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RhabdomyosarcomaRhabdomyosarcoma, EmbryonalRhabdomyosarcoma, AlveolarMacrophages, AlveolarPulmonary AlveoliMuscle NeoplasmsPAX7 Transcription FactorPaired Box Transcription FactorsAlveolar ProcessSoft Tissue NeoplasmsOrbital NeoplasmsPulmonary Alveolar ProteinosisUrogenital NeoplasmsSarcomaAlveolar Bone LossChromosomes, Human, Pair 13Forkhead Transcription FactorsMesenchymomaSarcoma, EwingOncogene Proteins, FusionDesminChromosomes, Human, Pair 2Translocation, GeneticCombined Modality TherapyVaginal NeoplasmsMyogeninMyoD ProteinSertoli-Leydig Cell TumorCell Line, TumorVincristineTumor Cells, CulturedThoracic NeoplasmsBronchoalveolar Lavage FluidPneumocytesPulmonary SurfactantsGene Expression Regulation, NeoplasticDactinomycinPelvic NeoplasmsImmunohistochemistryGene FusionCell DifferentiationEnterovirus A, HumanPulmonary BlastomaNephroblastoma Overexpressed ProteinDimethylformamideMyoblastsEncyclopedias as TopicMedlinePlusGross Domestic ProductEncyclopedias
Embryonal and alveolarTumorsARMSHistologicCause of rhabdomyosarcomaBotryoidDownregulated in embryonalSkeletalERMSOccurNeuroblastomaChemotherapyCases of rhabdomyosarcomaPatients with rhabdomyosarcomaMalignant tumorPrognosisCancerPediatric soft tissueSymptomsDifferentiationChildhoodASSAYFOXO1MetastasisGenitourinaryParameningealSurvivalEpidemiologySarcomasPrimarySoftFKHRExtremitiesIncidenceBoneChildrenTreatmentAlterationType
Embryonal and alveolar4
  • Rhabdomyosarcoma includes two major histological subtypes, embryonal and alveolar. (umich.edu)
  • Rhabdomyosarcoma, the most common pediatric soft tissue malignancy arises in 2 major histologic forms: embryonal and alveolar. (sahmri.org.au)
  • among which embryonal and alveolar RMSs are the most common ones under the age of 20, while pleomorphic and spindle cell variants of the tumor may also occur in adults, with a peak at the 4th-5th and 6th -7th decades of lifetime, respectively. (biomedcentral.com)
  • The paternally imprinted DLK1-GTL2 locus is differentially methylated in embryonal and alveolar rhabdomyosarcomas. (louisville.edu)
Tumors9
  • ARMS tumors resemble the alveolar tissue in the lungs. (wikipedia.org)
  • Immunostaining for myogenin and for MyoD can be used to determine ARMS from other rhabdomyosarcoma tumors and immunostaining for AP2β and p-cadherin can distinguish fusion positive ARMS from fusion negative. (wikipedia.org)
  • ARMS accounts for roughly 20 to 30 percent of all rhabdomyosarcoma tumors and therefore accounts for roughly 1 percent of malignancies found in children and adolescents. (wikipedia.org)
  • Rhabdomyosarcoma is a member of the group of "small round blue cell" tumors, and must be distinguished from morphologically similar pediatric tumors. (umich.edu)
  • Testing can aid in distinguishing alveolar rhabdomyosarcoma from other similar tumors. (umich.edu)
  • Approximately 20% of tumors diagnosed as alveolar rhabdomyosarcoma based on histologic grounds have been found to be negative for a PAX/FOXO1 translocation or fusion transcript. (umich.edu)
  • Rhabdomyosarcoma is a type of sarcoma made up of tumors that arise from muscle tissue and spread throughout the body. (medicinenet.com)
  • Children with genitourinary tract cancers may manifest with a painless scrotal lump (paratesticular tumors), a projecting grape-like mass in the vagina ("botryoid" rhabdomyosarcoma), blood in the urine (bladder tumors), or frequent urination , often with a burning sensation or hesitation. (medicinenet.com)
  • Rhabdomyosarcoma belongs to a group of tumors known as soft-tissue sarcomas and is the most common cancer in this group. (merckmanuals.com)
ARMS6
  • Alveolar rhabdomyosarcoma (ARMS) is a subtype of the rhabdomyosarcoma soft tissue cancer family whose lineage is from mesenchymal cells and are related to skeletal muscle cells. (wikipedia.org)
  • these share considerably more with the genomic profiles and biological behavior of embryonal rhabdomyosarcoma than with fusion-positive ARMS. (sahmri.org.au)
  • From a pediatric oncology point of view, RMS has traditionally been classified into alveolar (ARMS) and embryonal (ERMS) subtypes. (biomedcentral.com)
  • Primary alveolar rhabdomyosarcoma of the bone as a subtype of ARMS, seems to be a distinct clinico-pathological entity with challenging diagnostic difficulties and different, yet better, biological behavior in comparison to soft tissue ARMS. (biomedcentral.com)
  • Alveolar rhabdomyosarcoma (ARMS) makes up about 25-40% of RMS. (childrensoncologygroup.org)
  • Her research allowed for developing an easy PCR-based assay to phenotype embryonal (ERMS) and alveolar (ARMS) rhabdomyosarcoma based on the methylation of imprinted regions within DLK1-MEG3 locus. (louisville.edu)
Histologic1
  • Postmortem histologic examination showed focal areas of alveolar damage and hyaline membrane formation with lymphocytic infiltrates. (cdc.gov)
Cause of rhabdomyosarcoma2
Botryoid1
Downregulated in embryonal1
Skeletal5
  • Rhabdomyosarcoma (RMS), a malignant tumor derived from the neoplastic proliferation of striated skeletal muscle cells , is the most common pediatric soft tissue sarcoma . (bvsalud.org)
  • Rhabdomyosarcoma is a rare type of cancer that starts in the cells that develop into skeletal muscle cells. (childrensnational.org)
  • Rhabdomyosarcoma (RMS) is a malignant tumor with skeletal muscle cell morphology. (radiopaedia.org)
  • Rhabdomyosarcomas are thought not to arise from skeletal muscle, but rather to differentiate into a tumor that resembles skeletal muscle 7 . (radiopaedia.org)
  • Rhabdomyosarcoma is a cancer arising from embryonal mesenchymal cells that have potential to differentiate into skeletal muscle cells. (merckmanuals.com)
ERMS3
Occur2
Neuroblastoma1
  • La amplificación o sobreexpresión de N-Myc ocurre en más del 20 por ciento de los tumores y se asocia a mal pronóstico en casos de NEUROBLASTOMA, RABDOMIOSARCOMA ALVEOLAR, CARCINOMA PULMONAR DE CÉLULAS PEQUEÑAS y cáncer de próstata neuroendocrino. (bvsalud.org)
Chemotherapy3
  • Many different chemotherapy drugs are active against rhabdomyosarcoma. (medlineplus.gov)
  • Intergroup Rhabdomyosarcoma Study-4 risk stratification was used, with treatment based on a multimodality-regimen with chemotherapy (Vincristine/Ifosfamide/Etoposide and Vincristine/Actinomycin-D/Cyclophosphamide) and appropriate local therapy. (ecancer.org)
  • Treatment for patients with rhabdomyosarcoma involves a combination of surgery, chemotherapy, and radiation therapy. (medscape.com)
Cases of rhabdomyosarcoma2
Patients with rhabdomyosarcoma1
  • In a phase II trial of 87 patients with rhabdomyosarcoma who had experienced a first relapse or disease progression and whose prognosis was unfavorable, temsirolimus (Torisel) proved superior to bevacizumab (Asvastin) as add-on therapy. (medscape.com)
Malignant tumor3
  • Rhabdomyosarcoma is a malignant tumor of striated muscle origin. (medscape.com)
  • Rhabdomyosarcoma is a cancerous (malignant) tumor of the muscles that are attached to the bones. (medlineplus.gov)
  • Rhabdomyosarcoma (RMS) is a malignant tumor of mesenchymal origin and comprises the largest category of soft-tissue sarcomas both in children and adolescents. (biomedcentral.com)
Prognosis2
  • Of these, the alveolar type is associated with a worse prognosis. (umich.edu)
  • SUMMARY: Children who are diagnosed with pediatric rhabdomyosarcoma (RMS), a mesenchymal-derived soft tissue cancer that comprises 3.5% of childhood cancers, are often delivered a bleak prognosis with little hope of a future. (tdl.org)
Cancer8
  • The term "alveolar" refers to cancer cells that develop little hollow pockets or "alveoli. (medicinenet.com)
  • Rhabdomyosarcoma is a type of cancer. (childrensnational.org)
  • She was diagnosed with Stage 4 alveolar rhabdomyosarcoma , a rare soft tissue cancer. (yahoo.com)
  • On June 24 2011 our 3 year old daughter Lauren was diagnosed with a rare, aggressive cancer called alveolar rhabdomyosarcoma. (who.int)
  • Sam was diagnosed with a rare form of cancer known as Alveolar Rhabdomyosarcoma and had only a 15% chance of survival. (gofundme.com)
  • The plaintiff alleged that the provider failed to diagnose cancer in the form of a lump that grew on the infant for a year before a pediatric oncologist ultimately diagnosed the lump as stage IV alveolar rhabdomyosarcoma. (ecri.org)
  • More than 1,100 cars and motorcycles took part Saturday morning in a birthday celebration at the Willamette Speedway for Thomas Radley, now 14, who's battling alveolar rhabdomyosarcoma, a rare stage 4 bone cancer. (sweethomenews.com)
  • Family history of cancer and childhood rhabdomyosarcoma: a report from the Children's Oncology Group and the Utah Population Database. (cancercentrum.se)
Pediatric soft tissue1
  • Rhabdomyosarcomas are the most common soft tissue tumor in children and account for 5-8% of childhood cancers 6,7 , and 19% of all pediatric soft tissue sarcomas 7 . (radiopaedia.org)
Symptoms4
Differentiation1
  • Rhabdomyosarcoma (RMS) is a soft tissue tumor derived from mesenchymal tissue with myogenic differentiation and associated with the embryogenesis of striated muscle. (biomedcentral.com)
Childhood5
  • Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma of childhood and adolescence. (duke.edu)
  • [ 1 ] Rhabdomyosarcoma of the head and neck is primarily a disease of the first decade of life, and it is the most common soft tissue sarcoma in childhood. (medscape.com)
  • Childhood rhabdomyosarcoma treatment (PDQ) health professional version. (medlineplus.gov)
  • Rhabdomyosarcoma (RMS) is among the most common soft tissue sarcomas in childhood and adolescence with 4.5 new cases/1 million person/year in the USA and incidences in Europe share similar numbers [ 1 , 2 ]. (biomedcentral.com)
  • Treatment of rhabdomyosarcoma (RMS), the most common a soft tissue sarcoma in childhood, provides intensive multimodal therapy, with radiotherapy (RT) playing a critical role for local tumor control. (saludintegral.hn)
ASSAY1
  • This assay is expected to be positive in approximately 80% of all alveolar rhabdomyosarcomas. (umich.edu)
FOXO11
  • The t(2;13)(q35;q14) translocation joins the PAX3 and FOXO1 (FKHR) genes in approximately 60% of alveolar rhabdomyosarcomas, while the less common t(1;13)(p36;q14) joins PAX7 with FOXO1 in approximately 20% of cases. (umich.edu)
Metastasis1
Genitourinary1
  • Future directions in risk stratification and therapy for advanced pediatric genitourinary rhabdomyosarcoma. (nih.gov)
Parameningeal2
  • In the head and neck, the most common sites of rhabdomyosarcoma are parameningeal and orbital locations, which account for 16% and 9% of all cases of the disease, respectively. (medscape.com)
  • According to the histopathological analysis , the diagnosis was established corresponding to parameningeal alveolar RMS that was unresectable. (bvsalud.org)
Survival4
  • As a result, 5-year survival rates increased from 25% in 1970 to 73%, as shown in the Intergroup Rhabdomyosarcoma Study (IRS)-IV reported in 2001. (medscape.com)
  • In a retrospective study of 28 pediatric patients with head and neck rhabdomyosarcoma published in 2018, Häußler et al found the 5-year overall survival rate to be 91.3%, with the median period of progression-free survival reported to be 46 months. (medscape.com)
  • Dr. Schneider's work led to the identification of changes in genomic imprinting of DLK1-MEG3 locus in rhabdomyosarcoma and acute myeloid leukemia (AML) and pointed to their role in diagnosis and patient survival prediction. (louisville.edu)
  • In a second study of 461 children with intermediate-risk rhabdomyosarcoma, adding irinotecan to combination treatment with vincristine, dactinomycin, and cyclophosphamide (VAC) did not improve overall or event-free survival. (medscape.com)
Epidemiology2
  • Comparing adult and pediatric rhabdomyosarcoma in the surveillance, epidemiology and end results program, 1973 to 2005: An analysis of 2,600 patients. (merckmanuals.com)
  • 3. Linardic CM, Wexler LH: Rhabdomyosarcoma: Epidemiology and Genetic Susceptibilty. (merckmanuals.com)
Sarcomas1
  • Vinorelbine and low-dose cyclophosphamide in the treatment of pediatric sarcomas: pilot study for the upcoming European Rhabdomyosarcoma Protocol. (nih.gov)
Primary1
Soft2
FKHR1
Extremities1
  • When present in the extremities in children, embryonal rhabdomyosarcomas may cause bowing of the adjacent long bones. (radiopaedia.org)
Incidence1
Bone1
Children9
Treatment4
Alteration1
  • Alveolar RMS can be characterized by a recurrent cytogenetic alteration involving FOXO-1 and PAX3 or PAX7 genes, and the consecutive translocations (t(2;13) or t(1;13) respectively) lead to the excess synthesis of fusion proteins with oncogenic effects [ 5 , 6 ]. (biomedcentral.com)
Type1