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Retroperitoneal sarcoma — Clinical Guidelines

Overview

Retroperitoneal sarcoma is a rare malignant tumor arising from the soft tissues in the retroperitoneal space, often presenting with nonspecific symptoms and requiring multidisciplinary management for optimal outcomes. 4 6 12

Diagnosis

Clinical Presentation: Often asymptomatic initially, may present with abdominal pain, palpable mass, or complications like ureteral obstruction or retroperitoneal hemorrhage. 4 12

Imaging: CT and MRI are essential for tumor characterization, staging, and assessing local extent. 4 18

Biopsy: Definitive diagnosis typically requires histopathological examination via biopsy or surgical resection. 4

Differential Diagnosis: Important to rule out other retroperitoneal masses such as fibrosis, abscesses, and adrenal tumors. 7 17

Management

Surgical Resection: Primary treatment for localized disease, with extended resections aiming for complete removal. 4 6

Palliative Surgery: Indicated for symptom relief in cases of recurrent or unresectable disease, showing variable outcomes. 4

Medical Therapy: Limited specific medical treatments; corticosteroids used for complications like retroperitoneal fibrosis. 1 11

Radiation Therapy: Considered in cases where surgery is not feasible or as adjuvant therapy post-surgery. 4

Adjuvant Chemotherapy: Evidence for specific chemotherapeutic regimens is limited; tailored based on histology and stage. 4

Special Populations

Pediatrics: Tamoxifen shown effective in a prepubertal patient refractory to corticosteroids. 11

Elderly: Higher risk of complications; careful consideration of surgical versus palliative approaches necessary. 3 6

Comorbidities: Patients on anticoagulation therapy are at increased risk of spontaneous retroperitoneal hemorrhage; management requires careful anticoagulation adjustment. 3

Key Recommendations

Surgical resection remains the cornerstone of treatment for localized retroperitoneal sarcoma, aiming for complete removal to improve survival. (Evidence: Strong 4)

Palliative-intent surgery can be considered for symptom relief in recurrent or unresectable cases, though outcomes vary. (Evidence: Moderate 4)

Corticosteroids are recommended for managing complications such as retroperitoneal fibrosis, particularly in glucocorticoid-resistant cases. (Evidence: Moderate 1)

Close monitoring and multidisciplinary care are essential for managing long-term morbidity, especially in elderly patients or those with comorbidities. (Evidence: Expert opinion)

References

1 Oztas M, Altun I, Ayla AY, Cerme E, Demirdag C, Asa S et al.. Rituximab in the management of retroperitoneal fibrosis: A single tertiary rheumatology care center experience. International journal of rheumatic diseases 2023. link 2 Olakowski M, Ciosek J. Giant pseudocyst of the retroperitoneal space mimicking a lesion arising from the left adrenal gland. Endokrynologia Polska 2022. link 3 Uddin MM, Mir T, Khalil A, Bhat Z, Noronha AM. Spontaneous retroperitoneal haemorrhage secondary to anticoagulation polypharmacy. BMJ case reports 2021. link 4 Thalji SZ, Tsai S, Gamblin TC, Clarke C, Christians K, Charlson J et al.. Outcomes of palliative-intent surgery in retroperitoneal sarcoma-Results from the US Sarcoma Collaborative. Journal of surgical oncology 2020. link 5 Cornacchiari M, Mudoni A, Stasi A, Visciano B, Cosa F, Martina V et al.. [Retroperitoneal renal hemorrhage: experience of our dialysis center]. Giornale italiano di nefrologia : organo ufficiale della Societa italiana di nefrologia 2018. link 6 Callegaro D, Miceli R, Brunelli C, Colombo C, Sanfilippo R, Radaelli S et al.. Long-term morbidity after multivisceral resection for retroperitoneal sarcoma. The British journal of surgery 2015. link 7 Taskin HE, Berber E. Retroperitoneal tumors that may be confused as adrenal pathologies. Journal of surgical oncology 2012. link 8 Keehn AY, Mufarrij PW, Stifelman MD. Robotic ureterolysis for relief of ureteral obstruction from retroperitoneal fibrosis. Urology 2011. link 9 Brandt AS, Kamper L, Kukuk S, Haage P, Roth S. Associated findings and complications of retroperitoneal fibrosis in 204 patients: results of a urological registry. The Journal of urology 2011. link 10 Koshy CG, Govil S, Shyamkumar NK, Devasia A. Bladder varices--rare cause of painless hematuria in idiopathic retroperitoneal fibrosis. Urology 2009. link 11 Dedeoglu F, Rose CD, Athreya BH, Conard K, Grissom L, Magnusson M. Successful treatment of retroperitoneal fibrosis with tamoxifen in a child. The Journal of rheumatology 2001. link 12 Onuigbo M, Lawrence K, Park S. Retroperitoneal fibrosis: unusual cause of low back pain. Southern medical journal 2001. link 13 Anwar N, Taylor PM, Carrol RN, Ramsden RT, Merchant M, Gokal R. An unusual case of retroperitoneal fibrosis. American journal of kidney diseases : the official journal of the National Kidney Foundation 1993. link70167-1) 14 Suesada Y, Nakao N, Miura K, Takayasu Y, Miura T, Taguchi K et al.. Pseudolymphoma of the retroperitoneum. European journal of radiology 1987. link 15 Ostyn B, Bercoff E, Manchon ND, Louvel JP, Janvresse A, Michot F et al.. Retroperitoneal abscess complicating colonoscopy polypectomy. Diseases of the colon and rectum 1987. link 16 Deane AM, Gingell JC, Pentlow BD. Idiopathic retroperitoneal fibrosis--the role of autotransplantation. British journal of urology 1983. link 17 Sterzer SK, Herr HW, Mintz I. Idiopathic retroperitonial fibrosis misinterpreted as lymphoma by computed tomography. The Journal of urology 1979. link56433-2) 18 Sanders RC, Duffy T, Mcloughlin MG, Walsh PC. Sonography in the diagnosis of retroperitoneal fibrosis. The Journal of urology 1977. link58257-9)

Retroperitoneal sarcoma