MCID: UND005
MIFTS: 59

Undifferentiated Pleomorphic Sarcoma

Categories: Rare diseases, Cancer diseases, Bone diseases

Aliases & Classifications for Undifferentiated Pleomorphic Sarcoma

MalaCards integrated aliases for Undifferentiated Pleomorphic Sarcoma:

Name: Undifferentiated Pleomorphic Sarcoma 49 55
Malignant Fibrous Histiocytoma 49 69
Ups 49 55
Malignant Fibrohistiocytic Tumors 49

Characteristics:

Orphanet epidemiological data:

55
undifferentiated pleomorphic sarcoma
Inheritance: Not applicable; Prevalence: 1-9/1000000 (Europe),1-9/1000000 (United States); Age of onset: Adolescent,Adult,Childhood,Elderly; Age of death: any age;

Classifications:

Orphanet: 55  
Rare bone diseases


External Ids:

Orphanet 55 ORPHA2023
MESH via Orphanet 42 D051677
UMLS via Orphanet 70 C0334463
ICD10 via Orphanet 33 C49.9

Summaries for Undifferentiated Pleomorphic Sarcoma

NIH Rare Diseases : 49 The following summary is from Orphanet, a European reference portal for information on rare diseases and orphan drugs.Orpha Number: 2023Disease definitionUndifferentiated pleomorphic sarcoma (UPS) is an aggressive sarcoma of soft tissues or bone that can arise from any part of the body, clinically presenting as swelling, mass, pain, pathological fracture and occasional systemic features and is characterized by high local recurrence and significant metastasis.EpidemiologyUPS ranks the 4th most common soft tissue sarcoma with a slight male preponderance. The incidence has been evaluated to be close to 0.8-1 new case per 100000 per year in one European series.Clinical descriptionThe tumor arises most commonly during the sixth and seventh decades of life. The most common sites of involvement include lower extremities (mainly thigh) followed by upper arms, retroperitoneum, viscera, head and neck (in childhood). Primary osseous UPS most commonly occurs in distal femur, proximal tibia, proximal femur and humerus. Patients may present pain, swelling / mass and pathological fractures. In the skin, UPS presents as a relatively painless, rapidly enlarging nodule. Anorexia, malaise, fever and weight loss are present in retroperitoneal and inflammatory forms of UPS. Most UPS recur locally; distant metastases are common (the most frequent is lung). Regional metastases are rare.EtiologyUPS is thought to be derived from a primitive mesenchymal cell capable of differentiating into histiocytes, fibroblasts, myofibroblasts and osteoclasts. The etiology of the tumor remains unknown. Prior radiation therapy is a likely risk factor in some cases.Diagnostic methodsAny tumor mass over 5 cm is suspected to be a sarcoma. Magnetic resonance imaging (MRI) is the imaging method of choice for limbs and shows a high signal on T2 weighted images. Histology of biopsy specimen prior to any treatment is crucial to reach diagnosis and shows pleomorphic spindle cell population with large atypical cells frequently exhibiting numerous irregular mitotic figures, associated regions of hemorrhage and necrosis, associated lymphohistiocytic infiltrate and invasion of dermis. Immunohistochemical staining is negative for S-100, HMB-45, CD34 and cytokeratin which assists in ruling out other soft tissue tumors. Most cases previously diagnosed as malignant fibrous histiocytoma have been reclassified into other histological types of sarcoma.Differential diagnosisWhen occurring in skin, UPS is difficult to differentiate from atypical fibroxanthoma or dermatofibrosarcoma protuberans (see this term). Histological differential diagnoses include leiomyosarcoma, rhabdomyosarcoma, lymphoma, and melanoma (see these terms).Management and treatmentUPS should be referred to an expert/ reference center for primary biopsy, expert pathology review, and multidisciplinary treatment. Immediate surgery of a mass without knowledge of its histological nature is strongly discouraged because it is associated with an increased risk of death due to inappropriate resection and increased risk of relapse. UPS is best treated by wide surgical excision. Sometimes amputation may be necessary to remove the whole lesion. Adjuvant radiotherapy is given for high-grade, large (>5 cm), deep-seated tumors, in limb sparing surgeries and when negative margins are not obtained. For non-operable sarcomas, primary radiation therapy could be an option, but usually doxorubicin containing regimens are preferred options in first-line setting for locally irresectable and/or metastatic lesions. Chemotherapy (CHT) with ifosfamide, trabectedin, dacarbazine, pazopanib have demonstrated efficacy in UPS and are registered and available in most European Union countries.PrognosisA 5-year overall survival rate of 48% has been reported for patients with head and neck tumors versus 77% for patients with tumors arising on the trunk and extremities. The childhood variant appears have better prognosis.Visit the Orphanet disease page for more resources. Last updated: 6/15/2014

MalaCards based summary : Undifferentiated Pleomorphic Sarcoma, also known as malignant fibrous histiocytoma, is related to neurofibromatosis, type iv, of riccardi and thyroid cancer, nonmedullary, 1, and has symptoms including abnormality of the peritoneum, abnormality of the lower limb and soft tissue sarcoma. An important gene associated with Undifferentiated Pleomorphic Sarcoma is MET (MET Proto-Oncogene, Receptor Tyrosine Kinase), and among its related pathways/superpathways are HIV Life Cycle and Class I MHC mediated antigen processing and presentation. The drugs Doxorubicin and Ifosfamide have been mentioned in the context of this disorder. Affiliated tissues include bone, lung and skin, and related phenotypes are growth/size/body region and mortality/aging

Wikipedia : 72 Undifferentiated pleomorphic sarcoma (UPS), also undifferentiated pleomorphic sarcoma (PUS) and... more...

Related Diseases for Undifferentiated Pleomorphic Sarcoma

Diseases related to Undifferentiated Pleomorphic Sarcoma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 422)
# Related Disease Score Top Affiliating Genes
1 neurofibromatosis, type iv, of riccardi 31.5 KIT KRAS PDGFRA
2 thyroid cancer, nonmedullary, 1 31.2 MET PIK3CA STAT3
3 oligodendroglioma 31.0 IDH1 IDH2 PDGFRA
4 desmoid tumor 30.4 KIT PDGFRA
5 malignant peripheral nerve sheath tumor 29.6 KIT MLANA PDGFRA
6 diaphyseal medullary stenosis with malignant fibrous histiocytoma 12.8
7 malignant fibrous histiocytoma of bone 12.5
8 ichthyosis tapered fingers midline groove up 11.9
9 kabuki syndrome 1 11.9
10 porphyria, acute intermittent 11.5
11 malignant fibroxanthoma 11.4
12 urticaria pigmentosa 11.2
13 frontotemporal lobar degeneration with tdp43 inclusions, grn-related 11.2
14 peripheral vascular disease 11.0
15 muscular dystrophy, duchenne type 11.0
16 carpal tunnel syndrome 11.0
17 pulmonary embolism 11.0
18 gout 11.0
19 hemochromatosis, type 1 10.9
20 lung cancer 10.9
21 esophageal cancer 10.9
22 narcolepsy 10.9
23 neuronal ceroid lipofuscinosis 10.9
24 refractive error 10.9
25 hernia, hiatus 10.9
26 rippling muscle disease 2 10.9
27 glaucoma-related pigment dispersion syndrome 10.9
28 alzheimer disease 10.9
29 sarcoma 10.8
30 ulcerative colitis 10.8
31 amyloidosis 10.8
32 psoriasis 10.8
33 cystic fibrosis 10.8
34 coronary heart disease 1 10.8
35 phenylketonuria 10.8
36 sinusitis 10.8
37 congenital toxoplasmosis 10.8
38 hyperthyroidism 10.8
39 bipolar disorder 10.8
40 liver cirrhosis 10.8
41 west syndrome 10.8
42 acute promyelocytic leukemia 10.8
43 testicular seminoma 10.8
44 wilson disease 10.8
45 langerhans cell histiocytosis 10.8
46 al amyloidosis 10.8
47 intrahepatic cholestasis 10.8
48 cystinosis 10.8
49 poliomyelitis 10.8
50 niemann-pick disease 10.8

Graphical network of the top 20 diseases related to Undifferentiated Pleomorphic Sarcoma:



Diseases related to Undifferentiated Pleomorphic Sarcoma

Symptoms & Phenotypes for Undifferentiated Pleomorphic Sarcoma

Human phenotypes related to Undifferentiated Pleomorphic Sarcoma:

55 31 (show all 10)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 abnormality of the peritoneum 55 31 hallmark (90%) Very frequent (99-80%) HP:0002585
2 abnormality of the lower limb 55 31 hallmark (90%) Very frequent (99-80%) HP:0002814
3 soft tissue sarcoma 55 31 hallmark (90%) Very frequent (99-80%) HP:0030448
4 abnormal test result 55 31 hallmark (90%) Very frequent (99-80%) HP:0500014
5 fever 55 31 frequent (33%) Frequent (79-30%) HP:0001945
6 abnormality of the musculature 55 31 frequent (33%) Frequent (79-30%) HP:0003011
7 weight loss 55 31 occasional (7.5%) Occasional (29-5%) HP:0001824
8 anorexia 55 31 occasional (7.5%) Occasional (29-5%) HP:0002039
9 abnormality of the upper limb 55 31 occasional (7.5%) Occasional (29-5%) HP:0002817
10 fatigue 55 31 occasional (7.5%) Occasional (29-5%) HP:0012378

MGI Mouse Phenotypes related to Undifferentiated Pleomorphic Sarcoma:

43
# Description MGI Source Accession Score Top Affiliating Genes
1 growth/size/body region MP:0005378 10.13 PDGFRA KIT PIK3CA KRAS CD74 EZR
2 mortality/aging MP:0010768 10.07 PDGFRA KIT PIK3CA KRAS CD74 EZR
3 immune system MP:0005387 10.06 PDGFRA KIT PIK3CA KRAS CD74 EZR
4 digestive/alimentary MP:0005381 9.93 PDGFRA KIT KRAS EZR MET STAT3
5 muscle MP:0005369 9.8 PDGFRA KIT KRAS MET PIK3CA STAT3
6 neoplasm MP:0002006 9.8 PDGFRA IDH2 KIT PIK3CA KRAS MET
7 no phenotypic analysis MP:0003012 9.63 PDGFRA KIT PIK3CA KRAS MET MLANA
8 respiratory system MP:0005388 9.43 PDGFRA KIT KRAS MET IDH1 STAT3
9 skeleton MP:0005390 9.17 PDGFRA KIT PIK3CA KRAS CD74 IDH1

Drugs & Therapeutics for Undifferentiated Pleomorphic Sarcoma

Drugs for Undifferentiated Pleomorphic Sarcoma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 99)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Doxorubicin Approved, Investigational Phase 3,Phase 2,Phase 1 23214-92-8 31703
2
Ifosfamide Approved Phase 3,Phase 2,Phase 1 3778-73-2 3690
3
Mechlorethamine Approved, Investigational Phase 3,Phase 2,Phase 1 51-75-2 4033
4
Dacarbazine Approved, Investigational Phase 3,Phase 2 4342-03-4 5351166
5
Vincristine Approved, Investigational Phase 3 2068-78-2, 57-22-7 5978
6
Etoposide Approved Phase 3,Phase 2 33419-42-0 36462
7
Carboplatin Approved Phase 3 41575-94-4 10339178 38904 498142
8
Cyclophosphamide Approved, Investigational Phase 3,Phase 2 50-18-0, 6055-19-2 2907
9
Lenograstim Approved, Investigational Phase 3 135968-09-1
10
Epirubicin Approved Phase 3 56420-45-2 41867
11
Dactinomycin Approved, Investigational Phase 3 50-76-0 457193 2019
12
Trabectedin Approved, Investigational Phase 3,Phase 2 114899-77-3 108150
13
Gemcitabine Approved Phase 3,Phase 1,Phase 2 95058-81-4 60750
14
Doxil Approved June 1999 Phase 3,Phase 2,Phase 1 31703
15
Docetaxel Approved May 1996, Investigational Phase 3,Phase 1,Phase 2 114977-28-5 148124 9877265
16 Isophosphamide mustard Phase 3,Phase 2,Phase 1 0
17 Alkylating Agents Phase 3,Phase 2,Phase 1
18 Topoisomerase Inhibitors Phase 3,Phase 2,Phase 1
19 Anti-Bacterial Agents Phase 3,Phase 2,Phase 1
20 Anti-Infective Agents Phase 3,Phase 2,Phase 1
21 Antibiotics, Antitubercular Phase 3,Phase 2,Phase 1
22 Antiviral Agents Phase 3,Phase 2,Phase 1
23 Nucleic Acid Synthesis Inhibitors Phase 3,Phase 2
24 Etoposide phosphate Phase 3,Phase 2
25 Immunosuppressive Agents Phase 3,Phase 2,Phase 1
26 Antimitotic Agents Phase 3,Phase 2,Phase 1
27 Antineoplastic Agents, Phytogenic Phase 3,Phase 2
28 Antirheumatic Agents Phase 3,Phase 2,Phase 1
29 Antimetabolites Phase 3,Phase 1,Phase 2
30 Antimetabolites, Antineoplastic Phase 3,Phase 1,Phase 2
31
Ritonavir Approved, Investigational Phase 2 155213-67-5 392622
32
Indinavir Approved Phase 2 150378-17-9 5362440
33
Temozolomide Approved, Investigational Phase 2 85622-93-1 5394
34
Sorafenib Approved, Investigational Phase 2 284461-73-0 216239 406563
35
Miconazole Approved, Investigational, Vet_approved Phase 1, Phase 2 22916-47-8 4189
36
Everolimus Approved Phase 1, Phase 2 159351-69-6 6442177
37
Sunitinib Approved, Investigational Phase 2 341031-54-7, 557795-19-4 5329102
38
Sirolimus Approved, Investigational Phase 1, Phase 2 53123-88-9 46835353 6436030 5284616
39
Romidepsin Approved, Investigational Phase 2 128517-07-7 5352062
40
nivolumab Approved Phase 2,Phase 1 946414-94-4
41
Pembrolizumab Approved Phase 1, Phase 2 1374853-91-4
42
Cisplatin Approved Phase 2 15663-27-1 2767 441203 84093
43
Methotrexate Approved Phase 2 1959-05-2, 59-05-2 126941
44
Bevacizumab Approved, Investigational Phase 2 216974-75-3
45
Niacin Approved, Investigational, Nutraceutical Phase 2 59-67-6 938
46
Nicotinamide Approved, Investigational, Nutraceutical Phase 2 98-92-0 936
47
Folic Acid Approved, Nutraceutical, Vet_approved Phase 2 59-30-3 6037
48
leucovorin Approved, Nutraceutical Phase 2 58-05-9 143 6006
49
Camptothecin Experimental Phase 2 7689-03-4
50 Exatecan Investigational Phase 2 171335-80-1

Interventional clinical trials:

(show top 50) (show all 61)

# Name Status NCT ID Phase Drugs
1 Ifosfamide and Doxorubicin, Radiation Therapy, and/or Surgery in Treating Young Patients With Localized Soft Tissue Sarcoma Unknown status NCT00334854 Phase 3 doxorubicin hydrochloride;ifosfamide
2 Surgery Followed by Chemotherapy in Treating Young Patients With Soft Tissue Sarcoma Completed NCT00002898 Phase 3 carboplatin;cyclophosphamide;epirubicin hydrochloride;etoposide;ifosfamide;vincristine sulfate
3 Observation, Radiation Therapy, Combination Chemotherapy, and/or Surgery in Treating Young Patients With Soft Tissue Sarcoma Completed NCT00346164 Phase 3 doxorubicin hydrochloride;ifosfamide
4 Surgery With or Without Radiation Therapy in Treating Patients With Primary Soft Tissue Sarcoma of the Retroperitoneum or Pelvis Completed NCT00091351 Phase 3
5 Surgery With or Without Combination Chemotherapy in Treating Patients With Lung Metastases From Soft Tissue Sarcoma Completed NCT00002764 Phase 3 doxorubicin hydrochloride;ifosfamide
6 Ifosfamide or Doxorubicin in Treating Patients With Advanced or Metastatic Soft Tissue Sarcoma Completed NCT00003212 Phase 3 doxorubicin hydrochloride;ifosfamide
7 Doxorubicin With or Without Ifosfamide and Pegfilgrastim in Treating Patients With Locally Advanced or Metastatic Soft Tissue Sarcoma Completed NCT00061984 Phase 3 doxorubicin hydrochloride;ifosfamide
8 Combination Chemotherapy With or Without Hyperthermia Therapy in Treating Patients With Soft Tissue Sarcoma Completed NCT00003052 Phase 3 doxorubicin hydrochloride;etoposide;ifosfamide
9 Surgery With or Without Chemotherapy in Treating Patients With Soft Tissue Sarcoma Completed NCT00002641 Phase 3 doxorubicin hydrochloride;ifosfamide;isolated perfusion
10 Localized High-Risk Soft Tissue Sarcomas Of The Extremities And Trunk Wall In Adults: An Integrating Approach Comprising Standard Vs Histotype-Tailored Neoadjuvant Chemotherapy Recruiting NCT01710176 Phase 3 epirubicin 60 mg/m2/day (days 1, 2) and ifosfamide 3 g/m2/day (days 1, 2, 3);gemcitabine 900 mg/m2 (days 1 and 8) and docetaxel 75 mg/m2 (day 8);trabectedin 1.3 mg/m2;high-dose ifosfamide 14 g/m2, given in in 14 days;etoposide 150 mg/m2/day (days 1, 2, 3) and ifosfamide 3g/m2/day (days 1, 2, 3);gemcitabine 1800 mg/m2 (day 1) and dacarbazine 500 mg/m2 (day 1)
11 Radiation Therapy With or Without Combination Chemotherapy or Pazopanib Hydrochloride Before Surgery in Treating Patients With Newly Diagnosed Non-rhabdomyosarcoma Soft Tissue Sarcomas That Can Be Removed by Surgery Recruiting NCT02180867 Phase 2, Phase 3 Doxorubicin Hydrochloride;Ifosfamide;Pazopanib Hydrochloride
12 Surgery With or Without Radiation Therapy in Untreated Nonmetastatic Retroperitoneal Sarcoma Recruiting NCT01344018 Phase 3
13 A Study of Doxorubicin Plus Olaratumab (LY3012207) in Participants With Advanced or Metastatic Soft Tissue Sarcoma Active, not recruiting NCT02451943 Phase 3 Olaratumab;Doxorubicin;Placebo
14 Randomised Trial of Volume of Post-operative Radiotherapy Given to Adult Patients With eXtremity Soft Tissue Sarcoma Active, not recruiting NCT00423618 Phase 3
15 Doxorubicin Hydrochloride or Trabectedin in Treating Patients With Previously Untreated Advanced or Metastatic Soft Tissue Sarcoma Terminated NCT01189253 Phase 2, Phase 3 doxorubicin hydrochloride;trabectedin
16 External-Beam Radiation Therapy With or Without Indinavir and Ritonavir in Treating Patients With Brain Metastases Unknown status NCT00637637 Phase 2 indinavir sulfate;ritonavir
17 Brostallicin or Doxorubicin as First-Line Therapy in Treating Patients With Relapsed, Refractory, or Metastatic Soft Tissue Sarcoma Unknown status NCT00410462 Phase 2 brostallicin;doxorubicin hydrochloride
18 Temozolomide in Treating Patients With Advanced Soft Tissue Sarcoma Unknown status NCT00003718 Phase 2 temozolomide
19 Phase II Study of Sunitinib Malate for Metastatic and/or Surgically Unresectable Soft Tissue Sarcoma Completed NCT00400569 Phase 2 Sunitinib Malate (SU011248)
20 Imatinib Mesylate in Treating Patients With Advanced Soft Tissue Sarcoma or Bone Sarcoma Completed NCT00031915 Phase 2 imatinib mesylate
21 Sunitinib in Treating Patients With Metastatic, Locally Advanced, or Locally Recurrent Sarcomas Completed NCT00474994 Phase 2 sunitinib malate
22 Vismodegib and Gamma-Secretase/Notch Signalling Pathway Inhibitor RO4929097 in Treating Patients With Advanced or Metastatic Sarcoma Completed NCT01154452 Phase 1, Phase 2 Gamma-Secretase Inhibitor RO4929097;Vismodegib
23 Alisertib in Treating Patients With Advanced or Metastatic Sarcoma Completed NCT01653028 Phase 2 Alisertib
24 Safety and Efficacy Study of REOLYSIN® in the Treatment of Bone and Soft Tissue Sarcomas Metastatic to the Lung Completed NCT00503295 Phase 2
25 Imatinib Mesylate in Treating Patients With Locally Advanced or Metastatic Dermatofibrosarcoma Protuberans or Giant Cell Fibroblastoma Completed NCT00085475 Phase 2 imatinib mesylate
26 AZD0530 in Treating Patients With Recurrent Locally Advanced or Metastatic Soft Tissue Sarcoma Completed NCT00659360 Phase 2 saracatinib
27 Sorafenib in Treating Patients With Metastatic, Locally Advanced, or Recurrent Sarcoma Completed NCT00245102 Phase 2 sorafenib tosylate
28 Depsipeptide (Romidepsin) in Treating Patients With Metastatic or Unresectable Soft Tissue Sarcoma Completed NCT00112463 Phase 2 romidepsin
29 Safety and Efficacy Study of Torisel and Liposomal Doxorubicin for Patients With Recurrent Sarcoma Completed NCT00949325 Phase 1, Phase 2 temsirolimus (Torisel) plus liposomal doxorubicin (Doxil)
30 Soblidotin in Treating Patients With Advanced or Metastatic Soft Tissue Sarcoma Completed NCT00064220 Phase 2 soblidotin
31 BI 2536 in Treating Patients With Recurrent or Metastatic Solid Tumors Completed NCT00526149 Phase 2 BI 2536
32 Ecteinascidin 743 in Treating Patients With Advanced Soft Tissue Sarcoma Completed NCT00003939 Phase 2 trabectedin
33 STI571 in Treating Patients With Recurrent or Refractory Soft Tissue Sarcoma Completed NCT00006357 Phase 1, Phase 2 imatinib mesylate
34 Perifosine in Treating Patients With Metastatic or Locally Advanced Soft Tissue Sarcoma Completed NCT00053794 Phase 2 perifosine
35 Brostallicin in Treating Patients With Locally Advanced or Metastatic Soft Tissue Sarcoma Completed NCT00041249 Phase 2 brostallicin
36 Exatecan Mesylate in Treating Patients With Advanced Soft Tissue Sarcoma Completed NCT00041236 Phase 2 exatecan mesylate
37 Pembrolizumab and Gemcitabine Chemotherapy in Leiomyosarcoma and Undifferentiated Pleomorphic Sarcoma Recruiting NCT03123276 Phase 1, Phase 2 Pembrolizumab;Gemcitabine
38 Study of Neoadjuvant Checkpoint Blockade in Patients With Surgically Resectable Undifferentiated Pleomorphic Sarcoma and Dedifferentiated Liposarcoma Recruiting NCT03307616 Phase 2 Nivolumab;Ipilimumab
39 Nivolumab (Opdivo®) Plus ABI-009 (Nab-rapamycin) for Advanced Sarcoma Recruiting NCT03190174 Phase 1, Phase 2 Nab-Rapamycin
40 Multi-Arm Study to Test the Efficacy of Immunotherapeutic Agents in Multiple Sarcoma Subtypes Recruiting NCT02815995 Phase 2 Durvalumab;Tremelimumab
41 Sapanisertib or Pazopanib Hydrochloride in Treating Patients With Locally Advanced or Metastatic Sarcoma Recruiting NCT02601209 Phase 1, Phase 2 Pazopanib Hydrochloride;Sapanisertib
42 Doxorubicin With Upfront Dexrazoxane Plus Olaratumab for the Treatment of Advanced or Metastatic Soft Tissue Sarcoma Recruiting NCT02584309 Phase 2 Dexrazoxane;Doxorubicin;Olaratumab
43 Nivolumab With or Without Ipilimumab in Treating Patients With Metastatic Sarcoma That Cannot Be Removed by Surgery Recruiting NCT02500797 Phase 2
44 Gemcitabine Hydrochloride With or Without Pazopanib Hydrochloride in Treating Patients With Refractory Soft Tissue Sarcoma Recruiting NCT01532687 Phase 2 Gemcitabine Hydrochloride;Pazopanib Hydrochloride
45 A Study of Epacadostat, an IDO1 Inhibitor, in Combination With Pembrolizumab in Patients With Metastatic and/or Locally Advanced Sarcoma Recruiting NCT03414229 Phase 2 Epacadostat;Pembrolizumab
46 A Study of Bevacizumab in Combination With Chemotherapy for Treatment of Osteosarcoma Active, not recruiting NCT00667342 Phase 2 Cisplatin;Doxorubicin;Methotrexate;Ifosfamide;etoposide
47 Talimogene Laherparepvec and Radiation Therapy in Treating Patients With Newly Diagnosed Soft Tissue Sarcoma That Can Be Removed by Surgery Suspended NCT02923778 Phase 2
48 Gemcitabine and Docetaxel in Combination With Pazopanib (Gem/Doce/Pzb) for the Neoadjuvant Treatment of Soft Tissue Sarcoma (STS) Terminated NCT01418001 Phase 1, Phase 2 Pazopanib 400 mg QD - Gemcitabine and Docetaxel in Combination
49 Intensity-Modulated Radiation Therapy in Treating Patients Undergoing Surgery for Stage IB, Stage II, or Stage III Soft Tissue Sarcoma Terminated NCT00740597 Phase 2
50 Celecoxib and Radiation Therapy in Treating Patients With Stage II or Stage III Soft Tissue Sarcoma of the Arm, Hand, Leg, or Foot That Has Been Removed by Surgery Completed NCT00450736 Phase 1 celecoxib

Search NIH Clinical Center for Undifferentiated Pleomorphic Sarcoma

Genetic Tests for Undifferentiated Pleomorphic Sarcoma

Anatomical Context for Undifferentiated Pleomorphic Sarcoma

MalaCards organs/tissues related to Undifferentiated Pleomorphic Sarcoma:

38
Bone, Lung, Skin, Testes, Liver, Brain, Endothelial

Publications for Undifferentiated Pleomorphic Sarcoma

Articles related to Undifferentiated Pleomorphic Sarcoma:

(show top 50) (show all 79)
# Title Authors Year
1
Biology and Management of Undifferentiated Pleomorphic Sarcoma, Myxofibrosarcoma, and Malignant Peripheral Nerve Sheath Tumors: State of the Art and Perspectives. ( 29220302 )
2018
2
Establishment and proteomic characterization of a novel cell line, NCC-UPS2-C1, derived from a patient with undifferentiated pleomorphic sarcoma. ( 29359268 )
2018
3
Extended right hepatectomy with inferior vena cava replacement as an aggressive rescue procedure for threatening venous outflow occlusion in a pretreated undifferentiated pleomorphic sarcoma of the liver. ( 29366308 )
2018
4
Primary undifferentiated pleomorphic sarcoma of the thyroid: A case report and review of the literature. ( 29443775 )
2018
5
Analysis of the immune infiltrate in undifferentiated pleomorphic sarcoma of the extremity and trunk in response to radiotherapy: Rationale for combination neoadjuvant immune checkpoint inhibition and radiotherapy. ( 29308306 )
2018
6
LN2, CD10, and Ezrin Do Not Distinguish Between Atypical Fibroxanthoma and Undifferentiated Pleomorphic Sarcoma or Predict Clinical Outcome. ( 28079637 )
2017
7
Undifferentiated pleomorphic sarcoma of the skin - a UV-induced occupational skin disease? ( 28220622 )
2017
8
A case of a resected benign myxoma-like hemorrhagic cyst, which later recurred as undifferentiated pleomorphic sarcoma in the left atrium. ( 28422827 )
2017
9
Radiation therapy for recurrent cardiac undifferentiated pleomorphic sarcoma after three operations. ( 28434140 )
2017
10
Cardiac undifferentiated pleomorphic sarcoma incidentally diagnosed during mitral valve replacement. ( 28127801 )
2017
11
Aggressively progressing primary undifferentiated pleomorphic sarcoma in the eyelid: A case report and review of the literature. ( 28471957 )
2017
12
Undifferentiated pleomorphic sarcoma of the neck - A case report. ( 29031178 )
2017
13
Efficacy In Vitro of Caffeine and Valproic Acid on Patient-Derived Undifferentiated Pleomorphic Sarcoma and Rhabdomyosarcoma Cell Lines. ( 28739691 )
2017
14
Diagnostic utility of IDH1/2 mutations to distinguish dedifferentiated chondrosarcoma from undifferentiated pleomorphic sarcoma of bone. ( 28552826 )
2017
15
Radiation-induced undifferentiated pleomorphic sarcoma of the heart: A case report. ( 29305110 )
2017
16
Undifferentiated pleomorphic sarcoma: indolent, tail-like recurrence of a high-grade tumor. ( 28932893 )
2017
17
Primary Culture of Undifferentiated Pleomorphic Sarcoma: Molecular Characterization and Response to Anticancer Agents. ( 29292724 )
2017
18
PDGFRa amplification in multiple skin lesions of undifferentiated pleomorphic sarcoma: A clue for intimal sarcoma metastases. ( 28105789 )
2017
19
miR-152 down-regulation is associated with MET up-regulation in leiomyosarcoma and undifferentiated pleomorphic sarcoma. ( 27900663 )
2017
20
A novel anionic-phosphate-platinum complex effectively targets an undifferentiated pleomorphic sarcoma better than cisplatinum and doxorubicin in a patient-derived orthotopic xenograft (PDOX). ( 28968995 )
2017
21
Primary Undifferentiated Pleomorphic Sarcoma of the Penis. ( 29230132 )
2017
22
A Trp53fl/flPtenfl/fl mouse model of undifferentiated pleomorphic sarcoma mediated by adeno-Cre injection and in vivo bioluminescence imaging. ( 28841687 )
2017
23
Conception and Management of a Poorly Understood Spectrum of Dermatologic Neoplasms: Atypical Fibroxanthoma, Pleomorphic Dermal Sarcoma, and Undifferentiated Pleomorphic Sarcoma. ( 28762020 )
2017
24
Undifferentiated Pleomorphic Sarcoma and the Importance of Considering the Oncogenic and Immune-Suppressant Role of the Human T-Cell Lymphotropic Virus Type 1: A Case Report. ( 28596939 )
2017
25
Corrigendum to "Diagnostic utility of IDH1/2 mutations to distinguish dedifferentiated chondrosarcoma from undifferentiated pleomorphic sarcoma of bone" [Hum Pathol 2017;65:239-46]. ( 29146074 )
2017
26
Co-targeting PI3K, mTOR, and IGF1R with small molecule inhibitors for treating undifferentiated pleomorphic sarcoma. ( 29099264 )
2017
27
Identifying actionable variants using next generation sequencing in patients with a historical diagnosis of undifferentiated pleomorphic sarcoma. ( 28891048 )
2017
28
A Rare Cause of Dyspnea: Undifferentiated Pleomorphic Sarcoma in the Left Atrium. ( 29118888 )
2017
29
Optimal Percent Myxoid Component to Predict Outcome in High-Grade Myxofibrosarcoma and Undifferentiated Pleomorphic Sarcoma. ( 26759307 )
2016
30
Successful Salvage Radiotherapy for a Chemo-refractory, Non-resectable, Undifferentiated Pleomorphic Sarcoma Lung Metastasis with Pericardial Involvement: A Case Report. ( 26918213 )
2016
31
Undifferentiated pleomorphic sarcoma of the vocal fold. ( 27929601 )
2016
32
Analysis of Clinical and Molecular Factors Impacting Oncologic Outcomes in Undifferentiated Pleomorphic Sarcoma. ( 26847678 )
2016
33
High prevalence of MiTF staining in undifferentiated pleomorphic sarcoma: caution in use of melanocytic markers in sarcoma. ( 27926791 )
2016
34
Primary undifferentiated pleomorphic sarcoma of the breast in a young female: a case report. ( 27421259 )
2016
35
Phosphorylation of STAT3 in Undifferentiated Pleomorphic Sarcoma Is Correlated with a Favorable Prognosis. ( 27652596 )
2016
36
Undifferentiated Pleomorphic Sarcoma Presenting as Abdominal Pain with a Pulsatile Mass. ( 27563479 )
2016
37
High-grade undifferentiated pleomorphic sarcoma of the pelvis: A case report and review of literature. ( 27453666 )
2016
38
Intraventricular Undifferentiated Pleomorphic Sarcoma: A Case Report. ( 28003940 )
2016
39
Radiation-induced undifferentiated pleomorphic sarcoma after radiation therapy for a desmoid tumour. ( 26725422 )
2016
40
Study of Epithelial to Mesenchymal Transition in Atypical Fibroxanthoma and Undifferentiated Pleomorphic Sarcoma to Discern an Epithelial Origin. ( 26999338 )
2016
41
RAS/MAPK pathway hyperactivation determines poor prognosis in undifferentiated pleomorphic sarcomas. ( 26479291 )
2016
42
An unusual cause of life-threatening right-sided heart failure: undifferentiated pleomorphic sarcoma in the right ventricular outflow tract. ( 26670651 )
2016
43
Tail of Superficial Myxofibrosarcoma and Undifferentiated Pleomorphic Sarcoma After Preoperative Radiotherapy. ( 27127141 )
2016
44
Xenograft models for undifferentiated pleomorphic sarcoma not otherwise specified are essential for preclinical testing of therapeutic agents. ( 27446424 )
2016
45
Undifferentiated pleomorphic sarcoma with focally rhabdomyosarcomatous differentiation of the ovary. ( 27352574 )
2016
46
Primary Mesenteric Undifferentiated Pleomorphic Sarcoma Masquerading as a Colon Carcinoma: A Case Report and Review of the Literature. ( 26380135 )
2015
47
Undifferentiated pleomorphic sarcoma with co-existence of KRAS/PIK3CA mutations. ( 26339434 )
2015
48
MET gene copy number alterations and expression of MET and hepatocyte growth factor are potential biomarkers in angiosarcomas and undifferentiated pleomorphic sarcomas. ( 25844809 )
2015
49
Recurrent PRDM10 gene fusions in undifferentiated pleomorphic sarcoma. ( 25516889 )
2015
50
Elevated expression of HSP90 and the antitumor effect of an HSP90 inhibitor via inactivation of the Akt/mTOR pathway in undifferentiated pleomorphic sarcoma. ( 26502919 )
2015

Variations for Undifferentiated Pleomorphic Sarcoma

Cosmic variations for Undifferentiated Pleomorphic Sarcoma:

9 (show all 21)
# Cosmic Mut ID Gene Symbol COSMIC Disease Classification
(Primary site, Site subtype, Primary histology, Histology subtype)
Mutation CDS Mutation AA Conf
1 COSM5154 PTEN soft tissue,retroperitoneum,malignant fibrous histiocytoma-pleomorphic sarcoma,NS c.697C>T p.R233* 12
2 COSM532 KRAS soft tissue,upper arm,malignant fibrous histiocytoma-pleomorphic sarcoma,NS c.38G>A p.G13D 12
3 COSM483 HRAS soft tissue,upper arm,malignant fibrous histiocytoma-pleomorphic sarcoma,NS c.35G>T p.G12V 12
4 COSM5033 PTEN soft tissue,upper extremity,malignant fibrous histiocytoma-pleomorphic sarcoma,NS c.389G>A p.R130Q 11
5 COSM575 NRAS soft tissue,skin,sarcoma,NS c.38G>C p.G13A 11
6 COSM520 KRAS soft tissue,lung,sarcoma,NS c.35G>T p.G12V 11
7 COSM487 HRAS soft tissue,neck,sarcoma,NS c.37G>A p.G13S 11
8 COSM10648 TP53 soft tissue,arm,leiomyosarcoma,NS c.524G>A p.R175H 4
9 COSM45253 TP53 soft tissue,arm,rhabdomyosarcoma,NS c.611A>G p.E204G 4
10 COSM44683 TP53 soft tissue,arm,leiomyosarcoma,NS c.652G>A p.V218M 4
11 COSM43632 TP53 soft tissue,bone,leiomyosarcoma,NS c.493C>T p.Q165* 4
12 COSM45256 TP53 soft tissue,arm,rhabdomyosarcoma,NS c.871A>C p.K291Q 4
13 COSM5923 PTEN soft tissue,retroperitoneum,leiomyosarcoma,NS c.1027-7T>C p.? 4
14 COSM775 PIK3CA soft tissue,retroperitoneum,gastrointestinal stromal tumour,spindle c.3140A>G p.H1047R 4
15 COSM36289 PIK3CA soft tissue,retroperitoneum,gastrointestinal stromal tumour,spindle c.3143A>G p.H1048R 4
16 COSM27375 PIK3CA soft tissue,retroperitoneum,gastrointestinal stromal tumour,spindle c.3103G>A p.A1035T 4
17 COSM584 NRAS soft tissue,retroperitoneum,rhabdomyosarcoma,NS c.182A>G p.Q61R 4
18 COSM580 NRAS soft tissue,retroperitoneum,rhabdomyosarcoma,NS c.181C>A p.Q61K 4
19 COSM1745010 MYOD1 soft tissue,NS,rhabdomyosarcoma,spindle cell c.365T>G p.L122R 4
20 COSM516 KRAS soft tissue,lung,leiomyosarcoma,NS c.34G>T p.G12C 4
21 COSM133687 KIT soft tissue,retroperitoneum,gastrointestinal stromal tumour,spindle c.1372G>A p.D458N 4

Expression for Undifferentiated Pleomorphic Sarcoma

Search GEO for disease gene expression data for Undifferentiated Pleomorphic Sarcoma.

Pathways for Undifferentiated Pleomorphic Sarcoma

Pathways related to Undifferentiated Pleomorphic Sarcoma according to GeneCards Suite gene sharing:

(show all 49)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.62 IDH1 KIT KRAS MET PDGFRA PIK3CA
2
Show member pathways
13.34 CD74 KIT KRAS MET PDGFRA PIK3CA
3
Show member pathways
13.28 EZR KIT KRAS MET PDGFRA PIK3CA
4
Show member pathways
13.22 KIT KRAS MET PDGFRA PIK3CA STAT3
5
Show member pathways
12.92 KIT KRAS MET PDGFRA STAT3
6
Show member pathways
12.83 KIT KRAS MET PDGFRA PIK3CA
7
Show member pathways
12.73 KIT KRAS MET PDGFRA PIK3CA STAT3
8
Show member pathways
12.7 KRAS MET PDGFRA PIK3CA STAT3
9
Show member pathways
12.67 KIT KRAS MET PIK3CA
10 12.66 KIT KRAS MET PDGFRA
11
Show member pathways
12.64 KIT KRAS MET PDGFRA PIK3CA
12
Show member pathways
12.6 KRAS MET PIK3CA STAT3
13 12.5 EZR KRAS PDGFRA PIK3CA
14
Show member pathways
12.5 KIT KRAS MET PDGFRA PIK3CA
15
Show member pathways
12.47 KIT KRAS MET PDGFRA PIK3CA
16
Show member pathways
12.46 KRAS PDGFRA PIK3CA STAT3
17 12.46 KIT KRAS MET PDGFRA PIK3CA STAT3
18 12.43 CD74 KIT MLANA STAT3
19
Show member pathways
12.35 KRAS MET PDGFRA PIK3CA
20
Show member pathways
12.33 KRAS MET PIK3CA STAT3
21
Show member pathways
12.27 KRAS MET PDGFRA PIK3CA
22
Show member pathways
12.2 KIT KRAS PDGFRA PIK3CA
23
Show member pathways
12.14 KRAS MET PIK3CA STAT3
24
Show member pathways
12.08 KIT PIK3CA STAT3
25
Show member pathways
12.08 KRAS MET PDGFRA PIK3CA
26
Show member pathways
12.06 KRAS PIK3CA STAT3
27
Show member pathways
12.04 KRAS PIK3CA STAT3
28
Show member pathways
12.01 EZR PIK3CA STAT3
29 12.01 KIT MET PDGFRA
30 11.98 KRAS PIK3CA STAT3
31 11.98 EZR KRAS MET MIR152 PDGFRA PIK3CA
32
Show member pathways
11.97 KRAS PIK3CA STAT3
33
Show member pathways
11.95 KRAS PIK3CA STAT3
34 11.94 KRAS PIK3CA STAT3
35 11.86 EZR KRAS MET PIK3CA STAT3
36
Show member pathways
11.8 KRAS PDGFRA PIK3CA STAT3
37 11.78 KRAS PIK3CA STAT3
38 11.75 KRAS PIK3CA STAT3
39
Show member pathways
11.73 KRAS PIK3CA STAT3
40
Show member pathways
11.66 KIT KRAS PIK3CA
41 11.24 KRAS PDGFRA PIK3CA STAT3
42
Show member pathways
11.2 MET PIK3CA STAT3
43 11.14 KRAS PIK3CA
44 11.09 PIK3CA STAT3
45 11.07 PDGFRA PIK3CA
46 11.04 PDGFRA PIK3CA
47 11.02 PDGFRA PIK3CA
48 10.91 IDH1 KIT KRAS MET PDGFRA PIK3CA
49
Show member pathways
10.74 IDH1 IDH2

GO Terms for Undifferentiated Pleomorphic Sarcoma

Biological processes related to Undifferentiated Pleomorphic Sarcoma according to GeneCards Suite gene sharing:

(show all 15)
# Name GO ID Score Top Affiliating Genes
1 phosphorylation GO:0016310 9.85 KIT MET PDGFRA PIK3CA STAT3
2 positive regulation of gene expression GO:0010628 9.83 EZR KIT KRAS STAT3
3 peptidyl-tyrosine phosphorylation GO:0018108 9.72 KIT MET PDGFRA
4 cytokine-mediated signaling pathway GO:0019221 9.71 KIT KRAS STAT3
5 MAPK cascade GO:0000165 9.71 KIT KRAS MET PDGFRA
6 transmembrane receptor protein tyrosine kinase signaling pathway GO:0007169 9.65 KIT MET PDGFRA
7 cell chemotaxis GO:0060326 9.54 KIT PDGFRA PIK3CA
8 energy homeostasis GO:0097009 9.51 PIK3CA STAT3
9 2-oxoglutarate metabolic process GO:0006103 9.49 IDH1 IDH2
10 positive regulation of protein kinase B signaling GO:0051897 9.46 KIT MET PDGFRA PIK3CA
11 positive regulation of phospholipase C activity GO:0010863 9.43 KIT PDGFRA
12 phosphatidylinositol-mediated signaling GO:0048015 9.43 EZR PDGFRA PIK3CA
13 isocitrate metabolic process GO:0006102 9.32 IDH1 IDH2
14 glyoxylate cycle GO:0006097 8.96 IDH1 IDH2
15 phosphatidylinositol phosphorylation GO:0046854 8.92 KIT MET PDGFRA PIK3CA

Molecular functions related to Undifferentiated Pleomorphic Sarcoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 nucleotide binding GO:0000166 9.67 KIT KRAS MET PDGFRA
2 protein tyrosine kinase activity GO:0004713 9.61 KIT MET PDGFRA
3 Ras guanyl-nucleotide exchange factor activity GO:0005088 9.5 KIT MET PDGFRA
4 transmembrane receptor protein tyrosine kinase activity GO:0004714 9.43 KIT MET PDGFRA
5 cytokine binding GO:0019955 9.4 CD74 KIT
6 isocitrate dehydrogenase activity GO:0004448 9.16 IDH1 IDH2
7 isocitrate dehydrogenase (NADP+) activity GO:0004450 8.96 IDH1 IDH2
8 phosphatidylinositol-4,5-bisphosphate 3-kinase activity GO:0046934 8.92 KIT MET PDGFRA PIK3CA

Sources for Undifferentiated Pleomorphic Sarcoma

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