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 667 records · Page 37Linked to original sources
PubMed1999

Does debulking improve survival rate in advanced-stage retroperitoneal embryonal rhabdomyosarcoma?

UNLABELLED: BACKGROUND, METHODS, AND PURPOSE: The authors examined demographic and clinical features, therapy, and outcome of patients with advanced (group III or IV) rhabdomyosarcoma (RMS) of the retroperitoneum and nongenitourinary pelvis treated in the Intergroup Rhabdomyosarcoma Study Group (IRSG) III (1984 to 1991, n = 41) or IV pilot (1987 to 1991, n = 53) studies to assess the role of initial debulking surgery. RESULTS: Ninety-four patients with retroperitoneal primary tumors and gross locoregional residual tumor (group III, n = 53) or metastatic disease (group IV tumors, n = 41) were treated with combination chemotherapy (ie, vincristine, dactinomycin, and cyclophosphamide with or without other agents plus radiation therapy, RT) after biopsy only or subtotal resection. These retroperitoneal tumors usually were invasive (T2, 76%). Most patients were younger than 10 years of age (n = 69, 73%), the male to female ratio was 1.4, and tumors usually were embryonal (n = 64, 68%). Overall 4-year failure-free survival (FFS) was 50%; survival was 60%. Survival rate was better for girls (4-year survival rate, 75% v49% for boys; P = .05) and was not significantly different for patients treated in IRS-III (66%) or IRS-IV pilot (52%). However, it was better for patients with embryonal versus alveolar or undifferentiated tumors (4-year survival rate, 70% v 42%; P = .002). In adolescents, RMS is different from that seen in children less than 10 years old; most cases are alveolar or undifferentiated (16 of 29, 55%). Surgery for most (21 of 24) patients with alveolar tumors comprised biopsy only. By contrast, of 64 patients with embryonal tumors, 39 (61%) underwent biopsy only, whereas 25 (39%) had debulking surgery. Patients whose tumors were debulked fared better than those whose tumors underwent biopsy only (4-year FFS rate, 72% v48%; P = 0.03). Patients with group IV embryonal tumors fared unexpectedly better than those with group IV alveolar or undifferentiated tumors (70% versus 42% 4-year survival rate, P < .05), and patients less than 10 years of age with group IV embryonal tumors had 4-year survival rate of 77%, indicating the importance of the biology of these tumors. CONCLUSIONS: Multimodal therapy, including multiagent chemotherapy plus RT, appears to improve survival rate in patients with advanced embryonal RMS arising in the retroperitoneum. These data suggest that debulking tumors of embryonal histology improves outcome further. This approach will be assessed in IRSG V.

Adolescent↗
PubMed1996

Selection of probes for fluorescence in situ hybridization analysis by differential display polymerase chain reaction of mRNA from rhabdomyosarcoma.

Rhabdomyosarcoma (RMS) is a malignancy of skeletal muscle derivation encompassing two major subtypes, embryonal and alveolar, which differ in clinical behavior and genetic markers. Because RMS is a relatively circumscribed tumor system for which the beginnings of a molecular genetic framework are in place, it becomes an ideal model for the application of improved methods of molecular genetic analysis. We have applied the technique known as differential display polymerase chain reaction (DD-PCR) to characterize expression of RNA in rhabdomyosarcoma subtypes. Our studies have shown that DD-PCR generates a characteristic electrophoretic profile that can be used to isolate subtype specific probes for fluorescence in situ hybridization (FISH) analysis. We have isolated two cDNA fragments and obtained clones suitable for FISH mapping to metaphase chromosomes. One probe was mapped to the centromeric region of human chromosome 22 and the other probe to the human chromosome band 6q25-26. This approach demonstrates the utility of DD-PCR as a technique for isolating novel cDNA expressed in tumors and their subsequent use as probes for FISH analysis. As more genes are identified by DD-PCR and their roles in tumorigenesis become defined, they are likely to provide novel targets for future molecular cytogenetic analysis.

Base Sequence↗
PubMed2001

Results from the IRS-IV randomized trial of hyperfractionated radiotherapy in children with rhabdomyosarcoma--a report from the IRSG.

PURPOSE: To evaluate the outcome and toxicity of hyperfractionated radiotherapy (HFRT) vs. conventionally fractionated radiotherapy (CFRT) in children with Group III rhabdomyosarcoma (RMS). METHODS AND MATERIALS: Five hundred fifty-nine children were enrolled into the Intergroup Rhabdomyosarcoma Study IV with Group III RMS. Sixty-nine were ineligible for the analysis because of incorrect group or pathologic findings. Of the 490 remaining, 239 were randomized to HFRT (59.4 Gy in 54 1.1-Gy twice daily fractions) and 251 to CFRT (50.4 Gy in 28 1.8-Gy daily fractions). The age range was <1-21 years. All patients received chemotherapy. RT began at Week 9 after induction chemotherapy for all but those with high-risk parameningeal tumors who received RT during induction chemotherapy. The patient groups were equally balanced. The median follow-up was 3.9 years. RESULTS: Analysis by randomized treatment assignment (intent to treat) revealed an estimated 5-year failure-free survival (FFS) rate of 70% and overall survival (OS) of 75%. In the univariate analysis, the factors associated with the best outcome were age 1-9 years at diagnosis; noninvasive tumors; tumor size <5 cm; uninvolved lymph nodes; Stage 1 or 2 disease; primary site in the orbit or head and neck; and embryonal histologic features (p = 0.001 for all factors). No differences in the FFS or OS between the two RT treatment methods and no differences in the FFS or OS between HFRT and CFRT were found when analyzed by age, gender, tumor size, tumor invasiveness, nodal status, histologic features, stage, or primary site. Treatment compliance differed by age. Of the children <5 years, 57% assigned to HFRT received HFRT and 77% assigned to CFRT received CFRT. Of the children >or=5 years, 88% assigned to both HFRT and CFRT received their assigned treatment. The reasons for not receiving the appropriate randomized treatment were progressive disease, early death, parent or physician refusal, young age, or surgery. The toxicity assessment revealed more mucositis with HFRT (66%) than with CFRT (46%) (p = 0.03) for the parameningeal patients, and more skin reactions (16%) and nausea/vomiting (13%) with HFRT than with CFRT (7% and 5%, respectively) for patients with nonparameningeal primary tumors (p = 0.03 and p = 0.02, respectively). The analysis by treatment actually received revealed a 5-year FFS rate of 73% and OS rate of 77%, with no difference between CFRT and HFRT. As well, there was no difference in FFS or OS between CFRT and HFRT when analyzed by age, gender, tumor size, tumor invasiveness, modal status, histology, stage or site of primary. The 5-year estimated cumulative incidence of failure for the irradiated patients was local, 13%; regional, 3%; and distant, 13%; with no differences between HFRT and CFRT. The 5-year local failure rate by site was orbit, 5%; head and neck, 12%; parameningeal, 16%; bladder/prostate, 19%; extremity, 7%; and all others, 14%. The 5-year regional failure rate was parameningeal,1%; extremity, 20%; and all others, 5%. The 5-year distant failure rate was orbit, 2%; head and neck, 6%; parameningeal, 11%; bladder/prostate, 15%; extremity, 28%; and all others, 17%. CONCLUSIONS: HFRT, as given in this study, did not improve local/regional control, FFS, or OS compared with CFRT. The risk of local/regional failure was comparable to that of distant failure in children with Group III RMS. The standard of care for Group III RMS continues to be CFRT with chemotherapy.

Adolescent↗
PubMed2003

The truth about alveolar rhabdomyosarcoma.

The alveolar variant of rhabdomyosarcoma continues to be underdiagnosed by pathologists. Yet, the correct recognition of this subtype can be of significant import to the oncologist for specifics of patient treatment. This review discusses the important aspects of alveolar rhabdomyosarcoma and hopefully can be of some help in the proper diagnosis of this tumor.

Diagnosis, Differential↗
PubMed2001

Treatment of orbital rhabdomyosarcoma: survival and late effects of treatment--results of an international workshop.

PURPOSE: Orbital rhabdomyosarcoma (RMS) historically has been associated with an excellent survival rate. The majority of patients are cured with the use of both chemotherapy and radiation therapy, but a significant number experience important late sequelae of treatment. In an attempt to determine optimal therapy in relation both to cure and to sequelae, the experience of the four international collaborative groups (Intergroup Rhabdomyosarcoma Study Group [IRSG], International Society of Paediatric Oncology [SIOP] Sarcoma Committee, German Collaborative Soft Tissue Sarcoma Group [CWS], and Italian Cooperative Soft Tissue Sarcoma Group [ICG] studies) was shared at an international workshop. PATIENTS AND METHODS: A total of 306 eligible patients were identified from group records (186 from IRS, 43 from SIOP MMT, 40 from CWS, and 37 from ICG). Median age was 6.8 years, and median follow-up was 6.5 years. Eighty percent of patients received radiation therapy (RT) as part of primary therapy, but there were significant differences in the use of RT between the individual groups (93% in IRSG, 76% in ICG, and 70% in CWS, but only 37% in the SIOP MMT group). RESULTS: At 10 years, event-free and overall survival for the whole cohort were 77% (range, 71% to 81%) and 87% (range, 82% to 92%), respectively. There was no difference in overall survival between the collaborative groups regardless of differences in the use of initial RT. In total, 34 (12%) of 273 survivors had not received RT, although this varied between the different groups (41% in the SIOP MMT group, 20% in CWS, 7% in ICG, and 6% in IRSG). There was no difference in overall survival for the whole cohort regardless of whether radiotherapy was used as part of initial therapy (86% at 10 years for both). CONCLUSION: These data suggest that a subset of patients with orbital RMS can be cured without systematic local therapy, although the total burden of treatment (primary therapy and treatment for relapse) must be taken into account when assessing the implications for late sequelae.

Adolescent↗
PubMed2000

Rhabdomyosarcoma in children: report of two cases.

Rhabdomyosarcoma of soft tissue is the commonest sarcoma in pediatric medicine and comprises 15% of the solid tumors treated in pediatric oncology. It has an ample variety of clinical presentations in diverse organs and also presents a gamut of histopathological patterns, which sometimes make diagnosis difficult. We report two cases of rhabdomyosarcoma: one of the embryonic histological variety and the second one of the alveolar variety, which presented themselves in the preauricular and masseteric areas respectively. Both patients were male, diagnosed and treated.

Child↗
PubMed1993

Orbital rhabdomyosarcoma: a twenty-year survey in Turkey.

Orbital rhabdomyosarcoma is the most frequently encountered primary malignant orbital tumor in children. Between 1970 and 1991, 68 primary orbital rhabdomyosarcoma cases were diagnosed and treated in our clinic. Mean age at the time of diagnosis was 8.8 years. Seventy-two percent of the cases were of the embryonal type while 14% had alveolar and 14% had botryoid tumors. Following tissue diagnosis by anterior orbitotomy, radiotherapy and multimodal chemotherapy were instituted promptly. Subtotal exenteration was used in massive orbital involvement and total exenteration in recurrences. Using the Kaplan-Meier method of analysis, the survival rate was 84% at three years and 80% at five years. While morbidity due to the disease itself and treatment complications may still be a problem, the survival rates achieved in the long term are promising.

Adolescent↗
PubMed1997

Amplification of the t(2; 13) and t(1; 13) translocations of alveolar rhabdomyosarcoma in small formalin-fixed biopsies using a modified reverse transcriptase polymerase chain reaction.

Detection of characteristic chromosomal translocations has aided diagnosis of the small round cell tumors of childhood and may help to stratify patients into clinical groups. The detection of the abnormalities by classical cytogenetic techniques has been supplemented by fluorescent in situ hybridization and reverse transcriptase polymerase chain reaction (RT-PCR). These techniques allow diagnoses to be made using only very small amounts of tumor tissue. We here describe a technique for the rapid and specific detection by modified reverse transcriptase polymerase chain reaction of characteristic chromosomal translocations of alveolar rhabdomyosarcoma with small amounts of formalin-fixed tissue as the starting material. Of 27 samples studied, 4 cases are described in which the detection of translocations by this method cast doubt on the original histopathological diagnosis. These cases demonstrate the critical diagnostic importance of the detection of these translocations in rhabdomyosarcoma.

Adolescent↗
PubMed1991

Primary rhabdomyosarcoma of the iris.

We describe herein a 4-year-old girl with primary rhabdomyosarcoma of the right iris. Malignant cells were aspirated from the aqueous humor, and the eye was enucleated 7 months after the tumor appeared. To our knowledge, only two cases of primary rhabdomyosarcoma of the iris have been reported. Both of these patients received radiation therapy prior to enucleation.

Anterior Chamber↗
PubMed1993

Alveolar rhabdomyosarcoma that metastasized to the orbit.

A 22-year-old woman with a history of hyperthyroidism and a paravaginal alveolar rhabdomyosarcoma developed diplopia 3 months after successful autologous bone marrow transplantation. Results of computed tomographic scan showed apparent enlargement of the right inferior rectus muscle, and thyroid eye disease was suspected. However, results of further evaluation revealed an alveolar rhabdomyosarcoma that metastasized to the orbit.

Adult↗
PubMed1990

Rhabdomyosarcoma of the head and neck in children.

Rhabdomyosarcoma is the most common soft-tissue sarcoma in infants and children, with the head and neck being the most frequent site of involvement. Treatment for this neoplasm has undergone many changes, with a much improved prognosis using a combination of surgery, radiation therapy, and chemotherapy. This retrospective analysis presents the management and outcome of 60 children (aged 3 months to 18 years) with rhabdomyosarcoma of the head and neck evaluated at the Children's Hospital of Philadelphia (Pa) between 1970 and 1987. The overall death rate for all head and neck sites decreased from 50% in 1970 to 1979 to 23% in 1980 to 1987, reflecting the improved management protocol.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1989

Rhabdomyosarcoma: contemporary status and future directions. The Lucy Wortham James Clinical Research Award.

Approximately two thirds of children with rhabdomyosarcoma will be cured of their tumor and become long-term survivors. Multidisciplinary care has brought about improvements in survival. Yet our therapies of today are suboptimal in many situations and demand intense effort toward improving cure rates and quality of life. Rhabdomyosarcoma is a complex childhood malignancy with differing anatomic sites of presentation and varying histologic subtypes, each presenting with unique patterns of growth, making it inappropriate to consider the disease a single entity. Avenues of future research involve cytogenetic and molecular genetic investigations that will aid in diagnosis and screening and, it is hoped, guide clinicians to more specific therapy.

Adolescent↗
PubMed1999

Child with mosaic variegated aneuploidy and embryonal rhabdomyosarcoma.

We report on a 7-year-old boy with mosaic variegated aneuploidy (MVA) who developed embryonal rhabdomyosarcoma of the soft palate. This patient is the 11th case report of MVA and represents further documentation of the true existence of this rare mitotic mutant. Clinical findings share similarities to those previously described patients including microcephaly and growth retardation as the two most common abnormalities. Notably, mental retardation is not universally present. Results of serial cytogenetic analyses performed on somatic and neoplastic tissues are reviewed and compared with those of other previously reported patients. We postulate that mosaic variegated aneuploidy is causally related to the development of rhabdomyosarcoma in our patient. This is the first report of a patient with MVA who developed cancer and suggests that these patients may be at risk for malignancy and require long-term follow-up and cancer surveillance.

Adolescent↗
PubMed1996

Primary pulmonary rhabdomyosarcoma in childhood: clinico-biologic features in two cases with review of the literature.

The cases of two children under three years of age with primary pulmonary rhabdomyosarcoma and no associated lung malformations are reported and a review of the literature is presented. In both, complete surgical removal of the tumor was performed and histologic examination revealed embryonal subtype. Flow cytometric assessment showed a tumor-cell diploid DNA content. Postoperative radio- and chemotherapy were carried out, but in spite of treatment both girls died because of disease progression, fourteen and nine months after diagnosis. The importance of associated cystic lung malformations and DNA content in predicting clinical outcome of primary pulmonary rhabdomyosarcoma is evaluated.

Child, Preschool↗
PubMed1997

Long-lasting complete remission after prolonged administration of etoposide in a child with a second recurrence of alveolar rhabdomyosarcoma.

The authors report a case of alveolar rhabdomyosarcoma (RMS) of the thigh complicated by two successive distant relapses shortly after radio- and chemotherapy, treated with etoposide, and resulting in complete long-lasting remission. The schedule of etoposide was 100 mg/ m2/d intravenously for three days weekly for 3 weeks, with an interval of 1 week between courses. This was administered for 11 months. The child is alive without disease and off therapy 20 months after completion of etoposide treatment. Preclinical studies and experience in adults have suggested that the cytotoxic effects of etoposide show a marked dependence on schedule. A divided dose regimen of single-agent etoposide has previously been shown to have activity in relapsed rhabdomyosarcoma, but the outcome or the duration of complete response has not yet been fully evaluated. In out poor-prognosis case, the prolonged administration of etoposide achieved a long-lasting complete response.

Antineoplastic Agents, Phytogenic↗
PubMed1997

Undifferentiated sarcomas of children: pathology and clinical behavior--an Intergroup Rhabdomyosarcoma study.

Undifferentiated soft tissue sarcoma (UND-STS) is the most poorly defined tumor eligible for intergroup Rhabdomyosarcoma Studies (IRS). Recent IRS UND-STS experience was reviewed to assess the histologic characteristics and clinical behavior of undifferentiated sarcomas. Of the 1,527 patients entered on IRS-III and IRS pilot-IV, 96 had tumors classified by the IRS Pathology Committee as UND-STS. Of these, 52 had adequate histologic material for this study. After application of immunohistochemistry, 18 tumors were reclassified, mostly as embryonal rhabdomyosarcomas (RMS), primitive neuroectodermal tumors, and intraabdominal desmoplastic small found cell tumors. The remaining 34 UND-STS had a diffuse hypercellular histologic pattern made up of sheets of medium-sized cells. The tumor cells had a minimal to moderate amount of cytoplasm and a variable nuclear morphology, predominately vesicular with finely granular chromatin. Except for reactivity with antibodies against vimentin, most tumors had a negative immunohistochemical profile. The 5 year Kaplan-Meier survival estimate for patients with non-metastatic disease was 72%, a significant improvement when contrasted with patients diagnosed to have UND-STS in IRS-I and IRS-II.

Adolescent↗
PubMed1996

Interphase fluorescence in situ hybridization detection of t(2;13)(q35;q14) in alveolar rhabdomyosarcoma--a diagnostic tool in minimally invasive biopsies.

The identification of t(2;13)(q35;q14) is a useful aid in the accurate diagnosis of rhabdomyosarcoma, distinguishing it from other small round cell tumours and supporting the distinction between alveolar and embryonal forms. Cytogenetic analysis is difficult and with the increased use of minimally invasive biopsy methods and primary chemotherapy, adequate tumour material is not always available. To overcome these difficulties, two-colour interphase fluorescence in situ hybridization (FISH) to detect t(2;13)(q35;q14) was developed and its utility in assessing minimally invasive biopsies was investigated. Two cosmid clones which mapped proximal or distal to the breakpoint region 13q14 and one yeast artificial chromosome clone that mapped distal to the 2q35 breakpoint were identified. In interphase cells containing t(2;13)(q35;q14), the configuration of cosmid and yeast artificial chromosome signals demonstrated the presence of the translocation. Five cases of rhabdomyosarcoma were analysed by interphase FISH. The t(2;13)(q35;q14) was detected in all four alveolar tumours and was confirmed by cytogenetics in two cases, but was absent in one embryonal tumour. This sensitive detection method is applicable to minimal amounts of fresh or frozen tumour.

Adolescent↗
PubMed1996

Minimal disease detection in patients with alveolar rhabdomyosarcoma using a reverse transcriptase-polymerase chain reaction method.

BACKGROUND: Polymerase chain reaction (PCR) assays that detect fusion genes resulting from consistent chromosomal translocations have been used to detect minimal residual disease, primarily in hematologic malignancies. Molecular assays have been developed recently that detect the PAX3-FKHR or PAX7-FKHR fusion transcript resulting from the t(2; 13) or t(1; 13) translocation consistently observed in alveolar rhabdomyosarcoma. Because of the tumor's propensity to disseminate widely, our aim was to determine whether or not reverse transcriptase-PCR assays could detect submicroscopic disease in bone marrow or peripheral blood specimens. METHODS: We analyzed 19 bone marrow samples from 11 patients with a known gene fusion in their primary tumor. Specimens were collected at diagnosis, remission, and relapse. Fourteen peripheral blood samples were obtained serially from 5 of these patients. A reverse transcriptase-PCR assay was used to detect the presence of the PAX3-FKHR or PAX7-FKHR fusion transcript. These results were compared with the results of microscopic examination of the bone marrow. Medical records of all 11 patients were reviewed. RESULTS: Adequate amplifiable RNA was obtained in 17 of 19 marrow samples. The PCR assay detected fusion products in all 4 specimens obtained from 3 patients with histologic evidence of bone marrow involvement. In addition, PAX3-FKHR fusion products were detected in bone marrows from 2 patients for whom there was no histologic evidence of disease. The fusion transcripts were not detected in any of the peripheral blood samples. CONCLUSIONS: The detection of submicroscopic disease is possible in alveolar rhabdomyosarcoma patients using PCR. These assays may have a role in staging, monitoring therapy, purging protocols for autologous bone marrow transplantation, and post therapy follow-up. Larger prospective studies are needed to address the clinical relevance of these results.

Bone Marrow↗