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PubMed1980

Clinicomorphologic markers for predicting behavior and guiding therapy for brain tumors.

More specific therapy can be achieved for brain tumors by identifying morphologic markers like primitive neuroectodermal tumor cells that tend to be radioresponsive and spread diffusely through the cerebrospinal fluid (CSF). Utilizing clinical manifestations to suspect and then localize the brain tumor, morphologic markers, that can be appreciated with computerized tomography and cerebral angiography as well as at operation, provide a basis for more aggressive extirpation and the usefulness of radiation and chemotherapy. Metastatic neoplasms possess such markers as tumor hemorrhage and meningeal involvement suggesting specific extraneural sources. In almost half of these patients, cytologic examination of the CSF should help to identify the malignant cells. Although most gliomas do not metastasize either within or outside the central nervous system, the occurrence of primitive neuroectodermal tumor cells (undifferentiated malignant small cells) in about 20% of all glioblastomas implies spread throughout the CSF and some radioresponsiveness. With thorough clinicomorphologic evaluations of all patients with brain tumors in concert with liberal clinical consultations, the aggressive, newer therapeutic modalities can be used more effectively.

Aged↗
PubMed2006

Central nervous system tumors in patients under three years of age: treatment results of a single institute.

Eighty-six patients under 3 years of age with central nervous system tumors were retrospectively analyzed between 1972 and 2003. Surgical resection was done in all patients except for those with optic glioma, pons glioma and pineal tumor. Three different chemotherapy regimens were used in different time periods. In 48 patients, the tumor was located in the posterior fossa, and 29 patients had a supratentorial tumor. We had 32 (37.2%) patients with embryonic tumors (21 medulloblastoma, 4 ependymoblastoma, 5 with atypical teratoid rhabdoid and 2 with supratentorial primitive neuroectodermal tumors), 21 (24.4%) with ependymoma, 14 (16.3%) with optic glioma, 10 (11.6%) with astrocytoma, 3 (3.5%) with pons glioma and 6 (7.0%) with others. Overall (OS) and event-free survival rates were 49.5 and 40.9%. OS rates according to the tumor localizations were 40.9 and 68.1% in the posterior fossa and supratentorial localizations, respectively (p=0.001). OS rates were 33.7, 41.3 and 88.8% for the medulloblastoma+primitive neuroectodermal tumor groups, ependymoma and astrocytoma, respectively (p=0.0001). Most of the patients had primitive embryonic tumors (37.2%). The best prognostic factors were tumor localization and histology.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1992

Cytogenetic analysis of 39 pediatric central nervous system tumors.

Consistent cytogenetic abnormalities have been described in many pediatric solid tumors, including Ewing's sarcoma, Wilms' tumor, and neuroblastoma. Similar analysis of pediatric central nervous system (CNS) tumors has been hampered by technical problems. We report chromosome results from 39 pediatric CNS tumors. Abnormalities of chromosome 17 were noted in 3 of 11 primitive neuroectodermal tumors (including i(17q) in 2 tumors), confirming data observed by other investigators. Cells from 2 of 11 primitive neuroectodermal tumors (PNET) exhibited loss or structural abnormalities involving chromosome 11. Loss or distal deletion of chromosome 7q was noted in cells from two PNETs. Because other investigators have shown loss of heterozygosity on 17p in about one-third of PNET, we propose that chromosome regions 7q and 11 are areas worthy of further study in pediatric PNET. Numerical abnormalities were noted in 6 of 21 astrocytomas. Hyperdiploidy was demonstrated in 1 of 4 pilocytic astrocytomas and pseudopolyploidy was demonstrated in 4 of 13 anaplastic astrocytomas. Structural chromosome abnormalities (translocations, deletions) were noted in 4 of 13 anaplastic astrocytomas. Complex structural anomalies were observed in one craniopharyngioma. A rhabdoid tumor of the brain exhibited multiple complex structural rearrangements but did not exhibit the monosomy 22 observed in some rhabdoid tumors. Hypodiploidy and loss of chromosome 22 were noted in a clinically aggressive meningioma, corroborating observations by other investigators.

Astrocytoma↗
PubMed2002

[Renal tumors in adults: rare tumors and new tumor entities].

International agreement was reached on the histologic classification of renal epithelial neoplasms during the last years. This classification defines malignant neoplasms as clear-cell (conventional) renal carcinoma, papillary renal carcinoma, chromophobe renal carcinoma, collecting duct carcinoma and renal cell carcinoma, unclassified. Benign neoplasms are papillary adenoma, renal oncocytoma and metanephric nephroadenoma/adenofibroma. Over the past few years, new or rare distinctive kidney tumors have been described. The aim of this review is to present examples of recently recognized clinicopathologic tumor entites and to discuss the value of immunohistochemical and molecular tests for the differential diagnosis. The following tumors will be described: mixed epithelial/stromal renal tumors, primary renal synovial sarcomas, primary renal primitive neuroectodermal tumors, low grade myxoid renal epithelial neoplasms with distal nephron differentiation and epitheloid angiomyolipoma. Detection of SYT-SSX gene fusion transcripts resulting from the t(X;18) and the EWS-FLI-1 gene fusion are described as molecular tests for the diagnosis of renal synovial sarcomas and renal primitive neuroectodermal tumors. Immunohistochemical expression of hormone receptor is helpful to diagnose mixed epithelial/stromal renal tumors. It is important to distinguish these tumor entities from adult Wilms' tumors and sarcomatoid renal cell carcinomas because of a different biological behaviour and different therapeutical approaches.

Adult↗
PubMed1991

Ewing's sarcoma.

Ewing's sarcoma and primitive neuroectodermal tumor are rare tumors of childhood, both considered to be small round blue cell tumors of childhood. Recent advances have been made regarding the histogenesis and biology of these tumors. Current thinking links Ewing's sarcoma and primitive neuroectodermal tumor by a shared chromosomal translocation and by evidence of neuroectodermal origin. Advances in chemotherapy have resulted in the cure of most patients presenting with Ewing's sarcoma. This review briefly outlines the year's literature on the histogenesis, biology, and treatment of this tumor.

Adolescent↗
PubMed2003

Medulloblastoma.

Medulloblastomas, primitive neuroectodermal tumors of the cerebellum, comprise 20% of all pediatric brain tumors and are the most common solid neoplasm in children. Primitive neuroectodermal tumors are believed to arise from cerebellar granule cell precursors. Occasionally, medulloblastoma occurs in children with genetically linked disorders, such as Turcot's syndrome or Gorlin's syndrome, which is also called basal cell nevus syndrome. Several genes have been implicated in the development of medulloblastoma in children, including Patched-1 and Smoothened. The protein products of these genes function within the sonic hedgehog molecular signaling pathways, which are important in neural development and disease. Through analysis of several well-designed multi-institutional trials, much has been learned about the clinical factors that influence outcome in children with medulloblastomas. Age younger than 3 years, bulky residual disease postoperatively, and metastasis constitute adverse prognostic features and indicate patients who are considered "high risk" for recurrence with standard therapy using 3600 cGy craniospinal radiation in conjunction with a posterior fossa dose of 5400 cGy. Patients lacking these features are considered "standard risk." Chemotherapeutic trials have been developed to assess the safety and efficacy of various multi-agent therapies to improve the poor results of high-risk patients and to allow reduction in the dose of radiation needed to cure standard-risk patients, which may allow a decrease in late cognitive sequelae. Currently, it is policy to evaluate all children with posterior fossa tumors characteristic of medulloblastoma with preoperative, staging neuroimaging studies of the craniospinal axis. Surgical resection is undertaken with the goal of gross total resection. Postoperative neuroimaging studies are compared with preoperative studies to determine the amount of residual disease. Cerebrospinal fluid is obtained from a lumbar puncture done at the conclusion of the surgical resection or 2 weeks after surgery in order to determine microscopic leptomeningeal spread. Children with tumor histopathology consistent with medulloblastoma are enrolled, when possible, in open clinical trials. Chemotherapy and radiation are given as per protocol. The goal of current treatment approaches is to tailor therapy based on clinical risk factors, with intensification of treatment for children with high-risk disease and reduction of radiation therapy for those with standard-risk disease. Evaluation of biologic predictors of outcome, which may further refine treatment stratification, is in progress.

Journal Article↗
PubMed1998

Farm and animal exposures and pediatric brain tumors: results from the United States West Coast Childhood Brain Tumor Study.

Nineteen counties from San Francisco and Los Angeles, California and Seattle, Washington were the United States sites for a large population-based case-control study of childhood brain tumors (CBTs), sponsored by the National Cancer Institute. CBT patients who were < 20 years of age and were diagnosed between 1984 and 1991 were reported to each region's cancer registry. The 801 control subjects were obtained by random digit dial and were frequency-matched to the 540 CBT patients in San Francisco and Seattle (one patient to two controls) and in Los Angeles (one patient to one control). Data collected by in-person interview with subjects' mothers were analyzed to investigate an association between risk for CBTs and life on a farm, exposure to farm animals (dairy cattle, beef cattle, pigs, sheep/goats, poultry, and horses), and some cat and non-farm horse exposures. Elevated risks for CBTs were observed in association with mothers' exposure to pigs [odds ratio (OR) = 3.8, 95% confidence interval (CI) = 1.2-12] and horses (OR = 2.2, 95% CI = 1.0-4.8) on a farm during the index pregnancy. Children diagnosed with primitive neuroectodermal tumors showed elevated risks for CBTs with personal and maternal prenatal exposure to pigs (child, OR = 4.0, 95% CI = 1.2-13; mother, OR = 11.9, 95% CI = 2.8-51) and poultry (child, OR = 3.0, 95% CI = 1.1-8.0; mother, OR = 4.0, 95% CI = 1.2-14). No other animal exposures of children or mothers were found to be consistently related to CBTs. Children diagnosed with primitive neuroectodermal tumors who were on a farm for > 1 year and were first on a farm when they were < 6 months of age also had increased risk for CBTs (OR = 3.9, 95% CI = 1.2-13). A somewhat increased risk for CBTs was found for children of mothers who ever had worked on livestock farms compared with mothers who never had worked on a farm (OR = 7.4, 95% CI = 0.86-64, based on five case mothers and one control mother who worked on livestock farms during the 5 years preceding the birth of the index child). The associations are consistent with those of two previous studies in Norway (P. Kristensen et al., Int. J. Cancer, 65: 39-50, 1996) and the United States and Canada (G. R. Bunin et al., Cancer Epidemiol. Biomark. Prev., 3: 197-204, 1994) that investigated the role of farm-related exposures in the etiology of CBTs.

Adolescent↗
PubMed2000

Tumor-derived p53 mutant C174Y is a gain-of-function mutant which activates the fos promoter and enhances colony formation.

SV40 large T antigen-induced primitive neuroectodermal tumors of the rat provide a model system to study induction and progression of primitive neuroectodermal tumors at the molecular level. A cell line derived from such a tumor reproducibly gave rise to malignant derivatives that ceased large T-antigen expression but harbored a mutant p53 allele with a common mutation at Cys(174) to Tyr (C174Y). To determine whether this p53 mutation contributes to tumor progression, we analyzed mutant C174Y functionally. Co-transfection experiments in Saos-2 cells with mutant or wild-type p53 and reporter genes linked to various p53-responsive promoters revealed that mutant C174Y failed to transcriptionally transactivate the Mdm2, Waf1, Cyclin G and Bax promoters. Loss of transcriptional activation correlated with loss of DNA-binding activity. Moreover, mutant C174Y exhibited a dominant negative effect on co-expressed wild-type p53. The ability of mutant p53 to repress the viral RSV, LTR or SV40 early promoters or the cellular fos promoter was likewise impaired. In contrast, it showed even induction of the fos promoter. Consistent with these observations, mutant C174Y was non-functional in the suppression of Saos-2 cell growth and even conferred a growth advantage to the cells. Surprisingly, mutant C174Y was also impaired in nuclear transport, as revealed by immunofluorescence analyses. Taken together, our results demonstrate that mutant C174Y possesses features that can positively contribute to cancer progression.

Amino Acid Substitution↗
PubMed1985

Leptomeningeal dissemination of primary central nervous system tumors of childhood.

Presymptomatic craniospinal radiation therapy improves the rate of survival for children with brain tumors, which frequently metastasize to the leptomeninges. Radiotherapy may cause neurological damage and should be used only in patients considered to be at highest risk for leptomeningeal dissemination (LMS) at either the time of initial diagnosis or onset of disease relapse. We reviewed 314 consecutive patients with brain tumors to determine the incidence, timing, and importance of LMS. LMS occurred in 60 (19%) children. LMS occurred before diagnosis in 30 patients, as the only site of relapse or simultaneously with local first disease recurrence in 17 patients, and after local disease recurrence in 13 patients. Children with primitive neuroectodermal tumors, anaplastic gliomas, and ependymomas most frequently had LMS. Patients with primitive neuroectodermal tumors and posterior fossa anaplastic gliomas frequently had LMS before diagnosis or at the onset of relapse, whereas patients with ependymomas had LMS after local disease relapse. Both myelography and cerebrospinal fluid cytological examination are required to diagnose LMS.

Arachnoid↗
PubMed1987

PCNU and recurrent childhood brain tumors.

PCNU, the latest nitrosourea analogue to be subjected to clinical trials, held promise as a superior chemotherapy agent for brain tumors because of more favorable biochemical and cytotoxic characteristics in laboratory studies. Thirty-nine children with a variety of recurrent primary CNS tumors, all of whom had evaluable disease, participated in a phase II PCNU trial. Their mean age was 9.7 (3-20) years. PCNU was administered as a 2 hour intravenous infusion in one of 2 dose schedules at 6-7 week intervals; 100-125 mg/m2 for minimally treated patients and 70-90 mg/m2 for heavily treated patients. Response was assessed after 2 courses of chemotherapy after attempting to taper the steroid dose. The overall objective response rate was 18% (7/39) for a mean of 5.9 months (2+ -12). Only partial responses were observed. Disease-specific responses rates were: brainstem glioma--18% (3/17); cerebral glioma--27% (3/12); ependymoma--1/1; and primitive neuroectodermal tumors--(0/9) including 5 medulloblastomas, 2 pineoblastomas and 3 cerebral primitive neuroectodermal tumors. Toxicity was primarily hematologic and clinically significant thrombocytopenia (less than 50,000 mm3) was encountered in 30/38 (79%) patient trials. Modest activity of PCNU in recurrent childhood gliomas is confirmed. Our response rates, using objective CT criteria, are somewhat lower than those reported for BCNU and CCNU. Because of comparable hematologic toxicity and efficacy, intravenous PCNU does not appear to offer a clinical advantage to existing nitrosoureas for children with recurrent brain tumors using a 2 hour intravenous infusion schedule.

Adolescent↗
PubMed1993

The Ewing's sarcoma EWS/FLI-1 fusion gene encodes a more potent transcriptional activator and is a more powerful transforming gene than FLI-1.

EWS/FLI-1 is a chimeric protein formed by a tumor-specific 11;22 translocation found in both Ewing's sarcoma and primitive neuroectodermal tumor of childhood. EWS/FLI-1 has been shown to be a potent transforming gene, suggesting that it plays an important role in the genesis of these human tumors. We now demonstrate that EWS/FLI-1 has the characteristics of an aberrant transcription factor. Subcellular fractionation experiments localized the EWS/FLI-1 protein to the nucleus of primitive neuroectodermal tumor cells. EWS/FLI-1 specifically bound in vitro an ets-2 consensus sequence similarly to normal FLI-1. When coupled to a GAL4 DNA-binding domain, the amino-terminal EWS/FLI-1 region was a much more potent transcriptional activator than the corresponding amino-terminal domain of FLI-1. Finally, EWS/FLI-1 efficiently transformed NIH 3T3 cells, but FLI-1 did not. These data suggest that EWS/FLI-1, functioning as a transcription factor, leads to a phenotype dramatically different from that of cells expressing FLI-1. EWS/FLI-1 could disrupt normal growth and differentiation either by more efficiently activating FLI-1 target genes or by inappropriately modulating genes normally not responsive to FLI-1.

3T3 Cells↗
PubMed1997

An intra-abdominal small round cell neoplasm with features of primitive neuroectodermal and desmoplastic round cell tumor and a EWS/FLI-1 fusion transcript.

We report an intra-abdominal round cell tumor in a young man which exhibited the light and electron microscopic appearance of a peripheral primitive neuroectodermal tumor (PNET), in addition to the clinical and topographic characteristics, desmoplasia and a complex immunophenotypic profile of the intra-abdominal desmoplastic round cell tumor (DSRCT). Reverse transcription polymerase chain reaction revealed a EWS/FLI-1 fusion transcript as in PNET/Ewing's sarcoma, instead of the EWS/WT1 transcript of DSRCT. The tumor was also strongly positive for the mic2 protein. This is a unique case of a hybrid tumor arising in the peritoneal cavity of a young male. The existence of such a hybrid tumor in this location suggests that DSRCT and PNET may be related and possibly share a common histogenesis.

Abdominal Neoplasms↗
PubMed1998

Intraabdominal desmoplastic small round cell tumor with EWS/ERG fusion transcript.

This report describes an intraabdominal small cell tumor in a 37-year-old woman, with clinical, topographic, and morphologic features highly suggestive of the desmoplastic small round cell tumor. Immunohistochemical analysis revealed a polyphenotypic profile consistent with this tumor--positivity for keratin, epithelial membrane antigen, neuron-specific enolase, vimentin, and desmin--but, in addition, a strong membranous immunoreactivity for CD99 (MIC2 protein). Reverse transcription polymerase chain reaction revealed a EWS/ERG fusion transcript characteristic of the Ewing's sarcoma/peripheral primitive neuroectodermal tumor group of tumors, rather than the EWS/WT1 chimeric transcript typical of the desmoplastic small round cell tumor. This is the third report of a hybrid tumor with features of the desmoplastic small round cell tumor and Ewing's sarcoma/peripheral primitive neuroectodermal tumor, and the first one with the EWS/ERG fusion gene. Our case shows the existence of some overlap between these two groups of tumors, which are considered to be histogenetically different, and the need for further studies of molecular characterization of small cell tumors, especially in those with atypical morphologic or immunohistochemical features.

Abdominal Neoplasms↗
PubMed2005

Temporally regulated neural crest transcription factors distinguish neuroectodermal tumors of varying malignancy and differentiation.

Neuroectodermal tumor cells, like neural crest (NC) cells, are pluripotent, proliferative, and migratory. We tested the hypothesis that genetic programs essential to NC development are activated in neuroectodermal tumors. We examined the expression of transcription factors PAX3, PAX7, AP-2alpha, and SOX10 in human embryos and neuroectodermal tumors: neurofibroma, schwannoma, neuroblastoma, malignant nerve sheath tumor, melanoma, medulloblastoma, supratentorial primitive neuroectodermal tumor, and Ewing's sarcoma. We also examined the expression of P0, ERBB3, and STX, targets of SOX10, AP-2alpha, and PAX3, respectively. PAX3, AP-2alpha, and SOX10 were expressed sequentially in human NC development, whereas PAX7 was restricted to mesoderm. Tumors expressed PAX3, AP-2alpha, SOX10, and PAX7 in specific combinations. SOX10 and AP-2alpha were expressed in relatively differentiated neoplasms. The early NC marker, PAX3, and its homologue, PAX7, were detected in poorly differentiated tumors and tumors with malignant potential. Expression of NC transcription factors and target genes correlated. Transcription factors essential to NC development are thus present in neuroectodermal tumors. Correlation of specific NC transcription factors with phenotype, and with expression of specific downstream genes, provides evidence that these transcription factors actively influence gene expression and tumor behavior. These findings suggest that PAX3, PAX7, AP-2alpha, and SOX10 are potential markers of prognosis and targets for therapeutic intervention.

Biomarkers, Tumor↗
PubMed1993

Detection of the (11;22)(q24;q12) translocation of Ewing's sarcoma and peripheral neuroectodermal tumor by reverse transcription polymerase chain reaction.

Ewing's sarcoma and the related primitive neuroectodermal tumor (PNET) share a unique and specific t(11;22)(q24;q12) chromosomal translocation. The breakpoints have recently been cloned and shown to involve the EWS gene on chromosome 22 and the FLI-1 gene on chromosome 11. Translocation results in the fusion of these genes on the der(22) chromosome, resulting in the production of a novel chimeric EWS/FLI-1 message. Using oligonucleotide primers derived from EWS and FLI-1 complementary DNAs, we were able to amplify a specific fusion transcript from 18 of 18 cases containing t(11;22) and 10 of 14 cases of Ewing's sarcoma/PNET that had unsuccessful cytogenetics. No EWS/FLI-1 fusion transcripts were detected in five cell lines derived from cases of pediatric sarcomas having a histological diagnosis other than Ewing's sarcoma/PNET. The sensitivity and specificity of this PCR analysis demonstrates the usefulness of this approach for the primary diagnosis of t(11;22)-containing Ewing's sarcoma/PNET and for the detection of metastatic or residual disease.

Amino Acid Sequence↗
PubMed2004

Male breast disorders in Jordan. Disease patterns and management problems.

OBJECTIVE: To highlight the features and management problems of male breast disorders in an eastern country such as Jordan. METHODS: Data regarding 33 male patients who underwent surgery for breast diseases in the Department of Surgery at Jordan University of Science and Technology, Irbid, Jordan, between the year 1996 and 2002 were analyzed. RESULTS: Gynecomastia (45.5%), ductal carcinoma (18.2%), and lipoma (12.1%) were the most frequent lesions. Endocrine testing when the clinical diagnosis was physiologic gynecomastia was not yielding. Many features of male breast carcinoma in Jordan (symptomatology, male to female ratio, high education rate, age, diagnostic yield of fine-needle aspiration, histological type, and estrogen receptor status) did not depart from the experience of others. Delayed presentation is evident from the fact that 57% of tumors were stage III. Loco-regional control was achieved by modified radical mastectomy. Tamoxifen was used in 5 patients, and chemotherapy in 4 patients. The patient with stage I is still disease free 6 years after the diagnosis. The 5-year survival rate for stages II and III was zero. Rare lesions (cystic hygroma, cystic mastopathy, fibroadenoma, duct papilloma, tuberculosis, periductal mastitis, and the previously unreported primary primitive neuroectodermal tumor of the breast) accounted for the rest of the group. The patient with primary primitive neuroectodermal tumor of right breast was treated by mastectomy and adjuvant chemotherapy. He remains disease free 31 months after the diagnosis. CONCLUSION: Unawareness and the fact that male breast enlargement is considered a social stigmata are responsible for the delayed presentation. The value of fine needle aspiration cytology and mammography is not widely appreciated. The wide spectrum of potential pathologies calls for referring all patients to specialized breast units.

Adolescent↗
PubMed1990

Insulin-like growth factor I expression by tumors of neuroectodermal origin with the t(11;22) chromosomal translocation. A potential autocrine growth factor.

Expression of insulin-like growth factor I (IGF-I) mRNA by some tumor cell lines of neuroectodermal origin has been described. To further explore the significance of IGF-I mRNA expression in these tumors, a more extensive analysis was performed. Most (9 of 10) neuroectodermal tumor cell lines with a t(11;22) translocation (primitive neuroectodermal tumor [PNET], Ewing's sarcoma, esthesioneuroblastoma) expressed IGF-I mRNA, whereas 0 of 15 cell lines without the translocation (PNET, neuroblastoma) expressed IGF-I. Furthermore, inasmuch as all neuroblastoma (12 of 12) cell lines examined expressed IGF-II RNA, the pattern of IGF expression could distinguish between these closely related tumors. CHP-100, a PNET cell line with the t(11;22) translocation, was shown to secrete both IGF-I protein and an IGF binding protein, IGFBP-2. This cell line also expressed the type I IGF receptor mRNA, and blockade of this receptor by a monoclonal antibody (alpha IR3) inhibited serum-free growth. These data demonstrate that IGF-I expression is a property of neuroectodermal tumors with a t(11;22) translocation and that interruption of an IGF-I autocrine loop inhibits the growth of these tumor cells.

Cell Division↗
PubMed1997

[Pathologic diagnosis on bone and soft tissue tumors by molecular biological methods].

Characteristic chromosome aberrations and the rearranged genes resulting in chimeric fusion genes have been reported in some bone and soft tissue tumors; t(X; 18) in synovial sarcoma, t(11; 22) in Ewing's sarcoma and primitive neuroectodermal tumor, and t(2; 13) in alveolar rhabdomyosarcoma. We practically used the chromosome analysis and the reverse transcription-polymerase chain reaction (PCR) method as a tool for diagnosis and follow up. All of 10 cases of synovial sarcoma had a chimeric product of SYT/SSX gene. Eleven cases of Ewing's sarcoma and primitive neuroectodermal tumor showed 6 variants of chimeric products between EWS gene and Fli1 gene in the PCR-directed sequence analysis. Although PAX3/FKHD or PAX7/FKHD transcripts were amplified in alveolar rhabdomyosarcoma cases, MyoD1 and myogenin gene which are myogenic transcription factor were also expressed in most rhabdomyosarcomas. These findings indicate that molecular biological analysis may be a useful supplementary method for pathologic diagnosis of bone and soft tissue tumors.

Adolescent↗