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PubMed2004

Immune-mediated thrombocytopenia following dactinomycin therapy in a child with alveolar rhabdomyosarcoma: the unresolved issues.

The authors report a case of severe dactinomycin-induced thrombocytopenia in a child with alveolar rhabdomyosarcoma. The phenomenon is consistent with an immune process leading to the formation of platelet-specific antibodies. This study shows that this can be induced even with the first dose of actinomycin, and its persistence is unpredictably prolonged and does not correlate linearly in an inverted fashion with the platelet count. It will be important to identify the subsets of patients who can develop this phenomenon by molecular techniques and to define the exact mechanism in vitro leading to formation of these antibodies. This would facilitate profiling the therapy, preventing the need for multiple platelet transfusions with their obvious hazards.

Autoantibodies↗
PubMed2005

Caveolin-3 is a sensitive and specific marker for rhabdomyosarcoma.

Caveolin-3 (Cav-3) is a principal structural protein of caveolae membrane domains. Animal studies have revealed that Cav-3 is expressed in skeletal and cardiac myocytes but absent in other types of cells. Recent studies have shown that abnormalities in the Cav-3 gene are associated with some forms of muscular dystrophy, while skeletal muscle abnormalities have been observed in Cav-3 transgenic and knockout mice. In this study the authors evaluated the distribution of Cav-3 in normal human tissues and compared the expression of Cav-3 with that of myogenin and myoD1 in rhabdomyosarcoma (RMS), malignant mixed mullerian tumor (MMMT), and an array of neoplasms that mimic RMS to assess the utility of Cav-3 as a diagnostic marker for tumors with skeletal muscle differentiation. In nonneoplastic human tissues, crisp membrane staining for Cav-3 was present in cardiac and skeletal myocytes and occasionally in arterial smooth muscle cells and prostatic stromal cells, while other cell types were negative for Cav-3. Eighty-eight percent (21/24) of RMS studied were positive for Cav-3. Positive staining was generally observed in the more maturely differentiated tumor cells but not the primitive tumor cells. Eight of nine cases of MMMT stained strongly with Cav-3 in their rhabdomyosarcomatous component but not in other components. Fifty-four other neoplasms (13 leiomyosarcomas, 8 neuroblastomas, 5 lymphomas, 6 Wilms tumors without skeletal muscle differentiation, 5 Ewing sarcomas, 4 malignant fibrous histiocytomas, 4 angiosarcomas, 6 malignant melanomas, and 3 synovial sarcomas) were negative for Cav-3 expression. Nearly all (96% [23/24]) cases of RMS were positive for myogenin, while 88% (21/24) were positive for myoD1. Primitive tumor cells showed significantly increased expression of myoD1 and myogenin; conversely, more differentiated tumor cells were negative or weakly stained. The rhabdomyosarcomatous component of MMMT stained focally with myogenin and myoD1, in contrast to the strong Cav-3 labeling in these cells. These results demonstrate that Cav-3 is specifically expressed in human cardiac and skeletal myocytes. Furthermore, its high specificity and relatively high sensitivity (88%) for tumors with skeletal muscle differentiation suggest that Cav-3 is a valuable marker for these tumors and may be used to assess the degree of differentiation of RMS and to identify residual tumor cells in post-chemotherapy specimens.

Biomarkers, Tumor↗
PubMed2006

Unusual manifestations of disseminated neoplasia at presentation: right-sided heart failure due to a massive cardiac metastasis and autoimmune thrombocytopenia in pleomorphic rhabdomyosarcoma of the adult.

Although it is recognized that aggressive soft tissue sarcomas may give rise to cardiac metastases, these manifestations are usually late and clinically silent, being the prevailing finding in exceptional cases. This report describes the occurrence of a massive cardiac metastasis at diagnosis of leg rhabdomyosarcoma in a middle-age adult. This manifestation was the cause of rapidly progressive congestive heart failure and, together with the unusual occurrence of autoimmune thrombocytopenia, led to difficult patient care with a significantly negative influence on the outcome.

Adult↗
PubMed2005

Ulcerated rhabdomyosarcoma as portal of entry for tetanus in a previously nonimmunized child.

Infectious diseases represent one of the most important secondary problems related to the treatment of childhood cancer, being the leading cause of death in this population. They are predominantly of bacterial and fungal etiology. The association between tetanus, a bacterial vaccine-preventable disease, and cancer is virtually undescribed. The authors present the case of a previously nonimmunized child, due to his parents' choice, who developed severe tetanus with an ulcerated rhabdomyosarcoma as portal of entry. Due to an unfavorable evolution, the child underwent a hip disarticulation to provide tetanus control. The ulterior tumor management was successful: the child has been off therapy for more than 108 months with no evidence of disease.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2006

Temporal bone rhabdomyosarcoma presenting as acute peripheral facial nerve paralysis.

Facial palsy is not an uncommon presentation to an emergency department. Whereas most patients will ultimately receive a diagnosis of Bell palsy (idiopathic peripheral seventh cranial nerve palsy), a subset will have an identifiable cause for their facial paralysis. Children are more likely to have an identifiable cause than are adults. We present a case in which a child presented with acute peripheral facial nerve palsy and was found to have temporal bone rhabdomyosarcoma. The key clinical finding was the presence of both 7th and 12th cranial nerve palsy.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2006

Use of 2-deoxy-2-[F-18]-fluoro-D-glucose positron emission tomography to monitor therapeutic response by rhabdomyosarcoma in children: report of a retrospective case study.

PURPOSE: The purpose of this study was to study the use of 2-deoxy-2-[F-18]-fluoro-D-glucose positron emission tomography (F-18 FDG PET) for monitoring therapeutic response by rhabdomyosarcoma (RMSA) in children. PATIENTS AND METHODS: A retrospective case study was performed by searching a computer database for the patients with RMSA in whom F-18 FDG PET studies were performed pre- and posttreatment. The data of the PET studies from these patients were analyzed in conjunction with clinical treatment and other imaging studies to determine whether interval changes of F-18 FDG uptake by the RMSA reflect response of RMSA to treatment. RESULTS: Four patients with RMSA who received both pretreatment and posttreatment F-18 FDG PET studies were identified from the database and included in this study. A dramatic decrease of F-18 FDG uptake by the tumor was evident in the patients who had a favorable response to the therapy and prolonged remission of the disease. In contrast, persistent abnormal FDG uptake in one patient was associated with early relapse of the RMSA. CONCLUSIONS: F-18 FDG PET may be useful for monitoring therapeutic response by RMSA in children, which needs to be verified with a prospective study in a larger patient population.

Child↗
PubMed2000

Echovirus infection of rhabdomyosarcoma cells is inhibited by antiserum to the complement control protein CD59.

A number of echoviruses use decay accelerating factor (DAF) as a cellular receptor or attachment protein for cell infection. Binding of echovirus 7 to DAF at the cell surface, but not to soluble DAF in solution, triggers the formation of virus particles exhibiting an altered sedimentation coefficient ('A' particles) which are considered indicative of the particle uncoating process. We have previously demonstrated that antibodies to beta(2)-microglobulin block cell infection at a stage prior to 'A' particle formation and suggested that this reflects the involvement of beta(2)-microglobulin (or the associated MHC-I) in a virus-receptor complex that forms at the cell surface. We demonstrate here that antiserum to CD59 specifically blocks infection of rhabdomyosarcoma cells by a range of echoviruses, including viruses that bind DAF (e. g. echovirus 7) and those that use currently unidentified receptors other than DAF. The block occurs prior to 'A' particle formation and is cell-type specific. The potential role of CD59 as an active member, or passive participant, in the virus-receptor complex is discussed.

Animals↗
PubMed2003

Human rhabdomyosarcoma cells for rapid detection of enteroviruses by shell-vial assay.

The ability of the RD (rhabdomyosarcoma) and MRC-5 cell-lines to detect enteroviruses in 33 clinical samples (cerebrospinal fluid, stools and throat swabs) was evaluated. The samples had previously tested enterovirus-positive by traditional tube-culture and had been frozen after their initial processing. By traditional tube-culture, 100 and 85 % of samples were positive for enterovirus in RD and MRC-5 cells, respectively. By rapid shell-vial assay, 94 and 45.5 % were positive after 48 h incubation in RD and MRC-5 cells, respectively. RD cells supported growth of all enterovirus serotypes, whereas MRC-5 cells were not able to detect any of the three coxsackieviruses that were found (one coxsackievirus A9 and two coxsackievirus B5). The shell-vial assay with RD cell-lines may be a useful tool for rapid diagnosis of enteroviral infection.

Cerebrospinal Fluid↗
PubMed2004

Pax3:Fkhr interferes with embryonic Pax3 and Pax7 function: implications for alveolar rhabdomyosarcoma cell of origin.

To investigate the role of the translocation-associated gene Pax3:Fkhr in alveolar rhabdomyosarcomas, we generated a Cre-mediated conditional knock-in of Pax3:Fkhr into the mouse Pax3 locus. Exploring embryonic tumor cell origins, we replaced a Pax3 allele with Pax3:Fkhr throughout its expression domain, causing dominant-negative effects on Pax3 and paradoxical activation of the Pax3 target gene, c-Met. Ectopic neuroprogenitor cell proliferation also occurs. In contrast, activation later in embryogenesis in cells that express Pax7 results in viable animals with a postnatal growth defect and a moderately decreased Pax7+ muscle satellite cell pool, phenocopying Pax7 deficiency but remarkably not leading to tumors.

Animals↗
PubMed1992

Rhabdomyosarcoma of the head and neck in children.

Twenty-two children, 9 male and 13 female, with a non-orbital rhabdomyosarcoma of the head and neck, treated between 1970 and 1988, have been reviewed. Since 1972, treatment has consisted of combination chemotherapy, and where necessary radiotherapy and/or surgery. Complete clinical remission after initial chemotherapy was observed in 21 children. Six children were cured after primary treatment but 15 developed recurrent disease. Thirteen children had a parameningeal localized tumour, with eventual meningeal involvement in 5. Survival in this group was worse than in the non-meningeal group. Lymph node metastasis at first presentation (5 patients) had no influence on prognosis, whereas development of lymph node metastases during follow-up resulted in 100% mortality. All patients were retrospectively classified according to both the IRS-classification and TNM-descriptive system. No correlation with either system could be established. Fourteen of 15 children with recurrent disease were treated, 4 of whom were cured. Thus, 10 out of 22 (45%) children were long-term survivors.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1991

Primary oesophageal rhabdomyosarcoma.

A case is presented of a rhabdomyosarcoma of the oesophagus with a description of the cytology, light microscopy, and immunocytochemical findings and a discussion of spindle cell tumours occurring at this site. Cytologically, large bizarre shaped pleomorphic cells were seen in which desmin was demonstrated in order to confirm the diagnosis after destaining a Papanicolaou stained slide and restaining it with antibody to desmin.

Carcinoma↗
PubMed2006

Botryoid rhabdomyosarcoma of the biliary tract in children: a unique case report.

Rhabdomyosarcoma (RMS) is the most common tumour of the biliary tree in childhood. In children, it is a rare lesion, accounting for about 1% of all RMS. Hepatobiliary botryoid RMS is a disease affecting young children at a median age of about 3 years. In literature, the radiological findings of hepatobiliary RMS have been described in small series and some case reports. In this case report, we present a rare case of RMS of the extrahepatic biliary tree initially diagnosed as a choledochal cyst.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1990

Comparative phenotypes in rhabdomyosarcomas and developing skeletal muscle.

The morphological and immunohistochemical phenotypes of 51 rhabdomyosarcomas from young people have been described and contrasted with phenotypes in developing skeletal muscle from 20 fetuses and neonates. The tumours express markers in a cumulative and consistent sequence--vimentin, desmin, fast myosin, myoglobin--which evolves pari passu with morphological differentiation and follows the same pattern found in normal myogenesis. Changes in immunohistochemical phenotype are documented in residual and recurrent tumours excised after chemotherapy. The presumptive rhabdomyoblastic nature of some primitive tumours, marking with vimentin alone, is discussed.

Adolescent↗
PubMed1992

Molecular genetic study showing that the IN/157 'oligodendroglioma' cell line has been contaminated by rhabdomyosarcoma (RD) cells.

The IN/157 cell line was originally isolated from a human oligodendroglioma biopsy and has been used in recent years to study aspects of glioma cell biology. We established that IN/157 cells carry a relatively infrequent mutation at position three of codon 61 of the N-ras gene, suggesting that such a mutation may have contributed towards the genesis of the original tumour. However, the mutation was not detectable within the original paraffin-embedded glioma biopsy from which the cell line was supposedly derived. We thus considered the possibility that the cells had been contaminated by another cell line and, by means of DNA fingerprinting, have demonstrated that the contaminating cell line is the rhabdomyosarcoma line RD. We feel that this study makes several important points regarding experiments which make use of cell lines. We discuss the possible implications of contamination events with regard to erroneous conclusions about the biology of the cell lines and tumour types from which they supposedly derive. We also suggest ways in which future contamination-related errors can be minimized.

Base Sequence↗
PubMed1988

Neonatal myosin heavy chains are not expressed in Ni-induced rat rhabdomyosarcoma.

Myosin heavy chain (MHC) composition of chemically-induced rhabdomyosarcoma (RMS) was analyzed by gel electrophoresis and Western blotting using a panel of monoclonal antimyosin antibodies specific for embryonic-, neonatal-, slow- and adult fast-type MHC isoforms. Myosin extracted from tumours and electrophoresed on 6%-sodium dodecyl sulfate (SDS)glycerol gels was found to migrate as three distinct MHC components. These polypeptides were present in different relative amounts in the five RMS studied. Western blotting experiments revealed that variable proportions of embryonic-, slow- and adult fast-, but not neonatal-type, MHC isoforms are consistently expressed in RMS. Indirect and double immunofluorescence procedures applied to cryosections of tumoral tissue showed that: (a) RMS cells were unreactive with antineonatal-type-MHC antibody, (b) the majority of neoplastic, desmin-positive, cells contained embryonic- as well as adult fast-type MHCs and (c) a minority of cells were labelled by anti-slow MHC antibody. The results of this study indicate that there is no obligatory sequence of MHC isoform expression in the molecular transition (emb----neo----adult) which occurs during rat skeletal myogenesis.

Animals↗
PubMed1990

Experimentally induced murine rhabdomyosarcomas--correlation between cellular contacts, matrix formation and cellular differentiation.

Rhabdomyosarcomas (RMSs) consist of a mixture of primitive mesenchymal cells as well as cells showing various stages of rhabdomyomatous differentiation. The qualitative and quantitative degree of the rhabdomyomatous differentiation of the cells, evaluated by their morphology and expression of defined structural and functional proteins, is accepted as the basis of diagnosis and is considered to be related to the biological behaviour of RMSs. Therefore we investigated solid experimentally induced murine RMSs, adherent (subconfluent, confluent) cell cultures obtained therefrom, and also suspension cultures and studied the expression of muscular differentiation markers (vimentin, desmin, myoglobin) and the formation of extracellular matrix components (fibronectin, laminin). When we compared solid tumours with adherent cell cultures of decreasing cell densities (confluent up to single cells) and with cells grown in suspension, we found a gradual decline of differentiation ("dedifferentiation"). This decline paralleled the decrease of cell-cell and cell-substrate contacts. In suspension cultures, cells were prevented from interacting with each other and the substratum, no rhabdomyomatous differentiation of the cells took place. If restoration of cellular contacts was allowed, either by adherent growth or by reinoculation into nude mice, the process of dedifferentiation was completely reversible. Consequently, it was demonstrated that the increase of cell-cell and cell-substrate contacts was strongly associated with the appearance or increasing expression of the desmin intermediate filament cytoskeleton and with formation of the extracellular matrix components fibronectin and laminin. The microfilament (F-actin) system was modulated from an impressive stress-fiber system in subconfluent to a dense network in confluent monolayers. The extent of cell-substrate contacts, mediated by extracellular matrix components, and the number of cell-cell interactions are responsible for the capability of a malignant mesenchymal cell, which is able to undergo rhabdomyomatous differentiation, to achieve the various stages of maturation.

Animals↗
PubMed2006

Orbital rhabdomyosarcoma: multidisciplinary treatment experience.

Orbital rhabdomyosarcoma (RMS) accounts for 10% of childhood RMS and has a relatively good prognosis of up to 85% 5-year survival. Improved survival has led to increased interest in late effects of treatment. The objective of this study was to review the results of treating orbital RMS with multidisciplinary treatment at Women's and Children's Hospital and Royal Adelaide Hospital with emphasis on late effects of treatment. A retrospective review was carried out of all patients with orbital RMS treated with multidisciplinary treatment including radiation therapy and chemotherapy in the two institutions between 1982 and 2002. A total of five patients (age range 5.5-12 years) satisfied the eligibility requirements. Late effects were significant and included facial bone hypoplasia, cataract formation and growth hormone deficiency. Overall survival was 80% (4/5) with mean follow up of 8 years (range 3-13 years). Given the high cure rates achieved, future treatments must aim to maintain the good results but to reduce the high incidence of late effects of treatment. Standardised rating of late toxicity, long-term follow-up clinics, and implementation of modern radiation techniques (3-D conformal radiotherapy, intensity modulated radiotherapy, proton therapy) for patients with orbital RMS are important to improving outcome.

Child↗
PubMed1978

Growth and cytogenetic characteristics of nickel sulphide-induced rhabdomyosarcomas in rats.

Rhabdomyosarcomas induced by single intramuscular injections of nickel sulphide (Ni3S2) in Fischer and Hooded rats were cultured in vivo and in vitro to study their growth characteristics and chromosomal constitution. The tumor cell suspensions cultured in vitro exhibited more myogenic differentiation on the coverslips than those cells grown in vivo in diffusion chambers. A characteristic feature of in vivo cultures was the appearance of microclusters which resembled the primary tumors. Chromosome analyses of primary tumors revealed that a majority of these had a modal number in the diploid or near diploid range. Fischer rat primary tumor cells exhibited abnormal configurations including rings, dicentrics and triradials. A comparison of the chromosome make-up of the primary tumors and their metastases was performed on four sets of tumors. Three out of four metastases examined showed the diploid chromosome make-up characteristic of the primary tumors suggesting that the tumors with the diploid or near diploid chromosome constitution are more likely to produce metastases.

Animals↗