Myxoid\Round Cell Liposarcoma (MRCLS) Revisited: An Analysis of 418 Primarily Managed Cases

Moreau, Louis-Charles; Turcotte, Robert; Ferguson, Peter C.; Wunder, Jay S.; Clarkson, Paul W.; Masri, B A; Isler, Marc; Dion, Norbert; Werier, Joel; Ghert, Michelle; Deheshi, Benjamin

doi:10.1245/s10434-011-2127-z

Cited by 133 publications

(127 citation statements)

References 30 publications

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“…Our 7.4% local recurrence rate is similar to that reported by a recently published large MLPS cohort 11 and is better than the rates reported in other published series, [8][9][10]12,13 perhaps because of more frequent positive margins reported by Haniball et al (34%), Kilpatrick et al (49%), and ten Heuvel et al (24%) compared with our current series (17%); less frequent use of radiotherapy; and/or the inclusion of patients with primary retroperitoneal myxoid lesions. Our preferred therapeutic approach since the 1980 s has been radiotherapy (50 grays) followed by surgery, which facilitates excellent local control with smaller radiation fields 17 than postoperative radiotherapy, which we reserve for patients who are referred to us after undergoing primary tumor resection elsewhere.…”

Section: Discussionsupporting

confidence: 90%

“…Recently, the role of radiotherapy in local disease control has been demonstrated, 17 and the rates of radiotherapy receipt have increased, as we and others have demonstrated. 9,11 The role of chemotherapy for patients with localized MLPS is uncertain, and its rate of administration at some treatment centers is as low as 6% 11 compared with 40% at UTM-DACC, where any patient with localized disease who is considered high-risk (tumor size >10 cm, RCL histology, and positive resection margins) receives chemotherapy.…”

Section: Discussionmentioning

confidence: 99%

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Localized and metastatic myxoid/round cell liposarcoma

Hoffman

Ghadimi

Demicco

et al. 2013

Cancer

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BACKGROUND. Myxoid liposarcoma (MLPS), a disease especially of young adults with potential for local recurrence and metastasis, currently lacks solid prognostic factors and therapeutic targets. The authors of this report evaluated the natural history and outcome of patients with MLPS and commonly deregulated protein biomarkers. METHODS. Medical records were retrospectively reviewed for patients who presented to the authors' institution with localized (n ¼ 207) or metastatic (n ¼ 61) MLPS (1990MLPS ( to 2010. A tissue microarray of MLPS patient specimens (n ¼ 169) was constructed for immunohistochemical analysis of molecular markers. RESULTS. The 5-year and 10-year disease-specific survival rates among patients with localized disease were 93% and 87%, respectively; male gender, age >45 years, and recurrent tumor predicted poor outcome. The local recurrence rate was 7.4%, and the risk of local recurrence was associated with recurrent tumors and nonextremity disease location. Male gender was the main risk factor for metastatic disease, which occurred in 13% of patients. Forty percent of patients who had localized disease received chemotherapy, mostly in the neoadjuvant setting. Immunohistochemical analysis revealed significantly higher expression of C-X-C chemokine receptor type 4 (CXCR4) and platelet-derived growth factor beta (PDGFR-b) in metastatic lesions versus localized lesions. Tumors with a round cell phenotype expressed increased levels of CXCR4, p53, adipophilin, PDGFR-a, PDGFR-b, and vascular endothelial growth factor relative to myxoid phenotype. Only the receptor tyrosine kinase encoded by the AXL gene (AXL) was identified as a prognosticator of disease-specific survival in univariate analysis. CONCLUSIONS. In this study, the authors identified clinical and molecular outcome prognosticators for patients with MLPS as well as several potential therapeutic targets.

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Section: Discussionsupporting

confidence: 90%

Section: Discussionmentioning

confidence: 99%

Localized and metastatic myxoid/round cell liposarcoma

Hoffman

Ghadimi

Demicco

et al. 2013

Cancer

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“…However, a local recurrence rate similar to R0 surgery can be obtained when the excision procedure is planned by a specialized surgeon who has decided before the surgery to accept narrow margins in high-risk areas or areas in close proximity to vascular, nerve or bone structures [12,[23][24][25]. The local recurrence rate was 23% in the current study when the surgery was planned accordingly, which is comparable to published rates [5,6,[17][18][19][20][21].…”

Section: Discussionsupporting

confidence: 73%

“…There are only seven published studies on myxoid/round-cell liposarcoma [5,6,[17][18][19][20][21]. The small number of patients also limits the interpretation of any statistical tests for prognostic factors.…”

Section: Discussionmentioning

confidence: 99%

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Prognostic factors for the recurrence of myxoid liposarcoma: 20 cases with up to 8 years follow-up

Lemeur

Mattei

Souteyrand

et al. 2015

Orthopaedics & Traumatology: Surgery & Research

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Dose Reduction of Preoperative Radiotherapy in Myxoid Liposarcoma

et al. 2021

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IMPORTANCE Currently, preoperative radiotherapy for all soft-tissue sarcomas is identical at a 50-Gy dose level, which can be associated with morbidity, particularly wound complications. The observed clinical radiosensitivity of the myxoid liposarcoma subtype might offer the possibility to reduce morbidity. OBJECTIVE To assess whether a dose reduction of preoperative radiotherapy for myxoid liposarcoma would result in comparable oncological outcome with less morbidity. DESIGN, SETTING, AND PARTICIPANTSThe Dose Reduction of Preoperative Radiotherapy in Myxoid Liposarcomas (DOREMY) trial is a prospective, single-group, phase 2 nonrandomized controlled trial being conducted in 9 tertiary sarcoma centers in Europe and the US. Participants include adults with nonmetastatic, biopsy-proven and translocation-confirmed myxoid liposarcoma of the extremity or trunk who were enrolled between November 24, 2010, and August 1, 2019. Data analyses, using both per-protocol and intention-to-treat approaches, were conducted from November 24, 2010, to January 31, 2020. INTERVENTIONSThe experimental preoperative radiotherapy regimen consisted of 36 Gy in once-daily 2-Gy fractions, with subsequent definitive surgical resection after an interval of 4 or more weeks. MAIN OUTCOMES AND MEASURESAs a short-term evaluable surrogate for local control, the primary end point was centrally reviewed pathologic treatment response. The experimental regimen was regarded as a success when 70% or more of the resection specimens showed extensive treatment response, defined as 50% or greater of the tumor volume containing treatment effects. Morbidity outcomes consisted of wound complications and late toxic effects. RESULTS Among the 79 eligible patients, 44 (56%) were men and the median (interquartile range) age was 45 (39-56) years. Two patients did not undergo surgical resection because of intercurrent metastatic disease. Extensive pathological treatment response was observed in 70 of 77 patients (91%; posterior mean, 90.4%; 95% highest probability density interval, 83.8%-96.4%). The local control rate was 100%. The rate of wound complication requiring intervention was 17%, and the rate of grade 2 or higher toxic effects was 14%. CONCLUSIONS AND RELEVANCEThe findings of the DOREMY nonrandomized clinical trial suggest that deintensification of preoperative radiotherapy dose is effective and oncologically safe and is associated with less morbidity than historical controls, although differences in radiotherapy techniques and follow-up should be considered. A 36-Gy dose delivered in once-daily 2-Gy fractions is proposed as a dose-fractionation approach for myxoid liposarcoma, given that phase 3 trials are logistically impossible to execute in rare cancers.

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Myxoid\Round Cell Liposarcoma (MRCLS) Revisited: An Analysis of 418 Primarily Managed Cases

Abstract: MLS and RCLS showed different metastatic risk but equally good local control. Radiotherapy was effective in preventing local recurrence and should be delivered as neoadjuvant. New staging strategies are to be defined to account for the unusual metastatic pattern.

Cited by 133 publications

References 30 publications

Localized and metastatic myxoid/round cell liposarcoma

Localized and metastatic myxoid/round cell liposarcoma

Prognostic factors for the recurrence of myxoid liposarcoma: 20 cases with up to 8 years follow-up

Dose Reduction of Preoperative Radiotherapy in Myxoid Liposarcoma

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