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Adhesion proteins, cellular morphology and fibrous components around the cell/extracellular-matrix interface in myxoid liposarcomas.

We examined the cell/extracellular-matrix interface in 13 myxoid liposarcomas by determining the distribution of collagen and reticular fibers in the myxoid matrix, the presence of adhesion proteins and the morphological features of the cytoplasmic border. Adhesion proteins (fibronectin, integrin alpha3) and the cytoplasmic border were examined by immunofluorescence and a differential interference-contrast image analysis respectively. A network of reticular fibers and collagen fibers was present in the myxoid matrix of 11 cases (85%) and 3 cases (23%) respectively. Tumor cells with dendritic cytoplasmic processes were observed in 8 cases (62%). Adhesion proteins were sparsely present in tumor cells and there was no correlation between those proteins and collagen fibers, reticular fibers or cytoplasmic processes. Collagen fibers, dense reticular fibers or well-developed cytoplasmic processes were more frequently observed in the cases of long-term-surviving patients than those with recently developed tumors or patients who died. All 3 cases positive for collagen fibers also contained both dense reticular fibers and cytoplasmic processes. Our findings suggest that the fibrous components in the myxoid matrix and the well-developed cytoplasmic processes may limit the invasiveness of malignant cells. This peculiar architecture may also explain the slowly progressive nature of myxoid liposarcomas.

Adult↗

Characterization of the CHOP breakpoints and fusion transcripts in myxoid liposarcomas with the 12;16 translocation.

Myxoid liposarcomas are cytogenetically characterized by t(12;16)(q13;p11). The translocation results in rearrangements of the CHOP gene in 12q13 and the FUS gene in 16p11, creating a fusion gene where the RNA-binding domain of FUS is replaced by the DNA-binding and leucine zipper dimerization domain of CHOP. In the present study, we have mapped 16 genomic breakpoints in the region of the CHOP gene and isolated and sequenced a new variant (type II) of the chimeric FUS/CHOP transcript. The genomic breakpoints were dispersed along a 7.50-kilobase pair region from a SstI cleavage site upstream of the promoter of CHOP to a PstI cleavage site within intron 1. Reverse transcriptase-polymerase chain reaction analysis of tumor samples demonstrated the presence of two variant fragments, 654 base pairs (type I) and 378 base pairs (type II) in size. Of the 13 samples analyzed, 7 showed the smaller, 3 showed the larger, and 3 showed both types of transcripts. We cloned and sequenced the two fragments and found in type II a novel fusion point in the FUS mRNA 275 base pairs upstream of that present in the type I transcript. In both types of transcripts the interrupted FUS is followed by the entire exon 2 of CHOP. As a consequence the normally nontranslated exon 2 is translated and in both types there is in the junction between FUS and CHOP a shift from a FUS glycine codon to a valine codon in the chimeric mRNA.

Base Sequence↗

[Cytologic diagnosis of myxoid liposarcomas of soft tissues].

Myxoid sarcomas are characterized by the presence of branching capillaries in preparations around which lipogenesis is proceeded (unilocular and multilocular). Fat is chemically neutral. The cell content is represented by two basic kinds of cells: 1. stellate lipoblast -- a cambial element of the tumor, and 2. mature lipocyte -- a terminal stage of the differentiation of stellate lipoblast. Moreover, variable intermediate forms demonstrating the stage of lipoge nesis may be encountered. The differential diagnosis between the former and intramuscular myxoma and neurinoma (Antony B type) was made. The work is based on the analysis of 10 cases of myxoid liposarcomas.

Adult↗

[Myxoid liposarcoma of the mediastinum].

We describe a mediastinal myxoid liposarcoma case in a 47-year-old woman who complained of dyspnea. This kind of tumor is rare and becomes symptomatic by compression on mediastinal structures, especially on the respiratory tract. These neoplasms are therefore often voluminous at the time the diagnosis is made. Histopathologic examination is always necessary as much for diagnosis as prognosis. Surgery, whether radical or palliative, obtain the best therapeutic survival results in myxoid liposarcoma of the mediastinum.

Female↗

Unusual skeletal metastases from myxoid liposarcoma only detectable by MR imaging.

We present two cases of skeletal metastases from myxoid liposarcoma, occurring several years after treatment of the primary tumors in the lower limb. The present two case reports have unusual radiological features only detectable by MR imaging and not by plain radiographs or bone scans. From the present two cases, we found that a negative plain radiograph of the spine or a negative bone scan could not exclude skeletal metastases from myxoid liposarcoma, and MRI was a more sensitive screening procedure for their detection, especially in T1-weighted images. Unusual radiological features of skeletal metastases from myxoid liposarcoma are not well documented and only a few cases have been previously reported. Our aim is to document two more patients exhibiting the unusual radiological features of skeletal metastases from myxoid liposarcoma to improve their early detection and management.

Adult↗

Myxoid liposarcoma metastatic to the thoracic epidural space without bone involvement: report of two cases.

Myxoid liposarcoma can frequently metastasize to extrapulmonary sites. We present two cases of myxoid liposarcoma metastatic to the epidural space. Both patients complained of back pain, but plain radiography revealed no abnormality. MR imaging clearly demonstrated metastatic tumors in the epidural space, but no involvement of vertebra. When patients with myxoid liposarcoma complain of back pain, metastasis in the epidural space should be considered even in patients without bone involvement.

Adult↗

Myxoid liposarcoma metastatic to the thyroid gland: a case report and literature review.

We present the second reported case of a myxoid liposarcoma metastatic to the thyroid gland in a 51-year-old gentleman with previous liposarcoma of the right thigh. Myxoid liposarcoma has a relatively good prognosis but tends to recur locally. Metastases affecting the thyroid gland are a rare entity and most commonly arise from the kidney, lung or breast. Clinical presentation, patterns of recurrence and prognosis of myxoid liposarcoma and metastases to the thyroid gland are discussed.

Antineoplastic Agents↗

Cytogenetic and immunohistochemical profile of myxoid liposarcoma.

Cytogenetic and immunohistochemical studies were performed in nine myxoid liposarcomas. The tumor karyotype was determined after short-term culture of cells in vitro. Immunohistochemical studies were performed on frozen tissue in five cases and on paraffin-embedded tissue in three cases. Chromosomal analysis demonstrated a balanced translocation t(12;16) (q13;p11) as the sole abnormality in four cases. Two cases showed an association with other abnormalities. Three tumors showed variants of the t(12;16) translocation involving other chromosomes. In all cases studied, the 12q13 breakpoint was involved in rearrangements. In the majority of cases, immunohistochemical studies demonstrated vimentin (9 of 9) and S-100 protein (8 of 9). Strong focal expression of desmin was observed in two tumors. Weak focal expression was observed in three tumors. Two tumors, which were both desmin positive, showed focal expression of MSA and alpha-SMA. Strong expression of CD36 was present in all four cases that were studied for this marker. CD34 was negative in tumor cells, but it highlighted an intricate capillary network in the tumor. Close relationship between the tumor cells and pericapillary pericytes was demonstrated with CD34 and alpha-SMA strains. The authors conclude that myxoid liposarcoma is characterized by a specific chromosomal rearrangement. Its immunohistochemical profile is wider than previously believed, including expression of muscle markers.

Adult↗

Myxoid liposarcoma: magnetic resonance imaging appearances with clinical and histological correlation.

Myxoid liposarcoma is the most common type of liposarcoma. The magnetic resonance imaging (MRI) features of this tumor were evaluated and correlated with its clinical and histological features in seven patients to determine under what circumstances the tumor should be considered in differential diagnosis and why its signal intensity differs from those of lipoma and lipoma-like (lipoblastic) liposarcoma. In all patients the tumor presented in a lower extremity (5 thigh, 2 calf) as a painless, slowly growing mass which had been present for several months to several years. MRI examination revealed the tumors to be encapsulated, noninfiltrating, and usually septated. On T1-weighted sequences five of seven lesions (71%) showed lacy, amorphous, or linear foci of high signal within a low signal mass. These foci are believed to represent fat within the tumor and distinguish it from several other benign and malignant masses. If an indolent mass in a lower extremity demonstrates a predominantly low signal with a few amorphous or linear high signal foci on T1-weighted sequences, one should consider the possibility of myxoid liposarcoma even if it appears benign by all other criteria. Histologic evaluation showed that myxoid liposarcomas contain less than 10% mature fat, which accounts for their low signal on T1-weighted sequences in contrast to the high signal of lipomas and lipoblastic liposarcomas.

Connective Tissue↗

Translocation t(12;16)(q13;p11) in myxoid liposarcoma and round cell liposarcoma: molecular and cytogenetic analysis.

Translocation t(12;16)(q13;p11) is regarded as a diagnostic marker for myxoid liposarcoma. Cytogenetic data on round cell liposarcomas and combined myxoid and round cell tumors is scarce, and the genetic basis of progression of myxoid tumors to high grade, round cell lesions is unknown. We have accumulated six round cell, four combined myxoid and round cell, and three myxoid liposarcomas for analysis. t(12;16)(q13;p11) was present in three round cell lesions and was detectable in all of the tumors by DNA analysis. In each tumor type, the CHOP gene in 12q13 was rearranged and fused to the TLS gene in 16p11. A variant TLS-CHOP RNA transcript was detected by polymerase chain reaction but did not correlate with clinicopathological data. No distinguishing cytogenetic or molecular markers for round cell or mixed lesions were found. The histogenic and genetic relatedness of myxoid and round cell liposarcomas is apparent from these data.

Adult↗

Metastatic myxoid liposarcoma in the mesentery: what is debated? Case report and a review of the literature.

Myxoid liposarcoma, unlike other types of liposarcoma, has an unusual propensity to metastasize to extrapulmonary sites including the abdominal cavity. Metastatic myxoid liposarcoma is an important condition because it may indicate distant micrometastatic disease. We report the case of a 47-year-old woman with a metastatic myxoid liposarcoma in the mesentery. Surgical excision with a tumor-free margin was achieved, but cardiac and liver metastases developed. We review the available literature and discuss what is still debated about this disease.

Diagnosis, Differential↗

Physical mapping of chromosome 12q breakpoints in lipoma, pleomorphic salivary gland adenoma, uterine leiomyoma, and myxoid liposarcoma.

We report here the physical mapping of recurrent chromosome 12q13-q15 breakpoints in cell lines derived from primary myxoid liposarcoma, lipoma, uterine leiomyoma, and pleomorphic adenoma of the salivary glands. In fluorescence in situ hybridization (FISH) experiments, we first mapped the position of the chromosome 12 translocation breakpoint in uterine leiomyoma cell line LM-30.1/SV40 relative to loci COL2A1, D12S4, D12S17, D12S6, D12S6, D12S19, D12S8, and D12S7. It mapped between linkage probes CRI-C86 (D12S19) and p7G11 (D12S8). We then isolated YAC clones using CRI-C86- and p7G11-derived sequence-tagged sites, constructed corresponding YAC contigs of 310 and 800 kb, respectively, and established long-range physical maps of these. Cosmid clones LLNL12NCO1-98C10 and LLNL12NCO1-113D12 were isolated using STSs within the CRI-C86- and the p7G11-derived YAC contigs, respectively, and a mixture of them was used to routinely study the various tumor cell lines by FISH analysis. The chromosome 12 breakpoints of all tumor cell lines tested mapped between cosmids LLNL12NCO1-98C10 and LLNL12NCO1-113D12. None of the breakpoints appeared to map within any of the isolated YAC clones. Furthermore, FISH analysis using cosmid LLNL12-NCO1-144G3, which maps at the CHOP locus, revealed that the chromosome 12 breakpoints in all cell lines of the three benign solid tumors that were tested were located distal to the chromosome 12 translocation breakpoint with the CHOP gene in myxoid liposarcoma cells with t(12;16). In conclusion, our studies seem to indicate that the chromosome 12 breakpoints of myxoid liposarcoma, lipoma, uterine leiomyoma, and pleomorphic adenoma of the salivary glands are all clustered within the 7-cM interval between D12S19 and D12S8, with those of the benign solid tumors distal to CHOP. Finally, the MYF5 gene mapped telomeric to LLNL12NCO1-113D12, and the MIP gene mapped centromeric to the chromosome 12 translocation breakpoint in myxoid liposarcoma cells.

Adenoma↗

Combined use of surgery and radiation in the treatment of an intradural myxoid liposarcoma in a dog.

An intradural-extramedullary myxoid liposarcoma of the high cervical spine was diagnosed in a 9-year-old, spayed female Cavalier King Charles spaniel that was presented for a 2-month history of cervical pain and tetraparesis. Radiation therapy applied after surgery resulted in complete remission of the neurological deficits. The tumor recurred 18 months after surgical excision. A second surgery and another course of radiotherapy again resulted in complete remission of the clinical signs. The dog was euthanized 11 months after the second surgery because of tumor recurrence.

Animals↗

Cytogenetic analysis of a case of myxoid liposarcoma with cartilaginous differentiation.

The cytogenetic analysis of a patient with a myxoid liposarcoma exhibiting cartilaginous differentiation is presented. A complex translocation involving chromosome 12, 16, and 19 was found, instead of the t(12;16), specific for myxoid liposarcoma. The involvement of 19q13 in a tumor with cartilaginous differentiation, and the assignment of TGF beta 1 to 19q13.1-13.2, which appears to play a role in the formation of bone and cartilage, suggest a possible relation between both.

Adult↗

A parapharyngeal myxoid liposarcoma.

We present a case of a parapharyngeal space myxoid liposarcoma. This case highlights the importance of wide surgical resection margins, and the difficult histological diagnosis.

Aged↗

Treatment of myxoid liposarcoma in pregnancy.

Two cases of pregnancy associated with myxoid liposarcoma are presented. Both patients were treated with hyperthermoradiotherapy after the induced delivery of healthy infants and by surgical excision of the tumor 5 to 7 weeks after delivery. No local recurrence or distant metastasis occurred; the children have progressed normally for 4 and 5 years, respectively, after surgery. Because coexistence of pregnancy and sarcoma is rare, it is difficult for the clinician to develop an appropriate approach to the management of bone and soft tissue sarcoma during pregnancy. The literature was reviewed regarding the effect that pregnancy may have on the clinical behavior of sarcoma and the effect treatment for sarcoma may have on the fetus.

Adult↗

Myxoid liposarcoma of the scalp: case report and literature review.

Myxoid liposarcoma of the head and neck is an extremely rare entity. The scalp region represents a risk factor to the patient because the diagnosis is usually made late, and the surgeon must have a high index of suspicion for this entity because suctioning the tumor without taking a biopsy further delays an accurate diagnosis.

Biopsy↗