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Current Oncology (Toronto, Ont.) Apr 2023Surgery is the cornerstone of treatment for retroperitoneal sarcoma (RPS). Surgery should be performed by a surgical oncologist with sub-specialization in this disease... (Review)
Review
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.
Topics: Humans; Neoplasm Recurrence, Local; Sarcoma; Treatment Outcome; Retroperitoneal Neoplasms; Soft Tissue Neoplasms
PubMed: 37232807
DOI: 10.3390/curroncol30050349 -
Current Oncology (Toronto, Ont.) Feb 2023Recurrence after resection of retroperitoneal sarcoma is common and varies by histological subtype. Pattern of recurrence is similarly affected by histology (e.g.,... (Review)
Review
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.
Topics: Humans; Retrospective Studies; Neoplasm Recurrence, Local; Sarcoma; Liposarcoma; Leiomyosarcoma; Retroperitoneal Neoplasms; Soft Tissue Neoplasms
PubMed: 36975422
DOI: 10.3390/curroncol30030209 -
Cancer Treatment Reviews Jun 2020Retroperitoneal liposarcomas are rare tumours that carry a poorer prognosis than their extremity counterparts. Within their subtypes - well differentiated (WDL),... (Review)
Review
Retroperitoneal liposarcomas are rare tumours that carry a poorer prognosis than their extremity counterparts. Within their subtypes - well differentiated (WDL), dedifferentiated (DDL), myxoid (MLS) and pleomorphic (PLS) - they exhibit a diverse genomic landscape. With recent advances in next generation sequencing, the number of studies exploring this have greatly increased. The recent literature has deepened our understanding of the hallmark MDM2/CDK4 amplification in WDL/DDL and addressed concerns about toxicity and resistance when targeting this. The FUS-DDIT3 fusion gene remains the primary focus of interest in MLS with additional potential targets described. Whole genome sequencing has driven identification of novel genes and pathways implicated in WDL/DDL outside of the classic 12q13-15 amplicon. Due to their rarity; anatomical location and histologic subtype are infrequently mentioned when reporting the results of these studies. Reports can include non-adipogenic or extremity tumours, making it difficult to draw specific retroperitoneal conclusions. This narrative review aims to provide a summary of retroperitoneal liposarcoma genomics and the implications for therapeutic targeting.
Topics: Animals; Antineoplastic Agents, Alkylating; Chromosome Aberrations; Genomics; Humans; Liposarcoma; Oncogene Proteins, Fusion; Phosphatidylinositol 3-Kinases; Retroperitoneal Neoplasms; Trabectedin
PubMed: 32278233
DOI: 10.1016/j.ctrv.2020.102013 -
Current Oncology (Toronto, Ont.) Feb 2023Significant multidisciplinary scientific effort has been undertaken to understand the heterogeneous family of neoplasms that comprise soft tissue sarcomas. Within this... (Review)
Review
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.
Topics: Humans; Sarcoma; Retroperitoneal Neoplasms; Immunotherapy; Soft Tissue Neoplasms; Biomarkers, Tumor; Tumor Microenvironment
PubMed: 36826126
DOI: 10.3390/curroncol30020165 -
Cancer Control : Journal of the Moffitt... Jul 2016Retroperitoneal sarcoma is rare. Using initial specimens on biopsy, a definitive diagnosis of histological subtypes is ideal but not always achievable. (Review)
Review
BACKGROUND
Retroperitoneal sarcoma is rare. Using initial specimens on biopsy, a definitive diagnosis of histological subtypes is ideal but not always achievable.
METHODS
A retrospective institutional review was performed for all cases of adult retroperitoneal sarcoma from 1996 to 2015. A review of the literature was also performed related to the distribution of retroperitoneal sarcoma subtypes. A meta-analysis was performed.
RESULTS
Liposarcoma is the most common subtype (45%), followed by leiomyosarcoma (21%), not otherwise specified (8%), and undifferentiated pleomorphic sarcoma (6%) by literature review. Data from Moffitt Cancer Center demonstrate the same general distribution for subtypes of retroperitoneal sarcoma. A pathology-based algorithm for the diagnosis of retroperitoneal sarcoma is illustrated, and common pitfalls in the pathology of retroperitoneal sarcoma are discussed.
CONCLUSIONS
An informative diagnosis of retroperitoneal sarcoma via specimens on biopsy is achievable and meaningful to guide effective therapy. A practical and multidisciplinary algorithm focused on the histopathology is helpful for the management of retroperitoneal sarcoma.
Topics: Female; Humans; Male; Retroperitoneal Neoplasms; Retrospective Studies; Sarcoma
PubMed: 27556665
DOI: 10.1177/107327481602300308 -
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 Apr 2015Management of retroperitoneal sarcomas presents technical and oncological challenges. Imaging is crucial for diagnosis and to define local tumor extent. Complete gross... (Review)
Review
Management of retroperitoneal sarcomas presents technical and oncological challenges. Imaging is crucial for diagnosis and to define local tumor extent. Complete gross resection at initial presentation is the best chance for cure, but there is controversy as to how this can be best achieved. There is a long-term risk of local recurrence, which is best treated with repeat resection if feasible. The roles of radiation and chemotherapy remain undefined.
Topics: Humans; Neoplasm Staging; Pelvic Neoplasms; Retroperitoneal Neoplasms; Sarcoma
PubMed: 25482329
DOI: 10.1002/jso.23840 -
The Israel Medical Association Journal... Jan 2020Primary retroperitoneal neoplasms (PRN) arise from diverse retroperitoneal tissues. Soft tissue sarcomas (STS) comprise the majority and are well studied. Other...
BACKGROUND
Primary retroperitoneal neoplasms (PRN) arise from diverse retroperitoneal tissues. Soft tissue sarcomas (STS) comprise the majority and are well studied. Other non-sarcomatous PRN are very rare and less familiar.
OBJECTIVES
To evaluate the clinicopathologic and radiologic features of non-sarcomatous PRN, as well as the outcome of complete tumor resection (TR).
METHODS
Retrospective data were collected on consecutive patients (June 2006 to January 2015) who underwent resection of retroperitoneal lesions at our department. Final pathology of non-sarcomatous PRN was included.
RESULTS
The study population included 36 patients (26% with PRN). PRN were neurogenic (17%), fat-containing (3%), and cystic (6%). The preoperative diagnosis was correct in only 28%. All patients underwent TR via laparotomy (72%) or laparoscopy (28%), for mean operative time of 120 ± 46 minutes. En bloc organ resection was performed in 11%. Complete TR was achieved in 97%. Intra-operative spillage occurred in 8%. Intra-operative, 90-day postoperative complications, and mortality rates were 11%, 36%, and 0%, respectively. The mean length of stay was 6.5 ± 5.5 days. The median overall survival was 53 ± 4.9 months.
CONCLUSIONS
Familiarity with radiologic characteristics of PRN is important for appropriate management. Counter to STS, other PRN are mostly benign and have an indolent course. Radical surgery is not required, as complete TR confers good prognosis. Expectant management is reserved for small, asymptomatic, benign neoplasms.
Topics: Aged; Female; Ganglioneuroma; Humans; Lipoma; Male; Middle Aged; Neurilemmoma; Neurofibroma; Paraganglioma; Retroperitoneal Neoplasms; Retrospective Studies; Tomography, X-Ray Computed
PubMed: 31927807
DOI: No ID Found -
Current Oncology (Toronto, Ont.) Jan 2023Locoregional recurrence (LRR) is the predominant pattern of relapse and often the cause of death in patients with retroperitoneal sarcomas (RPS). As a result, reducing... (Review)
Review
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.
Topics: Humans; Radiotherapy, Adjuvant; Neoplasm Recurrence, Local; Sarcoma; Soft Tissue Neoplasms; Retroperitoneal Neoplasms
PubMed: 36661696
DOI: 10.3390/curroncol30010047