739:, which kill cancer cells with focused doses of radiation, is often indicated in the treatment of rhabdomyosarcoma, and the exclusion of this treatment from disease management has been shown to increase recurrence rates. Radiation therapy is used when resecting the entirety of the tumor would involve disfigurement or loss of important organs (eye, bladder, etc.). Generally, in any case where a lack of complete resection is suspected, radiation therapy is indicated. Administration is usually following 6–12 weeks of chemotherapy if tumor cells are still present. The exception to this schedule is the presence of parameningeal tumors that have invaded the brain, spinal cord, or skull. In these cases radiation treatment is started immediately. In some cases, special radiation treatment may be required. Brachytherapy, or the placement of small, radioactive "seeds" directly inside the tumor or cancer site, is often indicated in children with tumors of sensitive areas such as the testicles, bladder, or vagina. This reduces scattering and the degree of late toxicity following dosing. Radiation therapy is more often indicated in higher stage classifications.
697:. Surgery is generally the first step in a combined therapeutic approach. Resectability varies depending on tumor site, and RMS often presents in sites that don't allow for full surgical resection without significant morbidity and loss of function. Less than 20% of RMS tumors are fully resected with negative margins. Rhabdomyosarcomas are highly chemosensitive, with approximately 80% of cases responding to chemotherapy. In fact, multi-agent chemotherapy is indicated for all patients with rhabdomyosarcoma. Before the use of adjuvant and neoadjuvant therapy involving chemotherapeutic agents, treatment solely by surgical means had a survival rate of <20%. Modern survival rates with adjuvant therapy are approximately 60–70%.
212:(ARMS) is the second-most common type. ARMS comprises around 20–25% of RMS-related tumors, and it is equally distributed among all age groups with an incidence of about one case per million people ages 0 to 19. For this reason, it is the most common form of RMS observed in young adults and teenagers, who are less prone to the embryonal variant. This type of RMS is characterized by densely packed, round cells that arrange around spaces similar in shape to pulmonary alveoli, although variants have been discovered without these characteristic alveolar spacings. ARMS tends to form more often in the extremities, trunk, and peritoneum. It is also typically more aggressive than ERMS.
617:(tumor-nodes-metastasis) system originally developed by the IRSG. This system accounts for tumor size (> or <5 cm), lymph node involvement, tumor site, and presence of metastasis. It grades on a scale of 1 to 4 based on these criteria. In addition, patients are sorted by clinical group (from the clinical groups from the IRSG studies) based on the success of their first surgical resection. The current Children's Oncology Group protocols for the treatment of RMS categorize patients into one of four risk categories based on tumor grade and clinical group, and these risk categories have been shown to be highly predictive of outcome.
295:. Extremity tumors generally present as a rapidly enlarging, firm mass in the relevant tissue. The cancer's prevalence in the head, face, and neck will often allow for earlier signs of the disease simply due to the obvious nature of tumors in these locations. Despite the varying presentation and typically aggressive nature of the disease, RMS has the potential to be diagnosed and treated early. The fourth IRSG study found that 23% of patients were diagnosed in time for a complete resection of their cancer, and 15% had resection with only minimal remnants of the diseased cells.
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children or adolescents, two-thirds of reported cases occur in youths under the age of 10. RMS also occurs slightly more often in males than in females, with a ratio of approximately 1.3–1.5:1. In addition, slightly lower prevalence of the disease has been reported in black and Asian children relative to white children. In most cases, there are no clear predisposing risk factors for the development of RMS. It tends to occur sporadically with no obvious cause. However, RMS has been correlated with familial cancer syndromes and congenital abnormalities including
659:
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pulled from the patient and training to attack tumors through presentation with tumor antigen, or other experimental methods. A specific example here would be presenting some of the patient's dendritic cells, which direct the immune system to foreign cells, with the PAX3-FKHR fusion protein in order to focus the patient's immune system to the malignant RMS cells. All cancers, including rhabdomyosarcoma, could potentially benefit from this new, immune-based approach.
186:(ERMS) is the most common histological variant, comprising about 60–70% of childhood cases. It is most common in children birth to four years old, with a maximum reported incidence of four cases per million children. ERMS is characterized by spindle-shaped cells with a stromal-rich appearance, and the morphology is similar to the developing muscle cells of a 6- to 8-week-old embryo. Tumors often present in the head and neck, as well as the genitourinary tract.
228:
correlating to a worse prognosis. It occurs most often in adults, rarely in children, and is often discovered in the extremities. Due to the lack of discernible separation among cancers of this type, clinicians often label undiagnosed sarcomas with little to no discernible features as anaplastic RMS. It is the most aggressive type of RMS, and often requires intensive treatment.
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32:
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specific genetic lesions can allow for accurate classification of the ARMS subtype when the histopathological findings are equivocal or unclear. This is valuable for clinical practice as the alveolar type presents a higher risk to the patient and will often require more aggressive treatment than the embryonal type. Thus, ARMS is also referred to as
451:, and the potential loss-of-function of this region is likely associated with the loss of a tumor suppressor. However, the specific consequences of this LOH at (p11,15.5) have yet to be determined. The short arm of chromosome 11 is also the site of the insulin-like growth factor 2 gene (IGF-2), which is often over-expressed in RMS.
272:
Multiple classification systems have been proposed for guiding management and treatment, and the most recent and widely used classification system is the "International
Classification of Rhabdomyosarcoma" or ICR. It was created by the IRSG in 1995 after their series of four multi-institutional trials
197:
is almost always found in mucosal-lined organs, including the vagina, bladder, and nasopharynx (although presentation in the nasopharynx typically affects older children). It often presents in infants younger than a year old, as a round, grape-like mass on the affected organ. Histologically, cells of
819:
against such cells are in progress. Epigenetic therapy for rhabdomyosarcoma is becoming more important. A recent study by
Bharathy et al. found that deacetylase inhibitor, entinostat works in aggressive subtype, alveolar rhabdomyosarcoma (aRMS) by specifically blocking the activity of HDAC3, thereby
136:
RMS can occur in any soft-tissue site in the body, but is primarily found in the head, neck, orbit, genitourinary tract, genitals, and extremities. No clear risk factors have been identified, but the disease has been associated with some congenital abnormalities. Signs and symptoms vary according to
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in children as well as the third most common solid tumor in children. Recent estimates place the incidence of the disease at approximately 4.5 case per 1 million children/adolescents with approximately 250 new cases in the United States each year. With the vast majority of cases of RMS occurring in
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is a more recent treatment modality that is still in development. This method involves recruiting and training the patient's immune system to target the cancer cells. This can be accomplished through administering small molecules designed to pull immune cells towards the tumors, taking immune cells
152:
Treatment usually involves a combination of surgery, chemotherapy, and radiation. 60 to 70% of newly diagnosed patients with nonmetastatic disease can be cured using this combined approach to therapy. Despite aggressive multimodality treatment, less than 20% of patients with metastatic RMS are able
2899:
Raney, Richard
Beverly; Meza, Jane; Anderson, James R.; Fryer, Christopher J.; Donaldson, Sarah S.; Breneman, John C.; Fitzgerald, Thomas J.; Gehan, Edmund A.; Michalski, Jeff M. (2002-01-01). "Treatment of children and adolescents with localized parameningeal sarcoma: experience of the Intergroup
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Rhabdomyosarcoma was first described by Weber, a German physician, in 1845, but it was not until the paper by Arthur Stout in 1946 that RMS was formally classified. The first thirty years of investigation were conducted by the
Intergroup Rhabdomyosarcoma Study Group (IRSG), an independent National
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Raney, R. B.; Tefft, M.; Newton, W. A.; Ragab, A. H.; Lawrence, W.; Gehan, E. A.; Maurer, H. M. (1987-01-01). "Improved prognosis with intensive treatment of children with cranial soft tissue sarcomas arising in nonorbital parameningeal sites. A report from the
Intergroup Rhabdomyosarcoma Study".
335:
There are multiple genetic lesions associated with rhabdomyosarcoma, but there has been little consistent data demonstrating an association between specific genetic abnormalities and outcome. However, alveolar and embryonal types of RMS can be distinguished cytogenetically, and identification of
281:
RMS can occur in almost any soft-tissue site in the body; the most common primary sites are genitourinary (24%), parameningeal (16%), extremity (19%), orbit (9%), other head and neck (10%), and miscellaneous other sites (22%). RMS often presents as a mass, but signs and symptoms can vary widely
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to the VAC regimen was shown to increase survival rates of patients with alveolar-type, early-stage RMS in IRS study III, and this same addition improved survival rates and doubled bladder salvage rates in patients with stage III RMS of the bladder. In children and young adults with stage IV
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rhabdomyosarcoma, is defined by the presence of pleomorphic cells with large, lobate hyperchromatic nuclei and multipolar mitotic figures. These tumors display high heterogeneity and extremely poor differentiation. The pleomorphic cells may be diffuse or localized, with the diffuse variation
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1. Myogenin, in particular, has been shown to be highly specific to RMS, although the diagnostic significance of each protein marker may vary depending on the type and location of the malignant cells. The alveolar type of RMS tends to have stronger muscle-specific protein staining. Electron
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aimed at studying the presentation, histology, epidemiology, and treatment of RMS (IRSG I–IV). The ICR system is based on prognostic indicators identified in IRSG I–IV. Pleomorphic rhabdomyosarcoma usually occurs in adults rather than children, and is therefore not included in this system.
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pointing to a positive diagnosis of RMS. Classification into types and subtypes is accomplished through further analysis of cellular morphology (alveolar spacings, presence of cambium layer, aneuploidy, etc.) as well as genetic sequencing of tumor cells. Some genetic markers, such as the
1704:
Folpe, Andrew L.; McKenney, Jesse K.; Bridge, Julia A.; Weiss, Sharon W. (2002-09-01). "Sclerosing rhabdomyosarcoma in adults: report of four cases of a hyalinizing, matrix-rich variant of rhabdomyosarcoma that may be confused with osteosarcoma, chondrosarcoma, or angiosarcoma".
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to rule out metastasis to the meninges. A paratesticular presentation will often require an abdominal CT to rule out local lymph node involvement, and so on. Outcomes are strongly tied to the extent of the disease, and its early mapping is important for treatment planning.
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and mediastinum can become quite large before producing signs and symptoms. Parameningeal tumors may present with cranial nerve dysfunction, symptoms of sinusitis, ear discharge, headaches, and facial pain. Orbital tumors often present with orbital swelling and
539:, and a diagnosis of RMS requires confident elimination of these morphologically similar diseases. The defining diagnostic trait for RMS is confirmation of malignant skeletal muscle differentiation with myogenesis (presenting as a plump, pink cytoplasm) under
170:
Given the difficulty in diagnosing rhabdomyosarcoma, definitive classification of subtypes has proven difficult. As a result, classification systems vary by institute and organization. Rhabdomyosarcoma in the 2020 WHO classification, though, is listed as four
165:
Photomicrograph showing nodules of tumor cells separated by hyalinised fibrous septae (50×, HE stain). Inset: Discohesive large tumor cells with hyperchromatic nucleus and scant cytoplasm (200×, HE stain). The diagnosis was postauricular congenital alveolar
254:), and it has a fascicular, spindled, and leiomyomatous growth pattern with notable rhabdomyoblastic differentiation . It occurs most commonly in the paratesticular region, and the prognosis for this particular form of RMS is excellent with a reported
458:
is associated with many cancers including rhabdomyosarcoma, and approximately 50% of RMS cases have been shown to carry some form of mutation to the P53 gene. Other oncogenes often associated with rhabdomyosarcoma, albeit with less frequency, include
3304:
Cohen, P. R.; Kurzrock, R. (1995-01-01). "Miscellaneous genodermatoses: Beckwith-Wiedemann syndrome, Birt-Hogg-Dube syndrome, familial atypical multiple mole melanoma syndrome, hereditary tylosis, incontinentia pigmenti, and supernumerary nipples".
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Weber-Hall, S.; Anderson, J.; McManus, A.; Abe, S.; Nojima, T.; Pinkerton, R.; Pritchard-Jones, K.; Shipley, J. (1996-07-15). "Gains, losses, and amplification of genomic material in rhabdomyosarcoma analyzed by comparative genomic hybridization".
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Hays, D. M.; Lawrence, W.; Wharam, M.; Newton, W.; Ruymann, F. B.; Beltangady, M.; Maurer, H. M. (1989-01-01). "Primary reexcision for patients with 'microscopic residual' tumor following initial excision of sarcomas of trunk and extremity sites".
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Malkin, D.; Li, F. P.; Strong, L. C.; Fraumeni, J. F.; Nelson, C. E.; Kim, D. H.; Kassel, J.; Gryka, M. A.; Bischoff, F. Z. (1990-11-30). "Germ line p53 mutations in a familial syndrome of breast cancer, sarcomas, and other neoplasms".
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Cessna, M. H.; Zhou, H.; Perkins, S. L.; Tripp, S. R.; Layfield, L.; Daines, C.; Coffin, C. M. (2001-09-01). "Are myogenin and myoD1 expression specific for rhabdomyosarcoma? A study of 150 cases, with emphasis on spindle cell mimics".
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Crist, W. M.; Anderson, J. R.; Meza, J. L.; Fryer, C.; Raney, R. B.; Ruymann, F. B.; Breneman, J.; Qualman, S. J.; Wiener, E. (2001-06-15). "Intergroup rhabdomyosarcoma study-IV: results for patients with nonmetastatic disease".
2040:
Barr, F. G.; Chatten, J.; D'Cruz, C. M.; Wilson, A. E.; Nauta, L. E.; Nycum, L. M.; Biegel, J. A.; Womer, R. B. (1995-02-15). "Molecular assays for chromosomal translocations in the diagnosis of pediatric soft tissue sarcomas".
724:, vincristine, and actinomycin D. These drugs are administered in 9–15 cycles depending on the staging of the disease and other therapies used. Other drug and therapy combinations may also show additional benefit. Addition of
3252:
Smith, A. C.; Squire, J. A.; Thorner, P.; Zielenska, M.; Shuman, C.; Grant, R.; Chitayat, D.; Nishikawa, J. L.; Weksberg, R. (2001-12-01). "Association of alveolar rhabdomyosarcoma with the
Beckwith-Wiedemann syndrome".
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include the lungs, bone marrow, and bones. There are many classification systems for RMS and a variety of defined histological types. Embryonal rhabdomyosarcoma is the most common type and comprises about 60% of cases.
1301:
Newton WA, Gehan EA, Webber BL, et al. (September 1995). "Classification of rhabdomyosarcomas and related sarcomas. Pathologic aspects and proposal for a new classification--an
Intergroup Rhabdomyosarcoma Study".
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link between mutant RAS isoforms and a block of myogenic differentiation has been demonstrated. Furthermore, it has been shown that this differentiation block can be overcome with a clinical stage inhibitor of the
2807:
Admiraal, Rick; van der Paardt, Marcel; Kobes, Jasmijn; Kremer, Leontien CM; Bisogno, Gianni; Merks, Johannes HM (2010-12-08). "High-dose chemotherapy for children and young adults with stage IV rhabdomyosarcoma".
758:, presence of metastasis, site and extent of metastasis, and biological and histopathological characteristics of the tumor cells. Survival after recurrence is poor, and new salvage therapy strategies are needed.
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3780:
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Li, RF; Gupta, M; McCluggage, WG; Ronnett, BM (March 2013). "Embryonal rhabdomyosarcoma (botryoid type) of the uterine corpus and cervix in adult women: report of a case series and review of the literature".
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preventing epigenetic suppression of a microRNA that inhibits PAX3:FOXO1 translation. These findings and ongoing clinical trials (ADVL1513) shows promise for an effective therapy for some patients with aRMS.
2302:
1928:
Barr, F. G.; Galili, N.; Holick, J.; Biegel, J. A.; Rovera, G.; Emanuel, B. S. (1993-02-01). "Rearrangement of the PAX3 paired box gene in the paediatric solid tumour alveolar rhabdomyosarcoma".
344:). Up to 90% of alveolar RMS cases present with a translocations of t(2;13)(q35, q14) or, less commonly, t(1;13)(p36, q15). Both involve the translocation of a DNA binding domain of either
149:
Outcomes vary considerably, with five-year survival rates between 35 and 95%, depending on the type of RMS involved, so clear diagnosis is critical for effective treatment and management.
1554:
Cecchetto, Giovanni; Bisogno, Gianni; De Corti, Federica; Dall'Igna, Patrizia; Inserra, Alessandro; Ferrari, Andrea; Garaventa, Alberto; Scagnellato, Angela; Carli, Modesto (2007-12-01).
3729:
Saboo, S. S.; Krajewski, K. M.; Zukotynski, K.; Howard, S.; Jagannathan, J. P.; Hornick, J. L.; Ramaiya, N. (2012). "Imaging
Features of Primary and Secondary Adult Rhabdomyosarcoma".
1205:
Koscielniak E, Rodary C, Flamant F, et al. (1992). "Metastatic rhabdomyosarcoma and histologically similar tumors in childhood: a retrospective
European multi-center analysis".
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Kelly, K. M.; Womer, R. B.; Sorensen, P. H.; Xiong, Q. B.; Barr, F. G. (1997-05-01). "Common and variant gene fusions predict distinct clinical phenotypes in rhabdomyosarcoma".
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Qualman, S. J.; Coffin, C. M.; Newton, W. A.; Hojo, H.; Triche, T. J.; Parham, D. M.; Crist, W. M. (1998-12-01). "Intergroup
Rhabdomyosarcoma Study: update for pathologists".
2419:
Yohe, Marielle E.; Gryder, Berkley E.; Shern, Jack F.; Song, Young K.; Chou, Hsien-Chao; Sindiri, Sivasish; Mendoza, Arnulfo; Patidar, Rajesh; Zhang, Xiaohu (2018-07-04).
2658:"Pretreatment TNM staging of childhood rhabdomyosarcoma: a report of the Intergroup Rhabdomyosarcoma Study Group. Children's Cancer Study Group. Pediatric Oncology Group"
1657:"Analysis of prognostic factors in patients with nonmetastatic rhabdomyosarcoma treated on intergroup rhabdomyosarcoma studies III and IV: the Children's Oncology Group"
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is usually required to obtain sufficient tissue for accurate diagnosis. All findings must be considered in context, as no one trait is a definitive indicator for RMS.
523:
Rhabdomyosarcoma is often difficult to diagnose due to its similarities to other cancers and varying levels of differentiation. It is loosely classified as one of the
1808:
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activation has been shown to block myogenic differentiation, which could help explain its potential role in rhabdomyosarcogenesis. More recently, a mechanistic and
543:. Cross striations may or may not be present. Accurate diagnosis is usually accomplished through immunohistochemical staining for muscle-specific proteins such as
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121:
It is generally considered to be a disease of childhood, as the vast majority of cases occur in those below the age of 18. It is commonly described as one of the
2132:
Gryder, Berkley E.; Yohe, Marielle E.; Chou, Hsien-Chao; Zhang, Xiaohu; Marques, Joana; Wachtel, Marco; Schaefer, Beat; Sen, Nirmalya; Song, Young (2017-08-01).
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1556:"Biopsy or debulking surgery as initial surgery for locally advanced rhabdomyosarcomas in children?: the experience of the Italian Cooperative Group studies"
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in order to determine the extent of local invasion and any metastasis. Further investigational techniques may be necessary depending on tumor sites. A
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Bisogno, G.; Murgia, A.; Mammi, I.; Strafella, M. S.; Carli, M. (1999-10-01). "Rhabdomyosarcoma in a patient with cardio-facio-cutaneous syndrome".
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at super enhancers to support cancer growth. Furthermore, it was demonstrated that FP-RMS subtypes were especially sensitive to inhibitors (such as
2943:
Healey, E. A.; Shamberger, R. C.; Grier, H. E.; Loeffler, J. S.; Tarbell, N. J. (1995-05-15). "A 10-year experience of pediatric brachytherapy".
1245:"Disease patterns and survival rate in children with metastatic soft-tissue sarcoma. A report from the Intergroup Rhabdomyosarcoma Study (IRS)-I"
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fusion product, which is indicative of classic cystic ARMS. Cases of FP-RMS are associated with a poorer prognosis than fusion-negative RMS.
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705:
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Ruymann, F. B.; Grovas, A. C. (2000-01-01). "Progress in the diagnosis and treatment of rhabdomyosarcoma and related soft tissue sarcomas".
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metastatic rhabdomyoscarcoma, a Cochrane review has found no evidence to support the use of high-dose chemotherapy as a standard therapy.
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has a demonstrated role in muscle cell development, which supports its potential role in RMS. The t(2;13) translocation can result in the
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The fusion protein presents a potential therapeutic target, and in recent years more research has been conducted to clarify the role of
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Rhabdomyosarcoma is difficult to diagnose. Risk factors that increase the likelihood of this cancer include inherited disorders such as
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Gripp, Karen W.; Scott, Charles I.; Nicholson, Linda; McDonald-McGinn, Donna M.; Ozeran, J. Daniel; Jones, Marilyn C.; Lin, Angela E.;
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Prognosis in rhabdomyosarcoma patients has been shown to be dependent on age, tumor site, resectability of tumor, tumor size, regional
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Pedrick, T. J.; Donaldson, S. S.; Cox, R. S. (1986-03-01). "Rhabdomyosarcoma: the Stanford experience using a TNM staging system".
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Treatment of rhabdomyosarcoma is a multidisciplinary practice involving the use of surgery, chemotherapy, radiation, and possibly
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in FP-RMS. PAX3-FOXO1 is now known to drive key oncogenes such as MYC and MYCN by creating long-distance genetic interactions by
3681:"Survivin-responsive conditionally replicating adenovirus kills rhabdomyosarcoma stem cells more efficiently than their progeny"
198:
the botryoid variant are defined by a dense tumor layer under an epithelium (cambium layer). This subtype has a good prognosis.
114:, and spindle-cell/sclerosing rhabdomyosarcoma. Embryonal and alveolar are the main groups, and these types are the most common
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1162:
Pappo, A. S.; Shapiro, D. N.; Crist, W. M.; Maurer, H. M. (1995-08-01). "Biology and therapy of pediatric rhabdomyosarcoma".
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Stiller, C. A.; Parkint, D. M. (1994-01-01). "International variations in the incidence of childhood soft-tissue sarcomas".
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3455:(2002-02-15). "Five additional Costello syndrome patients with rhabdomyosarcoma: proposal for a tumor screening protocol".
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3574:"Anatomische Untersuchung einer hypertrophischen Zunge nebst Bemerkungen über die Neubildung quergestreifter Muskelfasern"
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Maurer HM, Beltangady M, Gehan EA, et al. (January 1988). "The Intergroup Rhabdomyosarcoma Study-I. A final report".
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Visser, M.; Sijmons, C.; Bras, J.; Arceci, R. J.; Godfried, M.; Valentijn, L. J.; Voûte, P. A.; Baas, F. (1997-09-01).
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Yang, P.; Grufferman, S.; Khoury, M. J.; Schwartz, A. G.; Kowalski, J.; Ruymann, F. B.; Maurer, H. M. (1995-01-01).
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Kallen, ME; Hornick, JL (January 2021). "The 2020 WHO Classification: What's New in Soft Tissue Tumor Pathology?".
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369:, a member of the forkhead/HNF-3 transcription factor family. The t(2;13) translocation results in a fusion of the
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2596:"Myogenin is a specific marker for rhabdomyosarcoma: an immunohistochemical study in paraffin-embedded tissues"
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Meza, Jane L.; Anderson, James; Pappo, Alberto S.; Meyer, William H.; Children's Oncology Group (2006-08-20).
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3099:"Childhood cancer and ethnic group in Britain: a United Kingdom children's Cancer Study Group (UKCCSG) study"
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The current staging system for rhabdomyosarcoma is unusual relative to most cancers. It utilizes a modified
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has been suggested as their marker. Preclinical animal studies that try to use conditionally replicating
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1885:"Fusion of PAX7 to FKHR by the Variant t(1;13)(p36;q14) Translocation in Alveolar Rhabdomyosarcoma"
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Cancer Institute (NCI)-funded cooperative that has become a part of the Children's Oncology Group.
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Botryoid rhabdomyosarcoma is also sometimes present in adult women, found in the cervix or uterus.
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3500:"Parents' use of cocaine and marijuana and increased risk of rhabdomyosarcoma in their children"
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due to its appearance on an H&E stain. Other cancers that share this classification include
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424:(a transcription factor highly expressed in all RMS subtypes) but also (2) co-binds with these
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Arndt CA, Crist WM (July 1999). "Common musculoskeletal tumors of childhood and adolescence".
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3027:"Trends in childhood rhabdomyosarcoma incidence and survival in the United States, 1975–2005"
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3199:"Association of childhood rhabdomyosarcoma with neurofibromatosis type I and birth defects"
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Oliner, J. D.; Kinzler, K. W.; Meltzer, P. S.; George, D. L.; Vogelstein, B. (1992-07-02).
2134:"PAX3–FOXO1 Establishes Myogenic Super Enhancers and Confers BET Bromodomain Vulnerability"
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One study showed that 35% of embryonal RMS tumors contained activating mutations in either
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Stiller, C. A.; McKinney, P. A.; Bunch, K. J.; Bailey, C. C.; Lewis, I. J. (1991-09-01).
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Ognjanovic, Simona; Linabery, Amy M.; Charbonneau, Bridget; Ross, Julie A. (2009-09-15).
2421:"MEK inhibition induces MYOG and remodels super-enhancers in RAS-driven rhabdomyosarcoma"
965:"Diagnosis of the small round blue cell tumors using multiplex polymerase chain reaction"
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a member of the Paired Box family of transcription factors, to a transactivation site on
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Please help update this article to reflect recent events or newly available information.
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Merlino, G.; Helman, L. J. (1999-09-20). "Rhabdomyosarcoma—working out the pathways".
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2625:
2617:
2568:
2560:
2556:
2524:
2516:
2470:
2458:
2440:
2390:
2382:
2341:
2333:
2286:
2220:
2212:
2171:
2153:
2109:
2101:
2066:
2058:
2011:
2003:
1953:
1945:
1896:
1862:
1782:
1774:
1730:
1722:
1718:
1686:
1678:
1637:
1629:
1585:
1577:
1525:
1446:
1428:
1379:
1371:
1319:
1266:
1222:
1187:
1179:
1133:
1084:
1049:
994:
929:
910:
898:
808:
788:
736:
631:
Head and neck (orbit), biliary tract, genitourinary (excluding bladder and prostate)
598:
316:
55:
3597:
3547:
3342:
3238:
2885:
2793:
2699:
2637:
2580:
2402:
2023:
1597:
1537:
1391:
1331:
1278:
1145:
1096:
4637:
4326:
4264:
4200:
3738:
3702:
3692:
3651:
3635:
3585:
3511:
3464:
3417:
3374:
3314:
3290:
3262:
3210:
3163:
3126:
3110:
3054:
3038:
2952:
2909:
2857:
2817:
2813:
2765:
2722:
2669:
2607:
2552:
2506:
2448:
2432:
2372:
2298:
2278:
2270:
2232:
2202:
2161:
2145:
2093:
2050:
1995:
1937:
1852:
1842:
1766:
1742:
1714:
1668:
1621:
1567:
1517:
1436:
1418:
1363:
1311:
1256:
1214:
1171:
1123:
1076:
1041:
984:
976:
890:
717:
129:. Despite being relatively rare, it accounts for approximately 40% of all recorded
80:
42:
of head showing a large mass without any intracranial extension. The diagnosis was
3899:
1965:
282:
depending on the site of the primary tumor. Genitourinary tumors may present with
4553:
4417:
4245:
3842:
2511:
2494:
2436:
2149:
1521:
894:
606:
586:
320:
88:
1770:
1045:
4578:
4568:
4486:
4471:
4406:
4317:
4255:
4235:
3913:
3902:
3853:
2990:
2359:
Epstein, J. A.; Lam, P.; Jepeal, L.; Maas, R. L.; Shapiro, D. N. (1995-05-19).
2097:
1478:
1316:
10.1002/1097-0142(19950915)76:6<1073::aid-cncr2820760624>3.0.co;2-l
1262:
10.1002/1097-0142(19881001)62:7<1257::aid-cncr2820620703>3.0.co;2-k
1175:
1129:
10.1002/1097-0142(19930301)71:5<1904::aid-cncr2820710530>3.0.co;2-x
980:
564:
microscopy may also aid in diagnosis, with the presence of actin and myosin or
409:
247:
118:
of childhood and adolescence. The pleomorphic type is usually found in adults.
96:
2862:
10.1002/1097-0142(19870101)59:1<147::aid-cncr2820590129>3.0.co;2-8
2769:
2612:
2595:
1081:
10.1002/1097-0142(19880115)61:2<209::aid-cncr2820610202>3.0.co;2-l
4616:
3647:
3523:
3498:
Grufferman, S.; Schwartz, A. G.; Ruymann, F. B.; Maurer, H. M. (1993-05-01).
3476:
3452:
3429:
3386:
3326:
3274:
3266:
3222:
3175:
3122:
3050:
2964:
2921:
2869:
2825:
2777:
2734:
2726:
2683:
2675:
10.1002/(sici)1097-0142(19970915)80:6<1165::aid-cncr21>3.0.co;2-5
2621:
2594:
Kumar, S.; Perlman, E.; Harris, C. A.; Raffeld, M.; Tsokos, M. (2000-09-01).
2564:
2520:
2444:
2386:
2377:
2360:
2337:
2248:"Amplification of a gene encoding a p53-associated protein in human sarcomas"
2216:
2157:
2105:
2062:
2007:
1949:
1778:
1726:
1682:
1673:
1656:
1633:
1581:
1432:
1375:
1183:
742:
532:
528:
512:
3789:
3697:
3378:
3214:
1847:
4596:
4240:
4142:
4061:
4034:
4002:
3750:
3716:
3665:
3578:
Archiv für Pathologische Anatomie und Physiologie und für Klinische Medicin
3484:
3437:
3282:
3068:
3000:
2929:
2833:
2785:
2629:
2572:
2528:
2462:
2207:
2190:
2175:
2054:
2015:
1999:
1866:
1786:
1734:
1690:
1589:
1529:
1450:
1218:
1053:
998:
902:
816:
713:
701:
565:
292:
126:
3539:
3394:
3334:
3230:
3183:
3140:
2972:
2877:
2742:
2691:
2394:
2345:
2290:
2224:
2113:
2070:
1957:
1900:
1641:
1383:
1367:
1323:
1270:
1226:
1191:
1137:
1088:
791:. It has also been associated with parental use of cocaine and marijuana.
4588:
4426:
4300:
4295:
4195:
3971:
3883:
3114:
2282:
829:
725:
709:
574:
503:
175:
subtypes: embryonal, alveolar, pleomorphic, and spindle-cell/sclerosing.
39:
3772:
3742:
2992:
Childhood Rhabdomyosarcoma Treatment (PDQ®): Health Professional Version
4533:
4382:
3908:
3589:
3531:
3515:
3468:
3042:
2988:
2900:
Rhabdomyosarcoma Study Group protocols IRS-II through –IV, 1978–1997".
1941:
1572:
1555:
755:
721:
540:
142:
2656:
Lawrence, W.; Anderson, J. R.; Gehan, E. A.; Maurer, H. (1997-09-15).
141:
is closely tied to the location of the primary tumor. Common sites of
4459:
4402:
4351:
3894:
3859:
2913:
2274:
1553:
729:
283:
224:
172:
138:
3573:
3198:
3974:
3919:
2361:"Pax3 inhibits myogenic differentiation of cultured myoblast cells"
1423:
672:
https://www.worldcommunitygrid.org/about_us/article.s?articleId=777
602:
544:
536:
448:
92:
60:
3450:
4057:
4030:
3978:
3864:
3024:
2806:
767:
412:. In this context, PAX3-FOXO1 both (1) drives the expression of
263:
84:
3831:
1407:"Recent advances in understanding and managing rhabdomyosarcoma"
4291:
4191:
4183:
3784:
3497:
556:
552:
76:
3966:
3728:
573:
fusion gene expression in alveolar RMS, can aid in diagnosis.
4378:
2945:
International Journal of Radiation Oncology, Biology, Physics
2942:
1110:
Maurer HM, Gehan EA, Beltangady M, et al. (March 1993).
639:
Cranial parameningial, bladder, extremities, prostate, other
548:
485:
389:
377:
361:
3196:
2322:
585:
Following diagnosis and histopathological analysis, various
3407:
3251:
2655:
2039:
1883:
Davis RJ, D'Cruz CM, Lovell MA, Biegel JA, Barr FG (1994).
1882:
928:(Tenth ed.). Philadelphia, Pennsylvania. p. 830.
560:
473:
461:
433:
383:
371:
352:
346:
3096:
2245:
1243:
Raney RB, Tefft M, Maurer HM, et al. (October 1988).
963:
Chen QR, Vansant G, Oades K, et al. (February 2007).
2593:
590:
479:
455:
429:
381:, while the t(1;13) translocation involves the fusion of
240:
1703:
1610:
1506:
1204:
2188:
2083:
1353:
1161:
161:
31:
4492:
Multiple cutaneous and uterine leiomyomatosis syndrome
3355:
2989:
PDQ Pediatric Treatment Editorial Board (2002-01-01).
2898:
2541:
1927:
1755:
1654:
1549:
1547:
3084:
Principles and practice of pediatric oncology. 3rd ed
2846:
1829:
Robertson, JC; Jorcyk, CL; Oxford, JT (15 May 2018).
1828:
1405:
Hiniker, Susan M.; Donaldson, Sarah S. (2015-01-01).
1109:
1066:
3762:
2358:
924:
Kumar, Vinay; Abbas, Abul K.; Aster, Jon C. (2018).
447:(p11,15.5). This region is associated with multiple
3086:. Philadelphia: Lippincott-Raven. pp. 799–829.
2712:
2418:
2131:
1831:"DICER1 Syndrome: DICER1 Mutations in Rare Cancers"
1544:
223:(undifferentiated rhabdomyosarcoma), also known as
2995:. Bethesda (MD): National Cancer Institute (US).
1300:
4614:
1404:
1242:
962:
766:Rhabdomyosarcoma is the most common soft-tissue
16:Cancer originating in precursors to muscle cells
3672:
923:
3153:
3952:
3303:
2755:
1985:
880:
811:of rhabdomyosarcoma have been identified and
83:that have failed to fully differentiate into
3081:
2492:
3959:
3945:
3624:"Rhabdomyosarcoma of the Skeletal Muscles"
2545:The American Journal of Surgical Pathology
2191:"Allelotype of pediatric rhabdomyosarcoma"
1707:The American Journal of Surgical Pathology
1510:The American Journal of Surgical Pathology
1485:(6th ed.). Hamilton (ON): BC Decker.
1112:"The Intergroup Rhabdomyosarcoma Study-II"
1031:
948:: CS1 maint: location missing publisher (
917:
883:The American Journal of Surgical Pathology
231:
30:
3706:
3696:
3655:
3130:
3058:
2673:
2611:
2510:
2452:
2376:
2206:
2165:
1856:
1846:
1822:
1672:
1571:
1440:
1422:
1260:
1127:
988:
876:
874:
605:presentation of RMS will often require a
125:of childhood due to its appearance on an
3678:
3410:Journal of Pediatric Hematology/Oncology
1793:
246:This subtype is very similar to that of
243:classification of soft-tissue sarcomas.
237:Spindle-cell/sclerosing rhabdomyosarcoma
160:
2810:Cochrane Database of Systematic Reviews
239:is an added subtype listed in the 2020
4615:
2651:
2649:
2647:
2488:
2486:
2484:
2482:
2480:
2414:
2412:
2127:
2125:
2123:
1923:
1921:
871:
439:Embryonal RMS usually presents with a
3940:
3621:
3571:
3255:Pediatric and Developmental Pathology
3156:Paediatric and Perinatal Epidemiology
2984:
2982:
2493:Dagher, R.; Helman, L. (1999-01-01).
2035:
2033:
1981:
1979:
1977:
1975:
1878:
1876:
1476:
1472:
1470:
1468:
1466:
1464:
1462:
1460:
1356:Pediatric and Developmental Pathology
1349:
1347:
1345:
1343:
1341:
1296:
1294:
1292:
1290:
1288:
1238:
1236:
842:
720:, and the IVA regimen, consisting of
276:
4628:Connective and soft tissue neoplasms
3457:American Journal of Medical Genetics
1157:
1155:
1027:
1025:
1023:
1021:
1019:
969:The Journal of Molecular Diagnostics
652:
432:) of a super enhancer bound protein
189:
3998:Desmoplastic small-round-cell tumor
2644:
2477:
2409:
2365:The Journal of Biological Chemistry
2120:
1918:
1801:"Risk Factors for Rhabdomyosarcoma"
956:
813:fibroblast growth factor receptor 3
13:
3622:Stout, Arthur Purdy (1946-03-01).
3168:10.1111/j.1365-3016.1994.tb00439.x
2979:
2030:
1972:
1873:
1500:
1457:
1338:
1285:
1233:
14:
4654:
4075:Aggressive infantile fibromatosis
3758:
3731:American Journal of Roentgenology
1152:
1016:
75:) is a highly aggressive form of
4564:Clear-cell sarcoma of the kidney
3640:10.1097/00000658-194603000-00011
3422:10.1097/00043426-199909000-00016
2557:10.1097/00000478-200109000-00005
1719:10.1097/00000478-200209000-00008
704:treatment for RMS. There is the
657:
269:and pseudovascular development.
4332:Intradermal spindle cell lipoma
4043:Dermatofibrosarcoma protuberans
3615:
3604:from the original on 2022-11-14
3565:
3554:from the original on 2022-07-03
3491:
3444:
3401:
3349:
3297:
3245:
3190:
3147:
3090:
3082:Wexler, LH; Herman, LJ (1997).
3075:
3018:
3007:from the original on 2022-08-19
2936:
2892:
2840:
2800:
2749:
2706:
2587:
2535:
2495:"Rhabdomyosarcoma: an overview"
2352:
2316:
2305:from the original on 2022-10-16
2239:
2182:
2077:
1907:from the original on 2016-08-10
1811:from the original on 2022-08-06
1749:
1697:
1648:
1604:
1489:from the original on 2022-10-16
1398:
1005:from the original on 2020-02-13
860:from the original on 2020-09-17
785:cardio-facio-cutaneous syndrome
761:
298:
4160:Malignant fibrous histiocytoma
4108:Infantile digital fibromatosis
4094:Familial myxovascular fibromas
4090:Diffuse infantile fibromatosis
2902:Medical and Pediatric Oncology
2818:10.1002/14651858.cd006669.pub2
2425:Science Translational Medicine
1198:
1103:
1060:
700:There are two main methods of
215:
153:to be cured of their disease.
1:
4206:Superficial acral fibromyxoma
4113:Juvenile hyaline fibromatosis
3319:10.1016/S0733-8635(18)30121-9
1626:10.1016/s0022-3468(89)80290-8
835:
749:
4633:Small-blue-round-cell tumors
4507:Solitary cutaneous leiomyoma
4497:Multiple cutaneous leiomyoma
4218:Ossifying fibromyxoid tumour
2957:10.1016/0360-3016(95)00520-9
2715:Journal of Clinical Oncology
2512:10.1634/theoncologist.4-1-34
2437:10.1126/scitranslmed.aan4470
2150:10.1158/2159-8290.CD-16-1297
2086:Journal of Clinical Oncology
1759:Journal of Clinical Oncology
1661:Journal of Clinical Oncology
1614:Journal of Pediatric Surgery
1522:10.1097/PAS.0b013e31826e0271
1483:Holland-Frei Cancer Medicine
1164:Journal of Clinical Oncology
895:10.1097/PAS.0000000000001552
648:
525:small-blue-round-cell tumors
518:
498:and it is worth noting that
426:master transcription factors
221:Pleomorphic rhabdomyosarcoma
178:
123:small-blue-round-cell tumors
112:pleomorphic rhabdomyosarcoma
7:
4155:Benign fibrous histiocytoma
3504:Cancer Causes & Control
1771:10.1200/JCO.2001.19.12.3091
1046:10.1056/NEJM199907293410507
850:"What Is Rhabdomyosarcoma?"
823:
803:
777:Beckwith-Wiedemann syndrome
313:Beckwith-Wiedemann syndrome
204:
10:
4659:
4435:Embryonal rhabdomyosarcoma
4013:Connective tissue neoplasm
2098:10.1200/JCO.1997.15.5.1831
1176:10.1200/JCO.1995.13.8.2123
981:10.2353/jmoldx.2007.060111
794:
580:
443:(LOH) in the short arm of
330:
184:Embryonal rhabdomyosarcoma
104:embryonal rhabdomyosarcoma
4587:
4559:Malignant rhabdoid tumour
4526:Complex mixed and stromal
4524:
4455:
4447:Alveolar rhabdomyosarcoma
4415:
4391:
4369:
4360:
4282:
4254:
4226:
4182:
4141:
4056:
4029:
4020:
4011:
3985:
3874:
3766:
3103:British Journal of Cancer
2770:10.3109/07357900009031827
2613:10.1038/modpathol.3880179
666:This section needs to be
210:Alveolar rhabdomyosarcoma
195:Botryoid rhabdomyosarcoma
108:alveolar rhabdomyosarcoma
54:
48:alveolar rhabdomyosarcoma
38:
29:
24:
4098:Fibroma of tendon sheath
3267:10.1007/s10024001-0110-6
2727:10.1200/JCO.1986.4.3.370
2378:10.1074/jbc.270.20.11719
1674:10.1200/JCO.2005.05.3801
773:neurofibromatosis type 1
454:The loss-of-function of
309:Neurofibromatosis type 1
156:
4128:Oral submucous fibrosis
4070:Aggressive fibromatosis
3986:Not otherwise specified
3698:10.1186/1479-5876-12-27
3379:10.1126/science.1978757
3215:10.1002/gepi.1370120504
1848:10.3390/cancers10050143
926:Robbins basic pathology
670:. The reason given is:
589:may be used, including
256:five-year survival rate
232:Spindle-cell/sclerosing
4170:Solitary fibrous tumor
4165:Atypical fibroxanthoma
2208:10.1038/sj.onc.1201302
2055:10.1001/jama.273.7.553
2000:10.1038/sj.onc.1203038
1219:10.1002/mpo.2950200305
756:lymph node involvement
441:loss of heterozygosity
262:of this subtype has a
167:
102:The four subtypes are
4544:Mixed Müllerian tumor
3679:Tanoue K (Jan 2014).
1368:10.1007/s100249900076
365:(previously known as
164:
4623:Anatomical pathology
4549:Mesoblastic nephroma
4118:Plantar fibromatosis
4048:Desmoplastic fibroma
3307:Dermatologic Clinics
3203:Genetic Epidemiology
3115:10.1038/bjc.1991.347
2758:Cancer Investigation
781:Li–Fraumeni syndrome
626:Risk classification
456:tumor suppressor p53
305:Li-Fraumeni syndrome
252:smooth muscle tissue
131:soft-tissue sarcomas
116:soft tissue sarcomas
40:Non-contrast CT scan
4539:Pleomorphic adenoma
4347:Spindle cell lipoma
4147:histiocytic sarcoma
4123:Pleomorphic fibroma
4085:Collagenous fibroma
4080:Aponeurotic fibroma
3993:Soft-tissue sarcoma
3743:10.2214/AJR.11.8213
3371:1990Sci...250.1233M
3365:(4985): 1233–1238.
2371:(20): 11719–11722.
2267:1992Natur.358...80O
79:that develops from
4502:Neural fibrolipoma
4477:Angiolipoleiomyoma
4440:Sarcoma botryoides
4337:Pleomorphic lipoma
4306:Myxoid liposarcoma
4270:Clear-cell sarcoma
4103:Fibromatosis colli
3875:External resources
3590:10.1007/BF01936232
3572:Weber, CO (1854).
3516:10.1007/bf00051316
3469:10.1002/ajmg.10241
3043:10.1002/cncr.24465
1942:10.1038/ng0293-113
1573:10.1002/cncr.23079
1479:"Rhabdomyosarcoma"
1477:Meyer, WH (2003).
587:imaging techniques
547:, muscle-specific
509:MAP kinase pathway
340:rhabdomyosarcoma (
277:Signs and symptoms
168:
95:are identified as
4610:
4609:
4602:Adenomatoid tumor
4574:Pancreatoblastoma
4520:
4519:
4482:Genital leiomyoma
4342:Lipoblastomatosis
4278:
4277:
4178:
4177:
4133:Pachydermodactyly
3934:
3933:
3628:Annals of Surgery
3453:Zackai, Elaine H.
3037:(18): 4218–4226.
2431:(448): eaan4470.
2332:(14): 3220–3224.
2201:(11): 1309–1314.
1994:(38): 5340–5348.
1895:(11): 2869–2872.
1765:(12): 3091–3102.
1667:(24): 3844–3851.
1566:(11): 2561–2567.
1207:Med Pediatr Oncol
809:Cancer stem cells
789:Costello syndrome
737:Radiation therapy
691:
690:
646:
645:
317:Costello syndrome
260:sclerosing aspect
190:Embryonal subtype
166:rhabdomyosarcoma.
81:mesenchymal cells
66:
65:
19:Medical condition
4650:
4431:rhabdomyosarcoma
4420:
4396:
4372:
4367:
4366:
4327:Chondroid lipoma
4265:Synovial sarcoma
4201:Cutaneous myxoma
4027:
4026:
4018:
4017:
3961:
3954:
3947:
3938:
3937:
3914:Rhabdomyosarcoma
3764:
3763:
3754:
3737:(6): W694–W703.
3721:
3720:
3710:
3700:
3676:
3670:
3669:
3659:
3619:
3613:
3612:
3610:
3609:
3569:
3563:
3562:
3560:
3559:
3495:
3489:
3488:
3448:
3442:
3441:
3405:
3399:
3398:
3353:
3347:
3346:
3301:
3295:
3294:
3249:
3243:
3242:
3194:
3188:
3187:
3151:
3145:
3144:
3134:
3094:
3088:
3087:
3079:
3073:
3072:
3062:
3022:
3016:
3015:
3013:
3012:
2986:
2977:
2976:
2940:
2934:
2933:
2914:10.1002/mpo.1259
2896:
2890:
2889:
2844:
2838:
2837:
2812:(12): CD006669.
2804:
2798:
2797:
2753:
2747:
2746:
2710:
2704:
2703:
2677:
2668:(6): 1165–1170.
2653:
2642:
2641:
2615:
2600:Modern Pathology
2591:
2585:
2584:
2551:(9): 1150–1157.
2539:
2533:
2532:
2514:
2490:
2475:
2474:
2456:
2416:
2407:
2406:
2380:
2356:
2350:
2349:
2320:
2314:
2313:
2311:
2310:
2275:10.1038/358080a0
2252:
2243:
2237:
2236:
2210:
2186:
2180:
2179:
2169:
2138:Cancer Discovery
2129:
2118:
2117:
2092:(5): 1831–1836.
2081:
2075:
2074:
2037:
2028:
2027:
1983:
1970:
1969:
1925:
1916:
1915:
1913:
1912:
1880:
1871:
1870:
1860:
1850:
1826:
1820:
1819:
1817:
1816:
1797:
1791:
1790:
1753:
1747:
1746:
1713:(9): 1175–1183.
1701:
1695:
1694:
1676:
1652:
1646:
1645:
1608:
1602:
1601:
1575:
1551:
1542:
1541:
1504:
1498:
1497:
1495:
1494:
1474:
1455:
1454:
1444:
1426:
1402:
1396:
1395:
1351:
1336:
1335:
1298:
1283:
1282:
1264:
1240:
1231:
1230:
1202:
1196:
1195:
1170:(8): 2123–2139.
1159:
1150:
1149:
1131:
1107:
1101:
1100:
1064:
1058:
1057:
1029:
1014:
1013:
1011:
1010:
992:
960:
954:
953:
947:
939:
921:
915:
914:
878:
869:
868:
866:
865:
846:
718:cyclophosphamide
708:, consisting of
686:
683:
677:
661:
660:
653:
620:
619:
541:light microscopy
137:tumor site, and
69:Rhabdomyosarcoma
34:
25:Rhabdomyosarcoma
22:
21:
4658:
4657:
4653:
4652:
4651:
4649:
4648:
4647:
4613:
4612:
4611:
4606:
4583:
4516:
4451:
4418:Skeletal muscle
4416:
4411:
4392:
4387:
4370:
4356:
4274:
4250:
4246:Phyllodes tumor
4228:Fibroepithelial
4222:
4174:
4137:
4052:
4007:
3981:
3965:
3935:
3930:
3929:
3870:
3869:
3775:
3761:
3725:
3724:
3677:
3673:
3620:
3616:
3607:
3605:
3570:
3566:
3557:
3555:
3496:
3492:
3449:
3445:
3406:
3402:
3354:
3350:
3302:
3298:
3250:
3246:
3195:
3191:
3152:
3148:
3095:
3091:
3080:
3076:
3023:
3019:
3010:
3008:
2987:
2980:
2941:
2937:
2897:
2893:
2845:
2841:
2805:
2801:
2754:
2750:
2711:
2707:
2654:
2645:
2592:
2588:
2540:
2536:
2491:
2478:
2417:
2410:
2357:
2353:
2326:Cancer Research
2321:
2317:
2308:
2306:
2261:(6381): 80–83.
2250:
2244:
2240:
2187:
2183:
2130:
2121:
2082:
2078:
2038:
2031:
1984:
1973:
1930:Nature Genetics
1926:
1919:
1910:
1908:
1889:Cancer Research
1881:
1874:
1827:
1823:
1814:
1812:
1799:
1798:
1794:
1754:
1750:
1702:
1698:
1653:
1649:
1609:
1605:
1552:
1545:
1505:
1501:
1492:
1490:
1475:
1458:
1403:
1399:
1352:
1339:
1299:
1286:
1241:
1234:
1203:
1199:
1160:
1153:
1108:
1104:
1065:
1061:
1030:
1017:
1008:
1006:
961:
957:
941:
940:
936:
922:
918:
879:
872:
863:
861:
848:
847:
843:
838:
826:
806:
797:
764:
752:
687:
681:
678:
675:
662:
658:
651:
607:lumbar puncture
583:
521:
410:super enhancers
338:Fusion Positive
333:
325:DICER1 syndrome
321:Noonan syndrome
301:
288:retroperitoneum
279:
250:(cancer of the
234:
218:
207:
192:
181:
159:
97:rhabdomyoblasts
91:. Cells of the
89:skeletal muscle
20:
17:
12:
11:
5:
4656:
4646:
4645:
4640:
4635:
4630:
4625:
4608:
4607:
4605:
4604:
4599:
4593:
4591:
4585:
4584:
4582:
4581:
4579:Carcinosarcoma
4576:
4571:
4569:Hepatoblastoma
4566:
4561:
4556:
4551:
4546:
4541:
4536:
4530:
4528:
4522:
4521:
4518:
4517:
4515:
4514:
4509:
4504:
4499:
4494:
4489:
4487:Leiomyosarcoma
4484:
4479:
4474:
4472:Angioleiomyoma
4469:
4468:
4467:
4456:
4453:
4452:
4450:
4449:
4444:
4443:
4442:
4423:
4421:
4413:
4412:
4410:
4409:
4407:leiomyosarcoma
4399:
4397:
4389:
4388:
4386:
4385:
4375:
4373:
4364:
4358:
4357:
4355:
4354:
4349:
4344:
4339:
4334:
4329:
4323:
4322:
4321:
4320:
4318:Angiomyolipoma
4310:
4309:
4308:
4303:
4288:
4286:
4280:
4279:
4276:
4275:
4273:
4272:
4267:
4261:
4259:
4252:
4251:
4249:
4248:
4243:
4238:
4236:Brenner tumour
4232:
4230:
4224:
4223:
4221:
4220:
4215:
4210:
4209:
4208:
4203:
4188:
4186:
4180:
4179:
4176:
4175:
4173:
4172:
4167:
4162:
4157:
4151:
4149:
4139:
4138:
4136:
4135:
4130:
4125:
4120:
4115:
4110:
4105:
4100:
4095:
4092:
4087:
4082:
4077:
4072:
4066:
4064:
4054:
4053:
4051:
4050:
4045:
4039:
4037:
4024:
4015:
4009:
4008:
4006:
4005:
4000:
3995:
3989:
3987:
3983:
3982:
3964:
3963:
3956:
3949:
3941:
3932:
3931:
3928:
3927:
3916:
3905:
3891:
3879:
3878:
3876:
3872:
3871:
3868:
3867:
3856:
3845:
3834:
3823:
3808:
3793:
3776:
3771:
3770:
3768:
3767:Classification
3760:
3759:External links
3757:
3756:
3755:
3723:
3722:
3671:
3634:(3): 447–472.
3614:
3564:
3510:(3): 217–224.
3490:
3443:
3416:(5): 424–427.
3400:
3348:
3313:(1): 211–229.
3296:
3261:(6): 550–558.
3244:
3209:(5): 467–474.
3189:
3162:(1): 107–119.
3146:
3109:(3): 543–548.
3089:
3074:
3017:
2978:
2951:(2): 451–455.
2935:
2891:
2856:(1): 147–155.
2839:
2799:
2764:(3): 223–241.
2748:
2721:(3): 370–378.
2705:
2643:
2606:(9): 988–993.
2586:
2534:
2499:The Oncologist
2476:
2408:
2351:
2315:
2238:
2181:
2144:(8): 884–899.
2119:
2076:
2049:(7): 553–557.
2029:
1971:
1936:(2): 113–117.
1917:
1872:
1821:
1805:www.cancer.org
1792:
1748:
1696:
1647:
1603:
1543:
1499:
1456:
1424:10.12703/P7-59
1411:F1000Prime Rep
1397:
1362:(6): 550–561.
1337:
1310:(6): 1073–85.
1284:
1255:(7): 1257–66.
1232:
1197:
1151:
1122:(5): 1904–22.
1102:
1059:
1015:
955:
934:
916:
870:
854:www.cancer.org
840:
839:
837:
834:
833:
832:
825:
822:
805:
802:
796:
793:
763:
760:
751:
748:
689:
688:
665:
663:
656:
650:
647:
644:
643:
640:
636:
635:
632:
628:
627:
624:
582:
579:
520:
517:
332:
329:
300:
297:
278:
275:
248:leiomyosarcoma
233:
230:
217:
214:
206:
203:
191:
188:
180:
177:
158:
155:
64:
63:
58:
52:
51:
44:post-auricular
36:
35:
27:
26:
18:
15:
9:
6:
4:
3:
2:
4655:
4644:
4641:
4639:
4636:
4634:
4631:
4629:
4626:
4624:
4621:
4620:
4618:
4603:
4600:
4598:
4595:
4594:
4592:
4590:
4586:
4580:
4577:
4575:
4572:
4570:
4567:
4565:
4562:
4560:
4557:
4555:
4552:
4550:
4547:
4545:
4542:
4540:
4537:
4535:
4532:
4531:
4529:
4527:
4523:
4513:
4510:
4508:
4505:
4503:
4500:
4498:
4495:
4493:
4490:
4488:
4485:
4483:
4480:
4478:
4475:
4473:
4470:
4466:
4463:
4462:
4461:
4458:
4457:
4454:
4448:
4445:
4441:
4438:
4437:
4436:
4432:
4428:
4425:
4424:
4422:
4419:
4414:
4408:
4404:
4401:
4400:
4398:
4395:
4394:Smooth muscle
4390:
4384:
4380:
4377:
4376:
4374:
4368:
4365:
4363:
4359:
4353:
4350:
4348:
4345:
4343:
4340:
4338:
4335:
4333:
4330:
4328:
4325:
4324:
4319:
4316:
4315:
4314:
4311:
4307:
4304:
4302:
4299:
4298:
4297:
4293:
4290:
4289:
4287:
4285:
4281:
4271:
4268:
4266:
4263:
4262:
4260:
4257:
4253:
4247:
4244:
4242:
4239:
4237:
4234:
4233:
4231:
4229:
4225:
4219:
4216:
4214:
4211:
4207:
4204:
4202:
4199:
4198:
4197:
4193:
4190:
4189:
4187:
4185:
4181:
4171:
4168:
4166:
4163:
4161:
4158:
4156:
4153:
4152:
4150:
4148:
4144:
4140:
4134:
4131:
4129:
4126:
4124:
4121:
4119:
4116:
4114:
4111:
4109:
4106:
4104:
4101:
4099:
4096:
4093:
4091:
4088:
4086:
4083:
4081:
4078:
4076:
4073:
4071:
4068:
4067:
4065:
4063:
4059:
4055:
4049:
4046:
4044:
4041:
4040:
4038:
4036:
4032:
4028:
4025:
4023:
4019:
4016:
4014:
4010:
4004:
4001:
3999:
3996:
3994:
3991:
3990:
3988:
3984:
3980:
3976:
3973:
3969:
3962:
3957:
3955:
3950:
3948:
3943:
3942:
3939:
3926:
3922:
3921:
3917:
3915:
3911:
3910:
3906:
3904:
3901:
3897:
3896:
3892:
3890:
3886:
3885:
3881:
3880:
3877:
3873:
3866:
3862:
3861:
3857:
3855:
3851:
3850:
3846:
3844:
3840:
3839:
3835:
3833:
3829:
3828:
3824:
3822:
3818:
3814:
3813:
3809:
3807:
3803:
3802:
3798:
3794:
3791:
3787:
3786:
3782:
3778:
3777:
3774:
3769:
3765:
3752:
3748:
3744:
3740:
3736:
3732:
3727:
3726:
3718:
3714:
3709:
3704:
3699:
3694:
3690:
3686:
3682:
3675:
3667:
3663:
3658:
3653:
3649:
3645:
3641:
3637:
3633:
3629:
3625:
3618:
3603:
3599:
3595:
3591:
3587:
3583:
3579:
3575:
3568:
3553:
3549:
3545:
3541:
3537:
3533:
3529:
3525:
3521:
3517:
3513:
3509:
3505:
3501:
3494:
3486:
3482:
3478:
3474:
3470:
3466:
3462:
3458:
3454:
3447:
3439:
3435:
3431:
3427:
3423:
3419:
3415:
3411:
3404:
3396:
3392:
3388:
3384:
3380:
3376:
3372:
3368:
3364:
3360:
3352:
3344:
3340:
3336:
3332:
3328:
3324:
3320:
3316:
3312:
3308:
3300:
3292:
3288:
3284:
3280:
3276:
3272:
3268:
3264:
3260:
3256:
3248:
3240:
3236:
3232:
3228:
3224:
3220:
3216:
3212:
3208:
3204:
3200:
3193:
3185:
3181:
3177:
3173:
3169:
3165:
3161:
3157:
3150:
3142:
3138:
3133:
3128:
3124:
3120:
3116:
3112:
3108:
3104:
3100:
3093:
3085:
3078:
3070:
3066:
3061:
3056:
3052:
3048:
3044:
3040:
3036:
3032:
3028:
3021:
3006:
3002:
2998:
2994:
2993:
2985:
2983:
2974:
2970:
2966:
2962:
2958:
2954:
2950:
2946:
2939:
2931:
2927:
2923:
2919:
2915:
2911:
2907:
2903:
2895:
2887:
2883:
2879:
2875:
2871:
2867:
2863:
2859:
2855:
2851:
2843:
2835:
2831:
2827:
2823:
2819:
2815:
2811:
2803:
2795:
2791:
2787:
2783:
2779:
2775:
2771:
2767:
2763:
2759:
2752:
2744:
2740:
2736:
2732:
2728:
2724:
2720:
2716:
2709:
2701:
2697:
2693:
2689:
2685:
2681:
2676:
2671:
2667:
2663:
2659:
2652:
2650:
2648:
2639:
2635:
2631:
2627:
2623:
2619:
2614:
2609:
2605:
2601:
2597:
2590:
2582:
2578:
2574:
2570:
2566:
2562:
2558:
2554:
2550:
2546:
2538:
2530:
2526:
2522:
2518:
2513:
2508:
2504:
2500:
2496:
2489:
2487:
2485:
2483:
2481:
2472:
2468:
2464:
2460:
2455:
2450:
2446:
2442:
2438:
2434:
2430:
2426:
2422:
2415:
2413:
2404:
2400:
2396:
2392:
2388:
2384:
2379:
2374:
2370:
2366:
2362:
2355:
2347:
2343:
2339:
2335:
2331:
2327:
2319:
2304:
2300:
2296:
2292:
2288:
2284:
2283:2027.42/62637
2280:
2276:
2272:
2268:
2264:
2260:
2256:
2249:
2242:
2234:
2230:
2226:
2222:
2218:
2214:
2209:
2204:
2200:
2196:
2192:
2185:
2177:
2173:
2168:
2163:
2159:
2155:
2151:
2147:
2143:
2139:
2135:
2128:
2126:
2124:
2115:
2111:
2107:
2103:
2099:
2095:
2091:
2087:
2080:
2072:
2068:
2064:
2060:
2056:
2052:
2048:
2044:
2036:
2034:
2025:
2021:
2017:
2013:
2009:
2005:
2001:
1997:
1993:
1989:
1982:
1980:
1978:
1976:
1967:
1963:
1959:
1955:
1951:
1947:
1943:
1939:
1935:
1931:
1924:
1922:
1906:
1902:
1898:
1894:
1890:
1886:
1879:
1877:
1868:
1864:
1859:
1854:
1849:
1844:
1840:
1836:
1832:
1825:
1810:
1806:
1802:
1796:
1788:
1784:
1780:
1776:
1772:
1768:
1764:
1760:
1752:
1744:
1740:
1736:
1732:
1728:
1724:
1720:
1716:
1712:
1708:
1700:
1692:
1688:
1684:
1680:
1675:
1670:
1666:
1662:
1658:
1651:
1643:
1639:
1635:
1631:
1627:
1623:
1619:
1615:
1607:
1599:
1595:
1591:
1587:
1583:
1579:
1574:
1569:
1565:
1561:
1557:
1550:
1548:
1539:
1535:
1531:
1527:
1523:
1519:
1516:(3): 344–55.
1515:
1511:
1503:
1488:
1484:
1480:
1473:
1471:
1469:
1467:
1465:
1463:
1461:
1452:
1448:
1443:
1438:
1434:
1430:
1425:
1420:
1416:
1412:
1408:
1401:
1393:
1389:
1385:
1381:
1377:
1373:
1369:
1365:
1361:
1357:
1350:
1348:
1346:
1344:
1342:
1333:
1329:
1325:
1321:
1317:
1313:
1309:
1305:
1297:
1295:
1293:
1291:
1289:
1280:
1276:
1272:
1268:
1263:
1258:
1254:
1250:
1246:
1239:
1237:
1228:
1224:
1220:
1216:
1213:(3): 209–14.
1212:
1208:
1201:
1193:
1189:
1185:
1181:
1177:
1173:
1169:
1165:
1158:
1156:
1147:
1143:
1139:
1135:
1130:
1125:
1121:
1117:
1113:
1106:
1098:
1094:
1090:
1086:
1082:
1078:
1075:(2): 209–20.
1074:
1070:
1063:
1055:
1051:
1047:
1043:
1040:(5): 342–52.
1039:
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4643:Rare cancers
4597:Mesothelioma
4554:Wilms' tumor
4430:
4241:Fibroadenoma
4143:Histiocytoma
4062:fibromatosis
4035:fibrosarcoma
4003:Skin sarcoma
3918:
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173:histological
169:
151:
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135:
120:
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72:
68:
67:
4589:Mesothelial
4427:Rhabdomyoma
4301:Myelolipoma
4296:liposarcoma
4213:Angiomyxoma
4196:myxosarcoma
4022:Fibromatous
3972:soft tissue
3884:MedlinePlus
3584:: 115–125.
1620:(1): 5–10.
975:(1): 80–8.
830:Rhabdomyoma
726:doxorubicin
710:vincristine
706:VAC regimen
682:August 2023
623:Tumor site
575:Open biopsy
511:known as a
216:Pleomorphic
46:congenital
4617:Categories
4534:Adenomyoma
4383:myosarcoma
4284:Lipomatous
4184:Myxomatous
3968:Connective
3909:Patient UK
3849:DiseasesDB
3608:2022-11-14
3558:2020-03-29
3011:2017-09-10
2309:2019-09-16
1911:2016-05-23
1841:(5): 143.
1815:2021-02-11
1493:2017-09-10
1009:2021-02-07
864:2021-02-11
836:References
750:Prognostic
722:ifosfamide
634:Favorable
595:ultrasound
504:epigenetic
406:PAX3-FOXO1
375:gene with
225:anaplastic
143:metastasis
4465:Cutaneous
4460:Leiomyoma
4403:Leiomyoma
4362:Myomatous
4352:Hibernoma
3895:eMedicine
3860:SNOMED CT
3648:0003-4932
3524:0957-5243
3477:0148-7299
3430:1077-4114
3387:0036-8075
3327:0733-8635
3275:1093-5266
3223:0741-0395
3176:1365-3016
3123:0007-0920
3051:0008-543X
2965:0360-3016
2922:0098-1532
2870:0008-543X
2826:1465-1858
2778:0735-7907
2735:0732-183X
2684:0008-543X
2622:0893-3952
2565:0147-5185
2521:1083-7159
2471:206694560
2445:1946-6234
2387:0021-9258
2338:0008-5472
2217:0950-9232
2158:2159-8274
2106:0732-183X
2063:0098-7484
2008:0950-9232
1950:1061-4036
1779:0732-183X
1727:0147-5185
1683:1527-7755
1634:0022-3468
1582:0008-543X
1433:2051-7599
1376:1093-5266
1184:0732-183X
944:cite book
911:225430576
730:cisplatin
649:Treatment
599:bone scan
571:PAX3-FKHR
519:Diagnosis
449:oncogenes
420:and even
399:PAX3-FKHR
293:proptosis
284:hematuria
267:sclerosis
179:Embryonal
139:prognosis
56:Specialty
4256:Synovial
3979:sarcomas
3920:Orphanet
3903:ped/2005
3865:30924005
3751:23169742
3717:24467821
3666:17858752
3602:Archived
3598:34545149
3552:Archived
3548:26153806
3485:11857556
3438:10524458
3343:36360747
3283:11826361
3239:23924644
3069:19536876
3005:Archived
3001:26389243
2930:11835233
2886:22713795
2834:21154373
2794:25749451
2786:10754991
2700:37393818
2638:21756898
2630:11007039
2581:46691289
2573:11688574
2529:10337369
2463:29973406
2403:25993698
2303:Archived
2195:Oncogene
2176:28446439
2024:23071344
2016:10498887
1988:Oncogene
1905:Archived
1867:29762508
1809:Archived
1787:11408506
1735:12218574
1691:16921036
1598:25505511
1590:17941028
1538:26946298
1530:23348207
1487:Archived
1451:26097732
1392:25785779
1332:23179823
1279:22962204
1146:40778156
1097:46247372
1054:10423470
1003:Archived
999:17251339
903:32796172
858:Archived
824:See also
804:Research
597:, and a
557:D-myosin
545:myogenin
537:lymphoma
483:, and c-
205:Alveolar
85:myocytes
61:Oncology
4638:Sarcoma
4371:General
4058:Fibroma
4031:Fibroma
3900:ent/641
3843:D012208
3821:M8920/3
3817:M8900/3
3708:3925355
3657:1803493
3540:8318638
3532:3552846
3395:1978757
3367:Bibcode
3359:Science
3335:7712645
3291:8824095
3231:8557179
3184:8153013
3141:1654982
3132:1977662
3060:2953716
2973:7772200
2878:3791141
2743:3950676
2692:9305719
2454:8054766
2395:7744814
2346:8764111
2299:1056405
2291:1614537
2263:Bibcode
2233:8160066
2225:9315099
2167:7802885
2114:9164192
2071:7530783
1958:8098985
1901:8187070
1858:5977116
1835:Cancers
1743:1792514
1642:2723995
1442:4447051
1384:9724344
1324:8625211
1271:2843274
1227:1574030
1192:7636557
1138:8448756
1089:3275486
990:1867426
795:History
768:sarcoma
668:updated
581:Staging
566:Z bands
331:Genetic
264:hyaline
4313:PEComa
4292:Lipoma
4192:Myxoma
3975:tumors
3889:001429
3832:268210
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1052:
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932:
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787:, and
716:, and
559:, and
553:desmin
535:, and
342:FP-RMS
323:, and
77:cancer
4512:STUMP
4379:Myoma
4258:-like
3854:11485
3812:ICD-O
3806:171.9
3594:S2CID
3544:S2CID
3528:JSTOR
3339:S2CID
3287:S2CID
3235:S2CID
2882:S2CID
2790:S2CID
2696:S2CID
2634:S2CID
2577:S2CID
2467:S2CID
2399:S2CID
2295:S2CID
2251:(PDF)
2229:S2CID
2020:S2CID
1962:S2CID
1739:S2CID
1594:S2CID
1534:S2CID
1388:S2CID
1328:S2CID
1275:S2CID
1142:S2CID
1093:S2CID
907:S2CID
549:actin
422:MYOD1
390:FOXO1
387:with
378:FOXO1
367:FKHR)
362:FOXO1
157:Types
93:tumor
3977:and
3838:MeSH
3827:OMIM
3801:9-CM
3792:.M50
3747:PMID
3713:PMID
3662:PMID
3644:ISSN
3536:PMID
3520:ISSN
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950:link
930:ISBN
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728:and
561:myoD
496:KRAS
492:NRAS
474:KRAS
468:NRAS
462:NMYC
434:BRD4
418:MYCN
395:PAX3
384:PAX7
372:PAX3
353:PAX7
347:PAX3
3925:780
3797:ICD
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3781:ICD
3739:doi
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1996:doi
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