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: 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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49Orphanet, 62UMLS, 26ICD10 via Orphanet, 59SNOMED-CT via Orphanet, 63UMLS via Orphanet
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Cancer diseases, Rare diseases
Orphanet: 49 
Rare tumors


Characteristics (Orphanet epidemiological data):

49
dedifferentiated liposarcoma:
Inheritance: Sporadic; Age of onset: Adulthood; Age of death: Adult


Aliases & Descriptions:

dedifferentiated liposarcoma 49
liposarcoma, dedifferentiated 62
ddls 49


External Ids:

ICD10 via Orphanet26 C49
SNOMED-CT via Orphanet59 404072004, 67280001
UMLS via Orphanet63 C0205824

Related Diseases for Dedifferentiated Liposarcoma

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17GeneCards, 18GeneDecks
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Diseases related to Dedifferentiated Liposarcoma via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 93)
idRelated DiseaseScoreTop Affiliating Genes
1liposarcoma31.8CDK4, MDM2, HMGA2, DES
2fibrous histiocytoma30.8ACTC1, DES
3lipoma30.7CDK4, MDM2, HMGA2, DES
4sarcoma30.6DES, ACTC1, HMGA2, MDM2, CDK4
5leiomyosarcoma30.4DES, ACTC1, MDM2, CDK4
6myxoid liposarcoma30.4MDM2, CDK4
7rhabdomyosarcoma30.2DES, ACTC1, MDM2, CDK4
8leukemia30.2CEBPA, HMGA2, MDM2, CDK4
9histiocytoma10.5
10leukemoid reaction10.3
11pleomorphic liposarcoma10.3
12solitary fibrous tumor10.3
13acute pancreatitis10.2
14kaposi's sarcoma10.2
15paraganglioma10.2
16cervicitis10.2
17diabetes mellitus10.2
18esophagitis10.2
19hyperparathyroidism10.2
20hypertension10.2
21hyperthyroidism10.2
22laryngitis10.2
23pancreatitis10.2
24retroperitoneal sarcoma10.2
25hemangioendothelioma10.2
26inflammatory myofibroblastic tumor10.2
27myxofibrosarcoma10.2
28dermatofibrosarcoma10.1MDM2
29infantile myofibromatosis10.1DES
30sertoli cell tumor10.1DES
31chondroma10.1HMGA2
32fasciitis10.1ACTC1
33lipomatosis10.1HMGA2
34anaplastic oligodendroglioma10.1CDK4, MDM2
35uterine sarcoma10.1DES, MDM2
36intraocular melanoma10.1CDK4, MDM2
37anaplastic astrocytoma10.1CDK4, MDM2
38epithelioid leiomyosarcoma10.1ACTC1, DES
39pseudosarcomatous fibromatosis10.1DES, ACTC1
40spindle cell carcinoma10.1ACTC1, DES
41spindle cell sarcoma10.1DES, ACTC1
42desmoid tumor10.1DES, ACTC1
43oligodendroglioma10.1MDM2, CDK4
44cutaneous fibrous histiocytoma10.1ACTC1, DES
45adenosarcoma10.1DES, ACTC1
46pre-eclampsia10.1AFP
47dermatofibrosarcoma protuberans10.0ACTC1, DES
48acute lymphoblastic leukemia, childhood10.0MDM2, CDK4
49endometrial stromal sarcoma10.0ACTC1, DES
50rhabdoid tumor10.0ACTC1, DES

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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42NIH Clinical Center, 6ClinicalTrials
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Drug clinical trials:

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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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33MalaCards
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MalaCards organs/tissues related to Dedifferentiated Liposarcoma:

33
Bone, Lung, Colon, Liver, Lymph node, Adipocyte

Animal Models for Dedifferentiated Liposarcoma or affiliated genes

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37MGI
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MGI Mouse Phenotypes related to Dedifferentiated Liposarcoma:

37
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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52PubMed
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Articles related to Dedifferentiated Liposarcoma:

(show top 50)    (show all 133)
idTitleAuthorsYear
1
Dedifferentiated liposarcoma of the retroperitoneum with osteosarcomatous component. (23426961)
2013
2
High level of CDK4 amplification is a poor prognostic factor in well-differentiated and dedifferentiated liposarcoma. (23852861)
2013
3
Phase II trial of the CDK4 inhibitor PD0332991 in patients with advanced CDK4-amplified well-differentiated or dedifferentiated liposarcoma. (23569312)
2013
4
Novel dedifferentiated liposarcoma xenograft models reveal PTEN down-regulation as a malignant signature and response to PI3K pathway inhibition. (23416162)
2013
5
Copy number losses define subgroups of dedifferentiated liposarcoma with poor prognosis and genomic instability. (22241790)
2012
6
Dedifferentiated liposarcoma of the rectum: a case report. (23139616)
2012
7
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
8
Renal dedifferentiated liposarcoma with intra-caval tumor thrombus: A rare case. (22919143)
2012
9
Diagnostic utility of p16, CDK4, and MDM2 as an immunohistochemical panel in distinguishing well-differentiated and dedifferentiated liposarcomas from other adipocytic tumors. (22301498)
2012
10
Liposarcoma of the chest wall. Transformation of dedifferentiated liposarcoma from a recurrent lipoma. (22886125)
2012
11
Cervical dedifferentiated liposarcoma with meningothelial-like whorling. (22528826)
2012
12
High-resolution genomic mapping reveals consistent amplification of the fibroblast growth factor receptor substrate 2 gene in well-differentiated and dedifferentiated liposarcoma. (21793095)
2011
13
Primary (de novo) dedifferentiated liposarcoma in the extremities: a multi-institution Tohoku Musculoskeletal Tumor Society study of 18 cases in northern Japan. (21795247)
2011
14
Recurrent Dedifferentiated Liposarcoma Arising from the Small Bowel Mesentery: A Case Report. (22207349)
2011
15
The CDKN2A/CDKN2B/CDK4/CCND1 pathway is pivotal in well-differentiated and dedifferentiated liposarcoma oncogenesis: an analysis of 104 tumors. (21910158)
2011
16
Well-differentiated and dedifferentiated liposarcomas. (19688222)
2010
17
Dedifferentiated liposarcoma of retroperitoneum: spectrum of imaging findings in 15 patients. (20416485)
2010
18
Dedifferentiated liposarcoma of the retroperitoneum. (20369054)
2010
19
Can MDM2 and CDK4 make the diagnosis of well differentiated/dedifferentiated liposarcoma? An immunohistochemical study on 129 soft tissue tumours. (19946100)
2009
20
Recurrent dedifferentiated liposarcoma of mediastinum involving lung and pleura. (19589790)
2009
21
Dedifferentiated liposarcoma of the retroperitoneum with osteosarcomatous component: report of two cases. (19135935)
2009
22
A dedifferentiated liposarcoma of the anterior mediastinum. (19390952)
2009
23
Dedifferentiated liposarcoma in the retroperitoneum in an atomic bomb survivor: report of a case. (21490866)
2008
24
HMGA2 is the partner of MDM2 in well-differentiated and dedifferentiated liposarcomas whereas CDK4 belongs to a distinct inconsistent amplicon. (18214854)
2008
25
Dedifferentiated Liposarcoma of the Retroperitoneum with Extensive Leiomyosarcomatous Differentiation and beta-Human Chorionic Gonadotropin Production. (18382626)
2008
26
Dedifferentiated liposarcoma presenting as jejunal polyp. Case report. (18184419)
2007
27
Cytogenetic and molecular cytogenetic findings in dedifferentiated liposarcoma with neural-like whorling pattern and metaplastic bone formation. (17213023)
2007
28
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
29
Dedifferentiated liposarcoma arising from the sigmoid mesocolon: a case report. (17696239)
2007
30
A rare diagnosis of dedifferentiated liposarcoma of the orbit. (17510871)
2007
31
Surgery for dedifferentiated liposarcoma, presenting two radiologically and pathologically distinctive patterns. (16887838)
2006
32
alpha-fetoprotein expression in a dedifferentiated liposarcoma. (16489442)
2006
33
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
34
Inguinal dedifferentiated liposarcoma with meningothelial-like whorls and metaplastic bone formation. (15842647)
2005
35
Primary dedifferentiated liposarcoma of the lung. (15910606)
2005
36
Alterations of the RB1 gene in dedifferentiated liposarcoma. (15933756)
2005
37
Dedifferentiated liposarcoma with extensive lymphoid component. (15991843)
2005
38
Best cases from the AFIP: Dedifferentiated liposarcoma of soft tissue with high-grade osteosarcomatous dedifferentiation. (16009825)
2005
39
Dedifferentiated liposarcoma of the oral cavity with angiosarcomatous dedifferentiation. (15806379)
2005
40
Inflammatory malignant fibrous histiocytomas and dedifferentiated liposarcomas: histological review, genomic profile, and MDM2 and CDK4 status favour a single entity. (15221942)
2004
41
Establishment of a novel human dedifferentiated liposarcoma cell line, FU-DDLS-1: conventional and molecular cytogenetic characterization. (12579306)
2003
42
Most malignant fibrous histiocytomas developed in the retroperitoneum are dedifferentiated liposarcomas: a review of 25 cases initially diagnosed as malignant fibrous histiocytoma. (12640106)
2003
43
A leukemoid reaction in a patient with a dedifferentiated liposarcoma. (11391379)
2001
44
Fine needle aspiration cytology of a dedifferentiated liposarcoma: report of a case with histologic and immunohistochemical follow-up. (11480734)
2001
45
Dedifferentiated liposarcoma: a report of nine cases with a peculiar neurallike whorling pattern associated with metaplastic bone formation. (9706974)
1998
46
Dedifferentiated liposarcoma with an inflammatory malignant fibrous histiocytoma-like component presenting a leukemoid reaction. (9311018)
1997
47
Molecular abnormalities of the p53 pathway in dedifferentiated liposarcoma. (9071997)
1997
48
Demonstration of recurrent dedifferentiated liposarcoma of the spermatic cord by FDG-PET. (9310177)
1997
49
Dedifferentiated liposarcoma: a clinicopathological analysis of 155 cases with a proposal for an expanded definition of dedifferentiation. (9060596)
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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2BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Dedifferentiated Liposarcoma

Search GEO for disease gene expression data for Dedifferentiated Liposarcoma.

Pathways for genes affiliated with Dedifferentiated Liposarcoma

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50PathCards, 55Reactome, 60Thomson Reuters, 54R&D Systems, 38NCBI BioSystems Database, 30KEGG, 53QIAGEN
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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 Degradation38
Immune response Antigen presentation by MHC class I60
8.9CDK4, MDM2, CALR

Compounds for genes affiliated with Dedifferentiated Liposarcoma

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45Novoseek, 51PharmGKB, 11DrugBank, 29IUPHAR, 61Tocris Bioscience, 24HMDB
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Compounds related to Dedifferentiated Liposarcoma according to GeneCards/GeneDecks:

(show top 50)    (show all 65)
idCompoundScoreTop Affiliating Genes
1vitamin k2459.9AFP, CDK4
210-hydroxycamptothecin459.9MDM2, AFP
3roscovitine459.8CDK4, MDM2
4orcein459.7ACTC1, DES
5s 100459.7DES, ACTC1
6carboplatin45 51 1111.6AFP, MDM2, CDK4
7reticulin459.6DES, ACTC1
8adpribose459.6CDK4, MDM2, DES
9mucicarmine459.6ACTC1, DES
10ifosfamide45 1110.6MDM2, AFP
11valine459.5AFP, MDM2, CDK4
12diethylstilbestrol45 29 1111.5AFP, MDM2
135fluorouracil459.5AFP, MDM2, CDK4
14agar459.5CDK4, MDM2, AFP
15adriamycin459.4MDM2, AFP, DES
16paclitaxel45 51 1111.4AFP, MDM2, CDK4
17imatinib45 51 1111.3MDM2, ACTC1, DES
18b 723459.2ACTC1, AFP, DES
19con a459.2CALR, AFP
20cisplatin45 51 61 1112.2AFP, HMGA2, MDM2, CDK4
21estradiol45 24 1111.1CDK4, MDM2, AFP, DES
22thymidine45 2410.1DES, AFP, MDM2, CDK4
23actinomycin d459.1DES, AFP, HMGA2, MDM2
24vitamin d459.0CDK4, CALR, AFP
25proline458.9CDK4, MDM2, DES
26hydrogen45 249.8CDK4, MDM2, CALR, AFP
27doxorubicin45 51 1110.7DES, AFP, HMGA2, MDM2, CDK4
28oligonucleotide458.7CDK4, MDM2, CALR, AFP
29arginine458.7DES, CALR, MDM2, CDK4
30zinc45 249.7CDK4, MDM2, CALR, AFP
31nitric oxide45 24 1110.6DES, CALR, MDM2, CDK4
32h2o2458.6DES, AFP, CALR, MDM2
33fatty acid458.5DES, AFP, CEBPA, CALR
34cysteine458.5DES, CALR, MDM2, CDK4
35progesterone45 29 61 24 1112.5DES, AFP, ACTC1, MDM2, CDK4
36vegf458.5DES, AFP, ACTC1, MDM2, CDK4
37threonine458.3DES, AFP, CALR, MDM2, CDK4
38serine458.2CDK4, MDM2, CALR, AFP, DES
39paraffin458.1DES, AFP, ACTC1, HMGA2, MDM2, CDK4
40glycogen45 249.1CDK4, MDM2, ACTC1, CEBPA, AFP, DES
41estrogen458.1DES, AFP, CEBPA, ACTC1, MDM2, CDK4
42dexamethasone45 51 29 1110.8AFP, CEBPA, ACTC1, CALR, MDM2, CDK4
43iron45 248.7DES, AFP, ACTC1, CALR, MDM2, CDK4
44testosterone45 61 24 1110.7DES, AFP, ACTC1, CALR, MDM2, CDK4
45steroid457.7DES, AFP, ACTC1, CALR, MDM2, CDK4
46oxygen45 248.7DES, AFP, ACTC1, CALR, MDM2, CDK4
47tyrosine457.7DES, AFP, ACTC1, CALR, MDM2, CDK4
48calcium45 51 24 1110.7DES, AFP, ACTC1, CALR, MDM2, CDK4
49glucose457.6DES, AFP, CEBPA, ACTC1, CALR, CDK4
50retinoic acid45 248.2DES, AFP, CEBPA, ACTC1, CALR, HMGA2

GO Terms for genes affiliated with Dedifferentiated Liposarcoma

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16Gene Ontology
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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:07115710.0CDK4, MDM2
2organ regenerationGO:0311009.7CDK4, CEBPA
3negative regulation of intracellular steroid hormone receptor signaling pathwayGO:0331449.5HMGA2, CALR
4response to testosteroneGO:0335749.5CDK4, CALR
5muscle filament slidingGO:0300499.4DES, ACTC1
6protein localization to nucleusGO:0345049.4CALR, MDM2
7cellular response to lithium ionGO:0712859.4CALR, CEBPA
8positive regulation of gene expressionGO:0106289.1CALR, HMGA2, MDM2
9negative regulation of apoptotic processGO:0430669.1ACTC1, MDM2, HMGA2
10positive regulation of cell proliferationGO:0082848.8MDM2, CDK4, CALR
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:0424938.6CALR, MDM2, CDK4, ACTC1

Products for genes affiliated with Dedifferentiated Liposarcoma

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Sources for Dedifferentiated Liposarcoma

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4CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
52PubMed
53QIAGEN
59SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet