title: Rare Aggressive Behavior ofMDM2-Amplified Retroperitoneal Dedifferentiated Liposarcoma, with Brain, Lung and Subcutaneous Metastases.
abstract: Dedifferentiated liposarcoma (DDL) is a histologically pleomorphic sarcoma, traditionally defined as well-differentiated liposarcoma with abrupt transition to high grade, non-lipogenic sarcoma. It can occur as part of recurrent well-differentiated liposarcoma, or may arisede 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 showedMDM2amplification with fluorescencein situhybridization, 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 inMDM2-amplified retroperitoneal liposarcoma.
authors.affiliation: Sarcoma Unit, Royal Marsden Hospital , London, UK.
authors.forename: Imen
authors.initials: I
authors.lastname: Ben Salha
authors.affiliation: Sarcoma Unit, Royal Marsden Hospital , London, UK.
authors.forename: Shane
authors.initials: S
authors.lastname: Zaidi
authors.affiliation: Sarcoma Unit, Royal Marsden Hospital , London, UK.
authors.forename: Jonathan
authors.initials: J
authors.lastname: Noujaim
authors.affiliation: Sarcoma Unit, Royal Marsden Hospital , London, UK.
authors.forename: Aisha B
authors.initials: AB
authors.lastname: Miah
authors.affiliation: Sarcoma Unit, Royal Marsden Hospital , London, UK.
authors.forename: Cyril
authors.initials: C
authors.lastname: Fisher
authors.affiliation: Sarcoma Unit, Royal Marsden Hospital , London, UK.
authors.forename: Robin L
authors.initials: RL
authors.lastname: Jones
authors.affiliation: Sarcoma Unit, Royal Marsden Hospital , London, UK.
authors.forename: Khin
authors.initials: K
authors.lastname: Thway
keywords: Dedifferentiated liposarcoma; MDM2 amplification; genetics; pathology; sarcoma