scholarly journals Double minute chromosomes harboring MDM2 amplification in a pediatric atypical lipomatous tumor

2019 ◽  
Author(s):  
Bérengère Dadone‐Montaudié ◽  
Fanny Burel‐Vandenbos ◽  
Christine Soler ◽  
Olivier Rosello ◽  
Corinne Boyer ◽  
...  
Keyword(s):  
Atypical Lipomatous Tumor ◽  
Lipomatous Tumor ◽  
Double Minute ◽  
Mdm2 Amplification ◽  
Double Minute Chromosomes

scholarly journals MDM2 Amplified Sarcomas: A Literature Review

Diagnostics ◽  
2021 ◽  
Vol 11 (3) ◽  
pp. 496
Author(s):  
Raf Sciot
Keyword(s):  
Suppressor Gene ◽  
Negative Regulator ◽  
Low Grade ◽  
P53 Mutations ◽  
Lipomatous Tumor ◽  
Double Minute ◽  
Genetic Features ◽  
Murine Double Minute ◽  
Well Differentiated ◽  
Mdm2 Amplification

Murine Double Minute Clone 2, located at 12q15, is an oncogene that codes for an oncoprotein of which the association with p53 was discovered 30 years ago. The most important function of MDM2 is to control p53 activity; it is in fact the best documented negative regulator of p53. Mutations of the tumor suppressor gene p53 represent the most frequent genetic change in human cancers. By overexpressing MDM2, cancer cells have another means to block p53. The sarcomas in which MDM2 amplification is a hallmark are well-differentiated liposarcoma/atypical lipomatous tumor, dedifferentiated liposarcoma, intimal sarcoma, and low-grade osteosarcoma. The purpose of this review is to summarize the typical clinical, histopathological, immunohistochemical, and genetic features of these tumors.

Atypical Spindle Cell Lipomatous Lesion Resected From Patient With History of CLL

2020 ◽  
Vol 86 (9) ◽  
pp. 1208-1211
Author(s):  
Chase J. Wehrle ◽  
J. Will Daigle ◽  
Asad Ullah ◽  
Suash Sharma ◽  
Edmond F. Ritter ◽  
...  
Keyword(s):  
Spindle Cell ◽  
Lymphocytic Leukemia ◽  
Left Shoulder ◽  
Spindle Cell Lipoma ◽  
Atypical Lipomatous Tumor ◽  
Lipomatous Tumor ◽  
Adipocytic Tumors ◽  
History Of ◽  
Pathologic Analysis ◽  
Mdm2 Amplification

Atypical spindle cell lipomatous neoplasm, also known as well-differentiated spindle cell liposarcoma, represents a newly discovered entity of adipocytic tumors. Recent research has shown this tumor variant to be more related to spindle cell lipoma, rather than the originally hypothesized atypical lipomatous tumor spectrum. Here we present a case of a 58-year-old man with a history of chronic lymphocytic leukemia with an enlarging mass on the posterior left shoulder, initially hypothesized to be a benign lipoma. However, magnetic resonance imaging showed a large, multiseptated, heterogeneous mass concerning for soft tissue sarcoma. After resection, pathologic analysis showed cells closely resembling spindle cell lipoma, with additional cellular and fascicular zones containing lipoblasts and mitotic figures. Molecular analysis showed no MDM2 amplification. This lack of amplification indicates this tumor is distinctly different from an atypical lipomatous tumor, which characteristically displays MDM2 amplification. However, tumor expression of RB1 was normal. The majority of atypical spindle cell lipomatous neoplasms are associated with RB1 deletions. We conclude that we have a unique example of an atypical spindle cell lipomatous tumor.

Atypical Lipomatous Tumor/Well-Differentiated Liposarcoma With Features Mimicking Spindle Cell Lipoma

2019 ◽  
Vol 28 (3) ◽  
pp. 336-340 ◽  
Author(s):  
Judith A. S. Jebastin ◽  
Kyle D. Perry ◽  
Dhananjay A. Chitale ◽  
Michael P. Mott ◽  
Jessica Sanchez ◽  
...  
Keyword(s):  
Spindle Cell ◽  
Spindle Cell Lipoma ◽  
Rb1 Gene ◽  
Prognostic Features ◽  
Atypical Lipomatous Tumor ◽  
Lipomatous Tumor ◽  
Well Differentiated ◽  
Mdm2 Amplification ◽  
Lipomatous Tumors

Atypical lipomatous tumor/well-differentiated liposarcoma (ALT/WDL) and spindle cell lipoma are lipomatous tumors with distinct clinical, molecular, and prognostic features. Although histological and immunophenotypic features can overlap between ALT/WDL and spindle cell lipoma, the oncogenesis and clinical behavior are markedly different. In borderline cases, molecular analysis for MDM2 or CDK4 amplification can aid in distinguishing ALT/WDL from spindle cell lipoma. Although dedifferentiated liposarcoma has been reported to harbor both MDM2 amplification and loss of the RB1 region, we are not aware of a reported RB1 loss in well-differentiated ALT/WDL. In this article, we present a 69-year-old woman with a lipomatous tumor in the gluteal region that histologically, immunohistochemically, and molecularly mimicked spindle cell lipoma (with positive immunohistochemical staining for CD34 and loss of the RB1 gene region), yet harbored amplification of MDM2 and CDK4 confirmed by fluorescence in situ hybridization, supporting classification as ALT/WDL. This case strengthens the argument that in atypical clinical contexts, molecular studies for MDM2/CDK4 should be considered in tumors resembling spindle cell lipoma.

scholarly journals Sensitivity of MDM2 amplification and unexpected multiple faint alphoid 12 (alpha 12 satellite sequences) signals in atypical lipomatous tumor

2012 ◽  
Vol 25 (10) ◽  
pp. 1384-1396 ◽  
Author(s):  
Takeshi Kashima ◽  
Dina Halai ◽  
Hongtao Ye ◽  
Sandra Nalini Hing ◽  
David Delaney ◽  
...  
Keyword(s):  
Atypical Lipomatous Tumor ◽  
Lipomatous Tumor ◽  
Satellite Sequences ◽  
Mdm2 Amplification

A Case of Atypical Lipomatous Tumor of the Lower Leg

2016 ◽  
Vol 78 (4) ◽  
pp. 379-381 ◽  
Author(s):  
Tatsuya OGAWA ◽  
Shijima TAGUCHI ◽  
Yoshio NAKAYAMA
Keyword(s):  
Lower Leg ◽  
Atypical Lipomatous Tumor ◽  
Lipomatous Tumor

Induction of metallothionein-I mRNA in cultured cells by heavy metals and iodoacetate: evidence for gratuitous inducers

1984 ◽  
Vol 4 (3) ◽  
pp. 484-491
Author(s):  
D M Durnam ◽  
R D Palmiter
Keyword(s):  
Heavy Metals ◽  
Cultured Cells ◽  
Mrna Accumulation ◽  
Mouse Hepatocyte ◽  
Hepatocyte Cell Line ◽  
Regulatory Molecule ◽  
Heavy Metal Detoxification ◽  
Double Minute ◽  
Double Minute Chromosomes

A mouse hepatocyte cell line selected for growth in 80 microM CdSO4 (Cdr80 cells) was used to test the role of metallothioneins in heavy metal detoxification. The cadmium-resistant (Cdr80) cells have double minute chromosomes carrying amplified copies of the metallothionein-I gene and accumulate ca. 20-fold more metallothionein-I mRNA than unselected cadmium-sensitive (Cds) cells after optimal Cd stimulation. As a consequence, the amount of Cd which inhibits DNA synthesis by 50% is ca. 7.5-fold higher in Cdr80 cells than in Cds cells. Cds and Cdr80 cells were compared in terms of their resistance to other heavy metals. The results indicate that although Zn, Cu, Hg, Ag, Co, Ni, and Bi induce metallothionein-I mRNA accumulation in both Cdr80 and Cds cells, the Cdr80 cells show increased resistance to only a subset of these metals (Zn, Cu, Hg, and Bi). This suggests that not all metals which induce metallothionein mRNA are detoxified by metallothionein and argues against autoregulation of metallothionein genes. Metallothionein-I mRNA is also induced by iodoacetate, suggesting that the regulatory molecule has sensitive sulfhydryl groups.

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