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 1,747 records · Page 97Linked to original sources
PubMed2002

Lactoferrin inhibits enterovirus 71 infection of human embryonal rhabdomyosarcoma cells in vitro.

Enterovirus 71 (EV71), the newest member of Enterovirudae, is notable for its etiological role in epidemics of severe neurological diseases in children. It appears to be emerging as an important virulent neurotropic enterovirus in the upcoming era of poliomyelitis eradication, whereas no effective vaccine or antiviral agents are available at this moment. Human and bovine lactoferrins, iron-binding proteins belonging to the nonimmune defense system, were assayed in vitro to assess their inhibiting capacity on the cytopathic effect of EV71 on human embryonal rhabdomyosarcoma (RD) cells. Both bovine and human lactoferrins were found to be potent inhibitors of EV71 infection (mean IC(50), 10.5-24.5 microg/mL and 103.3-185.0 microg/mL, respectively). Lactoferrin probably exerts its effect at the level of viral adsorption, since the ongoing infection could not be further inhibited after the EV71 penetrated RD cells.

Antiviral Agents↗
PubMed2005

Radioimmunotargeting of human rhabdomyosarcoma using monoclonal antibody 8H9.

Although metastatic rhabdomyosarcoma (RMS) is chemotherapy and radiotherapy responsive, few patients are cured. 8H9, a murine IgG(1) monoclonal antibody, recognizes a unique cell surface tumor antigen broadly distributed on neuroectodermal, epithelial, and mesenchymal tumors, including RMS. We now report on the in vitro characterization of radiolabeled 8H9 and its in vivo immunotargeting potential in mice with subcutaneous human RMS. Saturation-binding studies carried out to determine (125)I-8H9 affinity to the RMS cell line HTB82 demonstrated that (125)I-8H9 had a K(d) of 10.3nM with an estimated 115,000 binding sites on every HTB82 cell. (125)I-8H9 was retained on the cell surface without significant internalization. Biodistribution of (125)I-8H9 was studied in athymic mice bearing HTB82 xenografts. Following intravenous injection of 4.44MBq of (125)I-8H9, selective tumor uptake was evident 4 to 172 hours after injection. Average tumor uptake was 7.0 +/- 1.8, 11.5 +/- 3.9, 15.1 +/- 3.7, and 5.4 +/- 1.2% injected dose per gram at 4, 24, 48, and 172 hours, respectively. Mean tumor: tissue ratios were maximal at 172 hours (for lung, 4, kidney, 6, liver, 7, spleen, 11, femur, 14, muscle, 18, and brain, 48). Established RMS xenografts treated with a single injection of 18.5 MBq (131)I-8H9 were significantly suppressed compared to controls. Radiolabeled 8H9 effectively targeted RMS xenografts and may have a potential clinical role in radioimmunotherapy.

Animals↗
PubMed1997

Subtractive cloning and characterization of DRAL, a novel LIM-domain protein down-regulated in rhabdomyosarcoma.

A subtractive cloning procedure was used to characterize the molecular changes involved in transformation of normal myoblasts to rhabdomyosarcoma (RMS) cells. Here we describe the cloning of DRAL, a novel LIM-domain protein expressed in primary myoblasts but down-regulated in the RMS cell line RD. DRAL is a LIM-only protein with five LIM domains whereby one LIM domain consists only of the second half of the consensus motif. Interestingly, down-regulation of DRAL was not confined to the RD RMS cells, but was a phenomenon extended to other RMS cell lines of both embryonal and alveolar subtype, and to some breast cancer cell lines. Analysis of the expression pattern in normal human tissues revealed that DRAL is expressed at high levels in the heart, suggesting an important function in the specification of the terminally differentiated phenotype of heart muscle cells. Immunofluorescence studies using an antibody directed against recombinant DRAL localized the protein predominantly in the nucleus of cultured cells. On the basis of these results, we conclude that down-regulation of DRAL correlates with the tumor phenotype of RMS cells.

Amino Acid Sequence↗
PubMed1976

Rhabdomyosarcoma in children. A clinicopathologic study of 35 patients.

Thirty-five patients with rhabdomyosarcoma were treated at the Children's Hospital Medical Center from 1953 to 1973. Factors important in diagnosis and prognosis included age, sex, site, stage of tumor, and specific pathologic type. Effects that surgery, radiation, and chemotherapy had on survivals were also analyzed. Survivals ranged from 2 to 20 years. The rates were 86% for Stage I disease; 40% for Stage II disease, and 0 for the more advanced lesions. A useful prognostic indicator was found in pathologic subgroupings. Survivals for the myxoid spindle-cell variant were 80% for all stages. For the partial maturation series with moderately well-differentiated rhabdomyoblasts, survival was 10% for all stages. For a combination of the above two types, survival was 40% for all stages. The alveolar types had no survivors, although these patients seemed to live longer with their disease.

Adolescent↗
PubMed1999

Perinatal management of a neonate with airway obstruction caused by rhabdomyosarcoma of the tongue.

Intra-oral masses in neonates can seriously compromise the airway, potentially causing hypoxia and death if not recognized and managed appropriately. We report a case in which an intra-oral mass was diagnosed on antenatal ultrasound scan. Preparation for delivery involved a multidisciplinary team approach, with a strategy for management at delivery. The child was delivered by elective Caesarean section and had a patent airway. A tracheostomy was performed immediately after delivery. The infant underwent a debulking procedure 3 weeks after birth. A histological diagnosis of embryonal rhabdomyosarcoma was made and a course of chemotherapy commenced. The child had a partial response to treatment with considerable shrinkage of the tongue mass. We discuss the management options in neonates with intra-oral masses to provide an adequate airway and maintain fetal oxygenation. The differential diagnosis of fetal oral masses is reviewed.

Airway Obstruction↗
PubMed1996

In vivo amplification of the PAX3-FKHR and PAX7-FKHR fusion genes in alveolar rhabdomyosarcoma.

In the pediatric cancer alveolar rhabdomyosarcoma, characteristic t(2;13)(q35;q14) or variant t(1;13)(p36;q14) chromosomal translocations generate PAX3-FKHR or PAX7-FKHR fusion genes. Using fluorescence in situ hybridization, reverse transcriptase-polymerase chain reaction and quantitative Southern blot analyses, we demonstrate that these fusion genes are amplified in 20% of fusion-positive tumors. In particular, we found in vivo amplification of these fusions in one of 22 PAX3-FKHR-positive cases and five of seven PAX7-FKHR-positive cases. These findings indicate that translocation and amplification can occur sequentially in a cancer to alter both the structure and copy number of a gene and thereby activate oncogenic activity by complementary mechanisms.

Base Sequence↗
PubMed2006

Uniparental disomy at chromosome 11p15.5 followed by HRAS mutations in embryonal rhabdomyosarcoma: lessons from Costello syndrome.

Costello syndrome (CS; MIM 218040) is characterized by short stature, facial dysmorphism, cardiac defects and predisposition to embryonal rhabdomyosarcoma (CS/ERMS) and other neoplasias. CS is caused by germline mutations in the HRAS gene on chromosome 11p15.5, a region showing allelic imbalances in sporadic ERMS and CS/ERMS. The critical gene for ERMS development in this region is unknown. The association of CS and ERMS as well as previous reports illustrating that somatic HRAS mutations are found in a proportion of these tumors prompted us to clarify the significance and a possible correlation of HRAS mutations and genomic rearrangements at 11p15.5 in sporadic ERMS. We screened for somatic HRAS mutations and 11p15.5 imbalances in six sporadic ERMS samples. This analysis uncovered five ERMS samples with uniparental disomy (UPD) at the HRAS locus, two of which harbored HRAS mutations. By analyzing informative genetic variations in or at the HRAS gene locus, we show that one HRAS allele is entirely lost in specimens with UPD at 11p15.5. Notably, in both cases with UPD and HRAS mutations these mutations were heterozygous. Therefore, they must have succeeded the emergence of UPD. In contrast, HRAS germline mutations are the first step in CS/ERMS. Subsequent development of UPD at 11p15.5 may explain previous observations that CS/ERMS express mutant HRAS only. These data implicate that in sporadic ERMS, UPD at 11p15.5 is not driven by HRAS mutations and that imbalances at 11p15.5 and HRAS mutations represent independent but cooperating events during ERMS development.

Chromosomes, Human, Pair 11↗
PubMed1998

Rhabdomyosarcoma of the diaphragm: report of an adult case.

Diaphragmatic tumors, whether benign or malignant, may not generally reveal any symptoms in the early phase and may be found accidentally. During a pre-employment physical examination, a 20-year-old woman was found to have an abnormal shadow on the left diaphragm. An X-ray film, computed tomography and ultrasonography showed a giant mass on the left side, to the rear of the heart. She underwent surgery via a left thoraco-abdominal approach. The lesion was found to arise from the left diaphragm, and multiple disseminated lesions were scattered in the left thoracic cavity. Histological examination showed many large, oxyphilic rhabdoid cells between diffusely proliferating, spindle-shaped cells, and the tumor was subsequently diagnosed as a pleomorphic rhabdomyosarcoma of the diaphragm, of which the location and histological type were very rare. Despite adjuvant therapy, chest X-ray and CT revealed increasing tumor growth in the left cavity and she died one year after surgery.

Adult↗
PubMed1975

Studies on a transplantable murine rhabdomyosarcoma.

Well-differentiated rhabdomyosarcomas developed in 8 of 10 BALB/c mice inoculated with cell-free extracts of two lesions arising in bats previously inoculated with the Moloney strain of murine sarcoma virus. The tumors could be transplanted in BALB/c mice and other strains of mice compatible at the H-2 locus. A tumorigenic clonal cell culture line (R2) was established from a transplanted tumor. Ultrastructural studies of the primary and transplanted tumors and R2 cells revealed thick and thin myofilaments characteristic of myoblasts. These cells contained numerous cisternal type-A virus-like particles, but no type-C particles were found. Attempts to recover, rescue, or chemically induce transforming or nontransforming viruses from transplantable tumors and the R2 cell line were unsuccessful. The tumor cells lacked the murine type-C virus gs antigen. Hybridization data confirmed the apparent lack of viral RNA in the tumor cells.

Animals↗
PubMed1978

Transformation of human embryo cells with the use of cell-free extracts of a human rhabdomyosarcoma cell line (HUS-2): brief communication.

Cell-free extracts of the human rhabdomyosarcoma cell line HUS-2 caused the transformation of human embryo fibroblasts. This transformation included morphologic alteration, karyotypic change, and an increase in culture longevity. With the use of sex markers, multiple karyotypes confirmed that the human embryo fibroblasts were transformed, and the use of cell-free material further suggested the presence of a transforming virus. RNA-dependent DNA polymerase activity in a particle with a specific gravity of 1.16 g/cm3 indicated the presence of an RNA type C virus. Evidence also suggested that the known mammalian type C viruses, routine cytopathic effect-inducing viruses, or mycoplasma were not the agents responsible for the transformation. That both the donor (HUS-2) and converted (HUE-T) cell lines cross-reacted with antisera prepared against HUE-T indicated a common antigen arising in the process of conversion of HUS-2 cells to HUE-T cells.

Animals↗
PubMed1989

Promotion of micrometastasis proliferation in a rat rhabdomyosarcoma model by epidermal growth factor.

Using a rat rhabdomyosarcoma 9-4/0, we investigated the role of epidermal growth factor (EGF) in tumor dissemination. In vitro, we detected high-affinity EGF receptors on tumor cells and stimulation of their proliferation by EGF. When injected iv, EGF-pretreated cells demonstrated an increased capacity to form lung colonies and to invade lymphatic tissue. In vivo, EGF treatment led to increased metastatic spread of subcutaneous tumors. When primary tumors were ablated, and the treatment was given from the time of graft until ablation (seeding step), no effect on metastatic spread was noticed. When treatment was given from the time of ablation until death (growth step), EGF increased the number of lung metastases and of invaded lymph node sites.

Animals↗
PubMed1993

Paratesticular rhabdomyosarcoma in adult patients: 16-year experience at Institut Gustave-Roussy.

BACKGROUND: Children with paratesticular rhabdomyosarcoma (RMS) have both a good prognosis and a high survival rate. The clinical behaviour and outcome of the disease in adults is not well described. PATIENTS AND METHODS: We reviewed retrospectively our experience with paratesticular RMS in patients older than 16 years during a 16-year period (1975-1991). RESULTS: Thirteen adult patients with paratesticular RMS are reported. Median age was 21 years (range 16 to 31). Presentation characteristics were scrotal mass in 11 cases, lumbar pain and weight loss in 5 cases, hypercalcemia in 3 cases and thrombocytopenia in 3 cases. There were 5 patients with stage IV, 2 with stage IIB and 6 with stage IA (IRS classification). The 5 stage IV patients are reported in detail, with initial bone marrow infiltration encountered in 4 of them. Objective response to chemotherapy was achieved in all 6 patients with measurable disease (2 CR + 4 PR). Two of 7 patients who received adjuvant chemotherapy relapsed at 7 and 11 months. After a median follow-up of 90 months, 8 patients (5 stage IV, 3 stage IA) died from disease progression. CONCLUSION: Metastatic disease with bone marrow involvement at presentation and aggressive behaviour seem to be more relevant in adult paratesticular RMS patients compared with children.

Adolescent↗
PubMed2005

Full transcriptome analysis of rhabdomyosarcoma, normal, and fetal skeletal muscle: statistical comparison of multiple SAGE libraries.

Rhabdomyosarcoma (RMS) is the most frequent soft tissue sarcoma in children. Improved treatment strategies have increased overall survival, but the response of approximately one-third of the patients is still poor. To increase the knowledge of RMS pathogenesis, we performed the first full transcriptome analysis of RMS using serial analysis of gene expression (SAGE). With a G-test for the simultaneous comparison of subsets of SAGE libraries of normal skeletal muscle, embryonal (ERMS) and alveolar (ARMS) RMS, we identified 251 differentially expressed genes. A literature-mining procedure demonstrated that 158 of these genes have not previously been associated with RMS or normal muscle. Gene Ontology (GO) analysis assigned 198 of the 251 genes to muscle-specific classes, including those involved in normal myogenic development, as well as tumor-related classes. Prominent GO classes were those associated with proliferation and actin reorganization, which are processes that play roles during early muscle development, muscle function, and tumor progression. Using custom microarrays, we confirmed the (up- or down-) regulation of 80% of 98 differentially expressed genes. Another SAGE library of 19- to 22-week-old fetal skeletal muscle was compared with the RMS and normal muscle transcriptomes. Cluster analysis showed that the RMS and fetal muscle SAGE libraries formed one cluster distinct from normal muscle samples. Moreover, the expression profile of 86% of the differentially expressed genes between normal muscle and RMS was highly similar in fetal muscle and RMS. In conclusion, the G-test is a robust tool for analyzing groups of SAGE libraries and correctly identifies genes marking the difference between fully differentiated skeletal muscle and RMS. This study not only substantiates the close association between embryonic myogenesis and RMS development but also provides a rich source of candidate genes to further elucidate the etiology of RMS or to identify diagnostic and/or prognostic markers.

Cell Adhesion↗
PubMed1987

Use of anti-skeletal muscle antibody from myasthenic patients in the diagnosis of childhood rhabdomyosarcomas.

Rhabdomyosarcoma (RMS), a common soft tissue tumor in children, may often be difficult to distinguish from Ewing's sarcoma, neuroblastoma, and malignant lymphomas. Confirmation of the skeletal muscle origin of RMS depends partly on the demonstration of striations in tumor cells that are usually undetectable in poorly differentiated tumors. A number of tissue markers (e.g., myoglobin and desmin) are currently being used to establish the origin of RMS. However, most of these markers lack specificity and have relatively low sensitivity. We have investigated the specificity and sensitivity of anti-skeletal muscle antibody (ASMA) from patients with myasthenia gravis in the diagnosis of childhood RMS. Out of eight cases of childhood RMS (four embryonal and four alveolar) examined, two showed striations with hematoxylin and eosin and four with phosphotungstic acid hematoxylin. Myoglobin was detected in five tumors; only well-differentiated tumor cells contained myoglobin. Anti-desmin antibody and ASMA reacted with cells in all the eight tumors whether or not the tumor cells were well differentiated. Anti-skeletal muscle antibody did not react with nine lymphomas, four Ewing's sarcomas, four neuroblastomas, four osteogenic sarcomas, four lipomas, eight duct carcinomas of the breast, and eight squamous cell carcinomas of the lung. Eight leiomyomas and four leiomyosarcomas of the uterus were compared for their reactivity with anti-desmin antibody and ASMA. All the tumors stained with anti-desmin antibody and none with ASMA. The results show that ASMA is useful in the diagnosis of childhood RMS and is a more sensitive reagent than anti-myoglobin antibody. Unlike anti-desmin antibody, it can distinguish skeletal muscle tumors from smooth muscle tumors.

Antigens, Neoplasm↗
PubMed1989

Primary rhabdomyosarcoma of the adult liver.

A 53-year-old male Caucasian presented with a massive liver tumor composed entirely of spindle-shaped cells showing light-microscopic and ultrastructural evidence of rhabdomyoblastic differentiation. No epithelial or other sarcomatous elements were included. Detailed postmortem examination failed to reveal evidence of metastatic tumor or an alternative primary site. There have been only four previous cases of primary rhabdomyosarcoma of the adult liver, all occurring in Japanese men.

Carcinoid Tumor↗
PubMed1998

Spindle cell rhabdomyosarcoma (so-called) in adults: report of two cases with emphasis on differential diagnosis.

Spindle cell rhabdomyosarcoma (RMS) is a recently described variant of embryonal RMS that carries a relatively favorable prognosis when compared with other forms of RMS. To date, spindle cell RMS has been described only in children. The authors have identified two unusual cases occurring in adults using the following criteria: tumors composed mainly of fascicular, relatively monomorphic spindle-shaped cells that show unequivocal immunohistochemical and ultrastructural evidence of myogenic differentiation. The tumors were identified in a 38-year-old woman and a 56-year-old man, arising in the cheek and left hemidiaphragm, respectively. Both were treated with surgical resection and chemotherapy. The first patient died of uncontrolled local recurrence of her tumor at 27 months after diagnosis, and the second died of metastatic disease at 13 months follow-up. The tumors were composed mainly of fascicles of spindle cells with palely eosinophilic cytoplasm admixed diffusely with sparse polygonal, rounded, or strap-shaped rhabdomyoblasts with brightly eosinophilic cytoplasm and with cross-striations in the first case only. Immunostaining for muscle-related antigens showed staining for smooth-muscle actin (focal), pan-actin HHF-35, desmin, fast myosin, myoglobin, and MyoD1. Both cases were negative for S-100 protein. On electron microscopy, both cases showed neoplastic rhabdomyoblasts with clear-cut sarcomeric differentiation in many of the tumor cells. Spindle cell RMS poses special problems in differential diagnosis when arising in adults and should be distinguished from leiomyosarcoma, malignant peripheral nerve sheath tumor with heterologous rhabdomyoblastic differentiation (malignant Triton tumor), and fibrosarcoma. In view of the good prognosis afforded children with spindle cell RMS and in light of the chemoresponsive behavior of RMS in general, we feel that it is important to identify tumors that meet the criteria for spindle cell RMS occurring in the adult population. However, based on these two cases, it is possible that spindle cell RMS occurring in adults may not be associated with such a favorable outcome.

Actins↗
PubMed2001

Rhabdomyosarcoma of the urinary bladder and vagina: a clinicopathologic study with emphasis on recurrent disease: a report from the Kiel Pediatric Tumor Registry and the German CWS Study.

Rhabdomyosarcomas (RMS) of the urinary bladder and vagina vary in their biologic and clinical behavior and require different types of treatment. Anatomically the two organs are close, and the reason for these differences in behavior is unknown. We investigated tumor specimens of 51 urinary bladder RMS and 14 vaginal RMS with regard to histologic subtype, growth pattern, differentiation, and proliferation morphologically and immunohistochemically. Recurrences and/or "second look" specimens from 15 patients after chemotherapy were compared with the primary tumors. Within the 65 specimens we found 31 "classical" embryonal RMS, 26 embryonal RMS of botryoid subtype (BRMS), 3 embryonal RMS of spindle cell subtype, and 5 alveolar RMS. BRMS is more common in the vagina (11 BRMS of 14 cases) than in the urinary bladder RMS (15 BRMS of 54 cases). Classical embryonal RMS with a polypoid (exophytic) growth pattern is associated with a more favorable prognosis (92% 10-year survival) than the same type with a diffuse intramural (endophytic) growth pattern (68% 10-year survival, p = 0.02). The proliferation rate was associated with the degree of differentiation, but neither showed a correlation with prognosis. A marked maturation after chemotherapy was seen in the majority of recurrences and SL specimens, associated with lowered proliferation activity. Two of 12 patients with recurrences showing chemotherapy-induced maturation died of the disease. In conclusion, we determined that polypoid embryonal RMS of both the urinary bladder and the vagina have a comparably good prognosis. This includes all botryoid RMS. The poorer prognosis of the group of urinary bladder RMS as a whole was caused by a high incidence of diffusely growing RMS, which have a less favorable prognosis than polypoid RMS. Maturation after chemotherapy occurs frequently in RMS. In contrast to the excellent prognosis reported in other studies, we had two patients with fatal outcome despite chemotherapy-induced maturation in the recurrences.

Adolescent↗