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PubMed1996

The prognostic importance of nuclear morphometry and the MIB-1 index in rhabdomyosarcoma [corrected].

Both nuclear morphometry and an evaluation of the cell proliferative activity have been reported to be useful tools in predicting prognosis in malignant tumors. There have been few reports, however, on rhabdomyosarcoma regarding these evaluative techniques. We performed nuclear morphometry on 51 tumor specimens obtained either by biopsy or by resection of primary and untreated rhabdomyosarcomas, and we then evaluated MIB-1 staining on 25 of the 51 specimens. The morphometric analysis was semiautomatically performed on hematoxylin- and eosin-stained sections by using a personal computer. The areas, perimeters, and lengths of the major and minor axes of the best fitting ellipse of the nuclei were all measured. In addition, the Form Ell (nuclear ellipsoidity score: minor axis/major axis) was calculated. Using the monoclonal MIB-1 antibody, which detects Ki-67 antigen in formalin-fixed, paraffin-embedded tissue, immunohistochemical studies were performed. The values of the major and minor axes and the Form Ell showed significant differences between each histologic subtype whereas the values of nuclear areas did not. We defined the nucleus whose Form ELL was smaller than 0.25 as spindle-shaped nuclei and then divided all cases into two groups on the basis of the ratio of the spindle-shaped nuclei to the total number of nuclei of the tumor cells. The group with the higher ratio showed a significantly better prognosis (P < 0.05). The same trends were also found in the specimens with an embryonal subtype. Nuclear morphometry was a useful tool in predicting prognosis. In particular, the appearance of spindle tumor cells in rhabdomyosarcoma correlated with a better prognosis.

Adolescent↗
PubMed1996

Antisense epidermal growth factor receptor transfection impairs the proliferative ability of human rhabdomyosarcoma cells.

Human rhabdomyosarcoma cells express membrane epidermal growth factor receptor (ECF-R), which could confer responsiveness to EGF and transforming growth factor-alpha (TGF-alpha) of autocrine or paracrine origin. To study the role played by this growth factor circuit in the proliferation and differentiation of myogenic neoplastic cells, human rhabdomyosarcoma EGF-R-expressing cells (RD/18 clone) have been transfected with a plasmid containing a fragment of the EGF-R cDNA in the antisense orientation. In vitro growth and differentiative ability were studied on six antisense-transfected clones (AS) in comparison to parental RD/18 cells and to cells transfected with the plasmid containing only the neomycin resistance gene (NEO). A reduced EGF-R membrane expression was found in AS clones by decreased immunofluorescence with an anti-EGF-R monoclonal antibody. All AS transfectants had a greatly impaired proliferative ability, even when cultured in fetal bovine serum-containing medium. Proliferation of AS clones was completely blocked in medium supplemented with 2% horse serum. The differentiation ability of AS clones was heterogeneous, ranging from clones with a percentage of myosin-positive cells higher than controls to clones with a negligible myosin expression. Therefore, the growth impairment determined by the loop interruption is not sufficient to switch on the differentiation program. The role played by EGF-R in the proliferation of human rhabdomyosarcoma cells suggests that this receptor could constitute a target for a therapeutic approach.

Animals↗
PubMed1996

Low sample volume causes differentiation in human rhabdomyosarcoma cell line RD subjected to electroporation.

Gene transfection has been accomplished with a variety of techniques such as DEAE dextran, calcium phosphate coprecipitation, protoplast fusion, liposomes, microinjection and recombinant bacteriophages. However, transfection by electroporation, consisting of the reversible permeabilization of cell membranes after exposure to a pulsed electric field, has been shown to be the most rapid, simple and efficient method for the stable incorporation of genes in different cell lines. We studied rhabdomyosarcoma cells subjected to electroporation in two different vol. [400 microliters (group 1) and 150 microliters (group 2] of 140 mM NaCl/15 mM Hepes buffer, pH 7.2) and evaluated the effects of electroporation volume on growth and differentiation. Low sample volumes induced a terminal process of morphological and ultrastructural myogenic differentiation in rhabdomyosarcoma cells, which concluded with cell death. Our results suggest that in electroporation low sample vol. of rhabdomyosarcoma cells induced morphological and phenotypic differentiation, with increased expression of desmin, alpha-actinin and tropomyosin.

Actinin↗
PubMed1996

Evaluation of irinotecan in combination with 5-fluorouracil or etoposide in xenograft models of colon adenocarcinoma and rhabdomyosarcoma.

Irinotecan [7-ethyl-10-(4-[1-piperidino]-1-piperidino)-carbonyloxy-camptothec in] administered i.v. in two courses, each course consisting of administration every day for 5 days [(dx5)2] on days 1-5 and 8-12, has demonstrated significant activity against advanced human tumor xenografts derived from colon adenocarcinomas and several childhood cancers. To build on this therapy, we have evaluated the combination of irinotecan given on this schedule with 5-fluorouracil given on days 1, 7, and 14 with or without leucovorin [(dx5)3 i.v.] against colon tumors, or combined with etoposide administered (dx5)2 i.v. either 2 h before or 2 h after irinotecan for treatment of colon tumors and rhabdomyosarcomas. A combination of 5-fluorouracil at 75% and irinotecan at 50% of their respective maximum tolerated doses when administered as single agents on this schedule gave acceptable toxicity. Against colon adenocarcinoma xenografts, 5-fluorouracil did not enhance the response rate compared with that obtained with the optimum dose of irinotecan given as a single agent. Against GC3/TK- xenografts, which lack thymidine kinase and cannot salvage thymidine to circumvent the inhibition of thymidylate synthase, the addition of leucovorin to the combination increased the complete response rate from 10 to >90%, whereas the response rates for the optimal doses of irinotecan or 5-fluorouracil, as single agents, were 30 and <10%, respectively. Etoposide d x 5 i.v. for two or three courses or (d x 5)3 p.o. did not cause objective regression of any colon tumors. In contrast, three of five rhabdomyosarcoma lines demonstrated a high frequency of partial regressions or complete regressions when treated (d x 5)1 i.v. Repetitive courses [e.g., (d x 5)2 or (d x 5)3] i.v. or p.o. or by 4-h infusion d x3 i.v. were either equally effective or less effective. Irinotecan and etoposide were combined using the (d x 5)2 i.v. schedule for both drugs, in which irinotecan was given 2 h before or 2 h after the administration of etoposide. Each drug could be combined at only 38% of its respective maximum tolerated dose when administered as a single agent, indicating greater than additive toxicity. Toxicity was similar irrespective of the sequence of administration and was manifested by loss of weight (73% of the initial weight, nadir day 7), myelosuppression, and prolonged thrombocytopenia. The responses of colon carcinomas to the combination given in either sequence were similar to that achieved with irinotecan given alone at the same dose as used in the combination. Similarly, when etoposide was given before irinotecan, the responses of rhabdomyosarcomas were similar to those for irinotecan. However, in experiments in which etoposide was administered 2 h after each dose of irinotecan, there was significant antagonism of the antitumor activity of irinotecan.

Adenocarcinoma↗
PubMed1998

[Prostatic rhabdomyosarcoma in adults].

OBJECTIVE: To report on two adult patients with prostatic embryonal rhabdomyosarcoma. The literature is briefly reviewed and the clinical, diagnostic and therapeutic aspects of this unusual variety of prostate cancer are discussed. METHODS: Two patients, aged 27 and 34 years, with prostatic embryonal rhabdomyosarcoma are presented. Both cases showed tumor dissemination at the time of diagnosis. Both patients received chemotherapy. RESULTS: A 60% reduction in tumor volume was achieved in one patient, who subsequently underwent rescue surgery and, in spite of a recurrence, is still alive 3 years after the diagnosis. The other patient showed no response to chemotherapy. He refused rescue surgery and was lost to follow-up. CONCLUSION: Embryonal rhabdomyosarcoma of the prostate in the adult is an unusual and aggressive tumor, of rapid growth and progression. Early diagnosis and treatment without delay by radical surgery and chemotherapy are essential to improve the prognosis of this disease.

Adult↗
PubMed1998

Normal and rearranged PAX3 expression in human rhabdomyosarcoma.

PAX3, a member of the PAX-gene family, encodes a nuclear transcription factor that is transiently expressed in the neural tube and in muscle progenitor cells and regulates embryonal development in the mouse. Together with the FKHR gene it is involved in the t(2;13)(q35;q14), a specific translocation associated with alveolar rhabdomyosarcoma (ARMS). As a consequence of the rearrangement two chimeric transcripts originate: FKHR-PAX3 and PAX3-FKHR. We studied the expression of wild type PAX3 and the chimeric transcripts originating from the t(2;13) in a series of 23 rhabdomyosarcomas (RMS) of childhood, by reverse transcriptase polymerase chain reaction (RT-PCR). Wild type PAX3 was detected in 48% of the RMS, whereas another 39% were positive only after nested PCR. Normal adult-skeletal muscle showed a very weak expression of PAX3, but fetal muscle did not express PAX3. PAX3-FKHR was found in 11 of 15 alveolar RMS, 7 of which were positive also for the reciprocal transcript, whereas no RMS expressed FKHR-PAX3 alone. These results confirm that the PAX3-FKHR transcript is specifically associated with the alveolar RMS and that it is a more sensitive marker of the t(2;13) than the reciprocal product FKHR-PAX3. Furthermore, the finding of PAX3 expression with or without PAX3-FKHR transcript in the great majority of the cases raises the question of whether PAX3 expression could play a role in the pathogenesis of RMS.

Adult↗
PubMed2002

Rhabdomyosarcoma of the oral and maxillofacial region in Jordanians: a retrospective analysis.

OBJECTIVE: The objective was to study the clinicopathologic features of rhabdomyosarcoma (RMS) of the oral and maxillofacial region in Jordanians. STUDY DESIGN: Data were collected from records of patients treated between 1989 and 2000 at the Maxillofacial Unit of Jordan University of Science and Technology. The main outcome measures were age, gender, location, stage of disease, histopathologic type, treatment received, follow-up period, and eventual outcome. RESULTS: Nine patients with RMS aged 4 to 17 years were found, with a male to female ratio of 2:1. Six (67%) bony sites and 7 (78%) soft tissue sites were involved. The extent of disease was locoregional in 8 (89%) cases, nodal in 3 (33%) cases, and distant metastatic in 2 (22%) cases. The Intergroup Rhabdomyosarcoma Study classification of clinical groups was as follows: 2 (22%) cases in stage II, 5 (56%) cases in stage III, and 2 (22%) cases in stage IV. The histopathologic types found were 6 (67%) embryonal, 2 (22%) alveolar, and 1 (11%) undifferentiated. Triple agent chemotherapy was used in the treatment of 8 cases, singly or in combination with surgery or radiotherapy. Two patients are still alive, and 5 patients have died of disease. CONCLUSIONS: The clinicopathologic features of maxillofacial RMS in this group of Jordanians are different from those of people from other countries. Further studies are needed to have a better understanding of the behavior of RMS in the oral and maxillofacial area.

Adolescent↗
PubMedDate not supplied

Ultrastructural characterization of poorly differentiated rhabdomyosarcomas.

Twenty-five rhabdomyosarcomas, histopathologically and immunocytochemically classified as embryonal (12 cases), alveolar (11 cases), and pleomorphic (two cases), were studied by routine and immunogold electron microscopy, to identify, in poorly differentiated cells, reproducible markers useful for diagnostic purposes. Rhabdomyoblasts with rudimentary sarcomeres were observed in 12 cases (48%). Large cells (named oval cells) with an oval shape and an eccentric nucleus, like rhabdomyoblasts but lacking organized myofilaments, were observed in 20 cases (80%). By immunogold electron microscopy, performed on four of 25 cases, both oval cells and rhabdomyoblasts showed abundant intermediate filaments positive for desmin. Oval cells may be used as markers of poorly differentiated rhabdomyosarcomas.

Adolescent↗
PubMedDate not supplied

Orbital rhabdomyosarcoma: relationship between DNA ploidy, p53, bcl-2, MDR-1 and Ki67 (MIB1) expression and clinical behavior.

BACKGROUND: As for rhabdomyosarcoma (RMS) of other anatomic regions, the evaluation of traditional clinicopathological parameters does not allow the unequivocal outcome prediction of the single cases of orbital RMS. We investigated the role of DNA ploidy and immunohistochemical expression of p53, bcl-2, MDR-1 and Ki67 (MIB1) in the prognostic evaluation of orbital rhabdomyosarcomas. MATERIALS AND METHODS: The study population consisted of 11 selected cases. Serial sections of each tumor, stained with Feulgen's technique, were analyzed for the DNA content, using the QUANTIMET 500c Leica analyzer, QWINVO200A software. The results were compared with the immunohistochemical expression of p53 (wild plus mutated, W&M and mutated), bcl2, MDR-1 and Ki67 (MIB1), and with follow-up data. RESULTS: The statistical analysis of results showed that the cases of tetraploid and/or multiploid RMS, overexpressing p53 (W&M and mutated) and MDR-1, were characterized by an overall worse prognosis. On the contrary, the tumors with a favourable clinical course showed hyperexpression of MIB1 and absence of mutated p53 expression. Significantly higher MIB1 expression was found in the relapse-free group of tumors, with respect to the RMS with relapse (both in primary tumors and relative relapses, p<0.05). This finding could justify the higher sensibility to pharmacological therapy of RMS of the first group. The group of RMS with a worse prognosis (primary tumors and relapses) showed instead p53 overexpression (W&M and mutated), MDR-1 expression and multiploidy, with high 5cEE values and tetraploid peaks. No significant difference was found concerning the expression of bcl-2 among the two groups of RMS (p>0.05). CONCLUSION: The evaluation of DNA ploidy, p53, MIB1 and MDR-1 expression could be used for subtyping of orbital RMS into two prognostically different subcategories, respectively RMS responder to the therapy, with favourable clinical outcome, and RMS with a worse prognosis, requiring more aggressive therapeutic protocols.

ATP Binding Cassette Transporter, Subfamily B, Mem↗
PubMed1994

[A comparative study of rhabdomyosarcoma with cervicofacial involvement in children and adults. A therapeutic protocol].

Rhabdomyosarcoma is a malignant tumour of striated muscles. It is usually encountered in children and rarely in adults. In this study we compared sex ratios, aetiologic factors, different localizations, development stages, modes of extension and treatment as well as prognosis in children and adults with cervicofacial localizations. The differences observed led to the conclusion that cervicofacial rhabdomyosarcomas in children and adults are two very different pathological entities.

Adult↗
PubMed1997

Rhabdomyosarcoma. Morphologic, immunohistochemical, and DNA study.

In this study 80 cases of predominantly adult rhabdomyosarcoma are presented as follows: 20 cases of the embryonal type, 32 cases of the alveolar and 8 cases of the pleomorphic type. Additional histologic classification was performed in each type. In myotube stage the histologic picture of fetal muscles cannot be compared to the alveolar type of rhabdomyosarcoma. Desmin and sarcomeric actin are observed in 47.7% of all cases, and myoglobin and myosin in only 23.1%. The reactions were diffuse, disperse, or focal. Electron microscopic study subdivides desmin positive tumors into three groups of differentiation. DNA analysis shows that most desmin positive cells are diploid in comparison to all the tumor cells that are hyperdiploid and tetraploid.

Adult↗
PubMed1999

Primary orbital rhabdomyosarcoma in an adult.

PURPOSE. Rhabdomyosarcoma is the most common primary orbital malignant tumor in children. Though rare above the age of twenty, the authors present a case of primary orbital rhabdomyosarcoma in an adult and review the literature. DESIGN. Clinico-pathologic case report. METHODS. A 34-year-old man presented with a recurrent orbital tumor four years after initial excision. This tumor was initially misdiagnosed elsewhere as Merkel cell tumor on the basis of spurious immunohistochemical studies. Excision biopsy of the recurrent orbital mass was performed. MAIN OUTCOME MEASURES. MRI of the orbit and brain was done as well as histopathologic examination and immunohistochemistry of the excised mass. RESULTS. Histopathologic study of the excised orbital tumor revealed rhabdomyosarcoma. CONCLUSION. Careful microscopic evaluation and immunohistochemical stains are important in confirming the diagnosis of atypical cases of orbital tumors.

Journal Article↗
PubMed1992

Retinoic acid induces myogenin synthesis and myogenic differentiation in the rat rhabdomyosarcoma cell line BA-Han-1C.

Two clonal rat rhabdomyosarcoma cell lines BA-Han-1B and BA-Han-1C with different capacities for myogenic differentiation have been examined for the expression of muscle regulatory basic helix-loop-helix (bHLH) proteins of the MyoD family. Whereas cells of the BA-Han-1C subpopulation constitutively expressed MyoD1 and could be induced to differentiate with retinoic acid (RA), BA-Han-1B cells did not express any of the myogenic control factors and appeared to be largely differentiation-defective. Upon induction with RA, BA-Han-1C cells expressed also myogenin, in contrast to BA-Han-1B cells which never activated any of the genes encoding muscle bHLH factors. The onset of myogenin transcription in BA-Han-1C cells required de novo protein synthesis and DNA replication suggesting that RA probably did not act directly on the myogenin gene. Although MyoD1 was expressed in proliferating BA-Han-1C myoblasts, muscle-specific reporter genes were not activated indicating that MyoD was biologically inactive. However, transfections with plasmid expressing additional MyoD1 protein resulted in the transactivation of muscle genes even in the absence of RA. mRNA encoding the negative regulatory HLH protein Id was expressed in proliferating BA-Han-1C cells and disappeared later after RA induction which suggested that it may be involved in the regulation of MyoD1 activity. The myogenic differentiation of malignant rhabdomyosarcoma cells strictly correlated with the activation of the myogenin gene. In fact, stable transfections of BA-Han-1C cells with myogenin expressing plasmids resulted in spontaneous differentiation. Together, our results suggest that the transformed and undifferentiated phenotype of BA-Han-1C rhabdomyosarcoma cells is associated with the inactivation of the myogenic factor MyoD1 as well as lack of myogenin expression. RA alleviates the inhibition of myogenic differentiation, probably by activating MyoD protein and myogenin gene transcription. BA-Han-1B cells did not respond to RA and the differentiated phenotype could not be restored by overexpression of MyoD1 or myogenin.

Animals↗
PubMed1965

Embryonal sarcoma and embryonal rhabdomyosarcoma of the orbit.

A series of 34 cases of embryonal sarcoma and embryonal rhabdomyosarcoma of the orbit has been placed histologically into three groups according to their maximum degree of differentiation at any stage: (a) embryonal sarcoma, (b) non-striated embryonal rhabdomyosarcoma, and (c) striated embryonal rhabdomyosarcoma. The patients were then followed up. This paper presents a summary of the clinical course of the whole series and an account of eight typical cases; the age and sex incidence and survival rates are shown and the histology, treatment, and prognosis are discussed.

Adolescent↗
PubMed1997

Spontaneous Rhabdomyosarcomas in (A/J x CBA/J)Fl Mice.

Spontaneous rhabdomyosarcoma is rare in laboratory rodents. Incidence was estimated as 2.4/100,000 in BALB/c mice; incidence of <0.5% for rodents is generally accepted. We describe spontaneous rhabdomyosarcoma in 2 aged, female ([A/J x CBA/ J]F1) mice. Part of a small breeding colony, these mice were experimentally naive; incidence was estimated as 12.5%. Tumors were located in the muscles of the thoracic wall and muscles of the dorsolumbar region. Cross-striations were detected, using phosphotung- stic acid hematoxylin stain; presence of cross-striations within the neoplasm are diagnostic for rhabdomyosarcoma.

Journal Article↗
PubMed2026

DNA Methylation Profiling of Pediatric Ectomesenchymoma Supports Embryonal Rhabdomyosarcoma-Like Epigenetic Identity.

Ectomesenchymoma is a rare, biphenotypic pediatric tumor combining rhabdomyoblastic and neuroectodermal differentiation. We characterize two novel cases through integrated genomics and the first report of genome-wide DNA methylation profiling. Both tumors harbored RAS-pathway mutations (HRAS p.Gly13Arg; NRAS p.Gln61His). Methylation analysis, including microdissected components, consistently aligned ectomesenchymoma with the embryonal rhabdomyosarcoma superfamily, revealing a shared myogenic epigenetic program despite neural differentiation. Shared copy-number profiles across distinct histological regions supported a monoclonal origin. Overall, our data support a close biological relationship between ectomesenchymoma and embryonal rhabdomyosarcoma and indicate that RAS-pathway testing and methylation profiling can significantly refine diagnostic precision.

Humans↗
PubMedDate not supplied

Extremity rhabdomyosarcoma: biological principles, staging, and treatment.

Rhabdomyosarcoma is the most common soft tissue sarcoma in childhood and adolescence with 20% of the primary tumors anatomically located on extremities. It is a complicated entity that requires careful planning and coordination between the surgical oncologist and other members of the therapeutic team for successful treatment. Even with the most effective regimens more than 50% of patients will die from progressive, usually distant disease. Further progress may require new therapeutic agents or techniques. The surgical oncologist is a necessary and often prominent member of the team. Elements of the biological behavior, histopathology, clinical staging, and treatment of extremity rhabdomyosarcomas occurring in children are discussed. In particular, the importance of the alveolar subtype in determination of prognostic risk as well as new findings regarding the molecular biologic determinants of phenotypic behavior are mentioned. Finally, innovative methods of local control like regional arterial perfusion and rapid intraoperative brachytherapy are addressed.

Child↗
PubMed2004

Continent urinary reconstruction in rhabdomyosarcoma: a new approach.

PURPOSE: The authors present here 5 cases of continent urinary diversion in rhabdomyosarcoma applying a recently described technique for the Mitrofanoff Principle devised by the authors. METHODS: Two previously irradiated rhabdomyosarcoma patients presenting with residual bladder disease and massive sensitive urinary urgency underwent a transverse colonic reservoir with catheterizable stoma. Two other patients presenting with a Bricker conduit underwent conversion into an ileal reservoir. One patient underwent reconstruction after a cystectomy. RESULTS: All patients were continent and able to perform continent intermittent catheterization. CONCLUSIONS: The technique proved to be feasible for this group of patients. The authors believe that because of its simplicity, it should be an option of continent urinary diversion when the Mitrofanoff Principle is considered.

Adult↗