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目的 探讨原发性腹膜后脂肪肉瘤复发、转移机理及预后。方法 复习原发性腹膜后脂肪肉瘤临床资料及文献，总结伴有亚型转化的原发性腹膜后脂肪肉瘤病人的生存时间及复发率。分析反复复发与远外转移的关系。结果 伴有亚型转化的病人平均复发次数高于非亚型转化病人，但生存时间较长。肿瘤复发可诱导去分化危险。结论 亚型转化使腹膜后脂肪肉瘤的复发次数增加而不影响生存期。肿瘤复发诱导去分化危险，肿瘤可获得转移的能力。     

Atypical and malignant neoplasms showing lipomatous differentiation. A study of 111 cases

One-hundred-eleven cases of histopathologically atypical or malignant lipomatous lesions in the somatic soft tissue and retroperitoneum were studied. These consisted of 48 differentiated fatty neoplasms of the somatic soft tissues (DFT-S), 21 fatty neoplasms of the retroperitoneum (DFT-R), 33 myxoid liposarcomas from various sites and nine pleomorphic liposarcomas. DFT-S were defined as lipomatous lesions composed of mature fat and containing atypical stromal cells or lipoblasts. In the somatic soft tissues, this group included lesions that would be classified using published criteria as "atypical lipoma", "pleomorphic lipoma", "well-differentiated lipoma-like liposarcoma", and "sclerosing liposarcoma". All of the DFT-R met previously published criteria for "well differentiated liposarcoma" or "sclerosing liposarcoma". We found no consistent histologic differences between the DFT-S and DRT-R. No pure "round cell" liposarcomas were encountered although many myxoid liposarcomas had "round cell" areas. Follow-up data were available in 80 cases (72%) with a mean follow-up period of over 7 years. Among the DFT-S there were no uncontrollable recurrences, distant metastases, or tumor-related deaths. The depth of the neoplasm correlated with the tendency for local recurrence; no neoplasms primary in the subcutis recurred; 29% of the tumors recurred when they originated in the deep soft tissues or within the muscle. None of the recurrent tumors demonstrated "dedifferentiation." DFT-R had a recurrence rate of 67% and, although there were no distant metastases, nine patients (43%) died of tumor. Five retroperitoneal tumors dedifferentiated but did not metastasize. In light of this experience, we believe that the term "atypical lipoma" is warranted for the DFT-S and "well differentiated liposarcoma" is an appropriate label for the DFT-R. The overall mean survival for the 52 cases of liposarcoma (excluding DFT-S) was 13.6 years. The mean survival in "well differentiated liposarcoma" (11.25 years) was between that for myxoid liposarcoma (16.25 years) and that for pleomorphic liposarcoma (7 years). Six patients (29%) with myxoid liposarcoma developed local recurrences and 6 patients (29%) developed distant metastases and died. Metastasis was always associated with a round cell (or pleomorphic) component with increased numbers of mitotic figures in either the primary tumor or a local recurrence.     

Operative management of primary retroperitoneal sarcomas: a reappraisal of an institutional experience

OBJECTIVE: To review our recent experience with primary retroperitoneal sarcomas, determine prognostic factors for disease recurrence and patient survival, and compare them to our previous results. BACKGROUND: Medical therapies have shown little efficacy in the management of retroperitoneal sarcomas, making total surgical extirpation the best chance for patient cure. METHODS: The case histories of all patients operated upon for retroperitoneal sarcomas between January 1983 and December 1995 were retrospectively reviewed. RESULTS: Ninety-seven patients underwent attempted surgical resection of a primary retroperitoneal sarcoma. There were 54 (56%) men and 43 (44%) women, with a mean age of 59 years. Seventy-six (78%) patients underwent gross total resection, 13 (14%) had residual disease, and 8 (8%) underwent biopsy only with an actuarial 1-year survival of 88%, 51%, and 47%, respectively (P = 0.001). The actuarial 5- and 10-year survivals for patients who underwent gross total resection were 51% and 36%, respectively. Thirty-three patients (43%) developed locoregional recurrence, and 20 patients (26%) developed distant metastases at a median time of 12 months. The cumulative probability at 5 years was 44% for locoregional recurrence and 29% for distant metastases. On univariate analysis, factors associated with improved survival were complete resection of the tumor (P = 0.001), nonmetastatic disease at presentation (P = 0.01), low-grade tumors (P = 0.02), liposarcomas (P = 0.003), and no disease recurrence (P = 0.0001). Contrary to previous reports, the histologic subtype (P = 0.04) was the only significant factor predicting survival on multivariate analysis. CONCLUSIONS: Compared with our earlier experience, the rates of complete resection and overall survival have improved. Local control continues to be a significant problem in the management of retroperitoneal sarcomas. Because new surgical options for this problem are limited, further outcome improvement requires novel adjuvant therapies.     

Clinical features of multiply recurrent retroperitoneal liposarcoma: A single-center experience

BackgroundTo elucidate the clinicopathological features of multiply recurrent retroperitoneal liposarcoma referred to a tertiary center.MethodsWe retrospectively analyzed the clinical data of 40 patients with pathologically proven primary retroperitoneal liposarcoma treated between January 2015 and June 2019.ResultsThe initial pathology was well-differentiated liposarcoma (WDLS) in 23 patients, dedifferentiated liposarcoma (DDLS) in 15 patients, and myxoid liposarcoma in two patients. Before and after referral to our hospital, the patients underwent 142 surgeries (median number of surgeries: 3) for initial and recurrent tumors. Of these, 35 (87.5%) patients underwent surgeries for recurrent tumors. In 11 (47.8%) of the 23 patients with initial WDLS, pathological progression (PP) to DDLS was observed in recurrent tumors at the median interval of 7.8 years. In eight patients (72.7%), the PP occurred at the first recurrence. In contrast, all 15 patients with DDLS recurred as DDLS. Distant metastases developed in eight patients (34.8%) with WDLS and five patients (29.4%) with DDLS/myxoid subtype. All eight WDLS patients who developed metastasis were alive with disease at the median interval of 3.6 years, whereas four of the five patients with primary diagnosis of DDLS/myxoid subtype died at the median interval of 5.6 months from the development of metastasis. The 5-year overall survival of the patients with initial WDLS and those with DDLS/myxoid subtype were 100% and 67.4% (p = 0.0006), respectively.ConclusionsThe prognosis of the initial-WDLS patients was favorable despite multiple recurrences. In WDLS patients, if distant metastases develop, it is possible to remain alive with disease for years.     

Analysis of the prognostic significance of microscopic margins in 2,084 localized primary adult soft tissue sarcomas

OBJECTIVE: To define the significance of positive microscopic resection margins in a large cohort treated for soft tissue sarcoma. METHODS: The authors analyzed 2,084 patients with localized primary soft tissue sarcoma (all anatomic sites) treated from 1982 to 2000. Clinicopathologic variables studied included tumor site, size, depth, histologic type, grade, and resection margin status. Treatment other than resection was not analyzed. Study endpoints included local and distant recurrence-free and disease-specific survival rates, estimated by the Kaplan-Meier method. Univariate and multivariate analyses were performed using the log-rank test and the Cox proportional hazards model. RESULTS: Median follow-up was 50 months. After primary resection, 1,624 (78%) patients had negative and 460 (22%) had positive resection margins. Having positive margins nearly doubled the risk of local recurrence and increased the risk of distant recurrence and disease-related death. Seventy-two percent of patients with positive margins had no recurrence. Resection margin did not predict local control for retroperitoneal sarcomas or fibrosarcomas. Resection margin remained significantly associated with distant recurrence-free survival and disease-specific survival across all subsets after adjusting for other prognostic variables. The overall 5-year disease-specific survival rates for negative and positive margins were 83% and 75%. CONCLUSIONS: Positive microscopic resection margins significantly decrease the local recurrence-free survival rate for other-than-primary fibrosarcoma and retroperitoneal sarcomas, and independently predict distant recurrence-free survival rates and disease-specific survival rates for all patient subsets. Adjuvant therapy should be considered in the management of soft tissue sarcoma to increase local control. Because 72% of positive margins did not equate with inevitable local recurrence, considerable clinical judgment is required in considering additional treatment. Microscopic resection margins should be considered for inclusion in staging systems and treatment algorithms that address local recurrence.     

Long-term results of aggressive surgical treatment of primary and recurrent retroperitoneal liposarcomas

The aim of this study was to establish the role of surgery in the treatment of retroperitoneal liposarcomas. Data concerning 28 patients submitted to surgery for retroperitoneal liposarcoma in our department over the period from 1972 to 1999 were reviewed retrospectively and analysed. Seventy-four operations were performed; in 54% of the operations it was necessary to resect contiguous organs (kidney 60%, colon 50%, adrenal gland 35%). In 89%, grossly curative resection was achieved at the first operation; 20 patients had at least one local recurrence after first operation (median time interval: 22 months). The mean follow-up was 80 months; median survival time was 51 months and 5-year actuarial survival time 51%. Patients with low-grade liposarcoma showed a statistically significant improvement (P < 0.001) in median survival (153 months) versus those with medium- (37 months) and high-grade sarcomas (8 months). At present surgery is still the treatment of choice in the treatment of primary and recurrent liposarcoma; in the case of low-grade liposarcomas especially, an aggressive surgical approach can result in long-term survival.     

Locoregional Disease Patterns in Well-Differentiated and Dedifferentiated Retroperitoneal Liposarcoma: Implications for the Extent of Resection?

Background

Well-differentiated (WD)/dedifferentiated (DD) liposarcoma is the most common soft tissue sarcoma of the retroperitoneum. The frequency of distant metastasis is low and the major burden of disease is locoregional. We sought to define the patterns of locoregional disease to help guide surgical decision making.

Methods

Data were collected from 247 patients with de novo or recurrent tumors treated at our institution from 1993 to early 2012. The number and location of tumors at both initial presentation and subsequent locoregional recurrence were determined by combined analysis of operative dictations and radiologic imaging.

Results

Thirty-four percent of patients had multifocal locoregional disease (two or more tumors) at initial presentation to our institution, including 9 % who had tumors at synchronous remote retroperitoneal sites. The impact of multifocal disease on overall survival was dependent on histologic subtype (WD vs. DD) and disease presentation (de novo vs. recurrence) at the time of resection. Among patients with initial unifocal disease, 57 % progressed to multifocal locoregional disease with subsequent recurrence, including 11 % with new tumors outside of the original resection field. No clinicopathologic or treatment-related variable, including the type or extent of resection, was predictive of either multifocal or ‘outside field’ progression.

Conclusions

Multifocal disease is common in patients with WD/DD retroperitoneal liposarcoma, and tumors can also develop at remote, locoregional sites. Surgical resection remains the primary method of locoregional control in this disease; however, the aggressiveness of resection should be individualized, with consideration of both tumor and patient-related factors.     

原发性腹膜后脂肪肉瘤复发和预后的影响因素

目的:探讨原发性腹膜后脂肪肉瘤复发和预后的影响因素。方法:回顾性分析2011年6月至2020年1月郑州大学第一附属医院接受手术治疗的89例原发性腹膜后脂肪肉瘤患者的临床资料,男42例,女47例。中位年龄53(26~78)岁。我院首次手术治疗65例,外院术后复发再次手术治疗24例。初诊临床表现：体检发现腹膜后肿物41例,...     

Liposarcomas of the mediastinum and thorax: a clinicopathologic and molecular cytogenetic study of 24 cases, emphasizing unusual and diverse histologic features

Liposarcoma rarely occurs in the mediastinum, and most reports predate the current genetically based classification system. We report the clinicopathologic and molecular genetic features of a series of thoracic liposarcomas identified over a 60-year period. Twenty-four confirmed cases were reclassified using the most recent World Health Organization classification. Fluorescent in situ hybridization for CPM amplification and/or DDIT3 rearrangement was performed on selected cases. The 24 cases occurred in 13 men and 11 women (mean age, 53 y; range, 15 to 73 y) and arose in all mediastinal compartments. All subtypes were encountered with 8 well-differentiated liposarcomas, 6 dedifferentiated liposarcomas (3 of 6 confirmed CPM+), 7 pleomorphic liposarcomas (2 of 7 confirmed CPM-, 1 of 7 confirmed DDIT3-), 2 myxoid liposarcomas, and 1 unclassifiable liposarcoma (CPM- and DDIT3-). Unusual histologic features included myxoid well-differentiated liposarcoma mimicking myxoid liposarcoma (2 cases), lipoleiomyosarcoma (1 case), dedifferentiated liposarcoma with "meningothelial"-like dedifferentiation, differentiated myxoid liposarcoma mimicking well-differentiated liposarcoma (CPM-), and pleomorphic liposarcoma with epithelioid and myxoid change. Follow-up information was available for 19 patients (mean, 55 mo; range, 8 to 252 mo). Outcome was strongly associated with histologic subtype, with death from disease occurring in 1 of 6 well-differentiated, 1 of 4 dedifferentiated, 5 of 7 pleomorphic, and 2 of 2 myxoid liposarcomas. The mediastinum shows a preponderance of uncommon subtypes and unusual morphologic variants. Correct classification has important implications, with most patients with well-differentiated/dedifferentiated liposarcoma having a protracted clinical course, in contrast to the more rapid disease progression seen in patients with myxoid and pleomorphic liposarcoma.     

Liposarcoma of the lower extremity

Twenty patients with primary liposarcoma of the lower extremity were treated over a 25-year period. The adequacy of the initial surgical procedure and histological grade of malignancy both influenced survival rates. In addition local recurrence was noted only in patients who had undergone "inadequate" initial excision. Inguinal lymph nodes were uninvolved by tumor in all cases. In general, patients with liposarcomas of myxoid and/or round cell type survived for long periods of time. However, even patients with myxoid lesions occasionally exhibited evidence of early blood-borne metastases. This study suggests an important relationship between an inadequate primary resection, local recurrence, and eventual retroperitoneal spread. In the majority of cases, this may have resulted from failure to control the primary distal extremity tumor, with subsequent contiguous spread of metastases into the ipsilateral retroperitoneal space. If feasible, radical soft part resection should be performed as the primary surgical therapy of these neoplasms. Tumors contiguous to the knee or ankle joint should be treated by primary amputation. Failure to control local disease may result in blood-borne dissemination or local spread along musculoaponeurotic planes to involve proximal groin or retroperitoneal space.     

Management of high-grade retroperitoneal liposarcomas: personal experience

Sarcomas are uncommon malignant tumors arising from mesenchymal tissue, accounting for approximately 10% of soft-tissue sarcomas and less than 1% of all malignant neoplasms. The most frequent subtypes of retroperitoneal sarcomas are liposarcoma. Total surgical extirpation appears the only chance for patient cure since medical therapies have shown little efficacy. We have analyzed our series of 32 retroperitoneal liposarcoma that undergone surgical resection and with at least 5 years of follow-up. We have included 21 patients who underwent a complete resection (R0) and 6 patients who underwent an incomplete resection (R1). However, the operation's goal was a complete tumor resection with tumor-free resection margins. The 5-year survival was 85.7% when we performed a R0 resection, while it was 33.3% when we performed a R1 resection with a significant statistical difference (p = 0.02). Surgery is the gold standard for treatment of liposarcoma. Despite advances in diagnostic modalities, surgical techniques and the adoption of more aggressive procedure, such disease still has a propensity for local recurrence, even after an apparent complete resection. Therefore, locoregional recurrence remains the main cause of death in patients with a retroperitoneal liposarcoma. Moreover when the tumor recurs locally, the treatment of choice is to remove the recurrence. Our experience shows that a complete resection of primary lesions and further resection of recurrences early detected with CT examination could improve the overall survival of patients with retroperitoneal liposarcoma. Moreover in our series, the patients who undergo R1 resection had a worse prognosis.     

Subtype specific prognostic nomogram for patients with primary liposarcoma of the retroperitoneum, extremity, or trunk

OBJECTIVE: To determine the prognostic significance of histologic subtype in a large series of patients with primary liposarcoma (LS) and to construct a LS-specific postoperative nomogram for disease-specific survival (DSS). SUMMARY BACKGROUND DATA: Nomograms, used to define and predict outcome following operative intervention, may contain variables not conventionally used in standard staging systems. A 12-year DSS postoperative nomogram for all sarcomas has already been established. METHODS: From a single-institution prospective sarcoma database, patients with primary extremity, truncal, or retroperitoneal LS treated between 1982 and 2005 were identified. Histology was reviewed by a sarcoma pathologist and divided into 5 subtypes. A nomogram predictive of 5- and 12-year DSS was developed. RESULTS: Of 801 patients with primary LS resected with curative intent, 369 (46%) presented with well-differentiated, 143 (18%) dedifferentiated, 144 (18%) myxoid, 81 (10%) round cell, and 64 (8%) pleomorphic histology. The median tumor burden was 15 cm (range, 1-139 cm). At last follow-up, 560 patients were alive with a median follow-up time of 45 months (range, 1-264 months) and 51 months for surviving patients. The 5- and 12-year DSS rates were 83% (95% confidence interval [CI], 80%-86%) and 72% (95% CI, 67%-77%), respectively. The nomogram was drawn on the basis of a Cox regression model. The independent predictors of DSS were age, presentation status, histologic variant, primary site, tumor burden, and gross margin status. The nomogram was internally validated using bootstrapping and shown to have excellent calibration. The concordance index was 0.827 compared with 0.776 for the general sarcoma postoperative nomogram for 12-year DSS. CONCLUSION: The LS-specific nomogram based on histologic subtype provides more accurate survival predictions for patients with primary LS than the previously established generic sarcoma nomogram. DSS nomograms aid in more accurate counseling of patients, identification of patients appropriate for adjuvant therapy, and stratification of patients for clinical trials and molecular analysis.     

腹膜后巨大脂肪肉瘤一例报告并文献复习

目的探讨腹膜后巨大脂肪肉瘤术后腹腔复发患者多学科诊疗模式（MDT）。 方法回顾性分析腹膜后巨大脂肪肉瘤术后腹腔复发患者1例,男,45岁,复发肿瘤大小约10 cm×9 cm的临床病例资料及其两次诊治过程及MDT讨论要点分析,复习总结国内外相关文献。 结果患者行开放腹腔多发肿物切除+胆囊切除+大网膜切除术成功切除肿瘤,术后病理为去分化型脂肪内瘤。经治疗后恢复良好出院。一个半月后复发,继续靶向药物（阿帕替尼500 mg qd）治疗。 结论腹膜后脂肪肉瘤早期诊断困难,手术难度大,MDT及结合影像学检查及临床病理学特征可作出正确诊断,充分评估早期手术治疗可获得一定疗效,但去分化型脂肪内瘤总体预后不良。     

Midterm results after treatment of liposarcoma in the extremities

PURPOSE: In this retrospective analysis, survival time, local recurrence and rate of metastasis were appraised in patients with primary liposarcoma of the extremities depending upon the applied primary and adjuvant therapy procedures. Furthermore, we compared the representativity of histological results of the biopsy with the final histology of the resected tumour. MATERIAL AND METHOD: Between 1990 and 1998, 27 patients were surgically treated who suffered from a primary liposarcoma of the extremities. At the mean follow-up time of 62.4 months (39-141 months) postoperative survival rate, rate of metastasis and local recurrence were determined. The results were analysed with regard to intraoperative resection distance, tumour size and localisation as well as histological classification of previous biopsy and finally resected tumour. The influence of radiotherapy on the results was also determined. RESULTS: In 5 patients (18.5%) the amputation and in 22 cases (81.5%) the limb sparing resection of the liposarcoma was performed. 6 patients underwent a marginal resection and one patient an intralesional resection. At follow-up we observed a local recurrence rate of 22.2% (n = 6) and a survival rate of 74.1%. 18.5% of the patients had metastases. After adjuvant radiotherapy three patients (50%) showed local recurrence after marginal resection. In only 8 cases (29.6%) the results of primary biopsy and final tumour classification (entity, subtype and grading) were identical. CONCLUSION: The development of metastases (often pulmonary) and local recurrences even after long tumour-free interval makes adequate follow-up investigations mandatory in liposarcoma patients. With regard to the problems in assessing liposarcoma biopsies, MRI-orientated biopsies from several tumour areas via one biopsy approach are recommended.     

Retroperitoneal sarcomas: grade and survival

BACKGROUND: The survival of patients with retroperitoneal sarcomas depends on the feasibility of complete resection and the grade of the tumor. HYPOTHESIS: A high rate of complete resection, wide rather than local excision when feasible, and a policy of prompt reoperation for local recurrence all improve survival. METHODS: A review of 130 consecutive patients with retroperitoneal soft tissue sarcomas (1977-2001). RESULTS: The complete resectability rate was 95%, being 99% (78/79) for the primary tumors and 90% (46/51) for tumors referred with local recurrence. Local recurrence after complete resection occurred in 41% (32/79) of those with primary tumors and in 61% (31/51) of those referred with local recurrence (P =.06). The local recurrence rate was 63% after local excision and 39% after wide resection (P =.02). Of 83 patients with relapse, 37 (45%) were rendered surgically disease free. The estimated 5-year (10-year in parentheses) survival from the first surgery at our center was 65% (56%) for patients with primary tumors and 53% (34%) for patients referred with local recurrence (P =.23). For the primary tumors, the 5- and 10-year survival rates were 70% and 60%, respectively, after wide resection and 47% and 39%, respectively, after local excision (P =.04). For the primary tumors, the 5-year survival was 92%, 54%, and 48% for grades I, II, and III, respectively (P =.02). For those referred with local recurrence, the figures were 76%, 45%, and 19% for grades I, II, and III, respectively (P<.001). CONCLUSIONS: A high resectability rate (95%) is possible in retroperitoneal sarcomas. The survival estimates are similar to those following resection of extremity soft tissue sarcomas given an effective reoperation policy for local recurrences. Wide resection lowers the local recurrence and improves survival significantly. Survival varies significantly according to the grade of the tumor.     

Surgical management of primary and recurrent retroperitoneal liposarcoma

BACKGROUND: Surgery plays a dominant role in the initial and subsequent treatment of retroperitoneal liposarcoma (RPLS). This study was a review of outcomes of patients treated at the Royal Marsden Hospital. METHODS: Records of all patients who had surgery for RPLS since 1990 were reviewed, with particular attention to local recurrence and disease-specific survival. Patients with primary RPLS and those with recurrent RPLS, who had palliative surgery after a variable number of operations performed elsewhere, were considered separately. RESULTS: Seventy-two patients had surgery for primary RPLS, over half of whom underwent resection of a contiguous organ to achieve clearance. Follow-up of at least 12 months was available for 58 patients. Thirty-four patients had no evidence of recurrence after median follow-up of 26 (range 12-151) months. Low-grade tumour and macroscopic clearance of tumour were significantly associated with a reduced risk of local recurrence and improved survival. Forty-seven patients had palliative surgery for recurrent RPLS. Median survival from time of last operation to death was 27 (range 0-79) months. Follow-up was to a median of 68 (range 14-261) months. CONCLUSION: Patients with low-grade RPLS that has been completely resected at the initial operation have the most favourable prognosis. Palliative resection is worthwhile to treat troublesome symptoms of recurrence.     

A case of retroperitoneal dedifferentiated liposarcoma initially diagnosed as malignant fibrous histiocytoma: a case report

We report a case of retroperitoneal tumor which turned out to be liposarcoma by the histological evaluation of its recurrent tumor, although the initial tumor was diagnosed as malignant fibrous histiocytoma (MFH). A retroperitoneal tumor in a 62-year-old man was removed and pathologically diagnosed as MFH. Five years after the initial surgery, computed tomography (CT) demonstrated a recurrent tumor near the spleen. The tumor was resected together with the spleen, tail of pancreas, and connective tissue due to adhesion and diagnosed as well-differentiated liposarcoma with sclerosing component. Generally dedifferentiated liposarcoma is difficult to distinguish from MFH and the presence of a well-differentiated liposarcoma component in the adjacent adipose tissue leads to the diagnosis of dedifferentiated liposarcoma. The clinical course of the present case indicated that the initial tumor was dedifferentiated liposarcoma and the recurrent tumor developed from the surrounding well-differentiated liposarcoma.     

Prognostic factors for first recurrence in patients with retroperitoneal sarcoma

BACKGROUND: Retroperitoneal sarcomas are characterized by a high local recurrence rate despite optimal surgical treatment. The definition of prognostic factors for recurrence could help offer high-risk patients a closer follow-up and a multidisciplinary therapeutic approach. PATIENTS AND METHODS: A cohort of 40 patients treated for a primary retroperitoneal sarcoma was retrospectively analyzed. Median follow-up was 24 months. Patient (sex and age), tumor (maximal size, histologic type, tumor localization, and histologic grade), and treatment (complete vs. incomplete surgery) characteristics were included in univariate and multivariate prognostic factor analyses. RESULTS: After a median follow-up of 24 months (range 3-121), the overall recurrence rate was 65%. Median time between initial surgery and recurrence was 15 months (range 11.5-29.5). In univariate analysis, surgical positive margins (P = 0.011), bilateral tumors (P = 0.0034), nonliposarcoma histologic subtypes (P = 0.043), and a high histologic grade (P = 0.0072) were associated with an increased recurrence rate. All these factors except the histologic subtypes retained an independent prognostic value in the multivariate analysis. Death was strongly related to recurrence (P = 0.0033). CONCLUSION: The optimal treatment of patients with primary retroperitoneal sarcoma should be based on radical surgery, with en bloc organ resection if necessary, to minimize the risk of positive margins. In high-risk patients, close follow-up is mandatory to offer optimal subsequent surgical procedures. The impact of a multidisciplinary therapeutic approach remains to be proved.     

The impact of chemotherapy on the survival of patients with high-grade primary extremity liposarcoma

OBJECTIVE: To determine if chemotherapy offers a survival benefit to patients with large, high-grade, primary extremity liposarcoma. SUMMARY BACKGROUND DATA: The impact of chemotherapy on the survival of patients with primary extremity soft tissue sarcoma is controversial and its effect on individual histologic subtypes is not defined. PATIENT AND METHODS: Two prospectively collected sarcoma databases were used to identify all patients with >5 cm, high-grade, primary extremity liposarcoma that underwent surgical treatment of cure from 1975 to 2003 (n = 245). Clinical, pathologic and treatment variables were analyzed for disease-specific survival (DSS), distant recurrence-free survival (DRFS) and local recurrence-free survival (LRFS). RESULTS: Sixty-three (26%) patients were treated with ifosfamide based chemotherapy (IF), 83 (34%) with doxorubicin based chemotherapy (DOX) and 99 (40%) received no chemotherapy (NoC). To assess the impact of DOX, a contemporary cohort analysis of patients treated from 1975 to 1990 was performed. The 5 year DSS of the DOX treated patients was 64% (53%-74%) compared with 56% (51%-79%) for the NoC patients (log-rank P value = 0.28). To assess the impact of IF, a contemporary cohort analysis of patients treated from 1990 to 2003 was performed. The 5 year DSS of the IF treated patients was 92% (84%-100%) compared with 65% (51%-79%) for the NoC patients (log-rank P value = 0.0003). Independent prognostic factors for improved DSS were smaller size (HR = 0.7, P = 0.01), myxoid/round cell histologic subtype (HR = 0.3, P = 0.03) and treatment with IF (HR = 0.3, P = 0.01). The five-year DRFS of the IF treated patients was 81% (70%-92%) compared with 63% (50%-76%) for the NoC patients (log-rank P value = 0.02). The 5 year LRFS of the IF treated patients was 86% (76%-96%) compared with 87% (77%-97%) for the NoC patients (log-rank P value = 0.99). CONCLUSIONS: In patients with large, high-grade, primary extremity liposarcoma; DOX is not associated with improved DSS and IF is associated with an improved DSS. Treatment with IF should be considered in patients with high-risk primary extremity liposarcoma.