Re: Odaet al. Frequent alteration ofp16INK4a/p14ARF andp53 pathways in the round cell component of myxoid/round cell liposarcoma:p53 gene alterations and reduced p14ARF expression both correlate with poor prognosis.J Pathol 2005;207:410–421

Perrone, Francesco; Tamborini, Elena; Suardi, Simona; Ms, Lagonigro; Pilotti, Silvana

doi:10.1002/path.1994

Cited by 3 publications

(3 citation statements)

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“…The infrequent mutations and normal TP53 function in MLS/RCLS explains the genetic stability of this tumor entity. In the few cases where TP53 mutations occur, this is associated with progressive disease [15] – [17] .…”

Section: Resultsmentioning

confidence: 99%

“…Besides of the fusion oncogene, mutations in PIK3CA or loss of PTEN expression is seen in 10–15% of the cases and these changes are associated with poor prognosis [12] . TP53 mutations have also been reported and associated with progressive disease [13] , [15] – [17] . The vast majority of the tumors carry normal TP53 genes and secondary changes are few and rare even when relapses occur [14] .…”

Section: Introductionmentioning

confidence: 99%

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Normal and Functional TP53 in Genetically Stable Myxoid/Round Cell Liposarcoma

et al. 2014

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Myxoid/round-cell liposarcoma (MLS/RCLS) is characterized by either the fusion gene FUS-DDIT3 or the less commonly occurring EWSR1-DDIT3 and most cases carry few or no additional cytogenetic changes. There are conflicting reports concerning the status and role of TP53 in MLS/RCLS. Here we analysed four MLS/RCLS derived cell lines for TP53 mutations, expression and function. Three SV40 transformed cell lines expressed normal TP53 proteins. Irradiation caused normal posttranslational modifications of TP53 and induced P21 expression in two of these cell lines. Transfection experiments showed that the FUS-DDIT3 fusion protein had no effects on irradiation induced TP53 responses. Ion Torrent AmpliSeq screening, using the Cancer Hotspot panel, showed no dysfunctional or disease associated alleles/mutations. In conclusion, our results suggest that most MLS/RCLS cases carry functional TP53 genes and this is consistent with the low numbers of secondary mutations observed in this tumor entity.

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Section: Resultsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Normal and Functional TP53 in Genetically Stable Myxoid/Round Cell Liposarcoma

et al. 2014

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“…In cases of MLS/RCLS, reduced p14 protein expression and p53 mutations associate with poor prognosis. In addition, the RC component is identified as a negative prognostic factor, potentially involving the p14ARF/p53 pathway in its development [16].…”

Section: Introductionmentioning

confidence: 99%

Prognostic factors and nomogram construction for primary retroperitoneal myxoid/round cell liposarcoma: an analysis of population-based data

Zhuang,

Cheng,

et al. 2024

JCTR

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Background: It has been reported that the prognosis for myxoid/round cell liposarcoma (MLPS/RCLPS) is inconsistent across different sites. However, there are neither prognostic studies nor predictive models that focused on MLPS/RCLPS of retroperitoneal origin. Methods: Utilizing the Surveillance, Epidemiology, and End Results database, we selected 171 primary retroperitoneal MLPS/RCLPS cases from the period between 2000 and 2019. Prognostic factors influencing disease-specific survival were identified through Cox regression analysis. These independent prognostic factors were then used to construct a DSS nomogram prediction model. The accuracy and reliability of this nomogram were evaluated using the concordance index (C-index) and calibration plots. Furthermore, we categorized patient prognosis using an X-tile based on the nomogram score. Results: The observed 5-year and 10-year DSS rates for all patients were 64.0% (95% CI: 56.2% – 71.8%) and 47.1% (95% CI: 38.1% – 56.1%), respectively. The patient cohort had a median age of 64 years, ranging from 24 to 92 years, with a slight male predominance (n = 92, 53.8%) over females (n = 79, 46.2%). Distant metastases were diagnosed in 24 patients (14%). The distribution of MLPS and RCLPS was 89.5% and 10.5%, respectively. In terms of treatment, adjuvant radiotherapy was administered to 33 patients (19.3%), neoadjuvant radiotherapy to 9 patients (5.3%), and chemotherapy to 20 patients (11.7%), while a significant majority (83.6%) underwent surgical procedures. Independent prognostic factors for DSS included age (HR = 1.039, P < 0.001), marital status (P = 0.029), history of previous tumors (HR = 0.257, P = 0.007), presence of metastatic disease (HR = 2.206, P = 0.027), and surgical treatment (HR = 0.490, P = 0.036). A nomogram prediction model was constructed to forecast 1-, 5-, and 10-year DSS rates, with a C-index of 0.739. Calibration plots demonstrated a strong correlation between the nomogram’s predictions and actual observations. Based on the prediction model, patients were stratified into three groups, and significant differences in prognosis were observed between these groups. Conclusion: A poorer prognosis is associated with retroperitoneal-derived MLPS/RCLPS than with other sites. The nomogram prediction model we built can be used to assist patients in consulting with their doctors and selecting patients for clinical trials. Relevance for Patients: Our study highlights the unique challenges and prognosis variations in retroperitoneal myxoid/RCLPS. The developed nomogram serves as a valuable tool for patients, aiding informed discussions with doctors and guiding decisions on treatment and clinical trial participation.

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