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Low-grade myofibroblastic sarcoma

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89.6% received surgical treatment, 29.2% received radiation treatment, and 20% received chemotherapy. The study concluded that radiotherapy and chemotherapy had limited effects on survival and therefore should not be routinely used in LGMS, especially for cases in which all tumor cells are removed. These results and conclusions agree with previous reports finding that LGMS tumors are insensitive to radiotherapy and chemotherapy. Nonetheless, there have been case reports that local radiotherapy may allow longer survival periods and may, in select cases, be useful for treating LGMS. A similar situation exists with chemotherapy: some reports recommend chemotherapy as a potential treatment strategy, particularly when complete excision of the tumor is not possible, when the tumor is highly invasive, and/or when the tumor has spread to lymph nodes and/or distant tissues. There are case reports where chemotherapy following surgical excision may have been useful in prolonging progression-free survival, for example, in an individual with a pancreatic LGMS tumor (treatment regimen:
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75.0% and 71.6%, respectively. Their disease-specific survival (i.e. excluding deaths from causes unrelated to LGMS) at 3 and 5 years after treatment were 80.0% and 76.3%, respectively. These patients were treated with surgery in 93.9% of cases and radiotherapy in 26.5% of cases but no patients had lymph node metastasis and only 1 case had distant metastases (the presence of lymph node and distant tissue metastasis was unknown in 8 patients). The study of 96 individuals treated for LFMS reported 1, 3, 5, and 10-year disease-specific survival percentages of 88%, 77%, 70%, and 59%, respectively. Patient age >60 years was the only factor that clearly reduced survival times in this study (disease specific survival times for patients 60 years old or younger and >60 years were 167.1 and 92.5 months, respectively).
452:, have not been systematically investigated nor validated. Currently, the primary and most common treatment for non-metastatic LGMS is surgical resection with, where possible, removal of all tumor cells in order to reduce this tumor's recurrence rate (e.g. ~27% and ~38% in two different studies). Following this surgery, individuals should undergo long-term observation to check for post-surgical recurrences and the uncommon instances of metastases. One study suggested that tumor cells with high rates of proliferation, tumors containing areas of necrosis, tumor sizes >10 cm in largest diameters, and deep-seated tumors are at higher risks for metastasizing. 245:(which omentum not defined), heart, eye socket (in an 11 month old infant), and chest wall/breast. While typically presenting as slow growing, painless masses, some individuals have presented with increasingly painful subcutaneous or submucosal masses (16 of 50 individuals reported pain in one retrospective study). Rare cases of submucosal LGMS tumors have presented with more serious symptoms such as partial 136:. WHO, 2020, classifies HGMS (preferred name: undifferentiated pleomorphic sarcoma) as a soft tissue tumor in the category of tumors of uncertain differentiation. This article follows the WHO classification: here, LGMS includes IGMS but not HGMS which is a more aggressive and metastasizing tumor than LGMS and consists of cells of uncertain origin. 478:
The majority of studies on the prognosis of patients treated for LGMS have focused on short-term (i.e. one-year) follow-up times. One study of 49 patients (age range: 29.5–64.5; average age: 46.2 years; median age: 51.0 years) treated for LGFS reported overall survival percentages at 3 and 5 years of
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have been used with or without surgical resections to treat cases in which tumor resections were later found to leave tumor cells behind, in which tumors could not be safely resected, and in which metastases were present. In the study of 96 individuals presenting with LGMS tumors for the first time,
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of chromosomes 1, 12, and 5 and losses in genetic material on the long (i.e. 'q') arm of chromosome 15. These chromosome abnormalities are considered non-specific. Analysis of LGMS tumor cells for chromosome and gene abnormalities has not yet been helpful in understanding or diagnosing the disorder.
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studies. In another study, 103 individuals diagnosed with LGMS were aged 2–75 years (median: 43 years) with 12.6% < 18 years, 65.1% 18–60 years, and 22.3% >60  years old. Eighty-two percent of their LGMS tumors were located in soft tissues (28.2% in mucous membranes, 21.8% in muscle, 19.2% in
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LGMS should be suspected in cases presenting as nodular masses composed of spindle-shaped cells combining fibroblast and smooth muscle cell features that are arranged in bundles and express α-smooth muscle actin and/or desmin proteins but not vimentin, S-100, CD34 or other marker proteins cited in
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fibers. The tumors are not encapsulated and commonly infiltrate adjacent fibrous, fat, or skeletal muscle tissues. (The tumor's spindle-shaped cells may infiltrate between individual skeletal muscle fibers to create a characteristic checkerboard pattern.) LGMS tissues commonly have small or more
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LGMS tumor. A study of 96 individuals presenting for the first time with LGMS found that 51.0% had local disease, 25.0% had regional disease, 15.6% had metastases to the local lymph nodes, and 8.3% had distant metastases. (In the study 103 individuals, the distant metastasis rate was 4.4%.)
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LGMS's are commonly treated by surgical removal of the tumor along with all its cells, which if not removed increase the probability that the tumor will recur at the site of its removal. LGMS tumors typically show little or no sensitivity to radiotherapy and chemotherapy treatments.
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the previous section. Spindle-shaped cell infiltrations between individual skeletal muscle fibers that form a checkerboard pattern and the presence of foci containing epithelioid, polygonal cells that express cytokeratin and TP63 proteins are also indicative of a LGMS tumor.
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bones. They most often develop in middle-aged adults (average: 40 years old) but have been diagnosed in all age-groups. These tumors often recur at the sites of their surgical removal and may metastasize to nearby lymph nodes and distant tissues.
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protein found in all muscle forms including smooth muscle) proteins, with some tumors composed of cells expressing both of these proteins and other tumors composed of cells expressing only one of them. The tumor cells often express
116:) by Mentzel et al. in 1998. Myofibroblastic sarcomas had been divided into low-grade myofibroblastic sarcomas, intermediate‐grade myofibroblasic sarcomas, i.e. IGMS, and high‐grade myofibroblasic sarcomas, i.e. HGMS (also termed 1067:
Xu Y, Xu G, Wang X, Mao M, Wu H, Baklaushev VP, Chekhonin VP, Peltzer K, Wang G, Zhang C (February 2021). "Is there a role for chemotherapy and radiation in the treatment of patients with low-grade myofibroblastic sarcoma?".
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and/or giant marker chromosome, which commonly occur in the cells of various mesenchymal tumors, were found in one case of LGMS. In addition, these tumor cells may, in rare cases, contain
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Yonezawa H, Yamamoto N, Hayashi K, Takeuchi A, Miwa S, Igarashi K, Langit MB, Kimura H, Shimozaki S, Kato T, Morinaga S, Araki Y, Asano Y, Ikeda H, Nojima T, Tsuchiya H (December 2020).
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Tang L, Xu H, Gao H, Yang H, Chen S, Zhang P (November 2020). "Primary low-grade myofibroblastic sarcoma: A rare case report of this tumor in the orbit and literature review".
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skin, and 12.9% in other soft tissues) and 18% were in bone. Overall, 51.5% of their tumors were in the head and neck areas (most commonly the tongue, followed by the
108:. As it is currently recognized, LGMS was first described as a rare, atypical myofibroblastic tumor (i.e. a tumor consisting of cells with the microscopic features of 1602: 579:
Mentzel T, Dry S, Katenkamp D, Fletcher CD (October 1998). "Low-grade myofibroblastic sarcoma: analysis of 18 cases in the spectrum of myofibroblastic tumors".
128:, and malignancy features. LGMS and IGMS are now classified together by the World Health Organization (WHO), 2020, in the category of intermediate (rarely 1003:"Low-grade myofibroblastic sarcoma with abdominal pain, a stuffy nose, hearing loss, and multiple cavity effusion: a case report and literature review" 1597: 1364:"Evaluation and Management of an Uncommon Tumor of the Larynx: A Case Report and Literature Review of Laryngeal Low-Grade Myofibroblastic Sarcoma" 133: 117: 1114:
Chan JY, Gooi Z, Wong EW, Ng SK, Tong MC, Vlantis AC (January 2017). "Low-grade myofibroblastic sarcoma: A population-based study".
1552:"Characterization of giant marker and ring chromosomes in a pleomorphic leiomyosarcoma of soft tissue by spectral karyotyping" 1164:"Low-Grade Myofibroblastic Sarcoma of the Oral Cavity: A Report of Three Cases Illustrating an Emerging Disease in Children" 1316:
Oylumlu M, Yildiz A, Ercan S, Oylumlu M, Davutoglu V (2014). "Cardiac metastasis of a low-grade myofibroblastic sarcoma".
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Choi JH, Ro JY (January 2021). "The 2020 WHO Classification of Tumors of Soft Tissue: Selected Changes and New Entities".
205:, face, skull, and ear canal), 25.2% were in the trunk, and 23.3% were in an arm or leg. Bone tumors were located in the 616:"Malignant fibrous histiocytoma of the bone in a traumatic amputation stump: A case report and review of the literature" 302:
LGMS tissues generally show bundles of atypical spindle-shaped cells in a variably hyalinized (i.e. glassy appearing)
45: 419: 160: 342: 513:"Low-grade myofibroblastic sarcoma of the levator scapulae muscle: a case report and literature review" 475:). Further studies are needed to define the usefulness of radiation therapy and chemotherapy in LGMS. 330: 168: 121: 144: 1501:
Magro G, Salvatorelli L, Puzzo L, Piombino E, Bartoloni G, Broggi G, Vecchio GM (December 2019).
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Intermediate-grade myofibroblastic sarcoma is now regarded as a low-grade myofibroblastic sarcoma
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LGMS present as single tumors that ranged in size from 0.4 to 24.0 cm in three
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due to a LGMS tumor in the heart, difficulty in swallowing and breathing due to a
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Jayasooriya PR, Athukorala C, Attygalla M, Mendis BR, Lombardi T (January 2021).
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Metastasis have been reported to develop in various sites including the lungs,
1283: 873:"Limbal, low-grade myofibroblastic sarcoma: Case report and literature review" 687: 632: 472: 464: 411: 383: 320: 316: 279: 267: 109: 1518: 1434: 1417: 1267:"Giant low-grade primary myofibroblastic sarcoma of the posterior chest wall" 1235: 1018: 889: 734: 82: 1577: 1536: 1443: 1399: 1337: 1302: 1243: 1199: 1135: 1089: 1036: 970: 908: 836: 801: 783: 768:"The 2020 WHO Classification of Soft Tissue Tumours: news and perspectives" 752: 695: 651: 548: 1478: 600: 333:
analyses find that the LGMS tumors' spindle-shaped cells commonly express
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abnormalities have been found in the tumor cells of a few LGMS cases. A
1329: 1127: 351: 214: 105: 338: 263: 254: 226: 198: 156: 354:(also termed INI-1 and SNF5) proteins but typically fail to express 1362:
Nair NP, Kaushal D, Rao M, Soni K, Vaithankalath S (October 2020).
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Nishio J, Aoki M, Nabeshima K, Iwasaki H, Naito M (August 2012).
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and pleomorphic myofibrosarcoma ) based on their microscopic
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LGMS tumors are typically painless lesions that develop in:
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Wu X, Guo L, Li S, Zheng Y, Fan B, Zhou C (January 2020).
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proteins. The epithelioid, polygonal-shaped cells express
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due to intrabdominal LGMS tumors, shortness of breath and
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Zhao R, Wang J, Zhang H, Chi Y, Bi N (October 2020).
91: 81: 73: 65: 57: 44: 34: 26: 21: 766:Sbaraglia M, Bellan E, Dei Tos AP (April 2021). 614:Zhao KY, Yan X, Yao PF, Mei J (September 2021). 1496: 1494: 1492: 1490: 1488: 1411: 1409: 1357: 1355: 1217: 1215: 1213: 1211: 1209: 1157: 1155: 1153: 1109: 1107: 1062: 1060: 1058: 1056: 1054: 1052: 1050: 1048: 1046: 996: 994: 992: 990: 988: 986: 984: 982: 980: 229:. In other reports, the tumors occurred in the 930: 928: 926: 924: 922: 920: 918: 866: 864: 862: 860: 858: 856: 854: 674:Fisher C (September 2004). "Myofibrosarcoma". 669: 667: 665: 663: 661: 506: 504: 502: 500: 498: 496: 494: 492: 1416:Peng L, Tu Y, Li Y, Xiao W (September 2018). 1007:The Journal of International Medical Research 574: 572: 570: 568: 566: 564: 562: 560: 558: 444:Past treatments for LGMS, including surgery, 422:such as gains in the genetic material on the 8: 871:Mulay K, Sen M, Honavar SG (November 2020). 1422:Journal of Cancer Research and Therapeutics 581:The American Journal of Surgical Pathology 18: 1567: 1526: 1433: 1389: 1379: 1292: 1282: 1189: 1179: 1026: 960: 898: 888: 791: 742: 641: 631: 538: 528: 257:LGMS tumor, and abdominal pain due to a 147:, i.e. the lowermost layer of the skin; 488: 337:(also known α-smooth muscle actin) and 134:fibroblastic and myofibroblastic tumors 50:Post-surgical recurrences; uncommonly 1465:Lehoczky T, Halasy M (May 1966). "". 1070:Clinical & Translational Oncology 104:(LGMS) is a subtype of the malignant 7: 1603:Connective and soft tissue neoplasms 1318:Echocardiography (Mount Kisco, N.Y.) 935:Baranov E, Hornick JL (March 2020). 311:extensive foci of epithelioid (i.e. 118:undifferentiated pleomorphic sarcoma 1271:World Journal of Surgical Oncology 1265:Katalinic D, Santek F (May 2017). 159:that line passageways such as the 151:submucosa, i.e. the thin layer of 14: 1224:European Journal of Ophthalmology 406:Chromosome and gene abnormalities 102:Low-grade myofibroblastic sarcoma 22:Low-grade myofibroblastic sarcoma 593:10.1097/00000478-199810000-00008 1598:Dermal and subcutaneous growths 1181:10.3390/dermatopathology8010001 877:Indian Journal of Ophthalmology 620:World Journal of Clinical Cases 817:Advances in Anatomic Pathology 1: 517:BMC Musculoskeletal Disorders 300:hematoxylin and eosin stained 829:10.1097/PAP.0000000000000284 327:(i.e. dead or dying cells). 1624: 1082:10.1007/s12094-020-02425-4 953:10.1007/s12105-019-01104-3 530:10.1186/s12891-020-03857-3 221:, or in one case each the 1428:(Supplement): S796–S799. 1284:10.1186/s12957-017-1167-7 688:10.1007/s00428-004-1038-9 633:10.12998/wjcc.v9.i26.7930 77:Surgical removal of tumor 1519:10.32074/1591-951X-31-19 1435:10.4103/0973-1482.183551 1236:10.1177/1120672120970392 1019:10.1177/0300060519895661 40:Tumor, sometimes painful 941:Head and Neck Pathology 890:10.4103/ijo.IJO_2410_20 735:10.1111/1759-7714.13613 440:Treatment and prognosis 1013:(1): 300060519895661. 784:10.32074/1591-951X-213 420:copy number variations 306:background containing 343:intermediate filament 155:lying just below the 1569:10.3892/or.2012.1835 1381:10.7759/cureus.11072 424:short (i.e. 'p') arm 319:, sites of numerous 282:, heart, brain, and 145:subcutaneous tissues 331:Immunohistochemical 114:smooth muscle cells 1330:10.1111/echo.12370 1128:10.1002/lary.26146 247:bowel obstructions 883:(11): 2538–2540. 729:(10): 3011–3014. 626:(26): 7930–7936. 296:histopathological 194:literature review 99: 98: 16:Medical condition 1615: 1582: 1581: 1571: 1556:Oncology Reports 1547: 1541: 1540: 1530: 1498: 1483: 1482: 1469:(in Hungarian). 1462: 1456: 1455: 1437: 1413: 1404: 1403: 1393: 1383: 1359: 1350: 1349: 1313: 1307: 1306: 1296: 1286: 1262: 1256: 1255: 1230:(2): NP67–NP70. 1219: 1204: 1203: 1193: 1183: 1168:Dermatopathology 1159: 1148: 1147: 1116:The Laryngoscope 1111: 1102: 1101: 1064: 1041: 1040: 1030: 998: 975: 974: 964: 932: 913: 912: 902: 892: 868: 849: 848: 812: 806: 805: 795: 763: 757: 756: 746: 714: 708: 707: 671: 656: 655: 645: 635: 611: 605: 604: 576: 553: 552: 542: 532: 508: 276:abdominal cavity 161:gastrointestinal 157:mucous membranes 126:immunophenotypic 19: 1623: 1622: 1618: 1617: 1616: 1614: 1613: 1612: 1588: 1587: 1586: 1585: 1549: 1548: 1544: 1500: 1499: 1486: 1464: 1463: 1459: 1415: 1414: 1407: 1361: 1360: 1353: 1315: 1314: 1310: 1264: 1263: 1259: 1221: 1220: 1207: 1161: 1160: 1151: 1113: 1112: 1105: 1066: 1065: 1044: 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115: 111: 107: 103: 94: 90: 86: 84: 80: 76: 72: 68: 64: 60: 56: 53: 49: 47: 46:Complications 43: 39: 37: 33: 29: 25: 20: 1562:(2): 533–8. 1559: 1555: 1545: 1510: 1506: 1470: 1466: 1460: 1425: 1421: 1371: 1367: 1321: 1317: 1311: 1274: 1270: 1260: 1227: 1223: 1171: 1167: 1119: 1115: 1073: 1069: 1010: 1006: 947:(1): 43–58. 944: 940: 880: 876: 823:(1): 44–58. 820: 816: 810: 778:(2): 70–84. 775: 771: 761: 726: 722: 712: 679: 675: 623: 619: 609: 584: 580: 520: 516: 477: 460:chemotherapy 456:Radiotherapy 454: 450:chemotherapy 446:radiotherapy 443: 434: 409: 329: 298:analyses of 294:Microscopic 293: 251:palpitations 191: 188:Presentation 182: 176: 172: 148: 140: 138: 101: 100: 52:metastasizes 1507:Pathologica 1324:(1): E1–4. 772:Pathologica 469:pirarubicin 396:cytokeratin 380:cytokeratin 321:neutrophils 284:spinal cord 268:lymph nodes 231:oral mucosa 223:hard palate 165:respiratory 110:fibroblasts 58:Usual onset 27:Other names 1592:Categories 1174:(1): 1–9. 523:(1): 836. 483:References 473:nedaplatin 465:ifosfamide 412:chromosome 402:proteins. 317:mast cells 313:epithelial 280:peritoneum 1277:(1): 96. 1252:226988997 1098:220286531 845:221862064 704:220565385 431:Diagnosis 388:β-catenin 341:(i.e. an 290:Pathology 270:, bones, 255:laryngeal 83:Prognosis 74:Treatment 1578:22641359 1537:31965112 1452:52814348 1444:30249907 1400:33224666 1338:24111760 1303:28468630 1244:33198493 1200:33401376 1136:27377169 1090:32607812 1037:31975633 971:31950474 909:33120683 837:32960834 802:33179614 753:32815307 696:15173943 652:34621848 549:33302922 410:Various 392:myogenin 348:vimentin 325:necrosis 308:collagen 259:pancreas 211:mandible 203:mandible 201:, gums, 171:tracts; 106:sarcomas 95:Uncommon 61:All ages 36:Symptoms 1528:8145669 1479:5328880 1391:7676948 1346:7195357 1294:5415824 1191:7838777 1144:9627754 1028:7114293 962:7021862 900:7774147 793:8167394 744:7529570 643:8462244 601:9777985 540:7731512 352:SMARCB1 304:stromal 215:maxilla 87:Guarded 69:Unknown 1608:Cancer 1576:  1535:  1525:  1477:  1450:  1442:  1398:  1388:  1368:Cureus 1344:  1336:  1301:  1291:  1250:  1242:  1198:  1188:  1142:  1134:  1096:  1088:  1035:  1025:  969:  959:  907:  897:  843:  835:  800:  790:  751:  741:  702:  694:  650:  640:  599:  547:  537:  471:, and 448:, and 339:desmin 264:pleura 227:sacrum 219:tibias 207:femurs 199:larynx 153:tissue 92:Deaths 66:Causes 1448:S2CID 1342:S2CID 1248:S2CID 1140:S2CID 1094:S2CID 841:S2CID 700:S2CID 390:, or 368:STAT6 360:S-100 335:ACTA2 1574:PMID 1533:PMID 1475:PMID 1440:PMID 1396:PMID 1334:PMID 1299:PMID 1240:PMID 1196:PMID 1132:PMID 1086:PMID 1033:PMID 967:PMID 905:PMID 833:PMID 798:PMID 749:PMID 692:PMID 648:PMID 597:PMID 545:PMID 458:and 400:TP63 398:and 376:CD56 372:CD68 364:CD34 356:CD34 350:and 225:and 143:the 112:and 1564:doi 1523:PMC 1515:doi 1511:111 1471:107 1430:doi 1386:PMC 1376:doi 1326:doi 1289:PMC 1279:doi 1232:doi 1186:PMC 1176:doi 1124:doi 1120:127 1078:doi 1023:PMC 1015:doi 957:PMC 949:doi 895:PMC 885:doi 825:doi 788:PMC 780:doi 776:113 739:PMC 731:doi 684:doi 680:445 638:PMC 628:doi 589:doi 535:PMC 525:doi 384:ERG 241:or 124:, 1594:: 1572:. 1560:28 1558:. 1554:. 1531:. 1521:. 1509:. 1505:. 1487:^ 1446:. 1438:. 1426:14 1424:. 1420:. 1408:^ 1394:. 1384:. 1372:12 1370:. 1366:. 1354:^ 1340:. 1332:. 1322:31 1320:. 1297:. 1287:. 1275:15 1273:. 1269:. 1246:. 1238:. 1228:32 1226:. 1208:^ 1194:. 1184:. 1170:. 1166:. 1152:^ 1138:. 1130:. 1118:. 1106:^ 1092:. 1084:. 1074:23 1072:. 1045:^ 1031:. 1021:. 1011:48 1009:. 1005:. 979:^ 965:. 955:. 945:14 943:. 939:. 917:^ 903:. 893:. 881:68 879:. 875:. 853:^ 839:. 831:. 821:28 819:. 796:. 786:. 774:. 770:. 747:. 737:. 727:11 725:. 721:. 698:. 690:. 678:. 660:^ 646:. 636:. 622:. 618:. 595:. 585:22 583:. 557:^ 543:. 533:. 521:21 519:. 515:. 491:^ 467:, 386:, 382:, 378:, 374:, 370:, 366:, 362:, 358:, 286:. 278:, 274:, 266:, 237:, 217:, 213:, 209:, 177:4) 173:3) 167:, 163:, 149:2) 141:1) 132:) 1580:. 1566:: 1539:. 1517:: 1481:. 1454:. 1432:: 1402:. 1378:: 1348:. 1328:: 1305:. 1281:: 1254:. 1234:: 1202:. 1178:: 1172:8 1146:. 1126:: 1100:. 1080:: 1039:. 1017:: 973:. 951:: 911:. 887:: 847:. 827:: 804:. 782:: 755:. 733:: 706:. 686:: 654:. 630:: 624:9 603:. 591:: 551:. 527::

Index

Symptoms
Complications
metastasizes
Prognosis
sarcomas
fibroblasts
smooth muscle cells
undifferentiated pleomorphic sarcoma
morphological
immunophenotypic
metastasizing
fibroblastic and myofibroblastic tumors
subcutaneous tissues
tissue
mucous membranes
gastrointestinal
respiratory
genitourinary
literature review
larynx
mandible
femurs
mandible
maxilla
tibias
hard palate
sacrum
oral mucosa
small intestine
greater omentum

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