A Successful Chemoembolization of a Retroperitoneal Soft Tissue Sarcoma: A Case Report

Retroperitoneal sarcomas are rare entities. They have a tendency of growing slowly, rendering the patient apparently healthy for long periods of time, before diagnosis. Besides, they have a worse prognosis than sarcomas arising in extremities, with a higher local recurrence rate and lower 5-year survival rate. We describe a case of a 71-year-old male patient, who had a very well succeeded treatment of a large retroperitoneal sarcoma with the combination of chemoembolization, systemic chemotherapy, surgery, and radiation therapy. Initially, it was noticed in an incidental way he had a large retroperitoneal mass (15 cm × 10 cm × 9.2 cm) through magnetic resonance, when he was 63. The case was considered inoperable by the treating physicians. After neoadjuvant therapy, the residual tumor could be completely excised by the responsible surgeon. With a follow-up of >5 years, since the end of treatment, the patient remains in complete remission and, probably, cured from his illness. Large retroperitoneal sarcomas are still a great challenge for oncologists. According to the medical literature, chemoembolization can benefit some patients, but most of them in a palliative setting. In our report, we believe its contribution was critical for a great outcome. In selected cases, it is possible this procedure may be an additional therapeutic modality, as part of a multidisciplinary approach.

Neoadjuvant concurrent chemoradiotherapy with and without hyperthermia in retroperitoneal sarcomas: feasibility, efficacy, toxicity, and long-term outcome

Purpose Retroperitoneal (RPS) sarcomas are associated with poor local and abdominal tumor control. However, the benefit of preoperative radio- or chemotherapy alone for these entities is currently unclear. Moreover, as intermediate- and high-grade sarcomas have a tendency toward early metastasis, exploration of neoadjuvant strategies is of high importance. This analysis reports the results of our 20-year single-institution experience with preoperative neoadjuvant concurrent chemoradiation. Methods From 2000–2019, 27 patients with intermediate- or high-grade RPS (12 dedifferentiated liposarcoma, 10 leiomyosarcoma, 5 others) were treated with radiotherapy (median dose: 50.4 Gy; range 45–75 Gy) and two cycles of chemotherapy (doxorubicin 50 mg/m2 BSA/d3 q28 and ifosfamide 1.5 g/m2 BSA/d1‑5 q28) in neoadjuvant intent. Chemotherapy consisted of doxorubicin alone in two cases and ifosfamide alone in one case. Fifteen patients (56%) additionally received deep regional hyperthermia. Results The median follow-up time was 53 months (±56.7 months). 92% of patients received two cycles of chemotherapy as planned and 92% underwent surgery. At 5 and 10 years, abdominal-recurrence-free survival was 74.6% (±10.1%) and 66.3% (±11.9%), distant metastasis-free survival was 67.2% (±9.7%) and 59.7% (±11.1%), and overall survival was 60.3% (±10.5%) and 60.3% (±10.5%), respectively. CTC grade III and IV toxicities were leukocytopenia (85%), thrombocytopenia (33%), and anemia (11%). There were no treatment-related deaths. Conclusion Neoadjuvant chemoradiotherapy with and without hyperthermia for retroperitoneal sarcomas is feasible and provided high local control of intermediate- and high-grade sarcoma.

Adjuvant therapy for retroperitoneal sarcoma: a meta-analysis

Background Adjuvant therapy is a promising treatment to improve the prognosis of cancer patients, however, the evidence base driving recommendations for adjuvant radiotherapy (ART) or chemotherapy (ACT) in retroperitoneal sarcomas (RPS) primarily hinges on observational data. The aim of this study was to evaluate the effectiveness of adjuvant therapy in the management of RPS patients. Methods We searched PubMed, Web of Science, Embase, ASCO Abstracts, and Cochrane Library for comparative studies (until December 2020) of adjuvant therapy versus surgery alone. Data on the following endpoints were evaluated: overall survival (OS), local recurrence (LR), recurrence-free survival (RFS), and metastasis-free survival (MFS). Data were summarized as hazard ratios (HR) with 95% confidence intervals (CI). Risk of bias of studies was assessed with Begg’s and Egger’s tests. Results A total of 15 trials were eligible, including 9281 adjuvant therapy and 21,583 surgery alone cases (20 studies for OS, six studies for RFS, two studies for LR, and two studies for MFS). Meta-analysis showed that ART was associated with distinct advantages as compared to surgery alone, including a longer OS (HR = 0.80, P < 0.0001), a longer RFS (HR = 0.61, P = 0.0002), and a lower LR (HR = 0.31, P = 0.005). However, this meta-analysis failed to demonstrate a benefit of ACT for RPS patients, including OS (HR = 1.11, P = 0.19), RFS (HR = 1.30, P = 0.09) and MFS (HR = 0.69, P = 0.09). In the sensitivity analysis, ACT was associated with a worse OS (HR = 1.19, P = 0.0002). No evidence of publication bias was observed. Conclusions Overall, the quality of the evidence was moderate for most outcomes. The evidence supports that ART achieved a generally better outcome as compared to surgery alone.

EP.TH.96Recurrent Retroperitoneal Liposarcoma Presenting as Irreducible Inguinal Hernia

Introduction Retroperitoneal sarcomas protruding though the groin are extremely rare with only about 12 cases reported in the past 31 years to our knowledge. . As the retroperitoneal space communicates with the inguinal canal, large lipomatous tumors, may protrude through this natural weak spot which may present as irreduciible inguinal hernia. Case Report We report a 43 year old male, previously operated for retroperitoneal liposarcoma with 4 cycles of adjuvant chemotherapy and 5 cycles of radiotherapy 4 years back now presented with irreducible inguinal hernia. On examination, a firm fixed mass of about 15 x 10 cm, with indistinct margins was palpable in right hemiabdomen, abdomen was soft with audible bowel sounds. A firm swelling of 8 x 5 cm in right inguinal region extending into scrotum with no cough impulse was also present. On CT imaging a large mixed density lesion noted in Right lower abdomen measuring 16.2 x 14.8 x 23.5 cm. It showed internal fat and solid components and recurrence with herniation through right inguinal canal which is a rare entity. The patient was treated symptomatically, bilateral DJ stenting was done by the urology team and the patient was prepared for debulking surgery. Patient was classified as ASA IV. Unfortunately, the patient died 6th post admission day secondary to cardiopulmonary arrest. Conclusion A retroperitoneal tumor especially a liposarcoma should be ruled out in a large, painless, non-reducible inguinal mass with abdominal complaints. Patients need regular follow up after resection of retroperitoneal liposarcoma for early detection of recurrence.

Multidisciplinary Management of Retroperitoneal Sarcoma: Diagnosis, Prognostic Factors and Treatment

Retroperitoneal sarcomas (RPS) are rare cancers whose management can be challenging due to various presentation patterns, multiple organ involvement, and a high local and distant recurrence rate. Histopathology and prognostic factors analysis are essential to predict the behaviour of the disease and plan the best therapeutic strategy. To date, surgery is still the main therapeutic option that guarantees a chance of cure from the primary disease. While chemotherapy and radiotherapy seem to be good options for controlling metastatic and recurrent irresectable disease, their role in the treatment of primary RPS remains unclear. This literature review aims to provide a comprehensive overview of the multidisciplinary aspects of RPS management in high-volume centres, summarising the diagnostic path, the prognostic factors, and the most suitable therapeutic options.

Primary Adult Retroperitoneal Sarcoma: A Comprehensive Genomic Profiling Study

Background: Adult primary retroperitoneal sarcomas (RPSs) are a group of heterogeneous tumors with different histological subtypes. Comprehensive genomic profiling (CGP) analyses have recently provided significant insights into the biology of sarcomas by identifying genomic alterations (GAs) which could benefit from targeted therapies. Methods: RPS were evaluated by CGP using next-generation sequencing of up to 406 cancer-related genes. Tumor mutational burden (TMB) was determined on 0.83 to 1.14 mut/Mb of sequenced DNA. Finally, PD-L1 expression was determined. Results: Overall, 296 cases of primary RPS were analyzed. Liposarcoma (LPS) subtype had more GA/tumor than leiomyosarcoma (LMS) subtypes, with follicular dendritic cell sarcomas harboring the highest and synovial sarcomas the lowest. TP53 and Rb1 alterations were the highest in LMS, and CDK4/6 and MDM2 in LPS. However, both the TMB and targetable GA rates were low across subtypes. PD-L1 immunostaining was low positive in 21% and high positive in 5% of patients, respectively. Conclusions: CGP analysis revealed that potentially actionable genomic targets were rare in our cohort of RPS. Moreover, RPSs seem less likely to respond to immune checkpoint inhibitors based on putative biomarkers status. Nevertheless, genomic stratification according to histological subtypes led to description of GAs that can inform future clinical trials design.