Rare Aggressive Behavior of MDM2-Amplified Retroperitoneal Dedifferentiated Liposarcoma, with Brain, Lung and Subcutaneous Metastases.

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Authors

Ben Salha, I
Zaidi, S
Noujaim, J
Miah, AB
Fisher, C
Jones, RL
Thway, K

Document Type

Journal Article

Date

2016-09-05

Date Accepted

2016-02-25

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 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.

Citation

Rare tumors, 2016, 8 (3), pp. 6282 - ?

Source Title

Publisher

SAGE PUBLICATIONS LTD

ISSN

2036-3605

eISSN

2036-3613

Research Team

Sarcoma Clinical Trials (R Jones)
Targeted Therapy

Notes