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Introduction EORTC-62092 (STRASS) was a phase 3, randomized study that compared surgery alone versus surgery plus neoadjuvant radiotherapy (RT) for retroperitoneal sarcomas. RT was not associated with improved abdominal recurrence-free survival, the primary outcome measure, although on subanalysis, there may have been benefit for well-differentiated (WD) liposarcoma. This study investigated the real-world use and outcomes of RT (neoadjuvant and adjuvant) for the management of retroperitoneal liposarcoma. Methods We queried the National Cancer Database (NCDB) (2004–2017) for patients with nonmetastatic, primary retroperitoneal liposarcoma treated with resection with or without RT ( n = 3911). Patients were stratified by treatment type and histology [WD ( n = 2252), dedifferentiated (DD) ( n = 1659)]. Propensity score (PS) matching was used before comparison of treatment groups. Overall survival (OS) was the primary outcome measure. Results Median follow-up time was 4.1 years, and median OS was 10.7 years. There was no association between RT and OS for either WDLPS or DDLPS cohorts. We performed a subgroup analysis of neoadjuvant RT only, similar to STRASS. For WDLPS after PS matching ( n = 208), neoadjuvant RT was not associated with OS (hazard ratio [HR] 1.01, p = 0.0523) but was associated with longer postoperative hospital stay ( p = 0.012). For DDLPS after PS matching ( n = 290), neoadjuvant RT was not associated with OS (HR 1.02, p = 0.889). For both WD-LPS and DD-LPS, utilization of neoadjuvant RT was associated with treatment at high-volume (≥ 10 cases/year) and academic/network facilities. Conclusions For primary retroperitoneal liposarcoma treated with surgical resection, radiotherapy was not associated with an overall survival benefit in this propensity-matched, adjusted analysis of the NCDB.

We present a case of an extremely rare type of soft-tissue sarcoma with an atypical clinical presentation. The patient, a female in her 20s with Li Fraumeni syndrome, had prior surgery for a large intra-abdominal tumour that was given the diagnosis of malignant myxoid spindle cell neoplasm. Her recurrence manifested as diffuse intra-abdominal sarcomatosis for which she ultimately underwent subtotal debulking with palliative intent. Final pathology rendered the diagnosis of myxoid pleomorphic liposarcoma, a newly described entity, distinct from the more common liposarcoma subtypes. The optimal treatment for this typically aggressive disease is currently unknown; until that is better defined, management should be carried out by sarcoma specialists.

Background: Liposarcomas, which represent 20% of all adult sarcomas, are the most common histological type of malignant soft tissue tumors. The aim of this study was to define the prognostic factors that predict the postoperative survival period for patients with primary retroperitoneal liposarcoma. Methods: The clinical data and prognoses of 71 patients with primary retroperitoneal liposarcoma who were treated in the General Hospital of the People's Liberation Army of China between January 1, 2000 and December 31, 2007 were retrospectively reviewed and analyzed. Results: The primary tumor from each patient was resected; 54.9% (39/71) were deemed R0 resections, 31.0% (22/71) were R1 resections and 14.1% (10/71) were deemed R2 resections (palliative operations). The median follow up was 68 months (range: 1-160 months). Of the patients who received an R1 or R2 resection of their primary tumor, 96.7% (59/61) had tumor recurrence. The 1-year, 3-year, and 5-year recurrence-free rates were 77.0%, 29.8% and 19.7%, respectively. As of April 2013, 53 of the 71 patients had died from tumor recurrence. The overall 1-year, 3-year, 5-year, and 10-year survival rates were 88.7%, 76.1%, 61.7%, and 30.4%, respectively. The factors that were significantly associated with prognosis in the univariate analysis were age (as a categorical variable) (P = 0.006), modus operandi (P = 0.000), histologic subtype (P = 0.000), tumor grade (P = 0.000), ascites (P = 0.000), postoperative metastasis (P = 0.000) and adjuvant therapy (P = 0.030). However, in the multivariate analysis, the modus operandi (P = 0.000), tumor grade (P = 0.006), ascites (P = 0.027), postoperative metastasis (P = 0.023) and age (as a categorical variable) (P = 0.002) were the only significant predictors of survival. Conclusions: Complete resection remains the most effective method for treating liposarcoma. High grade, old age (>60 years old), postoperative metastasis, and ascites predict poor prognoses.

Liposarcomas (LS) of the extremities and trunk are aggressive soft-tissue sarcomas and surgical resection combined with multimodal therapy represents the cornerstone of curative treatment. Despite advances in surgical and medical management patients are still at risk of developing medical complications that negatively affect morbidity and mortality. Kidney dysfunction, sarcopenia and progressive loss of visceral adipose tissue have emerged as prognostically relevant and potentially treatable complications in surgical oncology. However, despite their growing relevance, little is known about their frequency and impact on survival and morbidity in the context of LS. We conducted a retrospective study of 47 adult patients with localized LS of the extremities and trunk who underwent curative-intent surgery. Kidney function, CT morphometry of muscle (skeletal muscle index, SMI) and visceral adipose tissue (VAT) as well as clinical assessments including ECOG score were recorded at diagnosis (t1) and after a median follow-up (t2) of 11 months. Kidney dysfunction, defined as a decrease in eGFR of ≥ 25% between time points, was analyzed in relation to survival, sequentially assessed CT-morphometry of muscle and adipose tissue as well as functional status assessed by ECOG scores. All patients underwent curative-intent surgical treatment with or without additional multimodal treatment (surgery only: 51.1%, additional radiation: 31.9%, additional chemotherapy: 38.3%). Kidney dysfunction was frequent in our cohort (53.2% of all patients) and significantly associated reduced overall survival in Kaplan-Meier, uni- and multivariate Cox proportional hazards regression models (multivariate hazard ratio: 6.7; p = 0.03). In addition, patients with kidney dysfunction experienced a significantly accelerated loss of SMI (p < 0.001) and VAT (p < 0.001) as well as accelerated functional deterioration measured by worsening ECOG scores between t1 and t2 compared to a stable ECOG score in patients without kidney dysfunction (odds ratio of ECOG increase of: 8.0; p = 0.0027). To our knowledge, this is among the first studies to investigate kidney dysfunction and its consequences in adult LS patients. In our cohort of surgically treated adult patients with LS of the extremities and trunk, kidney dysfunction was a frequent and clinically impactful complication. It was significantly associated with decreased overall survival, loss of muscle and adipose tissue in sequential CT morphometry assessments and progressive functional decline. Off note, CT-morphometry enabled objective, high-resolution tracking of body composition decline and may serve as a promising additional tool for risk stratification. Nonetheless, given the limited cohort size and retrospective single-center design, the generalizability of our findings is limited and the results should therefore be interpreted with caution. Despite these limitations, our findings call for future prospective studies and an awareness for heightened renal surveillance and integrated body composition assessments in the multimodal management of sarcoma patients.

Background The impact of radiotherapy on the oncologic outcome of retroperitoneal liposarcoma (RPLS) remains controversial. The aim of this study was to evaluate the effect of radiotherapy on disease-specific survival (DSS) in a cohort of patients with RPLSs. Methods In this population-based, retrospective cohort study, patients with localized RPLSs who underwent surgical therapy were identified from the Surveillance, Epidemiology, and End Results-17 cancer registry program. After propensity-score matching for potential confounders, multivariable logistic and Cox regression analyses were used to examine factors associated with DSS and radiotherapy. Results From 2004 to 2020, 1692 patients with localized RPLS who underwent surgical therapy were identified (84.2%  White, 44.6% female, mean age 62 years). Of those patients, 393 patients (23.2%) received perioperative radiotherapy. Patients who received radiotherapy had a higher rate of tumor size between 10 and 20 cm and unknown tumor grading. After propensity-score matching, multivariable adjusted Cox regression and Kaplan–Meier survival analysis demonstrated no improvement of DSS for patients who underwent radiotherapy (hazard ratio 1.04, confidence interval 0.81–1.32; log-rank p  = 0.47). Patient age ≥80 years, larger tumor size, and tumor grading G3 versus G1/2 were associated with an increased risk of death due to RPLS. Subgroup analyses stratified by grading showed similar outcomes. Conclusions The administration of perioperative radiotherapy did not improve DSS in patients undergoing surgery for localized RPLS in this population-based study. Therefore, the use of perioperative radiotherapy in these patients may be questioned. However, the findings should be interpreted with caution due to the inherent limitations of the Surveillance, Epidemiology, and End Results (SEER) database.

Background Esophageal lipomatous tumors, also reported as fibrovascular polyp, fibrolipoma, angiolipoma, and liposarcoma, account for less than 1% of all benign mesenchymal submucosal tumors of the esophagus. Clinical presentation and therapy may differ based on location, size, and morphology. A comprehensive and updated systematic review of the literature is lacking. Methods A systematic review of the literature was performed according to PRISMA guidelines. Pubmed, Embase, Cochrane, and Medline databases were consulted using MESH keywords. Non-English written articles and abstracts were excluded. Sex, age, symptoms at presentation, diagnosis, tumor location and size, surgical approach and technique of excision, pathology, and morphology were extracted and recorded in an electronic database. Results Sixty-seven studies for a total of 239 patients with esophageal lipoma or liposarcoma were included in the qualitative analysis. Among 176 patients with benign lipoma, the median age was 55. The main symptoms were dysphagia (64.2%), transoral polyp regurgitation (32.4%), and globus sensation (22.7%). The majority of lipomas (85.7%) were intraluminal polyps, with a stalk originating from the upper esophagus. Overall, 165 patients underwent excision of the mass through open surgery (65.5%), endoscopy (27.9%), or laparoscopy/thoracoscopy (3.6%). Only 5 (3%) of patients required esophagectomy. Of the 11 untreated patients with an intraluminal polyp, 7 died from asphyxia. Overall, liposarcoma was diagnosed in 63 patients, and 12 (19%) underwent esophagectomy. Conclusion Esophageal lipomatous tumors are rare but potentially lethal when are intraluminal and originate from the cervical esophagus. Modern radiological imaging has improved diagnostic accuracy. Minimally invasive transoral and laparoscopic/thoracoscopic techniques represent the therapeutic approach of choice.

Primary retroperitoneal liposarcoma (PRPLS) is a rare malignancy with a high risk of recurrence and poor prognosis. To evaluate the prognostic value of the systemic inflammation response index (SIRI) in patients with PRPLS undergoing curative-intent surgical resection and to compare its predictive performance with other inflammatory markers. This retrospective study included 122 patients with pathologically confirmed PRPLS who underwent surgical resection at Peking University International Hospital between January 2021 and January 2024. Patients were stratified into high-SIRI and low-SIRI groups based on an optimal cutoff value determined by receiver operating characteristic (ROC) curve analysis. Clinical characteristics, laboratory parameters, and surgical outcomes were compared between groups. Recurrence-free survival (RFS) and locoregional recurrence-free survival (LRFS) were assessed using Kaplan–Meier survival curves and log-rank tests. Univariate and multivariate Cox proportional hazards regression analyses were performed to identify independent prognostic factors. Post-hoc power analysis was conducted to ensure sample adequacy. SIRI demonstrated the highest prognostic accuracy among evaluated inflammatory markers, with an AUC of 0.831 ( P  < 0.001), outperforming NLR (AUC = 0.724), PLR (AUC = 0.730), and MLR (AUC = 0.747). Patients with high SIRI (≥ 1.95) had significantly shorter RFS and LRFS compared to those with low SIRI ( P  < 0.001 and P  = 0.008, respectively). High SIRI was associated with larger tumor size, higher Ki-67 index, abnormal liver function markers, elevated CRP, and lower albumin levels. In multivariate Cox regression analysis, SIRI remained the only independent predictor of RFS (HR = 5.19, 95% CI: 2.356, P  < 0.001). Preoperative SIRI is an independent and superior prognostic biomarker for recurrence in patients with PRPLS following surgical resection. Compared to conventional inflammatory indices, SIRI shows stronger predictive value for both RFS and LRFS, offering a simple and effective tool for risk stratification in clinical practice.

The efficacy of preoperative radiotherapy combined with surgery (preRT + S) for primary retroperitoneal sarcoma (RPS) remains unclear. This study aimed to compare preRT + S with surgery alone (SA) in patients with RPS. Core databases were searched for directly comparative studies depending on preRT. Thirteen studies included 2,439 patients with SA and 1,453 with preRT + S. PreRT + S in all RPS patients led to significantly low local recurrence (LR) (hazard ratios [HR],0.575; p = 0.008) compared to SA. Among the liposarcoma patients, PreRT + S did not clearly affect LR or abdominal recurrence-free survival (ARFS). Excluding dedifferentiated liposarcoma (DDLPS), the patients underwent preRT + S significantly improved LR (HR,0.430; p = 0.002) and ARFS (HR,0.706; p = 0.045). In another subgroup analysis of patients with well-differentiated liposarcoma and grade 1–2 DDLPS, preRT + S significantly extended ARFS (HR,0.601; p = 0.014) compared to SA. There was no significant difference in overall survival (OS) (HR,0.904; p = 0.362 [RPS]; HR,0.724; p = 0.348 [WDLPS + G1-2DDLPS]) between preRT + S and SA in all comparisons. PreRT + S group demonstrated higher incidence of total (odds ratio [OR],1.580; p = 0.007) and severe (OR,3.680; p = 0.004) complications than the SA group. PreRT + S prevent recurrence in patients with WDLPS and low-grade DDLPS but is associated with increased complications, resulting in similar OS compared to SA.

Background Surveillance imaging of patients with retroperitoneal liposarcoma (RP-LPS) after surgical resection is based on a projected risk of locoregional and distant recurrence. The duration of surveillance is not well defined because the natural history of RP-LPS after treatment is poorly understood. This study evaluated the long-term risk of recurrence and disease-specific survival (DSS) for a cohort of patients with at least 10 years of progression-free survival (10yr-PFS) from their primary resection. Methods The prospective University of California, Los Angeles (UCLA) Sarcoma Database identified RP-LPS patients with 10yr-PFS after initial resection. The patients in the 10yr-PFS cohort were subsequently evaluated for recurrence and DSS. The time intervals start at date of initial surgical resection. Cox proportional hazards models were used to determine factors associated with recurrence and DSS. Results From 1972 to 2010, 76 patients with RP-LPS had at least 10 years of follow-up evaluation. Of these 76 patients, 39 (51%) demonstrated 10yr-PFS. The median follow-up period was 15 years (range 10–33 years). Among the 10yr-PFS patients, 49% (19/39) experienced a recurrence at least 10 years after surgery. Of those who experienced recurrence, 42% (8/19) died of disease. Neither long-term recurrence nor DSS were significantly associated with age, sex, tumor size, LPS subtype, surgical margin, or perioperative treatment with radiation or chemotherapy. Conclusion Patients who have primary RP-LPS treated with surgical resection ± multimodality therapy face a long-term risk of recurrence and disease-specific death unacknowledged by current surveillance imaging guidelines. Among the patients with 10yr-PFS, 49% experienced a recurrence, and 42% of those died of disease. These findings suggest a need for lifelong surveillance imaging for patients with RP-LPS.

Retroperitoneal sarcomas are rare and heterogeneous tumors. We here report two cases of liposarcoma in the renal compartment with a literature review in order to emphasize on the anatomical and surgical features of this tumor location and on its possible prognostic implications. The first reported case was a 45-year old female patient with liposarcoma at the level of the left renal compartment. The diagnosis of myxoid liposarcoma was based on anatomo-pathological examination of the surgical specimen. The second reported case was a 70-year old man with liposarcoma at the level of the right renal compartment. The diagnosis of dedifferentiated liposarcoma was based on anatomo-pathological examination of the surgical specimen. Retroperitoneal sarcomas are often diagnosed with a palpable mass. The gold standard treatment is complete tumor resection with healthy resection margins and without damage or rupture. For this purpose resection involving adjacent organs is recommended by some authors. However the anatomical constraints of tretroperitoneal compartment and the often important tumor volume limit the possibilities to achieve a satisfactory resection. CT scan and MRI are very useful imaging techniques. Histological diagnosis poses some problems; therefore immunohistochemistry and sometimes molecular biology are significantly helpful. Patient evolution is marked by the frequent occurrence of recurrences. A specialized multidisciplinary management of these rare and very varied tumors is recommended in order to optimize therapeutic outcomes.