Co-Targeting of MDM2 and CDK4/6 with Siremadlin and Ribociclib for the Treatment of Patients with Well-Differentiated or Dedifferentiated Liposarcoma: Results From a Proof-of-Concept, Phase Ib Study

2021 ◽  
pp. clincanres.1291.2021
Author(s):  
Albiruni R. Abdul Razak ◽  
Sebastian Bauer ◽  
Cristina Suarez ◽  
Chia-Chi Lin ◽  
Richard Quek ◽  
...  
Keyword(s):  
Dedifferentiated Liposarcoma ◽  
Proof Of Concept ◽  
Phase Ib ◽  
Well Differentiated

scholarly journals Biology and Management of Dedifferentiated Liposarcoma: State of the Art and Perspectives

2021 ◽  
Vol 10 (15) ◽  
pp. 3230
Author(s):  
Jun Nishio ◽  
Shizuhide Nakayama ◽  
Kazuki Nabeshima ◽  
Takuaki Yamamoto
Keyword(s):  
Standard Treatment ◽  
Clinical Presentation ◽  
Current Knowledge ◽  
Single Agent ◽  
Dedifferentiated Liposarcoma ◽  
Cyclin Dependent Kinase ◽  
First Line ◽  
First Line Therapy ◽  
Well Differentiated ◽  
Genetic Events

Dedifferentiated liposarcoma (DDL) is defined as the transition from well-differentiated liposarcoma (WDL)/atypical lipomatous tumor (ALT) to non-lipogenic sarcoma, which arises mostly in the retroperitoneum and deep soft tissue of proximal extremities. It is characterized by a supernumerary ring and giant marker chromosomes, both of which contain amplified sequences of 12q13-15 including murinedouble minute 2 (MDM2) and cyclin-dependent kinase 4 (CDK4) cell cycle oncogenes. Detection of MDM2 (and/or CDK4) amplification serves to distinguish DDL from other undifferentiated sarcomas. Recently, CTDSP1/2-DNM3OS fusion genes have been identified in a subset of DDL. However, the genetic events associated with dedifferentiation of WDL/ALT remain to be clarified. The standard treatment for localized DDL is surgery, with or without radiotherapy. In advanced disease, the standard first-line therapy is an anthracycline-based regimen, with either single-agent anthracycline or anthracycline in combination with the alkylating agent ifosfamide. Unfortunately, this regimen has not necessarily led to a satisfactory clinical outcome. Recent advances in the understanding of the pathogenesis of DDL may allow for the development of more-effective innovative therapeutic strategies. This review provides an overview of the current knowledge on the clinical presentation, pathogenesis, histopathology and treatment of DDL.

scholarly journals Genomic profiling of dedifferentiated liposarcoma compared to matched well-differentiated liposarcoma reveals higher genomic complexity and a common origin

2018 ◽  
Vol 4 (2) ◽  
pp. a002386 ◽  
Author(s):  
Hannah C. Beird ◽  
Chia-Chin Wu ◽  
Davis R. Ingram ◽  
Wei-Lien Wang ◽  
Asrar Alimohamed ◽  
...  
Keyword(s):  
Common Origin ◽  
Dedifferentiated Liposarcoma ◽  
Genomic Profiling ◽  
Genomic Complexity ◽  
Well Differentiated

Dedifferentiated Liposarcoma With a Paraganglioma-like Histologic Pattern: A Case Report and Review of the Literature

2004 ◽  
Vol 128 (7) ◽  
pp. 788-791
Author(s):  
Dating Liu ◽  
Guillermo Quinonez ◽  
Steven Latosinsky
Keyword(s):  
Neuron Specific Enolase ◽  
Malignant Neoplasm ◽  
Neuroendocrine Differentiation ◽  
Growth Patterns ◽  
Dedifferentiated Liposarcoma ◽  
Tissue Mass ◽  
Histologic Pattern ◽  
History Of ◽  
Poorly Differentiated ◽  
Well Differentiated

Abstract A 53-year-old man presented with a 4-month history of increasing abdominal discomfort and distension. A large retroperitoneal mass was found on imaging. Image-guided needle core biopsy demonstrated a poorly differentiated malignant neoplasm. A 30 × 32 × 33-cm soft tissue mass was removed. Microscopically, the tumor consisted of predominantly epithelioid malignant cells arranged in a paraganglioma-like growth pattern. Immunohistochemically, these cells were strongly positive for neuron-specific enolase. Stains for synaptophysin and chromogranin, however, were negative. There was no ultrastructural evidence of neuroendocrine differentiation. Adjacent sarcomatous areas were composed of spindled cells arranged in storiform and fibrosarcoma-like growth patterns. A small area of well-differentiated liposarcoma was identified, and a diagnosis of dedifferentiated liposarcoma was established. To the best of our knowledge, this represents the first reported case of dedifferentiated liposarcoma with a paraganglioma-like histologic pattern. A brief review focusing on the morphologic variations of dedifferentiated liposarcoma is also presented.

scholarly journals PET/CT Imaging as a Diagnostic Tool in Distinguishing Well-Differentiated versus Dedifferentiated Liposarcoma

Sarcoma ◽  
2020 ◽  
Vol 2020 ◽  
pp. 1-6
Author(s):  
Amanda Parkes ◽  
Elizabeth Urquiola ◽  
Priya Bhosale ◽  
Heather Lin ◽  
Kelsey Watson ◽  
...  
Keyword(s):  
Diagnostic Tool ◽  
Standardized Uptake Value ◽  
Percutaneous Biopsy ◽  
Cutoff Point ◽  
Dedifferentiated Liposarcoma ◽  
Positron Emission ◽  
Pet Ct ◽  
Well Differentiated ◽  
Low Sensitivity ◽  
Cart Algorithm

Distinguishing well-differentiated liposarcoma (WDLPS) from dedifferentiated liposarcoma (DDLPS) is essential given distinct treatment paradigms and chemosensitivity. Percutaneous biopsy has a low sensitivity for detecting DDLPS. We sought to identify the diagnostic utility of positron emission tomography/computed tomography (PET/CT) in identifying WDLPS versus DDLPS. An independent radiologist reviewed PET/CT images to identify target lesions and determine the maximum standardized uptake value (SUVmax). An independent pathologist review confirmed WDLPS or DDLPS histology. A binary cutoff point of SUVmax was identified using a classification and regression trees (CART) algorithm. We identified 20 patients with WDLPS or DDLPS with 26 PET/CTs performed for separate recurrences that were followed by surgical sampling. Of the 26 records, 12 were DDLPS (46%) and 14 were WDLPS (54%). Patients with DDLPS had significantly higher SUVmax than those with WDLPS (p value = 0.0035). A SUVmax of 4 was identified as the cutoff point. Using this cutoff, the sensitivity of SUVmax identifying a case as DDLPS was 83.3% (95% CI: 51.6%, 97.9%) and the specificity was 85.7% (95% CI: 57.2%, 98.2%). PET/CT is a sensitive and specific diagnostic tool to identify the presence of dedifferentiation within the tumor.

scholarly journals Dedifferentiated Liposarcoma Arising in an Esophageal Polyp: A Case Report

2012 ◽  
Vol 2012 ◽  
pp. 1-3 ◽  
Author(s):  
Jorge Torres-Mora ◽  
Ann Moyer ◽  
Mark Topazian ◽  
Jeffrey Alexander ◽  
Tsung-Teh Wu ◽  
...  
Keyword(s):  
In Situ Hybridization ◽  
Case Report ◽  
Fluorescence In Situ Hybridization ◽  
Gene Amplification ◽  
Dedifferentiated Liposarcoma ◽  
Well Differentiated

Liposarcoma is one of the most common sarcomas in adults, but only rarely presents as an esophageal primary. There have been several reports of well-differentiated liposarcoma (WDL) arising in the esophagus, but we present a case of dedifferentiated liposarcoma (DL) presenting as a large esophageal polyp. We believe this is the first reported case of DL of the esophagus with morphologic evidence of both well-differentiated and dedifferentiated components. The diagnosis was confirmed by demonstration of CPM gene amplification by fluorescence in situ hybridization (FISH).

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