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Infantile myofibromatosis

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294:. Among 36 identified familial carriers of these mutant genes, 4 had no history of IMF, 3 had developed a solitary IMF, 29 had developed multiple tumors (no viscera involvement) or multiple tumors (with viscera involvement) IMF, and 2 had died. Most of these individuals were diagnosed with IMF in their infancy or early childhood. Subsequent studies have found that up to 70% of individuals with IMF and no family history of the disease also carry mutations in one of their two PDGFRB genes in the cells of their tumor tissues. These mutations are 412:
typically due to a tumor's compression of vital organs or tissues (e.g. blood vessels). However, there are uncommon cases of multiple tumors (with viscera involvement) that were not associated with serious symptoms, were observed without treatment, and over time showed spontaneous regressions of all or almost all of their tumors.
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spontaneiously. Single tumors located in vital areas (e.g. intracranial tumors) and tumors that do not regress over suitable observation periods are often treated by surgical removal. Multiple tumors (with viscera involvement) and surgically inaccessible life-threatening IMF tumors have been treated with one or a combination of
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Individuals with multiple tumors (with viscera involvement) have numerous tumors one or more of which are located in and often injure a vital internal organ and may be life-threatening. Cases in which one of the tumors is in a vital organ and seriously symptomatic have been successfully treated with
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Wenger TL, Bly RA, Wu N, Albert CM, Park J, Shieh J, Chenbhanich J, Heike CL, Adam MP, Chang I, Sun A, Miller DE, Beck AE, Gupta D, Boos MD, Zackai EH, Everman D, Ganapathi S, Wilson M, Christodoulou J, Zarate YA, Curry C, Li D, Guimier A, Amiel J, Hakonarson H, Webster R, Bhoj EJ, Perkins JA, Dahl
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gene mutations have been found in 29% of individuals with solitary IMF tumors and 68% of individuals with multiple tumors (with viscera involvement) plus multiple tumors (no visceral involvement) IMF. In about 50% of these cases, these somatic mutations were the same as those found in familial IMF
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Treatment of IMF tumors depends upon the tumor numbers, locations, and genetic abnormalities found in each individual and often include more than one therapeutic regimen. Individuals with solitary tumors are usually treated by observation with the expectation that many of these tumors will regress
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tests to determine the extent of their disease, particularly with respect to determining if a visceral tumor(s) is present and therefore that the proper diagnosis is multiple tumors (with viscera involvement). The treatment of multiple tumors (no viscera involvement) is similar to that used for
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as indicated in the following section on multiple tumors (with visceral involvement). Surgical excision has also be used if the diagnosis of the tumor is in doubt. In rare cases, aggressive IMF tumors have recurred after regressing and required further observation and/or treatment. Overall, the
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of brain, and lung. This form of IMF, particularly in cases with tumors involving the gastrointestinal tract, heart, and/or lung, has a far higher morbidity and mortality than the other forms with death occurring in infants during the first weeks to 4 months of life in 30–70% of cases Death is
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IMF tumors are usually painless, well-encapsulated, rubbery to hard, and freely movable-to-fixed masses. They may be evident at birth in up to 60% of cases but generally go undetected until they are diagnosed in the first year of life, uncommonly in older infants and young (<10 years/old)
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Multiple tumors (no viscera involvement) typically occur as dozens to hundreds of mostly small tumors located in various areas such as the skin, muscles, and frontal eye socket, These tumors do not involve visceral organs, often take a benign course, and may regress spontaneously.
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analyses of IMF tumor tissues commonly show repeated patterns of a central zone consisting of spindle-shaped cells lining capillaries and round-to-oval cells arranged around prominent "antler-shaped" vascular spaces all of which are surrounded by a peripheral zone of clustered
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strategy is commonly used for these tumors. Visceral solitary IMF tumors that cause significant tissue injury, are located in vital areas, and/or are life-threatening have been treated by surgical excision or, if surgery is deemed inappropriate, are treated with drugs and/or
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Hettmer S, Dachy G, Seitz G, Agaimy A, Duncan C, Jongmans M, Hirsch S, Kventsel I, Kordes U, de Krijger RR, Metzler M, Michaeli O, Nemes K, Poluha A, Ripperger T, Russo A, Smetsers S, Sparber-Sauer M, Stutz E, Bourdeaut F, Kratz CP, Demoulin JB (October 2021).
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El Demellawy D, Cundiff CA, Nasr A, Ozolek JA, Elawabdeh N, Caltharp SA, Masoudian P, Sullivan KJ, de Nanassy J, Shehata BM (2016). "Congenital mesoblastic nephroma: a study of 19 cases using immunohistochemistry and ETV6-NTRK3 fusion gene rearrangement".
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Multiple tumors (no viscera involvement) also may regress spontaneously and carry a good to excellent prognosis even when they invade local tissues. However, neonates presenting with multiple IMF tumors should undergo a through examination including
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multiple tumors (with viscera involvement) (also called generalized myofibromatosis) which rarely regress spontaneously and consist of numerous IMF lesions in non-visceral tissues plus one or more visceral tumors that may be life-threatening.
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Proust S, Benchimol G, Fraitag S, Starck J, Giacobbi V, Pierron G, Bodemer C, Orbach D (January 2021). "Major response to imatinib and chemotherapy in a newborn patient prenatally diagnosed with generalized infantile myofibromatosis".
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surgical resection. Cases where surgical resection of a single symptomatic tumor is not a good option and cases with multiple visceral tumors are treated by non-surgical methods. These tumors typically respond to conventional systemic
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There are no controlled studies to define the optimal treatment(s) for IMF. The following give the currently widely used and recommended treatments along with the result of these treatments for the three categories of the IMF tumors.
530:) mutation in the carrier family was p. Leu1519Pro. The p.Arg561Cys, p.Pro560Leu, p.Arg561Ser, and p. Lys567Glu mutations are regarded as contributing to the development and/or progression of IMF while the p.Pro660Thr 391:
Multiple tumors (with viscera involvement) typically occur as dozens to hundreds of tumors located in the same sites as multiple tumors (no viscera involvement) but also occur in one or more visceral organs such as those in the
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masses. Most of these tumors are <1 cm in diameter but can be much larger, e.g. 5.2 cm in diameter. At least 9 IMF cases described in the English literature were diagnosed in fetuses based on the findings of fetal
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genes has been reported to occur in a single family with autosomal dominant inheritance of IMF: 9 of this family's members had IMF plus the mutation while 7 of its members did not have IMF or the mutation. Since the mutated
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blocks the activity of the p.Arg561Cys and p.Asn666Lys mutants as well as the p.Arg561Cyt plus Asp666Lys and p.Trp566Arg plus AspN666Lys double mutants but not the p.Asp850Val mutant. Two other tyrosine kinase inhibitors,
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Pekar-Zlotin M, Levinsohn-Tavor O, Livneh A, Sher O, Melcer Y, Maymon R (October 2019). "Gynecology and Oncology Fetal Myofibromatosis: A Challenge for Prenatal Diagnosis Mini Review of the English Literature".
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the tyrosine kinase inhibitor should be selected based on the PDGFRB oncoprotein's sensitivity to it (e.g. dasatinib or ponatinib should be used to treat patients with the imatinib-resistant p.Asp850Val
614:, i.e. a normal gene that when mutated could form a cancer-causing oncoprotein. Studies show that the PDGFRB mutant proteins in IMF are constitutively overactive and that the tyrosine kinase inhibitor 271:. IMF tumors in the viscera or deep tissues may present with serious or life-threatening signs and symptoms of organ and/or deep tissue damage due to these tumors space-filling and pressuring effects. 1985:
Laskin WB, Miettinen M, Fetsch JF (January 2009). "Infantile digital fibroma/fibromatosis: a clinicopathologic and immunohistochemical study of 69 tumors from 57 patients with long-term follow-up".
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the inhibitors precise dosages for use in newborns and infants are not well-defined, have serious side-affects, and therefore should only be considered for refractory, life-threatening IMF tumors.
663:, a tumor that develops primarily in the fingers and toes, had been regarded as a type of IMF. However its tumors have a distinctly different clinical presentation and histopathology than IMF. The 1336:
Arts FA, Sciot R, Brichard B, Renard M, de Rocca Serra A, Dachy G, Noël LA, Velghe AI, Galant C, Debiec-Rychter M, Van Damme A, Vikkula M, Helaers R, Limaye N, Poirel HA, Demoulin JB (May 2017).
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Manisterski M, Benish M, Levin D, Shiran SI, Sher O, Gortzak Y, Elhasid R (February 2021). "Diverse presentation and tailored treatment of infantile myofibromatosis: A single-center experience".
571:) plus AspN666Lys in another case. Three cases had more complex mutations involving multiple gene sites with two of them having the p.Asn666Lys mutation gene in combination with other mutations. 586:
gene product, the PDGFβ receptor, is normally highly expressed in fibroblasts and other cells of mesenchymal origin including vascular smooth muscle cells. When activated, the PDGFβ receptor's
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with reductions in their sizes; these responses are usually observed only after several weeks of therapy. Chemotherapy regimes that have successfully treated these cases include low dosage:
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Sparber-Sauer M, Vokuhl C, Seitz G, Sorg B, Tobias M, von Kalle T, Münter M, Bielack SS, Ladenstein R, Ljungman G, Niggli F, Frühwald M, Loff S, Klingebiel T, Koscielniak E (October 2021).
2182: 1084:"Infantile myofibromatosis: Excellent prognosis but also rare fatal progressive disease. Treatment results of five Cooperative Weichteilsarkom Studiengruppe (CWS) trials and one registry" 2717: 643:, and tumorous lesions particular in cases which involve multiple tumors. However, the presence of two zone histopathology, family history of disease, and presence of the above cited 338:
Solitary IMF tumors represent 50 to 74% of all IMF cases. These tumors commonly present with a mass that infiltrates the surrounding tissue in the head and neck areas (e.g. in the
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Hassan M, Butler E, Wilson R, Roy A, Zheng Y, Liem P, Rakheja D, Pavlick D, Young LL, Rosenzweig M, Erlich R, Ali SM, Leavey PJ, Parsons DW, Skapek SX, Laetsch TW (October 2019).
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genes produce highly overactive platelet-derived growth factor receptor beta (i.e. PDGFβ receptor) proteins that contribute to the development and/or progression of IMF.
298:(i.e. non-inherited mutations developing only after conception) that are identical or similar to those seen in familial cases. The familial and non-familial mutated 651:
have been suggested to be the same disease because of their very similar histopathology. However, mesoblastic nephroma tumor cells, unlike IMF tumor cells, express
1476:"Recurrent SRF-RELA Fusions Define a Novel Subset of Cellular Myofibroma/Myopericytoma: A Potential Diagnostic Pitfall With Sarcomas With Myogenic Differentiation" 627:, do inhibit the p.Asp850Val mutant's tyrosine kinase activity. Limited initial studies suggest that these inhibitors are useful in treating severe cases of IMF. 2175: 784:, have successful treated several cases and chemotherapy plus imatinib have successfully treated a few cases of life-threatening IMF. Studies suggest that: 174:
A minority of infantile myofibromatosis tumors present in individuals with a strong family history of the disease. These familial cases are associated with
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gene in this family produces an overactive neurogenic locus notch homolog protein 3 product that in cell culture studies increases the expression of the
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Iwafuchi H, Tsuzuki T, Ito R, Miyake H, Okita H, Hamazaki M (August 2015). "Generalized infantile myofibromatosis with a monophasic primitive pattern".
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multiple tumors (no viscera involvement) which consists of numerous (i.e. dozens to >100) IMF lesions most of which are located in the skin and
668: 1688:"The infantile myofibromatosis NOTCH3 L1519P mutation leads to hyperactivated ligand-independent Notch signaling and increased PDGFRB expression" 141:, carry guarded to poor prognoses and can be life-threatening, particularly in newborns and young infants. The condition was first described by 688:
Solitary IMF tumors typically grow slowly, produce few or no symptoms, and often regress spontaneously within 18 to 24 months of diagnosis. A
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with the remaining cases having mutations in other sites of the gene. These other somatic mutation sites included 2 cases of p.Asp850Val (Asp=
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children, or rarely in older children and adults (one individual was diagnosed with IMF at 85 years of age). The tumors may be flesh-colored
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Wu D, Wang S, Oliveira DV, Del Gaudio F, Vanlandewijck M, Lebouvier T, Betsholtz C, Zhao J, Jin S, Lendahl U, Karlström H (January 2021).
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which in turn promote their parent cells to grow, proliferate, and survive for abnormally prolonged times. The PDGFβ receptor gene is a
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mutant genes, i.e. by increasing the number and therefore overall activity of the PDGFβ receptor. Further studies are needed to define
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Some individuals with IMF present with a family history of IMF tumors. To date (2021), 20 families have been reported to carry various
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imatinib should be the initial choice and dasatinib or ponatinib be used for cases failing to respond to imatinib in cases where the
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JP, Dobyns WB (July 2020). "Activating variants in PDGFRB result in a spectrum of disorders responsive to imatinib monotherapy".
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Based on their clinical presentation and gross appearance IMF tumors can be confused with a wide range of benign and malignant
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Zhao G, Zhu M, Qin C, Liu X, Zhao X (November 2020). "Infantile Myofibromatosis: 32 Patients and Review of Literature".
819: 2380: 429:-like cells. The relative proportions of the two zones can vary within different areas of a tumor and between tumors. 44:
Myofibromas, Multicentric fibromatosis, Congenital generalized fibromatosis, and Congenital multicentric fibromatosis
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Beck, Jill C.; Devaney, Kenneth O.; Weatherly, Robert A.; Koopmann, Charles F.; Lesperance, Marci M. (1999-01-01).
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of the genes product protein, the PDGFβ receptor. Four of the remaining families carried either a p.Pro560Leu (Pro=
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Soundararajan M, Kannan S (December 2018). "Fibroblasts and mesenchymal stem cells: Two sides of the same coin?".
1155:"Genetic testing and surveillance in infantile myofibromatosis: a report from the SIOPE Host Genome Working Group" 374:, lung, or heart. Solitary IMF tumors often (i.e. 25 to 61% of cases in different studies) regress spontaneously. 2784: 2672: 2453: 664: 158:
solitary IMF tumors (also called myofibromas) which often regress spontaneously and rarely cause serious issues;
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gene mutations. Regardless of these genetic variations, all IMF tumors consist of bland-appearing, benign (i.e.
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found in 1 in 150,000 to 1 in 400,000 live births. It is nonetheless the most common tumor derived from fibrous
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p. Leu1519Pro gene mutation require further study to determine more clearly their clinical significances.
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area, lower back, or soft tissue/bone in the lower leg; and also less commonly in visceral organs such as the
1636:"Novel PDGFRB rearrangement in multifocal infantile myofibromatosis is tumorigenic and sensitive to imatinib" 2751: 2509: 2353: 2293: 2245: 397: 2853: 2587: 2438: 2395: 2390: 393: 2148: 76:
Solitary tumor, Multiple tumors (no viscera involvement), and Multiple tumors (with viscera involvement)
1268:"Primary Resection and Immediate Autologous Reconstruction of Fronto-orbital Infantile Myofibromatoses" 2774: 2619: 2343: 2271: 1737:"Fibroblastic and myofibroblastic tumors of children: new genetic entities and new ancillary testing" 648: 287: 163: 197:
gene mutations in their tumor cells; these mutations are similar to those occurring in the familial
2764: 2690: 2572: 2372: 2348: 2308: 2303: 2216: 430: 347: 214: 1938:"Congenital mesoblastic nephroma: Clinical analysis of eight cases and a review of the literature" 1830:"PDGFRB platelet derived growth factor receptor beta [Homo sapiens (Human)] - Gene - NCBI" 2727: 2702: 2665: 2562: 2531: 2495: 2328: 2010: 1882: 1811: 1616: 1562: 1456: 1409: 1241: 1111: 898: 255: 142: 567:), and concurrent double mutations p.Arg561Cyt plus Asp666Lys in one case and p.Trp566Arg (Trp= 2827: 2799: 2707: 2567: 2358: 2191: 2059: 2002: 1967: 1918: 1874: 1803: 1768: 1717: 1665: 1608: 1554: 1505: 1448: 1401: 1359: 1297: 1233: 1184: 1103: 1057: 977: 969: 924: 890: 770: 766: 694: 640: 275: 247: 226: 126: 49: 2160: 254:(i.e. flat-topped papules that are equal to or greater than 10 mm in largest diameter); 2656: 2552: 2490: 2426: 2049: 2041: 1994: 1957: 1949: 1910: 1866: 1795: 1758: 1748: 1707: 1699: 1655: 1647: 1598: 1590: 1544: 1536: 1495: 1487: 1440: 1393: 1349: 1287: 1279: 1225: 1174: 1166: 1095: 1047: 1037: 959: 882: 758: 689: 667:(2020) redefined infantile digital fibromatosis as a benign tumor in the category of benign 471: 408: 295: 647:
gene mutations are almost always definitive indicators of IMF. IMF and the classic form of
2643: 2471: 708: 603: 587: 421: 343: 213:, the most common cell type in connective tissue, with a variable set of features seen in 138: 2779: 1083: 458:
Twenty families with inherited IMF have been shown to have mutations in one of their two
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gene mutation's frequency of occurrence in, and contribution to the development of, IMF.
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gene mutations, drugs directed specifically against this mutated gene's protein product.
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but not visceral organs, may regress spontaneously, and rarely cause serious issues; and
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mutation may promote the development and/or progression of IMF in a manner similar to
2842: 1620: 1566: 1460: 1413: 1245: 1115: 1026:"What to Look Out for in a Newborn with Multiple Papulonodular Skin Lesions at Birth" 902: 780:-associated IMF cases. Imatinib or imatinib plus a second tyrosine kinase inhibitor, 750: 659:
proteins and therefore likely have a very different cellular origin than IMF tumors.
527: 426: 405: 355: 339: 193:. However, most IMF cases have no family history of the disease but nonetheless have 2014: 1886: 1815: 1042: 100:
Guarded in multiple tumors (with visceral involvement); otherwise, good to excellent
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gene mutations, 15 carried a p.Arg561Cys mutation. This mutation substitutes the
2814: 2652: 2526: 2521: 2421: 2195: 814: 734: 726: 351: 264: 134: 54: 1540: 1170: 17: 2759: 2608: 964: 945: 754: 568: 560: 495: 483: 401: 291: 210: 202: 58: 973: 2685: 2628: 2577: 781: 652: 624: 620: 552: 519: 515: 467: 283: 175: 95: 2063: 2045: 2030:"The 2020 WHO Classification of Soft Tissue Tumours: news and perspectives" 2006: 1971: 1922: 1878: 1807: 1772: 1721: 1669: 1612: 1558: 1509: 1452: 1405: 1363: 1301: 1237: 1188: 1107: 1061: 894: 981: 2198: 2143: 1953: 1843: 1829: 1594: 1354: 1338:"PDGFRB gain-of-function mutations in sporadic infantile myofibromatosis" 1337: 762: 615: 487: 438: 367: 259: 137:
to distant tissues although when occurring in the viscera, i.e. internal
2088: 1651: 1603: 2281: 2254: 2202: 1703: 503: 499: 491: 359: 243: 1870: 1844:"NOTCH3 notch receptor 3 [Homo sapiens (Human)] - Gene - NCBI" 1799: 1444: 1099: 886: 2517: 2417: 2409: 2100: 1523:
Guérit E, Arts F, Dachy G, Boulouadnine B, Demoulin JB (April 2021).
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as congenital generalized fibromatosis – in which he coined the word
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prognosis in almost all cases of solitary IMF is good to excellent.
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that occurs primarily in infants and young children. IMF tumors are
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Antonescu CR, Sung YS, Zhang L, Agaram NP, Fletcher CD (May 2017).
286:(i.e. the mutant gene's product is overactive) and inherited in an 2604: 434: 122: 919:
Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007).
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analyses indicate that the spindle-shaped cells strongly express
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Lavie JL, Rogers CL, Stalder MW, St Hilaire H (January 2021).
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Recently, tyrosine kinase inhibitors have been used to treat
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at the 561st amino acid position as numbered starting at the
209:, i.e. cells that blend a variable set of features seen in 526:
gene (located in band 13.12 on the short (or "p") arm of
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Multiple cutaneous and uterine leiomyomatosis syndrome
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Rare tumor in infants originating in connective tissue
2078: 2813: 2750: 2641: 2617: 2595: 2586: 2508: 2480: 2452: 2408: 2367: 2280: 2253: 2244: 2235: 2209: 2134: 2082: 104: 94: 84:Familial and non-familial somatic mutations in the 80: 72: 64: 48: 40: 35: 951:Archives of Otolaryngology–Head & Neck Surgery 1936:Wang ZP, Li K, Dong KR, Xiao XM, Zheng S (2014). 2028:Sbaraglia M, Bellan E, Dei Tos AP (April 2021). 946:"Pediatric Myofibromatosis of the Head and Neck" 1681: 1679: 1425: 1423: 1379: 1377: 1375: 1373: 1331: 1272:Plastic and Reconstructive Surgery. Global Open 1147: 1145: 1019: 1017: 1015: 1013: 1011: 868: 866: 864: 862: 860: 278:(i.e. inherited) mutations in one of their two 1329: 1327: 1325: 1323: 1321: 1319: 1317: 1315: 1313: 1311: 1261: 1259: 1257: 1255: 1210: 1208: 1206: 1204: 1202: 1200: 1198: 1143: 1141: 1139: 1137: 1135: 1133: 1131: 1129: 1127: 1125: 1077: 1075: 1073: 1071: 1009: 1007: 1005: 1003: 1001: 999: 997: 995: 993: 991: 858: 856: 854: 852: 850: 848: 846: 844: 842: 840: 2176: 154:IMF tumors occur in three clinical patterns: 8: 1583:American Journal of Medical Genetics. Part A 358:); less commonly in other sites such as the 1525:"PDGF receptor mutations in human diseases" 2592: 2250: 2241: 2183: 2169: 2161: 2079: 1987:The American Journal of Surgical Pathology 1480:The American Journal of Surgical Pathology 914: 912: 716:Multiple tumors (with viscera involvement) 387:Multiple tumors (with viscera involvement) 32: 2053: 1961: 1762: 1752: 1711: 1659: 1640:Cold Spring Harbor Molecular Case Studies 1602: 1548: 1499: 1353: 1291: 1178: 1051: 1041: 963: 757:; and vincristine plus dactinomycin plus 534:gene mutation and the p. Leu1519Pro 318:gene's product, the PDGFβ receptor, this 1386:Journal of Pediatric Hematology/Oncology 801:gene mutation has not been defined; and 702:Multiple tumors (no viscera involvement) 378:Multiple tumors (no viscera involvement) 836: 669:fibroblastic and myofibroblastic tumors 1218:Obstetrical & Gynecological Survey 7: 1529:Cellular and Molecular Life Sciences 1024:Fraitag S, Boccara O (August 2021). 773:alone or combined with these drugs. 2222:Desmoplastic small-round-cell tumor 282:genes. All of these mutations were 671:and therefore different than IMF. 258:, calcified masses; or ulcerated, 25: 2299:Aggressive infantile fibromatosis 2790:Clear-cell sarcoma of the kidney 522:) mutation in this protein. The 2849:Dermal and subcutaneous growths 2558:Intradermal spindle cell lipoma 2267:Dermatofibrosarcoma protuberans 1692:Disease Models & Mechanisms 1043:10.3390/dermatopathology8030043 2386:Malignant fibrous histiocytoma 2334:Infantile digital fibromatosis 2319:Familial myxovascular fibromas 2314:Diffuse infantile fibromatosis 1859:Journal of Cellular Physiology 1754:10.12688/f1000research.16236.1 661:Infantile digital fibromatosis 559:), 2 case of p.Asn666Lys (Asn= 133:in the sense that they do not 1: 2432:Superficial acral fibromyxoma 2339:Juvenile hyaline fibromatosis 596:phosphatidylinositol-3 kinase 305:A mutation in one of the two 2733:Solitary cutaneous leiomyoma 2723:Multiple cutaneous leiomyoma 2444:Ossifying fibromyxoid tumour 1999:10.1097/PAS.0b013e3181788533 1915:10.1016/j.pathol.2015.11.007 1492:10.1097/PAS.0000000000000811 1433:Pediatric Blood & Cancer 1398:10.1097/MPH.0000000000001603 1284:10.1097/GOX.0000000000003261 1230:10.1097/OGX.0000000000000717 1088:Pediatric Blood & Cancer 875:Pediatric Blood & Cancer 820:List of cutaneous conditions 2381:Benign fibrous histiocytoma 229:or, in tumors with certain 2870: 2661:Embryonal rhabdomyosarcoma 2237:Connective tissue neoplasm 1541:10.1007/s00018-020-03753-y 1171:10.1007/s10689-020-00204-2 510:); p. Lys567Glu (Lys= 478:. Of the 20 families with 284:gain of function mutations 269:magnetic resonance imaging 2785:Malignant rhabdoid tumour 2752:Complex mixed and stromal 2681: 2673:Alveolar rhabdomyosarcoma 965:10.1001/archotol.125.1.39 921:Dermatology: 2-Volume Set 753:plus the glucocorticoid, 665:World Health Organization 400:and/or the heart, liver, 115:Infantile myofibromatosis 36:Infantile myofibromatosis 2324:Fibroma of tendon sheath 1342:Human Molecular Genetics 68:Infancy, early childhood 2354:Oral submucous fibrosis 2294:Aggressive fibromatosis 2210:Not otherwise specified 1788:Pathology International 741:; vinblastine plus the 675:Treatment and prognosis 518:); or p.Pro660Thr (Thr= 398:upper respiratory tract 2396:Solitary fibrous tumor 2391:Atypical fibroxanthoma 2046:10.32074/1591-951X-213 441:, and, less commonly, 394:gastrointestinal tract 2770:Mixed Müllerian tumor 712:solitary IMF tumors. 490:, for the amino acid 292:incomplete penetrance 2775:Mesoblastic nephroma 2344:Plantar fibromatosis 2272:Desmoplastic fibroma 1954:10.3892/ol.2014.2489 1595:10.1002/ajmg.a.61615 923:. St. Louis: Mosby. 825:mesoblastic nephroma 649:mesoblastic nephroma 506:); p.Arg561Ser (Ser= 354:, behind an ear, or 164:subcutaneous tissues 2765:Pleomorphic adenoma 2573:Spindle cell lipoma 2373:histiocytic sarcoma 2349:Pleomorphic fibroma 2309:Collagenous fibroma 2304:Aponeurotic fibroma 2217:Soft-tissue sarcoma 1652:10.1101/mcs.a004440 749:; vincristine plus 684:Solitary IMF tumors 466:gene is located in 435:smooth muscle actin 431:Immunohistochemical 348:infratemporal fossa 334:Solitary IMF tumors 215:smooth muscle cells 2728:Neural fibrolipoma 2703:Angiolipoleiomyoma 2666:Sarcoma botryoides 2563:Pleomorphic lipoma 2532:Myxoid liposarcoma 2496:Clear-cell sarcoma 2329:Fibromatosis colli 2135:External resources 1735:Parham DM (2018). 1704:10.1242/dmm.046300 1355:10.1093/hmg/ddx081 449:Gene abnormalities 288:autosomal dominant 143:Arthur Purdy Stout 2836: 2835: 2828:Adenomatoid tumor 2800:Pancreatoblastoma 2746: 2745: 2708:Genital leiomyoma 2568:Lipoblastomatosis 2504: 2503: 2404: 2403: 2359:Pachydermodactyly 2158: 2157: 1871:10.1002/jcp.26860 1865:(12): 9099–9109. 1800:10.1111/pin.12312 1445:10.1002/pbc.28576 1348:(10): 1801–1810. 1100:10.1002/pbc.29403 930:978-1-4160-2999-1 887:10.1002/pbc.28769 793:gene mutation.); 771:radiation therapy 695:radiation therapy 594:proteins such as 472:long (or "q") arm 296:somatic mutations 227:radiation therapy 127:connective tissue 112: 111: 30:Medical condition 16:(Redirected from 2861: 2657:rhabdomyosarcoma 2646: 2622: 2598: 2593: 2553:Chondroid lipoma 2491:Synovial sarcoma 2427:Cutaneous myxoma 2251: 2242: 2185: 2178: 2171: 2162: 2080: 2068: 2067: 2057: 2025: 2019: 2018: 1982: 1976: 1975: 1965: 1948:(5): 2007–2011. 1942:Oncology Letters 1933: 1927: 1926: 1897: 1891: 1890: 1854: 1848: 1847: 1840: 1834: 1833: 1826: 1820: 1819: 1783: 1777: 1776: 1766: 1756: 1732: 1726: 1725: 1715: 1683: 1674: 1673: 1663: 1631: 1625: 1624: 1606: 1589:(7): 1576–1591. 1577: 1571: 1570: 1552: 1535:(8): 3867–3881. 1520: 1514: 1513: 1503: 1471: 1465: 1464: 1427: 1418: 1417: 1381: 1368: 1367: 1357: 1333: 1306: 1305: 1295: 1263: 1250: 1249: 1212: 1193: 1192: 1182: 1149: 1120: 1119: 1079: 1066: 1065: 1055: 1045: 1030:Dermatopathology 1021: 986: 985: 967: 941: 935: 934: 916: 907: 906: 870: 767:interferon alpha 759:cyclophosphamide 690:watchful waiting 542:Non-familial IMF 33: 21: 2869: 2868: 2864: 2863: 2862: 2860: 2859: 2858: 2839: 2838: 2837: 2832: 2809: 2742: 2677: 2644:Skeletal muscle 2642: 2637: 2618: 2613: 2596: 2582: 2500: 2476: 2472:Phyllodes tumor 2454:Fibroepithelial 2448: 2400: 2363: 2276: 2231: 2205: 2189: 2159: 2154: 2153: 2130: 2129: 2091: 2077: 2072: 2071: 2027: 2026: 2022: 1984: 1983: 1979: 1935: 1934: 1930: 1899: 1898: 1894: 1856: 1855: 1851: 1842: 1841: 1837: 1828: 1827: 1823: 1785: 1784: 1780: 1734: 1733: 1729: 1685: 1684: 1677: 1633: 1632: 1628: 1579: 1578: 1574: 1522: 1521: 1517: 1473: 1472: 1468: 1429: 1428: 1421: 1383: 1382: 1371: 1335: 1334: 1309: 1265: 1264: 1253: 1224:(10): 607–610. 1214: 1213: 1196: 1159:Familial Cancer 1151: 1150: 1123: 1081: 1080: 1069: 1023: 1022: 989: 943: 942: 938: 931: 918: 917: 910: 872: 871: 838: 833: 811: 718: 709:medical imaging 704: 686: 677: 633: 604:phospholipase C 588:tyrosine kinase 580: 544: 456: 451: 422:histopathologic 418: 389: 380: 344:maxillary sinus 336: 239: 31: 28: 23: 22: 18:Myofibromatosis 15: 12: 11: 5: 2867: 2865: 2857: 2856: 2851: 2841: 2840: 2834: 2833: 2831: 2830: 2825: 2819: 2817: 2811: 2810: 2808: 2807: 2805:Carcinosarcoma 2802: 2797: 2795:Hepatoblastoma 2792: 2787: 2782: 2777: 2772: 2767: 2762: 2756: 2754: 2748: 2747: 2744: 2743: 2741: 2740: 2735: 2730: 2725: 2720: 2715: 2713:Leiomyosarcoma 2710: 2705: 2700: 2698:Angioleiomyoma 2695: 2694: 2693: 2682: 2679: 2678: 2676: 2675: 2670: 2669: 2668: 2649: 2647: 2639: 2638: 2636: 2635: 2633:leiomyosarcoma 2625: 2623: 2615: 2614: 2612: 2611: 2601: 2599: 2590: 2584: 2583: 2581: 2580: 2575: 2570: 2565: 2560: 2555: 2549: 2548: 2547: 2546: 2544:Angiomyolipoma 2536: 2535: 2534: 2529: 2514: 2512: 2506: 2505: 2502: 2501: 2499: 2498: 2493: 2487: 2485: 2478: 2477: 2475: 2474: 2469: 2464: 2462:Brenner tumour 2458: 2456: 2450: 2449: 2447: 2446: 2441: 2436: 2435: 2434: 2429: 2414: 2412: 2406: 2405: 2402: 2401: 2399: 2398: 2393: 2388: 2383: 2377: 2375: 2365: 2364: 2362: 2361: 2356: 2351: 2346: 2341: 2336: 2331: 2326: 2321: 2316: 2311: 2306: 2301: 2296: 2290: 2288: 2278: 2277: 2275: 2274: 2269: 2263: 2261: 2248: 2239: 2233: 2232: 2230: 2229: 2224: 2219: 2213: 2211: 2207: 2206: 2190: 2188: 2187: 2180: 2173: 2165: 2156: 2155: 2152: 2151: 2139: 2138: 2136: 2132: 2131: 2128: 2127: 2116: 2105: 2092: 2087: 2086: 2084: 2083:Classification 2076: 2075:External links 2073: 2070: 2069: 2020: 1977: 1928: 1892: 1849: 1835: 1821: 1778: 1727: 1675: 1646:(5): a004440. 1626: 1572: 1515: 1486:(5): 677–684. 1466: 1419: 1392:(8): 495–498. 1369: 1307: 1251: 1194: 1165:(4): 327–336. 1121: 1067: 1036:(3): 390–417. 987: 936: 929: 908: 835: 834: 832: 829: 828: 827: 822: 817: 810: 807: 743:glucocorticoid 717: 714: 703: 700: 685: 682: 676: 673: 632: 629: 612:proto-oncogene 592:cell signaling 579: 573: 543: 540: 455: 452: 450: 447: 417: 414: 388: 385: 379: 376: 335: 332: 238: 235: 207:myofibroblasts 178:in either the 110: 109: 106: 102: 101: 98: 92: 91: 82: 78: 77: 74: 70: 69: 66: 62: 61: 52: 46: 45: 42: 38: 37: 29: 26: 24: 14: 13: 10: 9: 6: 4: 3: 2: 2866: 2855: 2854:Rare diseases 2852: 2850: 2847: 2846: 2844: 2829: 2826: 2824: 2821: 2820: 2818: 2816: 2812: 2806: 2803: 2801: 2798: 2796: 2793: 2791: 2788: 2786: 2783: 2781: 2778: 2776: 2773: 2771: 2768: 2766: 2763: 2761: 2758: 2757: 2755: 2753: 2749: 2739: 2736: 2734: 2731: 2729: 2726: 2724: 2721: 2719: 2716: 2714: 2711: 2709: 2706: 2704: 2701: 2699: 2696: 2692: 2689: 2688: 2687: 2684: 2683: 2680: 2674: 2671: 2667: 2664: 2663: 2662: 2658: 2654: 2651: 2650: 2648: 2645: 2640: 2634: 2630: 2627: 2626: 2624: 2621: 2620:Smooth muscle 2616: 2610: 2606: 2603: 2602: 2600: 2594: 2591: 2589: 2585: 2579: 2576: 2574: 2571: 2569: 2566: 2564: 2561: 2559: 2556: 2554: 2551: 2550: 2545: 2542: 2541: 2540: 2537: 2533: 2530: 2528: 2525: 2524: 2523: 2519: 2516: 2515: 2513: 2511: 2507: 2497: 2494: 2492: 2489: 2488: 2486: 2483: 2479: 2473: 2470: 2468: 2465: 2463: 2460: 2459: 2457: 2455: 2451: 2445: 2442: 2440: 2437: 2433: 2430: 2428: 2425: 2424: 2423: 2419: 2416: 2415: 2413: 2411: 2407: 2397: 2394: 2392: 2389: 2387: 2384: 2382: 2379: 2378: 2376: 2374: 2370: 2366: 2360: 2357: 2355: 2352: 2350: 2347: 2345: 2342: 2340: 2337: 2335: 2332: 2330: 2327: 2325: 2322: 2320: 2317: 2315: 2312: 2310: 2307: 2305: 2302: 2300: 2297: 2295: 2292: 2291: 2289: 2287: 2283: 2279: 2273: 2270: 2268: 2265: 2264: 2262: 2260: 2256: 2252: 2249: 2247: 2243: 2240: 2238: 2234: 2228: 2225: 2223: 2220: 2218: 2215: 2214: 2212: 2208: 2204: 2200: 2197: 2193: 2186: 2181: 2179: 2174: 2172: 2167: 2166: 2163: 2150: 2146: 2145: 2141: 2140: 2137: 2133: 2126: 2122: 2121: 2117: 2115: 2111: 2110: 2106: 2103: 2102: 2098: 2094: 2093: 2090: 2085: 2081: 2074: 2065: 2061: 2056: 2051: 2047: 2043: 2039: 2035: 2031: 2024: 2021: 2016: 2012: 2008: 2004: 2000: 1996: 1992: 1988: 1981: 1978: 1973: 1969: 1964: 1959: 1955: 1951: 1947: 1943: 1939: 1932: 1929: 1924: 1920: 1916: 1912: 1908: 1904: 1896: 1893: 1888: 1884: 1880: 1876: 1872: 1868: 1864: 1860: 1853: 1850: 1845: 1839: 1836: 1831: 1825: 1822: 1817: 1813: 1809: 1805: 1801: 1797: 1793: 1789: 1782: 1779: 1774: 1770: 1765: 1760: 1755: 1750: 1746: 1742: 1741:F1000Research 1738: 1731: 1728: 1723: 1719: 1714: 1709: 1705: 1701: 1697: 1693: 1689: 1682: 1680: 1676: 1671: 1667: 1662: 1657: 1653: 1649: 1645: 1641: 1637: 1630: 1627: 1622: 1618: 1614: 1610: 1605: 1600: 1596: 1592: 1588: 1584: 1576: 1573: 1568: 1564: 1560: 1556: 1551: 1546: 1542: 1538: 1534: 1530: 1526: 1519: 1516: 1511: 1507: 1502: 1497: 1493: 1489: 1485: 1481: 1477: 1470: 1467: 1462: 1458: 1454: 1450: 1446: 1442: 1439:(1): e28576. 1438: 1434: 1426: 1424: 1420: 1415: 1411: 1407: 1403: 1399: 1395: 1391: 1387: 1380: 1378: 1376: 1374: 1370: 1365: 1361: 1356: 1351: 1347: 1343: 1339: 1332: 1330: 1328: 1326: 1324: 1322: 1320: 1318: 1316: 1314: 1312: 1308: 1303: 1299: 1294: 1289: 1285: 1281: 1277: 1273: 1269: 1262: 1260: 1258: 1256: 1252: 1247: 1243: 1239: 1235: 1231: 1227: 1223: 1219: 1211: 1209: 1207: 1205: 1203: 1201: 1199: 1195: 1190: 1186: 1181: 1176: 1172: 1168: 1164: 1160: 1156: 1148: 1146: 1144: 1142: 1140: 1138: 1136: 1134: 1132: 1130: 1128: 1126: 1122: 1117: 1113: 1109: 1105: 1101: 1097: 1094:(3): e29403. 1093: 1089: 1085: 1078: 1076: 1074: 1072: 1068: 1063: 1059: 1054: 1049: 1044: 1039: 1035: 1031: 1027: 1020: 1018: 1016: 1014: 1012: 1010: 1008: 1006: 1004: 1002: 1000: 998: 996: 994: 992: 988: 983: 979: 975: 971: 966: 961: 957: 953: 952: 947: 940: 937: 932: 926: 922: 915: 913: 909: 904: 900: 896: 892: 888: 884: 881:(2): e28769. 880: 876: 869: 867: 865: 863: 861: 859: 857: 855: 853: 851: 849: 847: 845: 843: 841: 837: 830: 826: 823: 821: 818: 816: 813: 812: 808: 806: 804: 800: 796: 792: 787: 783: 779: 774: 772: 768: 764: 760: 756: 752: 751:actinomycin D 748: 744: 740: 736: 732: 728: 724: 715: 713: 710: 701: 699: 696: 691: 683: 681: 674: 672: 670: 666: 662: 658: 654: 650: 646: 642: 638: 630: 628: 626: 622: 617: 613: 609: 605: 601: 600:STAT proteins 597: 593: 589: 585: 577: 574: 572: 570: 566: 562: 558: 554: 549: 541: 539: 537: 533: 529: 528:chromosome 19 525: 521: 517: 513: 509: 505: 501: 497: 493: 489: 485: 481: 477: 473: 469: 465: 461: 453: 448: 446: 444: 440: 436: 432: 428: 427:smooth muscle 423: 415: 413: 410: 407: 403: 399: 395: 386: 384: 377: 375: 373: 369: 365: 361: 357: 356:temporal bone 353: 349: 345: 341: 340:ethmoid sinus 333: 331: 329: 325: 321: 317: 313: 308: 303: 301: 297: 293: 289: 285: 281: 277: 272: 270: 266: 261: 257: 253: 249: 245: 236: 234: 232: 228: 224: 218: 216: 212: 208: 204: 203:non-malignant 200: 196: 192: 189: 188: 183: 182: 177: 172: 169: 165: 161: 157: 152: 150: 149: 144: 140: 136: 132: 128: 124: 120: 116: 107: 103: 99: 97: 93: 89: 88: 83: 79: 75: 71: 67: 63: 60: 56: 53: 51: 47: 43: 39: 34: 19: 2823:Mesothelioma 2780:Wilms' tumor 2467:Fibroadenoma 2369:Histiocytoma 2286:fibromatosis 2259:fibrosarcoma 2227:Skin sarcoma 2142: 2118: 2107: 2095: 2040:(2): 70–84. 2037: 2033: 2023: 1990: 1986: 1980: 1945: 1941: 1931: 1909:(1): 47–50. 1906: 1902: 1895: 1862: 1858: 1852: 1838: 1824: 1794:(8): 432–7. 1791: 1787: 1781: 1744: 1740: 1730: 1695: 1691: 1643: 1639: 1629: 1604:11343/275870 1586: 1582: 1575: 1532: 1528: 1518: 1483: 1479: 1469: 1436: 1432: 1389: 1385: 1345: 1341: 1278:(1): e3261. 1275: 1271: 1221: 1217: 1162: 1158: 1091: 1087: 1033: 1029: 958:(1): 39–44. 955: 949: 939: 920: 878: 874: 802: 798: 794: 790: 785: 777: 775: 747:prednisolone 739:methotrexate 731:dactinomycin 723:chemotherapy 719: 705: 687: 678: 657:Beta-catenin 644: 634: 583: 581: 575: 547: 545: 535: 531: 523: 479: 476:chromosome 5 463: 459: 457: 454:Familial IMF 420:Microscopic 419: 390: 381: 337: 327: 323: 319: 315: 311: 306: 304: 299: 290:manner with 279: 273: 256:pedunculated 240: 237:Presentation 230: 223:chemotherapy 219: 198: 194: 185: 179: 173: 167: 159: 155: 153: 148:fibromatosis 146: 121:) is a rare 118: 114: 113: 85: 2815:Mesothelial 2653:Rhabdomyoma 2527:Myelolipoma 2522:liposarcoma 2439:Angiomyxoma 2422:myxosarcoma 2246:Fibromatous 2196:soft tissue 2034:Pathologica 1993:(1): 1–13. 815:Skin lesion 735:vinblastine 727:vincristine 590:stimulates 462:genes. The 352:hard palate 265:spectrogram 211:fibroblasts 151:– in 1954. 135:metastasize 65:Usual onset 55:Neonatology 41:Other names 2843:Categories 2760:Adenomyoma 2609:myosarcoma 2510:Lipomatous 2410:Myxomatous 2192:Connective 831:References 755:prednisone 569:tryptophan 561:asparagine 496:N-terminus 486:Arg, i.e. 484:amino acid 470:32 on the 445:proteins. 404:, rectum, 402:cerebellum 350:, tongue, 59:Pediatrics 2691:Cutaneous 2686:Leiomyoma 2629:Leiomyoma 2588:Myomatous 2578:Hibernoma 1903:Pathology 1621:219331223 1567:231612187 1461:221540155 1414:208275551 1246:204966175 1116:238636902 974:0886-4470 903:222826034 782:sorafenib 653:cyclin D1 631:Diagnosis 625:ponatinib 621:dasatinib 553:aspartate 520:threonine 516:glutamate 416:Pathology 176:mutations 105:Frequency 96:Prognosis 50:Specialty 2482:Synovial 2203:sarcomas 2144:Orphanet 2064:33179614 2015:30315278 2007:18830128 1972:25295083 1923:27020209 1887:49410224 1879:29943820 1816:32983693 1808:25989870 1773:30613391 1747:: 1963. 1722:33509954 1670:31645346 1613:32500973 1559:33449152 1550:11072557 1510:28248815 1453:32896962 1406:31764512 1364:28334876 1302:33552804 1238:31670833 1189:32888134 1108:34636137 1062:34449594 895:33063933 809:See also 763:cytokine 616:imatinib 546:Somatic 488:arginine 439:calponin 406:parietal 368:pancreas 276:germline 260:necrotic 2597:General 2282:Fibroma 2255:Fibroma 2125:D018224 2104:: D48.1 2055:8167394 1963:4186628 1764:6305242 1713:7927659 1661:6824247 1501:5391281 1293:7858576 1180:8484085 1053:8395860 982:9932585 641:nodular 637:papular 606:γ, and 504:leucine 500:proline 492:cystine 360:scapula 267:and/or 252:plaques 248:nodules 244:papules 225:drugs, 2539:PEComa 2518:Lipoma 2418:Myxoma 2199:tumors 2114:228550 2062:  2052:  2013:  2005:  1970:  1960:  1921:  1885:  1877:  1814:  1806:  1771:  1761:  1720:  1710:  1668:  1658:  1619:  1611:  1565:  1557:  1547:  1508:  1498:  1459:  1451:  1412:  1404:  1362:  1300:  1290:  1244:  1236:  1187:  1177:  1114:  1106:  1060:  1050:  980:  972:  927:  901:  893:  799:PDGFRB 791:PDGFRB 778:PDGFRB 645:PDGFRB 584:PDGFRB 576:PDGFRB 565:lysine 563:, Lys= 557:valine 555:, Val= 548:PDGFRB 536:NOTCH3 532:PDGFRB 524:NOTCH3 514:, Glu= 512:lysine 508:serine 502:, Leu= 480:PDGFRB 464:PDGFRB 460:PDGFRB 443:desmin 409:cortex 372:spleen 364:axilla 328:NOTCH3 324:PDGFRB 320:NOTCH3 316:PDGFRB 312:NOTCH3 307:NOTCH3 300:PDGFRB 280:PDGFRB 231:PDGFRB 199:PDGFRB 195:PDGFRB 187:NOTCH3 181:PDGFRB 139:organs 131:benign 87:PDGFRB 81:Causes 2738:STUMP 2605:Myoma 2484:-like 2011:S2CID 1883:S2CID 1812:S2CID 1698:(2). 1617:S2CID 1563:S2CID 1457:S2CID 1410:S2CID 1242:S2CID 1112:S2CID 899:S2CID 737:plus 729:plus 123:tumor 73:Types 2201:and 2149:2591 2120:MeSH 2109:OMIM 2060:PMID 2003:PMID 1968:PMID 1919:PMID 1875:PMID 1804:PMID 1769:PMID 1718:PMID 1666:PMID 1609:PMID 1555:PMID 1506:PMID 1449:PMID 1402:PMID 1360:PMID 1298:PMID 1234:PMID 1185:PMID 1104:PMID 1058:PMID 978:PMID 970:ISSN 925:ISBN 891:PMID 761:. A 655:and 623:and 608:GRB2 582:The 578:gene 468:band 396:and 191:gene 108:rare 90:gene 2097:ICD 2050:PMC 2042:doi 2038:113 1995:doi 1958:PMC 1950:doi 1911:doi 1867:doi 1863:233 1796:doi 1759:PMC 1749:doi 1708:PMC 1700:doi 1656:PMC 1648:doi 1599:hdl 1591:doi 1587:182 1545:PMC 1537:doi 1496:PMC 1488:doi 1441:doi 1394:doi 1350:doi 1288:PMC 1280:doi 1226:doi 1175:PMC 1167:doi 1096:doi 1048:PMC 1038:doi 960:doi 956:125 883:doi 474:of 246:or 184:or 119:IMF 2845:: 2659:: 2147:: 2123:: 2112:: 2101:10 2058:. 2048:. 2036:. 2032:. 2009:. 2001:. 1991:33 1989:. 1966:. 1956:. 1944:. 1940:. 1917:. 1907:48 1905:. 1881:. 1873:. 1861:. 1810:. 1802:. 1792:65 1790:. 1767:. 1757:. 1743:. 1739:. 1716:. 1706:. 1696:14 1694:. 1690:. 1678:^ 1664:. 1654:. 1642:. 1638:. 1615:. 1607:. 1597:. 1585:. 1561:. 1553:. 1543:. 1533:78 1531:. 1527:. 1504:. 1494:. 1484:41 1482:. 1478:. 1455:. 1447:. 1437:68 1435:. 1422:^ 1408:. 1400:. 1390:42 1388:. 1372:^ 1358:. 1346:26 1344:. 1340:. 1310:^ 1296:. 1286:. 1274:. 1270:. 1254:^ 1240:. 1232:. 1222:74 1220:. 1197:^ 1183:. 1173:. 1163:20 1161:. 1157:. 1124:^ 1110:. 1102:. 1092:69 1090:. 1086:. 1070:^ 1056:. 1046:. 1032:. 1028:. 990:^ 976:. 968:. 954:. 948:. 911:^ 897:. 889:. 879:68 877:. 839:^ 803:3) 795:2) 786:1) 765:, 745:, 733:; 639:, 602:, 598:, 437:, 370:, 346:, 250:; 217:. 205:) 168:3) 160:2) 156:1) 57:, 2655:/ 2631:/ 2607:/ 2520:/ 2420:/ 2371:/ 2284:/ 2257:/ 2194:/ 2184:e 2177:t 2170:v 2099:- 2089:D 2066:. 2044:: 2017:. 1997:: 1974:. 1952:: 1946:8 1925:. 1913:: 1889:. 1869:: 1846:. 1832:. 1818:. 1798:: 1775:. 1751:: 1745:7 1724:. 1702:: 1672:. 1650:: 1644:5 1623:. 1601:: 1593:: 1569:. 1539:: 1512:. 1490:: 1463:. 1443:: 1416:. 1396:: 1366:. 1352:: 1304:. 1282:: 1276:9 1248:. 1228:: 1191:. 1169:: 1118:. 1098:: 1064:. 1040:: 1034:8 984:. 962:: 933:. 905:. 885:: 362:/ 342:/ 117:( 20:)

Index

Myofibromatosis
Specialty
Neonatology
Pediatrics
PDGFRB
Prognosis
tumor
connective tissue
benign
metastasize
organs
Arthur Purdy Stout
fibromatosis
subcutaneous tissues
mutations
PDGFRB
NOTCH3
gene
non-malignant
myofibroblasts
fibroblasts
smooth muscle cells
chemotherapy
radiation therapy
papules
nodules
plaques
pedunculated
necrotic
spectrogram

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