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European Journal of Surgical Oncology :... Jun 2023Retroperitoneal sarcomas (RPS) are rare malignancies that are potentially curable by complete surgical resection. A regular surveillance program is normally commenced...
Retroperitoneal sarcomas (RPS) are rare malignancies that are potentially curable by complete surgical resection. A regular surveillance program is normally commenced following surgery due to the risk of local recurrence (LR), especially in low-intermediate grade disease, and distant metastases (DM), especially in high-grade RPS. Consensus guidelines usually advocate for more frequent imaging during the first 2-3 years and less intensive imaging over a prolonged period thereafter, reflecting the incidence pattern of LR and DM. Definitive evidence for the most effective imaging schedule has never been provided, and retrospective studies have not shown an association between follow-up intensity and survival. Improvement in the prediction of recurrence patterns has been sustained by prognostic dynamic nomograms, which are now capable of forecasting disease behaviour in each patient according to specific features. Incorporation of such tools in clinical practice may help to stratify patients and tailor ongoing surveillance to the risk of recurrence. This may help to relieve patients' anxiety while awaiting results of surveillance investigations, and also reduce the economic and environmental burden of repeated imaging. A randomized controlled study (SARveillance Trial) is proposed to shed light on this controversial topic, allowing clinicians to harmonize the follow-up protocol of patients undergoing surgery for RPS.
Topics: Humans; Follow-Up Studies; Retrospective Studies; Sarcoma; Retroperitoneal Neoplasms; Prognosis; Neoplasm Recurrence, Local
PubMed: 35277304
DOI: 10.1016/j.ejso.2022.02.016 -
European Journal of Surgical Oncology :... May 2021Locoregional recurrence after resection of primary retroperitoneal sarcoma (RPS) is a challenging therapeutic issue. The objective of this study was to identify...
BACKGROUND AND PURPOSE
Locoregional recurrence after resection of primary retroperitoneal sarcoma (RPS) is a challenging therapeutic issue. The objective of this study was to identify clinicopathological factors predictive of overall survival (OS) and disease specific survival (DSS) after reoperation for recurrent RPS.
PATIENTS AND METHODS
We retrospectively collected data from the medical records of 800 patients who underwent resection for sarcoma at our Institution, from 1983 to 2015. Among these patients, 120 were treated for retroperitoneal sarcoma and 55 had a locoregional recurrence (LR). Four of them did not undergo surgery and thus were excluded from this study leaving 51 cases available for data analysis. Univariate and multivariate survival analyses were performed to identify prognostic factors.
RESULTS
Median overall survival was 33 months. The 1-year, 3-year and 5-year OS rates were 75.5%, 47.1% and 31.6% respectively. Multivariate Cox regression analysis suggested that extension of surgery (P = 0.026), surgical margin status (P = 0.015) and histological grade of recurrent tumor (P = 0.047) were independent prognostic factors for OS. Median DSS was 48 months. The 1-year, 3-year and 5-year DSS rates were 79.2%, 53.1% and 40.9%, respectively. At multivariate analysis, predictors of DSS were extension of surgery (P = 0.004), margin status (P = 0.011), histological grade of recurrent tumor (P = 0.008), and disease free interval (DFI) (P = 0.020). As regards histological subtype of recurrent RPS, at univariate analysis, well-differentiated liposarcoma (WDLS) was associated with better OS and DSS (P = 0.052 and P = 0.016 respectively) compared to dedifferentiated liposarcoma (DDLS).
CONCLUSIONS
According to our findings, surgery is more beneficial in patients with low-grade sarcoma, WDLS and long DFI. The achievement of clear resection margins, rather than performing a multivisceral resection, appears to be a key factor to improve OS and DSS.
Topics: Adult; Aged; Female; Humans; Male; Middle Aged; Neoplasm Grading; Neoplasm Recurrence, Local; Prognosis; Retroperitoneal Neoplasms; Retrospective Studies; Sarcoma
PubMed: 32950313
DOI: 10.1016/j.ejso.2020.08.030 -
BMC Cancer Nov 2023The clinical profiles of recurrent retroperitoneal liposarcoma (RLS) need to be explored. The recurrence patterns of RLS are controversial and ambiguous.
BACKGROUND
The clinical profiles of recurrent retroperitoneal liposarcoma (RLS) need to be explored. The recurrence patterns of RLS are controversial and ambiguous.
METHODS
A total of 138 patients with recurrent RLS were finally recruited in the study. The analysis of overall survival (OS) and recurrence-free survival (RFS) was performed by Kaplan‒Meier analysis. To identify independent prognostic factors, all significant variables on univariate Cox regression analysis (P ≤ 0.05) were subjected to multivariate Cox regression analysis. The corresponding nomogram model was further built to predict the survival status of patients.
RESULTS
Among patients, the 1-, 3-, and 5-year OS rates were 70.7%, 35.9% and 30.9%, respectively. The 1-, 3- and 5-year RFS rates of the 55 patients who underwent R0 resection were 76.1%, 50.8% and 34.4%, respectively. The multivariate analysis revealed that resection method, tumor size, status of pathological differentiation, pathological subtypes and recurrence pattern were independent risk factors for OS or RFS. Patients with distant recurrence (DR) pattern usually had multifocal tumors (90.5% vs. 74.7%, P < 0.05); they were prone to experience changes of pathological differentiation (69.9% vs. 33.3%, P < 0.05) and had a better prognosis than those with local recurrence (LR) pattern. R0 resection and combined organ resection favored the survival of patients with DR pattern in some cases.
CONCLUSIONS
Patients with DR pattern had better prognosis, and they may benefit more from aggressive combined resection than those with LR pattern. Classifying the recurrence patterns of RLS provides guidance for individualized clinical management of recurrent RLS.
Topics: Humans; Neoplasm Recurrence, Local; Liposarcoma; Retroperitoneal Neoplasms; Retroperitoneal Space; Prognosis; Survival Rate; Retrospective Studies
PubMed: 37936091
DOI: 10.1186/s12885-023-11586-8 -
BMC Cancer Sep 2020Myxoid liposarcoma (MLS) has the tendency to metastasize extrapulmonary. Although prognostic factors at the initial diagnosis of MLS have been reported, those at...
BACKGROUND
Myxoid liposarcoma (MLS) has the tendency to metastasize extrapulmonary. Although prognostic factors at the initial diagnosis of MLS have been reported, those at diagnosis of metastasis remain unclear. The purpose of this study was to investigate the prognostic factors for disease-specific survival at the initial diagnosis of metastasis.
METHODS
This retrospective observational study was conducted at three cancer centers and two university hospitals in Japan. Of 274 MLS patients pathologically diagnosed between 2001 and 2015, 48 metastatic patients were examined.
RESULTS
Lung metastases were detected in nine patients (18.8%) and extrapulmonary metastases in 45 (93.8%). Interval from primary diagnosis to the first metastasis was significantly shorter in patients with lung metastases than without (p = 0.007). Median disease-specific survival after diagnosis of metastases was 52.5 months in all patients. In multivariable analysis, liver metastasis (hazard ratio (HR), 2.71 [95% confidence interval (CI), 1.00-7.09]) and no evidence of disease (NED) achieved by radical treatment (resection with or without radiation therapy, or radiation therapy ≥60 Gy) or semi-radical (radiation therapy ≥40 Gy) treatment were significantly related to survival (HR, 0.36; 95%CI [0.13-0.95]). The number of metastases (odds ratio (OR), 0.44; 95%CI [0.25-0.78]) and abdominal/retroperitoneal metastases (OR, 0.09; 95%CI [0.008-0.95]) were the significant inhibitory factors of achieving NED.
CONCLUSIONS
This is the first study to statistically demonstrate the importance of achieving NED with surgical resection or radiation therapy for longer survival in metastatic MLS patients. As number of metastases was a significant factor for achieving NED, early detection of metastases might be important.
Topics: Adult; Aged; Disease-Free Survival; Female; Humans; Japan; Liposarcoma, Myxoid; Liver Neoplasms; Lung Neoplasms; Male; Middle Aged; Neoplasm Metastasis; Neoplasm Recurrence, Local; Prognosis; Retroperitoneal Neoplasms; Retrospective Studies
PubMed: 32928160
DOI: 10.1186/s12885-020-07384-1 -
Journal of Surgical Oncology May 2016The multi-modal treatment of retroperitoneal sarcoma has seen increased use of neoadjuvant radiation. However, its effect on local recurrence and survival remain... (Review)
Review
BACKGROUND AND OBJECTIVES
The multi-modal treatment of retroperitoneal sarcoma has seen increased use of neoadjuvant radiation. However, its effect on local recurrence and survival remain controversial. We aimed to synthesize and evaluate the literature.
METHODS
The review was conducted according the recommendation of the Meta-Analysis of Observational Studies in Epidemiology (MOOSE) group with pre-specified inclusion and exclusion criteria.
RESULTS
Of 8,701 citations collected, 15 articles reported on 464 patients. The median age was 56 years (45-64). The predominant histological subtypes were liposarcoma (51.54%) and leiomyosarcoma (23.26%). Tumor differentiation composed of 37.1% well-, 12.8% moderate-, 46.0% poorly-, and 4.1% undifferentiated. Most studies featured external beam radiation therapy (EBRT) treatment regimen with some who included patients treated with IMRT instead. Median follow-up averaged 41.4 months (19-106 months). Median 5-year OS, PFS, and LRR rates were 58%, 71.5%, and 25%. Using the NCI CTCAE, toxicities from Grade 1 (Mild) through Grade 5 (death) were experienced by 18.8%, 10.2%, 16.3%, 0.7%, and 1.6% of patients.
CONCLUSIONS
NART is a safe to use for RPS, but its effect toward survival and local control remains unclear. Without randomized control trials, common reporting criteria for pro- and retrospective studies are needed to allow comparison between studies. J. Surg. Oncol. 2016;113:628-634. © 2016 Wiley Periodicals, Inc.
Topics: Humans; Neoadjuvant Therapy; Neoplasm Recurrence, Local; Radiotherapy, Adjuvant; Retroperitoneal Neoplasms; Sarcoma; Treatment Outcome
PubMed: 26990903
DOI: 10.1002/jso.24221 -
Oncology (Williston Park, N.Y.) Sep 2018Retroperitoneal sarcoma (RPS) is a rare tumor whose diagnosis and management can be challenging and for which management requires a multidisciplinary team in a...
Retroperitoneal sarcoma (RPS) is a rare tumor whose diagnosis and management can be challenging and for which management requires a multidisciplinary team in a specialized center. An important part of the diagnosis-identification of the histologic subtype-depends on pathology; identifying the histologic subtype is important because this can affect prognosis and treatment options. Complete surgical resection with negative margins remains the cornerstone of treatment of nonmetastatic RPS and is the only chance for cure. In order to achieve negative margins, multivisceral en bloc resection is often necessary. Neoadjuvant therapies (chemotherapy, external beam radiation, or combination radiation and chemotherapy) are safe in well-selected patients and may be considered after careful review by a multidisciplinary sarcoma tumor board when the recurrence risk is high.
Topics: Dissection; Humans; Neoadjuvant Therapy; Neoplasm Staging; Patient Care Team; Patient Selection; Prognosis; Retroperitoneal Neoplasms; Sarcoma
PubMed: 30248168
DOI: No ID Found -
European Journal of Vascular and... Nov 2020Radical excision of retroperitoneal or intra-abdominal soft tissue sarcomas may necessitate vessel resection and reconstruction. The aim of this study was to assess... (Review)
Review
Multidisciplinary Oncovascular Surgery is Safe and Effective in the Treatment of Intra-abdominal and Retroperitoneal Sarcomas: A Retrospective Single Centre Cohort Study and a Comprehensive Literature Review.
OBJECTIVE
Radical excision of retroperitoneal or intra-abdominal soft tissue sarcomas may necessitate vessel resection and reconstruction. The aim of this study was to assess surgical results of retroperitoneal or intra-abdominal sarcomas involving major blood vessels.
METHODS
This was a retrospective single centre cohort study and a comprehensive review of literature. Patients with retroperitoneal or intra-abdominal sarcomas treated by the oncovascular team in Helsinki University Hospital from 2010 to 2018 were reviewed for vascular and oncological outcomes. A comprehensive literature review of vascular reconstructions in patients with retroperitoneal sarcoma was performed.
RESULTS
Vascular reconstruction was performed in 17 patients, 11 of whom required arterial reconstructions. Sixteen of the operations were sarcoma resections; the post-operative diagnosis for one patient was thrombosis instead of the presumed recurrent leiomyosarcoma. Early graft thrombosis occurred in two venous and one arterial reconstruction. Late thrombosis was detected in three (18%). The median follow up was 27 (range 0-82) months. Of the patients with sarcoma resections 5 (31%) died of sarcoma and further 4 (25%) developed local recurrence or new distant metastases. The comprehensive review of literature identified 37 articles with 110 patients, 89 of whom had inferior vena cava reconstruction only. Eight arterial reconstructions were described. Late graft thrombosis occurred in 14%. The follow up was 0-181 months, during which 57% remained disease free and 7% died of sarcoma.
CONCLUSION
Vascular reconstructions enable radical resection of retroperitoneal and intra-abdominal sarcomas in patients with advanced disease. The complex operations are associated with an acceptable rate of serious peri-operative complications and symptomatic thrombosis of the repaired vessel is rare. However, further studies are needed to assess the performance of the vascular reconstructions in the long term.
Topics: Adult; Aged; Arteries; Blood Vessel Prosthesis Implantation; Female; Follow-Up Studies; Graft Occlusion, Vascular; Humans; Male; Middle Aged; Neoplasm Staging; Postoperative Complications; Retroperitoneal Neoplasms; Retroperitoneal Space; Retrospective Studies; Sarcoma; Thrombosis; Treatment Outcome; Vascular Patency; Vena Cava, Inferior
PubMed: 32741678
DOI: 10.1016/j.ejvs.2020.05.029 -
Functional & Integrative Genomics Dec 2022Based on a case report, this review explores the genomic landscape for patients with liposarcomas and possible relationships with gene mutations related to... (Review)
Review
Based on a case report, this review explores the genomic landscape for patients with liposarcomas and possible relationships with gene mutations related to craniosynostosis. We describe the case of a 40-year-old man, known for a surgical correction of craniosynostosis before the age of 1 year, who underwent a radical resection of a voluminous retroperitoneal liposarcoma; histopathological analysis revealed a low-grade well-differentiated, mostly sclerosing, liposarcoma. A genetic analysis searching for mutations in blood DNA was performed and did not detect any specific mutation. A literature review was also conducted. Several tumors related to syndromic and non-syndromic craniosynostosis are mentioned in the literature; no specific link with retroperitoneal liposarcoma is established but the FGFR3 mutation is detected in dedifferentiated liposarcomas. To date, no case has been reported in the literature demonstrating a genetic relationship between craniosynostosis and low-grade differentiated retroperitoneal liposarcoma. We conclude that further studies for gene complex mutations should be conducted to show a possible genetic relationship between retroperitoneal liposarcoma and craniosynostosis.
Topics: Male; Humans; Adult; Female; Liposarcoma; Retroperitoneal Neoplasms; Mutation; Genomics
PubMed: 36538187
DOI: 10.1007/s10142-022-00924-x -
Rozhledy V Chirurgii : Mesicnik... 2016Retroperitoneal sarcomas are rare malignant neoplasms that are often diagnosed late because of their typical nonspecific symptomatology. Radical surgical resection, if...
Retroperitoneal sarcomas are rare malignant neoplasms that are often diagnosed late because of their typical nonspecific symptomatology. Radical surgical resection, if possible given the local anatomical conditions, is the most important treatment modality. However, even if the surgery is feasible and successful, the prognosis of retroperitoneal sarcoma is not good. The most frequent type of recurrence is local recurrence, and radical surgical resection remains the most important treatment modality even in such cases.Our study presents a case report of a patient with a retroperitoneal leiomyosarcoma that was radically (R0) removed. Two years later a local recurrence developed, which was eventually diagnosed by MRI. Again, radical (R0) surgical resection was approached (because of tumor invasion into inferior caval vein, the vein had to be partially resected as well and substituted with a vascular prosthesis).The report describes a relatively typical case of retroperitoneal sarcoma, while pointing out the importance of timely diagnosis (which is definitely not easy to achieve) and especially of radical surgical treatment.Key words: retroperitoneal sarcoma recurrence surgery.
Topics: Aged; Blood Vessel Prosthesis Implantation; Female; Humans; Leiomyosarcoma; Magnetic Resonance Imaging; Male; Neoplasm Recurrence, Local; Prognosis; Retroperitoneal Neoplasms; Vena Cava, Inferior
PubMed: 27879144
DOI: No ID Found -
Journal of Surgical Oncology Jan 2018Sarcomas represent a highly heterogeneous group of tumors as reflected in the significant overlap between their histologic phenotypes between the different types, posing... (Review)
Review
Sarcomas represent a highly heterogeneous group of tumors as reflected in the significant overlap between their histologic phenotypes between the different types, posing diagnostic challenges for the pathologist. Definitive tumor classification is increasingly important because of prognostication and emergence of targeted therapies for some of the sarcoma types. In this review, we highlight pertinent pathologic and molecular aspects of sarcomas common in the retroperitoneum, relevant to the surgical oncologist.
Topics: Humans; Retroperitoneal Neoplasms; Sarcoma
PubMed: 29230829
DOI: 10.1002/jso.24928