Clinical lar approaches to well differentiated and dedifferentiated liposarcoma

Sarcoma Disease Management Program, Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, NY 10065, USA.
Current opinion in oncology (Impact Factor: 3.76). 07/2011; 23(4):373-8. DOI: 10.1097/CCO.0b013e32834796e6
Source: PubMed

ABSTRACT Liposarcoma, a rare disease, is classified into five histologic subtypes. These include well differentiated liposarcoma (WDLS) and dedifferentiated liposarcoma (DDLS), both characterized by chromosome 12q13-15 amplification. This review will focus on the clinical management of WDLS and DDLS and examine recent molecular studies that have the potential to affect clinical management.
Outcome of patients with WDLS and DDLS depends on completeness of surgical resection as well as tumor location and histologic subtype. Risk of recurrence is high for patients with dedifferentiated histology or retroperitoneal location. We now understand that surgical outcomes are poor for patients with rapidly growing or incompletely resectable tumors, so these patients should be managed nonoperatively. Radiation and chemotherapy have low response rates in WDLS and DDLS, but novel agents targeted at chromosome 12 gene products MDM2 and CDK4 have shown promise in preclinical studies and are being tested in clinical trials. Cell line, tissue microarray, and genomic analyses have identified additional targets including ZIC1, TOP2A, AURKA, and IGF-1R, which could form the basis of future therapies.
Although complete surgical resection is currently the most effective treatment for WDLS and DDLS, the majority of patients with retroperitoneal liposarcoma will eventually have recurrence and die of disease. It is hoped that a multimodality approach, which incorporates targeted therapies and complete surgical resection, will significantly improve patient outcomes.

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    • "Primary surgical resection, where technically possible , is the mainstay of treatment, often involving extensive surgical procedures in specialist units, with en bloc multivisceral resections [5]. On development of disease recurrence further surgical excision may be performed after taking into account the disease extent and time since primary surgery but until recently treatment options in multiply recurrent, inoperable, or metastatic disease have been limited [6]. "
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    Surgical Oncology Clinics of North America 04/2012; 21(2):333-40. DOI:10.1016/j.soc.2011.12.007 · 1.67 Impact Factor
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    ABSTRACT: This is a report of the surgical treatment and prognosis of retroperitoneal liposarcoma (RPLS) in Koreans. Nineteen patients treated for RPLS between July 1, 1984, and March 31, 2009, were included. Patient demographics, histopathologic subtypes, survival rate, disease recurrence rate and interval, and adjuvant therapy were reviewed and analyzed. Of the 19 patients diagnosed with RPLS, 26.3% presented with well-differentiated RPLS, 10.5% with dedifferentiated RPLS, 15.8% with myxoid/round cell type, and 47.4% with mixed-type liposarcoma. The mean follow-up period was 66.8 months (range, 6 to 165 months). Primary RPLS was treated in 17 patients. Nine patients (52.9%) had recurrent disease, and recurrence developed at a mean of 47.7 months after primary or repeated surgical treatment. The overall survival rate was 84.2% during a mean follow-up of 66.8 months. The 3- and 5-year survival rates were 86.9%, and the 10-year survival rate was 69.5%. The recurrence interval was significantly shorter in recurrent RPLS cases (p=0.023). The mean growth rate of locally recurrent tumors was 0.34 cm per month. The survival rates reported here were higher than in previous studies. Locally recurrent tumors presented with a low growth rate, which may have contributed to the relatively high survival rate. A high prevalence of mixed-type RPLS was also noted, and its cause and prognosis require further research.
    Korean journal of urology 05/2012; 53(5):310-6. DOI:10.4111/kju.2012.53.5.310
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