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PubMed1979

Primary rhabdomyosarcoma of the cerebrum. An ultrastructural study.

A case of primary cerebral rhabdomyosarcoma in a 51-year-old female is reported. The histogenesis of this tumor is discussed. The clinicopathological features of 10 previously reported similar tumors of the CNS are briefly reviewed. Histologically the tumor was polymorphic, but composed of poorly differentiated cells interpreted as rhabdomyoblasts without definite cross-striation. Electron microscopy established that the poorly differentiated cells were of rhabdomyosarcomatous nature, compatible with presumptive myoblasts and analogous to developing fetal muscle.

Brain Neoplasms↗
PubMed1986

Cellular differentiation and prognosis in embryonal rhabdomyosarcoma. A report from the Cooperative Soft Tissue Sarcoma Study 1981 (CWS 81).

Sixty-four cases of embryonal rhabdomyosarcoma (eRMS) were investigated for cellular differentiation by light microscopy. Of these 64 cases 20 were studied by means of immunohistochemistry. Histologically, three subgroups could be distinguished: primitive (less than 10% rhabdomyoblasts), intermediate (10-50% rhabdomyoblasts) and well differentiated (greater than 50% rhabdomyoblasts) eRMS. Vimentin-positive cells predominated in the primitive eRMS. Intermediate eRMS showed large proportions of desmin-positive cells but vimentin containing cells were also numerous. Myoglobin could only be demonstrated in well differentiated eRMS. Primitive and well differentiated eRMS mainly occurred in the head and neck area, whereas intermediate eRMS were predominantly located in the abdomen. Stage III and IV tumours predominated in cases of primitive eRMS, whereas lower stages were noted in cases of intermediate and well differentiated eRMS. Response to chemotherapy, evaluated after seven weeks of treatment, was achieved in 10/15 (66%) cases of primitive, in 16/19 (84%) cases of intermediate and 5/5 cases of well differentiated eRMS. It is concluded from the current study that the three subgroups of eRMS differ not only by cytological differentiation but also by site of predilection, stage at time of diagnosis and response to chemotherapy.

Abdominal Neoplasms↗
PubMed1993

Modulation of invasive potential in different clonal subpopulations of a rat rhabdomyosarcoma cell line (BA-HAN-1) by differentiation induction.

Three clonal subpopulations (A, B, C) isolated from the same rhabdomyosarcoma of the rat and differing in their degree of spontaneous differentiation were tested for their invasive potential before and after differentiation induction with retinoic acid (RA), N-monomethylformamide (NMF) and sodium butyrate (NaBut). Invasive potential was analysed in an in vitro assay using embryonic chick heart fragments in organotypic culture. In standard culture medium, all three subpopulations were shown to be invasive, progressively replacing the chick heart fragments within 7-11 days after confrontation. After exposure to RA, NMF or NaBut, marked differences in the invasive potential of these subpopulations were, however, observed. Subpopulation C exhibited a pronounced decline in invasive potential, as evidenced by a significant decrease (P = 0.005) in the proportion of chick heart fragments with advanced stages of invasion. This response, however, was confined to the differentiation-inducing agents RA and NaBut, which had also produced a marked increase in morphological and/or biochemical differentiation (P = 0.0001). In contrast, NMF, which had only minor effects on differentiation, failed to affect the invasive potential of subpopulation C. In subpopulation B, a transient inhibition of single cell invasion became evident after exposure to RA, whereas NMF and NaBut failed to affect the invasive potential in spite of minor effects on differentiation. In the least differentiated subpopulation A, which was shown to be refractory to the differentiation-inducing effects of RA, NMF and NaBut, there was also no observation of any reduction of invasive potential. The results of our study demonstrate that differentiation-inducing agents can significantly reduce the invasive potential of malignant tumors, although marked differences of response are to be expected between the different subpopulations of a tumor.

Animals↗
PubMed1992

Non-cytopathic infection of rhabdomyosarcoma cells by coxsackie B5 virus.

Infection of rhabdomyosarcoma (RD) cells by coxsackie B5 virus (CBV5) was non-cytopathic, although low titres of infectious virus were produced after 24 h post-infection. The extent of CBV5 replication in RD cells increased after sequential passage of the virus in these cells. The RD cells from the first cycle of CBV5 infection were recovered and maintained in culture for 3 months (equivalent to 21 passages) releasing infectious virus throughout this period; these cells were considered to be persistently infected with CBV5 and were designated piRD cells. Coxsackie virus antigen was demonstrated in a small proportion of piRD cells by immunofluorescence staining. High resolution two-dimensional polyacrylamide gel electrophoresis was used to analyse the intracellular proteins prepared from piRD cells, three proteins were detected which were absent in uninfected RD cells. These new proteins were similar in charge to virus proteins induced during CBV5 lytic infection of HEp-2 cells. Quantitative densitometry of 2-dimensional protein profiles of piRD and uninfected cells showed no significant disruption of RD cell protein synthesis by the persistent virus infection. Three cloned cell lines were recovered from piRD cells, none of which showed evidence of infectious virus or virus-induced protein synthesis suggesting that the parental cell line was a carrier culture for CBV5.

Antigens, Viral↗
PubMed1988

Studies on human immunodeficiency virus-induced cytopathic effects: use of human rhabdomyosarcoma (RD) cells.

A full-length molecular clone of human immunodeficiency virus (HIV) proviral DNA was transferred to human rhabdomyosarcoma (RD) cells by gene transfer method. RD cells released infectious virus within 12 hours after transfection and the viral particles present in the culture medium could be quantitated by monitoring reverse transcriptase activity. Chronic low level viral producer cell lines of RD were also established. Southern hybridization analysis revealed the presence of HIV sequences in transfected RD cells. Electron microscopic studies of the transfected cell revealed intracellular budding of HIV and also showed structural abnormalities such as giant cell phenotype and vacuolation. These features qualify RD cells as a useful system for studying the regulation and cytopathic effects of HIV.

Cytopathogenic Effect, Viral↗
PubMed1980

Congenitally absent thoracic pedicle in a child with rhabdomyosarcoma.

A case of congenital absence of a thoracic pedicle is reported in a 9 year old boy with embryonal rhabdomyosarcoma of the eyelid. In this clinical setting a surgical exploration was necessary in order to differentiate a developmental anomaly from an acquired metastatic deposit.

Child↗
PubMed1993

Treatment of the rat R-1 rhabdomyosarcoma with methotrexate and radiation; effects of timing on cell survival and tumour growth delay.

The interaction of radiation (10 Gy 300-kV X-rays) and methotrexate (MTX; 3 x 10 mg/kg at 3.5-h intervals) was investigated with respect to effects on cell survival and tumour regrowth of the transplantable rat R-1 rhabdomyosarcoma. The treatment with MTX alone caused acceptable toxicity and no lethality. On day 3 after treatment with MTX alone a maximum decrease in the fraction of clonogenic cells was observed, which is in accordance with data on MTX concentrations in tumour tissue, indicating that MTX is active in the tumour for at least 3 days after injection. The clonogenic capacity after combined treatments, i.e. MTX before or after radiation, was assessed 3 days after the MTX administration. The fractions of clonogenic cells determined after combined therapy with intervals of up to 4 days were not significantly different from those expected on the basis of simple addition of the effects from individual treatments. However, the excess growth delay was positive at specific intervals (6-8 days after X-rays plus MTX and 5-6 days after MTX plus X-rays), whereas negative excess delays were observed when the two treatments were separated by less than 3 days. It is concluded that expectations with respect to clinical application of the combination must be modest in view of the short duration of favourable intervals and the observed absence of synergistic effects with respect to cell killing. The discrepancy between the two assays indicates that erroneous conclusions can be obtained if one endpoint only is assessed.

Animals↗
PubMedDate not supplied

Laminin-mediated adhesion in metastatic rat rhabdomyosarcoma cell lines involves prominent interactions with the laminin E8 fragment.

In vitro attachment assays were carried out to assess adhesion between two basement membrane proteins, type IV collagen and laminin, and rat rhabdomyosarcoma (RMS) cell lines with different metastatic potentials. Whereas cells did not adhere to type IV collagen, adhesion to laminin appeared to be very sensitive as maximal adhesion was achieved in dose-response assays with only nanograms of laminin. Adhesion was mediated by interactions between coated laminin and cell surface components, probably receptors, but not endogenous laminin. Laminin-mediated adhesion of RMS cell lines was compared with that of the MCF-7 (human mammary carcinoma) and the L6 (rat myoblast) cell lines. In dose-response assays, RMS cell lines required 10 times less laminin to reach half-maximal attachment rates than MCF-7 and L6 cell lines. Two laminin fragments, P1 and E8, which are structurally and immunologically distinct as shown by alpha-helix content, SDS-PAGE and monoclonal antibody mapping, supported adhesion by RMS cells and L6 myoblasts, but MCF-7 adhered only to P1. This fragment was 10 times less active than laminin in RMS cell lines. Attachment in dose-response assays and adhesion inhibition studies by antibodies revealed that E8 accounted for the activity of laminin in RMS cell adhesion. Adhesion in the RMS cell lines was dominated by interaction with E8 regardless of metastatic potential.

Animals↗
PubMed1994

Molecular analysis of a patient with Beckwith-Wiedemann syndrome, rhabdomyosarcoma and renal cell carcinoma.

We described a patient with Beckwith-Wiedemann syndrome associated with rhabdomyosarcoma (RMS), and renal cell carcinoma (RCC). Karyotypes of peripheral lymphocytes and RMS cells were normal. DNA analyses showed maternal loss of heterozygosity (LOH) at 11p15 region in RMS but not in RCC. The insulin-like growth factor II gene (IGF2) was found to be expressed at a moderate level in RMS but not in RCC by in situ hybridization. Each of parental allele-derived IGF2 transcript was detected in RCC, while maternal allele-derived transcript was weak in RMS because of maternal LOH. These results suggest that (1) loss of imprinting (LOI) of IGF2 might be responsible for BWS, (2) on the other hand, LOI itself might not induce tumor occurrence in tissues where the control of tissue-specific expression of IGF2 is maintained, (3) increased expression of IGF2 due to maternal loss of a putative controller gene for IGF2 at 11p15 might predispose to sustaining tumorigenic mutations and tumor progression, (4) loss of a putative onco-suppressor gene at 11p15 might induce RMS occurrence. The cause of RCC was thought to be different from that of RMS.

Abdominal Neoplasms↗
PubMed1992

Three pediatric patients with extension of prostatic embryonal rhabdomyosarcoma anterior to the bladder into the space of Retzius.

Invasive embryonal rhabdomyosarcoma of the prostate often extends superiorly into the bladder base requiring partial cystectomy as part of excision of the primary tumor. Three patients were studied with ultrasonography CT and MRI and showed the extension of the tumor superiorly and anterior to the bladder in the prevesical space of Retzius. Complete excision with bladder sparing was successful in two of the patients. The imaging of pelvic tumors in children (although well studied with ultrasonography, CT) is helped by MRI in the sagittal plane and/or lateral films of intravenous pyelograms and cystograms to establish the relation of the tumor to the bladder.

Child↗
PubMed1993

Primary rhabdomyosarcoma of the diaphragm: an unusual cause of adolescent pseudo-achalasia.

We report an adolescent male who presented with progressive dysphagia. Barium examination and upper endoscopy revealed the presence of a smoothly tapered stricture at the lower end of a dilated oesophagus typical of achalasia. Further evaluation by computed tomography (CT), demonstrated the presence of a bulky infiltrating diaphragmatic mass encasing the lower oesophagus. CT guided biopsy of the mass and subsequent post mortem examination revealed the presence of a primary rhabdomyosarcoma of the diaphragm encasing the lower oesophagus. A diagnosis of pseudo-achalasia secondary to a primary diaphragmatic tumour was made.

Child↗
PubMed1995

Unusual course of an epidural rhabdomyosarcoma of the upper thoracic spine.

This report deals with a case of rhabdomyosarcoma in the upper thoracic spine. It is of particular interest, not only for the rarity of type and location of this tumour, but for its clinical course, which presented fluctuations of neurological status, included an acute demonstration of complete paraplegia followed by full recovery after conservative treatment, and gradual relapsing of neurological deficit, one year later.

Combined Modality Therapy↗
PubMed1982

Primary rhabdomyosarcoma of the heart.

A primary rhabdomyosarcoma of the heart observed in a 3-week-old infant, provides an opportunity to review this rare clinical entity; 61 reported cases are collated. The more common sites are right ventricle, left atrium, and right atrium; the tumour may protrude into the cavity and cause obstruction. Echocardiography can demonstrate the tumour, which may also been seen as a filling defect on angiography. Primary heart tumours seldom occur, especially in infancy and childhood. According to Van der Hauwaert, the most common cardiac tumour in infancy is the rhabdomyoma. No primary malignant tumour was seen in 15 European centres within a 5-year period.

Angiocardiography↗
PubMed1987

Ultrasonography and computed tomography for diagnosis and follow-up of biliary duct rhabdomyosarcomas in children.

The authors report eight cases of biliary duct rhabdomyosarcoma in children, examined by US and CT. There were five boys and three girls, aged 2 to 17 years. At presentation, US demonstrates the tumor mass within the liver or the hepatic hilum; it allows measurement of it and defines the relationship with portal vessels, biliary tract and other important structures. CT complements the US evaluation and determines operability. As US and CT cannot assess the histological origin of the tumor, a biopsy is mandatory before treatment. If complete surgical excision does not seem possible, percutaneous biopsy is preferable to incomplete excision and its possible complications. During the follow-up period, US can be repeated to measure tumor regression under chemotherapy. After surgery, CT seems preferable because of gas interposition. Both US and CT proved to be valuable for the early detection of local recurrence. The prognosis of these tumors remains bad. However, with more aggressive and hopefully more efficient chemotherapy a precise evaluation of the tumor extension by US and CT is very important. Surgery will then be performed only on localized tumors or on residual masses after chemotherapy.

Adolescent↗
PubMed1987

Ultrasonography and computed tomography for diagnosis and follow-up of pelvic rhabdomyosarcomas in children.

The authors report 18 cases of pelvic rhabdomyosarcomas in children examined at presentation and followed on US and CT. There were 11 boys, 7 girls; tumor location was bladder, prostate or uterus in 12, perineum in 4, buttock in 2. US is good to define tumoral extension within the bladder CT is better to delineate the extension outside the bladder and regional spread to iliac nodes. CT is the only useful examination for perineal tumors. During chemotherapy US can be frequently repeated to appreciate tumoral regression and to give measurement of the mass. Preoperative CT should be performed when tumoral regression seems sufficient to permit complete tumoral excision without pelvic exenteration. Correlation between US and CT and surgical findings proved to be good. US and CT are also very useful in the follow-up when the child has finished treatment as it can demonstrate a local recurrence before clinical symptoms appear. However, post-surgical and post-radiation changes may be difficult to recognize. These aspects are discussed.

Adolescent↗
PubMed1987

Embryonal rhabdomyosarcoma of the biliary tree in children: a case report.

Embryonal rhabdomyosarcoma (ERS) in children is a very rare tumor. Jaundice is the capital symptom. Diagnosis can be made by echography, which shows echogenic formations without posterior conic shadows in a dilated biliary duct. The prognosis is bleak in spite of the combination of surgery, radiotherapy and chemotherapy.

Bile Duct Neoplasms↗
PubMed1989

CT and ultrasound imaging of pelvic rhabdomyosarcoma in children. A review of 56 patients.

We have retrospectively reviewed the radiographic and clinical features of 56 cases of pelvic rhabdomyosarcoma seen in three Pediatric Hospitals between 1960 and 1986. There were 35 boys and 21 girls. The study aimed at better defining the role played by the various imaging techniques in the investigation of these tumors. The role of diagnostic radiology is the detection and delineation of the primary tumor, its local spread and distant metastases at the time of diagnosis and on follow-ups. Intravenous urography (IVU) and to a lesser extent barium enema (BE) and cystography (VCUG) were used even after the availability of ultrasonography (US) and computed tomography (CT). These two modalities have only partly replaced the traditional radiographic techniques. All patients seen or followed after 1977 (28 patients) had both US and CT examinations. We have focused our discussion on these relatively new imaging modalities namely US and CT. Magnetic resonance was not utilized in any patient in this series. US was by far the best imaging technique available for lesions of the urinary bladder and those invading the bladder wall in children as it was possible to visualize, measure and follow these tumors. Scrotal US was used to confirm the clinical diagnosis of a paratesticular mass. CT showed to best advantage the tumour and its relationship to pelvic organs, musculature and bones.

Adolescent↗