UPS
MCID: UND005
MIFTS: 51

Undifferentiated Pleomorphic Sarcoma (UPS)

Categories: Bone diseases, Cancer diseases, Immune diseases, Rare diseases, Skin diseases

Aliases & Classifications for Undifferentiated Pleomorphic Sarcoma

MalaCards integrated aliases for Undifferentiated Pleomorphic Sarcoma:

Name: Undifferentiated Pleomorphic Sarcoma 54 60 6 17
Malignant Fibrous Histiocytoma 54 74
Ups 54 60
Malignant Fibrohistiocytic Tumors 54

Characteristics:

Orphanet epidemiological data:

60
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: 60  
Rare bone diseases


External Ids:

MESH via Orphanet 46 D051677
ICD10 via Orphanet 35 C49.9
UMLS via Orphanet 75 C0334463
Orphanet 60 ORPHA2023
UMLS 74 C0334463

Summaries for Undifferentiated Pleomorphic Sarcoma

NIH Rare Diseases : 54 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.

MalaCards based summary : Undifferentiated Pleomorphic Sarcoma, also known as malignant fibrous histiocytoma, is related to diaphyseal medullary stenosis with malignant fibrous histiocytoma and malignant fibrous histiocytoma of bone. An important gene associated with Undifferentiated Pleomorphic Sarcoma is KRAS (KRAS Proto-Oncogene, GTPase). The drugs Ifosfamide and Mechlorethamine have been mentioned in the context of this disorder. Affiliated tissues include bone, skin and brain, and related phenotypes are abnormality of the lower limb and abnormality of the peritoneum

Wikipedia : 77 Undifferentiated pleomorphic sarcoma (UPS), previously malignant fibrous histiocytoma, is a type of... 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 3942)
# Related Disease Score Top Affiliating Genes
1 diaphyseal medullary stenosis with malignant fibrous histiocytoma 13.0
2 malignant fibrous histiocytoma of bone 12.6
3 ichthyosis tapered fingers midline groove up 12.1
4 kabuki syndrome 1 12.1
5 malignant fibroxanthoma 11.8
6 porphyria, acute intermittent 11.6
7 malignant skin fibrous histiocytoma 11.5
8 mastocytosis, cutaneous 11.4
9 frontotemporal lobar degeneration with tdp43 inclusions, grn-related 11.4
10 histiocytoma, angiomatoid fibrous 11.3
11 urachal cancer 11.3
12 amyloidosis, hereditary, transthyretin-related 11.2
13 vascular disease 11.2
14 peripheral vascular disease 11.2
15 atherosclerosis susceptibility 11.2
16 carpal tunnel syndrome 11.1
17 muscular dystrophy, duchenne type 11.1
18 gout 11.1
19 migraine with or without aura 1 11.1
20 wilson disease 11.1
21 hemochromatosis, type 1 11.1
22 cystic fibrosis 11.1
23 psoriasis 11.1
24 esophageal cancer 11.1
25 lung cancer 11.1
26 phenylketonuria 11.1
27 sarcoma 11.1
28 hernia, hiatus 11.1
29 narcolepsy 11.1
30 nephrolithiasis, calcium oxalate 11.1
31 refractive error 11.0
32 alzheimer disease 11.0
33 sleep disorder 11.0
34 glaucoma-related pigment dispersion syndrome 11.0
35 rippling muscle disease 2 11.0
36 coronary heart disease 1 11.0
37 bipolar disorder 11.0
38 ulcerative colitis 11.0
39 spinal cord injury 11.0
40 cleft lip/palate 11.0
41 angina pectoris 11.0
42 fatty liver disease 11.0
43 amyloidosis 11.0
44 lymphedema 11.0
45 endometriosis 11.0
46 liver cirrhosis 11.0
47 hyperthyroidism 11.0
48 chronic kidney failure 11.0
49 fabry disease 11.0
50 bronchitis 11.0

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:

60 33 (show all 10)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 abnormality of the lower limb 60 33 hallmark (90%) Very frequent (99-80%) HP:0002814
2 abnormality of the peritoneum 60 33 hallmark (90%) Very frequent (99-80%) HP:0002585
3 soft tissue sarcoma 60 33 hallmark (90%) Very frequent (99-80%) HP:0030448
4 fever 60 33 frequent (33%) Frequent (79-30%) HP:0001945
5 abnormality of the musculature 60 33 frequent (33%) Frequent (79-30%) HP:0003011
6 fatigue 60 33 occasional (7.5%) Occasional (29-5%) HP:0012378
7 weight loss 60 33 occasional (7.5%) Occasional (29-5%) HP:0001824
8 anorexia 60 33 occasional (7.5%) Occasional (29-5%) HP:0002039
9 abnormality of the upper limb 60 33 occasional (7.5%) Occasional (29-5%) HP:0002817
10 abnormal test result 60 Very frequent (99-80%)

Drugs & Therapeutics for Undifferentiated Pleomorphic Sarcoma

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

(show top 50) (show all 106)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Ifosfamide Approved Phase 3,Phase 2,Phase 1,Not Applicable 3778-73-2 3690
2
Mechlorethamine Approved, Investigational Phase 3,Phase 2,Phase 1,Not Applicable 51-75-2 4033
3
Doxorubicin Approved, Investigational Phase 3,Phase 2,Phase 1,Not Applicable 23214-92-8 31703
4
Etoposide Approved Phase 3,Phase 2 33419-42-0 36462
5
Dactinomycin Approved, Investigational Phase 3 50-76-0 457193 2019
6
Vincristine Approved, Investigational Phase 3 2068-78-2, 57-22-7 5978
7
Carboplatin Approved Phase 3 41575-94-4 38904 10339178 498142
8
Epirubicin Approved Phase 3 56420-45-2 41867
9
Cyclophosphamide Approved, Investigational Phase 3,Phase 2 6055-19-2, 50-18-0 2907
10
Lenograstim Approved, Investigational Phase 3 135968-09-1
11
Trabectedin Approved, Investigational Phase 3,Phase 2 114899-77-3 108150
12
Dacarbazine Approved, Investigational Phase 3 4342-03-4 5351166
13
Gemcitabine Approved Phase 3,Phase 1,Phase 2 95058-81-4 60750
14
Docetaxel Approved, Investigational Phase 3,Phase 1,Phase 2 114977-28-5 148124
15 Olaratumab Approved, Investigational Phase 3,Phase 2 1024603-93-7
16
Doxil Approved June 1999 Phase 3,Phase 2,Phase 1,Not Applicable 31703
17
Isophosphamide mustard Phase 3,Phase 2,Phase 1,Not Applicable 0
18 Anti-Bacterial Agents Phase 3,Phase 2,Phase 1,Not Applicable
19 Antineoplastic Agents, Alkylating Phase 3,Phase 2,Phase 1,Not Applicable
20 Topoisomerase Inhibitors Phase 3,Phase 2,Phase 1,Not Applicable
21 Alkylating Agents Phase 3,Phase 2,Phase 1,Not Applicable
22 Antibiotics, Antitubercular Phase 3,Phase 2,Phase 1,Not Applicable
23 Anti-Infective Agents Phase 3,Phase 2,Phase 1
24 Antiviral Agents Phase 3,Phase 2,Phase 1
25 Antimitotic Agents Phase 3,Phase 2,Phase 1
26 Immunologic Factors Phase 3,Phase 1,Phase 2
27 Nucleic Acid Synthesis Inhibitors Phase 3,Phase 2
28 Antineoplastic Agents, Phytogenic Phase 3,Phase 2
29 Antirheumatic Agents Phase 3,Phase 2,Phase 1
30 Etoposide phosphate Phase 3,Phase 2
31 Immunosuppressive Agents Phase 3,Phase 2,Phase 1
32 Antimetabolites Phase 3,Phase 1,Phase 2
33 Antimetabolites, Antineoplastic Phase 3,Phase 1,Phase 2
34
Indinavir Approved Phase 2 150378-17-9 5362440
35
Parathyroid hormone Approved, Investigational Phase 2 9002-64-6
36
Ritonavir Approved, Investigational Phase 2 155213-67-5 392622
37
Temozolomide Approved, Investigational Phase 2 85622-93-1 5394
38
Sunitinib Approved, Investigational Phase 2 557795-19-4, 341031-54-7 5329102
39
Sorafenib Approved, Investigational Phase 2 284461-73-0 216239 406563
40
Romidepsin Approved, Investigational Phase 2 128517-07-7 5352062
41
Sirolimus Approved, Investigational Phase 1, Phase 2,Phase 2 53123-88-9 46835353 6436030 5284616
42
Miconazole Approved, Investigational, Vet_approved Phase 1, Phase 2,Phase 2 22916-47-8 4189
43
Everolimus Approved Phase 1, Phase 2,Phase 2 159351-69-6 6442177 70789204
44
Dasatinib Approved, Investigational Phase 2 302962-49-8 3062316
45
Pembrolizumab Approved Phase 1, Phase 2,Phase 2 1374853-91-4
46
nivolumab Approved Phase 2,Phase 1 946414-94-4
47
Ipilimumab Approved Phase 2 477202-00-9
48
Atezolizumab Approved, Investigational Phase 2 1380723-44-3
49
Mesna Approved, Investigational Phase 2,Not Applicable 3375-50-6 598
50
Cisplatin Approved Phase 2 15663-27-1 441203 84093 2767

Interventional clinical trials:

(show top 50) (show all 67)
# 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 With or Without Radiation Therapy in Untreated Nonmetastatic Retroperitoneal Sarcoma Unknown status NCT01344018 Phase 3
3 Surgery Followed by Chemotherapy in Treating Young Patients With Soft Tissue Sarcoma Completed NCT00002898 Phase 3 carboplatin;cyclophosphamide;epirubicin hydrochloride;etoposide;ifosfamide;vincristine sulfate
4 Observation, Radiation Therapy, Combination Chemotherapy, and/or Surgery in Treating Young Patients With Soft Tissue Sarcoma Completed NCT00346164 Phase 3 doxorubicin hydrochloride;ifosfamide
5 Randomised Trial of Volume of Post-operative Radiotherapy Given to Adult Patients With eXtremity Soft Tissue Sarcoma Completed NCT00423618 Phase 3
6 Surgery With or Without Radiation Therapy in Treating Patients With Primary Soft Tissue Sarcoma of the Retroperitoneum or Pelvis Completed NCT00091351 Phase 3
7 Surgery With or Without Combination Chemotherapy in Treating Patients With Lung Metastases From Soft Tissue Sarcoma Completed NCT00002764 Phase 3 doxorubicin hydrochloride;ifosfamide
8 Ifosfamide or Doxorubicin in Treating Patients With Advanced or Metastatic Soft Tissue Sarcoma Completed NCT00003212 Phase 3 doxorubicin hydrochloride;ifosfamide
9 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
10 Combination Chemotherapy With or Without Hyperthermia Therapy in Treating Patients With Soft Tissue Sarcoma Completed NCT00003052 Phase 3 doxorubicin hydrochloride;etoposide;ifosfamide
11 Surgery With or Without Chemotherapy in Treating Patients With Soft Tissue Sarcoma Completed NCT00002641 Phase 3 doxorubicin hydrochloride;ifosfamide;isolated perfusion
12 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)
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 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 Active, not recruiting NCT02180867 Phase 2, Phase 3 Doxorubicin Hydrochloride;Ifosfamide;Pazopanib Hydrochloride
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 plus liposomal doxorubicin
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 Trial of Dasatinib in Advanced Sarcomas Completed NCT00464620 Phase 2 Dasatinib
38 Pembrolizumab and Gemcitabine Chemotherapy in Leiomyosarcoma and Undifferentiated Pleomorphic Sarcoma Recruiting NCT03123276 Phase 1, Phase 2 Pembrolizumab;Gemcitabine
39 Study of Neoadjuvant Checkpoint Blockade in Patients With Surgically Resectable Undifferentiated Pleomorphic Sarcoma and Dedifferentiated Liposarcoma Recruiting NCT03307616 Phase 2 Nivolumab;Ipilimumab
40 Nivolumab (Opdivo®) Plus ABI-009 (Nab-rapamycin) for Advanced Sarcoma Recruiting NCT03190174 Phase 1, Phase 2 Nab-Rapamycin
41 Clinical and Biological Activity of an Anti-PD-L1 (Atezolizumab) in Operable Localised Soft Tissue Sarcomas Patients to be Treated With Radiotherapy Recruiting NCT03474094 Phase 2
42 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
43 Doxorubicin With Upfront Dexrazoxane Plus Olaratumab for the Treatment of Advanced or Metastatic Soft Tissue Sarcoma Recruiting NCT02584309 Phase 2 Dexrazoxane;Doxorubicin;Olaratumab
44 Hypofractionated Radiotherapy With Sequential Chemotherapy in Primary Unresectable or Marginally Resectable Soft Tissue Sarcomas of Extremities or Trunk Wall Recruiting NCT03651375 Phase 2 Sequential chemotherapy - 3 courses of AI
45 Gemcitabine Hydrochloride With or Without Pazopanib Hydrochloride in Treating Patients With Refractory Soft Tissue Sarcoma Recruiting NCT01532687 Phase 2 Gemcitabine Hydrochloride;Pazopanib Hydrochloride
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 Gemcitabine, Cisplatin, and Abraxane in Advanced Biliary Cancers Active, not recruiting NCT02392637 Phase 2 Abraxane (Nab-Paclitaxel);Cisplatin;Gemcitabine
48 Multi-Arm Study to Test the Efficacy of Immunotherapeutic Agents in Multiple Sarcoma Subtypes Active, not recruiting NCT02815995 Phase 2 Durvalumab;Tremelimumab
49 Sapanisertib or Pazopanib Hydrochloride in Treating Patients With Locally Advanced or Metastatic Sarcoma Active, not recruiting NCT02601209 Phase 1, Phase 2 Pazopanib Hydrochloride;Sapanisertib
50 A Phase II Study of Eribulin and Pembrolizumab in Soft Tissue Sarcomas Not yet recruiting NCT03899805 Phase 2 Eribulin;Pembrolizumab

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:

42
Bone, Skin, Brain, Kidney, Colon, Testes, Spleen

Publications for Undifferentiated Pleomorphic Sarcoma

Articles related to Undifferentiated Pleomorphic Sarcoma:

(show top 50) (show all 1144)
# Title Authors Year
1
Long-Term Survival after Wide Resection of Malignant Fibrous Histiocytoma of the Chest Wall. ( 30834216 )
2019
2
Large Undifferentiated Pleomorphic Sarcoma of the Posterior Thigh. ( 30853708 )
2019
3
The Outcome of Patients With Localized Undifferentiated Pleomorphic Sarcoma of the Lower Extremity Treated at Stanford University. ( 30557163 )
2019
4
Detection of Metastasis in a Patient-derived Orthotopic Xenograft (PDOX) Model of Undifferentiated Pleomorphic Sarcoma with Red Fluorescent Protein. ( 30591443 )
2019
5
Genomic and transcriptomic characterisation of undifferentiated pleomorphic sarcoma of bone. ( 30281149 )
2019
6
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
7
Establishment and proteomic characterization of a novel cell line, NCC-UPS2-C1, derived from a patient with undifferentiated pleomorphic sarcoma. ( 29359268 )
2018
8
Esophageal carcinosarcoma comprised of minimally invasive squamous cell carcinoma and undifferentiated pleomorphic sarcoma: A collision cancer? ( 29934989 )
2018
9
Undifferentiated pleomorphic sarcoma. ( 29641724 )
2018
10
Undifferentiated Pleomorphic Sarcoma of Pancreas: A Case Report and Review of the Literature for the Last Updates. ( 29955231 )
2018
11
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
12
Undifferentiated Pleomorphic Sarcoma: Factors Predictive of Adverse Outcomes. ( 29787841 )
2018
13
Biology and Management of Undifferentiated Pleomorphic Sarcoma, Myxofibrosarcoma, and Malignant Peripheral Nerve Sheath Tumors: State of the Art and Perspectives. ( 29220302 )
2018
14
Cardiac Undifferentiated Pleomorphic Sarcoma Mimicking Left Atrial Myxoma. ( 29551281 )
2018
15
Recurrent Acute Myocardial Infarction Caused by Intra-cardiac Metastatic Undifferentiated Pleomorphic Sarcoma during Cancer Treatment. ( 29629023 )
2018
16
Undifferentiated pleomorphic sarcoma of the orbital region: a case report. ( 29846420 )
2018
17
Undifferentiated Pleomorphic Sarcoma after Pirfenidone Use: A Case Report. ( 29702057 )
2018
18
Mouse genetic background influences whether <i>Hras<sup>G12V</sup></i> expression plus <i>Cdkn2a</i> knockdown causes angiosarcoma or undifferentiated pleomorphic sarcoma. ( 29731980 )
2018
19
Primary undifferentiated pleomorphic sarcoma of the thyroid: A case report and review of the literature. ( 29443775 )
2018
20
Undifferentiated pleomorphic sarcoma in a patient with Cowden syndrome after radiotherapy for breast cancer. ( 30047560 )
2018
21
Splenic malignant fibrous histiocytoma with concurrent hypertension and epistaxis in an Alaskan malamute dog. ( 30103739 )
2018
22
The first ever reported case of malignant fibrous histiocytoma of the scalp. ( 30168269 )
2018
23
Treatment of spinal malignant fibrous histiocytoma, a case report and literature review. ( 30527900 )
2018
24
Phase II study of neoadjuvant checkpoint blockade in patients with surgically resectable undifferentiated pleomorphic sarcoma and dedifferentiated liposarcoma. ( 30249211 )
2018
25
Prognostic significance of microscopic tumor extension in local recurrence of myxofibrosarcoma and undifferentiated pleomorphic sarcoma. ( 30094896 )
2018
26
Aggressive undifferentiated pleomorphic sarcoma of the stomach involving long-term survival: A case report and literature review. ( 30546898 )
2018
27
Undifferentiated Pleomorphic Sarcoma Metastatic to the Orbit. ( 30289824 )
2018
28
The histone deacetylase inhibitor LBH589 inhibits undifferentiated pleomorphic sarcoma growth via downregulation of FOS-like antigen 1. ( 30303565 )
2018
29
Radiation Therapy Combined With Checkpoint Blockade Immunotherapy for Metastatic Undifferentiated Pleomorphic Sarcoma of the Maxillary Sinus With a Complete Response. ( 30386736 )
2018
30
A primary undifferentiated pleomorphic sarcoma of the lumbosacral region harboring a LMNA-NTRK1 gene fusion with durable clinical response to crizotinib: a case report. ( 30134855 )
2018
31
A case of undifferentiated pleomorphic sarcoma of the breast with lung and bone metastases. ( 30165321 )
2018
32
Functional genomics identifies AMPD2 as a new prognostic marker for undifferentiated pleomorphic sarcoma. ( 30267407 )
2018
33
Recurrent Undifferentiated Pleomorphic Sarcoma in the Left Atrium. ( 30062306 )
2018
34
Undifferentiated Pleomorphic Sarcoma of Liver: Case Report and Review of the Literature. ( 30105111 )
2018
35
Aggressively progressing primary undifferentiated pleomorphic sarcoma in the eyelid: A case report and review of the literature. ( 28471957 )
2017
36
Cardiac undifferentiated pleomorphic sarcoma incidentally diagnosed during mitral valve replacement. ( 28127801 )
2017
37
Radiation therapy for recurrent cardiac undifferentiated pleomorphic sarcoma after three operations. ( 28434140 )
2017
38
Undifferentiated pleomorphic sarcoma of the neck - A case report. ( 29031178 )
2017
39
PDGFRa amplification in multiple skin lesions of undifferentiated pleomorphic sarcoma: A clue for intimal sarcoma metastases. ( 28105789 )
2017
40
Co-targeting PI3K, mTOR, and IGF1R with small molecule inhibitors for treating undifferentiated pleomorphic sarcoma. ( 29099264 )
2017
41
Conception and Management of a Poorly Understood Spectrum of Dermatologic Neoplasms: Atypical Fibroxanthoma, Pleomorphic Dermal Sarcoma, and Undifferentiated Pleomorphic Sarcoma. ( 28762020 )
2017
42
Radiation-induced undifferentiated pleomorphic sarcoma of the heart: A case report. ( 29305110 )
2017
43
Undifferentiated pleomorphic sarcoma: indolent, tail-like recurrence of a high-grade tumor. ( 28932893 )
2017
44
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
45
miR-152 down-regulation is associated with MET up-regulation in leiomyosarcoma and undifferentiated pleomorphic sarcoma. ( 27900663 )
2017
46
Identifying actionable variants using next generation sequencing in patients with a historical diagnosis of undifferentiated pleomorphic sarcoma. ( 28891048 )
2017
47
Primary Culture of Undifferentiated Pleomorphic Sarcoma: Molecular Characterization and Response to Anticancer Agents. ( 29292724 )
2017
48
Diagnostic utility of IDH1/2 mutations to distinguish dedifferentiated chondrosarcoma from undifferentiated pleomorphic sarcoma of bone. ( 28552826 )
2017
49
Primary Undifferentiated Pleomorphic Sarcoma of the Penis. ( 29230132 )
2017
50
LN2, CD10, and Ezrin Do Not Distinguish Between Atypical Fibroxanthoma and Undifferentiated Pleomorphic Sarcoma or Predict Clinical Outcome. ( 28079637 )
2017

Variations for Undifferentiated Pleomorphic Sarcoma

ClinVar genetic disease variations for Undifferentiated Pleomorphic Sarcoma:

6
# Gene Variation Type Significance SNP ID Assembly Location
1 RECQL4 NM_004260.3(RECQL4): c.1717C> T (p.Gln573Ter) single nucleotide variant Likely pathogenic GRCh38 Chromosome 8, 144514350: 144514350
2 RECQL4 NM_004260.3(RECQL4): c.1717C> T (p.Gln573Ter) single nucleotide variant Likely pathogenic GRCh37 Chromosome 8, 145739734: 145739734

Cosmic variations for Undifferentiated Pleomorphic Sarcoma:

9 (show top 50) (show all 232)
# Cosmic Mut ID Gene Symbol COSMIC Disease Classification
(Primary site, Site subtype, Primary histology, Histology subtype)
Mutation CDS Mutation AA GRCh38 Location Conf
1 COSM212945 XPO1 soft tissue,lower extremity,leiomyosarcoma,NS c.782G>A p.R261Q 2:61496985-61496985 0
2 COSM10648 TP53 soft tissue,retroperitoneum,leiomyosarcoma,NS c.524G>A p.R175H 17:7675088-7675088 0
3 COSM43632 TP53 soft tissue,bone,leiomyosarcoma,NS c.493C>T p.Q165* 17:7675119-7675119 0
4 COSM44151 TP53 soft tissue,lung,malignant fibrous histiocytoma-pleomorphic sarcoma,NS c.535C>A p.H179N 17:7675077-7675077 0
5 COSM11476 TP53 soft tissue,retroperitoneum,leiomyosarcoma,NS c.452C>A p.P151H 17:7675160-7675160 0
6 COSM45256 TP53 soft tissue,arm,rhabdomyosarcoma,NS c.871A>C p.K291Q 17:7673749-7673749 0
7 COSM10660 TP53 soft tissue,lower extremity,malignant fibrous histiocytoma-pleomorphic sarcoma,NS c.818G>A p.R273H 17:7673802-7673802 0
8 COSM11073 TP53 soft tissue,upper extremity,malignant fibrous histiocytoma-pleomorphic sarcoma,NS c.1024C>T p.R342* 17:7670685-7670685 0
9 COSM10654 TP53 soft tissue,NS,rhabdomyosarcoma,spindle cell c.637C>T p.R213* 17:7674894-7674894 0
10 COSM10672 TP53 soft tissue,retroperitoneum,leiomyosarcoma,NS c.577C>T p.H193Y 17:7674954-7674954 0
11 COSM45253 TP53 soft tissue,arm,rhabdomyosarcoma,NS c.611A>G p.E204G 17:7674920-7674920 0
12 COSM44683 TP53 soft tissue,arm,leiomyosarcoma,NS c.652G>A p.V218M 17:7674879-7674879 0
13 COSM10725 TP53 soft tissue,NS,rhabdomyosarcoma,pleomorphic c.701A>G p.Y234C 17:7674262-7674262 0
14 COSM43919 TP53 soft tissue,upper extremity,malignant fibrous histiocytoma-pleomorphic sarcoma,NS c.856G>T p.E286* 17:7673764-7673764 0
15 COSM44732 TP53 soft tissue,bone,leiomyosarcoma,NS c.512A>G p.E171G 17:7675100-7675100 0
16 COSM6897049 TOP1 soft tissue,lower extremity,leiomyosarcoma,NS c.1576G>C p.E526Q 20:41114093-41114093 0
17 COSM6911821 SUFU soft tissue,lower extremity,malignant fibrous histiocytoma-pleomorphic sarcoma,NS c.780G>T p.E260D 10:102597163-102597163 0
18 COSM6979022 SRC soft tissue,lung,sarcoma,NS c.205T>G p.S69A 20:37384358-37384358 0
19 COSM6945139 RICTOR soft tissue,NS,rhabdomyosarcoma,pleomorphic c.2898-1G>T p.? 5:38952426-38952426 0
20 COSM3399394 RB1 soft tissue,retroperitoneum,leiomyosarcoma,NS c.1004T>G p.L335* 13:48367558-48367558 0
21 COSM6964600 RB1 soft tissue,retroperitoneum,leiomyosarcoma,NS c.345C>G p.F115L 13:48342679-48342679 0
22 COSM389868 RAC1 soft tissue,lung,sarcoma,NS c.476C>T p.A159V 7:6402343-6402343 0
23 COSM5564171 PTPRT soft tissue,bone,malignant fibrous histiocytoma-pleomorphic sarcoma,NS c.3424C>T p.R1142W 20:42106818-42106818 0
24 COSM6927085 PTPRT soft tissue,lower extremity,leiomyosarcoma,NS c.1201C>T p.R401W 20:42472515-42472515 0
25 COSM6933928 PTPRD soft tissue,lower extremity,malignant fibrous histiocytoma-pleomorphic sarcoma,NS c.5686T>C p.S1896P 9:8317927-8317927 0
26 COSM6979014 PTPRD soft tissue,lung,sarcoma,NS c.4160C>T p.A1387V 9:8404587-8404587 0
27 COSM5154 PTEN soft tissue,retroperitoneum,malignant fibrous histiocytoma-pleomorphic sarcoma,NS c.697C>T p.R233* 10:87957915-87957915 0
28 COSM5923 PTEN soft tissue,retroperitoneum,leiomyosarcoma,NS c.1027-7T>C p.? 10:87965280-87965280 0
29 COSM5033 PTEN soft tissue,upper extremity,malignant fibrous histiocytoma-pleomorphic sarcoma,NS c.389G>A p.R130Q 10:87933148-87933148 0
30 COSM6959031 PTCH1 soft tissue,lung,sarcoma,NS c.2921T>G p.F974C 9:95458260-95458260 0
31 COSM1083818 PIK3CG soft tissue,NS,rhabdomyosarcoma,spindle cell c.2638G>A p.E880K 7:106883041-106883041 0
32 COSM1537052 PIK3CA soft tissue,retroperitoneum,leiomyosarcoma,NS c.1798G>A p.E600K 3:179219622-179219622 0
33 COSM6920563 PIK3CA soft tissue,lower extremity,malignant fibrous histiocytoma-pleomorphic sarcoma,NS c.168C>A p.Y56* 3:179198993-179198993 0
34 COSM36289 PIK3CA soft tissue,retroperitoneum,gastrointestinal stromal tumour,spindle c.3143A>G p.H1048R 3:179234300-179234300 0
35 COSM27375 PIK3CA soft tissue,retroperitoneum,gastrointestinal stromal tumour,spindle c.3103G>A p.A1035T 3:179234260-179234260 0
36 COSM775 PIK3CA soft tissue,retroperitoneum,gastrointestinal stromal tumour,spindle c.3140A>G p.H1047R 3:179234297-179234297 0
37 COSM6979006 PBRM1 soft tissue,lung,sarcoma,NS c.2834G>A p.R945H 3:52589201-52589201 0
38 COSM6979021 PAK5 soft tissue,lung,sarcoma,NS c.41C>A p.P14Q 20:9644288-9644288 0
39 COSM6955391 NTRK3 soft tissue,retroperitoneum,leiomyosarcoma,NS c.452G>A p.S151N 15:88147347-88147347 0
40 COSM6078150 NTRK3 soft tissue,retroperitoneum,leiomyosarcoma,NS c.1577C>A p.P526Q 15:88032865-88032865 0
41 COSM6964921 NTRK3 soft tissue,NS,rhabdomyosarcoma,spindle cell c.492C>A p.C164* 15:88137534-88137534 0
42 COSM575 NRAS soft tissue,skin,sarcoma,NS c.38G>C p.G13A 1:114716123-114716123 0
43 COSM580 NRAS soft tissue,retroperitoneum,rhabdomyosarcoma,NS c.181C>A p.Q61K 1:114713909-114713909 0
44 COSM584 NRAS soft tissue,retroperitoneum,rhabdomyosarcoma,NS c.182A>G p.Q61R 1:114713908-114713908 0
45 COSM6911819 NOTCH4 soft tissue,lower extremity,malignant fibrous histiocytoma-pleomorphic sarcoma,NS c.2738C>G p.S913C 6:32210879-32210879 0
46 COSM48604 NOTCH3 soft tissue,lower extremity,leiomyosarcoma,NS c.2741C>T p.P914L 19:15181627-15181627 0
47 COSM6915774 NKX2-1 soft tissue,upper extremity,malignant fibrous histiocytoma-pleomorphic sarcoma,NS c.314G>T p.R105M 14:36519044-36519044 0
48 COSM1745010 MYOD1 soft tissue,NS,rhabdomyosarcoma,spindle cell c.365T>G p.L122R 11:17720147-17720147 0
49 COSM6916495 MTOR soft tissue,retroperitoneum,malignant fibrous histiocytoma-pleomorphic sarcoma,NS c.4799A>G p.E1600G 1:11144721-11144721 0
50 COSM293555 MSH6 soft tissue,lower extremity,leiomyosarcoma,NS c.3991C>T p.R1331* 2:47806641-47806641 0

Expression for Undifferentiated Pleomorphic Sarcoma

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

Pathways for Undifferentiated Pleomorphic Sarcoma

GO Terms for Undifferentiated Pleomorphic Sarcoma

Sources for Undifferentiated Pleomorphic Sarcoma

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