Rare aggressive behavior of MDM2-amplified retroperitoneal dedifferentiated liposarcoma, with brain, lung and subcutaneous metastases

Rare Tumors

Robin L. Jones¹  Aisha B. Miah¹  Shane Zaidi¹  Cyril Fisher¹  Khin Thway¹  Imen Ben Salha¹  Jonathan Noujaim¹ 

[1] Sarcoma Unit, Royal Marsden Hospital, London;

关键词: Dedifferentiated liposarcoma;    sarcoma;    pathology;    genetics;    MDM2 amplification;   

DOI  :  10.4081/rt.2016.6282

来源: DOAJ

【 摘 要 】

Dedifferentiated liposarcoma (DDL) is a histologically pleomorphic sarcoma, traditionally defined as well-differentiated liposarcoma with abrupt transition to high grade, nonlipogenic sarcoma. It can occur as part of recurrent well-differentiated liposarcoma, or may arise de novo. DDL most frequently occurs within the retroperitoneum, and while it is prone to local recurrence, it usually has a lower rate of metastasis than other pleomorphic sarcomas. We describe a case of retroperitoneal dedifferentiated liposarcoma in a 63-year-old male, who showed MDM2 amplification with fluorescence in situ hybridization, which displayed unusually aggressive behavior, with brain, lung and subcutaneous soft tissue metastases. As previous reports of metastatic liposarcoma have largely grouped DDL in with other (genetically and clinically distinct) liposarcoma subtypes, we highlight and discuss the rare occurrence of brain metastasis in MDM2-amplified retroperitoneal liposarcoma.

【 授权许可】

Unknown