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Der Pathologe Jul 2019Soft-tissue sarcomas are rare malignant tumors. Surgery remains the most important treatment modality. Neoadjuvant and/or adjuvant chemo- and radiotherapy may be... (Review)
Review
Soft-tissue sarcomas are rare malignant tumors. Surgery remains the most important treatment modality. Neoadjuvant and/or adjuvant chemo- and radiotherapy may be administered to improve the local and systemic outcome. Advances in oncological and reconstructive surgery, combined with the use of multimodal therapies, have made mutilating surgery rare events in extremity sarcomas. In retroperitoneal sarcomas, local recurrences are life-threatening events and multivisceral resection has become the standard surgical procedure. The subjects of this review are diagnostics, multimodal therapy, and resection strategy from a surgical point of view.
Topics: Combined Modality Therapy; Extremities; Humans; Neoplasm Recurrence, Local; Retroperitoneal Neoplasms; Sarcoma; Soft Tissue Neoplasms
PubMed: 31240451
DOI: 10.1007/s00292-019-0631-2 -
European Journal of Surgical Oncology :... Jun 2023Benign retroperitoneal tumors (BRT) represent a rare group of heterogeneous diseases. The literature lacks high-quality evidence about the optimal management of BRT, and... (Review)
Review
Benign retroperitoneal tumors (BRT) represent a rare group of heterogeneous diseases. The literature lacks high-quality evidence about the optimal management of BRT, and most of the information available takes the form of case reports or case series. The aim of this review is to provide an overview of current management strategies for adult patients with BRT. A literature search using PubMed indexed articles was conducted and BRT were classified into five different biological subgroups: 1) lipomatous tumors, 2) smooth muscle tumors, 3) peripheral nerve sheath tumors, 4) myofibroblastic tumors, and 5) others. Tumors that are primarily pelvic in origin were excluded. Despite the significant heterogeneity of the disease, several generic considerations have emerged and can be applied to the management of BRT. Specifically, the risk of misdiagnosing a BRT with another pathology such as retroperitoneal sarcoma is notable. When encountered, suspected BRT should therefore be referred to a specialized sarcoma center. Multidisciplinary tumor boards, present at these centers, have a pivotal role in managing BRT. The decision of whether to offer surgery, nonsurgical treatment or a "watch-and-wait" approach should be made after multidisciplinary discussion, depending on tumor histology. Moving forward, collaborative research efforts dedicated to BRT remain crucial in gathering evidence and knowledge to further optimize patient care.
Topics: Adult; Humans; Retroperitoneal Neoplasms; Sarcoma; Combined Modality Therapy; Soft Tissue Neoplasms; Brachytherapy
PubMed: 35879135
DOI: 10.1016/j.ejso.2022.07.006 -
American Journal of Clinical Oncology Apr 2015Retroperitoneal liposarcomas are rare mesenchymal tumors of the retroperitoneum that typically present with advanced disease and often carry a poor prognosis. Because of... (Review)
Review
Retroperitoneal liposarcomas are rare mesenchymal tumors of the retroperitoneum that typically present with advanced disease and often carry a poor prognosis. Because of their rarity and anatomic location, these malignant tumors can cause a diagnostic dilemma and present several therapeutic challenges. They are usually associated with a high rate of recurrence despite grossly complete resection, thus requiring long-term and often indefinite follow-up. Relevant data on this topic was procured and synthesized with the aid of a comprehensive Medline search in addition to oncologic, pathologic, urologic, radiologic, and surgical literature review on retroperitoneal sarcomas. This article provides an in-depth review into the natural history, pathology, clinical manifestations, and prognostic features of retroperitoneal liposarcomas. It also discusses the reliability of diagnostic procedures and novel curative approaches that are currently being evaluated for the disease.
Topics: Humans; Liposarcoma; Retroperitoneal Neoplasms
PubMed: 24136142
DOI: 10.1097/COC.0b013e31829b5667 -
Surgical Oncology Clinics of North... Oct 2016After diagnosis of retroperitoneal sarcoma (RPS), detailed imaging and multidisciplinary discussion should guide treatment including surgical resection and in select... (Review)
Review
After diagnosis of retroperitoneal sarcoma (RPS), detailed imaging and multidisciplinary discussion should guide treatment including surgical resection and in select cases, neoadjuvant therapy. Local recurrence is common in RPS and is associated with grade, histologic subtype, completeness of resection, and size. As guidelines to standardize RPS patient management emerge, expert pathologic assessment and management in centers of excellence are benchmarks of quality of care. The efficacy of current chemotherapy is limited and there is a critical need to understand the molecular basis of sarcoma so that new drug therapies are developed. Multicenter clinical trials are needed to limit opinion and controversy in this complex and challenging disease.
Topics: Combined Modality Therapy; Humans; Neoadjuvant Therapy; Neoplasm Recurrence, Local; Retroperitoneal Neoplasms; Sarcoma; Treatment Outcome
PubMed: 27591493
DOI: 10.1016/j.soc.2016.05.003 -
Current Opinion in Oncology Jul 2022To review current knowledge and recent advances in retroperitoneal sarcoma management. (Review)
Review
PURPOSE OF REVIEW
To review current knowledge and recent advances in retroperitoneal sarcoma management.
RECENT FINDINGS
Surgery, radiotherapy, and medical treatments of retroperitoneal sarcomas should take into account the peculiarities of each histotype and the unique anatomical site. Surgery remains the mainstay of treatment and the only chance of cure for these diseases. In low-grade retroperitoneal sarcomas, like well differentiated liposarcoma, where the leading cause of death is dominated by local rather than distant relapses, treatment of the primary tumor encompasses extended surgery with multiorgan resection and evaluation of preoperative radiotherapy. Conversely, surgery is usually more conservative and without radiotherapy in those retroperitoneal sarcomas, such as leiomyosarcoma, characterized by a high risk of metastatic spread that prompted also the evaluation of neoadjuvant, histotype-driven chemotherapy. Surgery might have a role also for relapsed disease, despite long-term disease control probability declines at each recurrence. In advanced stages, anthracyclines still retain a key role and all medical treatment strategies should follow the specific chemosensitivity of each histotype to improve patient's outcomes.
SUMMARY
The rarity and heterogeneity in biological behavior and clinical presentation of retroperitoneal sarcomas deserves a multidisciplinary and histotype-driven treatment at all stages of the disease to be performed in highly specialized centers.
Topics: Humans; Liposarcoma; Neoplasm Recurrence, Local; Retroperitoneal Neoplasms; Sarcoma; Soft Tissue Neoplasms
PubMed: 35837704
DOI: 10.1097/CCO.0000000000000851 -
Expert Review of Anticancer Therapy 2023Retroperitoneal sarcomas (RPS) are rare mesenchymal tumors that account for only 0.1-0.2% of all malignancies. Management of this disease is challenging, and resection... (Review)
Review
INTRODUCTION
Retroperitoneal sarcomas (RPS) are rare mesenchymal tumors that account for only 0.1-0.2% of all malignancies. Management of this disease is challenging, and resection remains the cornerstone of treatment. Ongoing international collaboration has expanded our knowledge of this disease, allowing for a more personalized approach to RPS patients resulting in improved survival over time. Due to the heterogeneity of RPS, with differing recurrence patterns and sensitivities to neoadjuvant therapies based on histology and grade, management of RPS should be tailored to the individual patient.
AREAS COVERED
Our review focuses on a histology-driven approach in the management of primary RPS. We searched relevant articles from 1993 to 2023 that investigated prognostic factors and treatment of patients with RPS and summarized recent advances and future directions in the field.
EXPERT OPINION
Deeper understanding of the role of neoadjuvant radiotherapy and ongoing trials investigating the role of neoadjuvant chemotherapy will potentially contribute to the development of individualized treatment pathways.
Topics: Humans; Retrospective Studies; Sarcoma; Retroperitoneal Neoplasms; Neoadjuvant Therapy; Neoplasm Recurrence, Local
PubMed: 37791587
DOI: 10.1080/14737140.2023.2266137 -
Surgical Oncology Jun 2022Intraabdominal and retroperitoneal sarcomas (IaRS) are malignant connective tissue tumors. Surgical resection is often the only curative treatment. The primary objective...
BACKGROUND
Intraabdominal and retroperitoneal sarcomas (IaRS) are malignant connective tissue tumors. Surgical resection is often the only curative treatment. The primary objective was to report the mid-term outcomes following contemporary treatment protocols and identify prognostic factors.
METHODS
A retrospective review of consecutive patients (n = 107) with IaRS treated at single center from 2013 until 2018 was conducted. Histological diagnosis, tumor grade, perioperative complications, mortality, and long-time survival were registered and retrieved from patient records. Primary and recurrent tumors were analyzed separately.
RESULTS
A total of 107 patients were identified. Median follow-up time was 3.5 years. Thirty-day mortality was 3.4% and 90-day mortality was 5.6% for all tumors. The major complication rate was 18%. The 5-year estimated survival for primary and recurrent tumors was 55.4% and 48.4%, respectively. Multifocal disease was evident in 32% of the patient cohort, and 58% of patients in the recurrent group. Multivariate analysis for survival revealed a hazard ratio (HR) of 3.1 (95% CI 1.68-8.41) for multifocality, HR 2.9 (95% CI 1.28-6.98) for Clavien-Dindo grade, HR 2.3 (95% CI 1.21-4.31) for tumor grades 2 or 3, and HR 1.002 (95% CI 1.001-1.004) for surgical margins.
CONCLUSIONS
Our study found overall acceptable morbidity and mortality, and identified prognostic markers for overall survival. Recurrent tumors were not associated with worse survival. Multifocality is associated with a worse overall survival. The prognostic factors identified were; tumor grade, multifocality, intralesional margins and postoperative complications.
Topics: Humans; Margins of Excision; Neoplasm Recurrence, Local; Prognosis; Retroperitoneal Neoplasms; Retrospective Studies; Sarcoma; Soft Tissue Neoplasms; Survival Rate; Treatment Outcome
PubMed: 35643015
DOI: 10.1016/j.suronc.2022.101781 -
RoFo : Fortschritte Auf Dem Gebiete Der... May 2023Peritoneal and retroperitoneal tumors consist of a heterogenous group of benign and malignant lesions of different origin. Due to often complex multidisciplinary...
Peritoneal and retroperitoneal tumors consist of a heterogenous group of benign and malignant lesions of different origin. Due to often complex multidisciplinary treatment concepts in patients with peritoneal surface malignancies radiological imaging plays a pivotal role regarding the therapeutic options. Moreover, tumor entity, abdominal tumor distribution and common as well as rare differential diagnoses have to be taken into account. Using different radiological modalities non-invasive pretherapeutic diagnostics might be significantly improved. KEY POINTS:: · Diagnostic CT is a valuable part of the initial diagnostic approach to peritoneal surface malignancies.. · Sensitivity might be increased by the additional use of dwMRI and PET/CT considering tumor entity and individual diagnostic issues.. · The Peritoneal Cancer Index (PCI) should be determined independent of radiologic modality.. CITATION FORMAT: · Glockzin G, Helmberger T. Radiologic staging of peritoneal and retroperitoneal disease. Fortschr Röntgenstr 2023; 195: 377 - 384.
Topics: Humans; Peritoneal Neoplasms; Positron Emission Tomography Computed Tomography; Retroperitoneal Neoplasms; Radiography; Abdominal Neoplasms; Neoplasm Staging
PubMed: 36863365
DOI: 10.1055/a-1999-7057 -
Journal of Surgical Oncology Jan 2018Resection of retroperitoneal sarcoma (RPS) typically involves multivisceral resection. The morbidity of RPS resection has decreased over time despite widespread adoption... (Review)
Review
Resection of retroperitoneal sarcoma (RPS) typically involves multivisceral resection. The morbidity of RPS resection has decreased over time despite widespread adoption of radical resection. Certain patterns of resection are associated with higher complication rates and elderly patients are at increased risk of morbidity. Administration of preoperative radiotherapy does not increase morbidity, but intraoperative and brachytherapy techniques are associated with heightened toxicities. Long-term functional outcomes and quality of life scores after RPS resection are acceptable.
Topics: Humans; Morbidity; Quality of Life; Retroperitoneal Neoplasms; Sarcoma
PubMed: 29314041
DOI: 10.1002/jso.24902 -
European Journal of Surgical Oncology :... Jun 2023Until recently, the recommendation for primary retroperitoneal sarcomas (RPS) was to perform a complete en-bloc gross excision, (neo) adjuvant treatments being options... (Review)
Review
Until recently, the recommendation for primary retroperitoneal sarcomas (RPS) was to perform a complete en-bloc gross excision, (neo) adjuvant treatments being options which were not validated by randomized studies, with a large discrepancy of use between centers. The heterogeneity of RPS, with their different biological behaviour, renders a homogenous therapeutic and surgical approach probably inappropriate. Recent studies, both surgical and dedicated to adjuvant treatments, allow refining these recommendations. This review summarizes recent advances and directions.
Topics: Humans; Sarcoma; Retroperitoneal Neoplasms; Survival Rate; Neoplasm Recurrence, Local; Retrospective Studies
PubMed: 35599138
DOI: 10.1016/j.ejso.2022.05.010