DDLS
MCID: DDF001
MIFTS: 48

Dedifferentiated Liposarcoma (DDLS) malady

Cancer diseases, Rare diseases categories
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Summaries for Dedifferentiated Liposarcoma

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MalaCards based summary: Dedifferentiated Liposarcoma, also known as liposarcoma, dedifferentiated, is related to liposarcoma and fibrous histiocytoma. An important gene associated with Dedifferentiated Liposarcoma is CDK4 (cyclin-dependent kinase 4), and among its related pathways are Oncogene Induced Senescence and DNA damage ATM ATR regulation of G1 S checkpoint. The compounds vitamin k2 and 10-hydroxycamptothecin have been mentioned in the context of this disorder. Affiliated tissues include bone, lung and colon, and related mouse phenotypes are endocrine/exocrine gland and tumorigenesis.

Aliases & Classifications for Dedifferentiated Liposarcoma

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Sources:
48Orphanet, 62UMLS, 63UMLS via Orphanet, 26ICD10 via Orphanet
See all sources

Dedifferentiated Liposarcoma, Aliases & Descriptions:

Name: Dedifferentiated Liposarcoma 48
Liposarcoma, Dedifferentiated 62
 
Ddls 48


Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Cancer diseases, Rare diseases
Orphanet: 48 
Rare tumors


Characteristics (Orphanet epidemiological data):

48
dedifferentiated liposarcoma:
Inheritance: Sporadic; Age of onset: Adulthood; Age of death: Adult


External Ids:

UMLS via Orphanet63 C0205824
ICD10 via Orphanet26 C49

Related Diseases for Dedifferentiated Liposarcoma

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Diseases related to Dedifferentiated Liposarcoma via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 90)
idRelated DiseaseScoreTop Affiliating Genes
1liposarcoma31.8CDK4, MDM2, HMGA2, DES
2fibrous histiocytoma31.2ACTC1, DES
3myxoid liposarcoma30.8MDM2, CDK4
4lipoma30.6CDK4, MDM2, HMGA2, DES
5paraganglioma30.6DES, ACTC1
6leiomyosarcoma30.4DES, ACTC1, MDM2, CDK4
7sarcoma30.3DES, ACTC1, HMGA2, MDM2, CDK4
8rhabdomyosarcoma30.2DES, ACTC1, MDM2, CDK4
9leukemia30.1CEBPA, HMGA2, MDM2, CDK4
10histiocytoma10.6
11solitary fibrous tumor10.5
12infantile myofibromatosis10.5DES
13sertoli cell tumor10.5DES
14chondroma10.4HMGA2
15fasciitis10.4ACTC1
16lipomatosis10.4HMGA2
17anaplastic oligodendroglioma10.3CDK4, MDM2
18pleomorphic liposarcoma10.3
19leukemoid reaction10.3
20uterine sarcoma10.3DES, MDM2
21uveal melanoma10.3CDK4, MDM2
22grade iii astrocytoma10.3CDK4, MDM2
23epithelioid leiomyosarcoma10.2ACTC1, DES
24pseudosarcomatous fibromatosis10.2DES, ACTC1
25spindle cell carcinoma10.2ACTC1, DES
26spindle cell sarcoma10.2DES, ACTC1
27desmoid tumor10.2DES, ACTC1
28oligodendroglioma10.2MDM2, CDK4
29cutaneous fibrous histiocytoma10.2ACTC1, DES
30adenosarcoma10.2DES, ACTC1
31pre-eclampsia10.2AFP
32dermatofibrosarcoma protuberans10.2ACTC1, DES
33acute lymphoblastic leukemia, childhood10.2MDM2, CDK4
34endometrial stromal sarcoma10.2ACTC1, DES
35rhabdoid tumor10.2ACTC1, DES
36angiomyolipoma10.2ACTC1, DES
37malignant mesothelioma10.2CDK4, DES
38fibroma10.2DES, ACTC1
39fibromatosis10.2ACTC1, DES
40abrikosov's tumor10.2DES, ACTC1
41giant cell tumor10.2DES, ACTC1
42esophageal cancer10.2CDK4, MDM2
43gastrointestinal stromal tumor10.2ACTC1, DES
44diabetes mellitus10.2
45hypertension10.2
46acute pancreatitis10.2
47inflammatory myofibroblastic tumor10.2
48kaposi's sarcoma10.2
49cervicitis10.2
50esophagitis10.2

Graphical network of the top 20 diseases related to Dedifferentiated Liposarcoma:



Diseases related to dedifferentiated liposarcoma

Symptoms for Dedifferentiated Liposarcoma

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Drugs & Therapeutics for Dedifferentiated Liposarcoma

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Drug clinical trials:

Search ClinicalTrials for Dedifferentiated Liposarcoma

Search NIH Clinical Center for Dedifferentiated Liposarcoma

Genetic Tests for Dedifferentiated Liposarcoma

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Anatomical Context for Dedifferentiated Liposarcoma

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MalaCards organs/tissues related to Dedifferentiated Liposarcoma:

32
Bone, Lung, Colon, Lymph node, Adipocyte, Liver

Animal Models for Dedifferentiated Liposarcoma or affiliated genes

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MGI Mouse Phenotypes related to Dedifferentiated Liposarcoma:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053798.7CDK4, HMGA2, CEBPA, AFP
2MP:00020068.7CDK4, MDM2, CEBPA, AFP
3MP:00053898.4AFP, CEBPA, HMGA2, MDM2, CDK4
4MP:00053868.0DES, AFP, CEBPA, HMGA2, MDM2, CDK4
5MP:00053847.6CEBPA, ACTC1, CALR, MDM2, CDK4
6MP:00053697.6CDK4, MDM2, CALR, ACTC1, CEBPA, DES
7MP:00053787.5CEBPA, ACTC1, CALR, HMGA2, MDM2, CDK4
8MP:00053857.5DES, CEBPA, ACTC1, CALR, MDM2, CDK4
9MP:00053767.0DES, AFP, CEBPA, ACTC1, CALR, HMGA2
10MP:00107687.0DES, AFP, CEBPA, ACTC1, CALR, MDM2

Publications for Dedifferentiated Liposarcoma

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Articles related to Dedifferentiated Liposarcoma:

(show top 50)    (show all 145)
idTitleAuthorsYear
1
Primary dedifferentiated liposarcoma of the lung with rhabdomyoblastic and chrondroblastic differentiation. (24612243)
2014
2
Nuclear Expression of STAT6 in Dedifferentiated Liposarcomas With a Solitary Fibrous Tumor-like Morphology: A Diagnostic Pitfall. (25075469)
2014
3
Is the nonlipomatous component of dedifferentiated liposarcoma always soft tissue on CT? Analysis of CT densities and correlation with rate of growth in 60 patients. (25287680)
2014
4
Loss of heterozygosity and microsatellite instability are rare in sporadic dedifferentiated liposarcoma: a study of 43 well-characterized cases. (24878023)
2014
5
Challenging dedifferentiated liposarcoma identified by MDM2-amplification, a report of two cases. (25126005)
2014
6
MDM2 copy numbers in well-differentiated and dedifferentiated liposarcoma: characterizing progression to high-grade tumors. (24515760)
2014
7
Primary dedifferentiated liposarcoma of the femur presenting with malignant fibrous histiocytoma: A case report and review of the literature. (25013482)
2014
8
Dedifferentiated liposarcoma of the retroperitoneum with osteosarcomatous component. (23426961)
2013
9
High level of CDK4 amplification is a poor prognostic factor in well-differentiated and dedifferentiated liposarcoma. (23852861)
2013
10
Phase II trial of the CDK4 inhibitor PD0332991 in patients with advanced CDK4-amplified well-differentiated or dedifferentiated liposarcoma. (23569312)
2013
11
Novel dedifferentiated liposarcoma xenograft models reveal PTEN down-regulation as a malignant signature and response to PI3K pathway inhibition. (23416162)
2013
12
Copy number losses define subgroups of dedifferentiated liposarcoma with poor prognosis and genomic instability. (22241790)
2012
13
Dedifferentiated liposarcoma of the rectum: a case report. (23139616)
2012
14
Effect of the MDM2 antagonist RG7112 on the P53 pathway in patients with MDM2-amplified, well-differentiated or dedifferentiated liposarcoma: an exploratory proof-of-mechanism study. (23084521)
2012
15
Renal dedifferentiated liposarcoma with intra-caval tumor thrombus: A rare case. (22919143)
2012
16
Diagnostic utility of p16, CDK4, and MDM2 as an immunohistochemical panel in distinguishing well-differentiated and dedifferentiated liposarcomas from other adipocytic tumors. (22301498)
2012
17
Liposarcoma of the chest wall. Transformation of dedifferentiated liposarcoma from a recurrent lipoma. (22886125)
2012
18
High-resolution genomic mapping reveals consistent amplification of the fibroblast growth factor receptor substrate 2 gene in well-differentiated and dedifferentiated liposarcoma. (21793095)
2011
19
Primary (de novo) dedifferentiated liposarcoma in the extremities: a multi-institution Tohoku Musculoskeletal Tumor Society study of 18 cases in northern Japan. (21795247)
2011
20
Well-differentiated and dedifferentiated liposarcomas. (19688222)
2010
21
Dedifferentiated liposarcoma of retroperitoneum: spectrum of imaging findings in 15 patients. (20416485)
2010
22
Dedifferentiated liposarcoma of the retroperitoneum. (20369054)
2010
23
Can MDM2 and CDK4 make the diagnosis of well differentiated/dedifferentiated liposarcoma? An immunohistochemical study on 129 soft tissue tumours. (19946100)
2009
24
Recurrent dedifferentiated liposarcoma of mediastinum involving lung and pleura. (19589790)
2009
25
Dedifferentiated liposarcoma of the retroperitoneum with osteosarcomatous component: report of two cases. (19135935)
2009
26
Dedifferentiated liposarcoma in the retroperitoneum in an atomic bomb survivor: report of a case. (21490866)
2008
27
HMGA2 is the partner of MDM2 in well-differentiated and dedifferentiated liposarcomas whereas CDK4 belongs to a distinct inconsistent amplicon. (18214854)
2008
28
Dedifferentiated Liposarcoma of the Retroperitoneum with Extensive Leiomyosarcomatous Differentiation and beta-Human Chorionic Gonadotropin Production. (18382626)
2008
29
Dedifferentiated liposarcoma presenting as jejunal polyp. Case report. (18184419)
2007
30
Cytogenetic and molecular cytogenetic findings in dedifferentiated liposarcoma with neural-like whorling pattern and metaplastic bone formation. (17213023)
2007
31
Dedifferentiated liposarcoma of the pleura mimicking a malignant solitary fibrous tumor and associated with dedifferentiated liposarcoma of the mediastinum: usefulness of cytogenetic and molecular genetic analyses. (18036404)
2007
32
Dedifferentiated liposarcoma arising from the sigmoid mesocolon: a case report. (17696239)
2007
33
A rare diagnosis of dedifferentiated liposarcoma of the orbit. (17510871)
2007
34
Surgery for dedifferentiated liposarcoma, presenting two radiologically and pathologically distinctive patterns. (16887838)
2006
35
alpha-fetoprotein expression in a dedifferentiated liposarcoma. (16489442)
2006
36
MDM2 and CDK4 immunostainings are useful adjuncts in diagnosing well-differentiated and dedifferentiated liposarcoma subtypes: a comparative analysis of 559 soft tissue neoplasms with genetic data. (16160477)
2005
37
Inguinal dedifferentiated liposarcoma with meningothelial-like whorls and metaplastic bone formation. (15842647)
2005
38
Primary dedifferentiated liposarcoma of the lung. (15910606)
2005
39
Alterations of the RB1 gene in dedifferentiated liposarcoma. (15933756)
2005
40
Dedifferentiated liposarcoma with extensive lymphoid component. (15991843)
2005
41
Best cases from the AFIP: Dedifferentiated liposarcoma of soft tissue with high-grade osteosarcomatous dedifferentiation. (16009825)
2005
42
Dedifferentiated liposarcoma of the oral cavity with angiosarcomatous dedifferentiation. (15806379)
2005
43
Inflammatory malignant fibrous histiocytomas and dedifferentiated liposarcomas: histological review, genomic profile, and MDM2 and CDK4 status favour a single entity. (15221942)
2004
44
Establishment of a novel human dedifferentiated liposarcoma cell line, FU-DDLS-1: conventional and molecular cytogenetic characterization. (12579306)
2003
45
Most malignant fibrous histiocytomas developed in the retroperitoneum are dedifferentiated liposarcomas: a review of 25 cases initially diagnosed as malignant fibrous histiocytoma. (12640106)
2003
46
A leukemoid reaction in a patient with a dedifferentiated liposarcoma. (11391379)
2001
47
Fine needle aspiration cytology of a dedifferentiated liposarcoma: report of a case with histologic and immunohistochemical follow-up. (11480734)
2001
48
Dedifferentiated liposarcoma with an inflammatory malignant fibrous histiocytoma-like component presenting a leukemoid reaction. (9311018)
1997
49
Molecular abnormalities of the p53 pathway in dedifferentiated liposarcoma. (9071997)
1997
50
Dedifferentiated liposarcoma. Clinicopathologic analysis of 32 cases suggesting a better prognostic subgroup among pleomorphic sarcomas. (7977944)
1994

Variations for Dedifferentiated Liposarcoma

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Expression for genes affiliated with Dedifferentiated Liposarcoma

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Expression patterns in normal tissues for genes affiliated with Dedifferentiated Liposarcoma

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Pathways for genes affiliated with Dedifferentiated Liposarcoma

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Pathways related to Dedifferentiated Liposarcoma according to GeneCards/GeneDecks:

(show all 13)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.8CDK4, MDM2
29.8CDK4, MDM2
39.8CDK4, MDM2
49.8MDM2, CDK4
59.6CEBPA, MDM2
69.6MDM2, CEBPA
79.6MDM2, AFP
89.5CEBPA, AFP
9
Show member pathways
9.3CDK4, MDM2, HMGA2
10
Show member pathways
9.3ACTC1, DES
119.3CEBPA, MDM2, CDK4
129.2MDM2, HMGA2, CEBPA
13
Show member pathways
Proteasome Degradation37
Immune response Antigen presentation by MHC class I60
8.9CDK4, MDM2, CALR

Compounds for genes affiliated with Dedifferentiated Liposarcoma

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Compounds related to Dedifferentiated Liposarcoma according to GeneCards/GeneDecks:

(show top 50)    (show all 65)
idCompoundScoreTop Affiliating Genes
1vitamin k2449.9AFP, CDK4
210-hydroxycamptothecin449.9MDM2, AFP
3roscovitine449.8CDK4, MDM2
4orcein449.7ACTC1, DES
5s 100449.7DES, ACTC1
6carboplatin44 50 1111.6AFP, MDM2, CDK4
7reticulin449.6DES, ACTC1
8adpribose449.6CDK4, MDM2, DES
9mucicarmine449.6ACTC1, DES
10ifosfamide44 1110.6MDM2, AFP
11valine449.5AFP, MDM2, CDK4
12diethylstilbestrol44 28 1111.5AFP, MDM2
135fluorouracil449.5AFP, MDM2, CDK4
14agar449.5CDK4, MDM2, AFP
15adriamycin449.4MDM2, AFP, DES
16paclitaxel44 50 1111.4AFP, MDM2, CDK4
17imatinib44 50 1111.3MDM2, ACTC1, DES
18b 723449.2ACTC1, AFP, DES
19con a449.2CALR, AFP
20cisplatin44 50 61 1112.2AFP, HMGA2, MDM2, CDK4
21estradiol44 24 1111.1CDK4, MDM2, AFP, DES
22thymidine44 2410.1DES, AFP, MDM2, CDK4
23actinomycin d449.1DES, AFP, HMGA2, MDM2
24vitamin d449.0CDK4, CALR, AFP
25proline448.9CDK4, MDM2, DES
26hydrogen44 249.8CDK4, MDM2, CALR, AFP
27doxorubicin44 50 1110.7DES, AFP, HMGA2, MDM2, CDK4
28oligonucleotide448.7CDK4, MDM2, CALR, AFP
29arginine448.7DES, CALR, MDM2, CDK4
30zinc44 249.7CDK4, MDM2, CALR, AFP
31nitric oxide44 24 1110.6DES, CALR, MDM2, CDK4
32h2o2448.6DES, AFP, CALR, MDM2
33fatty acid448.5DES, AFP, CEBPA, CALR
34cysteine448.5DES, CALR, MDM2, CDK4
35progesterone44 28 61 24 1112.5DES, AFP, ACTC1, MDM2, CDK4
36vegf448.5DES, AFP, ACTC1, MDM2, CDK4
37threonine448.3DES, AFP, CALR, MDM2, CDK4
38serine448.2CDK4, MDM2, CALR, AFP, DES
39paraffin448.1DES, AFP, ACTC1, HMGA2, MDM2, CDK4
40glycogen44 249.1CDK4, MDM2, ACTC1, CEBPA, AFP, DES
41estrogen448.1DES, AFP, CEBPA, ACTC1, MDM2, CDK4
42dexamethasone44 50 28 1110.8AFP, CEBPA, ACTC1, CALR, MDM2, CDK4
43iron44 248.7DES, AFP, ACTC1, CALR, MDM2, CDK4
44testosterone44 61 24 1110.7DES, AFP, ACTC1, CALR, MDM2, CDK4
45steroid447.7DES, AFP, ACTC1, CALR, MDM2, CDK4
46oxygen44 248.7DES, AFP, ACTC1, CALR, MDM2, CDK4
47tyrosine447.7DES, AFP, ACTC1, CALR, MDM2, CDK4
48calcium44 50 24 1110.7DES, AFP, ACTC1, CALR, MDM2, CDK4
49glucose447.6DES, AFP, CEBPA, ACTC1, CALR, CDK4
50retinoic acid44 248.2DES, AFP, CEBPA, ACTC1, CALR, HMGA2

GO Terms for genes affiliated with Dedifferentiated Liposarcoma

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Cellular components related to Dedifferentiated Liposarcoma according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1cytosolGO:0058297.8CDK4, MDM2, CALR, ACTC1, DES

Biological processes related to Dedifferentiated Liposarcoma according to GeneCards/GeneDecks:

(show all 13)
idNameGO IDScoreTop Affiliating Genes
1negative regulation of cell cycle arrestGO:0711579.9MDM2, CDK4
2organ regenerationGO:0311009.7CDK4, CEBPA
3negative regulation of intracellular steroid hormone receptor signaling pathwayGO:0331449.5HMGA2, CALR
4response to testosteroneGO:0335749.5CALR, CDK4
5cellular response to lithium ionGO:0712859.4CEBPA, CALR
6muscle filament slidingGO:0300499.4ACTC1, DES
7protein localization to nucleusGO:0345049.4MDM2, CALR
8positive regulation of gene expressionGO:0106289.1MDM2, HMGA2, CALR
9negative regulation of apoptotic processGO:0430669.1MDM2, HMGA2, ACTC1
10positive regulation of cell proliferationGO:0082848.8CALR, MDM2, CDK4
11negative regulation of transcription from RNA polymerase II promoterGO:0001228.7CEBPA, CALR, HMGA2, MDM2
12negative regulation of transcription, DNA-templatedGO:0458928.6MDM2, HMGA2, CALR
13response to drugGO:0424938.6CDK4, MDM2, CALR, ACTC1

Products for genes affiliated with Dedifferentiated Liposarcoma

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Sources for Dedifferentiated Liposarcoma

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet