma, spectral differences on high-resolution, magic angle spinning proton nuclear magnetic resonance (hr-MAS 1H-NMR) spectroscopy relate to known biochemical changes and correlate with adipocyte tissue differentiation, histologic cell type, and cellularity. The NMR-visible level of triglyceride is shown to correlate with liposarcoma differentiation, since the triglyceride level in well-differentiated liposarcoma is 33-fold higher on average than for myxoid/round cell liposarcoma, which in turn is 6-fold higher than the dedifferentiated and/or pleomorphic subtypes. The NMR-visible phosphatidylcholine level serves as an estimate of total tissue cell membrane phospholipid mass and was found to correlate with liposarcoma subtype. Pleomorphic liposarcoma, the most aggressive and metastatic subtype, was found to have a threefold increase in NMR-visible phosphatidylcholine level compared with dedifferentiated liposarcoma. The level of NMR-visible phosphatidylcholine was twofold greater in well-differentiated liposarcoma compared with lipoma and was threefold larger for the hypercellular myxoid/round cell subtype compared with the pure myxoid histology. Thus, NMR-derived parameters of tissue lipid may be used for objective distinction of liposarcoma histologic subtype/grade and lipoma from liposarcoma. These biochemical parameters may ultimately improve prognostication in patients with liposarcoma.

Humans↗

PubMed2002

Distinct chromosomal imbalances in pleomorphic and in high-grade dedifferentiated liposarcomas.

Using comparative genomic hybridization, DNA copy number changes were studied in 14 pleomorphic liposarcomas and compared to those detected in high-grade areas of 9 dedifferentiated liposarcomas. A total of 251 gains and 84 losses were detected. The most frequent gains involved subregions of chromosomal arms 12q and 20q (70% each), 5p (57%), 6q and 9q (52% each), 1q, 7p and 17p (48% each), 1p (43%), 6p and 17q (39% each), 20p and 22q (35% each) as well as 7q and 12p (30% each). The same subregions were also affected by 30 high level amplifications. The most frequent losses were found in subregions of chromosomal arms 13q (35%) as well as 11q and 12p (30% each). Overall, gains of chromosomal material were more frequent than losses (p < 0.001). There were significant differences in the frequency and distribution of recurrent chromosomal imbalances between pleomorphic liposarcomas and the dedifferentiated areas of dedifferentiated liposarcomas. Gains of chromosomal material detected predominantly in pleomorphic liposarcomas involved subbands 5p13-p15 (p < 0.010), 1p21 (p < 0.019), 1q21-q22 (p < 0.040) and 7q22 (p < 0.049). Conversely, high level amplifications within chromosomal subregion 12q13-q21 were only found in the dedifferentiated components of dedifferentiated liposarcomas (p < 0.001). Overall, both gains and the less pronounced losses of chromosomal material were more frequent in pleomorphic than in dedifferentiated liposarcomas (p < 0.001 and p < 0.025, respectively). These results show that pleomorphic liposarcomas display a considerable number of recurrent chromosomal imbalances that are essentially different from those present in high-grade areas of dedifferentiated liposarcomas. Therefore, genetic data are considered as a helpful diagnostic adjunct for the discrimination between these 2 types of liposarcoma. The overall higher frequency of chromosomal imbalances in pleomorphic as compared to dedifferentiated liposarcomas could account for the more aggressive biological behavior of pleomorphic relative to dedifferentiated liposarcoma types.

Adult↗

PubMed2002

Liposarcoma of the oral and salivary gland region: a clinicopathologic study of 18 cases with emphasis on specific sites, morphologic subtypes, and clinical outcome.

Liposarcoma is rare in the oral and salivary gland region (OSG), previously described in only case reports and two small series. Clinicopathologic features of a large series of these tumors were studied. Cases coded as "liposarcoma or lipoma" from 1970 to 2000 were searched for in our files. Inclusion required an OSG location and diagnosis by established soft tissue criteria. Dermal, other soft tissue, and intraosseous liposarcomas were excluded. Clinical and pathologic material was reviewed and follow-up obtained. Eighteen liposarcomas were included: 10 from males and 8 from females. The median patient age was 51 years (range, 30-70 years). Specific anatomic locations included buccal mucosa (n = 7), tongue (n = 4), parotid gland (n = 3), soft tissue overlying the mandible (n = 2), and one each of palate and submandibular gland. The average tumor size was 4.2 cm (range, 1.5 to 6.0 cm). Histologically, most tumors were well differentiated, including one atypical lipoma (n = 10), followed by myxoid (n = 5) and dedifferentiated (n = 3). OSG liposarcomas of all subtypes had increased numbers of lipoblasts. All patients were treated with surgical excision alone. Follow-up on 15 patients (83%) over a mean of 16.5 years (range, 2 to 53 years) revealed that three patients had between one and six local recurrences over periods of 18 months to 6 years. Twelve patients were without recurrence, with a mean follow-up of 12.8 years (range, 2-23 years). No patients, including those with dedifferentiated liposarcoma, had metastases or died of disease. OSG liposarcomas are rare tumors of adults, occurring most commonly in the buccal mucosa, tongue, and then parotid gland. There were no pleomorphic liposarcomas in this series; well-differentiated liposarcoma was the most common subtype, which can locally recur but, even with high-grade dedifferentiation, does not necessarily predict poor outcome. Therefore, OSG liposarcomas have better prognosis than liposarcoma in other soft-tissue locations, perhaps based on smaller size at presentation. Complete local excision and careful patient follow-up, without adjuvant therapy, appears to be the best treatment for OSG liposarcoma.

Adult↗

PubMed2001

Well-differentiated liposarcoma (atypical lipomatous tumors).

Well-differentiated (WD) liposarcoma accounts for about 40% to 45% of all liposarcomas therefore representing the larger subgroup of adipocytic malignancies. It tends to occur equally in the retroperitoneum or the limbs followed by the paratesticular area and the mediastinum, with a peak incidence between the fifth and the seventh decades. WD liposarcoma is further subdivided in the adipocytic (lipoma-like), sclerosing, inflammatory, and spindle cell subtypes, of which the first two are by far the commoner. WD adipocytic liposarcoma is composed of a relatively mature adipocytic proliferation, featuring cell size variation as well as at least focal nuclear atypia. A varying number (from many to none) of lipoblasts may be found. Sclerosing WD liposarcoma is characterized microscopically by the presence of scattered distinctive bizarre stromal cells and multivacuolated lipoblasts set in a fibrillary collagenous background. Inflammatory liposarcoma represents a rare variant of WD liposarcoma in which a chronic inflammatory infiltrate predominates to the extent that the differential diagnosis is mainly with nonadipocytic lesions such as inflammatory myofibroblastic tumor, Castleman's disease, and Hodgkin's as well as non-Hodgkin's lymphomas. Spindle cell liposarcoma is the rarest variant and is composed neural-like spindle cell proliferation set in a fibrous and/or myxoid background and associated with an atypical lipomatous component which usually includes lipoblasts. Cytogenetically, WD liposarcoma appears to be relatively homogenous exhibiting characteristic ring as well as giant marker chromosomes containing amplified genetic material derived from the 12q13-15 chromosome region. As WD liposarcomas of any type have no potential for metastasis unless they undergo dedifferentiation, the opportunity to replace the term "WD liposarcoma" with a less frightening denomination has produced a long, sharp debate. WD liposarcoma and atypical lipoma should be considered as synonyms and their use should therefore be determined by the degree of reciprocal comprehension between the surgeon and the pathologist to prevent either inadequate or excessive treatment.

Adipocytes↗

PubMed2001

Myxoid and round cell liposarcoma: a spectrum of myxoid adipocytic neoplasia.

Myxoid and round cell liposarcoma accounts for about 30% to 35% of all liposarcomas and, even if still classified by the World Health Organization (WHO) as 2 distinct subtypes, share both clinical and morphologic features. Lesions combining both patterns are frequent and wide agreement exists in considering round cell liposarcoma as the high grade counterpart of myxoid liposarcoma. Furthermore, myxoid and round cell liposarcoma share the same characteristic chromosome change represented most frequently by a reciprocal translocation t(12;16)(q13;p11) that fuses the CHOP gene with the TLS gene. Clinically, myxoid and round cell liposarcoma tend to occur in the limbs with a peak incidence ranging between the third and the fifth decade and exhibit overall a metastatic rate of approximately 30%. A peculiar tendency to metastasize to the soft tissue is observed that should not be interpreted as multicentricity. Microscopically, purely myxoid liposarcoma is composed by a hypocellular spindle cell proliferation set in a myxoid background and associated with a varying number of monovacuolated lipoblasts. The most helpful morphologic clue is represented by the presence of a thin-walled capillary network organized in a plexiform pattern. The most important morphologic variation observed in myxoid liposarcoma is represented by the occurrence of hypercellular areas that may exhibits an undifferentiated round cell morphology. On the basis of the percentage of hypercellularity/round cell formation, a myxoid/round cell liposarcoma (more than 25% hypercellular/round cell areas) and a round cell liposarcoma (more than 75% hypercellular/round cell areas) are somewhat arbitrarily recognized. Both the recognition and the quantification of hypercellular/round cell areas represents a crucial step in the evaluation of this liposarcoma subtype because hypercellularity appears to correlate with the clinical outcome. In consideration of the intrinsic difficulty in establishing accurately the percentage of high grade areas as well as of application of different cut off values, it appears safer to consider any amount of hypercellularity as prognostically relevant. Careful as well as extensive sampling is mandatory to permit detection of the smallest amount of hypercellularity. The differential diagnosis of myxoid liposarcoma includes benign lesions, such as myxoid spindle cell lipoma, intramuscular myxoma and lipoblastoma, and malignant ones such as low grade myxofibrosarcoma, and extraskeletal myxoid chondrosarcoma. In consideration of the great morphologic variability, the application of both immunohistochemistry and genetics has proved helpful in sorting out the more challenging cases.

Adipocytes↗

PubMedDate not supplied

Correlation of lipid content and composition with liposarcoma histology and grade.

BACKGROUND: The determination of sarcoma grade, histologic type, and differentiation is often pathologist dependent and requires considerable expertise. METHODS: Lipid content and composition was analyzed in ex vivo fat, lipoma, and liposarcoma tissue samples using proton-decoupled 13C nuclear magnetic resonance (13C-NMR) spectroscopy and correlated with the histologic type and grade of liposarcoma. RESULTS: The well-differentiated liposarcomas were found to have threefold increases in fatty acyl chain content compared with benign lipomas. The fatty acyl chain content of the dedifferentiated and pleomorphic liposarcomas was 1% of that found in lipoma and < 0.2% of that found in well-differentiated liposarcoma. The 2.1- to 2.8-fold increase in the degree of polyunsaturation in the dedifferentiated and pleomorphic liposarcomas compared with well-differentiated liposarcoma could largely be accounted for by the 2.3-fold increase in the percentage of fatty acyl chains of lipid containing linoleic acid. The dedifferentiated and pleomorphic liposarcomas contained both free fatty acids and phospholipids that were not NMR detectable in normal fat, lipoma, and well-differentiated liposarcoma. CONCLUSION: Ex vivo 13C-NMR spectroscopy may be used to distinguish lipoma from well-differentiated, dedifferentiated, and pleomorphic liposarcoma based on changes in lipid and phospholipid metabolite profiles and may serve as adjunct to conventional light microscopy for the determination of liposarcoma histologic type and thus grade.

Biomarkers, Tumor↗

PubMed1979

The ultrastructure of liposarcoma. A study of 10 cases.

An ultrastructural study of 10 liposarcomas is reported. Four of the liposarcomas were wholly or predominantly of well-differentiated, lipoma-like or fibrosing type, 3 of myxoid type, 2 of round cell type and 1 pleomorphic type. The well-differentiated, lipoma-like liposarcomas showed cells with a few, large lipid droplets, few organelles and a peripherally located, fairly large nucleus, The well-differentiated liposarcomas of fibrosing type revealed mostly spindle-shaped, fibroblast-like cells, with abundant rough endoplasmic reticulum and inconspicuous lipid inclusions, surrounded by collagen. One well-differentiated liposarcoma contained an area which was similar to brown adipose tissue and hibernoma. The spindle and stellate shaped cells of the myxoid liposarcomas showed abundant rough endoplasmic reticulum and large smooth-membraned vacuoles filled with moderately dense amorphous material, which appeared to be extruded extracellularly by rupture of the vacuoles. Cytoplasmic lipid droplets were seen in most cells but were much less prominent than in the well-differentiated lipoma-like liposarcomas. Ultrastructurally there were many similarities between the myxoid and round cell liposarcoma, indicating a close relationship between the two types. The pleomorphic liposarcoma revealed cells with one or more large, irregular nuclei, numerous large vacuoles after dissolved lipids, abundant dilated cisternae of rough endoplasmic reticulum and rounded, electron-dense bodies corresponding to PAS-positive hyalin globules seen in the light microscope. The ultrastructural study suggests that the variegated cellular appearance of the different subtypes of liposarcoma reflects the wide cellular spectrum seen during the differentiation of adipose tissue and supports the view that all liposarcomas histogenetically represent a single entity.

Adipose Tissue↗

PubMed1996

[Anatomopathology, computerized tomography, and magnetic resonance correlations in soft tissue liposarcoma].

Liposarcomas are one of the most common soft tissue malignant tumors; they can be differentiated in four histologic subtypes: well-differentiated, myxoid, round cell and pleomorphic liposarcomas. The search of differential CT and MR patterns to better classify the lesions in the proper histologic subtype is justified by the different histologic features, clinical course and especially prognosis, of every lesion subtype. From 1990 to 1995, 50 liposarcoma patients were examined preoperatively with CT and MRI in our Institute of Radiology. We found 7 well-differentiated liposarcomas (14%), twenty myxoid liposarcomas (40%), ten round cell liposarcomas (20%) and, finally, 13 pleomorphic liposarcomas (26%). The thigh was the most common tumor site (60%). The following parameters were considered: lesion margins, tissue homogeneity, fat tissue ratio and the presence of calcifications. Well-differentiated liposarcomas presented well-defined and regular margins (72%), mildly heterogeneous appearance (44%) and more than 75% fat tissue (72%). Myxoid liposarcomas presented well-defined and regular margins (65%), heterogeneous appearance (65%) and less than 25% fat tissue (95%). The diagnosis of myxoid liposarcoma can be made in the presence of myxoid tissue, which has very low CT attenuation values and mildly hypointense signal on T1-weighted and progressively hyperintense signal on T2-weighted MR images. Round cell and pleomorphic liposarcomas are high-grade malignancies and they cannot be distinguished from other malignant soft tissue lesions. In these cases, the diagnosis can be made only at histology.

Adult↗

PubMed1998

Primary liposarcoma of the skin: a rare neoplasm with unusual high grade features.

Liposarcoma is the most common soft tissue sarcoma in adults. It presents in three main forms: well-differentiated liposarcoma (which includes adipocytic, sclerosing, inflammatory, spindle cell, and dedifferentiated subtypes), myxoid/round cell liposarcoma, and pleomorphic liposarcoma. Anatomic distribution depends largely on histologic subtype, but the deep soft tissue of the extremities and the retroperitoneum are most frequently affected. Whereas it is accepted that liposarcoma rarely occurs in the subcutaneous soft tissue, the dermis seems to represent an exceedingly rare site of occurrence. The clinicopathologic features of a series of seven primary cutaneous liposarcomas (two atypical lipomatous tumors/well-differentiated liposarcomas, one myxoid/round cell liposarcoma, and four pleomorphic liposarcomas) are analyzed here. Clinically, all of the patients were adults (four men, three women) with a median age of 72 years, and four of seven cases arose on the scalp. Local recurrences occurred in two patients, but no distant metastases or disease-related deaths have been observed. Although follow-up is relatively short, it appears that, exceptionally, liposarcoma occurs primarily in the skin and, despite an apparent tendency to show high-grade morphologic features, it seems to exhibit relatively indolent clinical behavior in this location.

Adipose Tissue↗

PubMed2002

Beta-catenin accumulation and gene mutation in exon 3 in dedifferentiated liposarcoma and malignant fibrous histiocytoma.

CONTEXT: beta-Catenin is an adhesion molecule that also plays a role in the Wnt signaling pathway. Objective.-To analyze beta-catenin mutation and accumulation in a series of liposarcomas and malignant fibrous histiocytomas. DESIGN: beta-Catenin mutation in exon 3 was studied using polymerase chain reaction-single-strand conformation polymorphism (PCR-SSCP) and direct sequencing analysis in 30 formalin-fixed, paraffin-embedded liposarcomas. The tumors included 12 dedifferentiated liposarcomas, characterized by both high-grade anaplastic components and well-differentiated liposarcoma components, plus 18 well-differentiated liposarcomas (10 lipoma-like and 8 sclerosing-type cases). The 2 components of dedifferentiated liposarcomas were analyzed independently. beta-Catenin accumulation in the nuclei or cytoplasm and Ki-67 expression (cell-proliferation marker, MIB-1 labeling index) were examined immunohistochemically. Nine storiform-pleomorphic-type malignant fibrous histiocytomas were also studied. RESULTS: Dedifferentiated liposarcomas showed mutation in 2 cases (17%) and accumulation in 5 cases (42%). One of the 2 cases that showed mutations had a mutation in the well-differentiated component; this mutation was silent. The other case had mutations that differed between the 2 components. In well-differentiated liposarcomas, mutation was not seen in any of the cases (0/18; 0%); however, accumulation was seen frequently in the sclerosing-type cases (5/8; 63%), but not in the lipoma-like cases (0/10; 0%). Malignant fibrous histiocytomas showed mutation and accumulation in 5 (56%) and 4 (44%) cases, respectively, without any exact correlation between the cases. Cases with accumulation had a higher MIB-1 labeling index than those without, among both the sclerosing-type well-differentiated liposarcomas (P <.05) and the malignant fibrous histiocytomas. CONCLUSIONS: Our results suggest the possible involvement of beta-catenin activation caused by beta-catenin mutation in liposarcoma and malignant fibrous histiocytoma, but the contribution would seem to be different, depending on the tumor type. beta-Catenin accumulation is also thought to be related to cell proliferation in some of the cases.

Adult↗

PubMed1997

Contribution of quantitative lectin histochemistry to characterizing well-differentiated, dedifferentiated and poorly differentiated liposarcomas.

OBJECTIVE: To find new diagnostic markers in the group of lipomatous tumors. STUDY DESIGN: The histochemical lectin staining pattern was characterized in a series of 45 lipomatous lesions, including 10 typical lipomas, 6 atypical lipomas, 8 well-differentiated, 6 myxoid, 5 dedifferentiated and 10 pleomorphic liposarcomas. Three lectins were used-peanut (Arachis hypogaea) agglutinin, which binds to terminal Gal(beta 1,3)GalNAc residues; wheat germ (Triticum vulgare) agglutinin (s-WGA, the succinylated form of WGA), which binds to ((1-4)-D-GlcNAc)n and Neu5NAc residues; and jack bean (Concanavalia ensiformis) agglutinin which binds to alpha-D-Man and alpha-D-Glc residues. Histochemical staining was quantitatively measured by means of a cell image processor. RESULTS: In the case of certain carbohydrate residues, typical lipomas closely resemble atypical lipomas, which in turn closely resemble well-differentiated liposarcomas; typical lipomas differ significantly from well-differentiated liposarcomas. This indicates that atypical lipomas, or at least some of them, could represent a biologic link between typical lipomas and well-differentiated liposarcomas. While well-differentiated and pleomorphic liposarcomas differed significantly from each other, the poorly differentiated component of dedifferentiated liposarcomas included histochemical lectin properties, which were common to both well-differentiated and pleomorphic liposarcomas. CONCLUSION: Some atypical lipomas exhibit glycohistochemical characteristics that are common to those of well-differentiated liposarcoma. The poorly differentiated component of dedifferentiated liposarcomas remains more differentiated in terms of glycohistochemical markers than do poorly differentiated pleomorphic liposarcomas.

Adult↗

PubMed1998

Lipoblastoma and liposarcoma in children: an analysis of 9 cases and a review of the literature.

OBJECTIVES: To review the experience at a children's hospital of lipoblastoma and liposarcoma and to identify any factors that would differentiate one type of tumour from the other. DESIGN: A retrospective case series. SETTING: British Columbia's Children's Hospital a tertiary-care pediatric centre. PATIENTS: All patients with a pathological diagnosis of lipoblastoma and liposarcoma recorded over 12 years. MAIN OUTCOME MEASURES: The frequency of lipoblastoma and liposarcoma, identified from biopsy specimens of pediatric adipose tumours. The clinical, pathological and cytogenetic variables between lipoblastoma and liposarcoma. RESULTS: One hundred and forty-nine adipose tumours were recorded. Seven (4.7%) were lipoblastomas and 2 (1.3%) were liposarcomas. All tumours presented as asymptomatic, slow-growing, soft-tissue masses. The children with lipoblastoma tended to be younger, but 29% were over 3 years of age. The liposarcoma patients were aged 9 and 14 years. One liposarcoma was of myxoid type and the other was a round cell variant. Karyotypes were reported for 1 lipoblastoma and 1 liposarcoma. The myxoid liposarcoma karyotype was 46,XY,t(12;16)(q13;p11), and the lipoblastoma was reported as 46,XY,der(8)?t(8q;?),+mar. CONCLUSIONS: Lipoblastoma is an unusual childhood neoplasm and liposarcoma is very rare in children. Both tumours may present in a similar fashion, and differentiating them histologically can be difficult. Age cannot be relied upon to accurately predict their behaviour. The tumour karyotype is very helpful in differentiating these neoplasms.

Adolescent↗

PubMed2000

High telomerase activity and high HTRT mRNA expression differentiate pure myxoid and myxoid/round-cell liposarcomas.

Molecular markers characterizing the transition of a myxoid to a more round-cell liposarcoma have not been described. To examine whether telomerase activity, hTRT and hTR mRNA expression were associated with tumor progression in myxoid liposarcoma, we investigated a total of 28 myxoid liposarcomas (13 pure myxoid tumors, 14 mixed-type tumors, and 1 pure round-cell variant) from 19 patients. Telomerase activity was detected by using the fluorescent PCR-based TRAP-assay. Expression of hTRT and hTR mRNAs was determined by the semi-quantitative RT-PCR. On the basis of only one tumor sample per patient, telomerase activity was found in 9 of 9 myxoid/round-cell liposarcomas and in 3 of 10 pure myxoid tumors. Elevated hTRT expression was found in 13 of 17 liposarcomas. All telomerase-positive tumors showed hTRT expression, whereas there were 3 cases showing hTRT expression without telomerase activity. HTR mRNA expression was elevated in all 19 liposarcomas. Thus, only the levels of telomerase activity and of hTRT mRNA expression differentiated pure myxoid liposarcoma and myxoid/round-cell liposarcoma (p < 0.003 and p = 0.029, respectively). We believe that high levels of telomerase activity and of hTRT expression are associated with tumor progression from low-grade pure myxoid to higher-grade malignant round-cell liposarcoma, and may consequently represent a useful prognostic marker for this histological sub-type of soft-tissue tumors.

Biomarkers↗