Initial local resection versus early referral for spermatic cord sarcoma: Any impact on treatment timelines and oncological outcomes?

OBJECTIVES: Spermatic cord sarcomas are exceedingly rare, often misdiagnosed and subsequently improperly treated at local hospitals. This retrospective study looked at the oncological outcomes of spermatic cord sarcoma cases managed with curative intent resection at a tertiary referral sarcoma centre. We specifically studied how initial inadequate resections impact the oncologic outcomes compared to primary tumour resections at the reference centre. METHODS: One hundred eighteen consecutive patients affected by primary, localized spermatic cord sarcoma surgically managed at our reference centre from January 2001 through January 2021 were included. Primary endpoints were local relapse free (LRFS), distant metastasis free (DMFS) and overall survival (OS). These outcomes were evaluated with multi-nomial logistic regression and Cox proportional hazards regression models for a co-relation to known patient, tumour and treatment-related prognostic factors, including a prior inadequate resection and time from diagnosis to a complete oncologic resection as independent variables. Secondarily, we compared the above variables and treatment intervals among the subgroups of primary versus re-resection surgery. RESULTS: Over a median follow-up of 54 months (IQR 25-105), 12 patients (10.2%) developed local recurrence (LR) and 14 (11.6%) had distant metastasis (DM). 5-year local relapse (LRFS) and distant metastasis-free survival (DMFS) were 89.3% and 86.5%, respectively. Higher tumour grade and size were associated with a worse DMFS (p=<0.05). Likewise, marginal (R1) resection correlated with an inferior LRFS (p=< 0.05). Eighty-four patients (71.2%) had their initial diagnosis established on an inadequate surgical excision performed in a local hospital, followed by a re-excision at our centre (Re-resection group). During the same period, 34 (28.8%) were managed primarily with biopsy and treatment at our reference centre (Primary-resection group). The two groups had statistically significant differences in tumour size, histopathology, surgery duration, rate of postoperative complication and R0 resection (p < 0.005). Additionally, the difference in time intervals to achieve the treatment targets was statistically insignificant and did not correlate to the risk of recurrence as an independent variable. Residual disease was present in 51.2 % (n = 43) of the re-excision specimens. However, following a complete R0 resection, this did not correlate with a higher risk of recurrence (p = 0.481). CONCLUSION: Prompt referral to a tertiary centre, where multidisciplinary evaluation and sound oncologic resections are the standard of treatment, can align the OS and DFS of patients receiving incomplete surgery elsewhere to those treated primarily in referral centres. The primary determinant of prognosis remains surgical margin, tumour size and grade.

Learning curve of laparoscopic cholecystectomy: a risk-adjusted cumulative summation (RA-CUSUM) analysis of six general surgery residents.

BACKGROUND: Laparoscopic cholecystectomy (LapC) is one of the most frequently performed surgical procedures worldwide. Reaching technical competency in performing LapC is considered one essential task for young surgeons. Investigating the learning curve for LapC (LC-LapC) may provide important information regarding the learning process and guide the training pathway of residents, improving educational outcomes. The present study aimed to investigate LC-LapC among general surgery residents (GSRs). METHODS: Operative surgical reports of consecutive patients undergoing LapC performed by GSRs attending the General Surgery Residency Program at the University of Milan were analysed. Data on patient- and surgery-related variables were obtained from the ICD-9-CM diagnosis codes and gathered. A multidimensional assessment of the LC was performed through Cumulative Sum (CUSUM) and Risk-Adjusted (RA)-CUSUM analysis. RESULTS: 340 patients operated by 6 GSRs were collected. The CUSUM and RA-CUSUM graphs based on surgical failures allowed to distinguish two defined phases for all GSRs: an initial phase ending at the peak, so-called learning phase, followed by a phase in which there was a significant decrease in failure incidence, so-called proficiency phase. The learning phase was completed for all GSRs at most within 25 procedures, but the trend of the curves and the number of procedures needed to achieve technical competency varied among operators ranging between 7 and 25. CONCLUSIONS: The present study suggested that at most 25 procedures might be sufficient to acquire technical competency in LapC. The variability in the number of procedures needed to complete the LC, ranging between 7 and 25, could be due to the heterogeneous scenarios in which LapC was performed, and deserves to be investigated through a prospective study involving a larger number of GSRs and institutions.

Risk Factors for the Development of Early Recurrence in Patients with Primary Retroperitoneal Sarcoma.

BACKGROUND: Disease recurrence after retroperitoneal sarcoma (RPS) surgery is common, and resection may offer no benefit for patients who experience recurrence early. This study examined the incidence of early recurrence (EREC) in RPS patients, and the association between EREC and prognosis, aiming to identify the factors associated with EREC. METHODS: Patients undergoing surgery for primary RPS from 2008 to 2019 at two tertiary RPS centers were analyzed. The study defined EREC as any evidence of local recurrence and/or distant metastases on the CT scan up to 6 months after surgery. Overall survival (OS) was calculated using the Kaplan-Meier method. A multivariable analysis was performed to identify independent predictors of EREC. RESULTS: Of the 692 patients who underwent surgery during the study period, 657 were included in the analysis. Sixty-five of these patients (9.9%; 95% confidence interval [CI], 7.7-12.4%) developed EREC. Five-year OS was 3% for the patients with EREC versus 76% for those without EREC (p < 0.001). Patient characteristics were compared between the EREC and non-EREC patients, and EREC was found to be significantly associated with Eastern Cooperative Oncology Group (ECOG) performance status (p = 0.006), tumor histology (p = 0.002), tumor grading (p < 0.001), radiotherapy (p = 0.04), and postoperative complications measured as a comprehensive complications index value (p = 0.003). However, the only significant independent predictor of EREC in the multivariable analysis was grade 3 tumors, with an odds ratio of 14.8 (95% CI, 4.44-49.2; p < 0.001). CONCLUSION: Early recurrence is associated with a poor prognosis, and a high tumor grade is an independent predictor for the development of EREC. Patients with EREC may benefit the most from new therapeutic options such as neoadjuvant chemotherapy.

Six Surgical Stages in the Resection of Primary Right Retroperitoneal Liposarcoma: A Standardized Comprehensive Approach.

BACKGROUND: Surgery is the treatment mainstay in retroperitoneal sarcoma (RPS), a frontline comprehensive approach based on tumor removal en bloc with adherent viscera is mandatory especially for liposarcoma, where the normal retroperitoneal fat is undistinguishable from the well-differentiated tumor component.1-5 In this video, a reproducible and standardized six-stage approach to a primary right retroperitoneal liposarcoma is presented. PATIENT AND METHODS: A 23-cm right retroperitoneal, well-differentiated liposarcoma was diagnosed in a 68-year-old female patient in December 2021. The tumor involved the right kidney and adrenal gland; displacing anteriorly the right colon, the duodenum, and the pancreatic head; and invading part of the ipsilateral psoas muscle. After the publication of the STRASS trial and STREXIT results,6,7 neoadjuvant radiotherapy was delivered to a total dose of 50.4 Gy in 28 fractions with stable disease. Virtual 3D reconstruction of regional anatomy by Visible Patient was performed preoperatively. RESULTS: The patient underwent right retroperitoneal mass resection en bloc with ipsilateral kidney and adrenal gland, colon, psoas muscle, and portion of ipsilateral diaphragm. Of note, the resection of the psoas muscle was performed to obtain a safe posterior margin and accomplish a better clearance of fat of the posterior abdominal wall. This can be limited to the psoas fascia whenever the tumor is not adherent to it. A six-stage approach was performed, as described in the supplementary video file. CONCLUSIONS: RPS resection is complex and requires a broad range of surgical expertise. A staged approach that can be followed in virtually all cases is highly recommended to achieve an optimal tumor resection.

Neoadjuvant Chemotherapy for Intrahepatic, Perihilar, and Distal Cholangiocarcinoma: a National Population-Based Comparative Cohort Study.

INTRODUCTION: Data supporting the utilization of neoadjuvant chemotherapy (NAC) in patients receiving resection for cholangiocarcinoma (CCA) remains uncertain. We aimed to determine whether NAC followed by resection improves long-term survival in intrahepatic (iCCA), perihilar (hCCA), and distal (dCCA) cholangiocarcinoma, analyzed separately. METHODS: Patients undergoing surgery for iCCA, hCCA, and dCCA, receiving either none, NAC, or adjuvant chemotherapy (AC) from 2010 to 2016 were identified from the National Cancer Database (NCDB). Cox regression was performed to account for selection bias and to assess the impact of surgery alone (SA) versus either NAC or AC on overall survival (OS). RESULTS: There were 9411 patients undergoing surgery for iCCA (n = 3772, 39.5%), hCCA (n = 1879, 20%), and dCCA (n = 3760, 40%). Of these, 10.6% (n = 399), 6.5% (n = 123), and 7.2% (n = 271) with iCCA, hCCA, and dCCA received NAC, respectively. On adjusted analyses, patients receiving NAC followed by surgery had significantly improved OS, compared to SA for iCCA (HR 0.75, CI95% 0.64-0.88, p < 0.001), hCCA (HR 0.72, CI95% 0.54-0.97, p = 0.033), and for dCCA (HR 0.65, CI95% 0.53-0.78, p < 0.001). However, sensitivity analyses demonstrated no differences in OS between NACs, followed by surgery or AC after surgery in iCCA (HR 1.19, CI95% 0.99-1.45, p = 0.068), hCCA (HR 0.83 CI95% 0.59-1.19, p = 0.311), and dCCA (HR 1.13 CI95% 0.91-1.41, p = 0.264). CONCLUSIONS: This study associated NAC with increased OS for all CCA subtypes, even in patients with margin-negative and node-negative disease; however, no differences were found between NAC and AC. Our results highlight that a careful and interdisciplinary evaluation should be sought to consider NAC in CCA and warrant the need of larger studies to provide robust recommendation.

Follow-up of patients with retroperitoneal sarcoma.

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.

Contemporary role of amputation for patients with extremity soft tissue sarcoma.

INTRODUCTION: limb-sparing surgery is the mainstream treatment for primary extremity soft tissue sarcoma (ESTS) at referral centers, following advances in surgical reconstructions and multimodal management. However, amputation is still needed in selected patients and has not yet been described for a ESTS cohort in a contemporary scenario. MATERIAL AND METHODS: consecutive patients who underwent surgery for primary ESTS from 2006 to 2018 were extracted from a prospectively collected database at our reference center. Patients receiving amputation for either primary tumor or local recurrence (LR) after limb-sparing surgery were selected for analysis. RESULTS: Among 1628 primary ESTS, 29 patients underwent primary amputation (1.8%), 22/1159 (1.9%) for upper limb and 7/469 (1.5%) for lower limb ESTS. Patients were mainly affected by grade III FNCLCC (89.6%) of notable dimension (median size 16 cm, IQR 10-24). 65.5% of patients received preoperative treatments (systemic or regional chemotherapy, radiotherapy or chemo-radiation). Secondary amputation for LR was performed after a median of 23 months in 16/1599 patients (1%). Median survival time was 16.2 and 29.6 months after primary or secondary amputation respectively. Factors prompting the need for a primary amputation were most often a combination of multifocal disease, bone invasion and pain or neurovascular bundle involvement and relevant comorbidities, mainly for grade III tumors in elderly patients. CONCLUSION: Contemporary rate of amputation for ESTS at a reference center is extremely low. Still, amputation is required in selected cases with advanced presentations, especially in elderly, frail patients.

Dealing with malignancy involving the inferior vena cava in the 21st century.

INTRODUCTION: Malignancies involving the inferior vena cava (IVC) have historically been considered not amendable to surgery. More recently, involvement of the IVC by neoplastic processes in the kidney, liver or in the retroperitoneum can be managed successfully. EVIDENCE ACQUISITION: In this systematic review we summarize the current evidence regarding the surgical management of the IVC in cases of involvement in neoplastic processes. Current literature was searched, and studies selected on the base of the PRISMA guidelines. Evidence was synthesized in narrative form due to heterogeneity of studies. EVIDENCE SYNTHESIS: Renal cell carcinoma accounts for the greatest proportion of studied patients and can be managed with partial or complete vascular exclusion of the IVC, thrombectomy and direct closure or patch repair with good oncological prognosis. Hepatic malignancies or metastases may involve the IVC, and the joint expertise of hepatobiliary and vascular surgeons has developed various strategies, according to the location of tumor and the need to perform a complete vascular exclusion above the hepatic veins. In retroperitoneal lymph node dissection, the IVC can be excised en-block to guarantee better oncological margins. Also, in retroperitoneal sarcomas not arising from the IVC a vascular substitution may be required to improve the overall survival by clearing all the neoplastic cells in the retroperitoneum. Leiomyoma can have a challenging presentation with involvement of the IVC requiring either thrombectomy, partial or complete substitution, with good oncological outcomes. CONCLUSIONS: A multidisciplinary approach with specialist expertise is required when dealing with IVC involvement in surgical oncology. Multiple techniques and strategies are required to deliver the most efficient care and achieve the best possible overall survival. The main aim of these procedures must be the complete clearance of all neoplastic cells and achievement of a safe margin according to the perioperative treatment strategy.

Emergency multivisceral resection for spontaneous haemorrhage rupture of huge solid pseudopapillary neoplasm of the pancreas during pregnancy†.

Solid pseudopapillary neoplasm (SPN) of the pancreas is a rare malignancy with a low malignant potential and strong female preponderance. Diagnosis during pregnancy is extraordinary, and management must consider the risks to the mother and foetus of tumour growth and rupture. A large 35-cm SPN was identified on magnetic resonance imaging (MRI) in a 24-year-old woman at 6 weeks of gestation following presentation with an abdominal mass. Surgery was delayed to allow the foetus to reach as close to term as possible because surveillance MRIs showed incremental mass growth. Emergency c-section was undertaken at 35 weeks of gestation due to persistent tachycardia and suspected haemorrhage into the tumour. A Hb of 70 g/l post-delivery despite four units of RBCs and an albumin of 11 g/l necessitated urgent multivisceral surgery. Surgical resection is the mainstay of treatment for SPN. However, the strategy of choice during pregnancy remains undetermined, with more recent reports delaying surgery until post-partum.

Retroperitoneal and Mesenteric Liposarcomas.

Retroperitoneal liposarcomas are a rare entity and are comprised mostly of the well-differentiated and dedifferentiated subtypes. Eight-year survival ranges from 30% to 80% depending on histologic subtype and grade. Surgery is the cornerstone of treatment and compartment resection is the current standard. Mesenteric liposarcomas are extremely rare and comprise more high-grade lesions, with poorer prognosis of 50% 5-year overall survival. They are managed with a similar aggressive surgical approach. This review presents the current management of retroperitoneal and mesenteric liposarcomas.

Overall survival in abdominal wall sarcoma treated with curative intent surgery: A retrospective study from two European referral centres.

INTRODUCTION: Primary abdominal wall sarcomas are rare, heterogeneous tumours. The mainstay of management is surgery, although local recurrences (LR) and distant metastases (DM) are common. OBJECTIVES: Overall survival (OS) and disease-free survival (DFS) were primary outcomes; factors associated with prognosis secondary outcomes. MATERIALS AND METHODS: Patients undergoing surgery of primary abdominal wall sarcomas between April 2008 and May 2018 were identified at two referrals centres for sarcoma surgery. Patient demographics, tumour and treatment-related characteristics were recorded and analysed. RESULTS: A cohort of 65 patients underwent surgical resection with a median follow-up of 56 months, 5-year OS and DFS were 69% and 71%, respectively. Eleven patients (16.9%) experienced a recurrence event: 6 LR (9.2%), 10 DM (15.4%) and 5 both (7.7%). At univariate analysis, size (p = 0.03), grade (p = 0.001) and depth (p = 0.04) were associated with OS while size (p = 0.02) was associated with DFS. No significant relationship with tumour depth, type of surgery, surgical margin status or neo-/adjuvant treatment was demonstrated. CONCLUSION: Recurrence events are less common following treatment of abdominal wall sarcomas if compared to extremities STSs, but size (≥5 cm), high malignancy grade (FNCLCC 3) and depth are associated with worse OS.

Residual Adrenal Function After Multivisceral Resection With Adrenalectomy in Adult Patients.

Importance: The risk of developing adrenal insufficiency (AI) following adrenalectomy has been insufficiently studied in the context of multivisceral resection (MVR). Objective: To evaluate the incidence of AI in patients undergoing MVR with en bloc adrenalectomy. Design, Setting, and Participants: Prospective observational longitudinal study in a single referral center including 56 consecutive adult patients undergoing retroperitoneal sarcoma surgery from June 2019 to August 2020. Those who were candidates for MVR with en bloc adrenalectomy and had no preexisting adrenal impairment were considered eligible. Of these, 4 individuals were excluded because they did not receive adrenalectomy at the time of surgery and 2 because they were not considered evaluable for the main end point. Follow-up was set at 4 months after surgery, and 49 patients completed follow-up. Data were analyzed from October 2020 to September 2021. Exposures: Diagnosis of AI was determined by low-dose (1 µg) adrenocorticotropic hormone (ACTH) stimulation test with a threshold of 20 µg/dL in blood samples retrieved 30 and 60 minutes after stimulation. ACTH test was repeated on postoperative days 1 and 10 and at 4 months' follow-up. Main Outcome and Measures: The primary end point was incidence and relevance of AI after MVR. Secondary end points were associations with patient- and tumor-related factors, impact on perioperative hemodynamic management, and association with postoperative morbidity and mortality. Results: Fifty patients (26 female; median [IQR] age, 59 [46-67] years) were evaluable. Incidence of AI was 64% (32 of 50 patients) in the early postoperative period and 38.5% (15 of 39 patients) at follow-up. Patients with AI showed lower postoperative cortisol values. Factors associated with risk of AI at univariate analysis were high American Society of Anesthesiologists score (odds ratio [OR], 0.31; 95% CI, 0.14-0.48) and high malignancy grade (OR, 0.35; 95% CI, 0.24-0.46). Clinical outcomes not associated with AI included morbidity, mortality, reoperation rate, admission to intensive care unit, length of intensive care unit stay, total hospital stay, and long-term quality of life. Conclusions and Relevance: In this study, AI after MVR with en bloc adrenalectomy was frequent, even in patients with adequate preoperative adrenal function. Despite this, adrenalectomy can be safely performed. Patients at risk should be monitored in the long term to exclude underrated impairment of adrenal function.

Delayed Gastric Emptying After Multivisceral Resection for Retroperitoneal Sarcoma.

INTRODUCTION: Delayed gastric emptying (DGE) is a common complication in surgery, but incidence and relevance following multivisceral resection are unknown. METHODS: Data from 100 consecutive patients treated for primary retroperitoneal sarcoma (RPS) were analyzed from our institutional prospectively maintained database from January 2019 to April 2020. DGE severity was graded according to the International Study Group of Pancreatic Surgery and classified as primary or secondary to other complications. The primary outcome was incidence and grade of clinically relevant DGE (grades B-C). Secondary outcomes were correlation with patient, tumor, and treatment characteristics, and non-DGE morbidity [Clavien-Dindo (CD) grade ≥ 3]. RESULTS: Forty-two patients developed DGE and 28 had clinically relevant DGE. DGE was primary in 10 patients and secondary in 18 patients; the most common associated complications were: infections (11/18, 61.1%), pancreatic leak (7/18, 38.9%), bleeding (6/18, 33.3%), and bowel leak (6/18, 33.3%). DGE was related to longer length of hospital stay (P < 0.001), ICU admission (P = 0.004), ICU length of stay (P = 0.001), postoperative complications (CD [Formula: see text] 3 in 14/28 in DGE patients vs 11/72 in no-DGE; P = 0.04), and re-operation (P = 0.03). With multivariate analysis, the independent risk factors for DGE were patient comorbidities (OR 1.05; 95% CI 1.01-1.1; P = 0.04) and tumor size (OR 1.05; 95% CI 1.0-1.1; P = 0.02). DISCUSSION: Following multivisceral resection, DGE is a clinically relevant event that can be caused by an underlying complication. Prompt diagnosis and treatment of both DGE and any underlying complications led to full recovery in all cases.

Dedifferentiation within well-differentiated liposarcoma of the extremity or trunk: Implications for clinical management.

BACKGROUND: In extremity or trunk liposarcoma, the implications of a dedifferentiated (DD) component within a well-differentiated (WD) tumor are unclear. We evaluated outcomes after surgery and identified potential predictors of survival in these patients compared to those with an entirely WD tumor. METHODS: Retrospective data were collected for patients who underwent complete resection from 2009 to 2019. Cumulative incidences of local recurrence (LR) and distant metastasis (DM) were calculated, and overall survival (OS) was estimated. Associations between OS and clinicopathologic variables were evaluated by univariable models. RESULTS: A total of 210 patients with MDM2-verified tumors were studied, including 58 (27.6%) with DD. In primary disease, LR occurred only in DD and worse OS was observed versus WD (p < 0.001). In recurrent disease, the LR incidences were similar between WD and DD (p = 0.559); however, worse OS persisted in DD (p = 0.004). The incidence of DM was extremely low (3.8%) and limited to DD. Higher grade (p < 0.001) and DD size (p = 0.043), but not overall tumor size were associated with worse OS. CONCLUSIONS: In extremity or trunk liposarcoma, the presence of DD leads to significantly worse outcomes in both primary and recurrence diseases. Further study is needed to determine if these patients benefit from adjunct therapies (e.g., radiation).

Clinicopathological features and treatment outcome of oesophageal gastrointestinal stromal tumour (GIST): A large, retrospective multicenter European study.

BACKGROUND: Oesophageal gastrointestinal stromal tumours (GISTs) account for ≤1% of all GISTs. Consequently, evidence to guide clinical decision-making is limited. METHODS: Clinicopathological features and outcomes in patients with primary oesophageal GIST from seven European countries were collected retrospectively. RESULTS: Eighty-three patients were identified, and median follow up was 55.0 months. At diagnosis, 59.0% had localized disease, 25.3% locally advanced and 13.3% synchronous metastasis. A biopsy (Fine Needle aspiration n = 29, histological biopsy n = 31) was performed in 60 (72.3%) patients. The mitotic count was low (<5 mitoses/50 High Power Fields (HPF)) in 24 patients and high (≥5 mitoses/50 HPF) in 27 patients. Fifty-one (61.4%) patients underwent surgical or endoscopic resection. The most common reasons to not perform an immediate resection (n = 31) were; unresectable or metastasized GIST, performance status/comorbidity, patient refusal or ongoing neo-adjuvant therapy. The type of resections were enucleation (n = 11), segmental resection (n = 6) and oesophagectomy with gastric conduit reconstruction (n = 33), with median tumour size of 3.3 cm, 4.5 cm and 7.7 cm, respectively. In patients treated with enucleation 18.2% developed recurrent disease. The recurrence rate in patients treated with segmental resection was 16.7% and in patients undergoing oesophagectomy with gastric conduit reconstruction 36.4%. Larger tumours (≥4.0 cm) and high (>5/5hpf) mitotic count were associated with worse disease free survival. CONCLUSION: Based on the current study, enucleation can be recommended for oesophageal GIST smaller than 4 cm, while oesophagectomy should be preserved for larger tumours. Patients with larger tumours (>4 cm) and/or high mitotic count should be treated with adjuvant therapy.

Inoperable Primary Retroperitoneal Sarcomas: Clinical Characteristics and Reasons Against Resection at a Single Referral Institution.

BACKGROUND: The outcome of patients with retroperitoneal sarcomas (RPS) depends mainly on tumor biology and completeness of surgical resection. However, some patients are deemed not resectable for various reasons. This study analyzed a series of primary RPS patients to describe rate and reasons of primary inoperability at a large referral center. METHODS: All consecutive patients affected by primary localized RPS referred for surgical treatment at our institution between January 1, 2013 and December 31, 2017 were analyzed. Patients were split in two groups: those who underwent surgical resection with curative intent, and those who were not resected. RESULTS: A total of 322 patients were available for the current analysis: 285 (88.5%) underwent resection with curative intent, and 37 (11.5%) did not. Twenty of 322 (6.2%) patients who did not undergo resection had a technically unresectable tumor, whereas the remaining 18 of 322 (5.6%) were not amenable to a major surgical procedure due to comorbidities/poor performance status. The dominant technical reason was involvement of the celiaco-mesenteric vessels. At a median follow-up from the diagnosis of 34 months, 24 of 37 (64.9%) nonoperated and 48 of 285 (16.8%) operated patients died. The corresponding 4-year overall survival were 10.3% and 83.4%, respectively (p < 0.001). CONCLUSIONS: Roughly, 10% of patients who presented with localized primary RPS at a large referral institution were not resected. An attempt to standardize the definition of resectability for primary localized RPS should be made considering anatomic, biologic, and patient-related factors.