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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 -
Urology Dec 1976Retroperitoneal teratoma is the third most common primary retroperitoneal neoplasm of childhood ranking behind neuroblastoma and nephroblastoma. Ten per cent of these...
Retroperitoneal teratoma is the third most common primary retroperitoneal neoplasm of childhood ranking behind neuroblastoma and nephroblastoma. Ten per cent of these tumors are malignant. Two cases of benign retroperitoneal teratoma are reported, and the first description of the angiographic appearance of such a tumor is presented.
Topics: Adolescent; Child, Preschool; Female; Humans; Radiography; Retroperitoneal Neoplasms; Teratoma
PubMed: 997059
DOI: 10.1016/0090-4295(76)90535-5 -
Seminars in Ultrasound, CT, and MR Oct 2011Retroperitoneal sarcomas are a rare and highly malignant group of tumors. Because of their anatomical location, they often reach a large size before detection and are... (Review)
Review
Retroperitoneal sarcomas are a rare and highly malignant group of tumors. Because of their anatomical location, they often reach a large size before detection and are difficult to diagnose clinically because of nonspecific symptoms. After surgical resection, the tumors display a high rate of recurrence, thus requiring long-term and often indefinite follow-up. Consequently, imaging plays a central role in the diagnosis and management of these tumors. This review article examines the epidemiology, staging criteria, histologic subtypes, diagnosis, treatment, and general imaging principles for all retroperitoneal sarcomas, with detailed focus on the most common subtype, retroperitoneal liposarcomas.
Topics: Contrast Media; Diagnosis, Differential; Diagnostic Imaging; Humans; Neoplasm Staging; Prognosis; Radiopharmaceuticals; Retroperitoneal Neoplasms; Sarcoma
PubMed: 21963163
DOI: 10.1053/j.sult.2011.06.002 -
Journal of Surgical Oncology Jan 2018Retroperitoneal liposarcomas (RPLPS) are rare tumors that represent at least 50% of all retroperitoneal sarcomas. Surgical resection remains the standard of care.... (Review)
Review
Retroperitoneal liposarcomas (RPLPS) are rare tumors that represent at least 50% of all retroperitoneal sarcomas. Surgical resection remains the standard of care. Unfortunately, many RPLPS patients will develop a local recurrence and subsequently die in the absence of distant metastasis. This review outlines the factors that predict local recurrence and influence the management of first and subsequent multiply recurrent RPLPS.
Topics: Disease Management; Humans; Liposarcoma; Neoplasm Recurrence, Local; Retroperitoneal Neoplasms
PubMed: 29266232
DOI: 10.1002/jso.24929 -
Current Treatment Options in Oncology Aug 2000The approach to the management of retroperitoneal tumors begins with a complete history and physical examination. Imaging of the abdomen and pelvis by computed... (Review)
Review
The approach to the management of retroperitoneal tumors begins with a complete history and physical examination. Imaging of the abdomen and pelvis by computed tomography (CT) provides both an imaging modality and a method by which to obtain tissue for diagnosis. Because a histologic diagnosis is essential in treatment planning, adequate tissue can usually be obtained by a CT-guided core biopsy. If the diagnosis is sarcoma, additional tests necessary for staging include plain chest radiography and evaluation of the liver by either CT scan or magnetic resonance imaging (MRI). The treatment options for primary retroperitoneal sarcomas include chemotherapy, radiation therapy, surgery, or a combination of these modalities; therefore, a multidisciplinary group best manages treatment planning. Primary radiation therapy for cure is seldom effective for retroperitoneal sarcomas but can provide palliation in select cases. Systemic chemotherapy for chemosensitive lesions, such as poorly differentiated liposarcoma, malignant fibrous histiocytoma (MFH), synovial cell sarcoma, and primitive neuroectodermal tumors (PNET), can be useful when used in a neoadjuvant manner. Consequently, surgical resection continues to be the mainstay of treatment for retroperitoneal sarcomas and requires en bloc resection of the primary tumor. Frequently this includes adjacent organs such as colon, small bowel, kidney, adrenal, and pancreas. Postoperative adjuvant therapy with chemotherapy or radiation has not been proven to be of any additional benefit. Overall treatment results are predominantly influenced by tumor stage, grade, size, and margins of surgical resection. Follow-up CT scans at 6-month intervals and surgical resection of recurrences can be valuable.
Topics: Antineoplastic Agents; Biopsy; Clinical Trials as Topic; Combined Modality Therapy; Humans; Neoplasm Recurrence, Local; Radiotherapy; Retroperitoneal Neoplasms; Sarcoma; Survival Rate
PubMed: 12057171
DOI: 10.1007/s11864-000-0040-y -
International Journal of Urology :... Dec 2020Retroperitoneal tumors are extremely rare tumors occurring in the retroperitoneum. Retroperitoneal tumors are divided into benign tumors and malignant tumors, including... (Review)
Review
Retroperitoneal tumors are extremely rare tumors occurring in the retroperitoneum. Retroperitoneal tumors are divided into benign tumors and malignant tumors, including retroperitoneal sarcoma. Approximately 70-80% of primary retroperitoneal soft-tissue tumors are malignant; however, these only account for 0.1-0.2% of all malignancies. Retroperitoneal sarcoma is an orphan malignant disease with a low incidence. The information on benign retroperitoneal tumors is limited. The American Joint Committee on Cancer/TNM classification updated to the 8th edition in 2017. In 2010, three new drugs for soft tissue sarcoma were approved based on the results of phase III trials, but the histological subtypes of the patients enrolled in the trials of each drug differed. Recently, in addition to surgery for retroperitoneal sarcoma, the effectiveness of perioperative radiation therapy has become interesting. For malignant retroperitoneal tumors and retroperitoneal sarcoma, survival improvement and locoregional recurrence prevention can be undertaken by carrying out surgery to secure negative margins with wide and combined resection of some adjacent organs, and cooperation with a trained medical team comprising of radiologists, pathologists and medical oncologists in centralized hospitals. Some clinical trials aimed at further improving treatment results by adding preoperative chemotherapy and radiation therapy based on histological confirmation using a correct needle biopsy are in progress. In recent years, molecular profiling has been used to select eligible patients for chemotherapy. In the future, precision medicine with next-generation sequencing technology will be expected among the diverse and potential future treatments for retroperitoneal sarcoma. In this review, we summarized the current state of retroperitoneal tumors and retroperitoneal sarcoma.
Topics: Humans; Margins of Excision; Neoplasm Recurrence, Local; Retroperitoneal Neoplasms; Sarcoma; Treatment Outcome
PubMed: 32914475
DOI: 10.1111/iju.14361 -
Cancer Imaging : the Official... Aug 2005Retroperitoneal 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...
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 -
Seminars in Surgical Oncology 1994Retroperitoneal sarcomas are rare malignant tumors, accounting for approximately 10-20% of all soft tissue sarcomas. Because of their anatomic location and indolent... (Review)
Review
Retroperitoneal sarcomas are rare malignant tumors, accounting for approximately 10-20% of all soft tissue sarcomas. Because of their anatomic location and indolent nature, these tumors are usually quite extensive when first detected with little hope of improvement in the early detection rate. Improved survival rates will therefore depend on obtaining better local control through an aggressive surgical approach, making every effort to achieve a complete resection with histologic negative margins, which in most cases will require the resection of adjacent organs or vascular structures. Even with such aggressive surgical approach, continued problems with local recurrence are reported and it is important to follow patients who had undergone surgical resection very carefully in order to identify those who would benefit from reresection. The results of postoperative adjuvant irradiation and/or chemotherapy have so far been discouraging. Until there is a national registry and a national intergroup study to develop strategies and innovative trials for the effective treatment of these tumors, it is unlikely that there will be much improvement in the current survival rates.
Topics: Biopsy; Combined Modality Therapy; Humans; Neoplasm Metastasis; Neoplasm Recurrence, Local; Prognosis; Retroperitoneal Neoplasms; Sarcoma; Survival Rate
PubMed: 7997730
DOI: 10.1002/ssu.2980100509 -
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 -
European Review For Medical and... 1997A case of 31-year-old male with a retroperitoneal tumor is described. Abdominal ultrasound revealed a left para-aortic calcific mass, adjacent to the left lobe of the...
A case of 31-year-old male with a retroperitoneal tumor is described. Abdominal ultrasound revealed a left para-aortic calcific mass, adjacent to the left lobe of the liver and to the upper pole of the left kidney. A CT-scan of the abdomen showed the mass to originate from the left adrenal gland. At operation, a large, retroperitoneal mass, adherent to the left kidney and the spleen, but not infiltrating, was excised. Histologically the tumor was diagnosed as a calcified osteo-producing fibroma. Benign retroperitoneal tumors represent about 25% of all retroperitoneal neoplasm. This reported case represents a retroperitoneal tumor of slow growth and benign clinical course whose characteristic consists of the heavy calcifications which are normally absent in a fibroma type mass.
Topics: Adult; Calcinosis; Fibroma; Humans; Male; Retroperitoneal Neoplasms; Ultrasonography
PubMed: 9558778
DOI: No ID Found