Authors: Brooke C Bredbeck, Lia D Delaney, Varun G Kathawate...

BACKGROUND AND OBJECTIVES

Retroperitoneal and abdominopelvic sarcomas are rare heterogeneous malignancies. The only therapy proven to improve disease-free survival (DFS) is R0/R1 surgical resection. We sought to analyze whether additional factors such as radiation and systemic therapy were associated with DFS and abdominal recurrence-free survival (RFS).

METHODS

Retrospective review of adults (≥18) with resectable abdominopelvic and retroperitoneal sarcomas who underwent intent-to-cure surgery at a high-volume tertiary referral center between 1998 and 2015. The main outcome measures were DFS and abdominal RFS.

RESULTS

Overall, 159 patients met the criteria for inclusion. Median follow-up was 4.8 years (range 0.1-18.9 years). The most common histology was liposarcoma (49%). Systemic therapy was administered to 48% of patients and was not associated with improved outcomes. The neoadjuvant radiotherapy group (11%) had improved adjusted DFS (5.46 years, 95% CI [3.68, 7.24] vs. 3.1 years, 95% CI [2.48, 3.73]) and abdominal RFS (6.14 years, 95% CI [4.38, 7.89] vs. 3.22 years, 95% CI [2.61, 3.84]). The adjuvant radiotherapy group (19%) had no improvement.

CONCLUSIONS

In a cohort of patients undergoing resection for retroperitoneal or abdominopelvic sarcoma, neoadjuvant radiation improved DFS and abdominal RFS. A follow-up of over three years was needed to appreciate a difference in outcomes.

Topics: Adult; Disease-Free Survival; Humans; Liposarcoma; Neoplasm Recurrence, Local; Retroperitoneal Neoplasms; Retrospective Studies; Sarcoma; Soft Tissue Neoplasms

PubMed: 35239187
DOI: 10.1002/jso.26828

Authors: L D Leffall

Topics: Humans; Retroperitoneal Neoplasms; Sarcoma; Soft Tissue Neoplasms; Survival Rate

PubMed: 2064464
DOI: No ID Found

Review

Authors: Joshua S Jolissaint, Chandrajit P Raut, Mark Fairweather...

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

Review

Authors: T Christopher Windham, Peter W T Pisters

BACKGROUND

The evaluation and treatment of retroperitoneal sarcomas are challenging because the tumors are relatively rare and frequently present with advanced disease in an anatomically complex location.

METHODS

We reviewed the literature on experience in the management of retroperitoneal sarcomas, and we present our own experience in the treatment of these tumors.

RESULTS

The identification of prognostic factors other than the adequacy of resection has been inconsistent. Due to a lack of associated symptoms, retroperitoneal sarcomas smaller than 5 cm are rare. Computed tomography is the most useful tool in the evaluation of retroperitoneal tumors. Surgery, radiation therapy, and chemotherapy are treatment options, but the most important factor in the treatment of primary tumors is complete surgical resection. The role of neoadjuvant and adjuvant therapies is not defined and should be considered within the context of clinical trials.

CONCLUSIONS

Early referral of patients with retroperitoneal soft tissue tumors will help to ensure that they will receive the benefits of multidisciplinary evaluation and treatment of their disease and ready access to clinical trials.

Topics: Antineoplastic Agents; Humans; Liver Neoplasms; Lung Neoplasms; Neoplasm Recurrence, Local; Prognosis; Radiotherapy; Retroperitoneal Neoplasms; Retroperitoneal Space; Sarcoma

PubMed: 15668651
DOI: 10.1177/107327480501200105

Review

Authors: Dana A Dominguez, Sagus Sampath, Mark Agulnik...

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

Authors: Anne Tranberg, Maja Kjer Nielsen, Flemming Brandt Sørensen...

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

Authors: Isaac R Francis, Richard H Cohan, Datla G K Varma...

Retroperitoneal sarcomas are rare neoplasms. CT or MR imaging is performed in patients with these tumors to detect local extent and distant metastases of the tumor and for preoperative surgical planning. Most sarcomas cannot be characterized as to cell type with CT or MR, with the exceptions being liposarcomas and intracaval leiomyosarcomas. Similarly histological grading cannot be made definitively with imaging alone, the exception being liposarcoma since well differentiated liposarcomas contain more macroscopic fat than do less differentiated liposarcomas. After surgery, follow up imaging with CT or MR and careful scrutiny of the tumor bed and resection site are essential to detect early recurrences, which can often be managed with re-resection.

Topics: Diagnostic Imaging; Humans; Magnetic Resonance Imaging; Neoplasm Staging; Positron-Emission Tomography; Prognosis; Retroperitoneal Neoplasms; Sarcoma; Tomography, X-Ray Computed

PubMed: 16154826
DOI: 10.1102/1470-7330.2005.0019

Authors: Gabriel Glockzin, Thomas Helmberger

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

Authors: Xiangdong Li, Renjie Ding, Zhenhua Liu...

Predicting the histopathology of residual retroperitoneal masses (RMMs) before post-chemotherapy retroperitoneal lymph node dissection in metastatic nonseminomatous germ cell tumors (NSGCTs) can guide individualized treatment and minimize complications. Previous single approach-based models perform poorly in validation. Herein, we introduce a machine learning model that evolves from a single-dimensional tumor diameter to incorporate high-dimensional radiomic features, with its effectiveness assessed using the macro-average area under the receiver operating characteristic curves (AUCs). In addition, we utilize more precise and specific microRNAs (miRNAs), not common clinical indicators, to construct an integrated radiomics-miRNA predictive system, achieving an AUC of 0.91 (0.80-0.99) in the prospective test set. We further develop a web-based dynamic nomogram for swift and precise calculation of the histopathological probabilities of RMMs based on radiomic scores and serum miRNA levels. The radiomics-miRNA integrated system offers a promising tool to select personalized treatments for patients with metastatic NSGCT.

Topics: Humans; Neoplasms, Germ Cell and Embryonal; Male; MicroRNAs; Testicular Neoplasms; Retroperitoneal Neoplasms; Adult; ROC Curve; Nomograms; Neoplasm Metastasis; Young Adult; Machine Learning; Neoplasm, Residual; Biomarkers, Tumor; Radiomics

PubMed: 39672156
DOI: 10.1016/j.xcrm.2024.101843

Review

Authors: Hiba Othman, Joel Shapiro, Peter Chung...

Surgical resection is the cornerstone of curative treatment for retroperitoneal sarcomas (RPS), aiming for complete excision, yet the complexity of RPS with its proximity to vital structures continues to lead to high local recurrence rates after surgery alone. Thus, the role of radiotherapy (RT) continues to be refined to improve local control, which remains an important goal to prevent RPS recurrence. The recently completed global randomized trial to evaluate the role of surgery with and without preoperative RT - STRASS1, did not demonstrate a significant overall benefit for neoadjuvant RT based on the pre-specified definition of abdominal recurrence-free survival, however, sensitivity analysis using a standard definition of local recurrence and analysis of outcomes by compliance to the RT protocol suggests histology-specific benefit in well- and some de-differentiated liposarcomas. Ultimately, multidisciplinary collaboration and personalized approaches that consider histological sarcoma types and patient-specific factors are imperative for optimizing the therapeutic strategy in the management of RPS.

Topics: Humans; Sarcoma; Retroperitoneal Neoplasms; Combined Modality Therapy; Radiotherapy, Adjuvant; Neoadjuvant Therapy; Neoplasm Recurrence, Local

PubMed: 38508781
DOI: 10.1016/j.semradonc.2024.02.002