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PubMed1982

Immuno-histochemical localization of myoglobin in human muscle tissue and embryonal and alveolar rhabdomyosarcoma.

An immuno-histochemical investigation of the presence and localization of myoglobin was performed on cardiac and skeletal muscle tissue and on 9 embryonal and 9 alveolar rhabdomyosarcomas, utilizing an immunoperoxidase technique. Cardiac muscle fibres were evenly stained whereas staining of skeletal muscle fibres varied, giving a mosaic-like pattern. Of the fixatives used (4 percent formaldehyde, 4 percent paraformaldehyde, formaldehyde-glutaraldehyde, and Bouin's fixative), 4 percent formaldehyde gave the most prominent staining; short fixation-time slightly increased the staining intensity. All the 9 embryonal and 9 alveolar rhabdomyosarcomas contained positively-stained tumour cells. The number of cells positively-stained and the intensity of the staining varied with differentiation: the most differentiated rhabdomyoblasts stained most intensely. However, also many poorly-differentiated tumour cells in highly cellular areas were positive. Myoglobin is considered a suitable marker of rhabdomyoblastic differentiation, thus the method used in this study may be valuable in the diagnosis.

Cell Differentiation↗
PubMed1982

Alveolar rhabdomyosarcoma: a cytogenetic and correlated cytological and histological study.

A correlative, histologic and cytologic study of seven cases of alveolar rhabdomyosarcoma and chromosome analysis using the G-banding technique (in case 1) is presented. The histologic pattern was characterized by pseudo-alveolar structures, separated by fibrous septa. Primitive, dark, round cells predominated, but differentiated rhabdomyoblasts were found in all cases, and so were multi-nucleated giant cells. Unequivocal cross striation was seen in two cases. Cytologically, the tumors were highly cellular and small, undifferentiated tumor cells predominated. Large polymorphous, sometimes multi-nucleated, cells often with an elongated cytoplasm and eccentric nucleus were observed in all cases. The cell-types found could be well correlated to those identified in the histologic sections. The cytogenetic study revealed a karyotype profile, showing three 2q markers and only one chromosome type No. 13. The observations are different from the karyotype profile of embryonal rhabdomyosarcoma. However, further studies are necessary to elucidate this question.

Adolescent↗
PubMed1996

Pericardial effusion in a dog with rhabdomyosarcoma in the right ventricular wall.

A seven-year-old male labrador retriever presented in right heart failure with weak femoral pulses, and pleural, abdominal and mild pericardial effusion. No diagnosis could be established initially. Two days later, the dog developed severe pericardial effusion causing cardiac tamponade. A tumour in the right ventricular wall was visualised on ultrasonographic examination. An exploratory thoracotomy was performed and biopsies of the mass submitted for histopathological examination. A diagnosis of rhabdomyosarcoma arising from the myocardium was established. Cardiac rhabdomyosarcoma has been reported in only two dogs. Neither report was associated with pericardial effusion.

Animals↗
PubMed1998

Rhabdomyosarcoma of the tongue in a dog.

A lingual rhabdomyosarcoma was diagnosed in a six-year-old Staffordshire bull terrier with clinical signs of dysphagia. The unsuccessful response to initial chemotherapy, to surgical resection and subsequent surgical resection and radiation therapy is documented. The accurate diagnosis and prognosis of such tumours is also discussed. Lingual rhabdomyosarcoma has not previously been reported in the dog.

Animals↗
PubMed2000

Pathological femoral fracture due to rhabdomyosarcoma in a cat.

An eight-year-old neutered male domestic shorthaired cat was presented with non-weightbearing lameness of the right hindlimb. On radiography, a pathological, metaphyseal femoral fracture was apparent, with areas of bony lysis directly surrounding the fracture site. The cat underwent amputation of the limb. Histopathological examination revealed the presence of a tumour of mesenchymal cells with cytoplasm-containing vacuoles which stained positive with the periodic acid-schiff reaction, indicative of glycogen granules that may be found in rhabdomyosarcoma cells. In addition, the tumour cells stained positive for vimentin and desmin on immunohistochemical examination, thus confirming the diagnosis of rhabdomyosarcoma. In spite of the radical surgery performed, the cat was euthanased due to a recurrence at the amputation site four and a half months later.

Amputation, Surgical↗
PubMed2002

Perianal rhabdomyosarcoma in a dog.

A perianal rhabdomyosarcoma was diagnosed in an 11-year-old neutered male Labrador retriever. Following two incomplete surgical excisions of the tumour, the dog was treated by means of surgery combined with local radiotherapy and systemic chemotherapy using one cycle of vincristine sulphate, doxorubicin and cyclophosphamide (VAC protocol). The dog died 252 days after the first combined therapy. Radiography at this time demonstrated enlargement of the iliac lymph nodes, suggesting metastasis of the tumour. To the authors' knowledge, this is the first report of treatment of canine perianal rhabdomyosarcoma.

Animals↗
PubMed2002

Rhabdomyosarcoma (botryoid sarcoma) of the urinary bladder in a Maltese.

A urinary bladder tumour was diagnosed in a two-year-old female Maltese with haematuria and pollakiuria on the basis of ultrasonography and pneumocystography findings. The mass was resected, and the bladder was preserved at surgery. Histological and immunohistochemical examination confirmed the tumour to be a rhabdomyosarcoma, which has rarely been reported in small breeds of dog. There was no recurrence of the tumour at the original site in the urinary bladder two months later, when the dog died due to metastasis to the liver. This is believed to be the first report of bladder rhabdomyosarcoma in a Maltese.

Animals↗
PubMed1995

The PAX3-FKHR fusion protein created by the t(2;13) translocation in alveolar rhabdomyosarcomas is a more potent transcriptional activator than PAX3.

Alveolar rhabdomyosarcomas are pediatric solid tumors with a hallmark cytogenetic abnormality: translocation of chromosomes 2 and 13 [t(2;13) (q35;q14)]. The genes on each chromosome involved in this translocation have been identified as the transcription factor-encoding genes PAX3 and FKHR. The NH2-terminal paired box and homeodomain DNA-binding domains of PAX3 are fused in frame to COOH-terminal regions of the chromosome 13-derived FKHR gene, a novel member of the forkhead DNA-binding domain family. To determine the role of the fusion protein in transcriptional regulation and oncogenesis, we identified the PAX3-FKHR fusion protein and characterized its function(s) as a transcription factor relative to wild-type PAX3. Antisera specific to PAX3 and FKHR were developed and used to examine PAX3 and PAX3-FKHR expression in tumor cell lines. Sequential immunoprecipitations with anti-PAX3 and anti-FKHR sera demonstrated expression of a 97-kDa PAX3-FKHR fusion protein in the t(2;13)-positive rhabdomyosarcoma Rh30 cell line and verified that a single polypeptide contains epitopes derived from each protein. The PAX3-FKHR protein was localized to the nucleus in Rh30 cells, as was wild-type PAX3, in t(2;13)-negative A673 cells. In gel shift assays using a canonical PAX binding site (e5 sequence), we found that DNA binding of PAX3-FKHR was significantly impaired relative to that of PAX3 despite the two proteins having identical PAX DNA-binding domains. However, the PAX3-FKHR fusion protein was a much more potent transcriptional activator than PAX3 as determined by transient cotransfection assays using e5-CAT reporter plasmids. The PAX3-FKHR protein may function as an oncogenic transcription factor by enhanced activation of normal PAX3 target genes.

Animals↗
PubMed1985

Unexpected cure in metastatic rhabdomyosarcoma.

A 3 year old boy presented with a presumably incurable form of rhabdomyosarcoma. Conventional treatment for advanced stage rhabdomyosarcoma (chemotherapy plus radiotherapy) was not used and intensive chemotherapy alone was given, resulting in an apparent cure. Our treatment rationale is discussed.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2001

Growth hormone and segmental growth in survivors of head and neck embryonal rhabdomyosarcoma.

AIMS: To assess the impact of treatment for embryonal rhabdomyosarcoma on spinal growth and limb length and examine the response of these parameters to growth hormone (GH) treatment. METHODS: We conducted a retrospective case note review of 17 survivors of head and neck rhabdomyosarcoma followed up at a single institution. All children had been treated with chemotherapy and local radiotherapy. Growth velocity, height, sitting height, and subischial limb length SDS scores were analysed. RESULTS: Growth failure secondary to isolated GH deficiency (GHD) developed in 7/17 patients. GHD occurred at a median (range) of 3.4 (1.3-9.9) years after radiotherapy tumour doses of 46 (40-50) Gy. Growth velocity, height, and subischial limb length SDS were significantly reduced in the GHD group and improved with GH therapy. CONCLUSIONS: GH treatment resulted in a significant improvement in sitting height SDS. We discuss the unexpected improvement in spinal growth in survivors with GHD.

Body Height↗
PubMed1977

Alveolar rhabdomyosarcoma presenting as subacute intravascular coagulation.

A patient with rhabdomyosarcoma is described in whom the presenting clinical and laboratory features were those of disseminated intravascular coagulation. The patient's rapid downhill course was primarily expressed by haemorrhagic tendency. An alveolar rhabdomyosarcoma, affecting many organs, including vascular and cardiac lumina, was found at necropsy and was considered to be the cause of the consumption coagulopathy.

Adult↗
PubMed1980

Perineal rhabdomyosarcoma in a newborn child: pathological and biochemical studies with emphasis on contractile proteins.

Histological and ultrastructural studies have been undertaken on a perineal rhabdomyosarcoma from a newborn child. The spontaneous tumour has the typical feature of mesenchymoma. The recurrent tumour, however, displays some rhabdopoietic characteristics. The myosin of the recurrent tumour has been extracted and compared with human fetal myosin. These two myosins are identical in their synthetic filaments and their light-chain composition. Nevertheless, whereas the ATPase activity of fetal myosin can be stimulated considerably by increasing the ca2+ concentration, that of tumoral myosin remains very low. These results show that there are isoenzymes of myosins and there must be differences in the myosin heavy chains, particularly in the active sites. These findings are identical with those seen in experimental rhabdomyosarcoma.

Female↗
PubMed1984

An investigation of beta enolase as a histological marker of rhabdomyosarcoma.

Sections from 21 tumours diagnosed as primary or metastatic rhabdomyosarcoma were stained for alpha and beta enolase. The cases were subdivided into embryonal and alveolar subtypes (38% and 62%, respectively). Positive cytoplasmic staining for alpha enolase was seen in all but one case, and cytoplasmic staining for beta enolase was seen in some cells in 18 of the 21 cases (86% of the total, 88% of the alveolar subgroup, and 85% of the embryonal subgroup). No cells stained positively for beta enolase in the control series of neuroblastomas, fibrosarcomas, Wilms' sarcomas, and an osteosarcoma. The results show that beta enolase is a sensitive marker of muscular differentiation in rhabdomyosarcoma.

Clinical Enzyme Tests↗
PubMed1994

Lipid rich rhabdomyosarcoma.

A lipid rich rhabdomyosarcoma of the paratesticular region was studied by light microscopy, histochemistry, immunohistochemistry and electron microscopy. The tumour was composed of primitive looking, vacuolated, and pleomorphic cells. Lipid was present in varying amounts in all cells but was especially abundant in the vacuolated and pleomorphic cells. Some cells showed eosinophilic fibrillary cytoplasm but cross-striations were not seen. Tumour cells were positive for desmin, muscle specific actin, and vimentin. A few cells were myoglobin positive. At electron microscopy, the presence of lipid was confirmed, while thick and thin filaments, Z disks, lamina and glycogen were observed, thereby confirming striated muscle differentiation. Although moderate amounts of lipid can be expected in almost any tumour, lipid rich rhabdomyosarcomas have received little attention. The present report provides a comprehensively examined case of such a tumour initially presenting diagnostic difficulty because of its possible confusion with liposarcoma.

Adult↗
PubMed1995

Desmin expression in rhabdomyosarcoma: influence of the desmin clone and immunohistochemical method.

AIM: To determine which, if any, of five commercially available desmin clones is most reliable at labelling desmin filaments and whether the enhanced polymer one step (EPOS) method of labelling is of any advantage in the routine diagnostic laboratory. METHODS: Thirty four rhabdomyosarcomas from the files at The Hospital for Sick Children, Great Ormond Street, London, were studied. Four different desmin clones, DE-R-11, D33, DE-U-10, and PDE, were applied to each using the conventional extravidin biotin peroxidase method. The D33 clone was also applied using the EPOS method. RESULTS: The EPOS method incorporating D33 persistently scored more cells as desmin positive and was positive in four cases which were negative on staining with the other clones. CONCLUSIONS: The D33 desmin clone used with the EPOS method is more reliable for identifying desmin filaments in tumours than other desmin antibodies tested. Different desmin clones using a routine technique label different rhabdomyosarcoma cells and therefore it is justifiable to use more than one clone.

Desmin↗
PubMed2005

New genetic tactics to model alveolar rhabdomyosarcoma in the mouse.

Using conditional knock-in and knock-out techniques, we designed a mouse model of the childhood muscle cancer alveolar rhabdomyosarcoma (ARMS) that is driven by the chromosomal translocation product, Pax3:Fkhr. Tumors that closely recapitulate the spectrum of molecular markers and histology seen in human ARMS are exclusively produced in this model. Unexpectedly, expression of Pax3:Fkhr in muscle satellite cells did not produce tumors, but it did in differentiating myofibers. Expression of Pax3:Fkhr in muscle is necessary but not sufficient to initiate tumorigenesis at high frequency. This model offers new insight into the roots of alveolar rhabdomyosarcoma and illustrates the utility of Cre-loxP technology for studying otherwise inaccessible cancers in the mouse.

Animals↗
PubMed2025

PAX3-FOXO1 Drives Targetable Cell State-Dependent Metabolic Vulnerabilities in Rhabdomyosarcoma.

UNLABELLED: PAX3-FOXO1, an oncogenic transcription factor, drives a particularly aggressive subtype of rhabdomyosarcoma (RMS) by enforcing gene expression programs that support malignant cell states. In this study, we show that PAX3-FOXO1+ RMS cells exhibit altered pyrimidine metabolism and increased dependence on enzymes involved in de novo pyrimidine synthesis, including dihydrofolate reductase (DHFR). Consequently, PAX3-FOXO1+ cells display increased sensitivity to inhibition of DHFR by the chemotherapeutic drug methotrexate, and this dependence is rescued by provision of pyrimidine nucleotides. Methotrexate treatment mimics the metabolic and transcriptional impact of PAX3-FOXO1 silencing, reducing expression of genes related to PAX3-FOXO1-driven malignant cell states. Accordingly, methotrexate treatment slows the growth of multiple PAX3-FOXO1+ tumor xenograft models but not their fusion-negative counterparts. Taken together, these data demonstrate that PAX3-FOXO1 induces cell states characterized by altered pyrimidine dependence and nominates methotrexate as an addition to the current therapeutic arsenal for treatment of these malignant pediatric tumors. SIGNIFICANCE: PAX3-FOXO1+ rhabdomyosarcoma cells and tumors exhibit increased sensitivity to DHFR inhibition via methotrexate, identifying a potential therapeutic vulnerability that can be exploited to treat this aggressive pediatric sarcoma.

Humans↗
PubMed2004

Influence of casein kinase II in tumor necrosis factor-related apoptosis-inducing ligand-induced apoptosis in human rhabdomyosarcoma cells.

Tumor necrosis factor-related apoptosis-inducing ligand (TRAIL) induces apoptosis via the death receptors DR4 and DR5 in transformed cells in vitro and exhibits potent antitumor activity in vivo with minor side effects. Protein kinase casein kinase II (CK2) is increased in response to diverse growth stimuli and is aberrantly elevated in a variety of human cancers. Rhabdomyosarcoma tumors are the most common soft-tissue sarcoma in childhood. In this investigation, we demonstrate that CK2 is a key survival factor that protects tumor cells from TRAIL-induced apoptosis. We have demonstrated that inhibition of CK2 phosphorylation events by 5,6-dichlorobenzimidazole (DRB) resulted in dramatic sensitization of tumor cells to TRAIL-induced apoptosis. CK2 inhibition also induced rapid cleavage of caspase-8, -9, and -3, as well as the caspase substrate poly(ADP-ribose) polymerase after TRAIL treatment. Overexpression of Bcl-2 protected cells from TRAIL-induced apoptosis in the presence of the CK2 inhibitor. Death signaling by TRAIL in these cells was Fas-associated death domain and caspase dependent because dominant negative Fas-associated death domain or the cowpox interleukin 1beta-converting enzyme inhibitor protein cytokine response modifier A prevented apoptosis in the presence of DRB. Analysis of death-inducing signaling complex (DISC) formation demonstrated that inhibition of CK2 by DRB increased the level of recruitment of procaspase-8 to the DISC and enhanced caspase-8-mediated cleavage of Bid, thereby increasing the release of the proapoptotic factors cytochrome c, HtrA2/Omi, Smac/DIABLO, and apoptosis inducing factor (AIF) from the mitochondria, with subsequent degradation of X-linked inhibitor of apoptosis protein (XIAP). To further interfere with CK2 function, JR1 and Rh30 cells were transfected with either short hairpin RNA targeted to CK2alpha or kinase-inactive CK2alpha (K68M) or CK2alpha' (K69M). Data show that the CK2 kinase activity was abrogated and that TRAIL sensitivity in both cell lines was increased. Silencing of CK2alpha expression with short hairpin RNA was also associated with degradation of XIAP. These findings suggest that CK2 regulates TRAIL signaling in rhabdomyosarcoma by modulating TRAIL-induced DISC formation and XIAP expression.

Adaptor Proteins, Signal Transducing↗