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 325 records · Page 18Linked to original sources
PubMedDate not supplied

Cytologic diagnosis of rhabdomyosarcoma in a child with a pleural effusion. A case report.

BACKGROUND: A pleural effusion in children is usually caused by infectious diseases; malignant effusion is very uncommon. In a case of a malignant effusion in a child, a pleura-based metastasis of a neoplasm with a typically high prevalence in childhood has to be considered. Examples are neuroblastoma, nephroblastoma, Wilms' tumor, hepatoblastoma, malignant germ cell tumor and rhabdomyosarcoma. CASE: A 4-year old boy presented with a unilateral pleural effusion. Cytologic examination of the effusion revealed malignant small round cells admixed with very large cells with atypical nuclei. In formalin-fixed cell blocks prepared for immunocytochemistry the cells expressed desmin and myf-4. The cytologic diagnosis was consistent with metastatic rhabdomyosarcoma. Subsequent computed tomography revealed a mass above the diaphragm, which was biopsied. Histologic examination of a needle biopsy specimen confirmed the diagnosis of rhabdomyosarcoma. Molecular examination revealed a PAX3-FKHR fusion transcript specific to the alveolar type of rhabdomyosarcoma. CONCLUSION: This case illustrates the usefulness of immunocytochemistry on cell block preparations in diagnosing difficult cases of effusion cytology.

Biomarkers, Tumor↗
PubMedDate not supplied

Fine needle aspiration of poorly differentiated rhabdomyosarcoma presenting with quadriparesis. A case report.

BACKGROUND: The diagnosis of metastatic poorly differentiated rhabdomyosarcoma (RMS) in lymph node specimens by fine needle aspiration presents a difficult problem since it is virtually indistinguishable from other small round cell neoplasms. CASE: Fine needle aspiration was performed under radiologic guidance on an extradural, space-occupying lesion of unknown etiology in the region of the C-6 and C-7 vertebrae in a 20-year-old male who was hospitalized with quadriparesis. Cytologic examination suggested a metastatic tumor consistent with the diagnosis of rhabdomyosarcoma. A subsequent search for the primary tumor site revealed a soft tissue swelling in the right calf muscle. Light microscopic, ultrastructural and immunocytochemical examination of multiple Tru-cut biopsy specimens from the swelling in the right calf muscle confirmed the diagnosis of poorly differentiated embryonal rhabdomyosarcoma. CONCLUSION: Immunostaining is useful for muscle proteins in the detection of poorly differentiated forms of embryonal rhabdomyosarcoma. Electron microscopy is of limited use in such cases.

Adult↗
PubMed1985

Current concepts in the management of paratesticular rhabdomyosarcoma.

Paratesticular rhabdomyosarcoma is a rare malignant disease most commonly seen in infants and children. In contrast with rhabdomyosarcomas in sites other than the genitourinary tract, paratesticular rhabdomyosarcomas tend to metastasize via a lymphatic route. This influences the therapeutic approach in which surgery, chemotherapy and, in selected cases, radiation therapy play an essential role. On hand of 7 of our own patients, uniformly treated at one institution, a review of the current approach of paratesticular rhabdomyosarcomas with excellent therapeutic results is given.

Adolescent↗
PubMed1994

Antisense-mediated reduction in insulin-like growth factor-I receptor expression suppresses the malignant phenotype of a human alveolar rhabdomyosarcoma.

The expression of the insulin-like growth factors (IGFs) and their receptors has been linked to cellular proliferation and tumorigenicity in a number of model systems. Since rhabdomyosarcoma cells express IGF-I receptors, an autocrine or paracrine loop involving this receptor and its ligands could be responsible in part for the growth characteristics of this tumor. To assess directly the role of the IGF-I receptor in rhabdomyosarcoma cell growth and tumorigenicity, a human alveolar rhabdomyosarcoma cell line with high IGF-I receptor expression was transfected with an amplifiable IGF-I receptor antisense expression vector. Four unique, transfected clones were analyzed and found to have reduced IGF-I receptor expression relative to the parental line. Integration of the antisense sequence was demonstrated by Southern blot analysis, and expression of antisense message in these clones was shown by S1 nuclease protection assay. Reduced IGF-I receptor surface expression in the transfectants was shown by decreased immunofluorescence with an IGF-I receptor monoclonal antibody and by decreased IGF-I binding as measured by Scatchard analysis. These clones had markedly reduced growth rates in vitro, impaired colony formation in soft agar, and failed to form tumors in immunodeficient mice when compared with vector-transfected clones. These results demonstrate that reduction of IGF-I receptor expression can inhibit both the in vitro and in vivo growth of a human rhabdomyosarcoma cell line and suggest a role for the IGF-I receptor in mediating neoplastic growth in this mesenchymally derived tumor.

Blotting, Southern↗
PubMed2006

Alveolar rhabdomyosarcoma of the paranasal sinuses in a 57-year-old woman with 1:16 translocation.

Alveolar rhabdomyosarcoma is an uncommon tumor. It is a rare malignancy in adults and rarely occurs in paranasal sinuses in those aged older than 50 years. This report describes a locally invasive and destructive alveolar rhabdomyosarcoma arising in the ethmoid sinus of a 57-year-old woman. The small round blue cell tumors are positive for myogenin by immunohistochemistry and have a karyotype of 45, XX, -5, -13, der(16)t(1;1) (q21;q13) by cytogenetic analysis. Fluorescence in situ hybridization demonstrated a complex translocation with break apart of the FKHR region, which supports a diagnosis of alveolar rhabdomyosarcoma. This report characterizes this tumor through microscopic and cyto-genetic analysis and emphasizes the importance of considering rhabdomyosarcoma in the differential diagnosis of small round cell tumors of the head and neck region in the middle-aged adults.

Carcinoma, Small Cell↗
PubMed1996

P-glycoprotein expression at diagnosis may not be a primary mechanism of therapeutic failure in childhood rhabdomyosarcoma.

PURPOSE: To evaluate the prognostic significance of tumor cell P-glycoprotein (Pgp) expression at diagnosis in children with rhabdomyosarcoma. PATIENTS AND METHODS: A panel of three anti-Pgp monoclonal antibodies (mAb) (C219, C494, and JSB-1) that recognize different Pgp epitopes was used to measure Pgp expression in rhabdomyosarcoma specimens obtained at diagnosis from 76 patients treated at St Jude Children's Research Hospital from 1969 to 1991. Two separate experiments using different immunohistochemical methods (immune alkaline phosphatase and immunoperoxidase) were performed to evaluate Pgp expression. The immunostaining was graded using a semiquantitative scale corresponding to the percentage of tumor cells with detectable staining. The influence of Pgp expression on outcome was assessed by the Kaplan-Meier method and Cox regression analysis with stepwise selection. The relationship between Pgp expression and clinical features was assessed using the Mantel-Haenszel method. RESULTS: Pgp expression at diagnosis did not predict worse overall survival or progression-free survival when tested in either experiment with C219, C494, or JSB-1 separately. No association was shown between Pgp expression and clinical features (clinical group, primary site, or histology) or response. However, in the immune alkaline phosphatase experiment, patients whose tumors had more than 10% tumor cell staining with all three mAbs had a significantly higher rate of estimated 5-year survival (78% +/- 10%) than did all other patients (38% +/- 8%; P = .025). In this instance, Pgp expression had independent prognostic value after adjusting for clinical group. CONCLUSION: We found no strong association between Pgp expression at diagnosis and clinical features or extent of disease in pediatric rhabdomyosarcoma. Depending on the criteria used to define it, high Pgp expression at diagnosis does not predict poor outcome. Although a large prospective study is needed to provide definitive conclusions, our findings suggest that Pgp-mediated multidrug resistance may not be a primary mechanism of therapeutic failure in rhabdomyosarcoma.

ATP Binding Cassette Transporter, Subfamily B, Mem↗
PubMed2001

Intergroup rhabdomyosarcoma study-IV: results for patients with nonmetastatic disease.

PURPOSE: The study goal was to improve outcome in children with rhabdomyosarcoma by comparing risk-based regimens of surgery, radiotherapy (RT) and chemotherapy. PATIENTS AND METHODS: Eight hundred eighty-three previously untreated eligible patients with nonmetastatic rhabdomyosarcoma entered the Intergroup Rhabdomyosarcoma Study-IV (IRS-IV) (1991 to 1997) after surgery and were randomized treatment by primary tumor site, group (1 to 3), and stage (I to III). Failure-free survival (FFS) rates and survival were the end points used in comparisons between randomized groups and between patient subgroups treated on IRS-III and IRS-IV. Most patients were randomized to receive vincristine and dactinomycin (VA) and cyclophosphamide (VAC, n = 235), or VA and ifosfamide (VAI, n = 222), or vincristine, ifosfamide, and etoposide (VIE, n = 236). Patients with group 3 tumors were randomized to receive conventional RT (C-RT) versus hyperfractionated RT (HF-RT). RESULTS: Overall 3-year FFS and survival were 77% and 86%, respectively. Three-year FFS rates with VAC, VAI, and VIE were 75%, 77%, and 77%, respectively (P =.42). No significant difference in outcome was noted with HF-RT versus C-RT (P =.85 and P =.90, respectively). Overall, patients with embryonal tumors benefited from intensive three-drug chemotherapy in IRS-IV (3-year FFS, 83%). The improvement was seen for patients with stage I or stage II/III, group 1/2 disease, many of whom received VA chemotherapy on IRS-III. Patients with stage 2/3, group 3 disease had similar outcomes on IRS-III and IRS-IV. Three-year FFS for the nonrandomized patient subsets was 75% with renal abnormalities; 81% for paratesticular, group 1 cases; and 91% for group 1/2 orbit or eyelid tumors. Patients with paratesticular primaries had poorer outcomes if they were more than 10 years old (3-year FFS, 63% v 90%). Myelosuppression occurred in most patients, but toxic deaths occurred in less than 1%. CONCLUSION: VAC and VAI or VIE with surgery (with or without RT), are equally effective for patients with local or regional rhabdomyosarcoma and are more effective for embryonal tumors than therapies used previously. Younger patients with group 1 paratesticular embryonal tumors and all patients with group 1/2 orbit or eyelid tumors can usually be cured with VA chemotherapy along with postoperative RT for group 2 disease.

Adolescent↗
PubMed2006

Pooled analysis of phase II window studies in children with contemporary high-risk metastatic rhabdomyosarcoma: a report from the Soft Tissue Sarcoma Committee of the Children's Oncology Group.

PURPOSE: The Soft Tissue Sarcoma Committee of the Children's Oncology Group has conducted five upfront window trials in patients with newly diagnosed metastatic rhabdomyosarcoma to identify promising new treatment agents. PATIENTS AND METHODS: This pooled analysis identified a total of 420 patients (115 from Intergroup Rhabdomyosarcoma Study III [IRS-III] and 305 from the five window trials). We assessed window therapy response rate, failure-free survival (FFS), and overall survival (OS). RESULTS: Response rates (complete + partial response) assessed at week 6 of window therapy ranged from 41% to 55% and did not predict FFS (P = .073) or OS (P = .31). FFS was influenced by trial (P = .048); patients enrolled onto IRS-III and the ifosfamide/etoposide and ifosfamide/doxorubicin trials fared best. When grouped and compared with topoisomerase I poison trials, ifosfamide/topoisomerase II inhibitor trials had superior FFS (P = .013). However, there was no difference in survival. CONCLUSION: Upfront phase II window trials can efficiently provide robust estimates of activity for new agents and combinations in newly diagnosed patients with high-risk rhabdomyosarcoma. Our data indicate that, for some phase II window trials, the risk of treatment failure may be increased but that the trend towards lower survival for some of the window trials compared with IRS-III is not statistically significant. Window nonresponders did not suffer worse FFS or OS than patients who responded to window therapy. Finally, these results provide a rationale for incorporating ifosfamide, etoposide, doxorubicin, and topoisomerase I poisons in future trials of high-risk metastatic rhabdomyosarcoma.

Adolescent↗
PubMed2005

Late effects of pelvic rhabdomyosarcoma and its treatment in female survivors.

PURPOSE: To document the spectrum and severity of late effects in female survivors of pelvic rhabdomyosarcoma. PATIENTS AND METHODS: We reviewed the demographic, diagnostic, treatment, and outcome data of the 26 females treated for pelvic rhabdomyosarcoma at our institution between March 1962 and December 1996 who survived free of disease for 5 or more years. Adverse effects that occurred 5 or more years after diagnosis were graded according to the National Cancer Institute Common Terminology Criteria for Adverse Events, version 3.0. RESULTS: The most common tumor sites were vagina (n = 7), pelvis/retroperitoneum (n = 6), and bladder (n = 4). All patients received chemotherapy (alkylating agent, n = 23; doxorubicin, n = 16); 22 received radiotherapy (median dose, 46 Gy). Median follow-up of the 23 survivors was 20.3 years. Late effects occurred in 24 patients, 23 of whom had grade 3/4 late effects (median grade 3/4 late effects per patient, three; range, zero to 14). Fourteen patients (54%) required surgery for late complications. The 22 patients who had received radiotherapy had a greater median number of late effects per patient than did the remaining four (9.5 v one; P = .002). The median number of late effects per patient was higher in the 12 patients treated during or after 1984 than in the 14 treated earlier (12.5 v 6.5; P = .041). CONCLUSION: The burden of late effects in girls treated for pelvic rhabdomyosarcoma is significant and does not seem to be diminishing with advances in treatment. Prospective studies are needed to better assess the impact of these late effects on quality of life and functional outcome, and to refine the treatment approach to pelvic rhabdomyosarcoma.

Adolescent↗
PubMed2004

Ultrasound diagnosis of paratesticular rhabdomyosarcoma.

Rhabdomyosarcoma is the most common tumour of the lower genitourinary tract in children in the first two decades. Paratesticular rhabdomyosarcoma is associated with a significantly better outcome than lesions elsewhere in the genitourinary tract. Although ultrasound is considered the imaging modality of choice for evaluating intrascrotal pathology, the ultrasound appearance of paratesticular rhabdomyosarcoma has rarely been reported and may be confused with other disease entities such as epididymitis, adenomatoid tumour and leiomyoma. We present the ultrasound features of a paratesticular rhabdomyosarcoma, discussing the clinical features and differential diagnosis.

Adolescent↗
PubMed1989

Rhabdomyosarcoma of the ear and temporal bone.

Rhabdomyosarcoma, the most common soft tissue sarcoma of childhood, involves the temporal bone in approximately 7% of reported cases. Until recently, the outcome of this disease was always fatal. The recent Intergroup Rhabdomyosarcoma Study Group (IRS-I) reported on the efficacy of multimodality therapy consisting of multiagent chemotherapy, radiation, and surgical resection when indicated. Twelve patients with rhabdomyosarcoma involving the temporal bone were treated between 1966 and 1988. Three patients were treated according to the IRS-I protocols and the remaining nine patients received various combinations of treatment modalities. Ten patients succumbed to their disease, most with distant metastases or intracranial extension. Two patients are alive; one at 5 1/2 years and one at 19 years. It is apparent that, although survival for rhabdomyosarcoma in general has improved with the use of IRS-I protocols, prognosis remains poor for disease involving the temporal bone and other parameningeal sites.

Adolescent↗
PubMed2005

Parathyroid hormone expression in a patient with metastatic nasopharyngeal rhabdomyosarcoma and hypercalcemia.

Ectopic PTH secretion by tumor cells has been described as the cause of hypercalcemia associated with malignancy in the absence of osteolytic bone lesions. Although there have been case reports of elevated PTH and hypercalcemia in patients with rhabdomyosarcoma, to date ectopic PTH secretion by malignant cells has not been definitively shown. The possibility of PTH production by pleural-based metastatic nasopharyngeal rhabdomyosarcoma cells in a 62-yr-old Japanese male with hypercalcemia was investigated. The patient's serum PTH level was found to be elevated at 62.22 pmol/L, and pleural fluid PTH level was 47.28 pmol/L and PTHrP level was 3.7 pmol/L. RT-PCR of mRNA extracted from rhabdomyosarcoma cells in the pleural fluid was performed with the addition of PTH and PTHrP exonic primer sets yielded only a cDNA fragment of approx 150 bp consistent with the expected PTH fragment. Sequence analysis of a nested primer PCR fragment confirmed PTH mRNA sequence. We believe this patient to have had hypercalcemia secondary to ectopic PTH secretion, as we have identified the presence of PTH mRNA in tumor cells. We speculate that the overexpression of PTH in rhabdomyosarcoma cells results from molecular rearrangement of the PTH gene. The finding of a normal PTH DNA sequence of the PCR fragment suggests the likelihood of alterations in regulatory sequences.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2005

Current chemotherapeutic strategies for rhabdomyosarcoma.

Rhabdomyosarcoma is a typical tumor of childhood and adolescence. Over the years there has been a gradual but important improvement in survival for patients with this tumor, despite its high grade of malignancy. These results are due to multidisciplinary treatment approaches including surgery, radiotherapy and especially chemotherapy. Rhabdomyosarcoma is a highly chemosensitive neoplasm, and the role of this therapeutic approach has also been clearly demonstrated in the adjuvant setting. This review covers current concepts on chemotherapy for rhabdomyosarcoma, with an overview of the results of the main clinical trials conducted over recent years and considerations of possible strategies for the near future. Recommendations for adult patients with rhabdomyosarcoma are also discussed, suggesting that these patients should be treated according to pediatric guidelines.

Adolescent↗
PubMed1993

Rhabdomyosarcoma in the inferior vena cava with secondary Budd-Chiari syndrome.

A 63-year-old man with symptoms of obstruction of the inferior vena cava was examined by computed tomography, ultrasound imaging and angiography. Examination revealed a tumor in the inferior vena cava, and transvenous biopsy revealed a rhabdomyosarcoma. The tumor was surgically resected and was easily separated from the surrounding tissues. Nevertheless, a local recurrence developed 43 days after the operation, and the patient's condition deteriorated rapidly. Hepatomegaly and ascites believed to represent the Budd-Chiari syndrome were noted. The patient died on the 163rd postoperative day. Autopsy revealed a tumor extending from the inferior vena cava just above the right renal vein to the right atrium and involving the lobus caudatus of the liver. Clinically, the tumor was thought to have arisen from the middle segment of the inferior vena cava. However, a diagnosis of primary hepatic rhabdomyosarcoma with extrahepatic growth could not be excluded. Only 12 cases of primary liver rhabdomyosarcoma have been reported, and none of those patients demonstrated Budd-Chiari syndrome. Our patient, diagnosed as rhabdomyosarcoma with secondary Budd-Chiari syndrome, is believed to be the first such report.

Budd-Chiari Syndrome↗
PubMedDate not supplied

[Rhabdomyosarcomas in the naso-paranasal area].

The nasoparanasal region rhabdomyosarcoma belongs to the group of the rare malignant diseases, however it nevertheless represents major diagnostic and therapeutic problem. The study is aimed at presenting clinical casuistry of the nasoparanasal region rhabdomyosarcoma in adults and children, for the purpose of assessment of modern diagnostic and therapeutic possibilities. The study was retrospective and included the patients with evidenced and pathohistologically verified rhabdomyosarcoma of the nasoparanasal region diagnosed and treated at the Institute of Otorhinolaryngology and Maxillofacial Surgery of the Clinical Centre of Serbia, during the period 1988-2001, and subsequently at the Institute of Oncology in Belgrade. The total of 9 patients aged 3-50 years (median 15) were assessed, with the female to male ratio being 4:5. Seven patients were previously hospitalized in other health institutions. The following parameters were analyzed: sex, age group, number of hospitalizations, diagnostic procedures, histological tumour types, mode of treatment and disease outcome. In 7 patients, the tumour spread beyond the nasoparanasal region--in 6 into the orbit, in 5 into the epipharynx, in 3 intracranially, in 1 into the pterygopalatine pit and in another 1 into the parapharyngeal space. Regional lymph nodes were affected only in one patient, while distant bone metastases were evidenced on another one. Embryonal type of the rhabdomyosarcoma was verified in 7 patients, while two had the alveolar type. In the affected children mean survival was significantly longer (76.3 months) in comparison to the one observed in adults (11.5 months). Therapeutic procedure included combined application of the chemotherapeutic protocols (adjusted with respect to the age group and stage of the disease) and radiotherapy. Radical surgical procedures were applied on in case of stage I tumours, and they may also be used in rest-relapse cases subsequent to the applied chemo-, i.e., radiotherapy.

Adolescent↗
PubMedDate not supplied

Ovarian Sertoli-Leydig cell tumor with rhabdomyosarcoma: an ultrastructural study.

The clinical and light and electron microscopic findings of a moderately differentiated, virilizing, Sertoli-Leydig cell tumor (SLT) with pleomorphic rhabdomyosarcoma of the ovary are presented. The tumor recapitulates the primitive embryonal testis and rhabdomyogenesis, respectively. The natural history, including pathogenesis, of this peculiar and rare tumor is discussed in the light of the pertinent literature on SLT and ovarian rhabdomyosarcoma. It seems that when rhabdomyosarcoma is a significant or predominant component of SLT, as occurred in this case, the prognosis is poor and is that of rhabdomyosarcoma in general.

Adolescent↗
PubMed1994

Flow cytometric analysis of DNA ploidy in childhood rhabdomyosarcoma.

Flow cytometric DNA analysis was performed on 17 rhabdomyosarcomas in conjunction with a histopathological review to determine the usefulness of this technique to predict the biologic behavior of the tumor and to establish the characteristic ploidy pattern of rhabdomyosarcoma compared to other small round cell tumors occurring in childhood. Aneuploidy including near-tetraploidy is the most common ploidy pattern encountered, followed by multiploidy and diploidy, and the presence of multiploidy in this tumor is useful for differentiating rhabdomyosarcoma from other kinds of small round cell tumors in which there are rare previous reports on occurrence of multiploidy. Even though there is no significant correlation between ploidy pattern and histologic type of rhabdomyosarcoma, patients with multiploid tumors or aneuploid tumors with a DNA index of 1.10-1.80 tend to have a high risk of treatment failure. Therefore, the ploidy pattern seems to be useful for predicting the patient's survival in concert with other variables.

Adolescent↗
PubMed2000

Reduced or absent expression and codon 201Gly/Arg polymorphism of DCC gene in rhabdomyosarcoma and Ewing's sarcoma/PNET family.

Genetic alterations occurring in various chromosomes have been described in many human tumors. The DCC gene was originally identified in colorectal cancer and was reported as a tumor suppressor gene that might be related to tumor metastasis. We investigated 10 cell lines and 15 fresh tumors of childhood rhabdomyosarcoma, 7 cell lines of Ewing's sarcoma, and 4 cell lines of primitive neuroectodermal tumor (PNET) for the expression and mutation of DCC gene by RT-PCR analysis and PCR-single stranded conformation polymorphism (SSCP) analysis. Twenty-five pairs of primers were used for PCR-SSCP. Six of ten (60%) cell lines of rhabdomyosarcoma and 3 of 7 (43%) cell lines of Ewing's sarcoma showed reduced or absent expression of DCC gene. There was no mobility shift within 24 exons by SSCP analysis, although 3 types of polymorphism were found at codon 201 in exon 3. Direct sequencing of different bands showed types I, II, and I/II representative of codon 201Gly, codon 201Arg, and codon 201Gly/Arg, respectively. The proportion of type I between fresh rhabdomyosarcoma and normal controls was not significant. Our results suggested that the inactivation of DCC gene may play a role in the pathogenesis of a subset of rhabdomyosarcoma and Ewing's sarcoma.

Adolescent↗