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 1,657 records · Page 92Linked to original sources
PubMed2005

Treatment for childhood rhabdomyosarcoma: the cost of cure.

Survival of children and young people with rhabdomyosarcoma has improved substantially during the past 30 years. The improvement can be attributed to the introduction of coordinated multimodality therapy through the efforts of collaborative clinical-trial groups in the USA and Europe. As survival has improved, important late sequelae of treatment have been characterised, and many relate to the local therapy used. Efforts have since been made by some groups to explore ways in which local treatment, particularly radiotherapy, can be omitted for some patients in an attempt to reduce the risk of late sequelae without compromising prospects for cure. An important issue is the overall cost of cure in relation to the treatment received and the need for better selection of patients most likely to benefit from this approach to therapy.

Child↗
PubMed1999

A comparison of MyoD1 and fetal acetylcholine receptor expression in childhood tumors and normal tissues: implications for the molecular diagnosis of minimal disease in rhabdomyosarcomas.

Detection of minimal residual disease or micrometastases in rhabdomyosarcoma (RMS) has been an unresolved problem in 70 to 80% of RMS patients. In patients with alveolar type RMS, which harbors chromosomal translocations and produces tumor-specific fusion products, polymerase chain reaction (PCR)-based diagnosis is clear-cut. In the more frequent embryonal RMS, however, no such PCR-based marker has been described. Recently it has been suggested that the PCR-based detection of MyoD1 may be a valuable adjunct in the diagnosis of minimal disease in embryonal RMS. We report here that MyoD1 mRNA is not specific for RMS, but can be amplified from ex vivo samples of many other childhood tumors and some normal tissues. By contrast, simultaneous amplification of alpha and gamma subunit message of the fetal type acetylcholine receptor (AChR), by a novel duplex PCR, and the quantification of both transcripts resulting in a alpha/gammaAChR ratio <1 was 100% sensitive in alveolar (n = 8) and embryonal (n = 10) RMS. Moreover, gammaAChR was not detected in other childhood (n = 27) or adult tumors (n = 12), or normal tissues, except thymus. The high sensitivity and specificity of the method were confirmed by the successful detection of five cases of cytologically or molecularly verified RMS bone marrow micrometastases among 47 bone marrow samples from childhood tumor patients. By contrast, MyoD1 showed no amplification because of its low level of transcription. We conclude that mRNA of the fetal type AChR is a more specific and (about 100 times) more sensitive marker for the molecular detection of RMS than MyoD1, and thus appears to be a promising candidate for the detection of minimal disease in RMS lacking tumor-specific translocations.

Base Sequence↗
PubMed1978

Rhabdomyosarcoma of the larynx. Report of two cases and a review of the literature.

The rhabdomyosarcoma is a relatively rare tumour form. Two cases in the larynx--a site which is extremely rare--are reported. So far, only 9 cases have been described and documented, and of one of our cases seems to be the first of the alveolar type found at this site. Diagnostic and differential-diagnostic considerations are discussed. Hoarseness is the commonest and earliest symptom. As stated by most authors, the treatment should be radical operation, often with laryngectomy, supplemented by irradiation and chemotherapy.

Adolescent↗
PubMed1985

Rhabdomyosarcoma of soft palate: a case of late relapse.

A patient with pleomorphic rhabdomyosarcoma of the soft palate is reported. The long disease-free interval is due to the success of the combined modalities of treatment and the late relapse is probably related to the biological activity of the tumour.

Adolescent↗
PubMed1988

Rhabdomyosarcoma of the middle ear and mastoid in children.

Three cases of embryonal rhabdomyosarcoma in the middle ear and mastoid in children are presented. Diagnosis was confirmed by histopathology. A multidisciplinary approach employing surgery, chemotherapy and radiation therapy is the method of choice in the management of this rare and highly lethal condition.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1988

Rhabdomyosarcoma of the trachea.

A 12-year-old girl was admitted on two occasions to our department because of severe dyspnoea, necessitating emergency tracheostomy. On the second admission, a lateral neck X-ray demonstrated a globular tumour which was found to be an embryonal rhabdomyosarcoma. The patient received post-operative radiation therapy followed by two years of continued chemotherapy. She is now five years post diagnosis and requiring no further therapy for about three years and her condition remains in remission.

Child↗
PubMed1994

Acute bilateral blindness in childhood caused by rhabdomyosarcoma and malignant lymphoma.

Sudden unilateral visual loss in children is usually caused by a complication of acute purulent sinusitis. Differential diagnosis must include malignant tumours of the orbit or the paranasal sinuses involving the optic nerve. Rapid and adequate diagnostic measures are required in this emergency situation. In comparison, acute or progressive bilateral blindness is a very rare disorder. We report two cases of bilateral visual loss caused by malignant tumours and diagnostic and therapeutic strategies are described. Our reports show that these life-threatening disorders and their severe complications can be managed successfully if well-coordinated interdisciplinary cooperation is conducted from the beginning. If surgery is performed in this vulnerable region, it may be facilitated by computer-aided modelling techniques. During the last decade the prognosis for rhabdomyosarcoma and malignant lymphoma, the most frequent mesenchymal tumours of childhood, has improved dramatically. Thus, any loss of time causing a delay in correct diagnosis and the onset of adequate therapy must be avoided. Intensive cooperation with paediatric oncologists has to be enlisted from the beginning to avoid unnecessary, repetitive, invasive, or imaging diagnostics and to accelerate the onset of specific therapy.

Acute Disease↗
PubMed1984

Rhabdomyosarcoma of the maxillary sinus. A case report.

A rare case of adult rhabdomyosarcoma originating from the left maxillary sinus is reported. A 22-year-old Japanese woman was referred to the Department of Otolaryngology, Hiroshima University, for investigation of the primary source of a metastatic tumor in the bone marrow. An X-ray of the sinuses revealed a tumor in the left maxillary sinus with extension into the nasal cavity, ethmoidal and sphenoidal sinuses. The patient was managed with a chemotherapeutic regimen consisting of high-dose methotrexate, adriamycin, vincristine, cyclophosphamide and prednisolone. After three courses of this chemotherapy, the tumor had decreased in size by about 90 per cent, as indicated by X-rays. The remaining tumor was removed by an extended Denker's operation. Following the surgery another seven courses of chemotherapy were given and during the eight months following the operation the patient has been free from any local recurrence.

Adult↗
PubMed1997

Cerebral rhabdomyosarcoma with rhabdoid tumor-like features.

The present paper describes a case of cerebral neoplasm presenting histological-immunohistochemical characteristics of malignant rhabdoid tumor (MRT) and ultrastructural features of both MRT and rhabdomyosarcoma (R). MRT was first described as an aggressive neoplasm of unknown histogenesis of the kidney, then many other sites of onset were reported, including the central nervous system. However, it has been shown that other tumors of known histogenesis can mimic histologic and ultrastructural features of MRT. On the basis of our findings we agree with authors who support the notion that extrarenal MRT has often a different histogenesis from MRT of kidney, and it probably is a 'phenotypic' entity rather than a distinct pathologic entity.

Brain Neoplasms↗
PubMed1983

Variable susceptibility to NK activity of cloned cell lines derived from a primary rat rhabdomyosarcoma: relationship to metastatic potential.

In vitro cloned lines derived from a primary nickel-induced rat rhabdomyosarcoma exhibited diverse levels of susceptibility to spontaneous NK activity. The presence of NK target structures was revealed by competition assays on all cloned cell lines, and the NK susceptibility of the tumour lines varied according to their osmotic fragility. Tumour cell lines derived from metastatic lung nodules presented similar NK susceptibilities to cells originating from the primary tumour. However, cloned cell lines differed in their capacity to form lung colonies after i.v. injection, and in their potential for invading lungs after s.c. primary tumour development. No correlation was found between lung colonization potential and NK resistance. Studies of the correlation between metastatic potential and NK sensitivity revealed that (1) all the NK resistant tumour cells were highly metastatic; (2) NK susceptible tumour cells could be either highly or weakly metastatic. Therefore, highly metastatic tumour cells could be either resistant or susceptible to NK lysis. We conclude that the property of resistance to NK contributes to a high metastatic potential. However, other properties could counterbalance and finally prevail over NK susceptibility thus enabling NK susceptible cell lines to be also highly metastatic.

Animals↗
PubMed1983

Childhood rhabdomyosarcoma: experience of the Children's Solid Tumour Group.

Seventy three children with rhabdomyosarcoma were treated by members of the Children's Solid Tumour Group during the period, 1974-1981. The extent of disease at diagnosis was found to be the major influence affecting outcome. Children with tumours confined to the tissue of origin with no evidence of nodal or metastatic spread, had a predicted actuarial 5-year survival rate of 86%. However children with 'unconfined' tumours, i.e. those with extension of disease outside the tissue of origin, had a much poorer prognosis with an actuarial 5-year survival rate of only 21%. Two other factors, histological type and site of primary tumour, appeared to affect prognosis but were not independent of the extent of disease at diagnosis. All children were treated according to protocol. Fifty-two patients showed a complete response to initial therapy and 4 of the 11 partial responders achieved a full remission after additional therapy. The overall complete response rate was therefore 77%. Nineteen children who achieved a complete response on initial treatment subsequently relapsed. Only 3 of these children were alive with no evidence of disease 3 years later, a salvage rate of 15%. "Late" relapses, defined as those occurring more than 2 years after diagnosis, were seen in only 5 children, 4 in boys with primary paratesticular tumours.

Adolescent↗
PubMed1986

RMZ: a new cell line from a human alveolar rhabdomyosarcoma. In vitro expression of embryonic myosin.

The RMZ cell line was established from a bone marrow metastasis of a human alveolar rhabdomyosarcoma. Since the beginning of the in vitro culture, RMZ cells showed differentiation-related morphological heterogeneity: actively proliferating polygonal or spindle-shaped cells were observed along with a few multinucleated myotube-like structures and giant cells, frequently multinucleated. All these cell types were still present after over 40 passages. A set of clonal derivatives has been obtained from the second in vitro subculture. All the clones showed the same morphological heterogeneity of the parental cells, but differed from one another in the degree of differentiation. Multinucleated myotube-like structures were strongly stained by anti-desmin antibody; most mononuclear cells were weakly stained. About 80% of RMZ and cloned cells were scored as desmin-positive in cytocentrifuged preparations. The expression of embryonic myosin heavy chain, specifically recognized by the monoclonal antibody BF-G6, was found in RMZ cell line and was localised in the myotube-like structures. Only a few giant cells and rare mononucleated polygonal cells were stained. The average proportion of BF-G6 positive cells in cytocentrifuged preparations was of about 6% of the total RMZ cells. In the two RMZ clones studied, the expression of embryonic myosin was correlated to the proportion of myotube-like structures: a BF-G6 positivity of 35% was found in the most differentiated one.

Cell Count↗
PubMed1987

Evaluation of desmin as a diagnostic and prognostic marker of childhood rhabdomyosarcomas and embryonal sarcomas.

The diagnostic and prognostic relevance of desmin expression in 80 rhabdomyosarcomas (RMS) and 5 embryonal sarcomas (ES) was examined using a peroxidase anti-peroxidase staining procedure. Fifty-nine RMS but only one ES stained for desmin (P less than 0.05). The maximum percentage of desmin containing cells was 49 in RMS compared with only 1% in ES. Desmin positivity correlated inversely with survival (P less than 0.02) in that RMS with high proportions of desmin positive cells were associated with poorer prognoses than those containing fewer desmin positive cells. If the degree of expression of desmin is related to myogenic differentiation, then our results indicate that poorly differentiated RMS tend to have a better prognosis than the well differentiated tumours. One possible explanation is that the poorly differentiated RMS respond better to chemotherapy than to well differentiated RMS. A multivariant analysis incorporating desmin staining, treatment, histology, age and gender revealed that the two most significant independent prognostic factors were treatment and histology.

Desmin↗
PubMed1988

In vivo emergence of a highly metastatic tumour cell line from a rat rhabdomyosarcoma after treatment with an alkylating agent.

Rats bearing a transplanted nickel-induced rhabdomyosarcoma (RMS 9-4/0), treated with chlorozotocin (CZT), an alkylating agent, showed an amplified metastatic invasion of the lung (median of 165 lung tumour nodules, compared to 3 for untreated controls). A higher level of metastatic invasion (200 nodules) was reached spontaneously after the grafting of the S4T line, which was obtained by successive in vivo passages of RMS 9-4/0 cells in CZT treated rats. S4T tumour cells also invaded the liver and a considerable proportion of the lymph nodes. The NT4T line, obtained by successive in vivo passages in untreated rats, showed a lesser degree of enhancement of metastatic capacity (57 nodules). Both derived lines proved to be more aggressive than the parental, proliferated more rapidly, and were resistant to CZT toxicity. Only the non-treated lineage became more resistant to NK lysis. The S4T line lost its myogenic differentiation and was best described as a fibrohistiosarcoma, whereas NT4T did not. Chromosome analysis demonstrated a reduced range of chromosome number per cell in both lines. We conclude that both S4T and NT4T tumours became more metastatic than RMS 9-4/0 as the result of tumour progression through in vivo passages, and that in addition S4T acquired a spontaneously higher metastatic potential, similar to that which occurred in rats grafted with RMS 9-4/0 or NT4T tumours and treated by CZT. This suggests an inheritable mutation in the S4T line.

Animals↗
PubMed1989

Heterogeneous response to differentiation induction with different polar compounds in a clonal rat rhabdomyosarcoma cell line (BA-HAN-1C).

The clonal rat rhabdomyosarcoma cell line BA-HAN-1C was tested for its susceptibility to differentiation induction with different polar compounds. This cell line is composed of proliferating mononuclear tumour cells, some of which spontaneously fuse to form terminally differentiated postmitotic myotube-like giant cells. Exposure of BA-HAN-1C cells to dimethylsulphoxide (DMSO), hexamethylene bisacetamide (HMBA), sodium butyrate (NaBut) and N-monomethylformamide (NMF) resulted in a significant inhibition of proliferation (P less than 0.001) and in a simultaneous increase in differentiation. The response was most pronounced after exposure to NMF as evidenced by a marked increase in the creatine kinase activity used as a biochemical marker of differentiation (P less than 0.05) and the number of terminally differentiated myotube-like giant cells (P less than 0.001). Furthermore, about 5% of the mononuclear cells exhibited thick and thin myofilaments which were never observed in the mononuclear cells of the control. In contrast, the effects of DMSO, HMBA and NaBut were exclusively confined to a significant increase in biochemical differentiation (P less than 0.05), whereas no increase in morphological differentiation was observed and the number of myotube-like giant cells even significantly (P less than 0.001) decreased. This heterogeneous response to differentiation induction with different polar compounds probably indicates different mechanisms of action and suggests that the induction of biochemical differentiation might be independently regulated from events leading to cell fusion and terminal differentiation.

Acetamides↗
PubMed1989

High intrinsic radiosensitivity of a newly established and characterised human embryonal rhabdomyosarcoma cell line.

A new human rhabdomyosarcoma cell line (HX170c) has been established from a paratesticular embryonal tumour in a 5-year-old male. The cells grew as an adherent monolayer with a doubling time of 32 h and showed pleomorphic features. Intermediate filament analysis revealed the line to be mesenchymal in origin (reactivity to vimentin and desmin antibodies). The line was tumorigenic in nude mice, possessed elevated levels of creatine phosphokinase (mainly of the MM isoenzyme form) and had a near diploid mean chromosome number of 50. In vitro cell cloning determinations gave colony forming efficiencies of 0.01% in soft agar and 24% in a monolayer anchorage-dependent assay. Radiosensitivity determinations using a monolayer clonogenic assay with feeder layer support showed the cells to be among the more radiosensitive human tumour cell types (surviving fraction at 2 Gy of 0.26) that have been investigated. Furthermore, experiments utilising continuous low dose rate radiation at 3.2 cGy min-1, showed that, under these experimental conditions, the cells possessed only a very low capacity to recover from radiation-induced damage (dose reduction factor at 1% cell survival of 1.07 for 150 versus 3.2 cGy min-1). As other human tumour cells of an embryonal cell origin (e.g. neuroblastoma and germ cell tumours of the testis) have also been shown to be radiosensitive it appears that sensitivity to radiation may be a common property of this group of tumours.

Animals↗
PubMed1992

Induction of myogenic differentiation in human rhabdomyosarcoma cells by ionising radiation, N,N-dimethylformamide and their combination.

Differentiation-inducing ability of gamma-radiation, N,N-dimethylformamide and their combination has been tested on human rhabdomyosarcoma RMZ-RC2 clone cells. Ionising radiation at 2-5 Gy doses induced a more differentiated morphology, with the appearance of an increased proportion of multinuclear myotube-like cells, and a significant increase in myosin-positive and multinuclear cells. Radiation appeared to act by inducing de novo differentiated elements. N,N-dimethylformamide was able to induce an increased myosin expression, but did not affect multinuclear cell proportion. The combined treatment (ionising radiation and N,N-dimethylformamide) resulted in an additive increase in the proportion of myosin-positive cells, approaching 25-35%, but de novo differentiated elements were not increased above the levels obtained with irradiation alone.

Cell Differentiation↗