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PubMed1997

Combined myxoid liposarcoma and angiolipoma of the spermatic cord.

To date, only 14 cases of myxoid liposarcoma of the spermatic cord have been reported in the literature. The growth is a rare, usually highly differentiated malignancy that tends to recur locally, rarely metastasizes, and has a good prognosis following complete removal. We describe a new case of a highly differentiated myxoid liposarcoma of the spermatic cord, combined with an angiolipoma. Left inguinal orchiectomy with high ligation of the cord was performed. Staging revealed no evidence of regional or distal metastases. By the latest visit, at 30 months, the tumor had not recurred. The diagnosis, management, and prognosis of this oncologic entity are discussed in the light of the literature.

Angiolipoma↗
PubMed1999

Induction of a secreted protein by the myxoid liposarcoma oncogene.

The TLS-CHOP oncoprotein, found in the majority of human myxoid liposarcomas, consists of a fusion between the transcription factor CHOP/GADD153 and the N terminus of an RNA-binding protein TLS/FUS. Clinical correlation and in vitro transformation assays indicate that the N terminus of TLS plays an important role in oncogenesis by TLS-CHOP. Until now, however, the only activity attributed to the oncoprotein is that of inhibiting the binding of transcription factors of the C/EBP class to certain adipogenic target genes, a function that TLS-CHOP shares with the nononcogenic CHOP protein. Here we report the isolation of a gene, DOL54, that is activated in primary fibroblasts by the expression of TLS-CHOP. DOL54 is expressed in the neoplastic component of human myxoid liposarcomas and increases the tumorigenicity of cells injected in nude mice. Activation of DOL54 requires an intact DNA-binding and dimerization domain in TLS-CHOP, a suitable cellular dimerization partner, and depends on the TLS N terminus. Normal adipocytic differentiation is associated with an early and transient expression of DOL54, and the gene encodes a secreted protein that is tightly associated with the cell surface or extracellular matrix. TLS-CHOP thus leads to the unscheduled expression of a gene that is normally associated with adipocytic differentiation.

Animals↗
PubMed2006

[A case of myxoid liposarcoma in the right inguinal region: a case report].

We report a case of myxoid liposarcoma developed in the right inguinal region. A 45-year-old man visited our hospital with a chief complaint of painless right inguinal mass. He was diagnosed with right direct inguinal hernia for the first time and received a hernia repair. However, the size of the inguinal mass increased gradually after the operation. Then, the tumor was re-evaluated by imaging study such as ultrasonography, computed tomography and magnetic resonance imaging. A fatty mass 4.0 cm in diameter was demonstrated and the tumor was diagnosed as a liposarcoma preoperatively. In addition to the right high orchiectomy, surrounding fatty tissue was also removed at the time of the operation. Histological examination revealed a myxoid liposarcoma and the existence of several daughter regions outside the pseudo-capsule. Right spermatic cord and testis were intact. Postoperatively, he received 54 Gy dose of radiation therapy to right inguinal region for the prevention of the local recurrence of the disease.

Abdominal Neoplasms↗
PubMed2000

Monoclonality of multifocal myxoid liposarcoma: confirmation by analysis of TLS-CHOP or EWS-CHOP rearrangements.

Multifocal presentation, defined as the presence of tumor at two or more anatomically separate sites, before the manifestation of disease in sites where sarcomas usually metastasize (e.g., lungs) occurs in about 1% of extremity soft tissue sarcomas (STSs). Debate still persists whether multifocal STSs represent an unusual pattern of metastasis or multiple separate primary tumors. Among STSs with multifocal presentation, myxoid liposarcoma is the predominant histological type. This subtype of liposarcoma contains the specific t(12;16) chromosomal translocation, which results in rearrangement of the TLS and CHOP genes that is clone specific at the DNA level. We, therefore, sought to address the question of clonality by molecular analysis in six patients who presented with either synchronous or metachronous multifocal myxoid liposarcoma. In all six cases, adequate frozen tumor was available for DNA extraction from at least two distinct anatomical sites. Southern blot analysis using CHOP, TLS, and EWS cDNA probes was performed on genomic DNA. Five cases contained a TLS-CHOP rearrangement, and one case had the variant EWS-CHOP fusion (seen in <5% of cases). The size of the rearranged CHOP fragment differed among the six patients, as expected, but was identical in all anatomically separate tumor samples from each patient. Likewise, the sizes of the rearranged bands observed with either the TLS or EWS probes supported the monoclonality of all cases. Our results confirm the monoclonal origin of multifocal myxoid liposarcoma, establishing the metastatic nature of distant soft tissue lesions in these cases. It remains unclear whether this unusual pattern of metastasis represents an intrinsic property of this subset of myxoid liposarcoma or merely a rare chance occurrence. The clinical outcomes observed in this small series suggest that the prognosis of multifocal myxoid liposarcoma is poor, regardless of its often bland or "low-grade" histological appearance.

Adult↗
PubMed2001

Analysis of human telomerase reverse transcriptase mRNA (hTERT) expression in myxoid liposarcomas using LightCycler real-time quantitative reverse transcriptase-polymerase chain reaction.

We describe a convenient, nonradioactive reverse transcription--polymerase chain reaktion (RT-PCR) method for the rapid and accurate quantitative detection of the human telomerase catalytic subunit human telomerase reverse transcriptase (hTERT) mRNA. The LightCycler TeloTAGGG hTERT Quantification Kit (Roche Molecular Biochemicals) was designed to be used for the highly sensitive and quantitative detection of hTERT mRNA relative to the house-keeping gene porphobilinogen deaminase (PBGD). As a tumor progression model, we investigated 26 myxoid liposarcomas (11 pure myxoid grade I, 15 myxoid/round cell grade II/III) for the hTERT expression level and compared the results of the new method with former measurements performed in silver-stained polyacrylamide gels. Both methods revealed similar results, with real-time RT-PCR being the more accurate quantification technique, which also saves time and material. Elevated hTERT expression (cut-off ratio x 100 at 1.3) was an indicator of round cell components and hence for tumor progression in myxoid liposarcoma. The new method is capable of differentiating between pure myxoid and myxoid/round cell liposarcomas for hTERT-expression more accurately.

Computer Systems↗
PubMed1996

Myxoid liposarcoma of the urinary bladder.

We report a case of myxoid liposarcoma of the urinary bladder, that occurred in a 36-year-old woman. We faced the difficulties in reaching the preoperative diagnosis, likewise in determining the extent of the tumor, as the results of the computed tomography differed from the pathologic findings of the tumor. The review of the literature concerning this unusual site for liposarcoma and methods of treatment is also included.

Adult↗
PubMedDate not supplied

Myxoid liposarcoma in a 12-year-old girl.

A 12-year-old girl developed a myxoid liposarcoma on the inner side of her right thigh. Liposarcomas are extremely rare at this age compared to benign lipoblastomas, which are the most frequent tumors of fatty tissue in childhood. The prognosis of myxoid liposarcoma is good, especially when, as in this patient, the tumor is located in the subcutis where a large and deep excision is possible and often curative.

Biopsy, Needle↗
PubMed1991

Trisomy 8 as a nonrandom secondary change in myxoid liposarcoma.

We report the cytogenetic findings in 5 cases of myxoid liposarcoma following short-term culture. In all 5 tumors a t(12:16)(q13:p11), characteristic of the myxoid form of liposarcoma, was observed. Trisomy 8 was present in two tumors as the only additional change and in a third in addition to other abnormalities. In the other two tumors the t(12:16) was present as the sole change. Three other myxoid liposarcomas with trisomy 8 as an additional aberration have been reported, suggesting that it could represent a non-random secondary event in these tumors.

Adult↗
PubMed2001

[Primary pulmonary myxoid liposarcoma discovered fortuitously].

We report a fortuitous discovery of primary pulmonary myxoid liposarcoma in an HIV-positive patient. Primary pulmonary localizations are uncommon. Generally, pulmonary localizations are metastatic. There is a male predominance and diagnosis is generally made around 40 years of age. The two main features of liposarcoma are the large tumor size and the complex histology that evolves over time. Pathology findings are rarely reproducible and vary from one pathologist to another. Macroscopically, liposarcomas can mimic benign tumors. The risk of recurrence is high after simple enucleation due to microscopic extracapsular extensions. Surgery remains the predominant treatment. Wide complete excision, if possible, provides long-term survival.

Diagnosis, Differential↗
PubMed2002

[Myxoid liposarcoma of the mediastinum: a case report].

We describe a mediastinal myxoid liposarcoma in a 47-year-old man who developed recurrent lung infection, treated by surgery associated with radiotherapy. The report of this case and a review of literature bring the authors to outline the epidemiological, clinical and therapeutic profile of this rare tumor.

Combined Modality Therapy↗
PubMed1996

Fusion of the EWS and CHOP genes in myxoid liposarcoma.

The translocation t(12;16)(q13;p11), which cytogenetically characterizes myxoid liposarcomas (MLS), results in a fusion of the CHOP gene in 12q13 and the FUS gene in 16p11, creating a chimeric FUS/CHOP gene. We have identified two cases of MLS with translocations giving rise to recombination between 12q13 and 22q12. The result was a fusion of the N-terminal part of the EWS gene in 22q12, involved in a number of mesenchymal tumor types, with the CHOP gene and the creation of an EWS/CHOP chimeric gene. The presence of the EWS/CHOP chimeric gene in MLS shows that (i) the N-terminal part of FUS may be replaced by the N-terminal part of EWS in a CHOP fusion oncoprotein (ii) the two N-terminal parts, when fused to certain transcription factors, have a common or very similar oncogenic potential and (iii) the tumorigenic process in MLS and the morphogenetically distinctly different EWS-associated tumor types may be related.

Amino Acid Sequence↗
PubMed1998

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↗
PubMed1997

Additional evidence of a variant translocation t(12;22) with EWS/CHOP fusion in myxoid liposarcoma: clinicopathological features.

It is well established that the majority of myxoid/round cell liposarcomas (LPS) are characterized by a reciprocal translocation t(12;16)(q13;p11) which at the molecular level results infusion of the CHOP and FUS/TLS genes. It is assumed that functional characterization of these genes may provide insight into the molecular pathogenesis of this tumour type. This study describes two new cases of myxoid/round cell LPS having a t(12;22). By reverse transcription-polymerase chain reaction (RT-PCR) it has been shown that this leads to fusion between the CHOP and EWS genes, thus indicating involvement of the EWS gene, at least occasionally, in yet another sarcoma type. Combining these two cases with two others which were recently similarly characterized at the molecular level, their clinicopathological features have been compared with cases having the more usual t(12;16). It was not possible to identify any clinical or pathological differences between these molecular genetic subsets. The relevance or significance of these gene fusion products in myxoid/round cell LPS remains to be determined.

Adult↗
PubMed2005

High 2-deoxy-2[F-18]fluoro-D-glucose uptake on positron emission tomography in hibernoma originally thought to be myxoid liposarcoma.

PURPOSE: The purpose of the study is to describe the rare tumor on 2-deoxy-2[F-18]fluoro-D-glucose (FDG) positron emission tomography (PET). PROCEDURE: A 33-year-old male was diagnosed with high uptake lesion on FDG-PET scanning, which was found to be hibernoma on excision. RESULTS: Hibernoma, originally confused with liposarcoma based on its PET and computed tomography presentation, was excised and correctly identified by pathology. CONCLUSION: Although found to be benign, radiological and FDG-PET scanning results were indistinguishable from malignancy, and biopsy is required to exclude neoplasm.

Adult↗