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PubMed2004

The role of nuclear morphometry in prediction of prognosis for rhabdomyosarcoma in children.

AIMS: To determine whether nuclear morphometry can be used in pretreatment diagnostic procedures to guide the therapy of childhood rhabdomyosarcoma (RMS). MATERIALS AND METHODS: Biopsy specimens obtained from 108 patients with rhabdomyosarcoma aged between 1 and 217 months treated in 12 paediatric oncology departments in Poland were evaluated. There were 65 (60.2%) specimens of embryonal rhabdomyosarcoma (RME), 32 (29.6%) of alveolar RMS (RMA) and 11 (10.2%) cases of undifferentiated RMS (RMU). The clinical data from all analysed patients were evaluated. Nuclear morphometry was performed semiautomatically on haematoxylin-eosin-stained sections using the MultiScan v.8.08 Computer Scanning System and an Olympus BX 50 microscope with a x 40 magnification lens. RESULTS: In the RMA subtype cells with spindle-shape nuclei were less common (P = 0.013) and cell nuclei were generally more round in comparison with RME (P = 0.033). The clinical outcome was better if the nuclei seen in biopsies of RMS were more spindle-shaped (event-free survival 0.51 and 0.23, respectively; P = 0.04) or more cells with spindle-shaped nuclei were observed (event-free survival 0.5 and 0.28, P = 0.035). RME patients with small nuclei had a better outcome then patients with large nuclei (P = 0.014). In the RMA/RMU group, patients with small tumour cell nuclei had a worse prognosis than patients with larg tumour cell nuclei (P = 0.046). CONCLUSIONS: Nuclear morphometry is a useful tool in the assessment of children with RMS. Additionally, certain morphometric parameters could be easily applied in a selection of patients with good prognosis.

Biomarkers, Tumor↗
PubMed1987

Actin typing of rhabdomyosarcomas shows the presence of the fetal and adult forms of sarcomeric muscle actin.

We analyzed actin expression in two human rhabdomyosarcomas as well as in three rhabdomyosarcomas induced in rats by the injection of nickel sulfide. All five tumors exhibited appreciable amounts of the sarcomeric alpha-actin types, in line with their myogenic differentiation. The level of these actins was particularly high in the rat tumors, which according to morphological criteria, all showed a higher degree of differentiation than the human tumors. Interestingly, in both human tumors and in two of the three rat tumors, the level of the cardiac alpha-actin type was significantly higher than that of adult skeletal muscle alpha-actin. Taken together with the results of recent reports indicating that the cardiac alpha-actin type is a marker of embryonic and fetal skeletal muscle, our findings indicate that rhabdomyosarcomas express the embryonic sarcomeric actin isoform.

Actins↗
PubMed1981

Morphological changes of myofibril in the carcinogenetic course of 20-methylcholanthrene-induced rhabdomyosarcoma.

In this study 20-methylcholanthrene (MC) was embedded in skeletal muscle tissue of mice. The resulting morphological changes were observed by light and electron microscope until the occurrence of rhabdomyosarcoma. The injected region of the muscle tissue in which MC was embedded went through three stages: destruction and degeneration, repair and appearance of atypical cells. The morphologic figures of the myofibrils seen in the skeletal muscle cell during destruction and degeneration were similar to nonspecific. The muscle tissue then regenerated in the same manner as seen during experimentally induced muscle tissue regeneration in both humans and animals. Abnormal mitotic figures were found 60 to 70 days after the injection of MC. Immature muscle cells, with tuft-formed myofibrils present, underwent abnormal mitosis in which one of the nuclei divided. The rhabdomyosarcoma cells produced by this abnormal mitosis began to appear in the lesion 70 days after the initial embedding of MC. The rhabdomyosarcoma cells in the induced tumor formed undifferentiated myofibrils in various degrees of maturation, and were therefore considered to play a part in the abnormal myofibrillar formation.

Animals↗
PubMed1982

Rhabdomyosarcoma of the right ventricle.

A case of a 66-year-old male with rhabdomyosarcoma of the right ventricle who presented first with a pleuritic chest pain and later developed congestive heart failure with A-V block is described. The total period of the disease was about 9 months. At autopsy the right ventricle and wall were found to be replaced by tumor tissue which was diagnosed histologically as rhabdomyosarcoma of the alveolar type. We described one case and 52 rhabdomyosarcomas of the heart from the literature; the ages ranged from 3 months to 92 years with 29 men and 23 women (unknown 1). The primary lesions were right ventricle 29%, right atrium 26%, left atrium 24%, left ventricle 6%, and others 15%.

Aged↗
PubMed1983

Paratesticular rhabdomyosarcoma.

Two cases of paratesticular rhabdomyosarcoma were reported. One was a 53-year-old male, who was admitted to the Hospital because of swelling of the scrotum, hemosputa, and complete left hemiplegia. Clinical diagnosis was testicular tumor with remote metastases to lung, brain, liver, and para-aortic lymph nodes. At operation, tumor of 340 g of the paratesticular appendage was removed. Pathological findings of the tumor revealed rhabdomyosarcoma of mixed pleomorphic and embryonal types. The other was a 32-year-old male, who was admitted to the Hospital because of left inguinal and scrotal masses. The patient had a history of bruise at the left inguinal region about 5 years prior to admission. An orchiectomy and an excision of the inguinal tumor were performed. Histologically, the tumor was rhabdomyosarcoma of pleomorphic type. Clinical and pathological features of this rare tumor were discussed with a review of literature.

Adult↗
PubMed1987

Exfoliative cytopathology of alveolar rhabdomyosarcoma.

Exfoliative cytopathology of alveolar rhabdomyosarcoma is presented. A 34-year-old male was found to have alveolar rhabdomyosarcoma of the retroperitoneum at autopsy. Ascites was recognized in the course of this disease. Cytological features in the ascites were characterized by relatively small-sized ovoid cells observed singly or in loose clusters. The neoplastic cells possessed small amount of rather vesicular cytoplasm and an eccentric nucleus. The nucleus characteristically revealed multiple deep indentations in the nuclear membrane, a few prominent eosinophilic nucleoli and finely granular or evenly distributed chromatin pattern. These cytological findings, especially the nuclear features, might be useful to differentiate alveolar rhabdomyosarcoma from other neoplasms which consist of relatively small-sized ovoid cells even if the striated muscle origin cannot be confirmed by immunoperoxidase staining.

Adult↗
PubMed1990

Adult embryonal rhabdomyosarcoma of the vagina complicating pregnancy: a case report and review of the literature.

Embryonal rhabdomyosarcoma of the vagina in adults per se is uncommon and pregnancy complicated by such tumors is extremely rare. We wish to report one case of vaginal embryonal rhabdomyosarcoma, diagnosed at the age of 23 years who was carrying at the time of initial presentation. The problem of management of rhabdomyosarcoma in adults, particularly when they are associated with pregnancy is discussed. We advocate the use of multiagent chemotherapy and pelvic radiotherapy in its overall management.

Adult↗
PubMed1989

Expression of the MyoD1 muscle determination gene defines differentiation capability but not tumorigenicity of human rhabdomyosarcomas.

Several human rhabdomyosarcoma cell lines, cultured primary tumor explants, and biopsies of tumor and normal skeletal muscle tissue expressed a 2.0-kilobase transcript that hybridized to the mouse muscle determination gene MyoD1. This transcript was found in tumor cell lines and primary explants that developed multinucleated myotubes but was absent in Wilms' tumors or cell lines and primary explants that developed multinucleated myotubes but was absent in Wilms' tumors or cell lines derived from other mesenchymal tumor cell types. Expression of the human homolog of MyoD1 therefore can define a tumor as a rhabdomyosarcoma. Transfection of the mouse MyoD1 gene into the human rhabdomyosarcoma cell line RD increased the ability of the tumor cells to differentiate into multinucleated myotubes and enhanced myosin heavy-chain gene expression but did not decrease tumorigenicity in nude mice.

Animals↗
PubMed2007

Embryonal rhabdomyosarcoma of the cervix with focal pleomorphic areas.

An embryonal rhabdomyosarcoma (sarcoma botryoides) of the cervix occurring in a 30-year-old woman is described. In addition to typical areas of the embryonal rhabdomyosarcoma, including cartilaginous elements, the neoplasm was characterised by the presence of foci composed of highly pleomorphic cells. The significance of this finding is uncertain. These foci may represent areas of dedifferentiation in an embryonal rhabdomyosarcoma.

Adult↗
PubMed1985

Comparison of beta enolase and myoglobin as histological markers of rhabdomyosarcoma.

A comparative study of beta enolase and myoglobin as markers of muscle differentiation in rhabdomyosarcoma was carried out, using an immunoperoxidase peroxidase antiperoxidase technique. Material from 26 cases of childhood rhabdomyosarcoma was studied and subdivided into embryonal and alveolar types. Positive cytoplasmic staining for beta enolase was seen in 85% of tumours studied (91% alveolar, 79% embryonal), whereas positive staining for myoglobin was detected in only 69% of tumours (82% alveolar, 64% embryonal). beta Enolase and myoglobin are useful in the histological diagnosis of rhabdomyosarcoma, and of the two, beta enolase seems to be the more sensitive.

Cell Differentiation↗
PubMed1990

Rhabdomyosarcomas in the head and neck: MR imaging evaluation.

To determine the typical magnetic resonance (MR) signal intensity characteristics of rhabdomyosarcomas, short repetition time (TR)/short echo time (TE) (T1-weighted) and long TR (proton density and T2-weighted) images of 13 patients with rhabdomyosarcomas of the head and neck were retrospectively reviewed. Seven patients received gadopentetate dimeglumine injections. The most common MR appearance was that of a homogeneous mass, hyperintense to both muscle and fat on long TR/long TE images and isointense or minimally hyperintense to muscle on short TR/short TE images. All lesions of the patients who received gadopentetate dimeglumine enhanced markedly. Two lesions had intratumoral hemorrhage, and six were markedly heterogeneous in signal intensity. Similar MR signal intensity patterns have been described for lymphomas and nasopharyngeal carcinomas. The forte of MR imaging lies in its ability to delineate precisely the extent of the rhabdomyosarcoma.

Child↗
PubMed2000

Nuclear DNA content and chromatin pattern of rat rhabdomyosarcoma cell sublines with different metastatic potentials.

There is a constant need of features able to characterize potentially metastatic cells among the heterogeneous cell subpopulations which constitute a tumor. Image cytometry of metastatic tumor cells give rise to variable results, partly because of a heterogeneous origin of cells, or potential drug effects. The aim of this work was to characterize nuclear changes observed in metastatic cell clones issued in vitro from the same parental cell population The nuclear phenotypes of 6 cell sublines isolated from a rat rhabdomyosarcoma cell line and differing in their metastatic ability were evaluated by image cytometry on Feulgen-stained preparations. Densitometric [5], geometric [3] and textural [9] features were computed from each nuclear image. For each cell subline, a metastatic score, ranging from 0 to 10, was calculated on the basis of in vivo invasivity data, by measuring the number of pulmonary metastases observed after s.c. graft of tumor cells in rats. Data obtained were compared to karyotype, growth characteristics, and oncogene expressions of cell lines. The nuclear DNA content, the chromosome numbers, the cell sublines doubling times, and the distribution of cells within the cell cycle appear unrelated with this score. On the contrary, increase in metastatic ability is accompanied by changes in chromatin pattern as assessed by textural features. Progressive increase in chromatin condensation can be observed in cell sublines with increasing metastatic score. These results were confirmed by an unsupervised multivariate partitioning of rhabdomyosarcoma cells which identified two separate subsets whose distributions within the analyzed cell lines correlate with their metastatic ability. These data suggest that, in rat rhabdomyosarcoma cell sublines, metastatic ability could be associated with nuclear morphological changes at the level of chromatin texture.

Animals↗
PubMed2006

Identification and epitope enhancement of a PAX-FKHR fusion protein breakpoint epitope in alveolar rhabdomyosarcoma cells created by a tumorigenic chromosomal translocation inducing CTL capable of lysing human tumors.

Fusion proteins created by chromosomal translocations in tumors can create neoantigenic determinants at the breakpoint, which are unique to the tumor cells but shared by the vast majority of tumors of that histologic type. If the fusion protein is responsible for the malignant transformation, its expression cannot be lost by the tumor to escape immune responses against this tumor antigen. Here, we identify such a fusion protein breakpoint epitope in the PAX-FKHR fusion protein created by the t(2;13) translocation present in 80% of cases of alveolar rhabdomyosarcoma, a highly aggressive pediatric soft-tissue sarcoma. We use autologous dendritic cells pulsed with the RS10 breakpoint fusion peptide to raise a human CTL line from a normal healthy HLA-B7+ blood donor specific for this peptide. These CTLs are CD8+ (CD4-CD56-) and restricted by HLA-B7. These human peptide-specific CTL lyse human HLA-B7+ rhabdomyosarcoma tumor cells. Therefore, the fusion protein is endogenously processed to produce this natural epitope presented by HLA-B7 and thus this peptide is a bone fide human tumor antigen. We also define a substitution that increases the affinity for HLA-B7 without loss of antigenicity. This epitope-enhanced peptide may serve as a candidate cancer vaccine for HLA-B7+ patients with alveolar rhabdomyosarcoma.

Amino Acid Sequence↗
PubMed2006

Identification of a PAX-FKHR gene expression signature that defines molecular classes and determines the prognosis of alveolar rhabdomyosarcomas.

Alveolar rhabdomyosarcomas (ARMS) are aggressive soft-tissue sarcomas affecting children and young adults. Most ARMS tumors express the PAX3-FKHR or PAX7-FKHR (PAX-FKHR) fusion genes resulting from the t(2;13) or t(1;13) chromosomal translocations, respectively. However, up to 25% of ARMS tumors are fusion negative, making it unclear whether ARMS represent a single disease or multiple clinical and biological entities with a common phenotype. To test to what extent PAX-FKHR determine class and behavior of ARMS, we used oligonucleotide microarray expression profiling on 139 primary rhabdomyosarcoma tumors and an in vitro model. We found that ARMS tumors expressing either PAX-FKHR gene share a common expression profile distinct from fusion-negative ARMS and from the other rhabdomyosarcoma variants. We also observed that PAX-FKHR expression above a minimum level is necessary for the detection of this expression profile. Using an ectopic PAX3-FKHR and PAX7-FKHR expression model, we identified an expression signature regulated by PAX-FKHR that is specific to PAX-FKHR-positive ARMS tumors. Data mining for functional annotations of signature genes suggested a role for PAX-FKHR in regulating ARMS proliferation and differentiation. Cox regression modeling identified a subset of genes within the PAX-FKHR expression signature that segregated ARMS patients into three risk groups with 5-year overall survival estimates of 7%, 48%, and 93%. These prognostic classes were independent of conventional clinical risk factors. Our results show that PAX-FKHR dictate a specific expression signature that helps define the molecular phenotype of PAX-FKHR-positive ARMS tumors and, because it is linked with disease outcome in ARMS patients, determine tumor behavior.

Biomarkers, Tumor↗
PubMed2004

Cyclophosphamide dose intensification during induction therapy for intermediate-risk pediatric rhabdomyosarcoma is feasible but does not improve outcome: a report from the soft tissue sarcoma committee of the children's oncology group.

PURPOSE: More than half of pediatric rhabdomyosarcoma cases have intermediate-risk features and suboptimal outcome (3-year failure-free survival estimates, 55 to 76%). Dose intensification of known active agents may improve outcome. EXPERIMENTAL DESIGN: This pilot study evaluated the feasibility of dose intensification of cyclophosphamide in previously untreated patients ages < 21 years with intermediate-risk rhabdomyosarcoma. Induction therapy comprised four 3-week cycles of VAC: vincristine (V) 1.5 mg/m2 on days 0, 7, and 14; actinomycin D (A) 1.35 mg/m2 on day 0; and dose-intensified cyclophosphamide (C) on days 0, 1, and 2. The three cyclophosphamide dose levels tested were as follows: (a) 1.2 g/m2/dose; (b) 1.5 g/m2/dose; and (c) 1.8 g/m2/dose. Continuation therapy comprised nine additional cycles of VAC with 2.2 g/m2/cycle of C. Radiotherapy was administered at week 0 (parameningeal tumors with intracranial extension) or week 12 or 15 (all others). RESULTS: Between October 1996 and August 1999, 115 eligible patients were enrolled. Three of 15 patients treated at dose level 2 experienced life-threatening dose-limiting toxicity (typhlitis +/- other severe toxicity). Dose level 1 was the maximum-tolerated dose, and 91 evaluable patients were treated at this level. The 3-year failure-free and overall survival estimates for patients treated at the maximum-tolerated dose were 52% (95% confidence interval, 41-64%) and 67% (95% confidence interval, 56-77%), respectively, at a median follow-up of 3 years. CONCLUSIONS: A 64% increase in the standard cyclophosphamide dosage during induction (to 3.6 g/m2/cycle) was tolerated. However, outcomes were similar to those observed at lower dosages, suggesting that alkylator dose intensification does not benefit patients with intermediate-risk rhabdomyosarcoma.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1998

Fluorescent in situ hybridization detection of HER-2/neu gene amplification in rhabdomyosarcoma.

Embryonal rhabdomyosarcoma is the most common malignant soft-tissue tumor in childhood, comprising 45-50% of childhood sarcomas. Cytogenetic studies of this tumor are rare. In view of the paucity of cytogenetic data on this cancer and based on the finding of HER-2/neu gene amplification in a number of cancers that was detected mostly using the traditional technique of immunohistochemistry, we decided to conduct a pilot study to investigate whether HER-2/neu gene amplification in this tumor can be detected using the newer technique of fluorescent in situ hybridization (FISH). Archival tissues of rhabdomyosarcoma were retrieved and FISH using an HER-2/neu probe was undertaken on formalin-fixed paraffin-embedded tissue sections using a protocol optimized for our laboratory at Rhode Island Hospital. Out of 9 cases of rhabdomyosarcoma studied to date, 1 case clearly showed HER-2/neu gene amplification. Thus, FISH is a sensitive technique suitable for the detection of oncogene amplification and the delineation of tumor heterogeneity in this tumor. Future experiments utilizing additional specimens from our centers as well as from other laboratories will be needed to extend the finding in the present pilot study.

Child↗
PubMed1998

Locally recurrent paratesticular rhabdomyosarcoma treated with surgery and radiotherapy.

Paratesticular rhabdomyosarcoma is a highly malignant neoplasm and is exceedingly rare in adults. Thus, its biological behavior is unclear and there is no standard established treatment. The prognosis of recurrent paratesticular rhabdomyosarcoma is dismal in the elderly. Herein we describe a case of locally recurrent paratesticular rhabdomyosarcoma in a 68-year-old patient treated with surgery and radiotherapy.

Aged↗
PubMedDate not supplied

Botryoid rhabdomyosarcoma of the nose: pitfalls of pathology.

A 2-year-old Swiss boy was referred to our hospital because of an obstructive sleep apnea syndrome with suspected recurrent choanal polyp. During surgery, a polypoid soft mass was found in the right nose and extirpated. The histology of the resected polypoid tumor seemed compatible with a polyp; however, immunohistochemistry revealed a botryoid rhabdomyosarcoma. Rhabdomyosarcomas are rare. The most important differential diagnoses for rhabdomyosarcoma are discussed and methods available for differentiating are included.

Child, Preschool↗