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MCID: LMY004
MIFTS: 62

Leiomyosarcoma

Categories: Cancer diseases, Muscle diseases, Rare diseases, Reproductive diseases
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Aliases & Classifications for Leiomyosarcoma

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MalaCards integrated aliases for Leiomyosarcoma:

Name: Leiomyosarcoma 11 19 58 75 53 5 43 14 16 71
Leiomyosarcomas 11

Characteristics:


Age Of Onset:

Adolescent,Adult,Childhood,Elderly 58

Classifications:

MalaCards categories:
Global: Rare diseases Cancer diseases
Anatomical: Muscle diseases Reproductive diseases
See all MalaCards categories (disease lists)
ICD10: 32


External Ids:

Disease Ontology 11 DOID:1967
MeSH 43 D007890
NCIt 49 C3158
SNOMED-CT 68 443719001
MESH via Orphanet 44 D007890
ICD10 via Orphanet 32 C49.9
UMLS via Orphanet 72 C0023269
Orphanet 58 ORPHA64720
UMLS 71 C0023269
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Summaries for Leiomyosarcoma

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Orphanet: 58 A rare soft tissue sarcoma characterized by a malignant space-occupying lesion most commonly located in the retroperitoneum or the inferior vena cava, but also other soft tissues, and composed of cells showing distinct features of smooth muscle cells. The tumor presents with mass effect depending on the location. It is capable of both local recurrence and distant metastasis, while lymph node metastasis is rare. Prognosis largely depends on tumor location and size.

MalaCards based summary: Leiomyosarcoma, also known as leiomyosarcomas, is related to uterus leiomyosarcoma and colon leiomyosarcoma. An important gene associated with Leiomyosarcoma is CHEK2 (Checkpoint Kinase 2), and among its related pathways/superpathways are Signal Transduction and ERK Signaling. The drugs Dacarbazine and Doxorubicin have been mentioned in the context of this disorder. Affiliated tissues include smooth muscle, uterus and lymph node, and related phenotypes are Decreased substrate adherent cell growth and Increased cell viability after pRB stimulation

GARD: 19 Leiomyosarcoma is a rare cancerous tumor that consists of smooth (involuntary) muscle cells. Leiomyosarcoma is a type of sarcoma. It spreads through the blood stream and can affect the lungs, liver, blood vessels, or any other soft tissue in the body. The exact cause of Leiomyosarcoma is not known, although genetic and environmental factors appear to be involved. It is most often found in the uterus or abdomen.

Disease Ontology: 11 A smooth muscle cancer that can arise almost anywhere in the body, but is most common in the uterus, abdomen, or pelvis.

Wikipedia: 75 Leiomyosarcoma is a malignant (cancerous) smooth muscle tumor. A benign tumor originating from the same... more...
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Related Diseases for Leiomyosarcoma

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Diseases in the Leiomyosarcoma family:

Inflammatory Leiomyosarcoma

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

(show top 50) (show all 1012)
# Related Disease Score Top Affiliating Genes
1 uterus leiomyosarcoma 32.9 PGR KIT FH DES ACTC1
2 colon leiomyosarcoma 32.7 MDM2 KIT
3 cutaneous leiomyosarcoma 32.6 KIT FH
4 gastric leiomyosarcoma 32.5 PDGFRA KIT
5 epithelioid leiomyosarcoma 32.5 VIM SERPINA3 MYOG KIT DES ACTC1
6 gallbladder leiomyosarcoma 32.5 MB KIT
7 vagina leiomyosarcoma 32.5 PGR MYOG KIT
8 breast leiomyosarcoma 32.3 MB KIT FH ENO2
9 heart leiomyosarcoma 32.2 PDGFRA MDM2
10 vascular cancer 32.1 SERPINA3 PDGFRB MYOG KIT
11 carney triad 32.0 PDGFRA KIT
12 uterine sarcoma 31.8 PDGFRB MDM2 MB KIT DES
13 myoma 31.3 PGR KIT FH ENO2 ACTC1
14 leiomyoma 31.2 VIM S100A1 PGR KIT IGF2 FH
15 fibrous histiocytoma 31.2 VIM SERPINA3 DES ACTC1
16 bizarre leiomyoma 31.0 PGR KIT FH
17 cutaneous fibrous histiocytoma 31.0 SERPINA3 S100A1 ENO2 DES ACTC1
18 smooth muscle tumor 30.9 VIM SERPINA3 S100A1 PGR KIT IGF2
19 mesenchymal cell neoplasm 30.9 SERPINA3 PDGFRB PDGFRA MYOG MDM2 KIT
20 malignant mesenchymoma 30.9 SERPINA3 MYOG MDM2 MB
21 fibrosarcoma 30.8 VIM PDGFRA KIT DES ACTC1
22 liposarcoma 30.7 VIM PDGFRA MYOG MDM2 MB DES
23 leiomyomatosis 30.7 VIM PGR FH DES ACTC1
24 chondroma 30.7 S100A1 PDGFRA KIT ACTC1
25 sarcomatosis 30.6 WT1 MYOG KIT
26 spindle cell sarcoma 30.6 VIM SERPINA3 MYOG MUC1 MDM2 KIT
27 heart cancer 30.6 WT1 SERPINA3 MYOG MDM2 KIT
28 retroperitoneal sarcoma 30.6 MYOG MDM2 KIT CDK4
29 epithelioid sarcoma 30.6 VIM SERPINA3 MUC1
30 fibroma 30.6 VIM SERPINA3 S100A1 PGR DES ACTC1
31 uterine carcinosarcoma 30.6 VIM MB KIT
32 lipomatosis, multiple 30.6 SERPINA3 S100A1 MDM2 KIT DES CDK4
33 paraganglioma 30.5 VIM MUC1 KIT IGF2 FH ENO2
34 carcinosarcoma 30.5 WT1 VIM S100A1 PGR MUC1 MB
35 pneumothorax 30.5 VIM PGR MUC1 DES ACTC1
36 breast sarcoma 30.5 PGR MYOG KIT
37 malignant fibrous histiocytoma 30.5 VIM SERPINA3 S100A1 MYOG MUC1 MDM2
38 benign breast phyllodes tumor 30.4 VIM PGR KIT
39 liver sarcoma 30.4 SERPINA3 MYOG KIT
40 teratoma 30.4 VIM MUC1 KIT ENO2 ACTC1
41 angiomyolipoma 30.4 VIM S100A1 PGR KIT DES ACTC1
42 prostate sarcoma 30.4 PGR MYOG
43 osteogenic sarcoma 30.3 MDM2 IGF2 CHEK2 CDK4
44 endometrial cancer 30.3 WT1 PGR MUC1 MDM2 KIT IGF2
45 gallbladder sarcoma 30.3 MYOG KIT
46 angiomyoma 30.3 MB KIT ENO2
47 cystadenoma 30.3 SERPINA3 PGR MUC1 KIT
48 bladder sarcoma 30.3 SERPINA3 MYOG
49 wilms tumor 5 30.3 WT1 IGF2 CHEK2
50 islet cell tumor 30.2 SERPINA3 IGF2 ENO2

Graphical network of the top 20 diseases related to Leiomyosarcoma:



Diseases related to Leiomyosarcoma
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Symptoms & Phenotypes for Leiomyosarcoma

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GenomeRNAi Phenotypes related to Leiomyosarcoma according to GeneCards Suite gene sharing:

25
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 no effect GR00402-S-1 10.19 ACTC1 CAPN6 CDK4 CHEK2 DES ENO2
2 no effect GR00402-S-2 10.19 ACTC1 CAPN6 CDK4 CHEK2 DES FH
3 Decreased substrate adherent cell growth GR00193-A-1 9.76 KIT
4 Decreased substrate adherent cell growth GR00193-A-2 9.76 CDK4 KIT
5 Decreased substrate adherent cell growth GR00193-A-3 9.76 CDK4 CHEK2
6 Decreased substrate adherent cell growth GR00193-A-4 9.76 CDK4 CHEK2 KIT
7 Increased cell viability after pRB stimulation GR00230-A-1 9.13 CDK4 CHEK2 KIT

MGI Mouse Phenotypes related to Leiomyosarcoma:

45 (show all 18)
# Description MGI Source Accession Score Top Affiliating Genes
1 homeostasis/metabolism MP:0005376 10.48 ACTC1 CDK4 CHEK2 DES FH IGF2
2 muscle MP:0005369 10.41 ACTC1 CAPN6 CDK4 DES IGF2 KIT
3 cellular MP:0005384 10.36 ACTC1 CDK4 CHEK2 DES ENO2 FH
4 normal MP:0002873 10.35 ACTC1 CDK4 KIT MDM2 MUC1 MYOG
5 cardiovascular system MP:0005385 10.25 ACTC1 CDK4 CHEK2 DES IGF2 KIT
6 embryo MP:0005380 10.24 CDK4 IGF2 KIT MB MDM2 MYOG
7 behavior/neurological MP:0005386 10.24 ACTC1 CDK4 DES ENO2 IGF2 KIT
8 endocrine/exocrine gland MP:0005379 10.21 CDK4 CHEK2 IGF2 KIT MDM2 MUC1
9 neoplasm MP:0002006 10.18 CDK4 CHEK2 KIT MDM2 MUC1 PDGFRA
10 no phenotypic analysis MP:0003012 10.16 IGF2 KIT MDM2 MUC1 MYOG PDGFRA
11 renal/urinary system MP:0005367 10.15 CDK4 FH IGF2 KIT MDM2 PDGFRA
12 digestive/alimentary MP:0005381 10.08 CAPN6 CDK4 IGF2 KIT MDM2 MUC1
13 craniofacial MP:0005382 10.06 CAPN6 CDK4 ENO2 IGF2 KIT MDM2
14 respiratory system MP:0005388 9.97 ENO2 IGF2 KIT MB MYOG PDGFRA
15 hematopoietic system MP:0005397 9.97 ACTC1 CDK4 CHEK2 IGF2 KIT MB
16 reproductive system MP:0005389 9.96 CDK4 CHEK2 IGF2 KIT MDM2 PDGFRA
17 mortality/aging MP:0010768 9.83 ACTC1 CDK4 CHEK2 DES FH IGF2
18 integument MP:0010771 9.32 CDK4 IGF2 KIT MDM2 MUC1 MYOG
Sources

Drugs & Therapeutics for Leiomyosarcoma

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

(show top 50) (show all 114)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Dacarbazine Approved, Investigational Phase 2, Phase 3 4342-03-4 2942 5351166
2
Doxorubicin Approved, Investigational Phase 2, Phase 3 23214-92-8 31703
3
Ifosfamide Approved Phase 2, Phase 3 3778-73-2 3690
4
Cyclophosphamide Approved, Investigational Phase 2, Phase 3 50-18-0, 6055-19-2 2907
5
Daunorubicin Approved Phase 2, Phase 3 20830-81-3 30323
6
Mechlorethamine Approved, Investigational Phase 2, Phase 3 51-75-2 4033
7
Temozolomide Approved, Investigational Phase 2, Phase 3 85622-93-1 5394
8
Olaparib Approved Phase 2, Phase 3 763113-22-0 23725625
9
Lenograstim Approved, Investigational Phase 3 135968-09-1
10
Bevacizumab Approved, Investigational Phase 3 216974-75-3 135329020
11
Palifosfamide Investigational Phase 2, Phase 3 31645-39-3 100427
12 Pharmaceutical Solutions Phase 3
13 Anti-Bacterial Agents Phase 2, Phase 3
14 Antibiotics, Antitubercular Phase 2, Phase 3
15 Liposomal doxorubicin Phase 2, Phase 3
16 Poly(ADP-ribose) Polymerase Inhibitors Phase 2, Phase 3
17 Immunoglobulins Phase 3
18 Antibodies, Monoclonal Phase 3
19 Antibodies Phase 3
20 Antineoplastic Agents, Immunological Phase 3
21 Angiogenesis Inhibitors Phase 3
22 Adjuvants, Immunologic Phase 3
23 Immunoglobulins, Intravenous Phase 3
24 Immunoglobulin G Phase 3
25 Endothelial Growth Factors Phase 3
26 Mitogens Phase 3
27
Coenzyme M Approved, Investigational Phase 2 3375-50-6 598 23662354
28
Sorafenib Approved, Investigational Phase 2 284461-73-0 216239
29
Progesterone Approved, Vet_approved Phase 2 57-83-0 5994
30
Sunitinib Approved, Investigational Phase 2 557795-19-4 5329102
31
Thalidomide Approved, Investigational, Withdrawn Phase 2 50-35-1 5426
32
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 2 1177-87-3 3680
33
Dexamethasone Approved, Investigational, Vet_approved Phase 2 50-02-2 3003 5743
34
Carboplatin Approved Phase 2 41575-94-4 10339178 38904
35
Benzocaine Approved, Investigational Phase 2 1994-09-7, 94-09-7 2337
36
Tannic acid Approved Phase 2 1401-55-4 16129878 16129778
37
Aflibercept Approved Phase 2 862111-32-8 124490314
38
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
39
Clotrimazole Approved, Vet_approved Phase 2 23593-75-1 2812
40
Romidepsin Approved, Investigational Phase 2 128517-07-7 5352062
41
Zoledronic acid Approved Phase 2 118072-93-8 68740
42
Dinutuximab Approved, Investigational Phase 2 1363687-32-4
43
Fulvestrant Approved, Investigational Phase 2 129453-61-8 17756771 104741
44
Rituximab Approved Phase 2 174722-31-7
45
Valganciclovir Approved, Investigational Phase 1, Phase 2 175865-60-8 64147
46
Cemiplimab Approved, Investigational Phase 1, Phase 2 1801342-60-8
47
Talimogene laherparepvec Approved, Experimental, Investigational Phase 2 1187560-31-1
48
Nivolumab Approved Phase 2 946414-94-4
49
Ipilimumab Approved Phase 2 477202-00-9
50
Rucaparib Approved, Investigational Phase 2 283173-50-2 9931954

Interventional clinical trials:

(show top 50) (show all 130)
# Name Status NCT ID Phase Drugs
1 Risk-Based Treatment for Non-Rhabdomyosarcoma Soft Tissue Sarcomas (NRSTS) in Patients Under 30 Years of Age Completed NCT00346164 Phase 3 doxorubicin hydrochloride;ifosfamide
2 Randomised Phase III Multicentric Study Comparing Efficacy of Doxorubicin With Trabectedin Followed by Trabectedin in Non-progressive Patients Versus Doxorubicine Alone as First-line Therapy in Patients With Metastatic or Unresectable Leiomyosarcoma (Uterine or Soft Tissue) Completed NCT02997358 Phase 3 Doxorubicin;Trabectedin
3 Multicenter, Open-label Study of YONDELIS (Trabectedin) in Subjects With Locally Advanced or Metastatic Liposarcoma or Leiomyosarcoma Completed NCT01692678 Phase 3 Trabectedin;Dacarbazine
4 A Randomized Controlled Study of YONDELIS (Trabectedin) or Dacarbazine for the Treatment of Advanced Liposarcoma or Leiomyosarcoma Completed NCT01343277 Phase 3 Trabectedin;Dacarbazine
5 A Phase 2/3 Study to Evaluate the Efficacy and Safety of Unesbulin in Unresectable or Metastatic, Relapsed or Refractory Leiomyosarcoma Recruiting NCT05269355 Phase 2, Phase 3 Unesbulin;Dacarbazine
6 A Randomized Phase III Study of Neoadjuvant Chemotherapy Followed by Surgery Versus Surgery Alone for Patients With High Risk RetroPeritoneal Sarcoma (RPS) Recruiting NCT04031677 Phase 3 Preoperative chemotherapy
7 A Phase III Study of AL3818 (Anlotinib, Catequentinib) Hydrochloride Monotherapy in Subjects With Metastatic or Advanced Alveolar Soft Part Sarcoma, Leiomyosarcoma and Synovial Sarcoma Active, not recruiting NCT03016819 Phase 3 AL3818;Dacarbazine;AL3818 or placebo
8 Pazopanib Neoadjuvant Trial in Non-Rhabdomyosarcoma Soft Tissue Sarcomas (PAZNTIS): A Phase II/III Randomized Trial of Preoperative Chemoradiation or Preoperative Radiation Plus or Minus Pazopanib (NSC# 737754) Active, not recruiting NCT02180867 Phase 2, Phase 3 Doxorubicin;Doxorubicin Hydrochloride;Ifosfamide;Pazopanib;Pazopanib Hydrochloride
9 A Randomized Phase 2/3 Study of Olaparib Plus Temozolomide Versus Investigator's Choice for the Treatment of Patients With Advanced Uterine Leiomyosarcoma After Progression on Prior Chemotherapy Not yet recruiting NCT05432791 Phase 2, Phase 3 Olaparib;Pazopanib;Temozolomide;Trabectedin
10 A Randomized Phase 2/3 Study of Olaparib Plus Temozolomide Versus Investigator's Choice for the Treatment of Patients With Advanced Uterine Leiomyosarcoma After Progression on Prior Chemotherapy Not yet recruiting NCT05633381 Phase 2, Phase 3 Olaparib;Temozolomide;Trabectedin;Pazopanib
11 A Randomized Phase III Evaluation of Docetaxel (NSC #628503) and Gemcitabine (NSC #613327) Plus G-CSF With Bevacizumab (NSC #704865) Versus Docetaxel (NSC #628503) and Gemcitabine (NSC #613327) Plus G-CSF With Placebo in the Treatment of Recurrent or Advanced Leiomyosarcoma of the Uterus Terminated NCT01012297 Phase 3 Docetaxel;Gemcitabine Hydrochloride
12 A Phase III Randomized Trial of Gemcitabine (NSC# 613327) Plus Docetaxel (NSC# 628503) Followed by Doxorubicin (NSC# 123127) Versus Observation for Uterus-Limited, High Grade Uterine Leiomyosarcoma Terminated NCT01533207 Phase 3 Docetaxel;Doxorubicin Hydrochloride;Gemcitabine Hydrochloride
13 Time to Secondary Resistance to Trabectedin After Interruption Versus Continuation in Responding Patients With Liposarcoma, Leiomyosarcoma and Synovial Sarcoma Withdrawn NCT03773510 Phase 3 Trabectedin discontinuation;Trabectedin continuation
14 A Phase II Study of Combination Therapy of a Protracted Oral Schedule of Temozolomide and Thalidomide as First-Line or Subsequent Therapy for Patients With Metastatic, Locally Advanced or Unresectable Leiomyosarcoma Unknown status NCT00033709 Phase 2 temozolomide;thalidomide
15 GEIS-27: Phase I/II Multicenter and Prospective Trial of Nilotinib and Adriamycin as Neoadjuvant Treatment in Liposarcomas and Leiomyosarcomas of Retroperitoneum Unknown status NCT02587169 Phase 1, Phase 2 Nilotinib-adriamycin
16 Hypofractionated 5x5 Gy Radiotherapy With Sequential Doxorubicin and Ifosfamide-based Chemotherapy in Marginally Resectable Soft Tissue Sarcomas of Extremities or Trunk Wall Unknown status NCT03651375 Phase 2 Sequential chemotherapy - 3 courses of AI
17 A Phase 2 Study of AZD0530 in Recurrent or Metastatic Soft Tissue Sarcoma Completed NCT00659360 Phase 2 saracatinib
18 A Multicentric Phase II Trial Studying Efficacy of Doxorubicin Associated With Trabectedin (Yondelis) in First Line Treatment on Patients With Metastatic Leiomyosarcoma (Uterus or Soft Tissue) and/or Inoperable Relapse Completed NCT02131480 Phase 2 Doxorubicin;Trabectedin
19 A Multicenter Phase II Study of Sorafenib (BAY43-9006) in Non-GIST Sarcomas Completed NCT00245102 Phase 2 sorafenib tosylate
20 A Phase 1B/II Study of GDC-0449 (NSC 747691) in Combination With RO4929097, a Gamma-Secretase Inhibitor (GSI) in Advanced/Metastatic Sarcomas Completed NCT01154452 Phase 1, Phase 2 Gamma-Secretase Inhibitor RO4929097;Vismodegib
21 A Phase II Randomized - Non Comparative - Study on the Activity of Trabectedin or Gemcitabine + Docetaxel in Metastatic or Locally Relapsed Uterine Leiomyosarcoma Pretreated With Conventional Chemotherapy Completed NCT02249702 Phase 2 gemcitabine + docetaxel;trabectedin
22 A Phase II Study of Mocetinostat Administered With Gemcitabine for Patients With Metastatic Leiomyosarcoma With Progression or Relapse Following Prior Treatment With Gemcitabine-Containing Therapy Completed NCT02303262 Phase 2 Mocetinostat;Gemcitabine
23 Open-label, Phase II Trial of Eribulin in Combination With Gemcitabine in Previously Treated Patients With Advanced Liposarcoma or Leiomyosarcoma Completed NCT03810976 Phase 2 Eribulin, gemcitabine
24 A Phase II Study on Trabectedin in Advanced Retroperitoneal Leiomyosarcoma and Well Differentiated/Dedifferentiated Liposarcoma Completed NCT02247544 Phase 2 Trabectedin
25 Phase 2 Clinical Study of Letrozole in Women With Advanced Estrogen/Progesterone Receptor Positive Uterine Leiomyosarcoma Completed NCT00856050 Phase 2 letrozole
26 A Phase II Evaluation of Sunitinib Malate (Sutent®, SU11248, NCI-Supplied Agent , NSC # 736511) in the Treatment of Recurrent or Persistent Leiomyosarcoma of the Uterus Completed NCT00378911 Phase 2 sunitinib malate
27 A Phase II Evaluation of Thalidomide (NSC #66847) in the Treatment of Recurrent or Persistent Leiomyosarcoma of the Uterus Completed NCT00025220 Phase 2 Thalidomide
28 Evaluation of Doxil in the Treatment of Recurrent or Advanced Leiomyosarcoma of the Uterus Completed NCT00005643 Phase 2 pegylated liposomal doxorubicin hydrochloride
29 A Phase II Evaluation of MLN8237 (NSC# 747888) in the Treatment of Recurrent or Persistent Leiomyosarcoma of the Uterus Completed NCT01637961 Phase 2 Alisertib
30 A Phase II Evaluation of Docetaxel and Gemcitabine Plus G-CSF in the Treatment of Recurrent or Persistent Leiomyosarcoma of the Uterus Completed NCT00031629 Phase 2 Docetaxel;Gemcitabine Hydrochloride
31 A Phase II Evaluation of Docetaxel (NSC #628503) and Gemcitabine (NSC #613327) Plus G-CSF in the Treatment of Recurrent or Advanced Leiomyosarcoma of the Uterus Completed NCT00101127 Phase 2 docetaxel;gemcitabine hydrochloride
32 A Phase II Trial of Gemcitabine and Docetaxel in Patients With Unresectable Leiomyosarcoma Completed NCT00004066 Phase 2 docetaxel;gemcitabine hydrochloride
33 A Phase 2 Multi Center Open Label Study of ARQ 501 in Adult Patients With Recurrent, Persistent or Metastatic Leiomyosarcoma Completed NCT00310518 Phase 2 ARQ 501
34 Adjuvant Treatment of High Risk Uterine Leiomyosarcoma With Gemcitabine/Docetaxel Followed by Doxorubicin: A Phase II Trial Completed NCT00282087 Phase 2 gemcitabine, docetaxel, doxorubicin
35 A Randomized, Multicenter, Open-label Study of Yondelis (ET-743 Ecteinascidin) Administered by 2 Different Schedules (Weekly for 3 of 4 Weeks vs. q3 Weeks) in Subjects With Locally Advanced or Metastatic Liposarcoma or Leiomyosarcoma Following Treatment With an Anthracycline and Ifosfamide Completed NCT00060944 Phase 2 Yondelis;Dexamethasone
36 A Phase II Evaluation of Ixabepilone (NSC #710428) in the Treatment of Recurrent or Persistent Leiomyosarcoma of the Uterus Completed NCT01220609 Phase 2 Ixabepilone
37 Phase II Open-Label Study of Sunitinib Malate (SU011248) in Adult Patients With Metastatic and/or Surgically Unresectable Soft Tissue Sarcoma Completed NCT00400569 Phase 2 Sunitinib Malate (SU011248)
38 A Five-Tier, Phase 2 Open-Label Study of IMC-A12 Administered as a Single Agent Every 2 Weeks in Patients With Previously-Treated, Advanced or Metastatic Soft Tissue and Ewing's Sarcoma/PNET Completed NCT00668148 Phase 2
39 Phase II Study Evaluating PegLiposomal Doxorubicin (PLD) and Carboplatin Combination Chemotherapy in Gynecological Sarcomas and Mixed Epithelial-Mesenchymal Tumors Completed NCT00815945 Phase 2 PegLiposomal Doxorubicin;Carboplatin
40 A Phase II Study of M6620 (VX-970) in Selected Solid Tumors Completed NCT03718091 Phase 2 M6620
41 Phase II Trial of Sorafenib and Dacarbazine in Soft Tissue Sarcoma Completed NCT00837148 Phase 2 Sorafenib and Dacarbazine
42 A Phase 2 Study of Intravenous REOLYSIN® (Wild-Type Reovirus) in the Treatment of Patients With Bone and Soft Tissue Sarcomas Metastatic to the Lung Completed NCT00503295 Phase 2
43 A Phase II Study of VEGF-Trap in Recurrent or Metastatic Gynecologic Soft-Tissue Sarcomas Completed NCT00390234 Phase 2 ziv-aflibercept
44 A Phase II Study of AP23573, An mTOR Inhibitor, in Patients With Advanced Sarcoma Completed NCT00093080 Phase 2 ridaforolimus
45 A Phase II Study of Cixutumumab (IMC-A12) in Combination With Temsirolimus in Pediatric Patients With Recurrent or Refractory Solid Tumors Completed NCT01614795 Phase 2 Temsirolimus
46 Phase II Trial Of Gemcitabine and Docetaxel With Bevacizumab in Selected Sarcoma Subtypes Completed NCT00887809 Phase 2 gemcitabine;docetaxel;bevacizumab
47 A Phase II Study of Neoadjuvant Bevacizumab and Radiation Therapy for Resectable Soft Tissue Sarcomas Completed NCT00356031 Phase 2 Bevacizumab
48 Phase II Study of MLN8237 in Advanced/Metastatic Sarcoma Completed NCT01653028 Phase 2 Alisertib
49 A Phase II Study of Single Agent Depsipeptide (FK228) in Metastatic or Unresectable Soft Tissue Sarcomas Completed NCT00112463 Phase 2 romidepsin
50 Phase II Multicenter Study to Determine the Efficacy of Gemcitabine With Pazopanib as Second Line Treatment in Patients With Metastatic or Relapsed Uterine or Soft Tissue Leiomyosarcomas Completed NCT01442662 Phase 2 pazopanib + gemcitabine

Search NIH Clinical Center for Leiomyosarcoma

Cochrane evidence based reviews: leiomyosarcoma

Sources

Genetic Tests for Leiomyosarcoma

About this section
Sources

Anatomical Context for Leiomyosarcoma

About this section

Organs/tissues related to Leiomyosarcoma:

MalaCards : Smooth Muscle, Uterus, Lymph Node, Liver, Small Intestine, Bone, Breast
Sources

Publications for Leiomyosarcoma

About this section

Articles related to Leiomyosarcoma:

(show top 50) (show all 11057)
# Title Authors PMID Year
1
Genomic profiling of pelvic genital type leiomyosarcoma in a woman with a germline CHEK2:c.1100delC mutation and a concomitant diagnosis of metastatic invasive ductal breast carcinoma. 62 5
28514723 2017
2
Carbonic anhydrase II. A novel biomarker for gastrointestinal stromal tumors. 53 62
20081808 2010
3
Expression of Ki-67, P53 and progesterone receptors in uterine smooth muscle tumors. Diagnostic value. 53 62
20437637 2010
4
WT-1 expression in gastrointestinal stromal tumours. 53 62
20025481 2010
5
Axillary and perianal leiomyosarcoma: report of two cases. 53 62
20495760 2010
6
A panel of antibodies to determine site of origin and malignancy in smooth muscle tumors. 53 62
19734847 2009
7
Gastric leiomyosarcoma manifesting peculiar findings: radiological-pathological correlation. 53 62
19432672 2009
8
[Acute abdomen for perforated gastrointestinal stromal tumor (GIST). A case report]. 53 62
19537128 2009
9
Utility of a comprehensive immunohistochemical panel in the differential diagnosis of spindle cell lesions of the urinary bladder. 53 62
18941404 2009
10
Absence of fumarate hydratase mutation in a family with cutaneous leiomyosarcoma and renal cancer. 53 62
18986479 2008
11
Selective progesterone receptor modulators 3: use in oncology, endocrinology and psychiatry. 53 62
18778186 2008
12
[Immunohistochemical study of endometrial stromal sarcoma and smooth-muscle tumors of the uterus]. 53 62
18565690 2008
13
Clinicopathological characteristics of gastrointestinal mesenchymal tumors and diagnostic value of endoscopic ultrasonography. 53 62
18505417 2008
14
Cytoplasmic overexpression of WT-1 in gastrointestinal stromal tumor and other soft tissue tumors. 53 62
18528287 2008
15
Breast metastasis from uterine leiomyosarcoma diagnosed by fine needle aspiration: a case report. 53 62
18702371 2008
16
Recurrent nodule on the nasal columella: a good reason to re-biopsy. 53 62
18613884 2008
17
Immunohistochemical distinction of cutaneous spindle cell carcinoma. 53 62
18496422 2008
18
A rare tumor in the adrenal region: neuron-specific enolase (NSE)-producing leiomyosarcoma in an elderly hypertensive patient. 53 62
18250540 2008
19
[Adult prostate sarcoma: a report of 6 cases with clinical analysis]. 53 62
17725305 2007
20
Expression of chemokines CCL5 and CCL11 by smooth muscle tumor cells of the uterus and its possible role in the recruitment of mast cells. 53 62
17368523 2007
21
Expression of cyclooxygenase-2, c-kit, progesterone and estrogen receptors in uterine smooth muscle tumors: differential diagnosis. 53 62
17550381 2007
22
Utility of ALK-1 protein expression and ALK rearrangements in distinguishing inflammatory myofibroblastic tumor from malignant spindle cell lesions of the urinary bladder. 53 62
17396140 2007
23
Comparative study using rabbit-derived polyclonal, mouse-derived monoclonal, and rabbit-derived monoclonal antibodies for KIT immunostaining in GIST and other tumors. 53 62
17316415 2007
24
C-kit protein expression in uterine and ovarian mesenchymal tumours. 53 62
17367465 2007
25
PDGFR-alpha as a potential therapeutic target in uterine sarcomas. 53 62
17049587 2007
26
Esophageal mesenchymal tumors: endoscopy, pathology and immunohistochemistry. 53 62
17278201 2007
27
Nestin expression as a new marker in malignant peripheral nerve sheath tumors. 53 62
17300669 2007
28
Expression of the O-linked N-acetylglucosamine containing epitope H in normal myometrium and uterine smooth muscle cell tumors. 53 62
17129677 2007
29
[Gastrointestinal mesenchymal tumors: a clinical pathologic and immunohistochemical study of 210 cases]. 53 62
17253165 2007
30
Increased expression of calpain 6 in uterine sarcomas and carcinosarcomas: an immunohistochemical analysis. 53 62
17291261 2007
31
Gastrointestinal stromal tumors: differential diagnosis. 53 62
17193824 2006
32
A case of cutaneous leiomyosarcoma with overexpression of KIT: do CD117 (KIT)-positive primary gastrointestinal stromal tumours of the skin exist? 53 62
16634920 2006
33
No evidence for epigenetic inactivation of fumarate hydratase in leiomyomas and leiomyosarcomas. 53 62
15949892 2006
34
Mammary NOS-type sarcoma with CD10 expression: a rare entity with features of myoepithelial differentiation. 53 62
16625090 2006
35
[Extragastrointestinal stromal tumors: a report of 4 cases]. 53 62
16472759 2006
36
Calretinin expression in tumors of adipose tissue. 53 62
16613326 2006
37
Analysis of allelic loss as an adjuvant tool in evaluation of malignancy in uterine smooth muscle tumors. 53 62
16330948 2006
38
Inflammatory myofibroblastic tumor of the uterus: a clinicopathologic study of 6 cases emphasizing distinction from aggressive mesenchymal tumors. 53 62
16160478 2005
39
The expression of Ki-67, p53, estrogen and progesterone receptors affecting survival in uterine leiomyosarcomas. A clinicopathologic study. 53 62
15992918 2005
40
KIT (CD117): a review on expression in normal and neoplastic tissues, and mutations and their clinicopathologic correlation. 53 62
16082245 2005
41
Primary osteosarcoma of the thoracic spine: report of an unusual elderly patient with autopsy findings. 53 62
15753962 2005
42
INI1 expression is retained in composite rhabdoid tumors, including rhabdoid meningiomas. 53 62
15761491 2005
43
Imatinib mesylate (gleevec)--targeted kinases are expressed in uterine sarcomas. 53 62
15894930 2005
44
Carcinosarcoma of the liver: a case report and review of the literature. 53 62
15913431 2005
45
Smooth muscle tumours of the uterine corpus: a clinicopathologic study with immunohistochemical aspects. 53 62
16033061 2005
46
So-called "inflammatory leiomyosarcoma'': a series of 3 cases providing additional insights into a rare entity. 53 62
15864383 2005
47
[Malignant fibrous histiocytoma: pleomorphic sarcoma NOS or pleomorphic fibrosarcoma]. 53 62
15702318 2005
48
[Morphology and immunohistochemical characteristics of hepatic primary and metastatic malignant spindle cell tumors]. 53 62
15774214 2005
49
Intimal sarcoma of the pulmonary artery: report of an autopsy case. 53 62
16136754 2005
50
[Gastrointestinal stromal tumor: a clinicopathological study of 74 cases]. 53 62
15796874 2005
Sources

Variations for Leiomyosarcoma

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ClinVar genetic disease variations for Leiomyosarcoma:

5
# Gene Name Type Significance ClinVarId dbSNP ID Position
1 CHEK2 NM_007194.4(CHEK2):c.1100del (p.Thr367fs) DEL Pathogenic
 128042 rs555607708 GRCh37: 22:29091857-29091857
GRCh38: 22:28695869-28695869

Cosmic variations for Leiomyosarcoma:

8 (show top 50) (show all 2128)
# Cosmic Mut ID Gene Symbol COSMIC Disease Classification
(Primary site, Site subtype, Primary histology, Histology subtype)		 Mutation CDS Mutation AA GRCh38 Location Conf
1 COSM149325379 ZFHX3 soft tissue,smooth muscle,leiomyosarcoma,NS c.2513A>G p.N838S 16:72957633-72957633 8
2 COSM102044775 ZFHX3 soft tissue,smooth muscle,leiomyosarcoma,NS c.-23-6668A>G p.? 16:72957633-72957633 8
3 COSM87293976 ZFHX3 soft tissue,smooth muscle,leiomyosarcoma,NS c.2513A>G p.N838S 16:72957633-72957633 8
4 COSM104236272 XPO1 soft tissue,smooth muscle,leiomyosarcoma,NS c.782G>A p.R261Q 2:61496985-61496985 8
5 COSM101976974 XPO1 soft tissue,smooth muscle,leiomyosarcoma,NS c.782G>A p.R261Q 2:61496985-61496985 8
6 COSM104882801 XPO1 soft tissue,smooth muscle,leiomyosarcoma,NS c.782G>A p.R261Q 2:61496985-61496985 8
7 COSM148791748 TSC1 soft tissue,smooth muscle,leiomyosarcoma,NS c.1079C>A p.T360N 9:132911064-132911064 8
8 COSM147982566 TSC1 soft tissue,smooth muscle,leiomyosarcoma,NS c.1079C>A p.T360N 9:132911064-132911064 8
9 COSM151103730 TSC1 soft tissue,smooth muscle,leiomyosarcoma,NS c.1122C>A p.N374K 9:132911064-132911064 8
10 COSM151874979 TSC1 soft tissue,smooth muscle,leiomyosarcoma,NS c.1079C>A p.T360N 9:132911064-132911064 8
11 COSM102541613 TSC1 soft tissue,smooth muscle,leiomyosarcoma,NS c.*317C>A p.? 9:132911064-132911064 8
12 COSM150523552 TSC1 soft tissue,smooth muscle,leiomyosarcoma,NS c.1079C>A p.T360N 9:132911064-132911064 8
13 COSM148126195 TSC1 soft tissue,smooth muscle,leiomyosarcoma,NS c.1079C>A p.T360N 9:132911064-132911064 8
14 COSM149038920 TSC1 soft tissue,smooth muscle,leiomyosarcoma,NS c.1079C>A p.T360N 9:132911064-132911064 8
15 COSM150677027 TSC1 soft tissue,smooth muscle,leiomyosarcoma,NS c.1079C>A p.T360N 9:132911064-132911064 8
16 COSM150536467 TSC1 soft tissue,smooth muscle,leiomyosarcoma,NS c.926C>A p.T309N 9:132911064-132911064 8
17 COSM111040244 TSC1 soft tissue,smooth muscle,leiomyosarcoma,NS c.1079C>A p.T360N 9:132911064-132911064 8
18 COSM148033864 TSC1 soft tissue,smooth muscle,leiomyosarcoma,NS c.1079C>A p.T360N 9:132911064-132911064 8
19 COSM133086425 TSC1 soft tissue,smooth muscle,leiomyosarcoma,NS c.926C>A p.T309N 9:132911064-132911064 8
20 COSM151895912 TSC1 soft tissue,smooth muscle,leiomyosarcoma,NS c.1079C>A p.T360N 9:132911064-132911064 8
21 COSM85719005 TSC1 soft tissue,smooth muscle,leiomyosarcoma,NS c.1079C>A p.T360N 9:132911064-132911064 8
22 COSM151333783 TSC1 soft tissue,smooth muscle,leiomyosarcoma,NS c.1079C>A p.T360N 9:132911064-132911064 8
23 COSM144100083 TP53 soft tissue,smooth muscle,leiomyosarcoma,NS c.331T>G p.F111V 17:7673812-7673812 8
24 COSM106055950 TP53 soft tissue,smooth muscle,leiomyosarcoma,NS c.374C>A p.T125K 17:7675995-7675995 8
25 COSM87912584 TP53 soft tissue,smooth muscle,leiomyosarcoma,NS c.376-1G>A p.? 17:7675237-7675237 8
26 COSM105657416 TP53 soft tissue,smooth muscle,leiomyosarcoma,NS c.641A>C p.H214P 17:7674890-7674890 8
27 COSM105620642 TP53 soft tissue,smooth muscle,leiomyosarcoma,NS c.742C>T p.R248W 17:7674221-7674221 8
28 COSM105625281 TP53 soft tissue,smooth muscle,leiomyosarcoma,NS c.782+402G>A p.? 17:7673779-7673779 8
29 COSM143945794 TP53 soft tissue,smooth muscle,leiomyosarcoma,NS c.-51G>A p.? 17:7675185-7675185 8
30 COSM143971570 TP53 soft tissue,smooth muscle,leiomyosarcoma,NS c.164A>C p.H55P 17:7674890-7674890 8
31 COSM122279858 TP53 soft tissue,smooth muscle,leiomyosarcoma,NS c.77G>A p.R26H 17:7675139-7675139 8
32 COSM143370705 TP53 soft tissue,smooth muscle,leiomyosarcoma,NS c.542A>G p.Y181C 17:7674872-7674872 8
33 COSM142837551 TP53 soft tissue,smooth muscle,leiomyosarcoma,NS c.742C>T p.R248W 17:7674221-7674221 8
34 COSM144659750 TP53 soft tissue,smooth muscle,leiomyosarcoma,NS c.356G>C p.R119P 17:7675139-7675139 8
35 COSM142568491 TP53 soft tissue,smooth muscle,leiomyosarcoma,NS c.356G>A p.R119H 17:7675139-7675139 8
36 COSM143944281 TP53 soft tissue,smooth muscle,leiomyosarcoma,NS c.83-1G>A p.? 17:7674972-7674972 8
37 COSM143167912 TP53 soft tissue,smooth muscle,leiomyosarcoma,NS c.-104A>T p.? 17:7675238-7675238 8
38 COSM121897862 TP53 soft tissue,smooth muscle,leiomyosarcoma,NS c.317G>T p.C106F 17:7674250-7674250 8
39 COSM142561826 TP53 soft tissue,smooth muscle,leiomyosarcoma,NS c.521G>A p.R174Q 17:7674893-7674893 8
40 COSM142560355 TP53 soft tissue,smooth muscle,leiomyosarcoma,NS c.334C>T p.P112S 17:7675161-7675161 8
41 COSM145036361 TP53 soft tissue,smooth muscle,leiomyosarcoma,NS c.596G>T p.C199F 17:7674250-7674250 8
42 COSM145034453 TP53 soft tissue,smooth muscle,leiomyosarcoma,NS c.706T>C p.C236R 17:7673797-7673797 8
43 COSM143162643 TP53 soft tissue,smooth muscle,leiomyosarcoma,NS c.308G>T p.G103V 17:7673835-7673835 8
44 COSM121893200 TP53 soft tissue,smooth muscle,leiomyosarcoma,NS c.121G>C p.V41L 17:7675095-7675095 8
45 COSM123055238 TP53 soft tissue,smooth muscle,leiomyosarcoma,NS c.179A>T p.Q60L 17:7674956-7674956 8
46 COSM122734176 TP53 soft tissue,smooth muscle,leiomyosarcoma,NS c.347G>A p.R116Q 17:7674220-7674220 8
47 COSM93183630 TP53 soft tissue,smooth muscle,leiomyosarcoma,NS c.743G>A p.R248Q 17:7674220-7674220 8
48 COSM144087106 TP53 soft tissue,smooth muscle,leiomyosarcoma,NS c.266G>A p.R89Q 17:7674220-7674220 8
49 COSM144652260 TP53 soft tissue,smooth muscle,leiomyosarcoma,NS c.464T>C p.L155P 17:7674950-7674950 8
50 COSM143157212 TP53 soft tissue,smooth muscle,leiomyosarcoma,NS c.58C>T p.H20Y 17:7675077-7675077 8

Copy number variations for Leiomyosarcoma from CNVD:

6
# CNVD ID Chromosome Start End Type Gene Symbol CNVD Disease
1 102985 16 58400001 69400000 Loss Leiomyosarcoma
Sources

Expression for Leiomyosarcoma

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Search GEO for disease gene expression data for Leiomyosarcoma.
Sources

Pathways for Leiomyosarcoma

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Pathways related to Leiomyosarcoma according to GeneCards Suite gene sharing:

(show all 22)
# Super pathways Score Top Affiliating Genes
1
Signal Transduction 66
13.6 VIM PGR PDGFRB PDGFRA MDM2 KIT
2
ERK Signaling 64
Show member pathways
 13.54 VIM PDGFRB PDGFRA MUC1 MDM2 KIT
3
PI3K-Akt signaling pathway 74
Show member pathways
 12.76 CDK4 IGF2 KIT MDM2 PDGFRA PDGFRB
4
Signaling by Receptor Tyrosine Kinases 66
12.66 IGF2 KIT PDGFRA PDGFRB PGR
5
Cytoskeletal Signaling 3
12.39 VIM MUC1 DES ACTC1
6
DNA Damage 3
12.37 VIM MDM2 CHEK2 CDK4
7
NF-kappaB Pathway 70
Show member pathways
 12.13 CAPN6 CHEK2 MDM2 PDGFRA PDGFRB
8
MAPK Signaling: Mitogens 65
Show member pathways
 12.07 PDGFRB PDGFRA MDM2 CDK4
9
Breast cancer pathway 74
Show member pathways
 11.86 PGR PDGFRB PDGFRA MDM2 KIT CDK4
10
Malignant pleural mesothelioma 74
11.78 PDGFRB PDGFRA MDM2 KIT IGF2 CDK4
11
Mesenchymal Stem Cells and Lineage-specific Markers 65
11.75 MYOG KIT DES
12
Hippo-Merlin signaling dysregulation 74
Show member pathways
 11.7 PDGFRB PDGFRA KIT
13
Clear cell renal cell carcinoma pathways 74
11.66 PDGFRB PDGFRA ENO2
14
Striated muscle contraction pathway 74
Show member pathways
 11.57 VIM DES ACTC1
15
Ponatinib Pathway, Pharmacokinetics/Pharmacodynamics 60
Show member pathways
 11.49 PDGFRB PDGFRA KIT
16
DNA damage_ATM/ATR regulation of G1/S checkpoint 22
11.31 MDM2 CHEK2 CDK4
17
Integrated cancer pathway 74
11.21 MDM2 CHEK2 CDK4
18
Cardiac progenitor differentiation 74
11.18 PDGFRA KIT IGF2 ACTC1
19
Pazopanib Pathway, Pharmacokinetics 60
Show member pathways
 11.14 PDGFRB PDGFRA KIT
20
G-protein signaling_Rap2B regulation pathway 22
10.78 VIM PDGFRB PDGFRA KIT IGF2 DES
21
Imatinib and chronic myeloid leukemia 74
10.73 PDGFRB PDGFRA KIT
22
Mammary gland development pathway - Puberty (Stage 2 of 4) 74
10.69 VIM PGR
Sources

GO Terms for Leiomyosarcoma

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Biological processes related to Leiomyosarcoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 positive regulation of cell population proliferation GO:0008284 10.17 PDGFRB PDGFRA MDM2 KIT IGF2 CDK4
2 positive regulation of fibroblast proliferation GO:0048146 9.95 PDGFRB PDGFRA CDK4
3 positive regulation of phosphatidylinositol 3-kinase activity GO:0043552 9.85 KIT PDGFRA PDGFRB
4 metanephric S-shaped body morphogenesis GO:0072284 9.71 WT1 PDGFRB
5 DNA damage response, signal transduction by p53 class mediator resulting in cell cycle arrest GO:0006977 9.63 MUC1 MDM2 CHEK2
6 metanephric glomerular capillary formation GO:0072277 9.62 PDGFRB PDGFRA
7 positive regulation of cell proliferation by VEGF-activated platelet derived growth factor receptor signaling pathway GO:0038091 9.56 PDGFRB PDGFRA
8 positive regulation of protein modification process GO:0031401 9.46 PDGFRB KIT
9 cardiac myofibril assembly GO:0055003 9.43 PDGFRB PDGFRA ACTC1
10 positive regulation of phospholipase C activity GO:0010863 9.1 PDGFRB PDGFRA KIT

Molecular functions related to Leiomyosarcoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 transmembrane receptor protein tyrosine kinase activity GO:0004714 9.43 PDGFRB PDGFRA KIT
2 vascular endothelial growth factor binding GO:0038085 8.92 PDGFRB PDGFRA
Sources

Sources for Leiomyosarcoma

About this section
2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 08-Dec-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 UMLS via Orphanet
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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