A case report of retroperitoneal liposarcoma.

BACKGROUND: Retroperitoneal liposarcoma is a rare and complex tumor originating from the mesenchymal tissues, with no specific manifestations in the early stage, and a large tumor size in the late stage. Patients often consult a physician because of large abdominal mass, increased abdominal circumference, and abdominal pain, and rarely because of leukocytosis. PATIENT CONCERNS: A 54-year-old female presented to our hospital with complaints of "abdominal distension for over 3 months, left lumbar pain for over 2 months." Considering the comprehensive symptoms, examinations, computed tomography scans, and pathological results, the possibility of retroperitoneal liposarcoma is high. DIAGNOSES: Retroperitoneal liposarcoma with leukocytosis. INTERVENTIONS: Open retroperitoneal mass excision along with transcystoscopic left ureteral Double-J Ureterl Stent Insertion tube placement and left nephrectomy. OUTCOMES: The postoperative pathological findings of the abdominal mass, combined with morphological and immunohistochemical results, are consistent with retroperitoneal liposarcoma. The patient had no recurrence in 7 months of postoperative follow-up conducted on the telephone and is now in continued follow-up. CONCLUSION: Retroperitoneal liposarcoma is highly malignant and prone to recurrence. Radical surgery is currently the primary treatment modality for patients with this condition. Analogous to cancer patients, those with elevated white blood cell counts and retroperitoneal liposarcoma may have poor prognoses, with a high likelihood of local recurrence and distant metastasis. Close postoperative follow-up is necessary. Therefore, regular postoperative review of blood routine may be a relatively economical and convenient method for the early detection of recurrence and metastasis of retroperitoneal liposarcoma.

Complications following biopsy of an intraabdominal or retroperitoneal mass compared with a renal mass.

INTRODUCTION: This study aimed to assess the short-term complication rate after US-guided core needle biopsies with an 18-gauge needle of retroperitoneal or intraabdominal masses (non-kidney group) compared with complications after biopsy from a renal mass (kidney group). METHODS: This was a retrospective analysis of 330 consecutive patients in the non-kidney group and 330 control patients in the kidney group. We recorded baseline characteristics, diagnostic yield, complications graded as Clavien-Dindo (CD) and readmissions within one and seven days. RESULTS: In all, 245 patients in the non-kidney and 281 patients in the kidney group had a biopsy performed. A total of 54 (22%) patients in the non-kidney group had a complication registered. However, 47 were minor complications (minor bleeding or localised pain, CD 1). In the kidney group, 47 (17%) patients had a complication, with 44 being graded as CD 1. No major complications (CD 3 or higher) were associated with the biopsies. Only 0.8% of patients in the non-kidney group and 0.7% in the kidney group had a treatment-requiring CD 2 complication (i.e. blood transfusion) directly caused by the US-guided biopsy. These complications were recognised less than 30 minutes or more than four hours after the procedure. We found no significant difference in the complication rate, diagnostic yield or risk of re-admission between the two groups. CONCLUSION: The observation period for patients who undergo an uncomplicated US-guided biopsy from an intraabdominal or retroperitoneal mass can safely be reduced to 30 minutes. FUNDING: None TRIAL REGISTRATION. Not relevant.

Giant retroperitoneal liposarcoma with multiple organ involvement: a case report and literature review.

BACKGROUND: Retroperitoneal liposarcoma (RPLPS) is a relatively rare disease. Liposarcomas vary in size, but sizeable RPLPS larger than 30 cm in diameter are very rare, and their diagnosis and treatment present significant challenges. CASE PRESENTATION: We report a 58-year-old male patient who was admitted to the hospital with an increased abdominal circumference and was later diagnosed with a giant RPLPS. The liposarcoma was found to adhere to the right kidney and the entire ureter, invading the ascending colon. The patient underwent complete combined surgical resection. The tumor was removed intact, measured 55.0 cm × 30.0 cm × 18.0 cm, and weighed 19.8 kg. Histopathologic analysis revealed well-differentiated liposarcoma (WDLPS). The patient was successfully discharged from the hospital and followed up for 6 months with no signs of recurrence. CONCLUSION: RPLPS is a rare tumor with atypical clinical presentation. Surgery remains the most effective method of treatment for retroperitoneal sarcomas, with complete removal if there is local invasion. Preoperative examination, including three-dimensional (3D) reconstruction, is essential for surgical success. The role of adjuvant radiotherapy or chemotherapy remains controversial. However, clinicians should not rule them out as viable options.

Primary retroperitoneal tumor: mucinous cystoadenocarcinoma.

Primary retroperitoneal mucinous cystadenocarcinomas (PRMCs) are extremely rare tumors with limited understanding of their pathogenesis and biological behavior. We describe a case of a 50-year-old female patient who underwent surgical treatment. The patient had a history of previous surgeries for mesenteric mucinous cystadenoma, without evidence of recurrence. During routine abdominal ultrasound a new tumor was found. An abdomen magnetic resonance imaging was done and confirmed the presence of a cystic lesion in the right iliac fossa. After discussion in multidisciplinary committee, surgical complete resection of the tumor, along with bilateral adnexectomy, was performed successfully. Histopathological examination revealed a mucinous adenocarcinoma adjacent to a mucinous cystadenoma. Immunohistochemical analysis supported the diagnosis of a primary retroperitoneal lesion. The patient had an uneventful recovery and has remained disease-free during the two-year postoperative follow-up. PRMCs are challenging to diagnose preoperatively due to nonspecific symptoms. Surgical excision is the mainstay of treatment. The long-term prognosis and optimal therapeutic strategies require further investigation.

Los cistoadenocarcinomas mucinosos primarios retroperitoneales (CMPR) son tumores extremadamente raros con una comprensión limitada de su patogénesis y comportamiento biológico. Describimos el caso de una mujer de 50 años sometida a tratamiento quirúrgico. La paciente tenía antecedentes de cirugías previas por cistodenoma mucinoso mesentérico, sin evidencia de recurrencia. Durante una ecografía abdominal de rutina se encontró un nuevo tumor. Se realizó una resonancia magnética abdomen que confirmó la presencia de una lesión quística en la fosa ilíaca derecha. Luego de discutir el caso en el comité multidisciplinario, se realizó con éxito la resección quirúrgica completa del tumor, junto con la anexectomía bilateral. El examen histopatológico reveló un adenocarcinoma mucinoso adyacente a un cistodenoma mucinoso. El análisis inmunohistoquímico apoyó el diagnóstico de lesión primaria retroperitoneal. La paciente tuvo una buena recuperación y permaneció libre de enfermedad durante dos años de seguimiento postoperatorio. Los CMPR son difíciles de diagnosticar debido a que presentan síntomas inespecíficos. La escisión quirúrgica es la base del tratamiento. El pronóstico a largo plazo y las estrategias terapéuticas óptimas requieren más investigación.

Molecular markers of proliferation, DNA repair, and immune infiltration defines high-risk subset of resectable retroperitoneal sarcomas.

INTRODUCTION: For retroperitoneal sarcomas (RPS), aggressive surgical resection offers the only chance for a cure; however, 5-year survival remains below 65%. Therefore, there is a critical need to identify drivers of poor clinical outcomes. MATERIALS AND METHODS: To identify biomarkers of tumors likely to recur following curative intent resection, we performed genomic and transcriptomic sequencing for 47 and 34 patients, respectively, with non-metastatic RPS at a single, high-volume sarcoma center. RESULTS: At the DNA level, alterations in TERT were associated with poor disease-free survival (DFS) and overall survival (OS). Increased RNA expression of gene sets related to growth signaling and DNA repair were associated with poor DFS and OS. Infiltration of CD8+ T-Cells and activated dendritic cells were associated with poor DFS and OS. CONCLUSION: These findings may help to better identify and treat non-metastatic, high-risk RPS.

Clinical features and surgical strategy of retroperitoneal liposarcoma involving the kidney capsule: a retrospective comparative cohort study.

BACKGROUND: Valid and generalizable data on the clinical features and surgical strategies for retroperitoneal liposarcoma (LPS) involving the kidney capsule remain scarce. This study aimed to investigate the clinical characteristics, morbidity, mortality, and long-term survival of patients with retroperitoneal LPS involving the kidney capsule. METHODS: The authors analyzed a prospectively maintained database of patients who underwent surgical resection for retroperitoneal LPS between 2015 and 2020. The patients were categorized into kidney capsule or no kidney capsule groups based on the presence or absence of kidney capsule involvement. A kidney-sparing strategy for retroperitoneal LPS involving the kidney capsule was developed. The primary outcome measure was overall survival (OS). The cumulative event probability curve was estimated using the Kaplan-Meier, and differences between groups using the Log-Rank. RESULTS: The study population consisted of 128 patients-54 with and 74 without kidney capsule involvement. Of these patients, 70 were female (54.7%) and 58 were male (45.3%), with a median age of 55. The median follow-up duration was 35 months. Postoperative morbidity, mortality, length of hospital stay, length of ICU stay, OS, and recurrence-free survival (RFS) did not differ significantly between the groups. Eleven patients developed postoperative acute kidney injury (AKI), and one patient required dialysis during the follow-up period. In multivariable logistic regression analysis, only nephrectomy was independently associated with postoperative AKI. Subgroup analysis of patients with kidney capsule involvement showed that nephrectomy did not improve OS or RFS but significantly decreased postoperative estimated glomerular filtration rate. CONCLUSION: Nephrectomy was associated with an increased risk of postoperative AKI after retroperitoneal LPS resection. A kidney-sparing strategy for retroperitoneal LPS involving the kidney capsule achieved optimal clinical outcomes.

TRAbectedin in adVanced rEtroperitoneal well differentiated/dedifferentiated Liposarcoma and Leiomyosarcoma (TRAVELL): results of a phase II study from the Italian Sarcoma Group.

BACKGROUND: This is a multicentre, single-arm, phase II study aimed at further exploring the activity of trabectedin as second-/further-line treatment in retroperitoneal leiomyosarcoma (LMS) and well-differentiated/dedifferentiated liposarcoma (LPS). MATERIALS AND METHODS: The primary endpoint was the growth modulation index (GMI) defined as the ratio between PFS under trabectedin (PFS) and during previous chemotherapy treatment: time to progression (TTP-1). Secondary endpoints were objective response rate (ORR) and PFS. As per protocol, patients were considered responders if the GMI was >1.33, non-responders if <0.75 and neither if 0.76-1.32. RESULTS: Overall 91 patients were assessable for the primary endpoint (32 patients with LMS and 59 patients with LPS): the median number of cycles received was 6.0 (Q1-Q3 3.0-12.0), and the main reason for treatment discontinuation was disease progression in 72% of patients. The median PFS was 6.0 months, while the median TTP1 was 7.5 months (8.1 and 6.4 months for LMS and LPS, respectively). Thirty-three patients [52%, 95% confidence interval (CI) 36% to 58%, P = 0.674, odds of response 1.1] had a GMI >1.33 (LMS 46%, 95% CI 26% to 67%, odds of response 0.85; LPS 56%, 95% CI 40% to 72%, odds of response 1.3). Overall, in LPS we observed 15/47 patients with a GMI <0.5 and 15/47 patients with a GMI >2. Among LMS patients, 9/26 had a GMI <0.5 and 10/26 had a GMI >2. Overall, ORR (complete response + partial response) was 16% (24% for LMS and 12% for LPS). CONCLUSIONS: While the primary endpoint of the study was not met, we noticed a subgroup of patients with a markedly discrepant TTP with trabectedin in comparison to previous therapy (GMI <0.5 or >2, the latter including some patients with a long TTP with trabectedin). A mismatch between PFS and overall survival was observed, possibly due to the natural history of the two different histologies and the availability of further lines in LMS.

Automated lattice radiation therapy treatment planning personalised to tumour size and shape.

PURPOSE: Lattice radiation therapy (LRT) alternates regions of high and low doses inside the tumour. Whilst this technique reported positive results in tumour size reduction, optimal lattice parameters are still unknown. We introduce an automated LRT planning method personalised to tumour shape and designed to allow investigation of lattice geometry. METHODS: Patients with retroperitoneal sarcoma were considered for inclusion. Automation was performed with the Eclipse Scripting Application Interface (v16, Varian Medical Systems, Palo Alto). By iterating over vertex size (V) and centre-to-centre distance (D), vertices were segmented within the gross tumour volume (GTV) in an alternating square pattern. Iterations stopped when the number of inserted vertices was contained between a prespecified lower and upper bound. Forty sets of lattices were considered, produced by varying V and D in five lower/upper bound pairs. Best-scoring sets were determined with a score favouring the maximization of GTV dose uniformity and heterogeneity whilst minimizing the maximum dose to organs at risk. RESULTS: Fifty patients with tumour volumes between 150 cm3 and 10,000 cm3 were included. Best-scoring sets were characterised by a low number of vertices (<15). Based on the best-scoring set, the predicted parameters to use for new patients were V = 0.19 (GTV volume)1/3 and D = 2V, in centimetres. The number of vertices (N) to insert in the GTV can be estimated with N ≤ (24 × 3% GTV volume)/(4πV3). CONCLUSIONS: The automated LRT treatment planning personalised to tumour size allows investigation of lattice geometry over a large range of GTV volumes.

A Rare Case of a Gigantic Retroperitoneal Schwannoma.

Introduction: Schwannomas (Schs) are benign tumor masses that rarely occur intra-abdominally and rarely reach larger diameters. When present, they occur as rare solitary nerve sheath tumors of peri-neural Schwann cells. Schwannoma mostly affects the nerves of the extremities, trunk, or the head and neck region. They are more common in female patients, mostly among patients between the third and fifth decade. They occur spontaneously but could also be found in association with a group of genetic autosomal dominant disorders called type 2. When present intra-abdominally, schwannomas grow slowly without significant clinical signs and symptoms. Clinical importance is presented in cases of occupying intra-abdominal space and impingement of surrounding structures, which causes intermittent pain. Only 0.5-5% of all retroperitoneal tumors are schwannomas and their malignant transformation is very rare. Case report: The authors present a case of a large intra-abdominal schwannoma in a 70-year-old female patient. She underwent CT scanning due to refractory left-sided subcostal pain, which revealed a large tumor mass in the left-sided hemiabdomen. Preoperative cytologic biopsy confirmed Sch. The patient underwent an MRI scan upon admission to our department, which revealed the origin of the tumor at the left-sided L3 level and intra-abdominal tumor spreading with the largest diameter of 25 cm. The patient underwent multidisciplinary surgical excision, confirmed by MRI scan in a period of five months postoperatively. Conclusions: Its rare presentation leads to the necessity to adequately evaluate such patients, especially to avoid any hidden diagnosis which might lead to further complications. The goal of a multidisciplinary approach should be emphasized as maintaining a good postsurgical condition without neurological deficits.

Retroperitoneal Lymph Node Dissection in Patients with Stage II Seminomatous Germ Cell Tumour.

Treatment de-escalation strategies in patients with seminoma with retroperitoneal metastases are being investigated in ongoing clinical trials. Primary retroperitoneal lymph node dissection conducted by expert surgeons may avoid any cytotoxic treatment and related long-term side effects in ≥70% of patients with clinical stage IIA/B seminoma.

Mini-review: Evaluation and Management of Retroperitoneal Masses in Patients with Testicular Cancer.

Testicular germ cell tumours (GCTs) account for the majority of testicular malignancies. Seminomas and nonseminomas differ in prognosis and management strategies. While cisplatin-based chemotherapy has significantly improved survival rates, identification of residual masses after chemotherapy is crucial for determining further treatment and survival. For seminomas, spontaneous resolution of residual masses occurs in a significant percentage of cases. Fluorodeoxyglucose positron emission tomography (FDG PET) is recommended for evaluation of residual masses after chemotherapy. Retroperitoneal lymph node dissection (RPLND) offers therapeutic benefits but is challenging because of an increase in desmoplasia after chemotherapy. For nonseminomas, residual masses are common after chemotherapy, with surgical resection necessary for masses larger than 1 cm. FDG PET has limited utility, and timely surgical intervention is crucial for favourable outcomes. Teratoma, if left unresected, can lead to serious complications, including growing teratoma syndrome, malignant transformation, and late relapse. Extraretroperitoneal residual masses, particularly those containing teratoma, are associated with poorer prognosis. Surgical resection remains the mainstay treatment, with significantly higher progression-free and recurrence-free survival rates for fibrosis/necrosis in comparison to teratoma or viable cancer. Understanding the characteristics and management of residual masses after chemotherapy is paramount for optimising treatment strategies and improving patient outcomes in testicular GCT. PATIENT SUMMARY: We reviewed treatment options for patients with testicular cancer who still have tumour tissue in the lower abdomen after chemotherapy. Surgical removal of the tumour is the main option; removal of lymph nodes can also help, but may be difficult because of tissue reactions to chemotherapy. Survival rates differ according to the tumour type and are lower for tumours beyond the lower abdomen.

[TFE3-rearranged perivascular epithelioid cell tumors: a clinicopathological analysis of eight cases].

Objective: To investigate the clinicopathological, immunohistochemical and molecular genetic characteristics of TFE3-rearranged perivascular epithelioid cell tumor (PEComa). Methods: Eight cases of PEComa with TFE3 rearrangement diagnosed in the First Affiliated Hospital of Air Force Medical University from January 2014 to July 2022 were collected. Three were consultation cases and 5 were collected from our hospital; 7 cases were resection specimens and 1 case was a needle biopsy specimen. Routine histolopathological analysis, immunohistochemical staining, fluorescence in situ hybridization (FISH) and the next-generation sequencing were performed. Clinical data were collected and the prognosis was assessed. Results: The 8 patients consisted of 5 females and 3 males with a median age of 45 years (ranged from 25 to 65 years). The tumor location included 1 uterus, 1 liver, 1 urachus, 2 kidneys, 1 abdominal cavity, 1 colon, and 1 retroperitoneum (3 subsequent recurrences in the abdominal cavity, pelvis and ovary, and abdominal cavity, respectively). Morphologically, the tumor cells were uniform and epithelioid with translucent or eosinophilic cytoplasm. They were arranged in nests or sheets, most of which were separated by thin-walled blood vessels. There were no papillary structures, and no overt smooth muscle or fat components. Atypical features were seen in 3 cases, with bizarre nuclei and tumor giant cells. Large areas of necrosis were visible, and mitosis was common (up to 28/50 HPF). Melanin deposition was present in 3 cases. Immunohistochemical staining showed diffuse and strong positivity for TFE3 in 8/8 cases and for HMB45 in 6/8 cases; focal positivity for Cathepsin K and Melan-A in 6/8 cases and for SMA in 2/8 of cases. All cases were negative for CKpan, PAX8 and Desmin. TFE3 gene break-apart was detected by FISH in all 8 cases, 4 of which underwent next-generation sequencing, and it revealed that 2 cases presented with SFPQ::TFE3 fusion, 1 case with ASPSCR1::TFE3 fusion, and 1 case with no chimeric fusion. Seven cases were followed up for 4-94 months. All cases were alive; 4 cases were disease-free, 2 cases showed recurrence, and 1 case had metastasis at initial diagnosis. Conclusions: TFE3-rearranged PEComa has unique histomorphological, immunohistochemical and molecular characteristics. The biological behavior is aggressive, which could lead to recurrence and metastasis, and warrants close clinical follow-up.

Laparoscopic removal of retroperitoneal schwannoma in renal hilum: a case report.

BACKGROUND: Schwannomas in the renal hilum are rare among retroperitoneal tumors. However, the possibility of malignant findings cannot be ruled out, and surgery is often indicated. This case was expected to be difficult to remove laparoscopically because the tumor was sandwiched between the arteriovenous veins of the renal portal. Sometimes, the tumor should be resected with a conservative approach to the kidney to preserve the renal function. CASE PRESENTATION: Our patient was a 51-year-old Asian-Japanese man who was referred to our department for a retroperitoneal tumor in the renal hilum. Since malignancy could not be ruled out due to its size (45 × 48 × 55 mm) on imaging, the tumor was excised laparoscopically. Histopathology revealed schwannoma. CONCLUSIONS: We herein report a case in which a renal hilar tumor between renal arteriovenous vessels was successfully resected laparoscopically.

A CARE-compliant article: A case report of retroperitoneal endometrial stromal sarcoma with multiple pulmonary metastases and literature review.

RATIONALE: Endometrial stromal sarcoma is an extremely rare mesenchymal neoplasm occurring in the extrauterine. Retroperitoneal endometrial stromal sarcoma with multiple pulmonary metastases, in particular, is extremely rare. PATIENT CONCERNS: Forty-seven-year-old woman (gravida 3, para 2), was referred to our institution with complaints of fever. DIAGNOSES: Ultrasound and computed tomographic imaging of the abdomen identified the presence of masses in the pelvic region. Additionally, computed tomographic scans and X-ray evaluations of the thorax detected dispersed masses in both the lungs and pelvic area. Histopathological analysis of the pelvic region indicated the presence of low-grade endometrial stromal sarcoma. A computed tomography-guided pneumocentesis was conducted to further characterize the pulmonary lesions, confirming the diagnosis of low-grade endometrial stromal sarcoma. INTERVENTIONS: The patient underwent tumor resection, subsequent treatment with Medroxyprogesterone acetate for 6 months, received microwave ablation for multiple lung metastases, PARP1 inhibitor for 4 courses, and has been undergoing chemotherapy (epirubicin/ifosfamide) up to the present time. OUTCOMES: Partial remission was obtained after the above treatment and this patient is now still alive maintaining for 3 years. LESSONS: The identification and management of this disease remain a significant challenge due to its low prevalence. Further research involving a larger number of cases is necessary to ensure consistency in diagnosis and to establish effective treatment guidelines.

Surgery for Infrarenal Retroperitoneal Node Metastases from Colon Cancer.

PURPOSE: Treatment of retroperitoneal lymph node metastases (RPN) from colon cancer (CC) is a therapeutic challenge. Available evidence supporting a curative approach is weak and uncertainties remain concerning the extent of the dissection, the optimal timing for surgery, and the role of adjuvant radiotherapy. We report the outcomes of a curative intent strategy in a recent monocentric series of patients. METHODS: We did a retrospective review of all curative intent surgical treatment of RPN from CC performed consecutively in a French university hospital from June 2015 to April 2021. Demographics, clinicopathological, and molecular characteristics were evaluated. We describe recurrence-free and overall survival and factors related to recurrence. RESULTS: Records from 18 patients were reviewed. The median age was 69 years. Most of the patients were male (55%), ASA 1-2 (94%), had a left-sided primary colon cancer (73%), and had metachronous RPN (62%). Thirteen patients (72%) experienced recurrence. Recurrence was often limited to RPN (27%) or liver (22%). Four patients underwent a second surgery for RPN recurrence. Median disease-free and overall survival were 22 months and 50 months after RPN surgery. We did not find any factor associated with recurrence. Short-term recurrence (< 6 months) was associated with shorter overall survival (0.031). CONCLUSION: The current results suggest that RPN resection is feasible and associated with long survival in selected patients. Further studies evaluating the benefit of curative strategies including radical surgery for patients with potentially resectable RPN are warranted.

Discordance of retroperitoneal and thoracic histologic findings in patients with metastatic germ cell tumors at postchemotherapy residual tumor resection.

INTRODUCTION AND OBJECTIVES: Postchemotherapy residual tumor resection (PC-RTR) is an important part of the multimodal treatment for patients with metastatic germ cell tumors. Simultaneous retroperitoneal and thoracic metastases often require consecutive surgical procedures. This study analyzes the histologic findings after abdominal and thoracic surgery in order to tailor the sequence and intensity of surgery. PATIENTS AND METHODS: From a total of 671 PC-RTRs from 2008 to 2021 we analyzed 50 patients with stage III non-seminomatous germ cell tumor (NSGCT) who had undergone both retroperitoneal and thoracic postchemotherapy residual tumor resection after first-line and salvage chemotherapy. RESULTS: All patients included had stage III NSGCT. 39 and 11 patients received first-line and salvage chemotherapy, respectively. 45 (90%) patients received retroperitoneal resection first, followed by thoracic surgery. Three patients (6%) underwent thoracic surgery before retroperitoneal surgery and two patients (4%) underwent simultaneous surgery. Overall, the histology of retroperitoneal and thoracic specimens was discordant in 23% of cases. After first-line chemotherapy, of fourteen patients with necrosis in retroperitoneal histology, four patients had vital carcinoma in lung histology. In patients with teratoma in the retroperitoneum, the thoracic findings were concordant in most cases (78%). When teratomatous elements were also present in the orchiectomy specimen, concordance was 100%. After salvage chemotherapy, the discordance rate was 55%. CONCLUSION: The data presented in this study underline that retroperitoneal residual masses with necrosis cannot reliably predict histologic findings of thoracic specimens. Patients with teratoma in the retroperitoneum have a high likelihood of teratoma in the thoracic specimen.

Optimizing surgical strategies for retroperitoneal liposarcoma: a comprehensive evaluation of standardized aggressive surgical policies.

BACKGROUND: Retroperitoneal liposarcoma (RLPS) constitutes the majority of retroperitoneal sarcomas. While surgical resection remains the sole curative approach, determining the optimal surgical strategy for RLPS remains elusive. This study addresses the ongoing debate surrounding the optimal surgical strategy for RLPS. METHODS: We recruited 77 patients with RLPS who underwent aggressive surgical policies. Patients were categorized into three surgical subtypes: suprapancreatic RLPS, pancreatic RLPS, and subpancreatic RLPS. Our standardized surgical strategy involved resecting macroscopically uninvolved adjacent organs according to surgical subtypes. We collected clinical, pathological and prognostic data for analyses. RESULTS: The median follow-up was 45.5 months. Overall survival (OS) and recurrence-free survival (RFS) were significantly correlated with multifocal RLPS, pathological subtype, recurrent RLPS and histological grade (P for OS = 0.011, 0.004, 0.010, and < 0.001, P for RFS = 0.004, 0.001, < 0.001, and < 0.001, respectively). The 5-Year Estimate OS of well-differentiated liposarcoma (WDLPS), G1 RLPS, de novo RLPS and unifocal RLPS were 100%, 89.4%, 75.3% and 69.1%, respectively. The distant metastasis rate was 1.4%. The morbidity rates (≥ grade III) for suprapancreatic, pancreatic, and subpancreatic RLPS were 26.7%, 15.6%, and 13.3%, respectively. The perioperative mortality rate is 2.6%. CONCLUSIONS: Standardized aggressive surgical policies demonstrated prognostic benefits for RLPS, particularly for G1 RLPS, WDLPS, unifocal RLPS, and de novo RLPS. This approach effectively balanced considerations of adequate exposure, surgical safety, and thorough removal of all fat tissue. G1 RLPS, WDLPS, unifocal RLPS, and de novo RLPS could be potential indications for aggressive surgical policies.