Retroperitoneal sarcomas: from diagnosis to treatment. Case series and review of the literature.

Abstract

Background: Retroperitoneal sarcoma is a rare malignancy arising from mesenchymal cells, most commonly presented as an abdominal mass and is associated with poor prognosis. The most effective treatment modality for retroperitoneal sarcomas is complete surgical resection, including sometimes adjacent organs infiltrated by the tumor. Radiotherapy is frequently applied and has shown some benefit, while the role of chemotherapy and molecular-targeted agents is still not clear. Local recurrence is common for retroperitoneal sarcomas and still remains the main cause of death. The major factors associated with the overall survival are tumor grade, histological subtype, complete macroscopic excision and multifocality.

Aim: To report our experience via the presentation of patients with retroperitoneal sarcomas managed in our department during the period 2014-18; and to review the current literature.

Patients and methods: Eight patients appeared with chronic non-specific complaints including abdominal distension and changes in bowel or bladder habit, while one patient presented with acute abdominal pain due to mass rupture. All of the patients underwent surgical resection of the tumor.

Results: Among the patients, seven were operated for primary disease and one only for recurrent. The most common histologic type was liposarcoma (well-differentiated, dedifferentiated), found in five patients; followed by leiomyosarcoma found in two cases. Fibrous histiocytoma was found in only one case. The masses were removed with macroscopically clear margins (R0 and R1 resections) in four cases. In five patient cases adjuvant therapy was required. Three patients are still alive and free of disease.

Conclusions: Retroperitoneal sarcomas present to be a therapeutic challenge based on their location, their extent at the time of diagnosis and the high risk of local recurrence or distant metastasis. Their management requires a multidisciplinary approach, with the surgical resection remaining the mainstay of curative treatment, combined with surveillance for early detection of recurrence or metastases.