New Frontiers in the Management of Retroperitoneal Soft Tissue Sarcoma

A special issue of Current Oncology (ISSN 1718-7729). This special issue belongs to the section "Bone and Soft Tissue Oncology".

Deadline for manuscript submissions: closed (30 November 2022) | Viewed by 40203

Special Issue Editor


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Guest Editor
Division of General Surgery, The Ottawa Hospital, Ottawa, ON, Canada
Interests: melanoma; soft tissue sarcoma; GIST; gastric cancer; database research

Special Issue Information

Dear Colleagues,

Soft tissue sarcomas are a rare and heterogeneous group of tumors with more than 100 histological subtypes and comprise only 1% of all adult cancers. They are usually caused by a variety of factors, such as exposure to chemical compounds, radiation, infections, and genetic diseases. Sarcomas can be divided into two large families: sarcomas of the soft tissues (smooth and striated muscles, adipose tissue, nerves, etc.) and sarcomas of the bone. They can occur anywhere in the body, but mainly in the extremities and the retroperitoneum. This Special Issue will focus on soft tissue sarcomas in the abdomen, pelvis, and retroperitoneum. Different histological subtypes have different biological behaviors and patterns of spread. The management of this disease requires a multidisciplinary team approach consisting of a wide range of members, such as medical and radiation oncologists, surgical oncologists, pathologists, nuclear medicine specialists, radiologists, and molecular biologists. The sarcoma mortality rate is relatively high: the global survival after 5 years is around 65%. In recent years, there has been an improvement in survival due to the improvement of diagnostic tools and better knowledge about the biology of these cancers and surgical techniques. Less defined is the role of chemotherapy and radiotherapy in modifying prognosis both in the adjuvant and metastatic setting.

In this Special Issue, we invite original studies and review articles on the diagnosis, biology, staging, and nomograms, genetic and epigenetic aspects, epidemiology, and novel therapeutic options of sarcomas (surgery, medical oncology, and radiation). This Special Issue also encourages papers evaluating aspects related to the quality of life of patients with sarcomas and any therapies and/or support procedures to improve patients’ quality of life.

Dr. Carolyn Nessim
Guest Editor

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Keywords

  • sarcoma
  • soft tissue sarcoma
  • retroperitoneal sarcoma
  • diagnosis
  • treatment
  • pathology
  • surveillance
  • surgery
  • chemotherapy
  • radiation

Published Papers (14 papers)

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Research

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9 pages, 231 KiB  
Article
Data Dissemination of the Role of Neoadjuvant Radiation in Retroperitoneal Sarcoma: A CTOS and CSSO Survey
by Sarah Corn, Carolyn Nessim, Christina L. Roland, Alessandro Gronchi, Carolyn Freeman and Sinziana Dumitra
Curr. Oncol. 2023, 30(6), 5807-5815; https://doi.org/10.3390/curroncol30060434 - 14 Jun 2023
Viewed by 1572
Abstract
Consensus guidelines call for complete resection of retroperitoneal sarcoma with consideration of neoadjuvant radiation for curative-intent treatment. The 15-month delay from the initial presentation of an abstract to the final publication of the STRASS trial results assessing the impact of neoadjuvant radiation led [...] Read more.
Consensus guidelines call for complete resection of retroperitoneal sarcoma with consideration of neoadjuvant radiation for curative-intent treatment. The 15-month delay from the initial presentation of an abstract to the final publication of the STRASS trial results assessing the impact of neoadjuvant radiation led to a dilemma of how patients should be managed in the interim. This study aims to (1) understand perspectives regarding neoadjuvant radiation for RPS during this period; and (2) assess the process of integrating data into practice. A survey was distributed to international organizations including all specialties treating RPS. Eighty clinicians responded, including surgical (60.5%), radiation (21.0%) and medical oncologists (18.5%). Low kappa correlation coefficients on a series of clinical scenarios querying individual recommendations before and after initial presentation as an abstract indicate considerable change. Over 62% of respondents identified a practice change; however, most also noted discomfort in adopting changes without a manuscript available. Of the 45 respondents indicating discomfort with practice changes without a full manuscript, 28 (62%) indicated that their practice changed in response to the abstract. There was substantial variability in recommendations for neoadjuvant radiation between the presentation of the abstract and the publication of trial results. The difference in the proportion of clinicians describing comfort with changing practice based on the presentation of the abstract versus those that had done so shows that indications for proper integration of data into practice are not clear. Endeavors to resolve this ambiguity and expedite availability of practice-changing data are warranted. Full article
19 pages, 1758 KiB  
Article
A Case Series of Metastatic Malignant Gastrointestinal Neuroectodermal Tumors and Comprehensive Genomic Profiling Analysis of 20 Cases
by Taylor Kandler, Eliane Cortez, Lani Clinton, Amanda Hemmerich, Osama Ahmed, Ralph Wong, Taylor Forns, Andrea J. MacNeill, Trevor D. Hamilton, Mohammadali Khorasani and Xiaolan Feng
Curr. Oncol. 2022, 29(2), 1279-1297; https://doi.org/10.3390/curroncol29020109 - 21 Feb 2022
Cited by 12 | Viewed by 3202
Abstract
Malignant gastrointestinal neuroectodermal tumor (GNET) is an ultra-rare soft tissue sarcoma, therefore often misdiagnosed and has no available standard treatment. Here, we report 3 cases of metastatic GNET with variable clinical courses. Our small case series as well as extensive literature review, further [...] Read more.
Malignant gastrointestinal neuroectodermal tumor (GNET) is an ultra-rare soft tissue sarcoma, therefore often misdiagnosed and has no available standard treatment. Here, we report 3 cases of metastatic GNET with variable clinical courses. Our small case series as well as extensive literature review, further support that GNET is a spectrum of diseases with variable inherent biology and prognosis. Surgical management in the setting of recurrent/metastatic disease may be appropriate for GNET with indolent nature. Response to systemic treatments including chemotherapy and targeted treatments is variable, likely related to heterogenous biology as well. Furthermore, we retrospectively identified 20 additional GNET cases from Foundation Medicine’s genomic database and expanded on their clinicopathological and genomic features. Comprehensive genomic profiling (CGP) with DNA and RNA sequencing of this cohort, in the course of clinical care, demonstrated recurrent EWSR1 chromosomal rearrangements and a sparsity of additional recurrent or driver genomic alterations. All cases had low tumor mutational burden (TMB) and were microsatellite stable. Full article
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Review

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20 pages, 1459 KiB  
Review
Practical Management of Adult Ultra-Rare Primary Retroperitoneal Soft Tissue Sarcoma: A Focus on Perivascular Epithelioid Tumours and Extraosseous Ewing Sarcoma
by Sameer S. Apte, Eyal Mor, Catherine Mitchell and David E. Gyorki
Curr. Oncol. 2023, 30(7), 5953-5972; https://doi.org/10.3390/curroncol30070445 - 21 Jun 2023
Cited by 1 | Viewed by 2137
Abstract
With the exception of well-differentiated liposarcoma, dedifferentiated liposarcoma, leiomyosarcoma, solitary fibrous tumour, malignant peripheral nerve sheath tumour, and undifferentiated pleomorphic sarcoma, the majority of the ≈70 histologic subtypes of retroperitoneal sarcoma are defined as ‘ultra-rare’ sarcomas, with an incidence of ≤1–5/1,000,000 persons/year. For [...] Read more.
With the exception of well-differentiated liposarcoma, dedifferentiated liposarcoma, leiomyosarcoma, solitary fibrous tumour, malignant peripheral nerve sheath tumour, and undifferentiated pleomorphic sarcoma, the majority of the ≈70 histologic subtypes of retroperitoneal sarcoma are defined as ‘ultra-rare’ sarcomas, with an incidence of ≤1–5/1,000,000 persons/year. For most of these ultra-rare RPS subtypes, diagnosis and treatment follows international guidelines for the management of more common RPS histologies, with en bloc surgical resection as the mainstay of curative treatment, and enrolment in clinical trials where possible. Because the treatment of RPS is heavily driven by histology, the surgeon must be familiar with specific issues related to the diagnosis and management of ultra-rare sarcoma subtypes. Expert radiological and surgeon reviews are required to differentiate similarly presenting tumours where surgery can be avoided (e.g., angiomyolipoma), or where upfront systemic therapy is indicated (e.g., extraosseous Ewing’s sarcoma). Thus, the management of all retroperitoneal sarcomas should occur at a sarcoma referral centre, with a multidisciplinary team of experts dedicated to the surgical and medical management of these rare tumours. In this focused review, we highlight how diagnosis and management of the ultra-rare primary RPS histologies of malignant perivascular epithelioid cell tumour (PEComa), extraosseous Ewing sarcoma (EES), extraosseous osteosarcoma (EOS), and rhabdomyosarcoma (RMS) critically diverge from the management of more common RPS subtypes. Full article
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11 pages, 290 KiB  
Review
The Role of Surgery in Oligometastatic Retroperitoneal Sarcoma
by Lindsay A. Janes and Christina V. Angeles
Curr. Oncol. 2023, 30(6), 5240-5250; https://doi.org/10.3390/curroncol30060398 - 24 May 2023
Viewed by 1577
Abstract
Retroperitoneal sarcomas are extremely rare, comprising <15% of primary sarcomas. Distant metastasis occurs in about 20% of cases, with pulmonary and hepatic metastasis as the most common sites of hematogenous spread. Although surgical resection is well established as the main treatment of localized [...] Read more.
Retroperitoneal sarcomas are extremely rare, comprising <15% of primary sarcomas. Distant metastasis occurs in about 20% of cases, with pulmonary and hepatic metastasis as the most common sites of hematogenous spread. Although surgical resection is well established as the main treatment of localized primary disease, there are limited guidelines for the surgical treatment of intra-abdominal and distant metastases. There are inadequate systemic treatment options for patients with metastatic sarcoma, thereby necessitating the consideration of surgical options in carefully selected patients. Key points to consider include tumor biology, patient fitness and co-morbidities, overall prognosis, and goals of care. Multidisciplinary sarcoma tumor board discussion for each case is an essential practice in order to deliver the best care to these patients. The purpose of this review is to summarize the published literature on the past and present role of surgery in the treatment of oligometastatic retroperitoneal sarcoma in order to inform the management of this difficult disease. Full article
14 pages, 3173 KiB  
Review
Surgical Management of Retroperitoneal Sarcoma
by Dana A. Dominguez, Sagus Sampath, Mark Agulnik, Yu Liang, Bao Nguyen, Vijay Trisal, Laleh G. Melstrom, Aaron G. Lewis, Isaac Benjamin Paz, Randall F. Roberts and William W. Tseng
Curr. Oncol. 2023, 30(5), 4618-4631; https://doi.org/10.3390/curroncol30050349 - 29 Apr 2023
Cited by 5 | Viewed by 4028
Abstract
Surgery is the cornerstone of treatment for retroperitoneal sarcoma (RPS). Surgery should be performed by a surgical oncologist with sub-specialization in this disease and in the context of a multidisciplinary team of sarcoma specialists. For primary RPS, the goal of surgery is to [...] Read more.
Surgery is the cornerstone of treatment for retroperitoneal sarcoma (RPS). Surgery should be performed by a surgical oncologist with sub-specialization in this disease and in the context of a multidisciplinary team of sarcoma specialists. For primary RPS, the goal of surgery is to achieve the complete en bloc resection of the tumor along with involved organs and structures to maximize the clearance of the disease. The extent of resection also needs to consider the risk of complications. Unfortunately, the overarching challenge in primary RPS treatment is that even with optimal surgery, tumor recurrence occurs frequently. The pattern of recurrence after surgery (e.g., local versus distant) is strongly associated with the specific histologic type of RPS. Radiation and systemic therapy may improve outcomes in RPS and there is emerging data studying the benefit of non-surgical treatments in primary disease. Topics in need of further investigation include criteria for unresectability and management of locally recurrent disease. Moving forward, global collaboration among RPS specialists will be key for continuing to advance our understanding of this disease and find more effective treatments. Full article
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13 pages, 940 KiB  
Review
Intra-Abdominal Desmoplastic Small Round Cell Tumor (DSRCT) and the Role of Hyperthermic Intraperitoneal Chemotherapy (HIPEC): A Review
by Sophie J. M. Reijers, Caroline C. H. Siew, Niels F. M. Kok, Charles Honoré and Winan J. van Houdt
Curr. Oncol. 2023, 30(4), 3951-3963; https://doi.org/10.3390/curroncol30040299 - 31 Mar 2023
Cited by 2 | Viewed by 2238
Abstract
Desmoplastic small round cell tumor is a very rare and highly aggressive soft tissue sarcoma, usually presenting with multiple intra-abdominal tumors in young males. Patients present with advanced disease and the overall survival is dismal. Multiple studies report relatively favorable outcomes with multimodal [...] Read more.
Desmoplastic small round cell tumor is a very rare and highly aggressive soft tissue sarcoma, usually presenting with multiple intra-abdominal tumors in young males. Patients present with advanced disease and the overall survival is dismal. Multiple studies report relatively favorable outcomes with multimodal treatment consisting of chemotherapy, surgery and radiotherapy. If resection is feasible, complete cytoreductive surgery is the cornerstone of surgical treatment. The benefit of hyperthermic intraperitoneal chemotherapy in addition to cytoreductive surgery is unclear, and few studies have evaluated this option. We sought to identify the role of hyperthermic intraperitoneal chemotherapy in patients with intra-abdominal desmoplastic small round cell tumor. Our review of the available literature revealed no clear survival benefit in performing hyperthermic intraperitoneal chemotherapy after cytoreductive surgery. Full article
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24 pages, 1434 KiB  
Review
Staging Systems and Nomograms for Soft Tissue Sarcoma
by Maria Danieli and Alessandro Gronchi
Curr. Oncol. 2023, 30(4), 3648-3671; https://doi.org/10.3390/curroncol30040278 - 26 Mar 2023
Cited by 7 | Viewed by 1866
Abstract
Reliable tools for prognosis prediction are crucially needed by oncologists so they can tailor individual treatments. However, the wide spectrum of histologies and prognostic behaviors of sarcomas challenges their development. In this field, nomograms could definitely better account for their granularity compared to [...] Read more.
Reliable tools for prognosis prediction are crucially needed by oncologists so they can tailor individual treatments. However, the wide spectrum of histologies and prognostic behaviors of sarcomas challenges their development. In this field, nomograms could definitely better account for their granularity compared to the more widely used AJCC/UICC TNM staging system. Nomograms are predictive tools that incorporate multiple risk factors and return a numerical probability of a clinical event. Since the development of the first nomogram in 2002, several other nomograms have been built, either general, site-specific, histology-specific, or both. Recently, some new “dynamic” nomograms and prognostic tools have been developed, allowing doctors to “recalculate” a patient’s prognosis by taking into account the time since primary surgery, the event history, and the potential time-dependent effect of covariates. Due to these new tools, prognosis prediction is no longer limited to the time of the first computation but can be adapted and recalculated based on the occurrence (or not) of any event as time passes from the first computation. In this review, we aimed to give an overview of the available nomograms for STS and to help clinicians in the process of selecting the best tool for each patient. Full article
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16 pages, 3025 KiB  
Review
Complex Vascular Resections for Retroperitoneal Soft Tissue Sarcoma
by Nicolas A. Devaud, Jean M. Butte, Juan C. De la Maza, Sebastian von Mühlenbrock Hugo and Kenneth Cardona
Curr. Oncol. 2023, 30(3), 3500-3515; https://doi.org/10.3390/curroncol30030266 - 20 Mar 2023
Cited by 5 | Viewed by 2259
Abstract
Retroperitoneal sarcomas (RPSs) are locally aggressive tumors that can compromise major vessels of the retroperitoneum including the inferior vena cava, aorta, or main tributary vessels. Vascular involvement can be secondary to the tumor’s infiltrating growth pattern or primary vascular origin. Surgery is still [...] Read more.
Retroperitoneal sarcomas (RPSs) are locally aggressive tumors that can compromise major vessels of the retroperitoneum including the inferior vena cava, aorta, or main tributary vessels. Vascular involvement can be secondary to the tumor’s infiltrating growth pattern or primary vascular origin. Surgery is still the mainstay for curing this disease, and resection of RPSs may include major vascular resections to secure adequate oncologic results. Our improved knowledge in the tumor biology of RPSs, in conjunction with the growing surgical expertise in both sarcoma and vascular surgical techniques, has allowed for major vascular reconstructions within multi-visceral resections for RPSs with good perioperative results. This complex surgical approach may include the combined work of various surgical subspecialties. Full article
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11 pages, 271 KiB  
Review
Surveillance Post Surgery for Retroperitoneal Soft Tissue Sarcoma
by John Whitaker, Carolyn Nessim, Max Almond and Samuel J. Ford
Curr. Oncol. 2023, 30(3), 2781-2791; https://doi.org/10.3390/curroncol30030211 - 26 Feb 2023
Cited by 2 | Viewed by 1652
Abstract
Complete en bloc surgical resection offers the best opportunity for the cure of primary retroperitoneal sarcomas (RPS). The potential for disease recurrence, in the form of both loco-regional recurrence and distant metastases, underpins the rationale for postoperative surveillance. There is a paucity of [...] Read more.
Complete en bloc surgical resection offers the best opportunity for the cure of primary retroperitoneal sarcomas (RPS). The potential for disease recurrence, in the form of both loco-regional recurrence and distant metastases, underpins the rationale for postoperative surveillance. There is a paucity of high-quality evidence underpinning follow-up for RPS patients, and most practice guidelines draw from expert opinion and evidence from soft tissue sarcomas of the extremities. The available observational retrospective data analysis has failed to demonstrate that high-intensity radiological surveillance improves the overall survival in patients. The lack of a robust evidence base has given rise to variations in approaches to post-operative surveillance strategies adopted by specialist centres managing RPS across the world. More high-quality prospective research is needed and planned to more clearly support surveillance approaches that balance oncologic outcomes, patient-centric care, and health service value. Risk stratification tools exist and are available for use in routine practice. Their use will likely support more individualised post-operative surveillance moving forward. Surveillance will likely be underpinned by serial radiological imaging for the medium term. However, developments in genomics offer hope for biomarkers such as ctDNA to impact patient care positively in the future and further support individualised patient care pathways. Full article
9 pages, 6609 KiB  
Review
Management of Recurrent Retroperitoneal Sarcoma
by Joshua S. Jolissaint, Chandrajit P. Raut and Mark Fairweather
Curr. Oncol. 2023, 30(3), 2761-2769; https://doi.org/10.3390/curroncol30030209 - 25 Feb 2023
Cited by 1 | Viewed by 1817
Abstract
Recurrence after resection of retroperitoneal sarcoma is common and varies by histological subtype. Pattern of recurrence is similarly affected by histology (e.g., well-differentiated liposarcoma is more likely to recur locoregionally, whereas leiomyosarcoma is more likely to develop distant metastases). Radiotherapy may provide effective [...] Read more.
Recurrence after resection of retroperitoneal sarcoma is common and varies by histological subtype. Pattern of recurrence is similarly affected by histology (e.g., well-differentiated liposarcoma is more likely to recur locoregionally, whereas leiomyosarcoma is more likely to develop distant metastases). Radiotherapy may provide effective locoregional control in limited circumstances and the data on the impact of chemotherapy are scant. Surgery for locally recurrent disease is associated with the greatest survival benefit; however, data are retrospective and from a highly selected subgroup of patients. Limited retrospective data have also suggested a survival association with the resection of limited distant metastases. Given the complexity of these patients, multidisciplinary evaluation at a high-volume sarcoma center is critical. Full article
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15 pages, 5792 KiB  
Review
The Landscape of Immunotherapy for Retroperitoneal Sarcoma
by Alicia A. Gingrich, Elise F. Nassif, Christina L. Roland and Emily Z. Keung
Curr. Oncol. 2023, 30(2), 2144-2158; https://doi.org/10.3390/curroncol30020165 - 9 Feb 2023
Cited by 2 | Viewed by 2558
Abstract
Significant multidisciplinary scientific effort has been undertaken to understand the heterogeneous family of neoplasms that comprise soft tissue sarcomas. Within this family of neoplasms, outcomes for retroperitoneal sarcomas (RPS) are currently limited given a lack of effective therapies. In this review, we focus [...] Read more.
Significant multidisciplinary scientific effort has been undertaken to understand the heterogeneous family of neoplasms that comprise soft tissue sarcomas. Within this family of neoplasms, outcomes for retroperitoneal sarcomas (RPS) are currently limited given a lack of effective therapies. In this review, we focus on immunotherapy and its relationship with the common RPS histologic subtypes. Although initial outcomes for RPS patients with immune checkpoint inhibition alone have been somewhat disappointing, subsequent analyses on histologies, the tumor microenvironment, sarcoma immune class, tumor infiltrating lymphocytes and genetic analysis for tumor mutational burden have yielded insight into the interplay between sarcomas and immunotherapy. Such approaches have all provided critical insight into the environment and characterization of these tumors, with targets for potential immunotherapy in future clinical trials. With this insight, molecularly tailored combination treatments for improving response rates and oncologic outcomes for RPS are promising. Full article
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12 pages, 462 KiB  
Review
Radiation Therapy for Retroperitoneal Sarcomas: A Strass-Ful Situation
by Ahsan S. Farooqi, B. Ashleigh Guadagnolo, Devarati Mitra and Andrew J. Bishop
Curr. Oncol. 2023, 30(1), 598-609; https://doi.org/10.3390/curroncol30010047 - 3 Jan 2023
Cited by 1 | Viewed by 4443
Abstract
Locoregional recurrence (LRR) is the predominant pattern of relapse and often the cause of death in patients with retroperitoneal sarcomas (RPS). As a result, reducing LRR is a critical objective for RPS patients. However, unlike soft tissue sarcomas (STS) of the superficial trunk [...] Read more.
Locoregional recurrence (LRR) is the predominant pattern of relapse and often the cause of death in patients with retroperitoneal sarcomas (RPS). As a result, reducing LRR is a critical objective for RPS patients. However, unlike soft tissue sarcomas (STS) of the superficial trunk and extremity where the benefits of radiation therapy (RT) are well-established, the role of RT in the retroperitoneum remains controversial. Historically, preoperative or postoperative RT, either alone or in combination with intraoperative radiation (IORT), was commonly justified for RPS based on extrapolation from the superficial trunk and extremity STS literature. However, long-awaited results were recently published from the European Organization for Research and Treatment of Cancer (EORTC) STRASS study of preoperative radiotherapy plus surgery versus surgery alone for patients with RPS; there was no statistical difference in the primary endpoint of abdominal recurrence-free survival. However, several subset analyses and study limitations complicate the interpretation of the results. This review explores and contextualizes the body of evidence regarding RT’s role in managing RPS. Full article
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14 pages, 1490 KiB  
Review
Morbidity and Mortality after Surgery for Retroperitoneal Sarcoma
by Samantha M. Ruff, Valerie P. Grignol, Carlo M. Contreras, Raphael E. Pollock and Joal D. Beane
Curr. Oncol. 2023, 30(1), 492-505; https://doi.org/10.3390/curroncol30010039 - 29 Dec 2022
Cited by 5 | Viewed by 3731
Abstract
Retroperitoneal sarcoma (RPS) is a rare disease with over 100 histologic types and accounts for 10–15% of all soft tissue sarcomas. Due to the rarity of RPS, sarcoma centers in Europe and North America have created the Transatlantic RPS Working Group (TARPSWG) to [...] Read more.
Retroperitoneal sarcoma (RPS) is a rare disease with over 100 histologic types and accounts for 10–15% of all soft tissue sarcomas. Due to the rarity of RPS, sarcoma centers in Europe and North America have created the Transatlantic RPS Working Group (TARPSWG) to study this disease and establish best practices for its management. Current guidelines dictate complete resection of all macro and microscopic disease as the gold standard for patients with RPS. Complete extirpation often requires a multi-visceral resection. In addition, recent evidence suggests that en bloc compartmental resections are associated with reduced rates of local recurrence. However, this approach must be balanced by the potential for added morbidity. Strategies to mitigate postoperative complications include optimization of the patient through improved preoperative nutrition and pre-habilitation therapy, referral to a high-volume sarcoma center, and implementation of enhanced recovery protocols. This review will focus on the factors associated with perioperative complications following surgery for RPS and outline approaches to mitigate poor surgical outcomes in this patient population. Full article
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19 pages, 3492 KiB  
Review
Updates in Pathology for Retroperitoneal Soft Tissue Sarcoma
by Tanner Mack and Bibianna Purgina
Curr. Oncol. 2022, 29(9), 6400-6418; https://doi.org/10.3390/curroncol29090504 - 7 Sep 2022
Cited by 13 | Viewed by 5093
Abstract
Retroperitoneal tumors are extremely rare. More than 70% of primary retroperitoneal soft tissue tumors are malignant. The most common sarcomas in the retroperitoneum include liposarcomas and leiomyosarcoma, however other sarcomas, along with benign mesenchymal tumors, can occur. Sarcomas are a heterogenous group of [...] Read more.
Retroperitoneal tumors are extremely rare. More than 70% of primary retroperitoneal soft tissue tumors are malignant. The most common sarcomas in the retroperitoneum include liposarcomas and leiomyosarcoma, however other sarcomas, along with benign mesenchymal tumors, can occur. Sarcomas are a heterogenous group of tumors with overlapping microscopic features, posing a diagnostic challenge for the pathologist. Correct tumor classification has become important for prognostication and the evolving targeted therapies for sarcoma subtypes. In this review, the pathology of retroperitoneal soft tissue sarcomas is discussed, which is important to the surgical oncologist. In addition, less common sarcomas and benign mesenchymal tumors of the retroperitoneum, which may mimic sarcoma clinically and pathologically, are also discussed. Full article
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