English (United Kingdom)

https://curated-unify.zendy.io/wp-json/zendy-region/v1/featured_content/oa?rat=en

https://curated-unify.zendy.io/wp-json/zendy-region/v1/highlighted_journal/

Global: Zendy Plus annual

Presents the access of premium content as premium feature

Premium Content

Presents the keyphrase highlighting as premium feature

Keyphrase Highlighting

Presents the summarisation as premium feature

Summarisation

Insights

Presents the pdf analysis as premium feature

PDF Analysis

Presents the zaia usage as premium feature

ZAIA

Global: Zendy Tools annual

Global: Zendy Free Plan

Global: Zendy Tools monthly

Global: Zendy Plus monthly

Liposarcoma is a malignancy of fat that commonly develops in the retroperitoneum (1,2). In this anatomic location, tumors can be low grade, well differentiated (WD) or have an additional high grade, dedifferentiated (DD) component. Entirely WD tumors are typically indolent and do not metastasize, while tumors with a DD component are more aggressive and have distant metastatic potential. WD and DD liposarcoma can be regarded as opposite ends of a spectrum of disease aggressiveness (e.g., lipoma-like WD to rhabdomyoblastic DD) (3). For patients with retroperitoneal (RP) liposarcoma, high resolution, contrast enhanced CT of the abdomen and pelvis is critical as part of the initial evaluation (4). This should be performed if not done already at the time of referral. WD liposarcomas are well-defined round or lobulated masses predominantly composed of macroscopic fat with few, thin septations (5). Non-fatty, soft tissue density areas with a ground glass appearance can occur and typically constitute less than 25% of the tumor (6). DD areas manifest as discrete nodular soft tissue density areas often with good demarcation from surrounding fat and show variable contrast enhancement. Internal septations are thicker (>2 mm) in DD. Calcifications may also be seen and would suggest DD liposarcoma if found in the nonfatty areas. For patients with suspected DD disease, CT of the chest should be obtained as part of staging to rule out distant metastasis to the lungs (4). To confirm the diagnosis of RP liposarcoma, percutaneous core needle biopsy can be performed. Biopsy may not always be necessary, especially in cases in which the radiologic features highly suggest the diagnosis. On histopathologic examination, WD liposarcoma consists of adipocytes of varying size with fibrous septae and interspersed hyperchromatic cells (7). DD areas are typically juxtaposed next to WD areas with an abrupt transition from the adipocytic (WD) to non-adipocytic, high grade appearing areas containing prominent mitotic figures (DD). Both WD and DD liposarcoma have amplification of 12q13-15 which includes several important genes such as MDM2 and CDK4. This molecular feature is considered pathognomonic and can be used for the definitive diagnosis if the histologic features alone are unclear. For all patients with nonmetastatic retroperitoneal liposarcoma, surgery is the mainstay of treatment. However, in the retroperitoneum, tumors are frequently massive in size and can potentially invade critical organs and major blood vessels, making surgery very difficult. Whether due to technical factors (e.g., “positive” margins) or disease biology [e.g., “field defect” (8)], recurrence rates after resection are high and do not plateau over time (9). Radiation therapy may offer improved local control, but the true benefit for retroperitoneal liposarcoma remains controversial (10). Sarcoma MDT Series

Multidisciplinary sarcoma tumor board: retroperitoneal liposarcoma