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Undifferentiated pleomorphic sarcoma

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about 70, 60, 50, and 0%, respectively; overall survival rates at 150 months for disease at these sites were about 90, 80, 75, and 35%, respectively. Patients who received surgery alone or surgery plus adjuvant treatment had disease-free survival rates of about 50 and 40%, respectively. A retrospective analysis of 266 patients with UPS were treated with surgery alone (6% of cases), surgery plus radiotherapy (91% of cases), or surgery plus chemotherapy (3% of cases). Post-treatment local recurrences and metastases were observed in 15% and 38% of cases; 5- and 10-year overall survival rates were 60% and 48%, respectively; Overall median survival time were 10.1 years; and patients with tumors ≄10 cm in longest diameter had an almost 6-fold higher rate of developing metastases than patients with tumors 4 cm or smaller. Poorer prognoses were seen in older patients; in patients with tumors that were large-sized, deep-seated, and/or located in a leg; in patients that presented with metastases; and in patients who had positive surgical margins and/or developed local recurrences after surgery. In another retrospective study, 203 individuals with UPS, 141 of whom had metastatic disease, were treated with regimens selected based on the severity of their disease. In this study, the overall 5 year survival rate was 4%. Patients with the most advanced disease had a median overall survival time 11 months.
638:; or etoposide, ifosfamide, and cisplatin. These treatment regimens have been reported to lower local recurrence rates, prolong disease-free survival rates (i.e. time after treatment when no disease is detected), and increase overall survival rates (i.e. time after treatment to death from any cause). However, other studies report that the addition of radiotherapy and/or chemotherapy to surgical resection does not improve recurrence or overall survival rates; addition of radiotherapy to surgery improves local control of UPS tumors but not disease-free survival rates (i.e. time from treatment to recurrence of disease); and adjuvant chemotherapy and radiotherapy have no significant effects on local recurrence-free survival rates, metastasis-free survival times, and overall survival rates. Further studies are needed to define the best treatments for UPS tumors. 685:, 3 had partial responses, and 6 had no response to pembrolizumab. (Further studies of the 4 pembrolizumab-responsive patients indicated that their tumor cells expressed PD-L1 in two cases but not in the other two cases.) In a retrospective study of 25 patients (21 patients treated with pembrolizumab, 4 treated with other immunotherapy agents), 7 attained stable disease, 7 attained partial responses, and 1 attained a complete response. In a study of 16 patients with UPS, 5 achieved short-term (lasting 1.2 to 1.4 months) stable disease in response to a regimen of pembrolizumab combined with the chemotherapy drug, 48: 262:
individuals with the neoplastic syndrome had similar tumor recurrence rates (57.14% vs 53.93% for the two respective groups) but a lower metastasis rate (14.29% vs 44.94%) and a higher 3-year survival rate (85.71% vs 59.55%). Fever symptoms disappeared in all patients after surgical removal of their tumors. It is suggested that individuals with UPS and neoplastic fever have a more favorable prognosis than individuals with UPS that do not evidence such fevers.
278:) because the histopathology of this disorder's tumors is non-specific. UPS tumor cells are undifferentiated (i.e. do not resemble any particular cell type) and pleomorphic (i.e. highly variable in size, shape, and/or color) when examined microscopically. Therefore, the diagnosis of UPS is commonly based on detecting a specific set of proteins that are expressed by UPS tumor cells but not by the cells of other undifferentiated and pleomorphic tumors or 229:(average 8.8 cm with 25%, 38%, and 38% having greatest diameters of 0–1, 5–9, and ≄10 cm, respectively). In a study of 205 individuals (median age 59 years) diagnosed with UPS, the tumors were located in the arm or leg (47.3% of cases), abdomen or pelvis (26.8%), thorax (17.6%), and head or neck (8.3%) areas. In rare cases, these tumors have also presented in other sites such as the 309:. In a later study that examined 83 individuals, 72.8% had UPS tumor cells that expressed PD-L1 with 53%, 35%, and 12% of these cases showing weak, intermediate, and strong PD-L1 expression, respectively. And, in a study of 73 patients with UPS, 39 cases showed no, 23 cases showed low, 10 showed intermediate, and 11 strong immunoreactivity for 241:. In a retrospective study, distant metastases were detected at the time of initial diagnosis in 6.4% of 266 individuals. Overall, metastases have or will develop in up to 40% of individuals with UPS. These metastases are reported to occur in lung (40% to 55% of cases) and less commonly in other sites such as 641:
In a retrospective study of 176 patients with localized UPS undergoing curative-intent treated with surgical resection or resection plus adjuvant treatment, disease-free survival rates at 120 months for patients with tumors in an extremity (leg or arm), heat/neck area, thorax, and abdomen/pelvis were
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with surgical resection is employed to reduce the risk of developing recurrent and metastatic disease in cases with high-risk disease (e.g. large tumors, tumors deemed highly aggressive based on their pathology and/or local invasiveness, inoperable tumors, and resections that did not remove all tumor
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The diagnosis of UPS depends on finding non-specific, undifferentiated tumor cells that have features suggestive of UPS and not features of other tumor types that also consist of pleomorphic, undifferentiated cells. The features primarily involve the expression of certain proteins by the tumor cells.
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located on the surface of normal tissue cells from binding to PD-1 receptors on activated T-cells and thereby blocks the T-cells from organizing an inflammatory response that kills the normal cells. Tumor cells may use this inflammation-evading tactic: they may express PD-L1/PD-L2 and thereby block
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termed neoplastic fever, i.e. these individuals suffered continuous, disabling fevers. Their tumors were located within a thigh muscle (4 cases), the upper arm (2 cases), or the lower leg (1 case). Compared to 89 individuals (median age 59.1 years) with a similar distribution of their UPS tumors,
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Gronchi A, Ferrari S, Quagliuolo V, Broto JM, Pousa AL, Grignani G, Basso U, Blay JY, Tendero O, Beveridge RD, Ferraresi V, Lugowska I, Merlo DF, Fontana V, Marchesi E, Donati DM, Palassini E, Palmerini E, De Sanctis R, Morosi C, Stacchiotti S, Bagué S, Coindre JM, Dei Tos AP, Picci P, Bruzzi P,
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UPS commonly presents as a deep-seated, rapidly enlarging, painless mass in individuals aged 50 to 70 years. These masses are rarely superficial lesions and rarely occur in the pediatric population. In a retrospective study of 266 individuals, UPS tumors ranged from 1–55 cm in greatest diameter
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protein (i.e. CKLF like MARVEL transmembrane domain containing 6 protein). Strong PD‑L1 expression proved to be a poor, while expression of IDO‑1 proved to be a favorable, prognostic factor for disease outcomes. Individuals with tumor cells that strongly expressed CMTM6 protein also had poor
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Ishihara S, Iwasaki T, Kohashi K, Yamada Y, Toda Y, Ito Y, Susuki Y, Kawaguchi K, Takamatsu D, Kawatoko S, Kiyozawa D, Mori T, Kinoshita I, Yamamoto H, Fujiwara T, Setsu N, Endo M, Matsumoto Y, Nakashima Y, Oda Y (July 2021). "The association between the expression of PD-L1 and CMTM6 in
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Orth MF, Gerke JS, Knösel T, Altendorf-Hofmann A, Musa J, Alba-Rubio R, Stein S, Hölting TL, Cidre-Aranaz F, Romero-PĂ©rez L, Dallmayer M, Baldauf MC, Marchetto A, Sannino G, Knott MM, Wehweck F, Ohmura S, Li J, Hakozaki M, Kirchner T, Dandekar T, Butt E, GrĂŒnewald TG (February 2019).
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Tawbi HA, Burgess M, Bolejack V, Van Tine BA, Schuetze SM, Hu J, D'Angelo S, Attia S, Riedel RF, Priebat DA, Movva S, Davis LE, Okuno SH, Reed DR, Crowley J, Butterfield LH, Salazar R, Rodriguez-Canales J, Lazar AJ, Wistuba II, Baker LH, Maki RG, Reinke D, Patel S (November 2017).
689:. It is clear that new treatment strategies as well as further studies on the efficacy of pembrolizumab and similarly acting immunotherapy drugs used with or without radiotherapy and/or chemotherapy over longer time periods are needed to evaluate their usefulness in treating UPS. 564:, and often associated with neural tissue. These tumors typically show undifferentiated, pleomorphic cells that are arranged in whorls, parallel bundles, or rosettes (i.e. circular arrangement resembling leaves in a flowering plant) and often contain large areas of 2684:
Keung EZ, Burgess M, Salazar R, Parra ER, Rodrigues-Canales J, Bolejack V, Van Tine BA, Schuetze SM, Attia S, Riedel RF, Hu J, Okuno SH, Priebat DA, Movva S, Davis LE, Reed DR, Reuben A, Roland CL, Reinke D, Lazar AJ, Wang WL, Wargo JA, Tawbi HA (March 2020).
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UPS tumors also show gene and chromosome abnormalities that further studies may find contribute to the development and/or progression of UPS. These abnormalities, which have not yet been reported to be helpful in diagnosing UPS, include the following.
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Kamat NV, Million L, Yao DH, Donaldson SS, Mohler DG, van de Rijn M, Avedian RS, Kapp DS, Ganjoo KN (February 2019). "The Outcome of Patients With Localized Undifferentiated Pleomorphic Sarcoma of the Lower Extremity Treated at Stanford University".
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quite distinct from UPS. Because of their low incidence and frequent grouping with what are now considered to be other sarcoma types, past findings on the clinical behaviour, proper treatment, and prognosis of UPS may be revised with further study.
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Lee K, Song JS, Kim JE, Kim W, Song SY, Lee MH, Chung HW, Cho KJ, Lee JS, Ahn JH (July 2020). "The clinical outcomes of undifferentiated pleomorphic sarcoma (UPS): A single-centre experience of two decades with the assessment of PD-L1 expressions".
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Casali PG (June 2017). "Histotype-tailored neoadjuvant chemotherapy versus standard chemotherapy in patients with high-risk soft-tissue sarcomas (ISG-STS 1001): an international, open-label, randomised, controlled, phase 3, multicentre trial".
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Toulmonde M, Lucchesi C, Verbeke S, Crombe A, Adam J, Geneste D, Chaire V, Laroche-Clary A, Perret R, Bertucci F, Bertolo F, Bianchini L, Dadone-Montaudie B, Hembrough T, Sweet S, Kim YJ, Cecchi F, Le Loarer F, Italiano A (December 2020).
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and intermediate‐grade myofibroblasic sarcomas. WHO has combined low- and intermediate-grade myofibroblastic sarcomas into a single entity, low-grade myofibroblastic sarcomas, and categorized it as one type of the intermediate (rarely
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Savina M, Le Cesne A, Blay JY, Ray-Coquard I, Mir O, Toulmonde M, Cousin S, Terrier P, Ranchere-Vince D, Meeus P, Stoeckle E, Honoré C, Sargos P, Sunyach MP, Le Péchoux C, Giraud A, Bellera C, Le Loarer F, Italiano A (April 2017).
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gene and patients with higher AMPD2 levels had poorer prognoses (5 year survivals for AMPD2 positive versus AMPD2 negative cases were ~38 and 59%, respectively). Other abnormalities found in some or isolated cases of UPS include:
136:, tendons, and ligaments. More than 70 sarcoma subtypes have been described. The UPS subtype of these sarcomas consists of tumor cells that are poorly differentiated and may appear as spindle-shaped cells, 577:
The most often used treatment for localized (i.e. no metastases) UPS tumors is complete surgical removal with the object of leaving no tumor cells behind as evidenced by microscopic examinations.
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Toulmonde M, Penel N, Adam J, Chevreau C, Blay JY, Le Cesne A, Bompas E, Piperno-Neumann S, Cousin S, Grellety T, Ryckewaert T, Bessede A, Ghiringhelli F, Pulido M, Italiano A (January 2018).
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Delespaul L, Lesluyes T, PĂ©rot G, Brulard C, Lartigue L, Baud J, Lagarde P, Le Guellec S, Neuville A, Terrier P, Vince-RanchĂšre D, Schmidt S, Debant A, Coindre JM, Chibon F (February 2017).
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Vodanovich DA, Spelman T, May D, Slavin J, Choong PF (September 2019). "Predicting the prognosis of undifferentiated pleomorphic soft tissue sarcoma: a 20-year experience of 266 cases".
113:), also termed pleomorphic myofibrosarcoma, high-grade myofibroblastic sarcoma, and high-grade myofibrosarcoma, is characterized by the World Health Organization (WHO) as a rare, poorly 3533: 2734:
Monga V, Skubitz KM, Maliske S, Mott SL, Dietz H, Hirbe AC, Van Tine BA, Oppelt P, Okuno S, Robinson S, O'Connor M, Seetharam M, Attia S, Charlson J, Agulnik M, Milhem M (July 2020).
1577:"High throughput profiling of undifferentiated pleomorphic sarcomas identifies two main subgroups with distinct immune profile, clinical outcome and sensitivity to targeted therapies" 2834:
Keung EZ, Lazar AJ, Torres KE, Wang WL, Cormier JN, Ashleigh Guadagnolo B, Bishop AJ, Lin H, Hunt KK, Bird J, Lewis VO, Patel SR, Wargo JA, Somaiah N, Roland CL (September 2018).
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The diagnosis of UPS initially included the malignant fibrous histiocytomas (MFH). MFH are now regarded as a wastebasket category of various sarcoma types including sarcoma-like
598:) may be used in these cases. In place or combined with surgery and/or radiotherapy, severe and/or metastatic cases of UPS are commonly treated with epirubicin plus ifosfamide; 3553: 292:
protein (i.e. programmed death-ligand 1 protein) either focally (36.5% of cases) or strongly (9.62% of cases); 48.1% of these individuals had tumor cells which also expressed
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Two other tumors that may be confused with UPS have microscopic histopathological and/or other features that help make this distinction. These tumors and features are:
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The majority of UPS tumors are highly aggressive, often recur after surgical removal, and often metastasize. They are treated with a combination of surgical resection,
1199:"Biology and Management of Undifferentiated Pleomorphic Sarcoma, Myxofibrosarcoma, and Malignant Peripheral Nerve Sheath Tumors: State of the Art and Perspectives" 257:
A review study conducted in China of 183 individuals with UPS reported that 7 (3.83%) individuals (age 51 to 73 years; median age 62.8 years) had a subtype of the
2991: 2836:"Phase II study of neoadjuvant checkpoint blockade in patients with surgically resectable undifferentiated pleomorphic sarcoma and dedifferentiated liposarcoma" 456:
The identifying proteins for UPS tumor cells are given in the preceding section. Identification proteins for tumors that have been confused with UPS inlclude:
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Gleason BC, Nascimento AF (February 2007). "HMB-45 and Melan-A are useful in the differential diagnosis between granular cell tumor and malignant melanoma".
2233:"Pathogenic and Targetable Genetic Alterations in Resected Recurrent Undifferentiated Pleomorphic Sarcomas Identified by Targeted Next-generation Sequencing" 2641:
Mazarico Gallego JM, Herrera JuĂĄrez M, Paz-Ares L (March 2020). "The safety and efficacy of pembrolizumab for the treatment of non-small cell lung cancer".
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Rodrigues GO, Cramer SD, Winer HY, Hixon JA, Li W, Yunes JA, Durum SK (May 2021). "Mutations that collaborate with IL-7Ra signaling pathways to drive ALL".
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Alomari AK, Glusac EJ, McNiff JM (November 2014). "p40 is a more specific marker than p63 for cutaneous poorly differentiated squamous cell carcinoma".
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Renn A, Adejolu M, Messiou C, Bhaludin B, Strauss DC, Thway K, Moskovic E (December 2021). "Overview of malignant soft-tissue sarcomas of the limbs".
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Liang J, Li S, Li W, Rao W, Xu S, Meng H, Zhu F, Zhai D, Cui M, Xu D, Cai J, Zhang B (November 2021). "CMTM6, a potential immunotherapy target".
1831:"PD‑L1 and IDO‑1 expression in undifferentiated pleomorphic sarcoma: The associations with tumor infiltrating lymphocytes, dMMR and HLA class I" 681:). From ~47% to ~73% of UPS cases contain tumor cells that express PD-L1. In a retrospective review of 10 patients with UPS, 1 patient had a 2687:"Correlative Analyses of the SARC028 Trial Reveal an Association Between Sarcoma-Associated Immune Infiltrate and Response to Pembrolizumab" 1254:"Pembrolizumab in advanced soft-tissue sarcoma and bone sarcoma (SARC028): a multicentre, two-cohort, single-arm, open-label, phase 2 trial" 3915: 3889: 3354: 3201: 1088:"Cutaneous soft tissue tumors: how do we make sense of fibrous and "fibrohistiocytic" tumors with confusing names and similar appearances?" 3037: 2592:"Patterns of care and outcomes of patients with METAstatic soft tissue SARComa in a real-life setting: the METASARC observational study" 3114: 330:
pathway that regulates cell proliferation and cell death; this amplification is associated with the overexpression of two proteins,
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Ali NM, Niada S, Brini AT, Morris MR, Kurusamy S, Alholle A, Huen D, Antonescu CR, Tirode F, Sumathi V, Latif F (February 2019).
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Ishihara S, Yamada Y, Iwasaki T, Yoshimoto M, Toda Y, Kohashi K, Yamamoto H, Matsumoto Y, Nakashima Y, Oda Y (January 2021).
331: 1626:"Good and sustained response to pembrolizumab and pazopanib in advanced undifferentiated pleomorphic sarcoma: a case report" 3247: 3154: 851:
Choi JH, Ro JY (January 2021). "The 2020 WHO Classification of Tumors of Soft Tissue: Selected Changes and New Entities".
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gene that encodes transcriptional regulator ATRX protein which contributes to regulating the expression of various genes;
313:, i.e. AMP deaminase 2 protein; the gains in AMPD2 protein immunoreactivity were associated with copy number gains in the 1775:"An unusual case of metastasis of a pulmonary undifferentiated pleomorphic sarcoma to the right ventricle: a case report" 3548: 3538: 3259: 430: 2423:"h-Caldesmon as a specific marker for smooth muscle tumors. Comparison with other smooth muscle markers in bone tumors" 3858: 3830: 3662: 3196: 658: 3785: 3476: 3052: 2787:"Use of PD-1 Targeting, Macrophage Infiltration, and IDO Pathway Activation in Sarcomas: A Phase 2 Clinical Trial" 3780: 3600: 3488: 3269: 3139: 2924: 3585: 296:
protein (i.e. indoleamine 2,3-dioxygenase protein). Tumor cells that strongly expressed PD-L1 also expressed
3863: 3835: 3790: 3567: 3325: 3169: 3109: 3061: 351: 323: 258: 3403: 3254: 3211: 3206: 1978:"Functional genomics identifies AMPD2 as a new prognostic marker for undifferentiated pleomorphic sarcoma" 1472:"Clinical implication of surgical resection for the rare cardiac tumors involving heart and great vessels" 275: 271: 376:
gene that encodes (i.e. is responsible for production of) the retinoblastoma protein that functions as a
354:(this activation has been shown to promote the growth and survival of various types of cancer cells; and 673: 441:(i.e. a hybrid gene formed from two previously independent genes as a result of a mutation) that merges 230: 129: 2331:"COMPASS Ascending: Emerging clues regarding the roles of MLL3/KMT2C and MLL2/KMT2D proteins in cancer" 3744: 3716: 3590: 3435: 3159: 3087: 205: 114: 3764: 3580: 3506: 3388: 3188: 3164: 3124: 3119: 3032: 894: 661:
that binds to and inhibits stimulation of the PD-1 receptors expressed on the surface of activated
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gene that encodes tumor protein P53 (a protein which regulates cell proliferation and cell death);
149: 1677:"Haemichorea-haemiballism secondary to brain metastasis from undifferentiated pleomorphic sarcoma" 3840: 3543: 3518: 3481: 3378: 3347: 3311: 3144: 2666: 2530: 2487: 2403: 2210: 2007: 1957: 1913: 1870: 1345: 1117: 1065: 950: 876: 833: 738: 418: 1524:"Primary Cardiac Undifferentiated High-Grade Intimal Pleomorphic Sarcoma: A Case Series Report" 41:
Pleomorphic myofibrosarcoma, high-grade myofibroblastic sarcoma, and high-grade myofibrosarcoma
17: 3812: 3643: 3615: 3523: 3383: 3174: 3007: 2935: 2867: 2816: 2767: 2736:"A Retrospective Analysis of the Efficacy of Immunotherapy in Metastatic Soft-Tissue Sarcomas" 2716: 2658: 2623: 2571: 2522: 2479: 2444: 2395: 2360: 2311: 2262: 2202: 2158: 2107: 2056: 1999: 1949: 1905: 1862: 1806: 1755: 1706: 1657: 1606: 1553: 1501: 1452: 1401: 1337: 1283: 1228: 1166: 1109: 1057: 1004: 942: 868: 825: 790: 730: 682: 678: 234: 220:) and thereby promote the ability of these T-cells to organizes an attack on UPS tumor cells. 176: 67: 2976: 2282:"Genomic and transcriptomic characterisation of undifferentiated pleomorphic sarcoma of bone" 3920: 3721: 3654: 3472: 3368: 3306: 3242: 2857: 2847: 2806: 2798: 2757: 2747: 2706: 2698: 2650: 2613: 2603: 2563: 2514: 2471: 2434: 2387: 2350: 2342: 2301: 2293: 2252: 2244: 2192: 2148: 2138: 2097: 2087: 2046: 2038: 1989: 1941: 1897: 1852: 1842: 1796: 1786: 1745: 1737: 1696: 1688: 1647: 1637: 1596: 1588: 1543: 1535: 1491: 1483: 1442: 1432: 1391: 1381: 1327: 1319: 1273: 1265: 1218: 1210: 1156: 1148: 1099: 1049: 994: 984: 934: 860: 817: 780: 772: 722: 686: 655: 619: 578: 474: 377: 362:, i.e. Dickkopf-related protein 1 (elevated in the tumor cells of various cancer types). 133: 301:
prognoses. Increases in the expression of CMTM6 protein were associated in some cases with
3850: 3804: 3759: 3459: 3287: 238: 153: 3739: 3595: 3868: 3822: 3754: 3693: 3689: 3620: 3610: 3528: 3513: 3448: 3359: 3297: 3277: 2862: 2835: 2811: 2786: 2762: 2735: 2711: 2686: 2618: 2591: 2518: 2355: 2330: 2306: 2281: 2257: 2232: 2153: 2126: 2102: 2075: 2051: 2026: 1801: 1774: 1750: 1725: 1701: 1676: 1652: 1625: 1601: 1576: 1548: 1523: 1496: 1471: 1447: 1420: 1396: 1369: 1278: 1253: 1223: 1198: 1161: 1136: 999: 972: 785: 761:"High-grade myofibroblastic sarcoma of the pleura: A case report and literature review" 760: 509:(also termed p63; which is detected with the p40 antibody), and various members of the 327: 2567: 2125:
Chu HY, Chen Z, Wang L, Zhang ZK, Tan X, Liu S, Zhang BT, Lu A, Yu Y, Zhang G (2021).
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and/or cell of origin). WHO classified it as one of the undifferentiated/unclassified
3904: 3701: 2670: 2407: 2027:"Comprehensive and Integrated Genomic Characterization of Adult Soft Tissue Sarcomas" 1961: 1917: 1874: 1349: 1121: 1069: 880: 837: 742: 651: 286: 185: 144:. UPS is considered a diagnosis that defies formal sub-classification after thorough 128:
in the category of tumors of uncertain differentiation. Sarcomas are cancers derived
2964: 2534: 2011: 1370:"Undifferentiated Pleomorphic Sarcoma: Long-Term Follow-Up from a Large Institution" 954: 3638: 3282: 3184: 3101: 3074: 3042: 2491: 2214: 973:"Undifferentiated Pleomorphic Sarcoma with Neoplastic Fever: A Retrospective Study" 635: 586: 582: 561: 524: 201: 197: 2702: 2654: 2197: 2180: 59: 2929: 2346: 938: 864: 560:: Typically occurs in young children, often develops in a preexisting inoperable 3731: 3630: 3468: 3342: 3337: 3237: 3011: 2802: 2439: 2422: 2391: 1592: 627: 623: 611: 607: 599: 547: 528: 484: 460: 443: 438: 242: 179:. UPS had also been regarded as a more aggressive and metastasizing form of the 168: 141: 2940: 2042: 1945: 1901: 1642: 1487: 1053: 821: 3575: 3424: 2852: 2608: 2143: 1104: 1087: 726: 603: 595: 591: 285:
A study of 52 individuals found that their UPS tumor cells expressed on their
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Lee M, Song JS, Hong SM, Jang SJ, Kim J, Song KB, Lee JH, Cho KJ (May 2020).
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Chen S, Huang W, Luo P, Cai W, Yang L, Sun Z, Zheng B, Yan W, Wang C (2019).
1214: 3501: 3444: 3393: 2959: 2913: 2752: 2092: 1791: 1741: 1692: 776: 631: 615: 551: 498: 468: 172: 160: 121: 87: 2871: 2820: 2771: 2720: 2662: 2627: 2575: 2526: 2483: 2448: 2399: 2364: 2315: 2266: 2206: 2162: 2111: 2060: 2003: 1953: 1909: 1866: 1810: 1759: 1710: 1661: 1610: 1557: 1505: 1456: 1405: 1341: 1287: 1232: 1170: 1152: 1137:"The 2020 WHO Classification of Soft Tissue Tumours: news and perspectives" 1113: 1061: 1008: 946: 872: 829: 794: 734: 3873: 3685: 3014: 1847: 1830: 1386: 989: 670: 565: 516: 478: 302: 246: 164: 125: 117: 72: 2896: 1857: 1726:"Sarcoma metastasis to the pancreas: experience at a single institution" 1332: 3097: 3070: 3018: 2248: 510: 213: 2475: 2297: 2076:"Notch Signaling Pathway in Cancer-Review with Bioinformatic Analysis" 1994: 1977: 1323: 425:
gene which encodes Interleukin-7 receptor-α protein (mutations in the
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which in the case of UPS bind to specific antigens on the surface of
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Recent studies have treated UPS by targeting the immune system with
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Alam L, Agrawal K, Kankanala V, Fishberg R, Powell D (April 2020).
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Wang J, Dong S, Zhang J, Gao S, Li Z, Li P, Yuan J, Tian Z (2021).
3420: 666: 520: 406: 310: 289: 2917: 1421:"High-grade myofibroblastic sarcoma in the liver: A case report" 506: 502: 492: 488: 447:
with other genes and is often found in other sarcoma subtypes.)
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gene which encodes lysine N-methyltransferase 2C protein (the
372: 634:; cyclophosphamide, doxorubicin, plus dacarbazine; high dose 2181:"Recurrent TRIO Fusion in Nontranslocation-Related Sarcomas" 132:
that typically develop in bone, muscle, fat, blood vessels,
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examinations fail to identify the type of cells involved.
2127:"Dickkopf-1: A Promising Target for Cancer Immunotherapy" 2074:
Anusewicz D, Orzechowska M, Bednarek AK (February 2021).
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Arora S, Rastogi S, Shamim SA, Barwad A, Sethi M (2020).
550:: At least some tumor cells have features of pleomorphic 523:(i.e. melanoma antigen recognized by T cells 1 protein), 2421:
Watanabe K, Tajino T, Sekiguchi M, Suzuki T (May 2000).
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Arnett AL, Smith TL, Gamez ME, Jhawar SR (August 2021).
120:(i.e., an abnormal growth of cells that have an unclear 3534:
Multiple cutaneous and uterine leiomyomatosis syndrome
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that increased the number of copies of its gene, i.e.
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Wen J, Zhao W, Li C, Shen JY, Wen TF (October 2017).
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Zhao R, Wang J, Zhang H, Chi Y, Bi N (October 2020).
2886: 3882: 3849: 3821: 3803: 3773: 3730: 3709: 3700: 3629: 3566: 3457: 3433: 3411: 3402: 3324: 3296: 3268: 3224: 3183: 3096: 3069: 3060: 3051: 3025: 2950: 2890: 204:. More recently, UPS tumors have been treated with 96: 86: 78: 66: 37: 32: 677:the T-cell-mediated immune responses to them (see 590:cells). A standard chemotherapy two-drug regimen ( 342:, i.e. yes-associated protein 1, a product of the 471:(i.e. high molecular form of caldesmon.) proteins 1934:Journal of Cancer Research and Clinical Oncology 1890:Journal of Cancer Research and Clinical Oncology 1135:Sbaraglia M, Bellan E, Dei Tos AP (April 2021). 535:gene which is detected using an antibody termed 2548: 2546: 2544: 2226: 2224: 2174: 2172: 1824: 1822: 1820: 1730:Journal of Pathology and Translational Medicine 1569: 1567: 1517: 1515: 1363: 1361: 1359: 1305: 1303: 1301: 1299: 1297: 1246: 1244: 1242: 1192: 1190: 1188: 1186: 1184: 1182: 1180: 2025:Abeshouse, Adam; et al. (November 2017). 1081: 1079: 1034: 1032: 1030: 1028: 1026: 1024: 1022: 1020: 1018: 966: 964: 919: 917: 915: 754: 752: 713:Fisher C (September 2004). "Myofibrosarcoma". 708: 706: 704: 702: 3670: 2992: 8: 2231:Zheng B, Qu Y, Wang J, Shi Y, Yan W (2019). 3706: 3677: 3663: 3655: 3408: 3066: 3057: 2999: 2985: 2977: 2887: 554:, i.e. variable shaped immature fat cells. 414:gene is mutated in various cancer types); 46: 29: 2861: 2851: 2810: 2761: 2751: 2710: 2617: 2607: 2438: 2354: 2305: 2256: 2196: 2152: 2142: 2101: 2091: 2050: 1993: 1856: 1846: 1800: 1790: 1749: 1700: 1651: 1641: 1600: 1547: 1495: 1446: 1436: 1395: 1385: 1331: 1277: 1222: 1160: 1103: 998: 988: 784: 58:of undifferentiated pleomorphic sarcoma ( 2507:The American Journal of Dermatopathology 1197:Widemann BC, Italiano A (January 2018). 1932:undifferentiated pleomorphic sarcoma". 698: 558:Malignant peripheral nerve sheath tumor 190:fibroblastic and myofibroblastic tumors 2427:American Journal of Clinical Pathology 346:gene, in the Hippo signaling pathway; 2329:Fagan RJ, Dingwall AK (August 2019). 1042:European Journal of Surgical Oncology 927:American Journal of Clinical Oncology 7: 370:Deletion and/or inactivation or the 107:Undifferentiated pleomorphic sarcoma 33:Undifferentiated pleomorphic sarcoma 3038:Desmoplastic small-round-cell tumor 1470:Oh SJ, Yeom SY, Kim KH (May 2013). 602:alone or combined with ifosfamide, 2519:10.1097/01.dad.0000249888.41884.6c 654:. Pembrolizumab is a manufactured 384:deletions and/or mutations in the 181:low-grade myofibroblastic sarcomas 25: 3710:Glandular and epithelial neoplasm 3115:Aggressive infantile fibromatosis 2380:Advances in Biological Regulation 1773:Xu G, Shi X, Shao G (June 2013). 1476:Journal of Korean Medical Science 1425:World Journal of Gastroenterology 3750:Clear-cell sarcoma of the kidney 3606:Clear-cell sarcoma of the kidney 2237:Cancer Genomics & Proteomics 3911:Dermal and subcutaneous growths 3374:Intradermal spindle cell lipoma 3083:Dermatofibrosarcoma protuberans 1982:International Journal of Cancer 1779:Journal of Medical Case Reports 659:therapeutic monoclonal antibody 501:(i.e. grade 3 or 4 carcinoma): 499:Poorly differentiated carcinoma 245:near the primary tumor, brain, 18:Histiocytoma, malignant fibrous 3890:Malignant fibrous histiocytoma 3202:Malignant fibrous histiocytoma 3150:Infantile digital fibromatosis 3135:Familial myxovascular fibromas 3130:Diffuse infantile fibromatosis 2464:Journal of Cutaneous Pathology 1374:Cancer Management and Research 977:Cancer Management and Research 853:Advances in Anatomic Pathology 332:vestigial-like family member 3 1: 3248:Superficial acral fibromyxoma 3155:Juvenile hyaline fibromatosis 2703:10.1158/1078-0432.CCR-19-1824 2655:10.1080/14740338.2020.1736554 2643:Expert Opinion on Drug Safety 2568:10.1016/S1470-2045(17)30334-0 2198:10.1158/1078-0432.CCR-16-0290 1270:10.1016/S1470-2045(17)30624-1 665:. This inhibition blocks the 3549:Solitary cutaneous leiomyoma 3539:Multiple cutaneous leiomyoma 3260:Ossifying fibromyxoid tumour 2347:10.1016/j.canlet.2019.05.024 1203:Journal of Clinical Oncology 939:10.1097/COC.0000000000000496 865:10.1097/PAP.0000000000000284 485:Defifferentiated liposarcoma 475:Pleomorphic rhabdomyosarcoma 431:acute lymphoblastic leukemia 274:(a diagnosis reached by the 171:(cells descended from blood 3916:Infectious causes of cancer 3859:Transitional cell carcinoma 3831:Transitional cell carcinoma 3197:Benign fibrous histiocytoma 2803:10.1001/jamaoncol.2017.1617 2440:10.1309/jnqx-f4km-q0q0-7xk8 2392:10.1016/j.jbior.2021.100788 1593:10.1016/j.ebiom.2020.103131 1086:Hornick JL (January 2020). 568:(i.e. dead or dying cells). 429:gene are commonly found in 3937: 3786:Juxtaglomerular cell tumor 3477:Embryonal rhabdomyosarcoma 3053:Connective tissue neoplasm 2043:10.1016/j.cell.2017.10.014 1946:10.1007/s00432-021-03616-4 1902:10.1007/s00432-021-03835-9 1643:10.1186/s13569-020-00133-9 1488:10.3346/jkms.2013.28.5.717 1054:10.1016/j.ejso.2020.02.029 822:10.1016/j.crad.2021.08.011 334:protein, a product of the 3781:Renal medullary carcinoma 3601:Malignant rhabdoid tumour 3568:Complex mixed and stromal 3497: 3489:Alveolar rhabdomyosarcoma 2853:10.1186/s12885-018-4829-0 2609:10.1186/s12916-017-0831-7 2144:10.3389/fimmu.2021.658097 1630:Clinical Sarcoma Research 1105:10.1038/s41379-019-0388-4 899:National Cancer Institute 727:10.1007/s00428-004-1038-9 461:Pleomorphic leimyosarcoma 282:(see Diagnosis section). 54: 45: 3140:Fibroma of tendon sheath 2691:Clinical Cancer Research 2286:The Journal of Pathology 2185:Clinical Cancer Research 1438:10.3748/wjg.v23.i38.7054 1215:10.1200/JCO.2017.75.3467 352:notch signaling pathways 237:of the lung, heart, and 3864:Squamous-cell carcinoma 3836:Squamous-cell carcinoma 3791:Renal medullary fibroma 3170:Oral submucous fibrosis 3110:Aggressive fibromatosis 3026:Not otherwise specified 2753:10.3390/cancers12071873 2131:Frontiers in Immunology 2093:10.3390/cancers13040768 1792:10.1186/1752-1947-7-165 1742:10.4132/jptm.2020.03.04 1693:10.1136/bcr-2021-242342 777:10.1111/1759-7714.13613 573:Treatment and prognosis 548:Pleomorphic liposarcoma 350:Abnormal activation of 324:Hippo signaling pathway 259:paraneoplastic syndrome 3212:Solitary fibrous tumor 3207:Atypical fibroxanthoma 1312:ANZ Journal of Surgery 1153:10.32074/1591-951X-213 816:(12): 940.e1–940.e16. 562:plexiform neurofibroma 276:process of elimination 272:diagnosis of exclusion 130:mesenchymal stem cells 3586:Mixed MĂŒllerian tumor 322:Amplification of the 231:retroperitoneal space 3745:Mesoblastic nephroma 3717:Renal cell carcinoma 3591:Mesoblastic nephroma 3160:Plantar fibromatosis 3088:Desmoplastic fibroma 2556:The Lancet. Oncology 1848:10.3892/or.2020.7837 1387:10.2147/CMAR.S226896 1258:The Lancet. Oncology 990:10.2147/CMAR.S339278 626:, doxorubicin, plus 3765:Metanephric adenoma 3581:Pleomorphic adenoma 3389:Spindle cell lipoma 3189:histiocytic sarcoma 3165:Pleomorphic fibroma 3125:Collagenous fibroma 3120:Aponeurotic fibroma 3033:Soft-tissue sarcoma 1528:Cardiology Research 513:family of proteins. 505:(also termed EMA), 326:, an intracellular 175:), but rather from 150:immunohistochemical 3841:Inverted papilloma 3544:Neural fibrolipoma 3519:Angiolipoleiomyoma 3482:Sarcoma botryoides 3379:Pleomorphic lipoma 3348:Myxoid liposarcoma 3312:Clear-cell sarcoma 3145:Fibromatosis colli 2951:External resources 2249:10.21873/cgp.20127 2037:(4): 950–965.e28. 1098:(Suppl 1): 56–65. 810:Clinical Radiology 531:(a product of the 358:Overexpression of 3898: 3897: 3813:Ureteral neoplasm 3799: 3798: 3652: 3651: 3644:Adenomatoid tumor 3616:Pancreatoblastoma 3562: 3561: 3524:Genital leiomyoma 3384:Lipoblastomatosis 3320: 3319: 3220: 3219: 3175:Pachydermodactyly 2974: 2973: 2476:10.1111/cup.12388 2298:10.1002/path.5176 1995:10.1002/ijc.31903 1431:(38): 7054–7058. 1324:10.1111/ans.15348 1264:(11): 1493–1501. 895:"What Is Cancer?" 771:(10): 3011–3014. 683:complete response 679:Immune checkpoint 404:mutations in the 394:mutations in the 287:surface membranes 177:mesenchymal cells 134:lymphatic vessels 104: 103: 27:Medical condition 16:(Redirected from 3928: 3722:Renal oncocytoma 3707: 3679: 3672: 3665: 3656: 3473:rhabdomyosarcoma 3462: 3438: 3414: 3409: 3369:Chondroid lipoma 3307:Synovial sarcoma 3243:Cutaneous myxoma 3067: 3058: 3001: 2994: 2987: 2978: 2888: 2876: 2875: 2865: 2855: 2831: 2825: 2824: 2814: 2782: 2776: 2775: 2765: 2755: 2731: 2725: 2724: 2714: 2697:(6): 1258–1266. 2681: 2675: 2674: 2638: 2632: 2631: 2621: 2611: 2586: 2580: 2579: 2550: 2539: 2538: 2502: 2496: 2495: 2459: 2453: 2452: 2442: 2418: 2412: 2411: 2375: 2369: 2368: 2358: 2326: 2320: 2319: 2309: 2277: 2271: 2270: 2260: 2228: 2219: 2218: 2200: 2176: 2167: 2166: 2156: 2146: 2122: 2116: 2115: 2105: 2095: 2071: 2065: 2064: 2054: 2022: 2016: 2015: 1997: 1972: 1966: 1965: 1940:(7): 2003–2011. 1928: 1922: 1921: 1885: 1879: 1878: 1860: 1850: 1835:Oncology Reports 1826: 1815: 1814: 1804: 1794: 1770: 1764: 1763: 1753: 1721: 1715: 1714: 1704: 1681:BMJ Case Reports 1672: 1666: 1665: 1655: 1645: 1621: 1615: 1614: 1604: 1571: 1562: 1561: 1551: 1519: 1510: 1509: 1499: 1467: 1461: 1460: 1450: 1440: 1416: 1410: 1409: 1399: 1389: 1365: 1354: 1353: 1335: 1318:(9): 1045–1050. 1307: 1292: 1291: 1281: 1248: 1237: 1236: 1226: 1194: 1175: 1174: 1164: 1132: 1126: 1125: 1107: 1092:Modern Pathology 1083: 1074: 1073: 1048:(7): 1287–1293. 1036: 1013: 1012: 1002: 992: 968: 959: 958: 921: 910: 909: 907: 906: 891: 885: 884: 848: 842: 841: 805: 799: 798: 788: 756: 747: 746: 710: 687:cyclophosphamide 620:cyclophosphamide 579:Adjuvant therapy 437:expression of a 378:tumor suppressor 253:Neoplastic fever 206:antibody therapy 50: 30: 21: 3936: 3935: 3931: 3930: 3929: 3927: 3926: 3925: 3901: 3900: 3899: 3894: 3878: 3845: 3817: 3795: 3769: 3760:Cystic nephroma 3726: 3696: 3694:genital systems 3683: 3653: 3648: 3625: 3558: 3493: 3460:Skeletal muscle 3458: 3453: 3434: 3429: 3412: 3398: 3316: 3292: 3288:Phyllodes tumor 3270:Fibroepithelial 3264: 3216: 3179: 3092: 3047: 3021: 3005: 2975: 2970: 2969: 2946: 2945: 2899: 2885: 2880: 2879: 2833: 2832: 2828: 2784: 2783: 2779: 2733: 2732: 2728: 2683: 2682: 2678: 2640: 2639: 2635: 2588: 2587: 2583: 2552: 2551: 2542: 2504: 2503: 2499: 2461: 2460: 2456: 2420: 2419: 2415: 2377: 2376: 2372: 2328: 2327: 2323: 2279: 2278: 2274: 2230: 2229: 2222: 2178: 2177: 2170: 2124: 2123: 2119: 2073: 2072: 2068: 2024: 2023: 2019: 1974: 1973: 1969: 1930: 1929: 1925: 1887: 1886: 1882: 1828: 1827: 1818: 1772: 1771: 1767: 1723: 1722: 1718: 1674: 1673: 1669: 1623: 1622: 1618: 1573: 1572: 1565: 1540:10.14740/cr1029 1521: 1520: 1513: 1469: 1468: 1464: 1418: 1417: 1413: 1380:: 10001–10009. 1367: 1366: 1357: 1309: 1308: 1295: 1250: 1249: 1240: 1196: 1195: 1178: 1134: 1133: 1129: 1085: 1084: 1077: 1038: 1037: 1016: 970: 969: 962: 923: 922: 913: 904: 902: 893: 892: 888: 850: 849: 845: 807: 806: 802: 765:Thoracic Cancer 758: 757: 750: 715:Virchows Archiv 712: 711: 700: 695: 648: 575: 453: 268: 255: 239:small intestine 226: 154:ultrastructural 28: 23: 22: 15: 12: 11: 5: 3934: 3932: 3924: 3923: 3918: 3913: 3903: 3902: 3896: 3895: 3893: 3892: 3886: 3884: 3880: 3879: 3877: 3876: 3871: 3869:Adenocarcinoma 3866: 3861: 3855: 3853: 3847: 3846: 3844: 3843: 3838: 3833: 3827: 3825: 3819: 3818: 3816: 3815: 3809: 3807: 3801: 3800: 3797: 3796: 3794: 3793: 3788: 3783: 3777: 3775: 3771: 3770: 3768: 3767: 3762: 3757: 3755:Angiomyolipoma 3752: 3747: 3742: 3736: 3734: 3728: 3727: 3725: 3724: 3719: 3713: 3711: 3704: 3698: 3697: 3684: 3682: 3681: 3674: 3667: 3659: 3650: 3649: 3647: 3646: 3641: 3635: 3633: 3627: 3626: 3624: 3623: 3621:Carcinosarcoma 3618: 3613: 3611:Hepatoblastoma 3608: 3603: 3598: 3593: 3588: 3583: 3578: 3572: 3570: 3564: 3563: 3560: 3559: 3557: 3556: 3551: 3546: 3541: 3536: 3531: 3529:Leiomyosarcoma 3526: 3521: 3516: 3514:Angioleiomyoma 3511: 3510: 3509: 3498: 3495: 3494: 3492: 3491: 3486: 3485: 3484: 3465: 3463: 3455: 3454: 3452: 3451: 3449:leiomyosarcoma 3441: 3439: 3431: 3430: 3428: 3427: 3417: 3415: 3406: 3400: 3399: 3397: 3396: 3391: 3386: 3381: 3376: 3371: 3365: 3364: 3363: 3362: 3360:Angiomyolipoma 3352: 3351: 3350: 3345: 3330: 3328: 3322: 3321: 3318: 3317: 3315: 3314: 3309: 3303: 3301: 3294: 3293: 3291: 3290: 3285: 3280: 3278:Brenner tumour 3274: 3272: 3266: 3265: 3263: 3262: 3257: 3252: 3251: 3250: 3245: 3230: 3228: 3222: 3221: 3218: 3217: 3215: 3214: 3209: 3204: 3199: 3193: 3191: 3181: 3180: 3178: 3177: 3172: 3167: 3162: 3157: 3152: 3147: 3142: 3137: 3132: 3127: 3122: 3117: 3112: 3106: 3104: 3094: 3093: 3091: 3090: 3085: 3079: 3077: 3064: 3055: 3049: 3048: 3046: 3045: 3040: 3035: 3029: 3027: 3023: 3022: 3006: 3004: 3003: 2996: 2989: 2981: 2972: 2971: 2968: 2967: 2955: 2954: 2952: 2948: 2947: 2944: 2943: 2932: 2921: 2900: 2895: 2894: 2892: 2891:Classification 2884: 2883:External links 2881: 2878: 2877: 2826: 2777: 2726: 2676: 2649:(3): 233–242. 2633: 2581: 2562:(6): 812–822. 2540: 2497: 2470:(11): 839–45. 2454: 2413: 2370: 2335:Cancer Letters 2321: 2292:(2): 166–176. 2272: 2243:(3): 221–228. 2220: 2191:(3): 857–867. 2168: 2117: 2066: 2017: 1988:(4): 859–867. 1967: 1923: 1880: 1841:(1): 379–389. 1816: 1765: 1736:(3): 220–227. 1716: 1687:(8): e242342. 1667: 1616: 1563: 1534:(2): 129–133. 1511: 1462: 1411: 1355: 1293: 1238: 1209:(2): 160–167. 1176: 1127: 1075: 1014: 960: 933:(2): 166–171. 911: 886: 843: 800: 748: 697: 696: 694: 691: 647: 644: 574: 571: 570: 569: 555: 541: 540: 514: 496: 482: 472: 452: 449: 328:cell signaling 267: 264: 254: 251: 249:, and heart. 225: 222: 115:differentiated 102: 101: 98: 94: 93: 90: 84: 83: 80: 76: 75: 70: 64: 63: 52: 51: 43: 42: 39: 35: 34: 26: 24: 14: 13: 10: 9: 6: 4: 3: 2: 3933: 3922: 3919: 3917: 3914: 3912: 3909: 3908: 3906: 3891: 3888: 3887: 3885: 3881: 3875: 3872: 3870: 3867: 3865: 3862: 3860: 3857: 3856: 3854: 3852: 3848: 3842: 3839: 3837: 3834: 3832: 3829: 3828: 3826: 3824: 3820: 3814: 3811: 3810: 3808: 3806: 3802: 3792: 3789: 3787: 3784: 3782: 3779: 3778: 3776: 3772: 3766: 3763: 3761: 3758: 3756: 3753: 3751: 3748: 3746: 3743: 3741: 3738: 3737: 3735: 3733: 3729: 3723: 3720: 3718: 3715: 3714: 3712: 3708: 3705: 3703: 3699: 3695: 3691: 3687: 3680: 3675: 3673: 3668: 3666: 3661: 3660: 3657: 3645: 3642: 3640: 3637: 3636: 3634: 3632: 3628: 3622: 3619: 3617: 3614: 3612: 3609: 3607: 3604: 3602: 3599: 3597: 3594: 3592: 3589: 3587: 3584: 3582: 3579: 3577: 3574: 3573: 3571: 3569: 3565: 3555: 3552: 3550: 3547: 3545: 3542: 3540: 3537: 3535: 3532: 3530: 3527: 3525: 3522: 3520: 3517: 3515: 3512: 3508: 3505: 3504: 3503: 3500: 3499: 3496: 3490: 3487: 3483: 3480: 3479: 3478: 3474: 3470: 3467: 3466: 3464: 3461: 3456: 3450: 3446: 3443: 3442: 3440: 3437: 3436:Smooth muscle 3432: 3426: 3422: 3419: 3418: 3416: 3410: 3407: 3405: 3401: 3395: 3392: 3390: 3387: 3385: 3382: 3380: 3377: 3375: 3372: 3370: 3367: 3366: 3361: 3358: 3357: 3356: 3353: 3349: 3346: 3344: 3341: 3340: 3339: 3335: 3332: 3331: 3329: 3327: 3323: 3313: 3310: 3308: 3305: 3304: 3302: 3299: 3295: 3289: 3286: 3284: 3281: 3279: 3276: 3275: 3273: 3271: 3267: 3261: 3258: 3256: 3253: 3249: 3246: 3244: 3241: 3240: 3239: 3235: 3232: 3231: 3229: 3227: 3223: 3213: 3210: 3208: 3205: 3203: 3200: 3198: 3195: 3194: 3192: 3190: 3186: 3182: 3176: 3173: 3171: 3168: 3166: 3163: 3161: 3158: 3156: 3153: 3151: 3148: 3146: 3143: 3141: 3138: 3136: 3133: 3131: 3128: 3126: 3123: 3121: 3118: 3116: 3113: 3111: 3108: 3107: 3105: 3103: 3099: 3095: 3089: 3086: 3084: 3081: 3080: 3078: 3076: 3072: 3068: 3065: 3063: 3059: 3056: 3054: 3050: 3044: 3041: 3039: 3036: 3034: 3031: 3030: 3028: 3024: 3020: 3016: 3013: 3009: 3002: 2997: 2995: 2990: 2988: 2983: 2982: 2979: 2966: 2962: 2961: 2957: 2956: 2953: 2949: 2942: 2938: 2937: 2933: 2931: 2927: 2926: 2922: 2919: 2915: 2911: 2910: 2906: 2902: 2901: 2898: 2893: 2889: 2882: 2873: 2869: 2864: 2859: 2854: 2849: 2845: 2841: 2837: 2830: 2827: 2822: 2818: 2813: 2808: 2804: 2800: 2796: 2792: 2791:JAMA Oncology 2788: 2781: 2778: 2773: 2769: 2764: 2759: 2754: 2749: 2745: 2741: 2737: 2730: 2727: 2722: 2718: 2713: 2708: 2704: 2700: 2696: 2692: 2688: 2680: 2677: 2672: 2668: 2664: 2660: 2656: 2652: 2648: 2644: 2637: 2634: 2629: 2625: 2620: 2615: 2610: 2605: 2601: 2597: 2593: 2585: 2582: 2577: 2573: 2569: 2565: 2561: 2557: 2549: 2547: 2545: 2541: 2536: 2532: 2528: 2524: 2520: 2516: 2512: 2508: 2501: 2498: 2493: 2489: 2485: 2481: 2477: 2473: 2469: 2465: 2458: 2455: 2450: 2446: 2441: 2436: 2432: 2428: 2424: 2417: 2414: 2409: 2405: 2401: 2397: 2393: 2389: 2385: 2381: 2374: 2371: 2366: 2362: 2357: 2352: 2348: 2344: 2340: 2336: 2332: 2325: 2322: 2317: 2313: 2308: 2303: 2299: 2295: 2291: 2287: 2283: 2276: 2273: 2268: 2264: 2259: 2254: 2250: 2246: 2242: 2238: 2234: 2227: 2225: 2221: 2216: 2212: 2208: 2204: 2199: 2194: 2190: 2186: 2182: 2175: 2173: 2169: 2164: 2160: 2155: 2150: 2145: 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1412: 1407: 1403: 1398: 1393: 1388: 1383: 1379: 1375: 1371: 1364: 1362: 1360: 1356: 1351: 1347: 1343: 1339: 1334: 1329: 1325: 1321: 1317: 1313: 1306: 1304: 1302: 1300: 1298: 1294: 1289: 1285: 1280: 1275: 1271: 1267: 1263: 1259: 1255: 1247: 1245: 1243: 1239: 1234: 1230: 1225: 1220: 1216: 1212: 1208: 1204: 1200: 1193: 1191: 1189: 1187: 1185: 1183: 1181: 1177: 1172: 1168: 1163: 1158: 1154: 1150: 1146: 1142: 1138: 1131: 1128: 1123: 1119: 1115: 1111: 1106: 1101: 1097: 1093: 1089: 1082: 1080: 1076: 1071: 1067: 1063: 1059: 1055: 1051: 1047: 1043: 1035: 1033: 1031: 1029: 1027: 1025: 1023: 1021: 1019: 1015: 1010: 1006: 1001: 996: 991: 986: 983:: 8481–8487. 982: 978: 974: 967: 965: 961: 956: 952: 948: 944: 940: 936: 932: 928: 920: 918: 916: 912: 900: 896: 890: 887: 882: 878: 874: 870: 866: 862: 858: 854: 847: 844: 839: 835: 831: 827: 823: 819: 815: 811: 804: 801: 796: 792: 787: 782: 778: 774: 770: 766: 762: 755: 753: 749: 744: 740: 736: 732: 728: 724: 721:(3): 215–23. 720: 716: 709: 707: 705: 703: 699: 692: 690: 688: 684: 680: 675: 672: 668: 664: 660: 657: 653: 652:pembrolizumab 646:Immunotherapy 645: 643: 639: 637: 633: 629: 625: 621: 617: 613: 609: 605: 601: 597: 593: 588: 584: 580: 572: 567: 563: 559: 556: 553: 549: 546: 545: 544: 538: 534: 530: 526: 522: 518: 515: 512: 508: 504: 500: 497: 494: 490: 486: 483: 480: 477:: Desmin and 476: 473: 470: 466: 462: 459: 458: 457: 450: 448: 446: 445: 440: 436: 432: 428: 424: 420: 419:amplification 417: 413: 409: 408: 403: 399: 398: 393: 389: 388: 383: 379: 375: 374: 369: 363: 361: 357: 353: 349: 345: 341: 337: 333: 329: 325: 321: 316: 312: 308: 304: 299: 295: 291: 288: 283: 281: 277: 273: 265: 263: 260: 252: 250: 248: 244: 240: 236: 232: 223: 221: 219: 215: 211: 207: 203: 199: 194: 191: 187: 186:metastasizing 182: 178: 174: 170: 166: 162: 157: 155: 151: 147: 143: 139: 135: 131: 127: 123: 119: 116: 112: 108: 99: 95: 91: 89: 85: 81: 77: 74: 71: 69: 65: 61: 60:H&E stain 57: 53: 49: 44: 40: 36: 31: 19: 3740:Wilms' tumor 3639:Mesothelioma 3596:Wilms' tumor 3283:Fibroadenoma 3185:Histiocytoma 3102:fibromatosis 3075:fibrosarcoma 3043:Skin sarcoma 2958: 2934: 2923: 2903: 2843: 2839: 2829: 2797:(1): 93–97. 2794: 2790: 2780: 2743: 2739: 2729: 2694: 2690: 2679: 2646: 2642: 2636: 2599: 2596:BMC Medicine 2595: 2584: 2559: 2555: 2510: 2506: 2500: 2467: 2463: 2457: 2433:(5): 663–8. 2430: 2426: 2416: 2383: 2379: 2373: 2338: 2334: 2324: 2289: 2285: 2275: 2240: 2236: 2188: 2184: 2134: 2130: 2120: 2083: 2079: 2069: 2034: 2030: 2020: 1985: 1981: 1970: 1937: 1933: 1926: 1896:(1): 47–56. 1893: 1889: 1883: 1858:2324/4474987 1838: 1834: 1782: 1778: 1768: 1733: 1729: 1719: 1684: 1680: 1670: 1633: 1629: 1619: 1584: 1581:eBioMedicine 1580: 1531: 1527: 1479: 1475: 1465: 1428: 1424: 1414: 1377: 1373: 1333:11343/286224 1315: 1311: 1261: 1257: 1206: 1202: 1147:(2): 70–84. 1144: 1140: 1130: 1095: 1091: 1045: 1041: 980: 976: 930: 926: 903:. 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303:mutations 270:UPS is a 266:Pathology 233:, liver, 200:, and/or 173:monocytes 165:melanomas 97:Frequency 88:Prognosis 68:Specialty 3874:Melanoma 3298:Synovial 3019:sarcomas 2920:C49.M10) 2872:30249211 2821:28662235 2772:32664595 2721:31900276 2663:32129104 2628:28391775 2576:28499583 2535:20167145 2527:17284958 2484:25263848 2449:10800398 2400:33578108 2365:31128216 2316:30281149 2267:31018952 2207:27528700 2163:34093545 2112:33673145 2061:29100075 2012:52883298 2004:30267407 1954:33811537 1910:34783871 1867:33155664 1811:23805953 1760:32311873 1711:34400423 1662:32670543 1611:33254023 1558:32256920 1506:23678263 1457:29097878 1406:31819633 1342:31364245 1288:28988646 1233:29220302 1171:33179614 1114:31653978 1062:32127249 1009:34795527 955:56178938 947:30557163 873:32960834 830:34607656 795:32815307 735:15173943 566:necrosis 517:Melanoma 479:myogenin 247:pancreas 126:sarcomas 122:identity 118:neoplasm 73:Oncology 3921:Sarcoma 3851:Urethra 3823:Bladder 3690:urinary 3688:of the 3413:General 3098:Fibroma 3071:Fibroma 2930:D051677 2863:6154892 2812:5833654 2763:7408640 2740:Cancers 2712:7731262 2619:5385590 2492:9053425 2356:6638576 2307:8033574 2258:6542646 2215:3756473 2154:8174842 2103:7918426 2080:Cancers 2052:5693358 1802:3750226 1785:: 165. 1751:7253956 1702:8378830 1653:7346343 1602:7708794 1549:7092767 1497:3653084 1448:5658323 1397:6885560 1279:7939029 1224:5759316 1162:8167394 1000:8592396 786:7529570 674:ligands 663:T-cells 585:or/and 511:keratin 421:of the 214:T-cells 208:, i.e. 92:Guarded 82:Unknown 3805:Ureter 3702:Kidney 3686:Tumors 3355:PEComa 3334:Lipoma 3234:Myxoma 3015:tumors 2870:  2860:  2819:  2809:  2770:  2760:  2719:  2709:  2669:  2661:  2626:  2616:  2574:  2533:  2525:  2490:  2482:  2447:  2406:  2398:  2363:  2353:  2314:  2304:  2265:  2255:  2213:  2205:  2161:  2151:  2110:  2100:  2059:  2049:  2010:  2002:  1960:  1952:  1916:  1908:  1873:  1865:  1809:  1799:  1758:  1748:  1709:  1699:  1660:  1650:  1636:: 10. 1609:  1599:  1556:  1546:  1504:  1494:  1455:  1445:  1404:  1394:  1348:  1340:  1286:  1276:  1231:  1221:  1169:  1159:  1120:  1112:  1068:  1060:  1007:  997:  953:  945:  879:  871:  836:  828:  793:  783:  741:  733:  537:HMB-45 527:, and 465:Desmin 433:) and 235:pleura 152:, and 140:, and 79:Causes 3883:Other 3554:STUMP 3421:Myoma 3300:-like 2941:31471 2667:S2CID 2531:S2CID 2488:S2CID 2404:S2CID 2211:S2CID 2008:S2CID 1958:S2CID 1914:S2CID 1871:S2CID 1346:S2CID 1118:S2CID 1066:S2CID 951:S2CID 877:S2CID 834:S2CID 739:S2CID 671:PL-L2 667:PD-L1 614:, or 594:plus 521:MLANA 412:KMT2C 407:KMT2C 336:VGLL3 315:AMPD2 311:AMPD2 307:CMTM6 298:CMTM6 290:PD-L1 3692:and 3017:and 2925:MeSH 2918:ILDS 2868:PMID 2817:PMID 2768:PMID 2717:PMID 2659:PMID 2624:PMID 2572:PMID 2523:PMID 2480:PMID 2445:PMID 2396:PMID 2361:PMID 2312:PMID 2263:PMID 2203:PMID 2159:PMID 2108:PMID 2057:PMID 2031:Cell 2000:PMID 1950:PMID 1906:PMID 1863:PMID 1807:PMID 1756:PMID 1707:PMID 1658:PMID 1607:PMID 1554:PMID 1502:PMID 1453:PMID 1402:PMID 1338:PMID 1284:PMID 1229:PMID 1167:PMID 1110:PMID 1058:PMID 1005:PMID 943:PMID 869:PMID 826:PMID 791:PMID 731:PMID 669:and 533:PMEL 529:PMEL 525:S100 507:TP63 503:MUC1 493:CDK4 491:and 489:MDM2 467:and 444:TRIO 427:IL7R 423:IL7R 397:ATRX 387:TP53 360:DKK1 344:YAP1 340:YAP1 294:IDO1 163:and 100:Rare 2914:C49 2905:ICD 2858:PMC 2848:doi 2807:PMC 2799:doi 2758:PMC 2748:doi 2707:PMC 2699:doi 2651:doi 2614:PMC 2604:doi 2564:doi 2515:doi 2472:doi 2435:doi 2431:113 2388:doi 2351:PMC 2343:doi 2339:458 2302:PMC 2294:doi 2290:247 2253:PMC 2245:doi 2193:doi 2149:PMC 2139:doi 2098:PMC 2088:doi 2047:PMC 2039:doi 2035:171 1990:doi 1986:144 1942:doi 1938:147 1898:doi 1894:148 1853:hdl 1843:doi 1797:PMC 1787:doi 1746:PMC 1738:doi 1697:PMC 1689:doi 1648:PMC 1638:doi 1597:PMC 1589:doi 1544:PMC 1536:doi 1492:PMC 1484:doi 1443:PMC 1433:doi 1392:PMC 1382:doi 1328:hdl 1320:doi 1274:PMC 1266:doi 1219:PMC 1211:doi 1157:PMC 1149:doi 1145:113 1100:doi 1050:doi 995:PMC 985:doi 935:doi 861:doi 818:doi 781:PMC 773:doi 723:doi 719:445 656:IG4 630:or 618:; 373:RB1 111:UPS 3907:: 3475:: 2963:: 2939:: 2928:: 2912:: 2909:10 2866:. 2856:. 2844:18 2842:. 2838:. 2815:. 2805:. 2793:. 2789:. 2766:. 2756:. 2744:12 2742:. 2738:. 2715:. 2705:. 2695:26 2693:. 2689:. 2665:. 2657:. 2647:19 2645:. 2622:. 2612:. 2600:15 2598:. 2594:. 2570:. 2560:18 2558:. 2543:^ 2529:. 2521:. 2511:29 2509:. 2486:. 2478:. 2468:41 2466:. 2443:. 2429:. 2425:. 2402:. 2394:. 2384:80 2382:. 2359:. 2349:. 2337:. 2333:. 2310:. 2300:. 2288:. 2284:. 2261:. 2251:. 2241:16 2239:. 2235:. 2223:^ 2209:. 2201:. 2189:23 2187:. 2183:. 2171:^ 2157:. 2147:. 2135:12 2133:. 2129:. 2106:. 2096:. 2084:13 2082:. 2078:. 2055:. 2045:. 2033:. 2029:. 2006:. 1998:. 1984:. 1980:. 1956:. 1948:. 1936:. 1912:. 1904:. 1892:. 1869:. 1861:. 1851:. 1839:45 1837:. 1833:. 1819:^ 1805:. 1795:. 1781:. 1777:. 1754:. 1744:. 1734:54 1732:. 1728:. 1705:. 1695:. 1685:14 1683:. 1679:. 1656:. 1646:. 1634:10 1632:. 1628:. 1605:. 1595:. 1585:62 1583:. 1579:. 1566:^ 1552:. 1542:. 1532:11 1530:. 1526:. 1514:^ 1500:. 1490:. 1480:28 1478:. 1474:. 1451:. 1441:. 1429:23 1427:. 1423:. 1400:. 1390:. 1378:11 1376:. 1372:. 1358:^ 1344:. 1336:. 1326:. 1316:89 1314:. 1296:^ 1282:. 1272:. 1262:18 1260:. 1256:. 1241:^ 1227:. 1217:. 1207:36 1205:. 1201:. 1179:^ 1165:. 1155:. 1143:. 1139:. 1116:. 1108:. 1096:33 1094:. 1090:. 1078:^ 1064:. 1056:. 1046:46 1044:. 1017:^ 1003:. 993:. 981:13 979:. 975:. 963:^ 949:. 941:. 931:42 929:. 914:^ 897:. 875:. 867:. 857:28 855:. 832:. 824:. 814:76 812:. 789:. 779:. 769:11 767:. 763:. 751:^ 737:. 729:. 717:. 701:^ 622:, 610:, 606:, 519:: 487:: 463:: 435:6) 416:5) 402:4) 392:3) 382:2) 368:1) 356:3) 348:2) 320:1) 188:) 148:, 3678:e 3671:t 3664:v 3471:/ 3447:/ 3423:/ 3336:/ 3236:/ 3187:/ 3100:/ 3073:/ 3010:/ 3000:e 2993:t 2986:v 2916:( 2907:- 2897:D 2874:. 2850:: 2823:. 2801:: 2795:4 2774:. 2750:: 2723:. 2701:: 2673:. 2653:: 2630:. 2606:: 2578:. 2566:: 2537:. 2517:: 2494:. 2474:: 2451:. 2437:: 2410:. 2390:: 2367:. 2345:: 2318:. 2296:: 2269:. 2247:: 2217:. 2195:: 2165:. 2141:: 2114:. 2090:: 2063:. 2041:: 2014:. 1992:: 1964:. 1944:: 1920:. 1900:: 1877:. 1855:: 1845:: 1813:. 1789:: 1783:7 1762:. 1740:: 1713:. 1691:: 1664:. 1640:: 1613:. 1591:: 1560:. 1538:: 1508:. 1486:: 1459:. 1435:: 1408:. 1384:: 1352:. 1330:: 1322:: 1290:. 1268:: 1235:. 1213:: 1173:. 1151:: 1124:. 1102:: 1072:. 1052:: 1011:. 987:: 957:. 937:: 908:. 883:. 863:: 840:. 820:: 797:. 775:: 745:. 725:: 109:( 62:) 20:)

Index

Histiocytoma, malignant fibrous

Micrograph
H&E stain
Specialty
Oncology
Prognosis
differentiated
neoplasm
identity
sarcomas
mesenchymal stem cells
lymphatic vessels
histiocytes
giant cells
histologic
immunohistochemical
ultrastructural
carcinomas
melanomas
histiocytes
monocytes
mesenchymal cells
low-grade myofibroblastic sarcomas
metastasizing
fibroblastic and myofibroblastic tumors
radiotherapy
chemotherapy
antibody therapy
antibodies

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