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Cancer Control : Journal of the Moffitt... Jul 2011Retroperitoneal sarcomas are rare neoplasms that often present with multivisceral involvement. Treatment for these tumors requires careful decision making requiring a... (Review)
Review
BACKGROUND
Retroperitoneal sarcomas are rare neoplasms that often present with multivisceral involvement. Treatment for these tumors requires careful decision making requiring a combination of surgery, chemotherapy, and radiation therapy.
METHODS
We reviewed the scientific literature pertaining to the diagnosis and management of retroperitoneal sarcomas. We also identify recent developments in treatment and discuss future trends in the care of patients with this disease.
RESULTS
Retroperitoneal tumors often present as large, locally advanced lesions. Evaluation of these tumors requires careful consideration of a multimodality approach. Retrospective data and historical prospective series have demonstrated the survival benefit of radical resection for these tumors with en bloc resection of involved structures. Compartmental resections in the retroperitoneum along with debulking of high-grade disease and regional therapy are controversial approaches with significant morbidity that can lead to long-term survival. The application of neoadjuvant and adjuvant therapies in select tumor histologies may improve local control and survival.
CONCLUSIONS
The management of retroperitoneal sarcomas requires a multidisciplinary approach and is best accomplished at high-volume centers specializing in the care of patients with these complex malignancies. Current data suggest that radical resection remains the only chance for cure and that chemotherapy and radiation therapy may confer a survival benefit.
Topics: Combined Modality Therapy; Disease-Free Survival; Humans; Neoadjuvant Therapy; Neoplasm Metastasis; Neoplasm Recurrence, Local; Patient Care; Prognosis; Retroperitoneal Neoplasms; Sarcoma
PubMed: 21666580
DOI: 10.1177/107327481101800305 -
Primary retroperitoneal soft tissue sarcoma: Imaging appearances, pitfalls and diagnostic algorithm.European Journal of Surgical Oncology :... Jul 2017Although retroperitoneal sarcomas are rare tumours, they can be encountered by a wide variety of clinicians as they can be incidental findings on imaging or present with... (Review)
Review
Although retroperitoneal sarcomas are rare tumours, they can be encountered by a wide variety of clinicians as they can be incidental findings on imaging or present with non specific symptoms and signs. Surgical resection can offer hope of cure and patient outcomes are improved when patients are managed in high-volume specialist centers. Failure to recognize retroperitoneal sarcomas on imaging can lead to inappropriate management in inexperienced centers. Therefore it is critical that a diagnosis of retroperitoneal sarcoma should be considered in the differential diagnosis of a retroperitoneal mass with prompt referral to a soft tissue sarcoma unit. In particular, the most common retroperitoneal sarcoma subtypes, liposarcoma and leiomyosarcoma, have characteristic imaging appearances which are discussed. This review therefore aims to set the context and guide clinicians through a diagnostic pathway for retroperitoneal masses in adults which arise extrinsic to the solid abdominal viscera.
Topics: Algorithms; Biopsy, Large-Core Needle; Contrast Media; Diagnosis, Differential; Humans; Image-Guided Biopsy; Magnetic Resonance Imaging; Retroperitoneal Neoplasms; Sarcoma; Tomography, X-Ray Computed
PubMed: 28057392
DOI: 10.1016/j.ejso.2016.10.032 -
The Journal of Surgical Research Dec 2019Sarcomas are malignant tumors of connective tissue that can vary widely in etiology. Parameters such as grade, extent of resection, and tumor integrity have been shown...
BACKGROUND
Sarcomas are malignant tumors of connective tissue that can vary widely in etiology. Parameters such as grade, extent of resection, and tumor integrity have been shown to affect prognosis. Our principal aim was to examine associations between the laterality of retroperitoneal sarcomas and tumor characteristics, treatment, and patient outcomes.
MATERIALS AND METHODS
We performed a retrospective study of patients treated at our tertiary referral center with a diagnosis of primary retroperitoneal sarcoma who underwent tumor resection. Categorical variables were compared using the chi-square test, whereas continuous variables were compared using one-way analysis of variance. Cox regression was used to estimate the risk of death.
RESULTS
Data from 106 patients were analyzed. A greater proportion of bilateral or midline tumors were leiomyosarcomas (P = 0.02), whereas right-sided tumors were more likely to be liposarcoma (P = 0.02). There was no significant relationship between laterality and tumor grade or stage. Two-thirds of patients had at least one contiguous organ resected (n = 68, 65.4%). Patients with nephrectomy during sarcoma resection were more likely to have right-sided disease (P = 0.02). Splenectomy and pancreatectomy were associated with left-sided disease (P < 0.01; P < 0.01), and pancreaticoduodenectomies with bilateral or midline disease (P < 0.001). Adjusting for age, sex, race, grade, stage, histology, and treatment, there was no increased risk of death or recurrence based on laterality.
CONCLUSIONS
Although laterality did not seem to have a measurable relationship with patient outcomes or survival, there was a significant association between laterality, tumor histology, and resection of contiguous organs. These preliminary findings warrant further investigation.
Topics: Aged; Female; Humans; Leiomyosarcoma; Liposarcoma; Male; Middle Aged; Neoplasm Grading; Neoplasm Recurrence, Local; Nephrectomy; Pancreatectomy; Pancreaticoduodenectomy; Prognosis; Retroperitoneal Neoplasms; Retroperitoneal Space; Retrospective Studies; Splenectomy; Treatment Outcome
PubMed: 31279261
DOI: 10.1016/j.jss.2019.05.016 -
The Journal of International Medical... May 2023Primary retroperitoneal mucinous cystic neoplasms are rare retroperitoneal tumors, which are histologically similar to mucinous cystic neoplasms of the ovaries. Only 31...
Primary retroperitoneal mucinous cystic neoplasms are rare retroperitoneal tumors, which are histologically similar to mucinous cystic neoplasms of the ovaries. Only 31 cases of primary retroperitoneal mucinous cystic neoplasm with borderline malignancy (PRMCN-BM) have been reported (26 in women and five in men). We describe an additional male patient with PRMCN-BM. A 39-year-old man presented to our hospital with back pain. Twelve years earlier, he had undergone an orchiectomy for a germ cell tumor. Computed tomography showed a 6.9- × 4.4-cm cystic mass in the left pararenal space. Laparoscopic mass excision was performed, and a unilocular cystic mass was found in the pararenal space near the lower pole of the left kidney. A histopathological examination showed a cyst lined by atypical mucinous intestinal epithelium without stromal invasion. Targeted next-generation sequencing identified two hotspot mutations, with one each in the and genes. Outpatient follow-up 10 months after surgery showed no evidence of tumor recurrence. PRMCNs are extremely rare retroperitoneal neoplasms, especially in men. These neoplasms are rarely considered in the differential diagnosis of retroperitoneal masses, and their preoperative diagnosis is difficult. Evaluation of additional patients is required to better determine the prognosis of PRMCNs and the optimal postoperative follow-up.
Topics: Adult; Humans; Male; Chromogranins; Cystadenoma, Mucinous; Cysts; GTP-Binding Protein alpha Subunits, Gs; Mutation; Neoplasm Recurrence, Local; Neoplasms, Cystic, Mucinous, and Serous; Proto-Oncogene Proteins p21(ras); Retroperitoneal Neoplasms
PubMed: 37198972
DOI: 10.1177/03000605231172469 -
European Journal of Vascular and... Nov 2020Radical excision of retroperitoneal or intra-abdominal soft tissue sarcomas may necessitate vessel resection and reconstruction. The aim of this study was to assess... (Review)
Review
Multidisciplinary Oncovascular Surgery is Safe and Effective in the Treatment of Intra-abdominal and Retroperitoneal Sarcomas: A Retrospective Single Centre Cohort Study and a Comprehensive Literature Review.
OBJECTIVE
Radical excision of retroperitoneal or intra-abdominal soft tissue sarcomas may necessitate vessel resection and reconstruction. The aim of this study was to assess surgical results of retroperitoneal or intra-abdominal sarcomas involving major blood vessels.
METHODS
This was a retrospective single centre cohort study and a comprehensive review of literature. Patients with retroperitoneal or intra-abdominal sarcomas treated by the oncovascular team in Helsinki University Hospital from 2010 to 2018 were reviewed for vascular and oncological outcomes. A comprehensive literature review of vascular reconstructions in patients with retroperitoneal sarcoma was performed.
RESULTS
Vascular reconstruction was performed in 17 patients, 11 of whom required arterial reconstructions. Sixteen of the operations were sarcoma resections; the post-operative diagnosis for one patient was thrombosis instead of the presumed recurrent leiomyosarcoma. Early graft thrombosis occurred in two venous and one arterial reconstruction. Late thrombosis was detected in three (18%). The median follow up was 27 (range 0-82) months. Of the patients with sarcoma resections 5 (31%) died of sarcoma and further 4 (25%) developed local recurrence or new distant metastases. The comprehensive review of literature identified 37 articles with 110 patients, 89 of whom had inferior vena cava reconstruction only. Eight arterial reconstructions were described. Late graft thrombosis occurred in 14%. The follow up was 0-181 months, during which 57% remained disease free and 7% died of sarcoma.
CONCLUSION
Vascular reconstructions enable radical resection of retroperitoneal and intra-abdominal sarcomas in patients with advanced disease. The complex operations are associated with an acceptable rate of serious peri-operative complications and symptomatic thrombosis of the repaired vessel is rare. However, further studies are needed to assess the performance of the vascular reconstructions in the long term.
Topics: Adult; Aged; Arteries; Blood Vessel Prosthesis Implantation; Female; Follow-Up Studies; Graft Occlusion, Vascular; Humans; Male; Middle Aged; Neoplasm Staging; Postoperative Complications; Retroperitoneal Neoplasms; Retroperitoneal Space; Retrospective Studies; Sarcoma; Thrombosis; Treatment Outcome; Vascular Patency; Vena Cava, Inferior
PubMed: 32741678
DOI: 10.1016/j.ejvs.2020.05.029 -
Journal of Surgical Oncology Jan 2018Patients with retroperitoneal sarcoma can present to a variety of clinicians with non-specific symptoms and retroperitoneal sarcomas can be incidental findings. Failure...
Patients with retroperitoneal sarcoma can present to a variety of clinicians with non-specific symptoms and retroperitoneal sarcomas can be incidental findings. Failure to recognize retroperitoneal sarcomas on imaging can lead to inappropriate management in non-specialist centers. Therefore it is critical that the possibility of retroperitoneal sarcoma should be considered with prompt referral to a soft tissue sarcoma unit. This review guides clinicians through a diagnostic pathway, introduces concepts in response assessment and new imaging developments.
Topics: Humans; Multimodal Imaging; Retroperitoneal Neoplasms; Sarcoma
PubMed: 29193092
DOI: 10.1002/jso.24891 -
BMC Cancer Sep 2020Myxoid liposarcoma (MLS) has the tendency to metastasize extrapulmonary. Although prognostic factors at the initial diagnosis of MLS have been reported, those at...
BACKGROUND
Myxoid liposarcoma (MLS) has the tendency to metastasize extrapulmonary. Although prognostic factors at the initial diagnosis of MLS have been reported, those at diagnosis of metastasis remain unclear. The purpose of this study was to investigate the prognostic factors for disease-specific survival at the initial diagnosis of metastasis.
METHODS
This retrospective observational study was conducted at three cancer centers and two university hospitals in Japan. Of 274 MLS patients pathologically diagnosed between 2001 and 2015, 48 metastatic patients were examined.
RESULTS
Lung metastases were detected in nine patients (18.8%) and extrapulmonary metastases in 45 (93.8%). Interval from primary diagnosis to the first metastasis was significantly shorter in patients with lung metastases than without (p = 0.007). Median disease-specific survival after diagnosis of metastases was 52.5 months in all patients. In multivariable analysis, liver metastasis (hazard ratio (HR), 2.71 [95% confidence interval (CI), 1.00-7.09]) and no evidence of disease (NED) achieved by radical treatment (resection with or without radiation therapy, or radiation therapy ≥60 Gy) or semi-radical (radiation therapy ≥40 Gy) treatment were significantly related to survival (HR, 0.36; 95%CI [0.13-0.95]). The number of metastases (odds ratio (OR), 0.44; 95%CI [0.25-0.78]) and abdominal/retroperitoneal metastases (OR, 0.09; 95%CI [0.008-0.95]) were the significant inhibitory factors of achieving NED.
CONCLUSIONS
This is the first study to statistically demonstrate the importance of achieving NED with surgical resection or radiation therapy for longer survival in metastatic MLS patients. As number of metastases was a significant factor for achieving NED, early detection of metastases might be important.
Topics: Adult; Aged; Disease-Free Survival; Female; Humans; Japan; Liposarcoma, Myxoid; Liver Neoplasms; Lung Neoplasms; Male; Middle Aged; Neoplasm Metastasis; Neoplasm Recurrence, Local; Prognosis; Retroperitoneal Neoplasms; Retrospective Studies
PubMed: 32928160
DOI: 10.1186/s12885-020-07384-1 -
Oncology (Williston Park, N.Y.) Sep 2018Retroperitoneal sarcoma (RPS) is a rare tumor whose diagnosis and management can be challenging and for which management requires a multidisciplinary team in a...
Retroperitoneal sarcoma (RPS) is a rare tumor whose diagnosis and management can be challenging and for which management requires a multidisciplinary team in a specialized center. An important part of the diagnosis-identification of the histologic subtype-depends on pathology; identifying the histologic subtype is important because this can affect prognosis and treatment options. Complete surgical resection with negative margins remains the cornerstone of treatment of nonmetastatic RPS and is the only chance for cure. In order to achieve negative margins, multivisceral en bloc resection is often necessary. Neoadjuvant therapies (chemotherapy, external beam radiation, or combination radiation and chemotherapy) are safe in well-selected patients and may be considered after careful review by a multidisciplinary sarcoma tumor board when the recurrence risk is high.
Topics: Dissection; Humans; Neoadjuvant Therapy; Neoplasm Staging; Patient Care Team; Patient Selection; Prognosis; Retroperitoneal Neoplasms; Sarcoma
PubMed: 30248168
DOI: No ID Found -
The Surgical Clinics of North America Aug 2022Radiation therapy is an integral component of local management with oncologic resection for soft tissue sarcoma. Radiotherapy is indicated in patients at an increased... (Review)
Review
Radiation therapy is an integral component of local management with oncologic resection for soft tissue sarcoma. Radiotherapy is indicated in patients at an increased risk of local recurrence so that improved local control may be achieved. Sequencing of radiotherapy and resection should be determined by multidisciplinary input before treatment initiation. For most patients, preoperative delivery of radiation therapy is preferred. In patients initially thought to be at low risk for local recurrence and found to have unexpected adverse pathologic features at resection, postoperative radiation therapy is indicated. The use of radiation therapy for retroperitoneal sarcoma is controversial; when used, preoperative delivery of radiation is recommended.
Topics: Humans; Neoplasm Recurrence, Local; Radiotherapy Dosage; Radiotherapy, Adjuvant; Retroperitoneal Neoplasms; Sarcoma; Soft Tissue Neoplasms
PubMed: 35952688
DOI: 10.1016/j.suc.2022.04.001 -
Polish Archives of Internal Medicine Nov 2022
Topics: Humans; Retroperitoneal Neoplasms; Sarcoma
PubMed: 35997239
DOI: 10.20452/pamw.16324