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Background: Dedifferentiated liposarcoma (DDL) juxtapose components of well-differentiated liposarcoma (WDL) and nonlipogenic sarcoma. Malignant fibrous histiocytoma (MFH) is no longer considered a homogeneous entity, but rather as the common morphologic appearance of various subtypes of sarcomas. The objectives of the current retrospective study were: 1( to analyze the relation between DDLs and tumors previously diagnosed as MFHs; 2) to trace the evolution of liposarcomas, and 3) to assess the consequences of dedifferentiation., Methods: Between 1974 and 2001, 86 patients with retroperitoneal liposarcoma (61 patients) or MFH (25 patients) underwent surgery at Institut Bergonie in Bordeaux, France. Histologic review was performed and tumors reclassified as WDL or DDL were retained for further clinicohistologic study. Subsequently, initial presentation and all recurrences were analyzed., Results: The 61 liposarcomas consisted of 21 WDLs and 35 DDLs; 17 MFHs were reclassified as DDL. In all, approximately half of the retroperitoneal liposarcomas and MFHs were found to be DDLs. The DDLs presented with a smaller size (20 cm vs.30 cm; P = .05) but a lower rate of complete resection (72% vs.90%; P = .015) and remission (72% vs. 100%; P = .0015). Dedifferentiated recurrence was evidenced in 7 WDLs. Ten DDLs presented with metastatic evolution. DDLs demonstrated a tendency toward lower rates of 5-year overall survival (55% vs. 82%; P = .075)., Conclusions: Most occurrences of retroperitoneal liposarcomas and MFHs are in fact DDLs and dedifferentiated recurrence of WDLs is frequent. Retroperitoneal DDLs present a lower rate of complete remission than WDLs and a risk of metastatic recurrence. Therefore, extensive histologic analysis of WDLs is required to identify an undifferentiated component and avoid misdiagnosis of DDL., (Copyright 2006 American Cancer Society.)

Background: Surgery is the main prognostic factor in retroperitoneal sarcoma. However, despite progress, surgery alone is rarely curative, and analysis of the causes of failures and of other prognostic factors are warranted to ascertain treatment orientations., Methods: Data of patients treated from 1.80 to 12.94 for primary retroperitoneal sarcoma were extracted from the French Federation of Cancer Centers Sarcoma Group registry. Univariate and multivariate analysis were performed for initial local control and for local and general outcome. One hundred sixty-five patients (median age, 54 years; range, 16--82 years) were identified. Median tumor size was 15 cm (range, 2--70 cm); 31% of tumors presented with neurovascular or bone involvement. Liposarcoma, leiomyosarcoma, and malignant fibrous histiocytoma represented 66% of the tumors. Eighty-four percent of the tumors were of high or intermediate grade. Twenty patients had initial metastases. Multimodality treatment included surgery (150 patients), radiotherapy (92 patients), and chemotherapy (77 patients). Complete excision was achieved in 94 of 145 nonmetastatic patients. Median follow-up was 47 months (range, 3--160 months)., Results: Actuarial overall 5-year survival rate (median) was 46% (51 months). The main prognostic factors for survival were initial metastases and surgery, which represented the major treatment-linked factor. High-grade of tumors affected local recurrence, metastatic recurrence, and survival. Adjuvant radiotherapy was significantly associated with reduced local recurrence. Various evolutive patterns were observed with histologic subtypes., Conclusions: Aggressive surgery remains mandatory in retroperitoneal sarcoma, but a randomized trial is needed to evaluate the place of radiotherapy for local control., (Copyright 2001 American Cancer Society.)

Background: Histologic grade is said to be the most important prognostic factor in adult soft tissue sarcomas (STS), but most grading systems have been tested in the overall sarcoma group and the predictive value of histologic grade needs to be assessed specifically for each of the histologic categories., Methods: From 1980 to 1994, 1240 nonmetastatic patients were entered in the French STS database. The following parameters were studied: patient's age and gender, previous history, tumor location, size and depth, neurovascular or bone involvement (NBI), histologic type and subtype, and grade (the French Federation of Cancer Centers [FNCLCC] system). Median follow-up for the survivors was 88 months; only 5% of patients were lost to follow-up. The authors performed univariate and multivariate analyses for metastasis-free survival for the overall sarcoma group and for every main histologic type., Results: In order of importance, parameters were respectively retained as independent predictors of metastasis as follows: grade, tumor size, NBI and tumor depth for the overall group, grade and NBI for malignant fibrous histiocytomas (n = 349), tumor size, histologic subtype and grade for liposarcomas (n = 188), NBI, grade and tumor size for leiomyosarcomas (n = 148), grade and NBI for synovial sarcomas (n = 125), grade for unclassified sarcomas (n = 140), and sarcomas of other types (n = 158). No parameter was significant for malignant schwannomas (n = 72) or for rhabdomyosarcomas (n = 60)., Conclusion: In this study, histologic grade appeared as an independent predictor of metastasis development in the main histologic types of adult STS, with the exception of malignant schwannomas and rhabdomyosarcomas., (Copyright 2001 American Cancer Society.)

Background: The authors determined the flow cytometric (FCM) DNA characteristics of 53 benign tumors and 132 malignant (71 primary, 61 recurrent) soft tissue sarcomas to investigate their heterogeneity and to evaluate the prognostic values of DNA ploidy status and S-phase fraction (SPF)., Methods: One to 11 frozen samples were collected from 185 tumors at 10 participating centers of the French Federation of Cancer Centers (FNCLCC). All FCM analyses were performed in the same laboratory. Histologic diagnoses were collegially reviewed, and grade was assessed by the pathologists of the FNCLCC Sarcoma Group. Relationships between FCM, clinical, and pathologic data were investigated using univariate and multivariate analyses for risks of mortality and metastasis., Results: All except 2 of the 53 benign lesions were DNA diploid. One schwannoma and one desmoid tumor exhibited small abnormal DNA peaks. SPF was low in all benign lesions. One-third of the sarcomas were DNA diploid, whereas two-thirds were DNA aneuploid. Relationships were found between aneuploidy and mitotic count, grade, and histologic subtype of malignant fibrous histiocytoma. DNA ploidy status did not influence the clinical outcome. Multiple sampling performed in 32 sarcomas showed both diploid and aneuploid samples in 6 tumors. Heterogeneity was related to tumor size. SPF evaluated in 85 sarcomas was related to DNA ploidy status, mitotic count, and grade. SPF > or = 4% was significantly associated with a low overall survival rate. In a multivariate analysis performed for the whole group of 132 patients, the single factors with independent prognostic value for patient mortality were disease free status after the treatment course (P < 0.0001) and SPF (P = 0.03). In the subgroup of patients who were initially free of metastases and free of tumor after the treatment course, SPF remained the only factor that significantly influenced the overall survival rate (P = 0.021)., Conclusions: Despite its high failure rate, SPF is an independent factor in the prognosis of soft tissue sarcoma and should be considered when patients with a high mortality risk need to be selected for adjuvant chemotherapy.

Background: The purpose of this study was to determine the independent prognostic variables in a well documented subset of 216 patients with localized primary malignant fibrous histiocytomas (MFH)., Methods: Between the years 1980 and 1989, 216 patients with localized, primary (International Union Against Cancer [UICC]/American Joint Committee on Cancer [AJCC] Stage I-IVA) MFH were evaluated and treated in 10 participating centers of the sarcoma group of the French Federation of Cancer Centers (FNCLCC). Clinicopathologic factors were collected retrospectively and entered into a cooperative database. Tissue slides of all cases were jointly reviewed microscopically by the pathology subcommittee. Surgical treatment was performed on all but 6 (3%) patients. One hundred ninety-five patients (90%) were free of gross disease, with complete local control at the end of the initial treatment. The adjuvant treatment was radiotherapy in 78 patients (36%), chemotherapy in 19 patients (9%), and both in 61 patients (28%)., Results: The median follow-up was 3.5 years (range, 45 days to 12 years). Five-year actuarial rates of disease specific (DSS), metastasis free (MFS), and local recurrence free (LRFS) survival were 70%, 63.3%, and 62.7%, respectively. Multivariate analyses showed that the adverse prognostic factors independently associated with decreased disease specific survival were UICC/AJC Stage III + IVA (P < 0.00001; relative risk [RR], 3.27; 95% confidence interval [CI], 1.6-6.58), residual macroscopic disease following primary local therapy (P = 0.00024; RR, 3.99, CI, 2.04-7.82), deep tumor location (P = 0.0045; RR, 3.37; CI, 1.21-9.38), non-myxoid histology (P = 0.0056; RR, 9.28; CI, 1.03-83.41), and age older than 50 years (P = 0.037; RR, 2.19; CI, 1.04-4.61). Two factors were significantly related to MFS in the patients with the poorest prognosis: histopathologic Grade 3 (P < 0.0001, RR, 3.46; CI, 2.02-5.91) and tumor size greater than 8 cm in largest dimension (P = 0.0012; RR, 2.78; CI, 1.36-3.66). With regard to LRFS, patients who did not undergo radiotherapy had reduced local control (P = 0.0043; RR, 2.36; CI, 1.46-3.83)., Conclusions: Resection of all macroscopic disease was independently associated with improved disease specific survival and adjuvant radiotherapy significantly decreased the local relapse risk. Histopathologic grade was the most important prognostic factor for DSS and MFS.

Tumor grade is currently the most important factor in the staging of patients with soft tissue sarcomas. In previous studies, a histopathologic grading system was described and its reproducibility was tested. The current study reports the value of this grading system in spindle cell sarcomas, which represent about one half of all adult soft tissue sarcomas, the precise identification of which is often difficult. One hundred twenty-five such tumors were studied retrospectively. Malignant fibrous histiocytoma was the most frequent histologic type, followed by leiomyosarcoma, neurosarcoma, and fibrosarcoma. Tumor grade was correlated with the advent of metastases and survival, and was the main prognostic factor according to multifactorial analysis introducing clinical prognostic factors.