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PubMed2004

Rhabdomyosarcoma, osteosarcoma, and adrenocortical carcinoma in a child with a germline p53 mutation.

A child with an unusual association of cancers is described. The patient first presented with a rhabdomyosarcoma of the right scapular muscle, and was successfully treated with chemotherapy. Six years after diagnosis of the first malignancy, the child presented with two synchronous malignancies: osteosarcoma of the jaw and adrenocortical carcinoma. Genetic mutation analysis was performed and revealed a germline p53 mutation of CGT > CAT at codon 273. The family history was negative for any other cancer consistent with the Li-Fraumeni syndrome. This case highlights the need for close surveillance of patients with p53 mutation for malignancy and describes the occurrence of two malignancies synchronously.

Adrenal Cortex Neoplasms↗
PubMed2005

Long-term medical effects of childhood and adolescent rhabdomyosarcoma: a report from the childhood cancer survivor study.

BACKGROUND: This study was conducted to evaluate the incidence of adverse medical conditions and to assess the risk of developing these conditions in a cohort of long-term survivors of rhabdomyosarcoma (RMS) diagnosed before age 21. PROCEDURE: Using data from the Childhood Cancer Survivor Study (CCSS), we evaluated the incidence of self-reported adverse medical conditions for 606 RMS survivors and 3,701 siblings of cancer survivors. Cancer and treatment data abstracted from medical records were used to evaluate the effects of primary tumor site and combined modality therapy on the risk of developing sequelae in survivors. RESULTS: The relative risk (RR) for developing sequelae among survivors compared with siblings was greatest within 5 years after diagnosis. RR was elevated more than 5 years after diagnosis for several conditions (RR, 95% CI) as follows: eye impairment (cataract: 7.4, 2.9-18.9; visual disturbances: 3.2, 2.0-5.1; very dry eyes: 2.0, 1.2-3.3), endocrine impairment (growth hormone deficiency: 83.9, 33.0-213.6; hypothyroidism: 6.9, 4.1-11.3; need for medications to induce puberty: 90.4, 30.2-270.5), cardiopulmonary impairment (congestive heart failure: 43.0, 12.7-145.5; angina-like symptoms: 2.0, 1.3-2.9), neurosensory impairment (legal blindness: 9.8, 4.8-20.0; abnormal sensations: 1.5, 1.0-2.2), and neuromotor impairment (repeated seizures: 2.3, 1.2-4.4; motor problems: 3.7, 2.2-6.4; problems chewing or swallowing: 3.8, 1.9-7.5). CONCLUSIONS: Survivors are at risk for developing sequelae many years after their initial diagnosis and treatment. Continued medical surveillance is necessary to ensure the long-term health and well-being of RMS survivors.

Adolescent↗
PubMed2006

Differentiation of rhabdomyosarcoma cell lines using retinoic acid.

BACKGROUND: Rhabdomyosarcoma (RMS) is the most frequent sporadic soft tissue sarcoma of childhood and adolescence. The overall 5-year survival rate for patients with RMS is 70% with the use of surgery, radiation, and chemotherapy. Novel therapeutic approaches are necessary to improve on these outcomes particularly among the more aggressive alveolar RMS (ARMS) and late stages of disease, where 5-year survival is less than 20%. Retinoids have been successfully used in the treatment of acute promyelocytic leukemia (APML) and neuroblastoma. PURPOSE: However, analysis of retinoids as a differentiating agent for RMS has been incomplete. This work examined the ability of retinoic acid (RA) to promote differentiation of RMS cell lines by examining the expression of myogenic proteins in five RMS cell lines in response to All-trans Retinoic Acid (ATRA) or 9-cis retinoic acid (CRA). RESULTS: Analysis of growth curves indicates that both retinoids suppress cell growth of Rh4 and Rh28. RD cells only responded to-CRA whereas Rh30 and Rh18 did not respond. Following treatment with ATRA FACS analysis showed an altered cell cycle with the same pattern as the growth curves. ATRA altered cellular morphology of two cell lines, Rh4 and Rh28, and induced Troponin T expression in these cells suggesting a differentiating effect. CONCLUSIONS: These studies suggest that retinoids are effective inducers of growth arrest and differentiation in some RMS cell lines, and offer a basis for further in vivo testing in mice of ATRA as a potential approach to ARMS treatment.

Alitretinoin↗
PubMed2007

Recognition of Li Fraumeni syndrome at diagnosis of a locally advanced extremity rhabdomyosarcoma.

A contemporaneous presentation of a second breast cancer in a mother and an extremity rhabdomyosarcoma (RMS) in her daughter led to the diagnosis of the Li Fraumeni syndrome (LFS). Although the association between LFS and RMS in young patients is well recognised 1 there are no guidelines as to how this knowledge should influence the optimal management of these patients. After reviewing the literature about the natural history of the LFS 2, the incidence of second malignancy (SMN) in RMS survivors 3-6 and the management of extremity RMS 7-9, we are concerned that contemporary RMS treatment, combining non-mutilating surgery with chemoradiotherapy, may be associated with an excessive SMN risk in LFS patients with advanced RMS. We question whether treatment should be individualised and, where possible and acceptable to the family, measures such as amputation should be the considered to attain local control for LFS patients with RMS as this will avoid the need for local radiotherapy without compromising long-term function and quality of life 10.

Adrenal Cortex Neoplasms↗
PubMed2008

Comparison of results of a pilot study of alternating vincristine/doxorubicin/cyclophosphamide and etoposide/ifosfamide with IRS-IV in intermediate risk rhabdomyosarcoma: a report from the Children's Oncology Group.

BACKGROUND: Over 50% of patients with rhabdomyosarcoma (RMS) have intermediate risk disease, with a 3-year failure-free survival (FFS) of 50%-70% depending on histology. Doxorubicin is active against RMS, but its role in improving outcome remains controversial. Ifosfamide is as active as cyclophosphamide in RMS, with the Fourth Intergroup RMS Study (IRS-IV) showing equivalent outcomes for patients treated with ifosfamide for the first 28 weeks compared to cyclophosphamide. Treatment with alternating cycles of non-cross-resistant chemotherapy has been used in a number of diseases with good results. PROCEDURE: The results of a pilot study utilizing alternating courses of vincristine, doxorubicin, cyclophosphamide, and etoposide/ifosfamide (VDC/IE) were compared for outcome and patient characteristics to a group of similar matched patients treated on IRS-IV. RESULTS: The 5-year FFS for patients with parameningeal (PM) primaries on IRS-IV and the VDC/IE study were 72% and 82%, respectively (P = 0.26); for patients with non-PM primaries, the estimated risk of failure for VDC/IE study versus IRS-IV was 0.54. Combining all disease sites and performing analysis for relative risk of failure for 46 VDC/IE patients and 342 IRS-IV patients, the relative risk of failure for the VDC/IE study compared to the IRS-IV study is 0.5 (P = 0.06). CONCLUSIONS: VDC/IE is as effective therapy for intermediate risk RMS as IRS-IV therapy. It is being explored along with irinotecan in relapsed patients and newly diagnosed high-risk patients.

Adolescent↗
PubMed2007

Physical impairment and social adaptation in adult survivors of childhood and adolescent rhabdomyosarcoma: A report from the Childhood Cancer Survivors Study.

BACKGROUND: Despite interest in the well-being of adult survivors of childhood rhabdomyosarcoma (RMS), few studies have examined their health-related quality of life (HRQOL). This study evaluated physical and social aspects of HRQOL among long-term childhood RMS survivors relative to a sibling comparison group, and assessed whether physical impairment among RMS survivors adversely affected their ability to achieve adult life goals. METHODS: Using baseline data from the Childhood Cancer Survivor Study, we evaluated self-reported physical impairment and social adaptation among 417 survivors of childhood RMS and 2685 siblings > or =18 years of age at survey completion. RESULTS: Survivors were more likely than siblings to report physical impairment, characterized by: at least one medically diagnosed condition, limitations in the performance of routine activities, a health-related inability to work or attend school, cancer-related pain. Survivors were less likely than siblings to have completed high school, ever worked a job, or ever been married. The odds of completing high school were lower among survivors with performance limitations, a health-related inability to work or attend school, or moderate to high levels of cancer-related pain. Survivors who reported cancer-related pain had an increased likelihood of ever being married. CONCLUSIONS: The majority of adult survivors of pediatric RMS are successful in attaining adult life goals despite higher reported occurrence of physical impairment than their sibling counterparts. Additional studies are needed to advance our understanding of other aspects of HRQOL in this population of pediatric cancer survivors.

Activities of Daily Living↗
PubMed1992

Clustering in sparse data and an analysis of rhabdomyosarcoma incidence.

Time series of epidemiologic events often contain periods of atypically low or high frequency. Correspondingly, for quite rate diseases there occur instances of long vacuous durations interrupted noticeably by periods of some disease activity. A recent community-based observation of the incidence of rhabdomyosarcoma (RMS), and an investigation of it, yielded sparse data of this general description. We introduce a combinatorial test for patchy time series and apply it to the RMS data. We comment on the prevalent practice of post hoc data analysis of alleged clusters, and on scale effects.

Adolescent↗
PubMed2002

Lipid peroxidation, DNA damage, and cellular morphology of R1 Rhabdomyosarcoma cell line irradiated in vitro by gamma-rays with different dose rates.

The study examines the relationship between lipid peroxidation, DNA damage, and cell morphology after the exposure of R1 Rhabdomyosarcoma cells to two different dose-rates of gamma rays. Exponential cultures of R1 cells were irradiated with single dose of 5 Gy at high dose rate (0.833 Gy/min) and low dose rate (0.0707 Gy/min). The concentration of two aldehydes, malondialdehyde (MDA) and 4-hydroxynonenal (4-HNE), were determined. DNA damage induction and repair were measured by using the alkaline version of the comet assay. Cellular alteration was also estimated microscopically as was the frequency of cells with micronuclei and proportion of apoptosis and necrosis. These parameters were evaluated immediately (time 0) and after different times up to 48 h of incubation in 37 degrees C, after irradiation. Results indicate that a low dose rate in comparison to high dose rate caused a significantly higher increase of aldehydes concentration observed at 12 h, followed by obviously higher DNA damage at 48 h and altered cellular morphology. The inverse dose-rate effect estimated for the gamma rays Co-60 source was found to be related to the measured biochemical and morphological parameters.

Apoptosis↗
PubMed2000

Human rhabdomyosarcoma cells retain insulin-regulated glucose transport activity through glucose transporter 1.

We evaluated the expression of glucose transporter (glut) isoforms and its function in RD cells, human rhabdomyosarcoma, which retain the potential to differentiate into muscle. Gluts 1, 3, and 4 were expressed in RD cells, as detected by reverse-transcription polymerase chain reaction and immunocytochemistry. Supraphysiological concentration (1 microM) of insulin treatment increased 2-deoxy glucose transport by up to 1.68-fold together with concomitant tyrosine phosphorylation of the insulin receptor beta subunit and of insulin receptor substrate 1. Suppression of glut 1 mRNA by 38% by antisense oligonucleotide transfection led to a reduction of basal and insulin-stimulated 2-deoxy glucose transport by 38 and 55%, respectively. Suppression of gluts 3 and 4 by antisense oligonucleotide transfection did not affect both basal and insulin-stimulated 2-deoxy glucose transport. Thus, glut 1 accounts for the major part of basal and insulin-stimulated glucose transport in RD cells. Next, we transfected expression vectors carrying human gluts 1 and 4 cDNAs into RD cells to add further support for the role of glut 1 in glucose transport. Overexpression of glut 1 stimulated basal and insulin-stimulated 2-deoxy glucose transport by 1.66- and 1.43-fold, respectively. Glut 4 overexpression did not affect basal and insulin-stimulated 2-deoxy glucose transport. Western blot analysis using glut 1 antibody showed that glut 1 was redistributed from intracellular membrane to plasma membrane. These observations support the notion that RD cells, with the potential to differentiate into muscle, retain insulin responsiveness. As human muscle cell lines are not available at this point, RD cells can serve as a useful alternative to human muscle for studies related to insulin signal transduction and glucose transport.

3-O-Methylglucose↗
PubMed2001

Self-cleaving-ribozyme-mediated reduction of betaAPP in human rhabdomyosarcoma cells.

A self-cleaving hammerhead ribozyme targeted to codon 47 in beta-amyloid precursor protein (betaAPP) mRNA was cloned as a eucaryotic transcription cassette into the 3' UTR of enhanced green fluorescence protein (EGFP) mRNA, producing a C-terminal fusion mRNA. CMV promotor-driven vectors bearing this construct or a mutationally inactive ribozyme construct were transiently transfected into human embryonic rhabdomyosarcoma (A-204) cells and their effects studied. Ribozyme self-cleavage in vivo was demonstrated by Northern blotting and the site of self-cleavage was delineated using site-specific deoxyoligonucleotide probes and primer extension arrest. Using this ribozyme reporter we demonstrated that ribozyme expression correlated with lower betaAPP levels in the transfected cells. Control studies with the inactive ribozyme construct showed that both ribozyme cleavage and antisense mechanisms combined to produce the observed effect. Furthermore, production of truncated EGFP mRNA via ribozyme self-cleavage reduced EGFP-reporter expression compared to full-length EGFP control mRNAs, indicating that truncation affects the translatability of the reporter. This occurred because of a slight decrease in the stability of the fusion mRNA. The results of these studies suggest that self-cleaving ribozyme vectors may be an effective means of delivering and visualizing the expression of small active ribozymes in vivo.

Alzheimer Disease↗
PubMed1993

Ara-C treatment leads to differentiation and reverses the transformed phenotype in a human rhabdomyosarcoma cell line.

Rhabdomyosarcoma (RMS) is an embryonal tumor of childhood that arises from primitive skeletal muscle-forming cells (rhabdomyoblasts) probably arrested and transformed along the normal myogenic pathway to maturation. Since Ara-C is an antitumor agent known to induce differentiation in human acute myelogenous leukemia, also presumably a disorder of cellular maturation, we treated RD, a human embryonal RMS cell line, with Ara-C and evaluated its effect on growth and differentiation. Ara-C treatment of RD cells in vitro caused a dose-dependent growth inhibition in the absence of cytotoxicity. Interestingly, RD cells treated with 5 x 10(-7) M Ara-C for 4 days were able to recover logarithmic growth after the removal of the drug from the media. A reexposure of these cells to Ara-C led to morphological and biochemical changes related to differentiation, including the appearance of an increased number of multinucleated cells that expressed muscle-specific actin and skeletal muscle myosin heavy chain (MHC) (fast). In vivo studies demonstrated that RD cells pretreated with 5 x 10(-7) M Ara-C lost their ability to form tumors in nude mice. We conclude that treatment of this human embryonal RMS cell line with Ara-C results in marked growth inhibition in vitro, loss of tumorigenicity in vivo, and the expression of biochemical markers present in a more differentiated phenotype. These data suggest a potential role for differentiation therapy as a therapeutic approach in RMS.

Animals↗
PubMed1993

TPA-induced differentiation of human rhabdomyosarcoma cells: expression of the myogenic regulatory factors.

RD cells (a cell line derived from a human rhabdomyosarcoma) undergo a very limited myogenic differentiation despite the fact that they express several myogenic determination genes. Since we have previously shown (Aguanno et al., Cancer Res. 50, 3377, 1990) that the tumor promoter 12-O-tetradecanoylphorbol-13-acetate (TPA) induces myogenic differentiation in these cells, in this paper we investigate the mechanism by which TPA interferes with the expression and/or function of the myogenic determination genes. Northern blot analysis revealed that RD cells express the myf3 (the human analog of MyoD) and myf4 (the human analog of myogenin) transcripts, but not myf5 or myf6 transcripts. The myf3 and the myf4 gene products are correctly translated and accumulated in the nuclei as shown by immunofluorescence analysis. The tumor promoter (TPA) does not modify the pattern of expression of the myf factors while it induces the accumulation of muscle-specific transcripts, such as alpha-actin and fast myosin light chain 1, and their corresponding proteins. On the other hand, within 1 day of treatment, TPA inhibits the expression of the Id gene, which is a negative regulator of MyoD activity. However, while the TPA-induced inhibition of Id message accumulation correlates with differentiation, cell confluence also causes a reduction in Id message accumulation, without inducing differentiation. Under our experimental conditions, overexpression of any of the myf cDNAs in RD cells does induce spontaneous differentiation but enhances the effect of TPA treatment independently from the level of the expressed message. These data suggest that differentiation of RD cells is likely to depend upon the activity of complexes containing the various members of the MyoD family, which can be regulated by proteins affecting MyoD dimerization such as Id, but also by other mechanisms induced by TPA, such as phosphorylation.

Cell Differentiation↗
PubMed2002

Identification of target genes regulated by PAX3 and PAX3-FKHR in embryogenesis and alveolar rhabdomyosarcoma.

PAX3 is a transcription factor important for neural, muscle, and facial development in vertebrates. To identify genes regulated by PAX3, we used a cyclic amplification and selection of targets (CASTing) strategy to isolate cis-regulatory elements bound by PAX3. CASTing libraries were constructed with mouse DNA fragments bound by mouse PAX3, and human genomic DNA fragments bound by human PAX3 and the fusion protein PAX3-FKHR. Approximately 1000 clones were sequenced from each of these three libraries. Numerous putative targets of PAX3 and PAX3-FKHR were identified and six genes, Itm2A, Fath, FLT1, TGFA, BVES, and EN2, were examined closely. The genomic DNA fragments near these genes contain PAX3 binding sites and confer PAX3-dependent regulation. The expression levels of these genes correlate with the PAX3 expression levels in mouse embryos or with PAX3-FKHR expression levels in rhabdomyosarcoma cell lines, and indicate they may be part of the PAX3 regulatory circuitry during embryogenesis and tumor formation.

Animals↗
PubMed1995

Rhabdomyosarcoma of the vagina in a postmenopausal woman: report of a case and review of the literature.

Most rhabdomyosarcomas (RMSs) of the female genital tract occur in infants and young children as sarcoma botryoides. Vaginal RMS occurring in a postmenopausal woman is extremely rare. To our knowledge, only three vaginal RMSs have been reported in patients who were over 50 years of age. Herein, the first case in Taiwan and the fourth case in the world is reported, and the pertinent literature is briefly reviewed. A 63-year-old woman complained of a few days of abnormal vaginal bleeding. Vaginal examination demonstrated two black polypoid tumors located at the left-lateral and posterior wall of the vagina. The patient had previously undergone a vaginal total hysterectomy for rectocele and urine incontinence, so surgical treatment including local wide excision and bilateral salpingo-oophorectomy were performed under the diagnosis of malignant vaginal tumor. The clinical, histopathological, and immunohistochemical studies revealed a group Ia pleomorphic RMS. Postoperative radiotherapy was performed. The patient remains alive and well 12 months after a combined therapy.

Combined Modality Therapy↗
PubMed1997

Pure alveolar rhabdomyosarcoma of the corpus uteri: description of a case with increased serum level of CA-125.

We report a morphological and immunohistochemical study of a case of pure alveolar rhabdomyosarcoma of the uterus in an 80-year-old woman. The diagnostic clues were the characteristic "alveolar" pattern of growth, the evidence of cross-striations in strap or elongated cells with abundant eosinophilic cytoplasms, the presence of multinucleated cells with peripherally placed "wreathlike" nuclei, and the expression of muscular antigens by the tumor cells. A thorough sampling of the tumor excluded areas of other types of heterologous or homologous sarcomas or the presence of coexisting adenoma or carcinoma. The other immunohistochemical data showed a high proliferative rate as well as a high rate of p53 overexpression in the small poorly differentiated rhabdomyoblasts. Interestingly, the large differentiated rhabdomyoblasts expressed CA-125, the antigenic determinant of nonmucinous epithelial ovarian tumors. The clinical course was very aggressive: the patient died 5 months after surgery because of disease progression. The pertinent literature is discussed.

Aged↗
PubMed1998

Embryonal rhabdomyosarcoma of the uterine cervix: case report and review of the literature.

Embryonal rhabdomyosarcoma (RMS) of the female genital tract usually occurs during childhood in the vagina. In rare cases, RMS can originate in the uterine cervix, with a peak incidence in the second decade. A combined modality approach to treating RMS using multidrug chemotherapy, radiotherapy, and surgery has markedly improved survival. Early stage embryonal RMS of the cervix has been found to have an excellent prognosis. The present case and literature review indicate that extensive surgery does not improve survival in patients with stage I disease.

Adult↗
PubMed2002

Differential display RT-PCR analysis of enterovirus-71-infected rhabdomyosarcoma cells reveals mRNA expression responses of multiple human genes with known and novel functions.

In order to better understand cellular responses to viral infection at the transcriptional level, we employed differential display RT-PCR to analyze mRNAs from RD rhabdomyosarcoma cells following infection with a neurovirulent enterovirus 71 (EV71) strain, compared with mRNAs from uninfected cells. Of 250 expressed sequence tags (ESTs) isolated, sequenced, and identified, all were of cellular origin except 1 that was of viral origin. Of these, 156 were individual distinctive clones, comprising 45 mRNAs showing unaltered expression and 111 mRNAs exhibiting upregulation or downregulation. Of the 45 uniformly expressed mRNAs, 14 represented unknown genes. Of the 111 differentially expressed mRNAs, 63 did not match any known genes. Forty-eight of the 111 mRNAs modified by EV71 infection matched known genes, including those encoding components of cell cycle, cytoskeleton, and cell death mediators; protein degradation mediators; mitochondrial-related proteins; components of protein translation and modification; and cellular transport proteins. The altered expression profiles of representative genes were authenticated by semiquantitative RT-PCR and real-time RT-PCR. We also identified a novel alternatively spliced transcript of TRIP7 thyroid receptor interactor protein; the putative human homolog of murine mc7 mRNA predominantly expressed in the brain; and a novel mRNA similar to that encoding vacuolar protein 8 involved in protein targeting. These results underscore the applicability of the mRNA differential display technique for elucidating the expression profiles of known and even novel genes in response to cellular infection with pathogenic viruses.

Amino Acid Sequence↗
PubMedDate not supplied

Hyaluronate-binding proteins in weakly and highly metastatic variants of rat rhabdomyosarcoma cells.

Weakly (RMS8) and highly (RMS0) metastatic rat rhabdomyosarcoma cells were assayed for their interaction with hyaluronate. The cells in subconfluent cultures were incubated with 35S methionine, the cells were fractionated and the labelled proteins were separated by affinity chromatography on hyaluronate-Sepharose and by HPLC. The RMS8 cells expressed about twice the amount of labelled hyaluronate-binding proteins seen in the RMS0 cells. The molecular sizes of the main hyaluronate-binding proteins were similar in both cell types. Unlike the RMS0 cells, the RMS8 cells took up exogenous, radioactively labelled hyaluronate at 4 degrees C in a saturable and specific way with high affinity. Cells were also incubated with 3H glucosamine. The isolation of the glycosaminoglycans from these cultures by ion-exchange chromatography indicated that the RMS8 cells retained more endogenous 3H hyaluronate in their pericellular domain than did the RMS0 cells. The attachment of trypsinized cells could be inhibited with exogenous hyaluronate, indicating that the proteins with affinity for hyaluronate may act as hyaluronate-binding sites on these cells.

Animals↗