SEARCH · Search PubMed
Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.
Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.
A case of successful operative treatment of an intracavitary liposarcoma of the right ventricle is presented. Comparison with the only other reported case is made and methods of diagnosis and treatment are discussed.
Concerning two cases of liposarcomas developing in the retroperitoneal space, the characteristics of these rare tumors are reviewed. They are the most frequent mesenchymal tumors. Often, they are uncovered by palpable abdominal mass or when they compress adjoining organs. Computed tomography has proved valuable in the diagnosis of these lesions. Surgical removal is the treatment of choice. Recurrences are frequent and prognosis is poor.
The FUS gene, which maps to 16p11, is fused to the CHOP gene in the t(12;16) (q13;p11) that characterizes myxoid liposarcomas (MLS) and to the ERG gene in acute myeloid leukemia (AML) with t(16;21) (p11;q22). In the present study we have mapped the breakpoints within FUS in 13 MLS with t(12;16) and in one AML with t(16;21). This region of FUS is about 3.9 kb and contains four exons. The breakpoints clustered to two zones (1 and 2). A strong association was found between the two known types of FUS/CHOP transcripts and the genomic localization of the breakpoints. In all cases expressing only type I or both type I and II FUS/CHOP transcript the genomic breakpoints mapped to zone 1. In all cases expressing only the type II transcript the breakpoints occurred in zone 2. The breakpoint in the AML case was in zone 1, suggesting that in-frame fusion transcripts are selected by similar mechanisms in both MLS and AML.
Summary of the cases presented in full in the August issue (Eur J Surg Oncol 1995; 21: 424-426 Patient 1. A 46-year-old female presented with a huge benign lipoma of the left axilla/breast in 1977. Repeated excisions led to three recurrences, the first reported as an intramuscular lipoma, the latter two as well-differentiated liposarcoma. She presented in 1990 with a large recurrence partially fixed to the chest wall. Patient 2. A 58-year-old man with cardiorespiratory impairment due to long-standing valvular disease presented in 1982 with a large poorly differentiated sarcoma over the lower posterior rib cage. He was treated by wide local excision (not including the ribs), and cover with a latissimus dorsi flap to allow completion of a radical perioperative course of radiotherapy. Recurrences around the periphery of the excision occurred in 1984, 1985, 1986, 1987, each treated by relatively wide local excision. He presented again in 1989 with further peripheral recurrences attached to the rib cage, still without evidence of distant metastasis, at which stage a further plan of management was sought.
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.
Central nervous system involvement in liposarcoma is rare, the only symptom reported in the literature being local compression of the spinal cord. An exceptional case with peripheral nerve involvement due to local recurrence, in addition to spinal cord compression and cavernous sinus syndrome due to metastases is reported.
The authors report 3 rare cases of liposarcoma occurring in the perineal or paratesticular regions. They review literature data concerning this particular form of soft tissue sarcoma and emphasize on prognosis which depends mainly on its histopathological type.
The authors report a case of liposarcoma in the sub-mandibular region, and they describe the histological species and the way of spreading. They suggest a surgical, non-demolitional therapy, that they carried radical if it is related to a strict follow-up. Anatomical and functional results, after demolishing surgical therapy, are sometimes unacceptable if the real benefits in terms of survival are considered. No supporting therapy has been used because of the presumed insensibility of the neoplasm to the chemo-radiotherapeutic treatments. At 2 years from surgical treatment no local relapses, on distance metastasis have been related.
Tumor-specific and skin-reactive antigen of a syngeneic liposarcoma (H-10) of Hartley/F guinea pig was solubilized with 3M potassium chloride and purified by precipitation with 2M ammonium sulfate, followed by Sephadex G-200 gel filtration. The antigenic activity of 7 fractions obtained was estimated by the delayed-type skin reaction elicited in syngeneic animals immunized with H-10 cells admixed with BCG. Accurate relative activity of the fractions comparable to the skin reaction elicited by living H-10 cells was calculated by the parallel line assay method in which the dose-response curves of the fractions are compared with that of living cells. About 30 approximately 50 microgram protein of the 3 fractions eluted slowly from the Sephadex column elicited the skin reaction equivalent to that elicited with 1 X 10(6) of living H-10 cells. Tumor-specific skin reactivity per microgram protein of these 3 fractions was roughly 20 approximately 40 times higher than that of lyophilized cells.
A case of spermatic cord liposarcoma is reviewed. Radical orchiectomy is an adequate form of treatment while retroperitoneal lymphadenectomy and adjunctive radiotherapy or chemotherapy appear to be controversial. A close follow-up is mandatory to detect early relapses or distant metastasis.
A 36-year-old woman with a dedifferentiated liposarcoma is described who had suffered from Sweet syndrome in the past. From the literature a relationship between this syndrome and malignancy is known to exist. However, the combination of Sweet syndrome with a sarcoma has not previously been reported.
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.
We report 20 cases of a peculiar fatty tumor that occurred in 16 female and four male patients who were 14-70 years old (median, 36 years). Most lesions were situated in the subcutis, superficial muscular fascia, or skeletal muscle of the limbs and limb girdles (15), trunk (3), and the head and neck (2). They were 1.5-11 cm in size (median, 4 cm) and usually described as yellow (13 of 15) and encapsulated (13 of 15). Microscopically they were well circumscribed and consisted of nests, strands, and sheets of eosinophilic and vacuolated cells, which contained glycogen and fat droplets, resembling brown fat cells, lipoblasts and chondroblasts. In all cases there was a variable background of mature adipose tissue associated with a prominent, partially fibrinous to hyalinized myxoid matrix that contained acid mucopolysaccharides usually resistant to hyaluronidase digestion. Several cases had foci of serous atrophy, perivascular fibrosis, and small thrombi; two were focally calcified. The lesions stained for S100 protein (11 of 12), vimentin (10 of 11), and CD68 antigen with KP1 (9 of 11); focal staining for keratin was also seen (4 of 11), but none stained for epithelial membrane antigen or actin or with HMB45. Follow-up in 12 cases (median, 9.5 years) revealed no local recurrences or metastases. Despite its deep location and atypical cellular features, the lesion's nonaggressive behavior suggests it is benign and neither a myxoid liposarcoma nor a myxoid chondrosarcoma, with which it is most frequently confused. The presence of glycogen in vacuolated fat cells is similar to brown fat, and the presence of sulfated stromal mucins supports focal chondroid differentiation. Although the pathogenesis remains uncertain, a lipoma with hibernomatous features, myxoid change, chondroid metaplasia, and secondary degenerative features is favored over a lipogranulomatous process.
Eighteen cases of perirenal liposarcoma were treated from 1969 to 1991. The average age of patients was 49 years (range 32-66 years). The main clinical features were abdominal mass and compression symptoms. Eight tumors originated from the right side and ten from the left. Sixty seven percent of the patients previously underwent one to three surgical procedures (laparotomy 3 cases, recurrence 9 cases). Tumors were resectable in all patients. The smallest tumor was 14 x 12 x 10 cm in size, the largest was 65 x 45 x 24 cm and 24 kg in weight. Preserved kidney was found to be highly related with local recurrence. Therefore, enbloc resection of the tumor with the kidney should be the operation of choice.
The coincidence of pulmonary and systemic emboli suggests paradoxical embolism. We present the case of a young man in whom a blood clot travelled from the veins in a retroperitoneal liposarcoma via a patent foramen ovalis to the systemic arterial circulation. Young patients with embolic disease but no obvious source of thrombus may have underlying neoplastic disease. Venous thrombosis is a frequent complication of neoplastic disease. Arterial embolism associated with neoplasm is, however, not widely recognised.
The authors report 3 rare cases of liposarcoma occurring in the perineal or paratesticular regions. They review literature data concerning this particular form of soft tissue sarcoma and emphasize on prognosis which depends mainly on its histo-pathologic type.
A case of giant retroperitoneal liposarcoma is described. Treatment was by radical surgery, using the anterior approach, with preservation of the renal unit. The diagnostic procedure is briefly discussed and the literature reviewed.
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.