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At least 883 records · Page 49Linked to original sources
PubMed2001

Is there a role for incomplete resection in the management of retroperitoneal liposarcomas?

Complete surgical resection is the most effective modality for the treatment of retroperitoneal sarcomas. Previous studies of all types of retroperitoneal sarcomas have not shown a survival benefit of incomplete resection over no resection. Because death often occurs as a result of local progression in retroperitoneal liposarcomas (RPLS), it is possible that incomplete resection may be beneficial in this histologic type. In this study we have sought to determine the clinical outcomes in patients with incompletely resected and unresected RPLS with the aim of defining patients who may benefit from palliative resection. From a prospective clinical database 55 patients with incompletely resected (n = 43) or unresected (n = 12) RPLS were identified between 1982 and 1999. Statistical analyses were performed using the log-rank test and Kaplan-Meier estimates with disease-specific survival as the primary end point. Variables studied included age, gender, recurrent versus primary disease, tumor grade, and tumor size. The patient population consisted of 34 men and 21 women with a mean age of 61 +/- 14 (SD) years. The median time to death was 10 months (range 1 to 83 months) with a median followup of 12 months (range 1 to 60 months) for survivors. Partial resection was an independent factor for increased survival as compared with exploration or biopsy only (median survival 26 versus 4 months, p < 0.0001). Of patients who received incomplete resections, locally recurrent presentation (n = 19) versus primary disease (n = 24) was a negative prognostic variable (median survival 17 versus 46 months, p = 0.009). Successful palliation of symptoms was achieved in 24 of 32 patients (75%) with preoperative symptoms. In select patients with unresectable RPLS, incomplete surgical resection can provide prolongation in survival and successful symptom palliation. Most likely to benefit are those patients presenting with primary tumors, suggesting that surgical resection should be attempted in the majority of patients.

Female↗
PubMed2002

A novel type of EWS-CHOP fusion gene in two cases of myxoid liposarcoma.

Fusion genes consisting of TLS/FUS and CHOP or EWS and CHOP are characteristic markers for myxoid/round cell liposarcomas (MLS/RCLS). Several different structures of the fusion genes were reported in the case of the TLS/FUS-CHOP form, whereas only one type of structure has so far been found for the EWS-CHOP form, which consisted of exons 1 to 7 of the EWS and exons 2 to 4 of the CHOP gene. Here we describe a novel type of EWS-CHOP fusion gene in two cases of MLS/RCLS, which were found in a consecutive analysis of 21 cases. This fusion gene consisted of exons 1 to 10 of the EWS and exons 2 to 4 of the CHOP gene. The two cases with this fusion gene shared several clinical features, such as a large tumor mass, rapid and invasive growth, and local recurrence within 12 months after surgical resection. Histopathological findings also showed common features characterized by the diffuse proliferation of small spindle cells with a primitive mesenchymal appearance. The association of these clinical and histopathological features suggests a distinct biological property for this rare type of fusion product.

Adolescent↗
PubMed1975

Primary liposarcoma of the mastoid.

A unique case of liposarcoma of the mastoid in a 4-year-old child presenting as acute mastoiditis and subperiosteal abscess has been reported.

Abscess↗
PubMed1999

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↗
PubMed2001

Liposarcoma of the spermatic cord.

Primary malignant tumors of the spermatic cord are rare. We report a case of paratesticular liposarcoma and discuss the prognosis of this neoplasm and its therapeutic implications.

Genital Neoplasms, Male↗
PubMed2003

Myxoid liposarcoma of the spermatic cord: a case report and review of the literature.

Myxoid liposarcoma of the spermatic cord (MLSC) is a rare variance of spermatic sarcomas, with only 14 previous cases having been reported. Typically it presents during the seventh decade of life as a painless scrotal or inguinal mass. Although local recurrences are not uncommon, prognosis following complete tumour removal is good because metastases are rare. We describe one new case of a 24-year-old male with incidental MLSC found during inguinal hernia repair, and also review the existing literature.

Adult↗
PubMed1970

Liposarcoma. A review of 60 cases.

Sixty cases of liposarcoma are presented. The pathological appearances, clinical features and methods of treatment are described. The overall 5-year survival was 64%.

Adolescent↗
PubMed1993

Fusion of the dominant negative transcription regulator CHOP with a novel gene FUS by translocation t(12;16) in malignant liposarcoma.

The search for tumour-specific markers is one of the chief goals in cancer biology. We show that the translocation t(12;16)(q13:p11) in malignant myxoid liposarcoma can be a fusion of the CHOP dominant negative transcription factor gene with a novel gene, FUS, which can result in fusion of the FUS glycine-rich protein with the whole CHOP coding region. The data support the concept that protein fusion may commonly occur in solid tumours resulting in tumour-specific markers of potential clinical importance. The data also indicate the importance of transcription disruption in the pathogenesis of solid tumours.

Amino Acid Sequence↗
PubMed2004

Late relapse of myelodysplasia after allogeneic transplantation concomitant with new presentation of invasive liposarcoma as a secondary neoplasm.

Second malignancies are uncommon events in the survivors of allogeneic transplant procedures, although they are increased compared to normal control populations. Among these malignancies, sarcomas are exceedingly rare. In addition, relapse of primary myelodysplasia rarely occurs after 5 years from the time of allogeneic transplantation. This report describes an unusual presentation of liposarcoma with concomitant relapse of underlying myelodysplasia developing in a patient 9 years after the first of two allogeneic transplantations.

Hematopoietic Stem Cell Transplantation↗
PubMed1997

Characteristic sequence motifs at the breakpoints of the hybrid genes FUS/CHOP, EWS/CHOP and FUS/ERG in myxoid liposarcoma and acute myeloid leukemia.

We have sequenced the breakpoint regions in one acute myeloid leukemia (AML) with t(16;21)(p11;q22) resulting in the formation of a FUS/ERG hybrid gene and in four myxoid liposarcomas (MLS), three of which had the translocation t(12;16) (q13;p11) and a FUS/CHOP fusion gene and one with t(12;22;20)(q13;q12;q11) and an EWS/CHOP hybrid gene. The breakpoints were localized to intron 7 of FUS, intron 1 of CHOP, an intronic sequence of ERG and intron 7 of EWS. In two MLS cases with t(12;16) and in the AML, the breaks in intron 7 of FUS had occurred close to each other, a few nucleotides downstream from a TG dinucleotide repeat region. The break in the two MLS had occurred in the same ATGGTG hexamer and in the AML 40 nucleotides upstream from the hexamer. The third case of t(12;16) MLS had a break upstream and near a TC-dinucleotide repeat region and a sequence similar to the chi bacterial recombination element was found to flank the breakpoint. In the MLS with the EWS/ CHOP hybrid gene, the break in intron 7 of EWS had occurred close to an Alu sequence. Similarly, in all 4 MLS, the breaks in intron 1 of CHOP were near an Alu sequence. No Alu or other repetitive sequences were found 250 bp upstream or downstream from the break in the ERG intron involved in the AML case. In the AML, the MLS with ESW/CHOP and in one MLS with FUS/CHOP there were one, two and six, respectively, nucleotide identity between the contributing germline sequences in the breakpoint. In the other two MLS cases, two and three extra nucleotides of unknown origin were inserted between the FUS and CHOP sequences. At the junction and/or in its close vicinity, identical oligomers, frequently containing a trinucleotide TGG, were found in both partner genes. Our data thus show that all four genes-FUS, EWS, CHOP and ERG-contain characteristic motifs in the breakpoint regions which may serve as specific recognition sites for DNA-binding proteins and have functional importance in the recombination events taking place between the chromosomes. Different sequence motifs may, however, play a role in each individual case.

Acute Disease↗
PubMed2004

Comparable transforming capacities and differential gene expression patterns of variant FUS/CHOP fusion transcripts derived from soft tissue liposarcomas.

The chromosomal translocation t(12;16)(q13;p11) is a common genetic alteration in myxoid and round-cell liposarcomas. It results in transcription of various chimeric FUS/CHOP fusion transcripts that encode different oncogenic proteins. Recent reports suggest that these may have different neoplastic transformation activities. To audit this hypothesis, we transfected expression plasmids for the two major variant FUS/CHOP transcripts I and II in NIH 3T3 cells and determined the number of outgrowing foci as well as their growth potential in soft agar. In addition, we compared tumour growth in nude mice upon subcutaneous injection of the respective transfectants. No significant differences in transformation assays in vitro and in vivo were observed, suggesting that both variant transcripts confer comparable transforming activities. The histopathological picture of tumours derived from both cell populations resembles high-grade spindle cell sarcomas. This suggests that both FUS/CHOP variants cause similar patterns of differential gene expression. This hypothesis was confirmed by mRNA-expression profiles of the respective cell clones. Strong overexpression of the pentaxin-related gene (PTX), the osteoblast-specific factor 2 (osf-2), the basic Kruppel-like factor (bklf), the leucoprotease inhibitor, and the cyclophilin B were observed in both types of FUS/CHOP-transfected cell clones. Taken together, our data suggest that different FUS/CHOP variants cause transformation of mesenchymal cells via the same pathways with comparable efficacy.

Animals↗
PubMed2003

Imaging features of primary pulmonary liposarcoma.

Primary liposarcoma of the lung is extremely rare. We report a 28-year-old pregnant woman who complained of dyspnoea during the third trimester. Chest radiography, thoracic ultrasound, CT and MRI showed a huge heterogeneous tumour involving all the left lung and the mediastinum. The tumour was composed of soft tissue, and fatty and cystic components with calcifications. Diagnosis was made on core biopsy under CT guidance. Surgical excision was performed but unfortunately the patient died during the operation.

Adult↗
PubMed2003

Myxoid liposarcoma confirmed by TLS/FUS-CHOP fusion transcripts.

Described here is the use of detecting TLS/FUS-CHOP fusion transcripts to confirm the diagnosis of a myxoid liposarcoma by nested reverse transcription-polymerase chain reaction assay using archival formalin-fixed, paraffin-embedded tissues of a young man with a benign-looking myxoid tumor on his upper extremity, which is often misdiagnosed clinically or histopathologically as a benign tumor.

Adult↗
PubMed2001

Cardiac metastasis from a myxoid liposarcoma.

Intracardiac metastases due to solid tumours are rare. We describe a patient with a myxoid liposarcoma of the thigh and a secondary lesion in the interventricular septum, which was treated with radiotherapy.

Antineoplastic Agents↗
PubMed2001

Primary orbital liposarcoma: case report and review of the literature.

Primary orbital liposarcoma is an extraordinarily rare tumour. Lack of specific diagnostic findings, scarcity of published series and a primary anatomical location make its management complicated. Surgery should conserve visual function in well located, low-grade tumours that do not invade ocular structures. Complementary RT should also be performed.

Biopsy, Needle↗