neoadjuvant chemotherapy combined with targeted treatment in high-risk retroperitoneal sarcoma

Conditions: Retroperitoneal Sarcoma

Summary: the goal of this clinical trial is to explore the potential survival benefits of neoadjuvant chemotherapy combined with target treatments followed by radical surgery in patients with primary high-risk/grade retroperitoneal sarcoma. the main questions it aims to answer are: * whether the 1,3-year progression-free survival time(pfs) is prolonged in the neoadjuvant therapy group, compared with the surgery-only group. * the overall survival time in the two groups. * the safety and tolerance in the neoadjuvant therapy group. participants will be allocated into two groups once they meet the inclusion criteria. * surgery-only group: patients will directly undergo surgeries after the confirmation of diagnosis through pre-operative biopsy. * neoadjuvant therapy group: patients will receive the neoadjuvant chemotherapy combined with target treatment for three circles before the following sarcoma resectional surgeries.


Eligibility criteria:
inclusion criteria: * histologically proven primary high-risk retroperitoneal sarcoma(rps) (including dedifferentiated liposarcoma (ddlps), leiomyosarcoma(lms), undifferentiated pleomorphic sarcoma(ups), solitary fibrous tumor(sft)). * primary localized rps without histories of surgical resection, chemo- or radio-therapy, targeted therapy. patients who underwent laparotomy with biopsy only will also be considered as primary cases. * without histories of second malignant tumors. * in ddlps * diagnosis should be confirmed based on mdm2(mouse double minute 2 homolog) and cdk4(cyclin-dependent kinase 4) expression on ihc (immunohistochemistry), while mdm2 amplification in the fish test is highly recommended. * all grade 2-3 ddlps can be included. * in lms * all grades of lms can be included. * tumor size ≥10cm * in ups or sft * all grades of ups can be included. * high-risk sft with a score ≥6 in 'risk stratification of sft for development of metastasis' introduced in the 5th edition of the who(world health organization) soft tissue sarcoma classification. * sarcoma without protruding across the diaphragm * possibility of r0/r1 resection evaluated through preoperative mdt(multi-disciplinary team) discussion * no multi-organ or system dysfunction/failure or patients with slight dysfunction of organ/system could be easily recovered * tolerable of chemotherapy and surgery through mdt evaluation and tests. * american society of anesthesiologist (asa) ≤3 * fully understand the informs and consent to participate in the study. exclusion criteria: * sarcoma confirmed originating from the gi tract, uterus, or urinal tract. * patients included in the study through preoperative biopsy or mdt discussion, with wdlps, grade 1 lps, or other types of tumors not mentioned in the inclusion criteria evaluating through final pathological findings. * sarcoma protruded into the chest cavity evaluated through preoperative imaging or surgical reports. * metastasis confirmed pathologically or highly suspicious metastatic lesions through radiological findings, pet-ct(positron emission tomography) is highly recommended, mdt discussion and evaluation are needed. * histories of administration of anthracyclines, ifosfamide, and targeted agents. * severe contradiction to surgery and chemotherapies, including persistent myelosuppression, myocardial/cerebral/pulmonary infarction, uncontrolled cardiac arrhythmia, etc within the last 6 months * indication of severe infection or undergoing surgeries with high-risk hemorrhages in one month. * persistent one or more organ or system dysfunctions, could not be recovered prior to the study. * female patients who are pregnant or breastfeeding or female and male patients of reproductive potential who are not willing to employ effective birth control methods. * patients with psychological conditions

Keywords: retroperitoneal sarcoma neoadjuvant therapy target therapy survival