Embryonal liposarcoma (myxoid liposarcoma): a study of six cases.
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Adipose Tissue↗
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Identification of genes differentially expressed in TLS-CHOP carrying myxoid liposarcomas.
Myxoid liposarcomas (MLS) carry a t(12;16) or, more rarely, a t(12;22) resulting in fusion of the transcription factor gene CHOP on chromosome 12 with TLS/FUS on chromosome 16 or EWS on chromosome 22. The chimeric TLS-CHOP or EWS-CHOP proteins most probably function as abnormal transcription factors, causing transcriptional de-regulation of several target genes and relaxation of functions critical for growth and differentiation control. A PCR-based subtractive hybridization technique was used to identify genes that are differentially expressed in TLS-CHOP-carrying MLS but not in normal fat tissue. Six myxoid-liposarcoma-associated transcripts, MLAT, were isolated. The genes identified as MLAT can be divided into 2 groups. MLAT1, 2 and 6 show high similarity to glia-derived nexin, neuronatin and the RET oncogene, respectively, all normally involved in development of tissues of neural origin. MLAT3 to MLAT5 represent new genes.
CCAAT-Enhancer-Binding Proteins↗
Genomic PCR detects tumor cells in peripheral blood from patients with myxoid liposarcoma.
Myxoid liposarcoma (MLS) is the most common subtype of liposarcoma. The cytogenetic hallmark of MLS is the pathognomonic t(12;16)(q13;p11), present in more than 85% of cases. The translocation leads to the fusion of the CHOP and FUS genes at 12q13 and 16p11, respectively, and the generation of a FUS/CHOP hybrid protein. The presence of a tumor-specific chimeric gene makes it possible to identify MLS cells by polymerase chain reaction (PCR). We have analyzed peripheral blood samples obtained during a 10-year period at diagnosis of primary and/or recurrent disease in 19 MLS patients with t(12;16) and in one MLS patient with t(12;22;20), resulting in the fusion of the CHOP and EWS genes. Nested PCR on genomic DNA from blood samples amplified FUS/CHOP hybrid fragments in three patients and EWS/CHOP in the patient with t(12;22;20). There was no obvious association between PCR findings and clinical outcome, but larger series are needed to draw any firm conclusions.
Adult↗
[Myxoid liposarcoma of the larynx].
The paper presents a rare case of tumor--liposarcoma myxoides of untypical location--in the area of subglottic larynx. Diagnostic problems that resulted in erroneous recognition of the case, leading to improper treatment, are discussed.
Adult↗
Chromosomal evolution and tumor progression in a myxoid liposarcoma.
A myxoid liposarcoma showed macroscopic, histologic, and cytogenetic heterogeneity. In one of three myxoid nodules and in the surrounding lipoma-like tumor tissue, the translocation t(12;16)(q13;p11), known to be specific for myxoid liposarcoma, was found as the sole chromosomal abnormality. In the other two nodules, additional rearrangements involving chromosomes 1, 12, and 16 were found. These aberrations were probably secondary to the primary t(12;16), and are cytogenetic evidence of clonal evolution. The complex chromosome aberrations were present in those tumor parts that had more malignant histology, indicating that the acquisition of secondary chromosomal aberrations parallels the histologic manifestations of tumor progression.
Chromosome Aberrations↗
Fusion of CHOP to a novel RNA-binding protein in human myxoid liposarcoma.
Human myxoid liposarcomas contain a characteristic chromosomal translocation, t(12;16)(q13;p11), that is associated with a structural rearrangement of the gene encoding CHOP, a growth arrest and DNA-damage inducible member of the C/EBP family of transcription factors residing on 12q13.1. Using a CHOP-specific complementary probe and antiserum we report here the presence of an abnormal CHOP transcript and protein in these tumours. Cloning of the translocation-associated CHOP gene product revealed a fusion between CHOP and a gene provisionally named TLS (translocated in liposarcoma). TLS is a novel nuclear RNA-binding protein with extensive sequence similarity to EWS, the product of a gene commonly translocated in Ewing's sarcoma. In TLS-CHOP the RNA-binding domain of TLS is replaced by the DNA-binding and leucine zipper dimerization domain of CHOP. Targeting of a conserved effector domain of RNA-binding proteins to DNA may play a role in tumour formation.
Amino Acid Sequence↗
RNA splicing mediated by YB-1 is inhibited by TLS/CHOP in human myxoid liposarcoma cells.
Human myxoid liposarcoma contains a characteristic t(12;16) chromosomal translocation that results in fusion of the N-terminal domain of the translocated in liposarcoma (TLS) protein to the C/EBP homologous protein (CHOP). TLS possesses structural motifs that suggest it may participate in RNA processing. We demonstrate that in human myxoid liposarcoma cells, wild-type TLS binds to RNA polymerase II (Pol II) via its N-terminal domain and to the transcription and translation factor Y-box binding protein-1 (YB-1) through its C-terminal domain. The liposarcoma fusion protein TLS/CHOP retains the ability to bind RNA Pol II but lacks the ability to recruit YB-1 due to replacement of the C-terminal domain of TLS by CHOP. In an in vivo splicing assay, YB-1 promotes splicing of adenovirus EIA pre-mRNA predominantly to the 13S isoform. The oncogenic TLS/CHOP fusion protein inhibits this splicing function of YB-1 in a dominant negative manner. When considered in conjunction with studies on other sarcoma fusion proteins, these data suggest that aberrant RNA splicing may be a common feature of human sarcomas.
Adenovirus E1A Proteins↗
[Myxoid liposarcoma. MRI imaging].
PURPOSE: Myxoid liposarcoma is the most common type of liposarcoma (approximately 40 to 50% of all liposarcomas). The main tissue component is a myxoid matrix present primarily in extracellular compartments; proliferating lipoblasts account for less 10% of the tumor: MRI appearances are not typical for lipomatous tumor. Nevertheless histological features may permit understanding MRI findings and identifying patients with myxoid liposarcoma. MATERIALS AND METHODS: Clinical history and radiologic images of 7 patients with histologically verified myxoid liposarcoma were retrospectively studied. In all patients the tumor presented in a lower extremity as a painless, slowly growing mass. MR images were available for review in all cases (T1- and T2-weighted images); in addition fat-suppression before and after gadolinium enhancement were assessed with T1-weighted sequences. RESULTS: MRI examination revealed an "encapsulated" tumor, non infiltrating and septated. On T1-weighted sequences all the lesions show lacy, amorphous and linear foci of high signal within a low signal of the tumor due to the predominance of a myxoid matrix. The high sensitivity of MRI demonstrates the presence of small areas of high signal and fat-suppression technique is valuable for characterizing soft-tissue tumors: suppression of high signal intensity on fat-saturated T1-weighted images indicates the presence of fatty tissue components. CONCLUSION: Clinical correlation with MRI appearances on T1-weighted sequences (in addition to fat-suppression technique) may suggest the possibility of myxoid liposarcoma.
Adult↗
Radiation response: an additional unique signature of myxoid liposarcoma.
PURPOSE: Historically, myxoid liposarcoma (MLS) has been reported to show a marked clinical response to radiotherapy (RT), but objective data to support that contention have been lacking. We performed a retrospective analysis of the response of a group of MLS tumors to preoperative RT using tumor dimensions calculated from pretreatment imaging and the subsequent surgical specimen obtained approximately 1 month after RT. METHODS AND MATERIALS: Data were abstracted from the Princess Margaret Hospital prospective database on a series of patients with MLS who had undergone preoperative RT between 1991 and 1999. A group of malignant fibrous histiocytoma (MFH) patients treated on the same protocol served as controls. Pretreatment and posttreatment three-dimensional measurements were obtained; comparison tumor volume measurements were estimated using the ellipsoid formula, and the analysis was also repeated using the maximal tumor dimension. Identical measurement procedures were used for the MLS (experimental) and MFH (control) cases. RESULTS: A total of 16 MLS tumor specimens were available for analysis, and 16 MFH cases were randomly chosen for comparison. The mean pretreatment and posttreatment volume of the MLS tumors was 415 and 199 cm(3), respectively (P = <0.0001). The mean pretreatment and posttreatment volume of the MFH tumors was 264 and 273 cm(3), respectively (p = 0.804). The proportional reduction in the median tumor volume was 59% and -7% for MLS and MFH tumors, respectively. Both the absolute and the proportional reduction in volume for MLS tumors vs. MFH tumors was statistically significant (p = 0.006 and p = 0.002, respectively). CONCLUSION: These results provide the first objective data to support the idea that MLS tumors show a statistically significant reduction in size when treated with RT and that this response is greater than that of MFH tumors given the same RT. These results may be relevant to the management of MLS with combined RT and surgery in difficult anatomic situations in which adequate surgical excision is not feasible. They also confirm another unique characteristic of this unusual subtype of soft tissue sarcoma.
Adult↗
[Pelvic myxoid liposarcoma. A case report].
Myxoid liposarcoma is a rare soft tissue tumour. The authors report one case of retroperitoneal tumour discovered during pregnancy, which recurred after initial surgery. Clinical features, clinical course and prognosis of this disease are examined together with management procedures.
Adult↗
[Myxoid liposarcoma and pleomorphic liposarcoma: cyto-histological correlations].
Myxoid liposarcoma and pleomorphic liposarcoma: cito-histological correlations. A correlative cytologic and histologic study of a myxoid liposarcoma of the shoulder in a 72 year-old man and a pleomorphic liposarcoma observed in the retroperitoneum of a 84 year-old woman, are presented. A preoperative FNAB cytology performed in both cases showed necrotic material containing spindle-stellate shaped cells, interspersed in a myxoid matrix, with rare classical monovacuolated lipoblasts and fragments of plessiform vessels were seen in the first and scattered pleomorphic and multinucleated cells, with prominent nucleoli and numerous atypical mitosis in the second. A malignant mesenchimal spindle-cells tumor, with myxoid matrix and pleomorphic cells, consistent with liposarcoma, respectively were suspected. Gross and histological specimens confirmed the cytological suspect. Authors discuss main cyto-histological differential diagnoses of myxoid tumors, and point out the importance and a correct differentiation between myxoid liposarcoma and intramuscular myxoma. The cytologic appearance of pleomorphic liposarcoma is similar to histologic type and therefore the problem of a differential diagnosis with soft tissue tumors is analogous. When mono or plurivacuolated lipoblasts are absent, differential diagnosis between pleomorphic histiocytoma and liposarcoma is impossible. Nevertheless this is not a important problem at cytological level because both tumors have a had prognosis and must be treated with radical surgery. Definition of correct histologic type will be more suitable on histologic specimens.
Aged↗
Pericardial metastasis of myxoid liposarcoma causing cardiac tamponade.
Although myxoid liposarcoma often metastasizes to various organs, cardiac metastasis is rare. We present herein a rare case with pericardial metastasis of myxoid liposarcoma, which expanded the pericardial sac extraordinarily and required an emergency operation because of acute cardiac tamponade. We undertook a review of the English literature regarding liposarcoma.
Cardiac Tamponade↗
Myxoid liposarcoma: appearance at MR imaging with histologic correlation.
Although myxoid liposarcoma is a subtype of liposarcoma, it may be difficult to establish the correct diagnosis with magnetic resonance (MR) imaging due to the lack of fat signal intensity. Without the administration of gadolinium contrast material, the tumor may even mimic a cystic tumor. A spectrum of MR imaging abnormalities occur in myxoid liposarcoma, depending on the amount of fat and myxoid material, the degree of cellularity and vascularity, and the presence of necrosis. Most myxoid liposarcomas have lacy or linear, amorphous foci of fat. Some myxoid liposarcomas appear to be cystic at nonenhanced MR imaging, although they enhance like other solid masses at contrast material-enhanced MR imaging. The enhancing areas within the tumor represent increased cellularity and vascularity; the nonenhancing areas represent necrosis, reduced cellularity, and accumulated mucinous material. Gadolinium-enhanced imaging is important in differentiating myxoid liposarcoma from benign cystic tumors. Characterization of the tumor with MR imaging plays an important role in the management of myxoid liposarcoma.
Adipose Tissue↗
A novel FUS/CHOP chimera in myxoid liposarcoma.
The cytogenetic hallmark of myxoid liposarcoma is the chromosomal aberration t(12;16)(q13;p11), which is pathognomonic for this tumor type. The translocation results in the hybrid gene FUS/CHOP, where the central and C-terminal parts of FUS, coding for the RNA binding domain and the RGG triplet motif, are replaced by the full length CHOP protein. Thus, CHOP is under the control of the FUS promoter and the FUS/CHOP chimera contains the 5'-terminal part of FUS which provides a transcriptional activation function. Although different structural variations of the FUS/CHOP chimeric transcript have been reported, none of them contains the parts of FUS encoding the RNA binding properties. An explanation is the location of the genomic breakpoint in FUS, which frequently occurs in the region spanning exon 5 to intron 8. We describe here a case of myxoid liposarcoma containing two novel FUS/CHOP chimeric transcripts and with the breakpoint occurring in intron 14 of FUS. Reverse transcription-polymerase chain reaction, using FUS forward and CHOP reverse primers, amplified strongly a 2.1-kbp DNA fragment and weakly a 0.9-kbp DNA fragment. Direct sequencing showed that in the 2.1-kbp transcript nt 1474, which corresponds to the third nucleotide of exon 14 of FUS, was in-frame fused to exon 2 of CHOP. In the 0.9-kbp DNA fragment, exon 3 of FUS was in-frame fused to exon 2 of CHOP. Genomic analyses revealed that the breaks were located at the end of exon 14/beginning of intron 14 of FUS and in intron 1 of CHOP and that microdeletions had occurred in the close vicinity of the breakpoints.
Amino Acid Sequence↗
Myxoid liposarcoma. Experience with chemotherapy.
BACKGROUND: Myxoid liposarcoma (ML) is the most common type of liposarcoma. It has been classified as an intermediate grade tumor with a definite metastatic potential but a relatively indolent natural history. Little is known about its sensitivity to chemotherapy. METHOD: The authors reviewed their experience with chemotherapy in ML from 1986 to 1992. The patient population was identified through a search of the database maintained by the Department of Melanoma-Sarcoma Medical Oncology of the M.D. Anderson Cancer Center. RESULTS: Forty-four patients each with a histologically confirmed diagnosis of ML were identified. Twenty-one were treated with chemotherapy. The median age was 45 years (31-69 years); there were 14 men and 7 women. The ML in 19 patients was in the lower extremity, one in the head and neck, and one pelvic. The median size of the primary tumor was 15 cm (range, 7-48 cm) in maximum dimension. Of the 18 patients who received doxorubicin- and dacarbazine-based chemotherapy as a frontline regimen [median of 3 (2-9) cycles] and were evaluable for response, 8 (1 completed response, 7 partial responses) achieved an objective response (44%, 95% confidence interval 21-67%). Two of the remaining three patients who were also treated with a similar regimen were not evaluable for response (one received chemotherapy postoperatively, and the other received concomitant radiation and doxorubicin), and the third patient received ifosfamide as frontline chemotherapy because of a significant cardiac history. Seven patients received chemotherapy in the neoadjuvant setting, 13 for recurrent or metastatic disease, and 1 postoperatively after complete tumor resection. At the last follow-up, 10 patients were alive with no evidence of disease, 3 were alive with disease, and 8 had died. The median follow-up was 51 months (range, 6-199 months) from diagnosis. CONCLUSION: The authors conclude that doxorubicin- and dacarbazine-based chemotherapy is effective in the treatment of ML.
Adult↗
Isolated pancreatic metastasis of extremity myxoid liposarcoma: Report of a case.
Myxoid liposarcoma (ML) has a high predilection for extrapulmonary sites of metastases, including intra-abdominal metastases, but pancreatic involvement is extremely rare. Here, we report the case of a 66-year-old male patient, who underwent pancreaticoduodenectomy for isolated pancreatic metastasis of ML of the left lower extremity that had been excised 6 years before. Completion pancreatectomy was necessitated afterwards for a delayed haemorrage associated with pancreatic fistula. Currently the patient is alive with no evidence of disease. Highly selected patients with isolated pancreatic metastasis of soft tissue sarcoma may benefit from a curative surgical resection.
Aged↗
Dedifferentiated myxoid liposarcoma: a clinicopathological study suggesting a closer relationship between myxoid and well-differentiated liposarcoma.
Myxoid/round cell liposarcoma is arguably the commonest type of liposarcoma occurring in the extremities and may show gradual progression from low-grade, pure myxoid liposarcoma to high-grade round cell liposarcoma. Rarely myxoid/round cell liposarcoma is associated with areas of well-differentiated or pleomorphic liposarcoma (mixed liposarcoma). We describe the clinicopathological features of three unusual myxoid/round cell liposarcomas which showed morphological features of de novo dedifferentiation. All patients were male and were aged 66, 70 and 76 years, respectively. One lesion each arose in the retroperitoneum, inguinal region and peritoneal cavity. Histologically, in one case the myxoid/round cell component was juxtaposed to a high-grade non-lipogenic component resembling non-pleomorphic storiform 'malignant fibrous histiocytoma' ('MFH'), one case showed a combination of myxoid liposarcoma and a high-grade myxofibrosarcoma-like component (so-called myxoid 'MFH'), and in the third case, a well-differentiated myxoid liposarcoma with a discontinuous micronodular pattern of dedifferentiation was seen. Follow-up information of 30, 28 and 26 months revealed two recurrences each in two patients. These patients died of postoperative pulmonary embolism and abdominal haemorrhage, respectively; systemic metastases were not noted. These cases demonstrate that myxoid/round cell liposarcoma can show, albeit very rarely, histological features of dedifferentiation. Cases like these, combined with the occurrence of mixed-type liposarcoma (well-differentiated/myxoid liposarcoma) and the vicinity of chromosomal regions involved by specific karyotypic aberrations in these tumours, suggest that myxoid/round cell liposarcoma and well-differentiated liposarcoma (including its dedifferentiated variant) are more closely related in biological terms than is generally believed.
Abdominal Neoplasms↗
Metastatic patterns of extremity myxoid liposarcoma and their outcome.
BACKGROUND AND OBJECTIVES: Extremity myxoid liposarcomas have a unique extrapulmonary metastatic potential. We studied the metastatic pattern of extremity liposarcomas to determine what types of posttreatment imaging may be of value in the follow-up these patients. METHODS: Twenty-two patients from a total of 128 patients with primary extremity liposarcoma were treated at a tertiary care institution for subsequent metastases from January 1981 to January 2000. Median follow-up was 45 months (range: 6-270 months). Data on these patients was prospectively collected and then retrospectively analyzed for effect of metastatic pattern and treatment on outcome. RESULTS: Of these 22 patients, extrapulmonary metastases developed in 10, combined pulmonary and extrapulmonary metastases developed in 6, and isolated pulmonary metastases developed in 6. Of the 16 patients with extrapulmonary metastases, 13 were of the myxoid subtype. Of the 49 patients with extremity myxoid liposarcomas, metastases developed in 14 (29%). The most common sites of metastases among these 14 patients include: the retroperitoneum, 10 patients (71)%; intra-abdominal extra-hepatic, 7 patients (50%); spinal/paraspinal, 6 patients (43%). Only 3 of the patients are alive and disease free and all 3 of these patients are from the subgroup of 10 patients with only extra-pulmonary metastases (2 intra-abdominal and 1 retroperitoneal). CONCLUSIONS: Extremity myxoid liposarcomas have an unusually high predilection for extra-pulmonary metastases, frequently without any pulmonary metastases. After treatment of the primary tumor, these patients should be followed with periodic chest X-ray and abdominal/pelvic computed tomography (CT) scans. Any back or neurologic complaints should prompt additional imaging of the appropriate spinal area. Consideration should be given to surgical and adjuvant treatment of metastatic disease when appropriate.
Abdominal Neoplasms↗