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 451 records · Page 25Linked to original sources
PubMed1994

Pericentric inversion (2)(p15q35) in an alveolar rhabdomyosarcoma.

We report a 7-year-old girl with a pericentric inversion of chromosome 2, inv(2)(p15q35), in a "solid variant" of alveolar rhabdomyosarcoma. The breakpoint in the long arm of chromosome 2 at band q35 is, at the cytogenetic level, identical to the breakpoint observed in the well-established reciprocal t(2;13)(q35;q14) associated with the alveolar subtype of rhabdomyosarcoma. In this case, however, no reciprocal translocation has occurred with chromosome 13 or any other chromosome, suggesting that the single critical breakpoint in alveolar rhabdomyosarcoma may be located at 2q35.

Child↗
PubMed1994

Embryonal rhabdomyosarcoma arising in the buccal mucosa: a case report with immunohistochemical and electronmicroscopic findings.

Rhabdomyosarcoma is one of the most common soft-tissue neoplasms in children. We report a case of a 9-year-old female with embryonal rhabdomyosarcoma arising in the buccal region with immunohistochemical and electronmicroscopic findings. Under a light microscope, it was observed that the tumor was composed of small round or spindle-shaped cells with pleomorphic and hyperchromatic nuclei. They were immunoreactive for actin, myoglobin and desmin. With an electron microscope, it was found that most tumor cells contained filamentous structures and free ribosomes. Some of them showed typical myofilaments, M-bands and Z-lines. These findings were specific evidence of rhabdomyosarcoma. Twenty courses of VAC (vincristine, D-actinomycin and cyclophosphamide) chemotherapy were administered, followed by surgical resection of the tumor.

Actins↗
PubMed1995

Three dimensional conformal radiation therapy in pediatric parameningeal rhabdomyosarcomas.

PURPOSE: We evaluated the utility of three dimensional (3D) treatment planning in the management of children with parameningeal head and neck rhabdomyosarcomas. METHODS AND MATERIALS: Five children with parameningeal rhabdomyosarcoma were referred for treatment at our radiation oncology center from May 1990 through January 1993. Each patient was evaluated, staged, and treated according to the Intergroup Rhabdomyosarcoma Study. Patients were immobilized and underwent a computed tomography scan with contrast in the treatment position. Tumor and normal tissues were identified with assistance from a diagnostic radiologist and defined in each slice. The patients were then planned and treated with the assistance of a 3D treatment planning system. A second plan was then devised by another physician without the benefit of the 3D volumetric display. The target volumes designed with the 3D system and the two-dimensional (2D) method were then compared. The dosimetric coverage to tumor, tumor plus margin, and normal tissues was also compared with the two methods of treatment planning. RESULTS: The apparent size of the gross tumor volume was underestimated with the conventional 2D planning method relative to the 3D method. When margin was added around the gross tumor to account for microscopic extension of disease in the 2D method, the expected area of coverage improved relative to the 3D method. In each circumstance, the minimum dose that covered the gross tumor was substantially less with the 2D method than with the 3D method. The inadequate dosimetric coverage was especially pronounced when the necessary margin to account for subclinical disease was added. In each case, the 2D plans would have delivered substantial dose to adjacent normal tissues and organs, resulting in a higher incidence of significant complications. CONCLUSIONS: 3D conformal radiation therapy has a demonstrated advantage in the treatment of sarcomas of the head and neck. The improved dosimetric coverage of the tumor and its margin for subclinical extensions may result in improvement in local control of these tumors. In addition, lowering of radiation dose to adjacent critical structures may help lower the incidence of adverse late effects in children.

Child↗
PubMed1995

Genomic organization of the human PAX3 gene: DNA sequence analysis of the region disrupted in alveolar rhabdomyosarcoma.

Mutations in the human PAX3 gene have previously been associated with two distinct diseases, Waardenburg syndrome and alveolar rhabdomyosarcoma. In this report we establish that the normal human PAX3 gene is encoded by 8 exons. Intron-exon boundary sequences were obtained for PAX3 exons 5, 6, 7, and 8 and together with previous work provide the complete genomic sequence organization for PAX3. Difficulties in obtaining overlapping genomic clone coverage of PAX3 were circumvented in part by RARE cleavage mapping, which showed that the entire PAX3 gene spans 100 kb of chromosome 2. Sequence analysis of the last intron of PAX3, which contains the previously mapped t(2;13)(q35;q14) translocation breakpoints of alveolar rhabdomyosarcoma, revealed the presence of a pair of inverted Alu repeats and a pair of inverted (GT)n-rich microsatellite repeats within a 5-kb region. This work establishes the complete structure of PAX3 and will permit high-resolution analyses of this locus for mutations associated with Waardenburg syndrome, alveolar rhabdomyosarcoma, and other phenotypes for which PAX3 may be a candidate locus.

Alleles↗
PubMedDate not supplied

Computed tomography in botryoid rhabdomyosarcoma of urinary bladder.

Genituorinary tract is a common site of origin of rhabdomyosarcomas. Not very many cases of urinary bladder rhabdomyosarcomas have been reported in the computed tomography literature. We present computed tomography appearances which were virtually classical to diagnose botryoid variety of embryonal rhabdomyosarcoma in a two year old male child.

Child, Preschool↗
PubMed2006

[Rhabdomyosarcoma of the genitourinary sinus in children].

Genitourinary rhabdomyosarcoma (RMS) accounts for approximately 25% of all rhabdomyosarcomas. Management of RMS at this site has changed during the last 5 consecutive Intergroup Rhabdomyosarcoma (IRS) trials, with increasing emphasis of bladder and vaginal conservation. As more effective treatment regimens has improved survival, surgical approaches have evolved to less aggressive management of the primary tumour to improve conservation. Various combinations of chemotherapy, irradiation and surgery have resulted in a decreased late sequelae in the group of patients with sarcoma arising in the genitourinary tract.

Child↗
PubMed2004

Fibronectin-induced COX-2 mediates MMP-2 expression and invasiveness of rhabdomyosarcoma.

Although accumulating evidence suggests the importance of cyclooxygenase-2 (COX-2) and prostaglandin E(2) (PGE(2)) in the pathogenesis of many cancers, the mechanism by which this enzyme and its metabolite promote cancer progression is unknown. In this study, we investigated the role of COX-2 in fibronectin-induced up-regulation of rhabdomyosarcoma matrix metalloproteinase (MMP)-2 activity and cellular invasiveness. We tested three human rhabdomyosarcoma cell lines: RMS559, RD, and SJRH30. Cell attachment to fibronectin up-regulated both COX-2 expression and PGE(2) production and concomitantly enhanced MMP-2 activity. Exogenous PGE(2) stimulated MMP-2 promoter activity, increased MMP-2 expression, and increased cellular invasiveness. Aspirin and rofecoxib (non-selective and selective COX-2 inhibitor, respectively) each abolished fibronectin-associated induction of MMP-2 and induced dose-dependent reductions in cellular invasiveness. These data implicated a role for inducible COX-2 and PGE(2) in the regulation of rhabdomyosarcoma cellular invasiveness and MMP-2 activity.

Anti-Inflammatory Agents, Non-Steroidal↗
PubMedDate not supplied

Rhabdomyosarcoma of the breast.

AIM: The aim of this study was to describe the first imaging studies of a primary embryonal rhabdomyosarcoma of the breast in an adult woman. PATIENT: This 45-year-old woman was seen for investigation of a palpable mass in the left breast. Examinations included mammography, Doppler ultrasound, and MRI. The diagnosis was made after surgical biopsy. Treatment consisted in neoadjuvant chemotherapy, followed by quadrantectomy and interstitial curietherapy. Imaging studies were performed both prior to and during therapy (i.e., after three then after six courses of chemotherapy). Imaging findings were correlated with histopathological findings. RESULTS: Mammography was not very contributive as the breasts were dense, particularly in the external left quadrants. On ultrasound, the mass had a tissue density and sharp contours; the mass was not highly vascularized on Doppler. MRI revealed a mass that enhanced markedly on both early and late contrast-enhanced T1-weighted sequences and was hyperintense on T2-weighted sequences. MRI appeared to be the best technique for the evaluation of the residual tissue. CONCLUSION: Rhabdomyosarcoma is a rare tumor in adults; to our knowledge, this is the first report describing the imaging features and value of MRI for the work-up and follow-up of mammary rhabdomyosarcoma.

Antibiotics, Antineoplastic↗
PubMed2004

Late events occurring five years or more after successful therapy for childhood rhabdomyosarcoma: a report from the Soft Tissue Sarcoma Committee of the Children's Oncology Group.

The aim of our study was to describe late failures in children who initially survived event-free five years from a diagnosis of rhabdomyosarcoma. Charts of children enrolled in the Intergroup Rhabdomyosarcoma Study Group (IRSG) trials III, IV pilot and IV (1984-1997) who survived five years event-free and subsequently experienced an adverse event (disease recurrence, second malignant neoplasm or death from other causes) were reviewed. Of the 2534 enrolled patients, 1160 were event-free at five years and 48 subsequently experienced a late event. The estimated 10-year event rate for the 1160 patients who were alive and event-free at five years was 9% (95% Confidence Interval (CI) 5%, 13%). Patients with both advanced disease (Group III/IV) and large primary tumours at diagnosis (> 5 cm) were at the highest risk for late events (19%; 95% CI 8%, 30%). Late events after successful treatment for rhabdomyosarcoma occur in 9%. Those with advanced disease and large primary tumours have the highest risk of late events.

Adolescent↗
PubMed2004

Rhabdomyosarcoma presenting as an anterior neck mass and possible thyroid malignancy in a seven-month-old.

A seven-month-old male presented with a one month history of an enlarging left neck mass and worsening inspiratory stridor. Upon excision of the mass, pathologic examination was consistent with embryonal rhabdomyosarcoma (RMS). Preoperative imaging and intraoperative exploration were consistent with tumor replacing the left lobe of the thyroid. No cases of either anterior neck rhabdomyosarcoma or thyroid rhabdomyosarcoma have been explicitly described in the literature. The distinction between the two malignancies becomes important when considering prognosis and treatment protocols.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2004

Outcome for children with group III rhabdomyosarcoma treated with or without radiotherapy.

PURPOSE: To analyze the sites of relapse, relapse-free survival, and overall survival in children with Group III rhabdomyosarcoma treated with or without radiotherapy (RT). METHODS AND MATERIALS: The outcomes of 48 children with Group III rhabdomyosarcoma treated between 1980 and 1997 were evaluated. The median overall survival follow-up was 7.3 years. Of the 48 patients, 65% had embryonal histology. Local treatment after induction chemotherapy included complete surgical resection (CSR) alone in 9 (19%), CSR plus RT in 13 (27%), partial resection or rebiopsy plus RT in 10 (21%), and RT alone in 15 patients (31%). One child developed distant disease before completing local therapy. RESULTS: Of the 48 patients, 12 developed relapse at local (n = 3), regional (n = 4), or distant (n = 5) sites. All 9 patients treated with CSR after induction chemotherapy had embryonal/botryoid histology. Only 1 of these 9 patients developed relapse. No statistically significant difference was found in overall survival (p = 0.95) or relapse-free survival (p = 0.67) between patients treated with or without RT. The Kaplan-Meier estimate of 5-year overall survival and relapse-free survival was 76% +/- 7% and 74% +/- 7%, respectively. Significant predictors of relapse-free survival on univariate analysis included CSR (p = 0.03), nodal positivity (p = 0.001), and embryonal histology (p = 0.0003). On multivariate analysis, embryonal histology was the most significant predictor of relapse-free (p = 0.001) and overall (p = 0.01) survival. CONCLUSION: The overall survival for children with Group III rhabdomyosarcoma in this series was favorable. Embryonal/botryoid histology was the most significant predictor of both overall and relapse-free survival. We found that for a selected subgroup of children with embryonal histology, induction chemotherapy followed by complete surgical resection alone may be adequate local therapy.

Adolescent↗
PubMed2006

Phacomatosis pigmentokeratotica associated with hemihypertrophy and a rhabdomyosarcoma of the abdominal wall.

Phacomatosis pigmentokeratotica (PPK) represents a specific "twin nevus" syndrome in which a speckled lentiginous nevus (SLN) is associated with an organoid nevus with sebaceous differentiation. A boy with a large nevus sebaceus on the left face and upper part of the trunk, a giant segmental SLN extending from the abdomen to the feet bilaterally, and right hemihypertrophy developed an embryonal rhabdomyosarcoma of the right abdominal wall at age 6 months. A variety of musculoskeletal, neurologic, and ocular anomalies have been observed in patients with PPK, reflecting the individual manifestations of both SLN and Schimmelpenning syndromes. This report adds hemihypertrophy to the spectrum of extracutaneous manifestations of PPK and, to our knowledge, represents the first observation of a rhabdomyosarcoma arising in contiguity with an SLN in a patient with PPK. The development of a rhabdomyosarcoma in our patient likely reflects both increased propensity for growth (as evidenced by the hemihypertrophy) and the pluripotent nature of neural-crest derived cells within the field defect that underlies an SLN.

Abdominal Wall↗
PubMed2005

Predictors of outcome in children and adolescents with rhabdomyosarcoma of the trunk--the St Jude Children's Research Hospital experience.

PURPOSE: The aim of this study was to determine predictors of outcome in childhood truncal rhabdomyosarcoma. METHODS: Retrospective chart review evaluating the impact of demographic features, disease characteristics, and the extent and timing of surgical intervention on outcome was performed. RESULTS: Thirty-three patients with a median age of 8 years were identified. Most had advanced Intergroup Rhabdomyosarcoma Study group III (n = 13) or group IV (n = 9) disease. Primary site included 20 (61%) chest wall, 6 (21%) paraspinal, 5 (15%) periscapular, and 1 (3%) abdominal wall. Most tumors were embryonal (n = 21), larger than 5 cm (n = 27), and locally invasive (n = 13); 7 had positive nodes. Tumor size, nodal status, and gross total tumor resection (upfront or delayed) were significant predictors of event-free and overall survival. Tumors 5 cm or smaller were amenable to upfront surgical resection (P = .007). In patients with tumors larger than 5 cm, resection at any time was associated with a 10-year overall survival 57% +/- 13% compared with 8% +/- 5% in those who had no surgery (P = .003). Tumor recurrence was local in 44% of cases, and survival after local recurrence was rare (1 of 8). CONCLUSION: Tumor size, nodal status, and gross total resection at any time are significant predictors of outcome in truncal rhabdomyosarcoma. Gross tumor excision should be the goal of surgical intervention in this disease.

Abdominal Neoplasms↗
PubMedDate not supplied

Puerperal renal rhabdomyosarcoma: case report and review of the literature.

Renal rhabdomyosarcoma is a rare malignant mesenchymal tumor with an aggressive clinical course. We describe the case of a 39-year-old woman with a large rhabdomyosarcoma of the left kidney serendipitously discovered ultrasonographically subsequent to cesarean section. To our knowledge, this is the first reported case of renal rhabdomyosarcoma during pregnancy. The importance of ultrasound examination in the differential diagnosis of gestational flank pain is discussed.

Adult↗
PubMed2005

Response of human rhabdomyosarcoma cell lines to retinoic acid: relationship with induction of differentiation and retinoic acid sensitivity.

The ability of retinoids to induce growth inhibition associated with differentiation of diverse cell types makes them potent anti-cancer agents. We examined the effect of retinoic acid (RA) in cell lines derived from rhabdomyosarcoma (RMS), a malignant soft-tissue tumor committed to the myogenic lineage, but arrested prior to terminal differentiation. We showed that several RMS derived cell lines, including RD human rhabdomyosarcoma cells, are resistant to the growth-inhibitory and differentiation effects of RA. We established that this RA-resistance correlates with reduced expression and activity of RA-receptors in RD cells. We stably expressed either RARalpha, RARbeta, RARgamma, or RXRalpha expression vector into RD cells and found that only RARbeta or RARgamma induced a significant RA growth arrest without promoting differentiation indicating that changes in the amounts of RARs and RXRs are not sufficient to determine the RA myogenic response of rhabdomyosarcoma cells. Activation of RD cell differentiation by ectopic MRF4 expression enhanced RA-receptor activity and led to RA induction of differentiation. These studies demonstrate that RA-resistance of RD cells is linked to their lack of differentiation and suggest that the differentiation-promoting activity of RA requires factors other than RAR-RXR heterodimers.

Animals↗
PubMed2006

Embryonal rhabdomyosarcoma of the uterus in a postmenopausal woman. Case report and review of the literature.

BACKGROUND: Embryonal rhabdomyosarcoma is a rare sarcoma which characteristically occurs in non genitourinary sites in children. CASE: We present a case of uterine embryonal rhabdomyosarcoma in a postmenopausal patient who presented with increasing abdominal girth, early satiety, weight loss, and pelvic pain. CONCLUSION: Embryonal rhabdomyosarcoma does not commonly originate from the uterine corpus, and it is rarely seen in postmenopausal patients. A review of the literature confirms the unique nature of this case.

Aged↗
PubMed1997

Pediatric rhabdomyosarcoma of the head and neck.

PURPOSE: Survival for pediatric rhabdomyosarcoma has improved with the use of multidrug chemotherapy and external beam radiotherapy. This study was performed to determine survival in a cohort of patients treated on one of three multidrug treatment protocols for head and neck rhabdomyosarcoma and to identify factors that place patients at risk for treatment failure. METHODS: Pertinent prognostic variables including age, sex, subsite of origin, resectability, and TNM stage were analyzed by the Kaplan-Meier methods with comparisons between variables performed using the Prentice-Wilcoxon test statistic. RESULTS: Overall 5-year survival was 74% (95% confidence interval 64% to 84%). Local failure accounted for the cause of death in 10 patients, and 8 died of disseminated disease. On univariate analysis, each variable contributing to the TNM staging system was significant in determining survival; invasiveness (P = 0.01), size (P = 0.02), nodal metastases (P <0.01), and distant disease (P <0.01). CONCLUSION: Survival has improved for head and neck rhabdomyosarcoma treated with multimodality therapy. Patients with advanced-stage disease are at greatest risk for treatment failure and require the most aggressive therapy.

Child↗
PubMed2001

Total orthotopic heart transplantation for primary cardiac rhabdomyosarcoma: factors influencing long-term survival.

BACKGROUND: Primary cardiac sarcomas are uncommon and rare, with an unequal distribution in the population. A dismal prognosis is usually admitted that is related to a high propensity to develop distant metastasis with survival rarely exceeding 2 years. We report a case of a patient with a primary cardiac rhabdomyosarcoma characterized by an exceptional long-term survival after surgical treatment by a total orthotopic heart transplantation. From this limited experience, we reviewed factors that may influence survival to optimize therapeutic strategy. METHODS: A 33-year-old man was found to have a 10-cm primary cardiac rhabdomyosarcoma located in the right atrium and extending to the atrioventricular groove; therefore, resection was not possible. Since no metastases were detected, the patient was scheduled for urgent cardiac transplantation, which was performed after adjuvant radiotherapy. RESULTS: Postoperative outcome was uneventful and the patient is still alive, with regular follow-up, at 102 months. CONCLUSIONS: In a case of primary rhabdomyosarcoma, heart transplantation, despite immunosuppressive therapy, can provide long-term survival and can be considered for selected patients after rigorous analysis of predictors of survival.

Adult↗