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PubMed1999

Patterns of recurrence in extremity liposarcoma: implications for staging and follow-up.

BACKGROUND: Liposarcoma is one of the most common histologic types of soft tissue sarcoma and presents a wide spectrum of clinical behavior. The authors examined the correlation among histologic subtypes, outcomes, and patterns of recurrence among patients with extremity liposarcomas. METHODS. A retrospective review of all patients with intermediate and high grade extremity liposarcoma referred to the University of Texas M. D. Anderson Cancer Center from January 1, 1980, to December 31, 1992, was performed. Data on clinical presentation, treatment, patterns of treatment failure, and outcome were evaluated. RESULTS: During the 13-year study period, 122 patients with intermediate or high grade extremity liposarcoma were identified: 102 patients (84%) with myxoid subtype, 18 patients (15%) with pleomorphic subtype, and 2 patients (2%) with mixed histology. There were no differences between the myxoid and pleomorphic subtype groups in tumor size (T1 vs. T2), depth in relation to the muscular fascia, or anatomic site. The median follow-up was 70 months. The 5-year overall survival rate for all intermediate and high grade extremity liposarcoma patients presenting with primary disease (n=85) was 74%; the 5-year local recurrence free survival, distant recurrence free survival, and disease free survival rates were 93%, 78%, and 73%, respectively. Among the 102 patients with myxoid tumors, 33 had distant recurrences; 31 of these were to extrapulmonary soft tissue sites (e.g., the retroperitoneum, chest wall, pleura, pericardium, pelvic sidewall, and soft tissue of the back), and 2 were to the lung only. Among the 18 patients with pleomorphic tumors, 10 had distant recurrences; 3 occurred at extrapulmonary sites, and 7 occurred in the lung only (P < 0.05 for myxoid vs. pleomorphic subtypes). CONCLUSIONS: Myxoid liposarcomas often metastasized to extrapulmonary sites and did so significantly more frequently than pleomorphic tumors. Imaging of the abdomen, retroperitoneum, and extrapleural chest should be performed for accurate staging and posttreatment follow-up of patients with myxoid liposarcoma. Patients presenting with "primary" myxoid liposarcoma of the trunk should be carefully evaluated for an occult primary tumor in an extremity.

Adolescent↗
PubMed2006

The role of genetic testing in soft tissue sarcoma.

Soft tissue tumours represent a heterogeneous group of mesenchymal lesions and their classification continues to evolve as a result of incorporating advances in cytogenetic and molecular techniques. In the last decade traditional diagnostic approaches were supplemented with a significant number of reliable molecular diagnostic tools, detecting tumour type-specific genetic alterations. In addition, the successful application of some of these techniques to formalin-fixed paraffin-embedded tissue made it possible to subject a broader range of clinical material to molecular analysis. Thus, molecular genetics has already become an integral part of the work-up in some tumours, such as paediatric small blue round cell tumours, which demonstrate characteristic translocations. Several lines of evidence suggest that sarcomas can be divided into two major genetic groups: (i) sarcomas with specific genetic alterations and usually simple karyotypes, such as reciprocal chromosomal translocations (e.g. FUS-DDIT3 in myxoid liposarcoma) and specific oncogenic mutations (e.g. KIT mutation in gastrointestinal stromal tumours); and (i) sarcomas with non-specific genetic alterations and complex unbalanced karyotypes. Some of these genetic abnormalities, including chromosomal numerical changes, translocations, gene amplifications or large deletions can be apparent at the cytogenetic level (karyotyping, fluorescence in situ hybridization), while others, such as small deletions, insertions or point mutations, require molecular genetic techniques (polymerase chain reaction and sequence analysis). This review focuses on the applicability of genetic testing in the diagnosis and prognosis of soft tissue sarcomas, and gives a realistic appraisal of the ancillary role of molecular techniques, including its advantages and limitations.

Base Sequence↗
PubMed2001

Amplification and overexpression of PRUNE in human sarcomas and breast carcinomas-a possible mechanism for altering the nm23-H1 activity.

PRUNE, the human homologue of the Drosophila gene, is located in 1q21.3, a region highly amplified in human sarcomas, malignant tumours of mesenchymal origin. Prune protein interacts with the metastasis suppressor nm23-H1, but shows impaired affinity towards the nm23-H1 S120G mutant associated with advanced neuroblastoma. Based on these observations, we previously suggested that prune may act as a negative regulator of nm23-H1 activity. We found amplification of PRUNE in aggressive sarcoma subtypes, such as leiomyosarcomas and malignant fibrous histiocytomas (MFH) as well as in the less malignant liposarcomas. PRUNE amplification was generally accompanied by high mRNA and moderate to high protein levels. The sarcoma samples expressed nm23-H1 mostly at low or moderate levels, whereas mRNA and protein levels were moderate to high in breast carcinomas. For the more aggressive sarcoma subtypes, 9/13 patients with PRUNE amplification developed metastases. A similar situation was observed in all breast carcinomas with amplification of PRUNE. Infection of NIH3T3 cells with a PRUNE recombinant retrovirus increased cell proliferation. Possibly, amplification and overexpression of PRUNE has the same effect in the tumours. We suggest that amplification and overexpression of PRUNE could be a mechanism for inhibition of nm23-H1 activity that affect the development or progression of these tumours.

3T3 Cells↗
PubMedDate not supplied

Sporadic amplification of the c-fms proto-oncogene in human musculoskeletal sarcomas.

Fresh samples of bone and soft tissue sarcoma from 31 patients were analyzed by the Southern technique for amplification or other structural abnormalities of the c-fms oncogene. As a sole finding, amplification of the c-fms (4- and 10-fold) was detected in 2 of 3 cases with a histologic diagnosis of liposarcoma. RFLP analysis disclosed no gene rearrangements associated with the c-fms amplification. The case with the highest amplification of the c-fms gene was disease-free at review. The 2 fms-amplified liposarcomas were aneuploid according to DNA flow cytometry. This is the first study demonstrating amplification of the c-fms proto-oncogene in human liposarcoma. The fact that this structural abnormality of the c-fms gene was only detected in liposarcomas might point to an uncommon but tissue-specific phenomenon.

Blotting, Southern↗
PubMed1986

Surgical treatment of 43 retroperitoneal sarcomas.

Forty-three cases of primary retroperitoneal sarcomas, observed and treated from 1970 to 1983 at this Institute, were analysed. The series consisted of 16 liposarcomas (37%), 10 leiomyosarcomas (23%), 7 rhabdomyosarcomas (16%), 5 fibrosarcomas (12%), 2 malignant histiocytomas (5%), 2 sarcomas NOS (5%) and 1 mesenchymoma (2%). All the patients underwent surgery. In order to evaluate the results of surgery, the patients were divided into three groups, according to the type of operation performed, that is open biopsy, resection and excision. Survival of the patients in the first group never exceeded 24 months. The symptom-free period for the patients treated by incomplete removal of the tumour (second group) lasted 3-24 months. Further surgery, in three cases for this group, did not result in a useful control of the disease. As regards radically treated cases, local recurrence was observed in 3 of the 7 liposarcomas, 2 of the 5 leiomyosarcomas, 1 of the 2 rhabdomyosarcomas and 1 fibrosarcoma. Out of 16 cases of the third group, regularly followed up, only 3 patients (liposarcomas) were alive and free of disease at 5 years from first operation. Overall 5-year survival for this group was 31.9%; disease-free survival was 18.7%. For the whole series of 43 cases, overall 5-year survival was 11.4%. As far as histology was concerned, liposarcomas showed the highest operability rate: out of 16, 7 were resected and 9 radically excised. There is a lack of convincing evidence for the utility of post-operative chemotherapy. On the contrary, post-operative radiotherapy seems to be worthwhile for liposarcomas, especially after non-radical operations.

Adult↗
PubMed1990

Spermatic cord sarcomas: sonographic and CT features.

Five patients with sarcomas of the spermatic cord were imaged with sonography and/or computed tomography (CT). The former modality is most helpful in demonstrating the extratesticular origin of the mass and evaluating its scrotal extension for local staging. CT is necessary for pelvic staging and searching for distant metastasis. The fat in liposarcomas was easily identified by CT, although it was not echogenic on sonography. Sonography should be the primary imaging modality for scrotal or inguinal masses. If a neoplasm is found, CT should be used for staging, prior to definitive surgical treatment.

Aged↗
PubMed2003

Expression of fatty acid synthase as a prognostic indicator in soft tissue sarcomas.

PURPOSE: Fatty acid synthase (FAS) is a key enzyme in the de novo biosynthesis of fatty acids. Carcinoma cells are dependent on endogenous fatty acid synthesis for growth in vitro. In a subset of human cancers, elevated FAS is associated with poor prognosis; however, the expression of FAS and the relationship between FAS and prognosis in soft tissue sarcomas (STSs) have not been studied. The objective of this study is to examine the expression of FAS in STSs and determine its relationship to clinicopathological features and prognosis. EXPERIMENTAL DESIGN: Sixty-four cases of STS were examined. The clinicopathological features and immunohistochemical expression of FAS and Ki-67 antigen were studied. Survival analysis was performed using the log-rank test and the Cox multivariate regression model. RESULTS: FAS expression was observed in 20 of 64 cases (31.3%) of STS. FAS-positive sarcomas were found in 13 of 23 malignant fibrous histiocytomas, 3 of 17 liposarcomas, 3 of 7 malignant peripheral nerve sheath tumors, and 1 extraskeletal mesenchymal chondrosarcoma. No expression of FAS was seen in the synovial sarcomas, leiomyosarcomas, or rhabdomyosarcomas that were examined. Clinicopathologically, FAS-positive tumors were significantly deep-seated (P = 0.02) and large in size (P = 0.03). FAS expression correlated with decreased disease-free survival (P = 0.006) and decreased overall survival (P = 0.003). In a multivariate analysis, expression of FAS was able to predict decreased disease-free survival but did not reach the level of significance for overall survival. CONCLUSIONS: FAS expression is one of the predictive indicators for disease prognosis in STS.

Adolescent↗
PubMed1984

[Four case reports of sarcoma combined with carcinoma].

We report 4 cases of double cancer, combined sarcoma and carcinoma. Case 1 was a 50-year-old woman, had a malignant fibrous histiocytoma in the leg and an advanced gastric carcinoma. The soft tissue sarcoma was resected and the gastric carcinoma was treated with chemotherapy. She died from pulmonary metastasis from the sarcoma. Case 2 was a 72-year-old woman with a liposarcoma in the leg and a thyroid carcinoma. Both tumors were resected, however, she died from pulmonary metastasis from the carcinoma. Case 3 was a 65-year-old man with a liposarcoma in the buttock and lung carcinoma. Both tumors were resected and he is alive and disease-free. Case 4 was a 47-year-old man with spindle cell sarcoma of the tibia and a rectal carcinoma. Both tumors were treated surgically, but he died from pulmonary metastasis from the sarcoma. We suggest that the combination of sarcoma and carcinoma is very rare and that the prognosis in these cases is very poor.

Adenocarcinoma↗
PubMed1995

t(9;22)(q22-31;q11-12) is a consistent marker of extraskeletal myxoid chondrosarcoma: evaluation of three cases.

Three cases of extraskeletal myxoid chondrosarcoma with typical histologic and ultrastructural features were investigated cytogenetically. All three cases showed a reciprocal chromosome translocation characterized as t(9;22)(q22-31)(q11-12), thus confirming the findings in three previously karyotyped cases of extraskeletal myxoid chondrosarcoma in the literature. These data add significantly to the evidence of t(9;22) being diagnostic for extraskeletal myxoid chondrosarcoma. Other previously published cases with a range of complex karyotypes were less well defined morphologically. In cases with limited diagnostic material this karyotype might facilitate distinction from myxoid liposarcoma, which consistently shows t(12;16). Clear cell sarcoma, Ewing's sarcoma/primitive neuroectodermal tumor, and desmoplastic round cell tumor also show involvement of chromosome 22 with formation of a hybrid gene between the Ewing's sarcoma gene on band q12 and a transcription factor gene. Whether rearrangement of the Ewing's sarcoma gene is also present in extraskeletal myxoid chondrosarcoma is not clear at present. Cloning of the (9;22) translocation might provide important clues to the pathogenesis of this type of chondrosarcoma.

Adult↗
PubMed1996

Liposarcoma: outcome and prognostic factors following conservation surgery and radiation therapy.

PURPOSE: Liposarcoma is among the more common types of soft-tissue sarcoma. This retrospective review was performed to determine the outcome and delineate prognostic factors for this disease when treated by conservation surgery and radiation. METHODS AND MATERIALS: Between 1964 and 1992, 112 consecutive patients with liposarcoma were treated with conservation surgery and radiotherapy. Univariate and multivariate statistics were used to evaluate the outcome with local control, metastatic relapse, and survival as end points, and to delineate independently significant prognostic factors. RESULTS: Preoperative radiation to a mean dose of 50 Gy was used in 36 patients and postoperative radiation to a mean dose of 61 Gy was used in 76 patients. At a median follow-up of 9 years (range 2-25), 34 patients (30%) developed disease progression at some site. Local recurrence developed in 14 patients (13%), and the actuarial local relapse rate was 13% at 10 years. Metastases developed in 23 patients (21%) with an actuarial metastatic rate of 23% at 10 years. Five-, 10-, and 15-year survival rates were 79%, 69%, and 61%, respectively. The most important factor influencing outcome--whether local control, metastases, or survival--was tumor histologic subtype. Well-differentiated (15 patients) and myxoid (including round cell) (71 patients) subtypes had a local control rate exceeding 90% at 10 years. No patient with well-differentiated liposarcoma developed metastases. The actuarial metastatic rate for myxoid tumors was 22% at 10 years and was higher for large than small tumors. The pattern of metastases for this variant was atypical with a preponderance of extrapulmonary sites. Pleomorphic liposarcoma (26 patients) was a highly malignant tumor with a high local recurrence rate of 37% and a metastatic rate of 41% at 10 years. Larger pleomorphic tumors had a significantly higher metastatic rate than smaller ones. These biologic factors were reflected in progressively decreasing 10-year survival rates from 87% for well-differentiated lesions, through 76% for myxoid tumors, to 39% for pleomorphic tumors. Although other factors such as positive resection margins and prior local recurrence were also independently significant determinants of outcome, histology was the overriding factor. CONCLUSION: Liposarcoma is a highly pleomorphic disease whose outcome is greatly determined by its histologic subtype: so greatly that results reported without histologic stratification are likely meaningless. Conservation surgery and radiation achieve excellent local results and with relatively little metastatic risk for patients with well-differentiated and myxoid liposarcomas. The same treatment strategy appears appropriate for pleomorphic liposarcoma, but with a much smaller expectation of a satisfactory outcome. The development of effective systemic therapy is an important goal for this variant of liposarcoma.

Adolescent↗
PubMed1989

Chromosomal analysis and the classification of soft tissue sarcomas.

Seventeen soft tissue sarcomas were karyotyped and the chromosomal findings were compared with those in the literature. Some cases were easily classified (immuno)histologically, whereas others defied classification. In four cases with unequivocal histology, a "characteristic" chromosomal abnormality was found. In one case a diagnosis that was doubtful on histologic grounds was supported by the chromosomal findings, whereas in three other cases, the chromosomal pattern led to reconsideration of the histologic diagnosis. The remaining cases either showed chromosomal abnormalities with hitherto undescribed breakpoints or breakpoints described in tumors with different histology. The results extend the knowledge of chromosomal abnormalities in soft tissue sarcomas. Most importantly, the present approach demonstrates that close collaboration between the surgical pathologist and the cytogeneticist may improve the classification of soft tissue sarcomas.

Adolescent↗
PubMedDate not supplied

Giant pedunculated liposarcomas of the esophagus: literature review and case report.

Although liposarcoma is the second most common soft-tissue sarcoma in adults, its incidence within the gastrointestinal tract is distinctly low. Esophageal involvement is exceedingly rare and only four cases have been described so far. A fifth case is presented here along with a thorough review of the literature of polypoid lipomatous tumors of the esophagus. Diagnostic and therapeutical strategies of these tumors are discussed in detail.

Diagnosis, Differential↗
PubMed1981

The role of radiotherapy in the treatment of soft tissue sarcoma: a retrospective study 1958--73.

Survival and local control data are presented for a series of 109 cases of soft tissue sarcoma treated 1958--73. A five-year minimum follow-up period is available. The two-year and five-year survival rates for the complete series are respectively 80 and 68%. In a comparison of survival results for different histological subgroups we found a 94% two-year survival for liposarcomas compared with 80% for fibrosarcomas and 74% for synovial sarcomas; and five-year survivals of 73, 68 and 51% respectively. Sixty-two per cent of the series achieved local control of the disease after initial treatment, and this figure increased to 83% after further treatment. Eighty-six per cent of tumours of the extremities (66/77) were controlled locally without amputation. Of those in the complete series whose disease recurred, either locally or distantly, 50% experienced this recurrence within one year of initial treatment. In only 7% did the recurrence occur more than five years after initial treatment. These results are encouraging and we conclude that they justify an initial treatment policy of conservative surgery combined with radical radiotherapy.

Humans↗
PubMed2006

[Three cases of retroperitoneal soft tissue sarcoma].

Soft tissue sarcomas (STS) are rare tumors that comprise only 1% of all malignancies. The retroperitoneum is an uncommon site of origin for them, accounting for about 10% of all STS. In case 1, a 30-year-old man complaining of chest pain was found to have a retroperitoneal tumor around the left kidney by computed tomography (CT). The tumor was removed along with the left kidney. Histologically, it was fibrosarcoma 3,000 g in weight. In case 2, a 31-year-old woman complaining of abdominal distension was found to have a retroperitoneal tumor by CT. The tumor was removed in the same way. It was undifferentiated sarcoma 2,000 g in weight. In case 3, a 73-year-old woman, who had no complaint was found to have mass in the left lateral region during follow up for cardiac failure. She was referred to our department for treatment of retroperitoneal tumor revealed by ultrasound and CT. The tumor was dedifferentiated liposarcoma 2,420 g in weight. All three patients had local recurrences, and two of them underwent a second surgical procedure.

Adult↗