Retroperitoneal Liposarcoma

Abstract

Retroperitoneal liposarcomas are rare mesenchymal tumors of the retroperitoneum that typically present with advanced disease and often carry a poor prognosis. Because of their rarity and anatomic location, these malignant tumors can cause a diagnostic dilemma and present several therapeutic challenges. They are usually associated with a high rate of recurrence despite grossly complete resection, thus requiring long-term and often indefinite follow-up. Relevant data on this topic was procured and synthesized with the aid of a comprehensive Medline search in addition to oncologic, pathologic, urologic, radiologic, and surgical literature review on retroperitoneal sarcomas. This article provides an in-depth review into the natural history, pathology, clinical manifestations, and prognostic features of retroperitoneal liposarcomas. It also discusses the reliability of diagnostic procedures and novel curative approaches that are currently being evaluated for the disease.

Massive retroperitoneal dedifferentiated liposarcoma in a young patient

“…However, when they produce symptoms they are usually non-specific; abdominal pain, and early satiety being the most common. Local invasion of the retroperitoneal structures can produce neurological, musculoskeletal and obstructive bowel disorders [ 2 , 3 ]. CT is the gold standard when it comes to diagnosis, staging and preoperative evaluation of DDLs [ 2 ].…”

“…Retroperitoneal liposarcomas are potentially curable with complete resection of the tumor with negative margins [ 9 ] nevertheless complete resection of these tumors is often challenging, as these lesions are very large when diagnosed and may involve many adjacent organs and structures due to the tumor size [ 2 , 6 ]. The prognostic factor most consistently associated with survival and recurrence is the complete surgical resection with negative margins [ 10 ].…”

“…The prognostic factor most consistently associated with survival and recurrence is the complete surgical resection with negative margins [ 10 ]. That being said due to the highly aggressive nature of retroperitoneal DDLs complementary therapy is usually necessary in spite of obtaining clear surgical margins [ 1 , 2 ].…”

“…Soft tissue sarcomas are an extremely rare malignancy accounting only 1% of all tumors in adult patients [ 1 ]. Liposarcomas are the most common type of sarcomas arising in the retroperitoneum that could become life-threatening [ 2 , 3 ]. These tumors can grow to a massive size and involve adjacent structures that could make their control even more difficult [ 4 ].…”

A novel long noncoding RNA PILRLS promote proliferation through TCL1A by activing MDM2 in Retroperitoneal liposarcoma

“…But, the majority of patients with high-grade RLS will develop locally recurrent following surgery, and this constitutes the cause of death in most patients [3, 4]. In spite of the progress in chemotherapy, radiotherapy and surgical techniques for RLS in recent years, the survival rate of RLS patients remains unsatisfactory [5, 6]. As a consequence, to explore the new insights and therapy targets based on histology, molecular biology, and systemic treatment were urgently needed in RLS.…”