Retroperitoneal sarcoma

Estimated that 13% of soft tissue sarcomas are retroperitoneal in origin.

One third of retroperitoneal malignant tumors are sarcomas.

The prognosis of retroperineal sarcoma is poorer than that of extremity sarcomas.

Liposarcoma and leiomyosarcoma are the most common retroperitoneal sarcoma histologies.

Median age at presentation is 50 years, but can occur at any age and is equally frequent among men and women.

Accounts for 15-20% of soft tissue sarcomas.

Accounts for approximately 0.1% of all malignancies.

Annual incidence of 0.76 new cases per hundred thousand people

Symptoms are usually vague due to the loose areolar tissue of the retroperitoneal space.

Most frequent presenting findings are weight loss, and/or an abdominal pain.

May have lower extremity swelling from pressure on the inferior vena cava or iliac veins.

The most common retroperitoneal sarcoma subtypes are liposarcomas (41%), leiomyosarcoma (28%), malignant fibrous histiocytoma (7%), fibrosarcoma (6%), and malignant peripheral nerve sheath tumors (3%).

Arise from the mesenchymal stem cells usually located in muscle, fat and connective tissues.

Leiomyoma sarcomas occur most commonly during adulthood and in women.

Histologically composed of spindle cells.

Tumor grade is a significant prognostic factor, with higher tumor grade associated with progression to both metastases and death.

Metastases are most common to the lungs and liver and develop in an estimated 50% of patients.

Liposarcomas constitute the most common subtype of retroperitoneal sarcoma constituting approximately 41% of such lesions.

Leiomyosarcoma (28%), malignant fibrous histiocytoma (7%), fibrosarcoma (6%) and malignant peripheral nerve sheath tumor (3%) are he most common subtypes of sarcomas behind liposarcomas.

Lesions are frequently large in size and their location lead to abdominal pain, nausea, vomiting, anorexia, weight loss, fatigue, and malaise.

Approximately half of the lesions are high grade.

Prognosis  factors include tumor size and multifocalitu,  patient age and comorbidities, and completeness of resection.

Surgery most effective modality for the treatment.

Complete surgical resection provides a survival benefit.

Median survival of patients with retroperitoneal sarcomas who undergo exploration and biopsy alone is invariably less than 1 year.

Given the complex anatomy of the retroperitoneal space and adjacent normal tissues, achieving wide negative margins is often impossible.

An appropriate balance between preservation of organ function and risk of local recurrence is the key consideration of therapy.

Local recurrence is frequent even with complete microscopic resection.

Incomplete restrictions are not curative.

Overall survival for patients undergoing definitive surgery at 5 years is about 46% and about 27% for 10 years.

Distant metastases are the main cause of treatment failure in patients with nonlipomatous retroperitoneal sarcomas.

Liposarcomas have a high local failure rate.

Liposarcoma patients have a tumor size at presentation more than double the size of other histological type sarcoma tumors.

Preoperative radiotherapy is not efficacious treatment for retroperitoneal sarcoma.

Postoperative radiation is associated with a reduced risk of recurrence, while chemotherapy is not beneficial.

The used of postoperative radiation is controversial with mixed results.

High-grade tumors associated with shorter survival recurrence-free survival and overall survival.

Virtually all lesions are greater than 5 cm at presentation so that tumor size is not a good predictor of survival.

CT is the most useful radiographic imaging study in the evaluation of a retroperitoneal tumor by defining its location, identifies its relationship to surrounding tissue as and can indicate the presence of metastases.

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