Rare Aggressive Behavior of MDM2-Amplified Retroperitoneal Dedifferentiated Liposarcoma, with Brain, Lung and Subcutaneous Metastases
Author(s) -
Imen Ben Salha,
Shane Zaidi,
Jonathan Noujaim,
Aisha Miah,
Cyril Fisher,
Robin L. Jones,
Khin Thway
Publication year - 2016
Publication title -
rare tumors
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.285
H-Index - 15
eISSN - 2036-3613
pISSN - 2036-3605
DOI - 10.4081/rt.2016.6282
Subject(s) - liposarcoma , medicine , pathology , soft tissue sarcoma , sarcoma , myxoid liposarcoma , metastasis , fluorescence in situ hybridization , brain metastasis , lung , soft tissue , cancer , biology , gene , biochemistry , chromosome
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
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