Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “RHABDOMYOSARCOMA”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 343 records · Page 19Linked to original sources
PubMed2000

Primary meningeal rhabdomyosarcoma in a child with hypomelanosis of Ito.

Intracranial rhabdomyosarcomas are rare neoplasms, and those thought to be primary meningeal tumors are even more rare. Hypomelanosis of Ito is a neurocutaneous disorder believed to involve a defect in cells of neural crest origin. We report the case of a 15-month-old boy with hypomelanosis of Ito who developed a primary meningeal rhabdomyosarcoma. The patient initially presented with hydrocephalus and 2 months later developed neurologic signs localizing to the spinal cord. Radiologic studies revealed widespread leptomeningeal enhancement with compression of the spinal cord at C5-C7. A brain biopsy revealed a tumor diffusely involving the meninges. Microscopically, the tumor was composed of rhabdomyoblasts, many of which showed prominent cross-striations on routine hematoxylin-eosin staining. To the best of our knowledge, this is the first reported case of meningeal rhabdomyosarcoma in a patient with hypomelanosis of Ito and the fourth reported case of a primary meningeal rhabdomyosarcoma reported in the world literature.

Actins↗
PubMed1998

[Alveolar rhabdomyosarcoma presenting as acute leukemia].

Rhabdomyosarcoma is the most common soft tissue sarcoma in adolescence and childhood, which manifests by the locally destructive growth of the primary tumor or its metastases. We report on a 29-year-old man with an alveolar rhabdomyosarcoma presenting with an unusual leukemia-like picture. On admission, the patient suffered from diffuse bone pain and renal insufficiency. Peripheral blood analysis showed anaemia, thrombocythaemia and blast-like cells. A bone marrow aspirate revealed extensive infiltration by atypical blast-like cells which were interpreted as acute lymphoblastic leukemia. Although confirmation of this diagnosis by immunophenotyping did not succeed chemotherapy was started immediately and led to partial remission. Histologic analysis of a bone marrow biopsy from the iliac crest, however, revealed an extensive solid tumor with alveolar spaces, lined by primitive round cells with positive PAS-reaction in the cytoplasm. Immunostaining demonstrated a positive reaction of the tumor cells for desmin and in a few tumor cells for smooth-muscle-actin. Chromosomal analysis showed a t(2;13) translocation typical for alveolar rhabdomyosarcoma. Although multiple lytic lesions of the skeletal system became evident during the further clinical course, the site of origin of the primary tumor could not be defined retrospectively. In conclusion, rhabdomyosarcoma should be included in the differential diagnosis of systemic diseases with extensive bone marrow infiltration by tumor cells that could otherwise be misinterpreted as a haematologic malignancy.

Acute Disease↗
PubMed1999

Genes, chromosomes, and rhabdomyosarcoma.

Rhabdomyosarcomas are a heterogeneous group of malignant tumors and are the most common soft-tissue sarcoma of childhood. Rhabdomyosarcomas resemble developing skeletal muscle, notably in their expression of the MRF family of transcription factors and the PAX3 and PAX7 genes. These PAX genes are also involved through specific translocations, t(2;13)(q35;q14) and variant t(1;13)(p36;q14) in the alveolar subtype, which result in PAX3-FKHR and PAX7-FKHR fusion genes, respectively. The fusion genes are thought critically to affect downstream targets of PAX3 and PAX7 or possibly have novel targets. Similar downstream changes may also be involved in embryonal and fusion gene negative cases. Genomic amplification of such genes as MYCN, MDM2, CDK4, and PAX7-FKHR is a feature mainly of the alveolar subtype, while specific chromosomal gains, including chromosomes 2, 8, 12, and 13, are associated with the embryonal subtype. Loss of alleles and imprinting at 11p15.5 and disruption of genes such as IGF2, ATR, PTC, P16, and TP53 have also been implicated in rhabdomyosarcoma development. Whereas there is now a realistic possibility of cure in the majority of cases, there remains a subset that is resistant to multimodality therapy, including high-dose chemotherapy. Characterization of the defining molecular features of tumors that are likely to behave aggressively represents a particular challenge. Current research is leading toward a better understanding of rhabdomyosarcoma tumorigenesis, which may ultimately result in novel therapeutic strategies that increase the overall cure. Genes Chromosomes Cancer 26:275-285, 1999.

Animals↗
PubMed2001

[Alveolar rhabdomyosarcoma: apropos of a rare location].

Rhabdomyosarcoma is a soft tissue tumor of striated muscle origin. It is observed mainly in children and adolescents. Rhabdomyosarcoma predominantly occurs in three regions: head and neck, genito-urinary tract and retroperitoneum, and upper and lower extremities. We report a case of primary alveolar rhabdomyosarcoma of the mesentery occurring in a 68-year-old man. Rhabdomyosarcomas can be diagnosed on microscopic findings and immunohistochemical features. Cytogenetic findings can be helpful in the diagnosis.

Aged↗
PubMed1975

Pleomorphic Rhabdomyosarcoma: response to irradiation.

Although the usefulness of radiation in treatment of many embryonal rhabdomyosarcomas is recognized, the pleomorphic rhabdomyosarcomas are frequently felt to be radioresistant and thus a potentially useful mode of therapy is overlooked. There is increasing evidence in the literature to document successful local management of pleomorphic rhabdomyosarcomas with radiation. The literature is reviewed briefly and a case of rhabdomyosarcoma of the heart is reported in which radiation in doses well tolerated by normal tissues successfully controlled several local lesions at various sites until the patient's demise from widespread disease 2 years after the initial treatment.

Aged↗
PubMed1975

[Effect of ectopic transplantations on the cytological structure of mouse rhabdomyosarcomas].

12 mouse rhabdomyosarcomas from V--XI transplant generations were investigated at their growing in the anterior chamber of the eye, in the testis, lungs and subcutaneous fibrous tissue. With these modes of transplantation rhabdomyosarcomas were seen differentiating to different levels and differed in their cell populations. The most differentiated rhabdomyosarcomas were in the anterior chamber of the eye: they formed myotubes with cross-striated myofibrilles; the least differentiated ones occurring in lungs. Rhabdomyosarcomas, inoculated in testes, intensively dessiminated in the lymph-nodes and in the organs of abdomen. It is concluded that the degree of tumor autonomy depends on the tumor--organism interactions.

Animals↗
PubMedDate not supplied

Primary pleomorphic rhabdomyosarcoma of the liver: a case report.

Primary intrahepatic rhabdomyosarcoma is extremely rare in children. We describe a case of pleomorphic rhabdomyosarcoma originating from the liver in an eight-year-old boy presenting with abdominal pain, spiking fever and a rapidly growing abdominal mass for one week. Preoperative imaging studies revealed a large solid tumor in the right lobe of the liver without any tumor elsewhere in the body. Serological study was negative for HBsAg and positive for anti-HBs. Biochemical tests including serum glutamic-oxalacetic and glutamic-pyruvic transaminase, alkaline phosphatase, bilirubin and alpha-fetoprotein were all within normal limits. The tumor was removed by an extended right hepatectomy. Histological and immunohistochemical examination confirmed a pleomorphic rhabdomyosarcoma. The patient eventually succumbed to tumor recurrence with massive internal hemorrhage two months after resection of the tumor. This is the first report of primary pleomorphic rhabdomyosarcoma of the liver in children. Abdominal ultrasonography, computed tomography scan and angiography are valuable for preoperative planning but the imaging findings are non-specific. Pathological examination with immunohistochemical stains remains the most important method in arriving at the exact diagnosis. The poor prognosis and early death of most previously reported cases imply the need for investigation of a more effective treatment method of this uncommon tumor.

Child↗
PubMed2003

Development of rhabdomyosarcoma in HER-2/neu transgenic p53 mutant mice.

Rhabdomyosarcomas derive from the skeletal muscle lineage and harbor a variety of genetic and molecular lesions. However, it is not clear which molecular alterations have a pathogenetic role. We show that activation of the HER-2/neu oncogene coupled with inactivation of the oncosuppressor gene p53 causes rhabdomyosarcoma in mice. At the age of 11-21 weeks, all male mice carrying both genetic lesions developed embryonal rhabdomyosarcomas expressing desmin, myosin, and insulin-like growth factor-II, in the genitourinary tract. Our findings led to the hypothesis that the interaction between HER family genes and the p53 pathway might be involved in the origin of human rhabdomyosarcoma.

Alleles↗
PubMed1992

The insulin-like growth factor II (IGF-II)/mannose 6-phosphate receptor mediates IGF-II-induced motility in human rhabdomyosarcoma cells.

Insulin-like growth factor-II (IGF-II) is an autocrine growth and motility factor for human rhabdomyosarcoma. It interacts with three different receptors: the IGF-I, the IGF-II, and the insulin receptor. A specific function of the IGF-II receptor in mediating IGF-II responses has not been defined. In this report we investigate the mechanism of IGF-II-mediated motility in rhabdomyosarcoma cells. We demonstrate that IGF-II and [Leu27]IGF-II, an analog selective for the IGF-II receptor, stimulate motility at concentrations in which they interact only with their own receptor. An antibody that blocks the IGF-I receptor does not inhibit either peptide activity, while an antibody specific for the IGF-II receptor suppresses the IGF-II-induced motility. This antibody does not interfere with rhabdomyosarcoma cell proliferation. We conclude that in rhabdomyosarcoma cells IGF-II stimulates two different responses mediated by distinct receptors: 1) a mitogenic response through the type I receptor and 2) a motility response through the type II receptor.

Affinity Labels↗
PubMed2004

Expression of multidrug resistance-1 and multidrug resistance-associated protein genes in pediatric rhabdomyosarcoma.

Overexpression of multidrug resistance-1 (MDR-1), and multidrug resistance-associated protein (MRP) genes has been linked with resistance to chemotherapy in vitro and in vivo. Their role in chemotherapy resistance in pediatric rhabdomyosarcoma is unclear. The study was undertaken to analyze the expression of MDR-1 and MRP genes in the embryonal and the alveolar subtypes of rhabdomyosarcoma and to elucidate its clinical relevance. Twenty-three rhabdomyosarcoma samples were analyzed for the expression of MDR-1 and MRP genes using a semi-quantitative competitive RT-PCR assay. MRP gene expression was associated with a reduction in survival (p=0.02). The overall survival of patients with tumors positive or negative for MRP expression were 50% (95% confidence interval, 30-70%) and 93% (95% confidence interval, 76-100%) respectively. In contrast, the expression of MDR-1 gene was not predictive of survival. These findings suggest that MRP expression could be a prognostic factor in patients with rhabdomyosarcoma.

ATP Binding Cassette Transporter, Subfamily B, Mem↗
PubMed2004

Veno-occlusive disease in pediatric patients receiving actinomycin D and vincristine only for the treatment of rhabdomyosarcoma.

OBJECTIVES: Veno-occlusive disease (VOD) following standard chemotherapy has been reported in patients receiving vincristine actinomycin D, and cyclophosphamide for the treatment of Wilms tumor and more rarely rhabdomyosarcoma. The dose and schedule of administration of actinomycin D in patients with Wilms tumor and the increased dose of cyclophosphamide administered to patients with rhabdomyosarcoma have been considered the likely etiology for VOD. METHODS: The authors report four cases of VOD in patients with rhabdomyosarcoma treated with vincristine and actinomycin D only. No risk factors for the development of VOD were identified. VOD was diagnosed clinically by the presence of at least two of three findings as defined by McDonald et al. VOD occurred after two to four doses of actinomycin D and approximately 7 to 14 days after the dose. All patients recovered with no evidence of permanent hepatic damage. CONCLUSIONS: VOD can occur in patients with "low-stage" rhabdomyosarcoma treated with vincristine and actinomycin D alone. Although chemotherapy-related VOD is a potentially severe disease, the outcome is good and resumption of chemotherapy is well tolerated.

Adolescent↗
PubMed2005

Rhabdomyosarcoma in childhood: a retrospective analysis of 190 patients treated at a single institution.

OBJECTIVES: The study goal was to retrospectively review the treatment results of childhood rhabdomyosarcoma and identify prognostic factors that affect treatment outcome. PATIENTS AND METHODS: The records of 190 patients with childhood rhabdomyosarcoma treated between January 1991 and December 1999 were reviewed. The data were analyzed for clinico-epidemiological factors and the impact of potential prognostic factors on failure-free survival. Factors evaluated were age, gender, histology type, primary site, tumor size, Intergroup Rhabdomyosarcoma Study (IRS) group, surgical procedure, and the use of radiation treatment. RESULTS: The 5-year actuarial FFS and OS were 40% and 50%, respectively. The only significant prognostic factors as estimated by univariate analysis were histology type (p=0.01), primary site (p=0.002), tumor size (p=0.049), IRS-group (p=0.003), surgical procedure (p=0.002), and radiation treatment (p=0.001). Multivariate analysis showed that histology type (p=0.02), primary site, and IRS-group (p=0.02) were the only independent prognostic factors. CONCLUSIONS: This analysis demonstrates that failure-free survival for rhabdomyosarcoma is dependent on several factors at the time of initial diagnosis, including histologic subtype, primary site and disease group. Our treatment results were inferior compared to IRS-studies as the patients during this period were treated on individual bases and not standardized protocol.

Adolescent↗
PubMed2006

Rhabdomyosarcoma of the extremities in adults.

Rhabdomyosarcomas are uncommon in adults and literature regarding their management is limited. Eight patients with an average age of 32.6 years (range: 21 to 75) who were treated for rhabdomyosarcomas on the extremities between 1991 and 2002 in a regional centre for the treatment of soft tissue sarcomas were studied retrospectively. Treatment consisted of en bloc resection of the primary tumour in all the patients, combined with radiotherapy or chemotherapy or both. The tumour size ranged from 5.5 cm to 15 cm (average 9.3 cm). Histologically there were four alveolar, two pleomorphic, one embryonal and one anaplastic subtype. Seven of the eight patients developed metastasis and died after an average period of 15.3 months (range 4-28 months). At the final follow-up, only one patient was disease free and alive 48 months after surgery. Extremity rhabdomyosarcoma is a highly malignant tumour and our results are poor compared to the reported results in children. Metastatic recurrence is high in adult rhabdomyosarcomas even with local control of the disease and therefore development of effective systemic therapy is an urgent priority.

Adult↗
PubMed2004

[Rhabdomyosarcoma development in Trp53/fos mutant mice: tumor suppressor functions of the Fos protooncogene].

Rhabdomyosarcoma is the most common soft tissue sarcoma in children, yet the genetic changes causing this disease are poorly understood. The Fos protein, a major component of the AP-1 transcription factor, is essential for osteoclast differentiation, acts as an oncogene, potentiates transforming signals and controls invasive growth and angiogenesis during tumor progression. To genetically investigate a potential interaction between the p53 and Fos pathways, Trp53/Fos double knock-out mice were generated. These mice develop highly proliferative and invasive rhabdomyosarcomas of the facial and orbital regions with more than 90% penetrance at 6 months of age. Expression of Fos in Trp53/Fos mutant rhabdomyosarcoma cell lines established from primary tumors is associated with enhanced apoptosis and downregulation of Pax7 expression. Our results show that Trp53/Fos double knock-out mice recapitulate major aspects of human rhabdomyosarcoma development and therefore provide a mouse model for the human disease. Furthermore, this study identifies a novel and unexpected tumor suppressive function of the Fos proto-oncogene.

Animals↗
PubMed1991

Genitourinary rhabdomyosarcoma in children.

The survival rates for childhood rhabdomyosarcomas have risen from 20% to 50% with the institution of a multidisciplinary approach involving the pediatric oncologist, radiation therapist, and surgeon. Increased survival has been particularly striking in the case of genitourinary rhabdomyosarcomas, which have a more favorable prognosis than rhabdomyosarcomas occurring in other anatomic sites. By stratifying genitourinary rhabdomyosarcomas by anatomic subgroup, i.e., paratesticular, bladder or bladder-prostate, and gynecologic, insight into specific predictors of failure can be obtained. Patients judged to be high risk at diagnosis can then be selected for more intensive or novel forms of therapy.

Child↗
PubMed1991

[A case of brain metastasis of rhabdomyosarcoma in a child].

We report here a specific case of metastatic brain tumor orienting from rhabdomyosarcoma. An 11-year-old boy came to our hospital with complaints including headache, exophthalmos and tumors in the right frontal and left occipital region. 6 months previously subtotal removal of rhabdomyosarcoma in his right foot had been performed. CT scan demonstrated tumors in the right orbit, right frontal region and left occipital region. The tumors existed bilaterally in the skull and markedly enhanced by contrast medium. But the changes of the cranium were mild, only the dipole was slightly dilated. MRI showed that the tumors were extraaxial masses. Right external carotid angiogram revealed numerous tumor vessels fed by the meningeal artery. On April 3rd, biopsy was performed at the right frontal region. The tumor had spread in the epidural space but the cortical surface seemed to be intact. Histologically, the tumor consisted of small round cells and revealed multinuclear giant cells. It involved the skull bone, dura mater and subdural tissues. Accordingly the tumor was diagnosed as rhabdomyosarcoma. Although tumor increased rapidly, the intracranial region did not change remarkably. It was an interesting phenomenon. Eventually, rhabdomyosarcoma metastasized to the lung, and the patient died due to respiratory distress.

Brain Neoplasms↗
PubMed1990

Bone tumors of mixed origin: osteo-liposarcoma and osteo-rhabdomyosarcoma.

1. A complete perusal of the literature revealed twenty cases of primary liposarcoma of bone acceptable as such to the authors. These were tabulated as to location and age. 2. Eight cases of osteo-liposarcoma, primary in bone, were encountered in the literature and an additional case was reported by the authors. 3. The authors described for the first time in the literature a new primary tumor of bone of mixed origin: osteo-rhabdomyosarcoma. After careful perusal of the literature they added three additional cases: two cases, previously reported as primary rhabdomyosarcoma of bone, which on careful evaluation of the radiographs in said publications and the paucity of microphotographs they considered to be osteo-rhabdomyosarcomas, and the other case, previously reported as malignant mesenchymoma of the sternum following radiotherapy for breast cancer. 4. The authors prefer to classify these tumors (osteo-liposarcoma and osteo-rhabdomyosarcoma) as "Tumors of Mixed Origin" and not as "Malignant Mesenchymomas". 5. A complete review of the literature revealed 219 reported "dedifferentiated" chondrosarcomas, or chondrosarcomas "with additional mesenchymal component", among which only nine (9) contained a bona fide rhabdomyosarcomatous component. The rest exhibited other mesenchymal tumors as osteogenic sarcoma, fibrosarcoma, malignant fibrous histiocytoma, angiosarcoma, and undifferentiated sarcoma. The authors recommend to continue classifying these tumors as chondrosarcomas with additional mesenchymal component or even as "dedifferentiated" chondrosarcomas but not as malignant mesenchymomas.

Adolescent↗
PubMed1990

[Comparative study on ultrastructure of rhabdomyosarcoma and embryonal striated muscle].

Twelve cases of rhabdomyosarcoma identified by electron microscopy were studied. Of them, 6 were diagnosed originally as rhabdomyosarcoma and 6 as other tumors by light microscopy. Five types of tumor cells, primitive mesenchymal cells, undifferentiated myoblasts, poorly differentiated myoblasts, well-differentiated myoblasts and leptomeric fibril myoblasts were observed under electron microscope. Striated muscle tissue from 5 cases of 5-8 week old human embryo was used as control. There was a correlation in differentiation between rhabdomyosarcoma and embryonal striated muscle. Based on their fine features, the diagnosis of rhabdomyosarcoma is discussed.

Adolescent↗