Embryonal Rhabdomyosarcoma

A form of RHABDOMYOSARCOMA arising primarily in the head and neck, especially the orbit, of children below the age of 10. The cells are smaller than those of other rhabdomyosarcomas and are of two basic cell types: spindle cells and round cells. This cancer is highly sensitive to chemotherapy and has a high cure rate with multi-modality therapy. (From Holland et al., Cancer Medicine, 3d ed, p2188)

Also Known As:

Networked: 337 relevant articles (15 outcomes, 28 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Sarcoma (Soft Tissue Sarcoma)
2.	Rhabdomyosarcoma
3.	Neoplasms (Cancer)
4.	Alveolar Rhabdomyosarcoma
5.	Neuroblastoma

Experts

1.	Marampon, Francesco: 8 articles (01/2022 - 12/2005)
2.	Keller, Charles: 6 articles (01/2019 - 06/2009)
3.	Parham, David M: 6 articles (09/2017 - 04/2005)
4.	Iseki, Ken: 6 articles (04/2016 - 07/2005)
5.	Kobayashi, Masaki: 6 articles (04/2016 - 07/2005)
6.	Ciccarelli, Carmela: 5 articles (01/2022 - 12/2005)
7.	Hawkins, Douglas S: 5 articles (01/2019 - 07/2013)
8.	Itagaki, Shirou: 5 articles (01/2012 - 07/2005)
9.	Megiorni, Francesca: 4 articles (01/2022 - 10/2017)
10.	Tombolini, Vincenzo: 4 articles (01/2019 - 01/2011)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Embryonal Rhabdomyosarcoma:

1.	Cyclophosphamide (Cytoxan)FDA LinkGeneric 01/01/2000 - "The IRSG has reported improvement in the prognosis of both nonmetastatic and metastatic embryonal rhabdomyosarcoma as attributable to three, three-drug regimens that use cyclophosphamide at 2.2 g/m2 in either maintenance or induction and maintenance therapy. " 06/01/2001 - "Described here is a case of transitional cell carcinoma of the bladder developing 8 years following exposure to cyclophosphamide for the treatment of embryonal rhabdomyosarcoma." 04/01/2011 - "Patients with localized, grossly resected, or gross residual (orbital only) embryonal rhabdomyosarcoma (ERMS) had 5-year failure-free survival (FFS) rates of 83% and overall survival rates of 95% on Intergroup Rhabdomyosarcoma Study Group (IRSG) protocols III/IV. IRSG D9602 protocol (1997 to 2004) objectives were to decrease toxicity in similar patients by reducing radiotherapy (RT) doses and eliminating cyclophosphamide for the lowest-risk patients. " 12/01/2019 - "This study is significant because it aims to establish that the use of irinotecan in patients with low-risk subset B embryonal rhabdomyosarcoma (aged 30 or younger) allows the dose of cyclophosphamide to be reduced and is associated with few short-term adverse effects and long-term complications. " 06/01/1992 - "We report a case of abdominal embryonal rhabdomyosarcoma who received a combined treatment modality with radical pelvic surgery and combination chemotherapy of vincristine, actinomycin-D and cyclophosphamide (VAC). "
2.	Vincristine (Oncovin)FDA LinkGeneric 07/01/2022 - "We present the case of vincristine induced fever in a child with embryonal rhabdomyosarcoma. " 04/01/2003 - "In vivo combination treatment using i.v. G207 and vincristine resulted in complete regression of alveolar rhabdomyosarcoma in five of eight animals and significant growth inhibition of embryonal rhabdomyosarcoma. " 03/01/2006 - "Stage I, II orbital and stage I para-testicular embryonal rhabdomyosarcomas received 32 weeks of vincristine and actinomycin- D (vincristine 1.5 mg/m2 weekly, actinomycin-D 0.015 mg/Kg/day day 1 to day 5). " 01/01/2019 - "Magnetic resonance imaging demonstrated absence of invasion into adjacent organs.  Histology confirmed embryonal rhabdomyosarcoma, botryoid subtype.  Subsequent to the initial excision of the polyp, he was started on an ifosfamine, vincristine and actinomycin (IVA) chemotherapy regime in three weekly cycles for nine cycles with concomitant radiotherapy. " 05/01/2007 - "In embryonal rhabdomyosarcoma strongest effects were found after treatment with Ifosfamide, Vincristine and Carboplatin (P < 0.05). "
3.	Dactinomycin (Actinomycin)FDA LinkGeneric 07/01/1997 - "We conclude that exposure of this human embryonal rhabdomyosarcoma cell line to low concentrations of actinomycin D released the neoplastic cells from their blockade, allowing them to recover normal myogenic development. " 03/01/2006 - "Stage I, II orbital and stage I para-testicular embryonal rhabdomyosarcomas received 32 weeks of vincristine and actinomycin- D (vincristine 1.5 mg/m2 weekly, actinomycin-D 0.015 mg/Kg/day day 1 to day 5). " 01/01/2019 - "Magnetic resonance imaging demonstrated absence of invasion into adjacent organs.  Histology confirmed embryonal rhabdomyosarcoma, botryoid subtype.  Subsequent to the initial excision of the polyp, he was started on an ifosfamine, vincristine and actinomycin (IVA) chemotherapy regime in three weekly cycles for nine cycles with concomitant radiotherapy. " 06/01/1992 - "We report a case of abdominal embryonal rhabdomyosarcoma who received a combined treatment modality with radical pelvic surgery and combination chemotherapy of vincristine, actinomycin-D and cyclophosphamide (VAC). " 08/01/1975 - "This has been best demonstrated in the long-term disease-free survival of a majority of children with inoperable or metastatic embryonal rhabdomyosarcoma treated with VAC chemotherapy (vincristine, actinomycin D, and cyclophosphamide). "
4.	Chloroquine (Aralen)FDA LinkGeneric 04/28/2015 - "Here, we identify a synthetic lethal interaction of BEZ235 together with the lysosomotropic agent chloroquine (CQ), which is effective against embryonal rhabdomyosarcoma (ERMS). " 04/28/2015 - "Dual phosphatidylinositol 3-kinase/mammalian target of rapamycin inhibitor NVP-BEZ235 synergizes with chloroquine to induce apoptosis in embryonal rhabdomyosarcoma."
5.	dactolisibIBA 04/28/2015 - "Here, we identify a synthetic lethal interaction of BEZ235 together with the lysosomotropic agent chloroquine (CQ), which is effective against embryonal rhabdomyosarcoma (ERMS). " 04/28/2015 - "Dual phosphatidylinositol 3-kinase/mammalian target of rapamycin inhibitor NVP-BEZ235 synergizes with chloroquine to induce apoptosis in embryonal rhabdomyosarcoma."
6.	ParaffinIBA 09/01/1996 - "Our study confirms that the t(2;13) translocation is not present in embryonal rhabdomyosarcomas, but can be detected in nearly half of alveolar rhabdomyosarcomas, whether fresh-frozen or paraffin-embedded. " 07/15/1993 - "To solve these problems, fluorescent in situ hybridization (FISH) technique was used on free nuclei isolated from formalin-fixed paraffin-embedded embryonal rhabdomyosarcoma (RMS) tissue using our modification of Hedley's method for isolation of nuclei. " 12/01/1980 - "Ultrastructural examination of embryonal rhabdomyosarcoma may be of value for a definite diagnosis also when only formalin-fixed and paraffin-embedded material is available." 11/01/1988 - "Nuclear deoxyribonucleic acid ploidy studies with paraffin-embedded archival tumor specimens were performed by flow cytometry on extracted nuclei from 13 embryonal rhabdomyosarcomas of the bladder and prostate. " 02/01/1997 - "Using monoclonal antibody from clone hVIN-1 by APAAP techniques on formalin-fixed, paraffin-embedded tissue, all but one tumor, which was a primitive embryonal rhabdomyosarcoma, demonstrated a significant positive vinculin staining. "
7.	Hydroxymethylglutaryl-CoA Reductase Inhibitors (HMG-CoA Reductase Inhibitors)IBA 04/01/2016 - "We previously reported that α-cyano-4-hydroxycinnamate, an MCT4 inhibitor, increased the inhibition of growth of RD cells, a prototypic embryonal rhabdomyosarcoma cell line (an RD cell line), as a model of in vitro skeletal muscle, induced by a statin. " 04/23/2008 - "In the present study, we examined the mechanisms underlying the cytotoxicity of pitavastatin, a new statin, and we compared the in vitro potencies of muscle cytotoxicity using a prototypic embryonal rhabdomyosarcoma cell line (RD cells), a typical side effect of statins and compared the cholesterol-lowering effects of statins using Hep G2 hepatoma cells. " 01/01/2012 - "The aim of this study was to determine the effects of α-cyano-4-hydroxycinnamic acid (CHC), a lactate efflux inhibitor, and citrate, an alkaline reagent, on statin-induced muscle injury using a human prototypic embryonal rhabdomyosarcoma cell line (RD) as a model of in vitro skeletal muscle and on statin-induced muscle damage in an in vivo study. "
8.	DesminIBA 01/01/2020 - "Although most cases are easily diagnosed by morphology alone, the morphology of FESPs is variable and in some instances can contain hypercellular stroma with numerous atypical desmin-positive cells, simulating botryoid embryonal rhabdomyosarcoma (ERMS). " 01/01/1988 - "The value of the embedding technique for ultrastructural analysis and immunohistochemistry in the demonstration of desmin in aspirates is emphasized in the diagnosis of embryonal rhabdomyosarcoma." 07/01/2005 - "We conclude that desmin, muscle-specific actin, smooth muscle actin, and WT1 are not specific for discriminating embryonal rhabdomyosarcoma from normal subepithelial cells in the female genital tract of children, although the number of immunopositive cells is consistently larger in rhabdomyosarcoma. " 05/01/2005 - "We conclude that desmin, muscle-specific actin, smooth muscle actin, and WT1 are not specific for discriminating embryonal rhabdomyosarcoma from normal subepithelial cells in the female genital tract of children, although the number of immunopositive cells is consistently larger in rhabdomyosarcoma. " 01/01/2022 - "However, the final diagnosis was established to be hepatic embryonal rhabdomyosarcoma based on postoperative histopathology and typical immunohistochemical staining, which was positive for desmin and myogenin. "
9.	AntibodiesIBA 02/08/2022 - " positive rate of both NKX3.1 antibodies in MC was 12/12, NKX3.1 was focal weakly positive in only one of 12 chondrosarcomas (grade 3), 5 alveolar rhabdomyosarcomas, 5 embryonal rhabdomyosarcomas, and 5 solitary fibrous tumors, respectively. " 09/01/2006 - "Tissue microarray sections representing 66 rhabdomyosarcoma tumors (34 embryonal rhabdomyosarcoma, 32 alveolar rhabdomyosarcoma) were surveyed by immunohistochemistry using antibodies specific for EGFR and ErbB-2. " 01/01/1982 - "Four embryonal rhabdomyosarcomas, one tumor diagnosed as an undifferentiated sarcoma, probably a rhabdomyosarcoma, and six different non-muscular sarcomas were investigated with antibodies specific for different intermediate filament types. " 01/01/2021 - "The mAbs did not recognize proteins in fusion-negative embryonal rhabdomyosarcoma cell lines, nor did they recognize PAX3 or FOXO1 alone when compared to anti-PAX3 and anti-FOXO1 antibodies. " 01/01/2021 - "We reviewed responses for patients with chemotherapy, decitabine or everolimus combination therapies with PD-1 antibodies, and found that PD-1 antibody combined with decitabine showed potential efficacy in pediatric patients with advanced embryonal rhabdomyosarcoma and lymphoepitheliomatoid-like carcinoma. "
10.	Messenger RNA (mRNA)IBA 11/01/2002 - "In this study, we investigated the mRNA level of several genes involved in cell cycle regulation in alveolar (ARMS) and embryonal rhabdomyosarcomas (ERMS). " 08/01/1996 - "To test this we have characterized total and allele-specific mRNA expression levels and DNA methylation of the 11p15.5 KIP2 gene in normal human tissues, WTs and embryonal rhabdomyosarcoma (RMS). " 11/01/1999 - "Assay of PAX3 and PAX7 mRNA expression in embryonal rhabdomyosarcoma, neuroblastoma, Ewing's sarcoma, and melanoma cell lines revealed tumor-specific expression patterns similar to the corresponding embryonic lineages. " 11/01/2003 - "The three tested cell lines of embryonal rhabdomyosarcomas demonstrated no or low (n = 2) telomerase activity, which was confirmed in two cases by a very low expression of hTERT mRNA. " 11/01/2002 - "GLUT4 mRNA and protein were detected in ARMS-derived human biopsies and in ARMS-derived RH30 myoblasts, which both express the PAX3/FKHR chimeric protein, but not in either C2C12 or embryonal rhabdomyosarcoma-derived myoblasts. "

Therapies and Procedures

1.	Radiotherapy 01/01/2008 - "We report a case of primary intracranial embryonal rhabdomyosarcoma in a 5-year-old girl who was treated successfully with local radiation therapy (RT) and a combination of two different chemotherapeutic regimens. " 01/01/1989 - "However, morphologic analyses suggest that VAC is effective in embryonal rhabdomyosarcomas in which undifferentiated rhabdomyoblasts predominate, while radiotherapy is preferable for those containing variously differentiated rhabdomyoblasts." 09/01/2017 - "Here, we analyze the efficacy of 45 Gray (Gy) local radiotherapy (RT) in patients with Group III orbital embryonal rhabdomyosarcoma (ERMS) enrolled on the COG low-risk study ARST0331. " 05/01/1997 - "Adult embryonal rhabdomyosarcoma showing marked response to radiotherapy: a case report." 04/01/1989 - "A case involving a 13-year-old patient who was found to have an embryonal rhabdomyosarcoma of the middle pharynx at the age of six and a half years will present the consequences of radiotherapy to the head of a growing child. "
2.	Therapeutics 06/01/2000 - "IRS-IV therapy benefited certain subgroups of patients with intermediate-risk embryonal rhabdomyosarcoma. " 01/01/2017 - "Pediatric Anaplastic Embryonal Rhabdomyosarcoma: Targeted Therapy Guided by Genetic Analysis and a Patient-Derived Xenograft Study." 01/01/2023 - "Patient-derived organoids facilitating individual therapy in an adolescent with embryonal rhabdomyosarcoma of the cervix: a case report and literature review." 09/01/2021 - "To report a case of aggressive infantile orbital embryonal rhabdomyosarcoma harboring germline ATM mutation and 2 somatic mutations as revealed by next-generation sequencing and the potential application for personalized therapy. " 01/01/2020 - "We present a classic case of AML with t(8;16)/KAT6A-CREBBP occurring in a patient with both a germline NF1 mutation and recent cytotoxic therapy for embryonal rhabdomyosarcoma."
3.	Drug Therapy (Chemotherapy) 06/01/1979 - "Survival with embryonal rhabdomyosarcoma of all sites has improved dramatically in recent years with the increased use of long-term, cyclic, multidrug chemotherapy. " 01/01/2023 - "Successful pregnancy in a female with embryonal rhabdomyosarcoma of the cervix who received biopsy and chemotherapy alone without recurrence after 16 years: a case report and literature review." 12/01/2022 - "Patients with completely resected nongenitourinary low-risk embryonal rhabdomyosarcoma are candidates for reduced duration low-intensity chemotherapy." 02/01/2022 - "Case Report: This 14- month- old baby was referred to us for recurrent left scrotal embryonal rhabdomyosarcoma (ERMS), first diagnosed at 8 months, status post chemotherapy. " 10/01/2020 - "We present a case of embryonal rhabdomyosarcoma that was successfully and conservatively managed with chemotherapy, allowing for future pregnancies. "
4.	Combination Drug Therapy (Combination Chemotherapy) 07/01/1999 - "To analyze MDR in RMS we used the RMS-GR cell line, obtained from an embryonal rhabdomyosarcoma treated in vivo with polychemotherapy. " 03/01/1974 - "Combination chemotherapy for embryonal rhabdomyosarcoma." 05/01/1985 - "Combination chemotherapy has dramatically improved the results of treatment for embryonal rhabdomyosarcoma of the vagina in children, but little attention has been directed toward vaginal sarcomas in adults. " 06/01/1992 - "We report a case of abdominal embryonal rhabdomyosarcoma who received a combined treatment modality with radical pelvic surgery and combination chemotherapy of vincristine, actinomycin-D and cyclophosphamide (VAC). " 01/01/1996 - "We present the case of a 12-year-old girl diagnosed with an embryonal rhabdomyosarcoma of the endocervix who received vincristine-Adriamycin-cyclophosphamide combination chemotherapy followed by a simple hysterectomy. "
5.	Aftercare (After-Treatment) 08/01/1987 - "Girls with localized embryonal rhabdomyosarcoma of the vagina have a 100% survival rate after treatment on the Intergroup Rhabdomyosarcoma Study (IRS) (IRS-1 or IRS-2). " 10/01/2004 - "Laryngeal embryonal rhabdomyosarcoma: a case of cervical metastases 13 years after treatment and a 25-year review of existing literature." 02/01/2013 - "Colonic adenocarcinoma as a secondary malignancy after treatment of embryonal rhabdomyosarcoma." 05/01/2007 - "In embryonal rhabdomyosarcoma strongest effects were found after treatment with Ifosfamide, Vincristine and Carboplatin (P < 0.05). "