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Retroperitoneal Liposarcoma

 

 

Related narrative: Retroperitoneal Liposarcoma

Retroperitoneal sarcomas are rare tumors that tend to attain tremendous size before recognition due to their location. The most common histologic subtype is the liposarcoma; others include malignant fibrous histiocytoma and leiomyosarcoma.

Retroperitoneal liposarcomas tend to occur in the 40-60 year old age group with a slightly male preponderance. The most common presenting symptom is a palpable abdominal mass, followed by vague abdominal pain. Symptoms may also occur from compression of adjacent viscera, such as the duodenum, kidney, ureter, or pancreas. The majority of patients present with a mass larger than 10 cm.

At the time of presentation, approximately 80% of these tumors are surgically respectable. Complete surgical resection confers a survival advantage; therefore, a liberal policy of en bloc resection to include contiguous organs should be employed. In one of the largest series published to date, the median survival of patients who underwent complete resection was 103 months. The median survival of patients who underwent incomplete resection was 18 months, which was the same as for those patients in whom no resection at all was performed. Partial resection, therefore, should be reserved only for a palliative intent.

Retroperitoneal sarcomas have very high local recurrence rates, reported in the literature anywhere from 40% to 80%. In contrast to extremity sarcomas, in which mortality is almost always a consequence of distant metastases, retroperitoneal liposarcomas tend to cause death from local recurrence. Because of this, patients should be liberally re-explored when recurrence occurs. The median survival after local recurrence is 60 months in resected patients, compared to 20 months in unresected patients.

The role of radiation therapy in the treatment of these tumors is unclear. In the neoadjuvant setting, it may shrink large tumors and allow for a lesser resection. Adjuvant postoperative radiation has been shown to decrease the rate of local recurrence. The use of intraoperative brachytherapy is under investigation.

References:

Lewis JJ, et al: Retroperitoneal soft-tissue sarcoma: Analysis of 500 patients treated and followed at a single institution. Ann Surg 228: 355-365, 1998

Moley JF, Eberlein TJ: Soft-tissue sarcomas. Surg Clin North Am 80(2): 687-708, 2000

Karakousis CP, et al: Retroperitoneal sarcomas and their management. Arch Surg 130(10): 1104-1109, 1995

Witz M, et al: Diagnosis and treatment of primary and recurrent retroperitoneal liposarcoma. J Surg Onc 47: 41-44, 1991


This page was last modified on 1-Mar-02.