DDLS
MCID: DDF001
MIFTS: 43

Dedifferentiated Liposarcoma (DDLS) malady

Categories: Rare diseases

Aliases & Classifications for Dedifferentiated Liposarcoma

Aliases & Descriptions for Dedifferentiated Liposarcoma:

Name: Dedifferentiated Liposarcoma 56
Liposarcoma, Dedifferentiated 69
Liposarcoma Dedifferentiated 52
Ddls 56

Characteristics:

Orphanet epidemiological data:

56
dedifferentiated liposarcoma
Inheritance: Not applicable; Prevalence: 1-9/1000000 (Europe); Age of onset: Adult; Age of death: adult;

Classifications:



External Ids:

Orphanet 56 ORPHA99970
UMLS via Orphanet 70 C0205824
ICD10 via Orphanet 34 C49.9

Summaries for Dedifferentiated Liposarcoma

MalaCards based summary : Dedifferentiated Liposarcoma, also known as liposarcoma, dedifferentiated, is related to liposarcoma and primary eye hypotony. An important gene associated with Dedifferentiated Liposarcoma is MDM2 (MDM2 Proto-Oncogene), and among its related pathways/superpathways are Cellular Senescence and Glioma. The drugs Rosiglitazone and Cyclophosphamide have been mentioned in the context of this disorder. Affiliated tissues include bone, lung and colon, and related phenotypes are cardiovascular system and cellular

Related Diseases for Dedifferentiated Liposarcoma

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

(show top 50) (show all 180)
id Related Disease Score Top Affiliating Genes
1 liposarcoma 10.9
2 primary eye hypotony 10.3 CDK4 MDM2
3 amelogenesis imperfecta-gingival hyperplasia syndrome 10.3 CDK4 MDM2
4 blood protein disease 10.3 CDK4 MDM2
5 liposarcoma of bone 10.3 CDK4 MDM2
6 bone leiomyosarcoma 10.3 CDK4 MDM2
7 localized hypertrophic neuropathy 10.3 CDK4 MDM2
8 malignant mixed mullerian tumor 10.2 DES HMGA2
9 intracranial liposarcoma 10.2 CDK4 MDM2
10 breast rhabdomyosarcoma 10.2 DES HMGA2
11 transitional cell carcinoma 10.2 DES HMGA2
12 heart sarcoma 10.2 CDKN2A HMGA2
13 carbon monoxide-induced parkinsonism 10.2 ACTC1 DES
14 endometrial disease 10.2 CDK4 CDKN2A
15 vagina leiomyosarcoma 10.2 ACTC1 DES
16 myoma 10.2 ACTC1 DES
17 spleen angiosarcoma 10.2 ACTC1 DES
18 malignant dermis tumor 10.2 ACTC1 DES
19 reticulohistiocytic granuloma 10.2 DES F13A1
20 acute mountain sickness 10.2 MDM2 TP53
21 polydactyly cleft lip palate psychomotor retardation 10.2 CDKN2A DES
22 biemond syndrome 10.2 ACTC1 DES
23 intestinal obstruction 10.2 ACTC1 DES
24 functionless pituitary adenoma 10.2 MDM2 TP53
25 scarlet fever 10.2 MDM2 TP53
26 cavernous sinus meningioma 10.2 DES F13A1
27 extraosseous osteosarcoma 10.2 CDK4 DES MDM2
28 male urethral cancer 10.2 ACTC1 DES
29 verruciform xanthoma of skin 10.2 DES TP53
30 pinta disease 10.2 CDK4 MDM2
31 sarcoma 10.2
32 rhabdomyosarcoma 2, alveolar 10.2 CDK4 DES MDM2
33 pustulosis palmaris et plantaris 10.2 ACTC1 DES
34 dystrophic epidermolysis bullosa, nails only 10.2 ACTC1 F13A1
35 secondary adrenal insufficiency 10.1 ACTC1 F13A1
36 pleural lipoma 10.1 CDK4 HMGA2 MDM2
37 childhood immature teratoma of ovary 10.1 CDK4 HMGA2 MDM2
38 embryonal extrahepatic bile duct rhabdomyosarcoma 10.1 CDK4 DES MDM2
39 clear cell adenofibroma 10.1 CDKN2A TP53
40 lacrimal gland adenoid cystic carcinoma 10.1 CDKN2A TP53
41 aortic aneurysm 10.1 CDK4 CDKN2A MDM2
42 apocrine sweat gland neoplasm 10.1 CDK4 CDKN2A MDM2
43 serous surface papilloma 10.1 ACTC1 HMGA2
44 hormone producing pituitary cancer 10.1 CDK4 MDM2
45 sebaceous adenoma 10.1 ACTC1 DES
46 multifocal osteogenic sarcoma 10.1 CDK4 CDKN2A MDM2
47 spondylarthropathy 10.1 CDKN2A TP53
48 histiocytoma 10.1
49 fibrous histiocytoma 10.1
50 bone deterioration disease 10.1 DES TP53

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



Diseases related to Dedifferentiated Liposarcoma

Symptoms & Phenotypes for Dedifferentiated Liposarcoma

MGI Mouse Phenotypes related to Dedifferentiated Liposarcoma:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 10.08 CDKN2A MET TP53 DES ACTC1 F13A1
2 cellular MP:0005384 10 CDK4 MDM2 MET CDKN2A DES ACTC1
3 homeostasis/metabolism MP:0005376 9.97 CDK4 MDM2 CDKN2A MET DES ACTC1
4 endocrine/exocrine gland MP:0005379 9.88 CDK4 MDM2 MET CDKN2A F13A1 TP53
5 mortality/aging MP:0010768 9.86 CDK4 MDM2 MET CDKN2A DES ACTC1
6 digestive/alimentary MP:0005381 9.83 CDK4 MDM2 MET CDKN2A TP53
7 liver/biliary system MP:0005370 9.72 CDK4 MDM2 MET CDKN2A TP53
8 muscle MP:0005369 9.7 CDKN2A MET TP53 DES ACTC1 CDK4
9 neoplasm MP:0002006 9.35 CDKN2A MET TP53 CDK4 MDM2
10 pigmentation MP:0001186 8.92 CDK4 MDM2 CDKN2A TP53

Drugs & Therapeutics for Dedifferentiated Liposarcoma

Drugs for Dedifferentiated Liposarcoma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 23)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Rosiglitazone Approved, Investigational Phase 2 122320-73-4 77999
2
Cyclophosphamide Approved, Investigational Phase 2 50-18-0, 6055-19-2 2907
3
Ifosfamide Approved Phase 2 3778-73-2 3690
4
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
5
Mechlorethamine Approved Phase 2 51-75-2 4033
6
Mesna Approved Phase 2 3375-50-6 598
7
Everolimus Approved Phase 2 159351-69-6 6442177
8
Pembrolizumab Approved Phase 2 1374853-91-4
9
Sirolimus Approved, Investigational Phase 2 53123-88-9 5284616 6436030 46835353
10
Trabectedin Approved, Investigational Phase 2 114899-77-3 108150
11
Maleic acid Experimental Phase 2 110-16-7 444266
12 Hypoglycemic Agents Phase 2
13 Alkylating Agents Phase 2
14 Anti-Bacterial Agents Phase 2
15 Immunoglobulins Phase 2
16 Anti-Infective Agents Phase 2
17 Immunosuppressive Agents Phase 2
18 Antifungal Agents Phase 2
19 Isophosphamide mustard Phase 2
20 Antibiotics, Antitubercular Phase 2
21 Antibodies Phase 2
22 Antirheumatic Agents Phase 2
23 Antineoplastic Agents, Alkylating Phase 2

Interventional clinical trials:

(show all 14)
id Name Status NCT ID Phase
1 Selinexor in Advanced Liposarcoma Recruiting NCT02606461 Phase 2, Phase 3
2 Rosiglitazone in Treating Patients With Liposarcoma Completed NCT00004180 Phase 2
3 Study of Abemaciclib in Dedifferentiated Liposarcoma Recruiting NCT02846987 Phase 2
4 A Study Assessing Efficacy & Safety of Ribociclib in Patients With Advanced Well/Dedifferentiated Liposarcoma Recruiting NCT03096912 Phase 2
5 Ph II Cabazitaxel DD Liposarcoma Recruiting NCT01913652 Phase 2
6 Efficacy Study on Trabectedin in Retroperitoneal Leiomyosarcoma and Well Differentiated/Dedifferentiated Liposarcoma Recruiting NCT02247544 Phase 2
7 SARC028: A Phase II Study of the Anti-PD1 Antibody Pembrolizumab (MK-3475) in Patients With Advanced Sarcomas Recruiting NCT02301039 Phase 2
8 Sirolimus and Cyclophosphamide in Metastatic or Unresectable Myxoid Liposarcoma and Chondrosarcoma Recruiting NCT02821507 Phase 2
9 A Phase II Study Assessing Efficacy & Safety of Ribociclib in Patients With Advanced Well/Dedifferentiated Liposarcoma Active, not recruiting NCT02571829 Phase 2
10 Activity and Tolerability of Pazopanib in Advanced and/or Metastatic Liposarcoma. A Phase II Clinical Trial Active, not recruiting NCT01692496 Phase 2
11 Phase II Trial of Ribociclib and Everolimus in Advanced Dedifferentiated Liposarcoma (DDL) and Leiomyosarcoma (LMS) Not yet recruiting NCT03114527 Phase 2
12 Efficacy and Safety of Plitidepsin in Patients With Advanced Unresectable or Metastatic, Relapsed/Refractory, Dedifferentiated Liposarcoma (DLPS): an Exploratory Phase II Multicenter Trial Terminated NCT01876043 Phase 2
13 Study Evaluating the Efficacy and Safety of IPI 504 in Patients With Advanced Dedifferentiated Liposarcoma Withdrawn NCT00969917 Phase 2
14 Phase 1 Safety Testing of SAR405838 Active, not recruiting NCT01636479 Phase 1

Search NIH Clinical Center for Dedifferentiated Liposarcoma

Genetic Tests for Dedifferentiated Liposarcoma

Anatomical Context for Dedifferentiated Liposarcoma

MalaCards organs/tissues related to Dedifferentiated Liposarcoma:

39
Bone, Lung, Colon, Liver, Adipocyte, Brain, Lymph Node

Publications for Dedifferentiated Liposarcoma

Articles related to Dedifferentiated Liposarcoma:

(show top 50) (show all 185)
id Title Authors Year
1
Dedifferentiated liposarcoma of the lower extremity with low-grade dedifferentiation and low-grade osteosarcomatous component. ( 27909788 )
2017
2
Primary Diaphragmatic Dedifferentiated Liposarcoma in a Young Female Patient after Delivery. ( 27247814 )
2016
3
Application of MDM2 Fluorescence In Situ Hybridization and Immunohistochemistry in Distinguishing Dedifferentiated Liposarcoma From Other High-grade Sarcomas. ( 27028243 )
2016
4
Preclinical evaluation of potential therapeutic targets in dedifferentiated liposarcoma. ( 27409346 )
2016
5
Differential diagnosis of atypical lipomatous tumor/well-differentiated liposarcoma and dedifferentiated liposarcoma: utility of p16 in combination with MDM2 and CDK4 immunohistochemistry. ( 27597521 )
2016
6
Dedifferentiated Liposarcoma of Sigmoid Mesocolon - A Case Report. ( 27604671 )
2016
7
Progression-Free Survival Among Patients With Well-Differentiated or Dedifferentiated Liposarcoma Treated With CDK4 Inhibitor Palbociclib: A Phase 2 Clinical Trial. ( 27124835 )
2016
8
Primary mediastinal dedifferentiated liposarcoma resected by lateral thoracotomy with video-assisted thoracoscopic surgery. ( 26724326 )
2016
9
Rare Aggressive Behavior of MDM2-Amplified Retroperitoneal Dedifferentiated Liposarcoma, with Brain, Lung and Subcutaneous Metastases. ( 27746879 )
2016
10
MDM2 Expression in Extranodal Abdominal and Retroperitoneal Follicular Dendritic Cell Sarcomas, Mimicking Dedifferentiated Liposarcomas. ( 26180935 )
2016
11
Dedifferentiated Liposarcoma: Updates on Morphology, Genetics, and Therapeutic Strategies. ( 26645460 )
2016
12
Paratesticular dedifferentiated liposarcoma with rhabdomyoblastic differentiation and hCG production: Case study and literature review. ( 27773101 )
2016
13
Dedifferentiated Liposarcoma Masquerading as Rhabdomyosarcoma. ( 27801731 )
2016
14
Giant dedifferentiated liposarcoma of small bowel mesentery: a case report. ( 27654578 )
2016
15
Plasma levels of miRNA-155 as a powerful diagnostic marker for dedifferentiated liposarcoma. ( 27186423 )
2016
16
Spermatic cord dedifferentiated liposarcoma presenting as a recurrent inguinal hernia. ( 27995856 )
2016
17
Homologous Lipoblastic Differentiation in Dedifferentiated Liposarcoma. ( 26644373 )
2016
18
Multiple Primary Dedifferentiated Liposarcoma of the Jejunal Mesentery: A Case Report and Review of Literature. ( 26894164 )
2016
19
Clinical outcome of dedifferentiated liposarcoma in the extremities: A retrospective case series of 7 patients. ( 27317086 )
2016
20
Dedifferentiated liposarcoma involving the spleen and splenic hilum: a report of a case with a rare growth pattern. ( 25594652 )
2015
21
Dedifferentiated Liposarcoma of the Anterior Mediastinum: A Rare Case. ( 26252913 )
2015
22
Immunohistochemical, cytogenetic, and molecular cytogenetic characterization of both components of a dedifferentiated liposarcoma: implications for histogenesis. ( 25550570 )
2015
23
Dedifferentiated Liposarcoma of the Gastroesophageal Junction. ( 25690861 )
2015
24
Paratesticular dedifferentiated liposarcoma with prominent myxoid stroma: report of a case and review of the literature. ( 25986682 )
2015
25
The hepatocyte growth factor receptor as a potential therapeutic target for dedifferentiated liposarcoma. ( 26006023 )
2015
26
Peritoneal sarcomatosis: site of origin for the establishment of an in vitro and in vivo cell line model to study therapeutic resistance in dedifferentiated liposarcoma. ( 26373732 )
2015
27
Seaweed floating in the pericardium: a rare case of primary dedifferentiated liposarcoma. ( 26525285 )
2015
28
Dedifferentiated Liposarcoma of the Spermatic Cord: A Series of 42 Cases. ( 25828386 )
2015
29
Metastatic uterine leiomyosarcoma with MDM2 amplification mimicking dedifferentiated liposarcoma: a diagnostic pitfall. ( 25675188 )
2015
30
Metastasis in dedifferentiated liposarcoma: Predictors and outcome in 148 patients. ( 25659772 )
2015
31
SAR405838: A novel and potent inhibitor of the MDM2:p53 axis for the treatment of dedifferentiated liposarcoma. ( 26475335 )
2015
32
Primary cardiac dedifferentiated liposarcoma with homologous and heterologous differentiation: a case report. ( 26464734 )
2015
33
Alternative lengthening of telomeres and loss of ATRX are frequent events in pleomorphic and dedifferentiated liposarcomas. ( 26022452 )
2015
34
Prognostic value of HMGA2, CDK4, and JUN amplification in well-differentiated and dedifferentiated liposarcomas. ( 26336885 )
2015
35
Dedifferentiated liposarcoma presenting as a large rectal mass. ( 26112679 )
2015
36
Syndecan-1 regulates adipogenesis: new insights in dedifferentiated liposarcoma tumorigenesis. ( 25344834 )
2014
37
Pazopanib, a Receptor Tyrosine Kinase Inhibitor, Suppresses Tumor Growth through Angiogenesis in Dedifferentiated Liposarcoma Xenograft Models. ( 25500074 )
2014
38
MDM2 copy numbers in well-differentiated and dedifferentiated liposarcoma: characterizing progression to high-grade tumors. ( 24515760 )
2014
39
Retroperitoneal dedifferentiated liposarcoma lacking MDM2 amplification in a patient with a germ line CHEK2 mutation. ( 24595525 )
2014
40
Prognostic relevance of FAcdAcration Nationale des Centres de Lutte Contre le Cancer grade and MDM2 amplification levels in dedifferentiated liposarcoma: a study of 50 cases. ( 25059573 )
2014
41
High-dose continuous-infusion ifosfamide in advanced well-differentiated/dedifferentiated liposarcoma. ( 25628856 )
2014
42
Ultrasound-guided fine-needle aspiration of a posterior neck dedifferentiated liposarcoma with MDM2 fluorescence in situ hybridization performed on a Pap-stained smear. ( 25132684 )
2014
43
Gankyrin is a predictive and oncogenic factor in well-differentiated and dedifferentiated liposarcoma. ( 25238053 )
2014
44
Primary dedifferentiated liposarcoma of the femur presenting with malignant fibrous histiocytoma: A case report and review of the literature. ( 25013482 )
2014
45
Are peripheral purely undifferentiated pleomorphic sarcomas with MDM2 amplification dedifferentiated liposarcomas? ( 24525499 )
2014
46
Predictors of outcomes in patients with primary retroperitoneal dedifferentiated liposarcoma undergoing surgery. ( 24315890 )
2014
47
Nuclear Expression of STAT6 in Dedifferentiated Liposarcomas With a Solitary Fibrous Tumor-like Morphology: A Diagnostic Pitfall. ( 25075469 )
2014
48
Primary dedifferentiated liposarcoma of the lung with rhabdomyoblastic and chrondroblastic differentiation. ( 24612243 )
2014
49
Loss of heterozygosity and microsatellite instability are rare in sporadic dedifferentiated liposarcoma: a study of 43 well-characterized cases. ( 24878023 )
2014
50
An unusual case of primary colonic dedifferentiated liposarcoma. ( 24394854 )
2014

Variations for Dedifferentiated Liposarcoma

Expression for Dedifferentiated Liposarcoma

Search GEO for disease gene expression data for Dedifferentiated Liposarcoma.

Pathways for Dedifferentiated Liposarcoma

Pathways related to Dedifferentiated Liposarcoma according to GeneCards Suite gene sharing:

(show all 36)
id Super pathways Score Top Affiliating Genes
1
Show member pathways
12.97 CDK4 CDKN2A HMGA2 MDM2 TP53
2
Show member pathways
12.85 CDK4 CDKN2A MDM2 MET TP53
3 12.45 CDK4 MDM2 MET TP53
4 12.45 CDK4 CDKN2A MDM2 MET TP53
5
Show member pathways
12.43 CDK4 CDKN2A MDM2 TP53
6 12.36 CDK4 CDKN2A MDM2 TP53
7
Show member pathways
12.23 MDM2 MET TP53
8
Show member pathways
12.23 CDK4 CDKN2A MDM2 TP53
9 12.22 CDK4 CDKN2A MDM2 TP53
10
Show member pathways
12.19 CDK4 CDKN2A TP53
11 12.14 MDM2 MET TP53
12 12.14 CDK4 CDKN2A MDM2 TP53
13
Show member pathways
12.1 CDK4 MDM2 TP53
14 12.05 HMGA2 MDM2 MET TP53
15 12.03 CDKN2A HMGA2 MDM2 MET TP53
16 12.01 CDK4 MDM2 TP53
17
Show member pathways
11.99 CDK4 MDM2 MET TP53
18 11.93 MDM2 MET TP53
19
Show member pathways
11.88 CDK4 CDKN2A MDM2 TP53
20 11.87 CDKN2A MDM2 TP53
21 11.84 CDK4 CDKN2A MDM2 TP53
22 11.82 CDKN2A MDM2 TP53
23 11.73 CDK4 MDM2 TP53
24 11.62 CDKN2A MDM2 TP53
25
Show member pathways
11.62 CDKN2A MDM2 TP53
26 11.6 CDK4 CDKN2A MDM2 TP53
27 11.56 CDKN2A MDM2 TP53
28 11.46 CDKN2A MDM2 TP53
29 11.36 CDK4 MDM2 TP53
30 11.33 CDK4 MDM2 TP53
31 11.17 CDK4 TP53
32 11.15 MDM2 TP53
33 11.12 CDK4 CDKN2A MDM2 TP53
34 10.89 CDKN2A MDM2 TP53
35
Show member pathways
10.84 CDKN2A MDM2 TP53
36 10.43 CDK4 CDKN2A MDM2 TP53

GO Terms for Dedifferentiated Liposarcoma

Cellular components related to Dedifferentiated Liposarcoma according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 senescence-associated heterochromatin focus GO:0035985 8.62 CDKN2A HMGA2

Biological processes related to Dedifferentiated Liposarcoma according to GeneCards Suite gene sharing:

(show all 17)
id Name GO ID Score Top Affiliating Genes
1 cell cycle GO:0007049 9.84 CDK4 CDKN2A HMGA2 TP53
2 negative regulation of transcription, DNA-templated GO:0045892 9.76 CDKN2A HMGA2 MDM2 TP53
3 negative regulation of apoptotic process GO:0043066 9.73 ACTC1 HMGA2 MDM2 TP53
4 positive regulation of gene expression GO:0010628 9.62 ACTC1 HMGA2 MDM2 TP53
5 muscle filament sliding GO:0030049 9.57 ACTC1 DES
6 base-excision repair GO:0006284 9.56 HMGA2 TP53
7 positive regulation of apoptotic process GO:0043065 9.56 CDK4 CDKN2A HMGA2 TP53
8 response to antibiotic GO:0046677 9.55 MDM2 TP53
9 adipose tissue development GO:0060612 9.52 CDK4 HMGA2
10 cellular response to gamma radiation GO:0071480 9.51 MDM2 TP53
11 positive regulation of cell cycle arrest GO:0071158 9.49 HMGA2 TP53
12 negative regulation of cell cycle arrest GO:0071157 9.48 CDK4 MDM2
13 positive regulation of protein export from nucleus GO:0046827 9.4 MDM2 TP53
14 replicative senescence GO:0090399 9.37 CDKN2A TP53
15 positive regulation of cellular senescence GO:2000774 9.16 CDKN2A HMGA2
16 senescence-associated heterochromatin focus assembly GO:0035986 8.96 CDKN2A HMGA2
17 cellular response to actinomycin D GO:0072717 8.62 MDM2 TP53

Molecular functions related to Dedifferentiated Liposarcoma according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 disordered domain specific binding GO:0097718 8.96 MDM2 TP53
2 SUMO transferase activity GO:0019789 8.62 CDKN2A MDM2

Sources for Dedifferentiated Liposarcoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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