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At least 1,459 records · Page 81Linked to original sources
PubMed1990

The clinical features and prognosis of rhabdomyosarcoma: follow-up studies on pediatric tumors from the Japanese Pediatric Tumor Registry 1971-1980. Part II. Committee of Malignant Tumors, Japanese Society of Pediatric Surgeons.

A retrospective study was conducted on 126 patients with rhabdomyosarcoma enrolled in the Japanese Pediatric Tumor Registry between 1971 and 1980. The age of the patients ranged from less than 1 year to 15 years, and the male to female ratio was 1.3:1.0. Primary sites included the pelvis (37.3 per cent), abdomen (23.8 per cent), head and neck (21.4 per cent), thorax (9.5 per cent), extremities (6.4 per cent) and unknown (1.6 per cent). According to the staging system of the Japanese Society of Pediatric Surgeons, the extent of disease was classified into stages Ia (26.2 per cent of the total); Ib (14.6 per cent); II (12.6 per cent), III (29.1 per cent) and IV (17.5 per cent). The clinical stage was significantly correlated with survival outcome in this series (p less than 0.05). Age, sex, histology and primary site per se had no independent prognostic influence on tumor-free survival. With regard to treatment modalities, surgery was performed in 94.0 per cent of the patients, and radiotherapy at a mean dose of 37 Gy, and/or multi-agent chemotherapy in 41.7 per cent and 80.0 per cent, respectively. The patients who underwent total excision had a better survival outcome than those who did not (p less than 0.05). Combination chemotherapy such as VAC was more commonly administered in the latter study period. The overall 2-year tumor-free survival rate (2YTFSR) significantly improved from 24.0 per cent in the former period between 1971 and 1975 to 48.7 per cent in the latter period between 1976 and 1980 (p less than 0.05).

Adolescent↗
PubMed1987

Primary intracranial rhabdomyosarcoma: case report and review of the literature.

Invasion of the meninges is a relatively common complication of head and neck rhabdomyosarcoma (RMS), while RMS arising primarily within the brain or meninges is rare. We report the case of an 11-year old child with a primary "primitive" frontal lobe tumor, subsequent leptomeningeal spread and fatal intratumoral hemorrhage; the diagnosis of RMS was discovered only at postmortem examination. The literature contains a total of 34 reported cases of primary intracranial RMS. This tumor has been observed to arise in a variety of central nervous system (CNS) locations in patients of all ages, but most commonly within the posterior fossa of children. Leptomeningeal dissemination and spontaneous intratumoral hemorrhage are important clinical features. Postoperative chemotherapy and craniospinal radiation may improve the anticipated poor prognosis of patients treated with surgery and radiation alone. The diagnosis of RMS may be missed unless electron microscopic and specific immunohistochemical studies are applied to "undifferentiated" or "primitive" CNS tumors.

Brain Neoplasms↗
PubMed2003

Embryonal rhabdomyosarcoma of the common bile duct.

A two-and half-year-old boy presented with a short history of fever, jaundice and abdominal distension. At laparotomy, a rhabdomyosarcoma (RMS) arising from the common bile duct (CBD) was found. The tumor including the CBD was successfully excised and a Roux-en-Y hepaticodochojejunostomy was performed. Postoperatively, the child received chemotherapy and is doing well on one-year follow-up the recent literature on the subject is reviewed.

Anastomosis, Roux-en-Y↗
PubMed1990

Uniform response of c-raf expression to differentiation induction and inhibition of proliferation in a rat rhabdomyosarcoma cell line.

The clonal rat rhabdomyosarcoma cell line BA-HAN-1C is composed of proliferating mononuclear cells, some of which spontaneously fuse to terminally differentiated myotube-like giant cells. Both the induction of differentiation by retinoic acid (RA) and by sodium butyrate (NaBut), as well as the inhibition of proliferation by fetal calf serum (FCS)-depleted medium uniformly resulted in the same effects. There was a significant (p less than 0.001) inhibition of proliferation and induction of cellular differentiation, as evidenced by a significant (p less than 0.05) increase in creatine kinase activity. Furthermore, after exposure to RA-supplemented or FCS-depleted medium, a significant (p less than 0.001) increase in the number of myotube-like giant cells was observed. These effects were preceded by a uniform enhancement of c-raf mRNA expression, which became evident 6 h after exposure to RA, NaBut and FCS-depleted media. C-raf mRNA expression persisted at an elevated level throughout the observation period of 5 days after exposure to RA or NaBut, whereas the increased expression of c-raf mRNA observed after FCS-depletion declined near to the basal level after only 24 h. Furthermore, a transient c-fos mRNA expression was observed 15 and 30 min after exposure to RA-supplemented and FCS-depleted medium but not after exposure to NaBut. The present results suggest a possible role of c-raf in the regulation of differentiation and proliferation of this cell line. Since all our experiments with RA, NaBut and FCS-depletion resulted in an early peak of c-raf mRNA expression, it is suggested that this early peak may be sufficient to trigger events crucial for differentiation and proliferation of BA-HAN-1C tumor cells.

Animals↗
PubMed1983

Rhabdomyosarcoma of the cord: ultrasonic evaluation.

A rare case of paratesticular rhabdomyosarcoma in a young adult is described, emphasizing the role of ultrasonic preoperative examination in evaluating the consistency and extension of the scrotal mass for surgical management.

Adult↗
PubMed1992

Magnetic resonance imaging for diagnosis and follow-up of genitourinary, pelvic, and perineal rhabdomyosarcoma.

Magnetic resonance (MR) imaging, ultrasound, and computed tomography (CT) were performed for diagnosis and follow-up of rhabdomyosarcoma (RMS) arising from the genitourinary tract in five children, pelvic musculature in one, and the perineum in three others. MR imaging performed at 1.0 T included the following: spin-density-, T1-, and T2-weighted images in all nine patients; gadopentatate dimeglumine (Gd-DTPA)-enhanced T1-weighted images in five; and short TI inversion recovery (STIR) images in two children. Longitudinal T1-weighted images were of additional help in localizing the primary tumor at diagnosis and detecting local spread. Residual or recurrent disease within the bladder was best detected by spin-density images. Gd-DTPA enhancement improved contrast in two studies, but paramagnetic artifacts obscured intravesical lesions in three other studies. STIR images disclosed pelvic and retroperitoneal lymphadenopathy. MR imaging is recommended as the key method of diagnosis and follow-up of pelvic RMS.

Adolescent↗
PubMed2001

Impact of treatment acceleration and its timing on the response of the rhabdomyosarcoma R1H of the rat to fractionated irradiation.

BACKGROUND AND PURPOSE: In clinical practice a concomitant boost is usually given as a second daily dose to a reduced field. The question arises which part of treatment should be accelerated to achieve optimal tumor control. An experiment was performed on tumor bearing rats to determine the optimal timing of treatment acceleration for this experimental tumor system. MATERIAL AND METHODS: Rhabdomyosarcoma R1H of the rat were treated applying 30 fractions in an overall treatment time of 40-42 days, up to total doses ranging from 67.5 to 97.5 Gy were administered. For control a standard treatment was given as continuous treatment applying one fraction per day. A boost of five additional fractions was given as a second fraction during 5 days. Three experimental arms received a boost either in the 1st, 4th, or in the last week of treatment. Treatment outcome was assessed using tumor control as endpoint. RESULTS: All experimental arms proved more effective than the standard treatment. Treatment was most effective when the boost was administered in the 1st week of treatment. A TCD37% of 87.1 Gy (95% CI: 82.8 ... 92.7 Gy), 96.5 Gy (89.9 ... 107.1), and 107.3 Gy (97.2 ... 131.0) was determined, when the boost was given in the 1st, 4th, or last week of treatment, respectively. The observed difference between the experimental arms was statistically significant (p = 0.004). CONCLUSIONS: Initially accelerated treatment schedules were found to be more effective for tumor control in an experimental tumor system.

Animals↗
PubMed2003

Cell-specific toxicity of fibrates in human embryonal rhabdomyosarcoma cells.

The effects of a variety of fibrates on the cell viability were examined in human embryonal rhabdomyosarcoma cells (HRMSC). Five fibrates, including fenofibrate, clofibrate, gemfibrozil, bezafibrate and ciprofibrate, all concentration-dependently reduced the cell viability determined by the mitochondrial enzyme activity. The cell injury occurred time-dependently and was marked at 24-48 h. The toxic action of fibrates was specific to HRMSC, since bezafibrate did not induce any marked changes in the viability of human microvascular endothelial cells or arterial smooth muscle cells. Synergistic cell injury was observed after a combined treatment with bezafibrate and simvastatin, although simvastatin alone reduced the cell viability. The cell injury was characterized by a typical nuclear damage, as evidenced by Hoechst 33342 staining and deoxynucleotidyl transferase dUTP nick-end label-positive staining. Similar cell-specific injury was induced by 8(S)-hydroxyeicosatetraenoic acid, a potent peroxisome proliferator-activated receptor alpha (PPARalpha) agonist. Consistent with these data, a marked expression for PPARalpha mRNA was observed in HRMSC but not in the endothelial or smooth muscle cells. Therefore, it is suggested that fibrates cause a cell-specific injury in HRMSC via activation of PPARalpha. Moreover, our present cell injury model using HRMSC may be useful for elucidating the mechanisms of clinical rhabdomyolysis induced by lipid-lowering agents.

Bezafibrate↗
PubMed2003

Hypertrophic osteoarthropathy in primary liver rhabdomyosarcoma.

Hypertrophic osteoarthropathy is a well-documented paraneoplastic phenomenon in adults. It is a rare, but important finding in children with malignant disease as it can indicate prognosis. We present a case of hypertrophic osteoarthropathy associated with primary liver rhabdomyosarcoma in a 14-year-old boy.

Adolescent↗
PubMed2006

Optic pathway glioma associated with orbital rhabdomyosarcoma and bilateral optic nerve sheath dural ectasia in a child with neurofibromatosis-1.

Neurofibromatosis-1 (NF-1) is a multisystem disorder presenting with a variety of clinical and imaging manifestations. Neural and non-neural tumours, and unusual benign miscellaneous conditions, separately or combined, are encountered in variable locations. We present a 2(1/2)-year-old boy with NF-1 who demonstrated coexisting optic pathway glioma with involvement of the chiasm and optic nerve, orbital alveolar rhabdomyosarcoma and bilateral optic nerve sheath dural ectasia.

Child, Preschool↗
PubMed1998

Hepatobiliary rhabdomyosarcoma in children: diagnostic radiology.

Rhabdomyosarcoma (RMS) occurs infrequently in the liver and biliary tree. Although the radiological diagnosis may be simple when the tumour involves the extrahepatic bile ducts, no specific imaging features are known for hepatic RMS. We present four cases and discuss the role of diagnostic radiology in the management of this tumour.

Biliary Tract Neoplasms↗
PubMed1999

Primary embryonal rhabdomyosarcoma of the diaphragm in a child: case report.

Primary rhabdomyosarcoma of the diaphragm presenting as an epigastric mass is extremely rare, with only three published cases. We highlight the unique imaging features in a 2(1)/(2)-year-old boy which predicted the correct anatomical site preoperatively. Awareness of this rare tumour and its imaging characteristics should help in differentiating it from other more common tumours in this location, especially primary hepatic tumours.

Child, Preschool↗
PubMed2004

Lack of effective T-lymphocyte response to the PAX3/FKHR translocation area in alveolar rhabdomyosarcoma.

PURPOSE: Alveolar rhabdomyosarcoma (ARMS) frequently contains the fusion transcription factor PAX3/FKHR. Therefore, clinical studies have been initiated to utilize the PAX3/FKHR translocation point area as a peptide vaccine against ARMS. Our study was directed at identifying antigenic T-lymphocyte epitopes at the PAX3/FKHR translocation point area. EXPERIMENTAL DESIGN: The peptide sequence surrounding the PAX3/FKHR translocation point was evaluated by MHC binding algorithms for potential T-lymphocyte antigenic epitopes (class I molecules HLA-A1, -A2 and -A3; class II molecules HLA-DR1, -DR4 and -DR7). Using in vitro techniques, dendritic cells loaded with PAX3/FKHR peptides were used to stimulate naive T-lymphocytes. T-lymphocyte activity was then evaluated by 51Cr release and 3H-thymidine uptake assays. RESULTS: Only one HLA-A3-restricted epitope was predicted by the algorithms. The peptide was prepared and tested for its ability to stimulate naive cytotoxic T-lymphocytes (CTLs). Unfortunately, the peptide was unsuccessful at stimulating naive CTL. However, induction of naive helper T-lymphocytes (HTL) to recognize and respond to the PAX3/FKHR translocation peptide was successful. Yet, this HTL peptide activity did not translate into recognition of PAX3/FKHR-containing ARMS tumor cells. CONCLUSIONS: It appears that the fusion area of PAX3/FKHR may not be a good source of antigenic anti-tumor peptide epitopes. These results raise serious concerns about the success and applicability of future peptide-based vaccine immunotherapy directed at the PAX3/FKHR translocation point.

Amino Acid Sequence↗
PubMed1995

[Sarcoma botryoides (embryonal rhabdomyosarcoma) as an unusual diagnosis of cervix polyps].

Embryonal rhabdomyosarcomas of the female genital tract usually occur during infancy in the vagina. Only in rare cases can they be found in the cervix, where they are most commonly seen in adolescence. Their prognosis seems to be good since the introduction of a combination of surgery and chemotherapy. We report a case of cervical embryonal rhabdomysarcoma in IRS stage Ia in a 32-year-old female. After surgical resection and chemotherapy there has been no evidence of disease for 7 months. The case is compared with the published literature and prognostically relevant features, and possible problems in the differential diagnosis in biopsies are discussed.

Adult↗
PubMed1999

Cardiac rhabdomyosarcoma: diagnosis by MR imaging.

We report a case of cardiac rhabdomyosarcoma the initial clinical features of which were pericardial effusion, clinical symptoms of congestive heart failure and probable pulmonary thromboembolism, in which echocardiography constituted the first approach to the diagnosis of cardiac tumor and MRI confirmed it, precisely delimiting the tumoral extension and possible infiltration of pericardiac structures. A brief literature review of this entity is given, the MRI findings obtained in our case are described, and we discuss the advantages and limitations of this technique as compared with other alternatives of image diagnosis.

Adult↗
PubMed1999

Metastases to the breast from rhabdomyosarcoma: appearances on MRI.

The authors report a case of blood-borne bilateral metastatic breast disease of alveolar rhabdomyosarcoma (RMS) in a 21-year-old patient. The possibilities of mammography, ultrasound, and MRI in the early detection of breast metastases and their appearance on these modalities are discussed. Whereas mammography rendered no additional information due to dense breast parenchyma and ultrasound showed only a solitary tumor without definite criteria of malignancy, multifocal bilateral spread was verified with MRI and early ring-like enhancement suggested malignancy. Therefore, we conclude that MRI may provide useful information in evaluating patients with sarcomas, even when there is no clinical evidence for metastatic disease of the breast.

Adult↗
PubMed2003

Sentinel lymph node mapping and biopsy: a potentially valuable tool in the management of childhood extremity rhabdomyosarcoma.

Multimodal therapy, involving surgery, chemotherapy and radiation, now dominates the management of rhabdomyosarcoma (RMS) in childhood. Yet, despite improvements in these practices, extremity tumors continue to fare relatively poorly. Several investigators have identified prognostic factors that can be used to direct therapy and predict outcome. These factors include histology and metastatic disease, the latter requiring accurate staging to identify. The presence of lymph node metastases has been shown to be of prognostic significance and is incorporated into pre-treatment staging schemes. Up to 50% of all surgically evaluated nodes and 17% of clinically negative nodes in extremity RMS may harbor tumor, underscoring the increased risk of understaging the disease if accurate lymph node dissection is not undertaken. Despite its importance, there appears to be no standard format by which regional nodal status is evaluated in extremity RMS. Sentinel lymph node mapping and biopsy are a minimally invasive technique, currently used in the staging of adult breast cancer and melanoma. In adults, the technique is associated with optimum nodal yield and low morbidity. We describe a case in which sentinel node mapping and dissection were used to easily and accurately stage a distal upper extremity alveolar RMS in a child with clinically and radiologically negative regional lymph nodes. The procedure yielded no positive nodes, was associated with no morbidity and spared the child more extensive radiotherapy. We propose the further evaluation of this simple and innovative technique in the overall management of this childhood malignancy.

Child↗