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[Title] Mouse mesenchymal stem cells expressing PAX-FKHR form alveolar rhabdomyosarcomas by cooperating with secondary mutations.

Alveolar rhabdomyosarcomas (ARMS) are highly malignant soft-tissue sarcomas that arise in children, adolescents, and young adults.

Despite exhibiting enhanced growth in vitro, the PAX-FKHR-expressing populations do not form colonies in soft agar or tumors in mice.

[MeSH-major] Antigens, Polyomavirus Transforming / genetics. Forkhead Transcription Factors / genetics. Genes, ras / physiology. Mesenchymal Stromal Cells / pathology. Mutation / genetics. Oncogene Proteins, Fusion / metabolism. PAX7 Transcription Factor / genetics. Paired Box Transcription Factors / genetics. Rhabdomyosarcoma, Alveolar / genetics. Rhabdomyosarcoma, Alveolar / pathology

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(PMID = 18701482.001).

[ISSN] 1538-7445

[Journal-full-title] Cancer research

[ISO-abbreviation] Cancer Res.

[Language] eng

[Publication-type] Journal Article; Research Support, Non-U.S. Gov't

[Publication-country] United States

[Chemical-registry-number] 0 / Antigens, Polyomavirus Transforming; 0 / Antigens, Viral, Tumor; 0 / Forkhead Transcription Factors; 0 / Foxo1 protein, mouse; 0 / Myogenin; 0 / Oncogene Proteins, Fusion; 0 / PAX7 Transcription Factor; 0 / Paired Box Transcription Factors; 0 / Pax7 protein, mouse; 0 / Tumor Suppressor Protein p53; 138016-91-8 / Pax3 protein, mouse

   

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[Title] Heparanase activity in alveolar and embryonal rhabdomyosarcoma: implications for tumor invasion.

BACKGROUND: Rhabdomyosarcoma (RMS) is a malignant soft tissue sarcoma of childhood including two major histological subtypes, alveolar (ARMS) and embryonal (ERMS) RMS.

[MeSH-major] Glucuronidase / metabolism. Neoplasm Invasiveness. Rhabdomyosarcoma, Alveolar / embryology. Rhabdomyosarcoma, Alveolar / enzymology

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(PMID = 19715595.001).

[ISSN] 1471-2407

[Journal-full-title] BMC cancer

[ISO-abbreviation] BMC Cancer

[Language] eng

[Publication-type] Journal Article; Research Support, Non-U.S. Gov't

[Publication-country] England

[Chemical-registry-number] EC 3.2.1.- / heparanase; EC 3.2.1.31 / Glucuronidase

[Other-IDs] NLM/ PMC2743710

   

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[Title] Spindle cell rhabdomyosarcoma in adults: clinicopathological and immunohistochemical analysis of seven new cases.

Rhabdomyosarcoma (RMS) is currently classified into embryonal RMS, including its botryoid and spindle cell variants, alveolar RMS, including a solid variant, and pleomorphic RMS.

In children and adolescents embryonal RMS occurs in a younger age group than alveolar RMS, and pleomorphic RMS is almost always seen in older adults.

All neoplasms arose in subcutaneous and deep soft tissues with dermal involvement in one case, and the size of the neoplasms ranged from 4 to 19 cm in largest diameter.

In summary, spindle cell rhabdomyosarcoma represents a rare neoplasm in adulthood characterized clinically by a rather poor prognosis, and shows a broad morphological spectrum including most likely the sclerosing, pseudovascular variant.

Immunohistochemically, tumour cells in RMS stain positively for CD 99 and WT1 as well, which is of importance in the differential diagnosis to other mesenchymal neoplasms, whereas fast myosin does not represent a reliable marker for RMS in adults.

[MeSH-major] Rhabdomyosarcoma / pathology. Sarcoma / pathology. Soft Tissue Neoplasms / pathology

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(PMID = 17013628.001).

[ISSN] 0945-6317

[Journal-full-title] Virchows Archiv : an international journal of pathology

[ISO-abbreviation] Virchows Arch.

[Language] eng

[Publication-type] Journal Article

[Publication-country] Germany

[Chemical-registry-number] 0 / Antigens, CD; 0 / Antigens, CD99; 0 / CD99 protein, human; 0 / Cell Adhesion Molecules

   

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Based on the data of the Surveillance, Epidemiology and End Results Section of the National Cancer Institute (SEER) program, soft tissue and bone sarcomas account for about 1% of all new malignancies diagnosed in the United States each year.

Anderson Cancer Center (MDACC) tumor registry was queried for all soft tissue sarcomas from 1990 through 2003, and all bone sarcomas from 1990 through 2002.

These sarcomas include rhabdomyosarcoma, synovial sarcoma, neurogenic sarcoma, epithelioid sarcomas, alveolar soft parts sarcoma, Ewing sarcoma, and osteosarcoma.

[MeSH-major] Bone Neoplasms / pathology. Sarcoma / pathology. Soft Tissue Neoplasms / pathology

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(PMID = 15838394.001).

[ISSN] 1077-4114

[Journal-full-title] Journal of pediatric hematology/oncology

[ISO-abbreviation] J. Pediatr. Hematol. Oncol.

[Language] eng

[Publication-type] Comparative Study; Journal Article; Review

[Publication-country] United States

[Number-of-references] 19

   

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[Title] Breast metastases in children and adolescents with rhabdomyosarcoma: Experience of the Italian Soft Tissue Sarcoma Committee.

BACKGROUND: Breast metastasis from rhabdomyosarcoma (RMS) is an uncommon event but may be problematic in treatment decision-making.

Aim of the study was to evaluate clinical characteristics, treatment, and subsequent outcome, of patients with RMS metastasis in the breast, enrolled in four consecutive Associazione Italiana di Ematologia ed Oncologia Pediatrica (AIEOP) Soft Tissue Sarcoma Committee protocols during the last 20 years, in order to obtain information to establish a more adequate diagnostic and therapeutic approach.

RESULTS: From 1988 to 2008, among 189 patients with metastatic RMS, we identified 7 (3.7%) patients with RMS with breast involvement at diagnosis.

All patients were females, aged 13-17 years with alveolar histology and multiple metastasis sites (2-5).

CONCLUSIONS: Our data suggest that investigations of the mammary region should be part of the usual diagnostic workup in adolescent girls with alveolar RMS, especially if the primary tumor arises in the extremities.

[MeSH-major] Breast Neoplasms / secondary. Muscle Neoplasms / pathology. Rhabdomyosarcoma / secondary

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[Copyright] Copyright © 2010 Wiley-Liss, Inc.

(PMID = 20730885.001).

[ISSN] 1545-5017

[Journal-full-title] Pediatric blood & cancer

[ISO-abbreviation] Pediatr Blood Cancer

[Language] eng

[Publication-type] Journal Article; Research Support, Non-U.S. Gov't

[Publication-country] United States

   

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[Title] FOXO1a acts as a selective tumor suppressor in alveolar rhabdomyosarcoma.

Rhabdomyosarcoma (RMS), the most common pediatric soft-tissue sarcoma, has two major histological subtypes: embryonal RMS (ERMS), which has a favorable prognosis, and alveolar RMS (ARMS), which has a poor outcome.

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(PMID = 16157701.001).

[ISSN] 0021-9525

[Journal-full-title] The Journal of cell biology

[ISO-abbreviation] J. Cell Biol.

[Language] ENG

[Grant] United States / NCI NIH HHS / CA / P01 CA071907; United States / NCI NIH HHS / CA / R01 CA087952; United States / NCI NIH HHS / CA / CA 71907; United States / NCI NIH HHS / CA / P01 CA023099; United States / NCI NIH HHS / CA / P30 CA021765; United States / NCI NIH HHS / CA / CA 96696; United States / NCI NIH HHS / CA / CA 87952; United States / NCI NIH HHS / CA / CA 23099; United States / NCI NIH HHS / CA / R01 CA096696; United States / NCI NIH HHS / CA / CA 21765

[Publication-type] Comparative Study; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.; Retracted Publication

[Publication-country] United States

[Chemical-registry-number] 0 / FOXO1 protein, human; 0 / Fluorescent Dyes; 0 / Forkhead Transcription Factors; 0 / Indoles; 0 / Recombinant Fusion Proteins; 47165-04-8 / DAPI; EC 1.13.12.- / Luciferases; EC 3.4.22.- / CASP3 protein, human; EC 3.4.22.- / Casp3 protein, mouse; EC 3.4.22.- / Caspase 3; EC 3.4.22.- / Caspases

[Other-IDs] NLM/ PMC2171446

   

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[Title] Identification of PAX3-FKHR-regulated genes differentially expressed between alveolar and embryonal rhabdomyosarcoma: focus on MYCN as a biologically relevant target.

Rhabdomyosarcoma is a family of myogenic soft tissue tumors subdivided into two main subtypes: alveolar (ARMS) and embryonal (ERMS).

[MeSH-major] Gene Expression Regulation, Neoplastic. Nuclear Proteins / physiology. Oncogene Proteins / physiology. Oncogene Proteins, Fusion / physiology. Rhabdomyosarcoma, Alveolar / genetics. Rhabdomyosarcoma, Embryonal / genetics. Soft Tissue Neoplasms / genetics

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(PMID = 18335505.001).

[ISSN] 1098-2264

[Journal-full-title] Genes, chromosomes & cancer

[ISO-abbreviation] Genes Chromosomes Cancer

[Language] eng

[Grant] United States / NCI NIH HHS / CA / CA104896; United States / NCI NIH HHS / CA / CA106450; United States / NCI NIH HHS / CA / CA64202; United States / NCI NIH HHS / CA / CA87812

[Publication-type] Comparative Study; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't

[Publication-country] United States

[Chemical-registry-number] 0 / MYCN protein, human; 0 / Neoplasm Proteins; 0 / Nuclear Proteins; 0 / Oncogene Proteins; 0 / Oncogene Proteins, Fusion; 0 / PAX3-FKHR fusion protein, human; 0 / Protein Synthesis Inhibitors; 0 / RNA, Messenger; 0 / RNA, Neoplasm; 0 / Recombinant Fusion Proteins; 98600C0908 / Cycloheximide

   

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[Title] MI-63: a novel small-molecule inhibitor targets MDM2 and induces apoptosis in embryonal and alveolar rhabdomyosarcoma cells with wild-type p53.

METHODS: Rhabdomyosarcoma (RMS), the most common childhood soft tissue sarcoma, expresses either wild-type or mutant p53 protein.

We examined the inhibitory effects of MI-63 in embryonal RMS (ERMS) and alveolar RMS (ARMS) cell lines expressing wild-type or mutated p53.

[MeSH-major] Antineoplastic Agents / pharmacology. Apoptosis / drug effects. Indoles / pharmacology. Proto-Oncogene Proteins c-mdm2 / antagonists & inhibitors. Rhabdomyosarcoma, Alveolar / pathology. Rhabdomyosarcoma, Embryonal / pathology. Spiro Compounds / pharmacology. Tumor Suppressor Protein p53 / metabolism

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(PMID = 19707204.001).

[ISSN] 1532-1827

[Journal-full-title] British journal of cancer

[ISO-abbreviation] Br. J. Cancer

[Language] eng

[Publication-type] Journal Article

[Publication-country] England

[Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Imidazoles; 0 / Indoles; 0 / MI-63 compound; 0 / Piperazines; 0 / Spiro Compounds; 0 / Tumor Suppressor Protein p53; 0 / nutlin 3; 80168379AG / Doxorubicin; EC 3.4.22.- / Caspase 3; EC 6.3.2.19 / MDM2 protein, human; EC 6.3.2.19 / Proto-Oncogene Proteins c-mdm2

[Other-IDs] NLM/ PMC2736841

   

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[Title] Treatment results for patients with localized, completely resected (Group I) alveolar rhabdomyosarcoma on Intergroup Rhabdomyosarcoma Study Group (IRSG) protocols III and IV, 1984-1997: a report from the Children's Oncology Group.

PURPOSE: To assess local control, event-free survival (EFS), and overall survival (OS) rates in 71 patients with localized, completely resected (Group I) alveolar rhabdomyosarcoma (ALV RMS) and their relation to radiation therapy (RT) on IRSG Protocols III and IV, 1984-1997.

PATIENTS AND TUMORS: Patients were 1-18 years at diagnosis (median, 6 years).

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[Copyright] Copyright 2010 Wiley-Liss, Inc.

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(PMID = 20806360.001).

[ISSN] 1545-5017

[Journal-full-title] Pediatric blood & cancer

[ISO-abbreviation] Pediatr Blood Cancer

[Language] ENG

[Grant] United States / NCI NIH HHS / CA / CA-98543; United States / NCI NIH HHS / CA / U10 CA072989; United States / NCI NIH HHS / CA / U10 CA029511; United States / NCI NIH HHS / CA / CA-24507; United States / NCI NIH HHS / CA / CA-72989; United States / NCI NIH HHS / CA / U10 CA098543; United States / NCI NIH HHS / CA / CA-29511; United States / NCI NIH HHS / CA / U10 CA024507

[Publication-type] Journal Article; Research Support, N.I.H., Extramural

[Publication-country] United States

[Other-IDs] NLM/ NIHMS263511; NLM/ PMC3128801

   

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[Title] A case report of rhabdomyosarcoma of the uterus associated with uterine inversion.

BACKGROUND: Alveolar rhabdomyosarcoma is an uncommon malignant soft tissue tumor rarely found in the genital tract.

On exam, a large pedunculated mass protruding from the cervix was seen and biopsy of this mass revealed an alveolar rhabdomyosarcoma.

The patient was treated with adjuvant chemotherapy consisting of VAC (Vincristine, Actinomycin, and Cyclophosphamide) for a presumed cervical rhabdomyosarcoma.

Final pathology showed alveolar rhabdomyosarcoma of the uterus.

The patient then received additional postoperative VAC regimen for a total of 10 treatments and remains in good health with no evidence of disease 20 months from diagnosis.

CONCLUSION: This case report describes the only reported case of uterine inversion secondary to alveolar rhabdomyosarcoma of the uterus and discusses current therapeutic options for adults.

[MeSH-major] Rhabdomyosarcoma, Alveolar / complications. Uterine Inversion / etiology. Uterine Neoplasms / complications

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(PMID = 15721436.001).

[ISSN] 0090-8258

[Journal-full-title] Gynecologic oncology

[ISO-abbreviation] Gynecol. Oncol.

[Language] eng

[Publication-type] Case Reports; Journal Article

[Publication-country] United States

[Chemical-registry-number] 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine; 8N3DW7272P / Cyclophosphamide; VAC protocol

   

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[Title] Rhabdomyosarcoma of nose and paranasal sinuses--a therapeutic challenge.

Rhabdomyosarcoma is a highly malignant tumour of striated muscle and it is the most common soft tissue sarcoma in the paediatric age group.

Histologically embryonal rhabdomyosarcoma is the most common variant.

The present series deals with seven such cases of different histopathological types including the embryonal, botryoid, and alveolar varieties in the paediatric age group.

[MeSH-major] Nose Neoplasms / therapy. Paranasal Sinus Neoplasms / therapy. Rhabdomyosarcoma / therapy

[MeSH-minor] Biopsy. Child. Child, Preschool. Combined Modality Therapy. Diagnosis, Differential. Female. Follow-Up Studies. Humans. India / epidemiology. Male. Retrospective Studies. Survival Rate. Tomography, X-Ray Computed. Treatment Outcome

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(PMID = 17388011.001).

[ISSN] 0019-5847

[Journal-full-title] Journal of the Indian Medical Association

[ISO-abbreviation] J Indian Med Assoc

[Language] eng

[Publication-type] Journal Article; Multicenter Study

[Publication-country] India

   

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[Title] Genetic and molecular alterations in rhabdomyosarcoma: mRNA overexpression of MCL1 and MAP2K4 genes.

Rhabdomyosarcoma, the most common soft tissue sarcoma in childhood, belongs to the small round cell tumor family and is classified according to its histopathological features as embryonal, alveolar and pleomorphic.

In this study we propose to explore genetic alterations involved in rhabdomyosarcoma tumorigenesis and assess the level of mRNA gene expression of controlling survival signalling pathways.

Our findings on rhabdomyosarcoma samples showed multiple copy number alterations in chromosome regions implicated in malignancy progression and indicated a strong expression of MAP2K4 and MCL1 genes, both involved in different biological functions of complicated signalling pathways.

[MeSH-major] MAP Kinase Kinase 4 / genetics. Proto-Oncogene Proteins c-bcl-2 / genetics. RNA, Messenger / analysis. Rhabdomyosarcoma / genetics. Soft Tissue Neoplasms / genetics

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(PMID = 19012245.001).

[ISSN] 1699-5848

[Journal-full-title] Histology and histopathology

[ISO-abbreviation] Histol. Histopathol.

[Language] eng

[Publication-type] Journal Article; Research Support, Non-U.S. Gov't

[Publication-country] Spain

[Chemical-registry-number] 0 / Myeloid Cell Leukemia Sequence 1 Protein; 0 / Proto-Oncogene Proteins c-bcl-2; 0 / RNA, Messenger; EC 2.7.12.2 / MAP Kinase Kinase 4; EC 2.7.12.2 / MAP2K4 protein, human

   

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[Title] [Clinical relevance of chromosomal aberrations in bone and soft tissue tumors in children and young adults].

BACKGROUND: We present the results of a cytogenetic and molecular cytogenetic analysis of a series of patients with bone and soft tissue tumors.

PATIENTS ANDMETHODS: We analyzed a cohort of 26 patients with Ewing sarcoma/PNET, 15 patients with rhabdomyosarcoma, 5 with synovial sarcoma and one patient with an undifferentiated sarcoma using the cytogenetic and molecular cytogenetic techniques M-FISH and arrayCGH.

In the RMS patients we detected the t(1;13)(p36;q14) once and the t(2;13)(q35;q14) four times, both of them characteristic for the alveolar subtype with poor prognosis and numerical aberrations, characteristic for the embryonal subtype, in five patients.

CONCLUSION: Cytogenetic and molecular cytogenetic analysis is a valuable diagnostic tool in bone and soft tissue tumors, especially in less differentiated subtypes, and as such it should be an integral part of curative care.

[MeSH-major] Bone Neoplasms / genetics. Chromosome Aberrations. Soft Tissue Neoplasms / genetics

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(PMID = 19522375.001).

[ISSN] 0862-495X

[Journal-full-title] Klinická onkologie : casopis Ceské a Slovenské onkologické spolecnosti

[ISO-abbreviation] Klin Onkol

[Language] cze

[Publication-type] English Abstract; Journal Article

[Publication-country] Czech Republic

   

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Eight had acute lymphoblastic leukaemia, one child had acute myeloid leukaemia and another had rhabdomyosarcoma.

[MeSH-major] Debridement / methods. Ecthyma / surgery. Practice Guidelines as Topic. Precursor Cell Lymphoblastic Leukemia-Lymphoma / complications. Rhabdomyosarcoma, Alveolar / complications. Soft Tissue Neoplasms / complications

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[Cites] Am Surg. 1988 Dec;54(12):693-5 [3195845.001]

[Cites] Clin Infect Dis. 1994 Mar;18(3):390-4 [8011821.001]

[Cites] Pediatr Infect Dis J. 1991 Jul;10(7):496-500 [1876464.001]

[Cites] J Am Acad Dermatol. 1992 Sep;27(3):415-8 [1401278.001]

[Cites] Ann Acad Med Singapore. 1990 Mar;19(2):290-4 [2346294.001]

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[Cites] Pediatr Infect Dis J. 2002 Dec;21(12):1161-4 [12488670.001]

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(PMID = 18427811.001).

[ISSN] 0179-0358

[Journal-full-title] Pediatric surgery international

[ISO-abbreviation] Pediatr. Surg. Int.

[Language] eng

[Publication-type] Comparative Study; Journal Article

[Publication-country] Germany

   

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[Title] Rhabdomyosarcoma presenting with destructive large lesion of the face.

Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children and adolescents.

Prognostic factors of RMS include tumor status, primary tumor site, localization in the organ or tissue of origin, patient age and histopathological type.

Alveolar histologic type is more aggressive than the other types and is seen in most patients with locally advanced and metastatic disease.

She was diagnosed with alveolar rhabdomyosarcoma on histopathological examination.

[MeSH-major] Facial Neoplasms / diagnosis. Rhabdomyosarcoma, Alveolar / diagnosis. Sarcoma / diagnosis

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(PMID = 18176304.001).

[ISSN] 1541-8243

[Journal-full-title] Southern medical journal

[ISO-abbreviation] South. Med. J.

[Language] eng

[Publication-type] Case Reports; Journal Article

[Publication-country] United States

   

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[Title] Emergency surgery for left atrial metastatic alveolar rhabdomyosarcoma manifesting as a right atrial mass.

We report the case of a 43-year-old woman with a history of alveolar rhabdomyosarcoma of the forearm, scalp, and paraspinal region who developed acute shortness of breath owing to a tumor arising from the left atrium and extending through the coronary sinus.

Emergency resection of the atrial mass was performed, and histologic analysis confirmed the presence of metastatic rhabdomyosarcoma.

To our knowledge, this is the first reported case of emergency surgical resection of intracardiac metastatic alveolar rhabdomyosarcoma.

[MeSH-major] Heart Neoplasms / secondary. Heart Neoplasms / surgery. Rhabdomyosarcoma, Alveolar / pathology. Rhabdomyosarcoma, Alveolar / secondary. Rhabdomyosarcoma, Alveolar / surgery. Soft Tissue Neoplasms / pathology

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(PMID = 18721606.001).

[ISSN] 1552-6259

[Journal-full-title] The Annals of thoracic surgery

[ISO-abbreviation] Ann. Thorac. Surg.

[Language] eng

[Publication-type] Case Reports; Journal Article

[Publication-country] Netherlands

   

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[Title] FGFR1 over-expression in primary rhabdomyosarcoma tumors is associated with hypomethylation of a 5' CpG island and abnormal expression of the AKT1, NOG, and BMP4 genes.

Rhabdomyosarcoma (RMS), the most common pediatric soft tissue sarcoma likely results from abnormal proliferation and differentiation during skeletal myogenesis.

Multiple genetic alterations are associated with the three RMS histopathological subtypes, embryonal, alveolar, and pleomorphic adult variant.

[MeSH-major] Bone Morphogenetic Proteins / genetics. Carrier Proteins / genetics. CpG Islands. DNA Methylation. Proto-Oncogene Proteins c-akt / genetics. Receptor, Fibroblast Growth Factor, Type 1 / genetics. Rhabdomyosarcoma / genetics

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[Copyright] Copyright (c) 2007 Wiley-Liss, Inc.

(PMID = 17696196.001).

[ISSN] 1045-2257

[Journal-full-title] Genes, chromosomes & cancer

[ISO-abbreviation] Genes Chromosomes Cancer

[Language] eng

[Publication-type] Journal Article; Research Support, Non-U.S. Gov't

[Publication-country] United States

[Chemical-registry-number] 0 / BMP4 protein, human; 0 / Bone Morphogenetic Protein 4; 0 / Bone Morphogenetic Proteins; 0 / Carrier Proteins; 0 / DNA Primers; 148294-77-3 / noggin protein; EC 2.7.10.1 / FGFR1 protein, human; EC 2.7.10.1 / Receptor, Fibroblast Growth Factor, Type 1; EC 2.7.11.1 / AKT1 protein, human; EC 2.7.11.1 / Proto-Oncogene Proteins c-akt

   

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[Title] Reverse transcriptase-polymerase chain reaction as an ancillary molecular technique in the diagnosis of small blue round cell tumors by fine-needle aspiration cytology.

The PAX3/7-FKHR fusion transcript was detected in 2 of 4 cases of alveolar rhabdomyosarcoma and the EWS-WT1 transcript in both cases of DSRCT.

[MeSH-major] Biopsy, Fine-Needle / methods. Neuroblastoma / diagnosis. Neuroectodermal Tumors, Primitive, Peripheral / diagnosis. Reverse Transcriptase Polymerase Chain Reaction / methods. Rhabdomyosarcoma / diagnosis. Sarcoma, Ewing / diagnosis. Sarcoma, Small Cell / diagnosis. Soft Tissue Neoplasms / diagnosis

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(PMID = 20231617.001).

[ISSN] 1943-7722

[Journal-full-title] American journal of clinical pathology

[ISO-abbreviation] Am. J. Clin. Pathol.

[Language] eng

[Publication-type] Journal Article; Research Support, Non-U.S. Gov't

[Publication-country] United States

   

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[Title] Molecular classification of rhabdomyosarcoma--genotypic and phenotypic determinants of diagnosis: a report from the Children's Oncology Group.

Rhabdomyosarcoma (RMS) in children occurs as two major histological subtypes, embryonal (ERMS) and alveolar (ARMS).

ERMS is associated with an 11p15.5 loss of heterozygosity (LOH) and may be confused with nonmyogenic, non-RMS soft tissue sarcomas.

Here, we describe a genomic-based classification scheme that is derived from the combined gene expression profiling and LOH analysis of 160 cases of RMS and non-RMS soft tissue sarcomas that is at variance with conventional histopathological schemes.

These objectively derived molecular classes are based solely on genomic analysis at the time of diagnosis and are highly reproducible.

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(PMID = 19147825.001).

[ISSN] 1525-2191

[Journal-full-title] The American journal of pathology

[ISO-abbreviation] Am. J. Pathol.

[Language] ENG

[Grant] United States / NCI NIH HHS / CA / U01 CA088199; United States / NCI NIH HHS / CA / U01 CA114757; United States / NCI NIH HHS / CA / CA-88199; United States / NCI NIH HHS / CA / U01CA-114757

[Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't

[Publication-country] United States

[Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / HMGA2 Protein; 0 / TFAP2B protein, human; 0 / Transcription Factor AP-2

[Other-IDs] NLM/ PMC2630563

   

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[Title] Genomic imbalances in rhabdomyosarcoma cell lines affect expression of genes frequently altered in primary tumors: an approach to identify candidate genes involved in tumor development.

Rhabdomyosarcomas (RMS) are the most common pediatric soft tissue sarcomas.

They resemble developing skeletal muscle and are histologically divided into two main subtypes; alveolar and embryonal RMS.

Characteristic genomic aberrations, including the PAX3- and PAX7-FOXO1 fusion genes in alveolar cases, have led to increased understanding of their molecular biology.

Copy number and expression of FGFR1 was validated in additional primary material and found amplified in 6 out of 196 cases and overexpressed relative to skeletal muscle and myoblasts, with significantly higher expression levels in the embryonal compared with alveolar subtypes.

[MeSH-major] Allelic Imbalance. Gene Dosage. Gene Expression Regulation, Neoplastic. Neoplasm Proteins / genetics. Rhabdomyosarcoma / genetics

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(PMID = 19235922.001).

[ISSN] 1098-2264

[Journal-full-title] Genes, chromosomes & cancer

[ISO-abbreviation] Genes Chromosomes Cancer

[Language] eng

[Grant] United Kingdom / Cancer Research UK / /

[Publication-type] Journal Article; Research Support, Non-U.S. Gov't

[Publication-country] United States

[Chemical-registry-number] 0 / Neoplasm Proteins; EC 2.7.10.1 / FGFR1 protein, human; EC 2.7.10.1 / Receptor, Fibroblast Growth Factor, Type 1

   

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[Title] A novel PAX3 rearrangement in embryonal rhabdomyosarcoma.

Rhabdomyosarcoma is the most common soft tissue tumor seen in children and young adults, and it can be classified into 2 major histological subtypes, alveolar and embryonal.

In the alveolar subtype, 2 recurrent chromosomal translocations, t(2;13)(q35;q14) and its variant t(1;13)(p36;q14), have been identified as the specific cytogenetic abnormalities.

In this study, we analyzed the complex chromosomal translocation in one case with embryonal rhabdomyosarcoma by means of spectral karyotyping (SKY) and identified a novel translocation involving chromosome band 2q35, which is the locus of PAX3 gene.

[MeSH-major] Mutation. Paired Box Transcription Factors / genetics. Rhabdomyosarcoma, Embryonal / genetics. Urogenital Neoplasms / genetics

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(PMID = 19215790.001).

[ISSN] 1873-4456

[Journal-full-title] Cancer genetics and cytogenetics

[ISO-abbreviation] Cancer Genet. Cytogenet.

[Language] eng

[Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't

[Publication-country] United States

[Chemical-registry-number] 0 / Oncogene Proteins, Fusion; 0 / PAX3 protein, human; 0 / PAX3-FOXO1A fusion protein, human; 0 / Paired Box Transcription Factors; 0 / Recombinant Fusion Proteins

   

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Treatment of these tumors usually is conducted according to standardized therapy protocols like the German Cooperative Soft Tissue Sarcoma Study (CWS) or the Intergroup Rhabdomyosarcoma Study (IRS).

Histologic subtypes encompassed 9 embryonal (e) and 6 alveolar (a) RMS.

[MeSH-major] Nose Neoplasms / therapy. Paranasal Sinus Neoplasms / therapy. Rhabdomyosarcoma / therapy

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(PMID = 16025051.001).

[ISSN] 0194-5998

[Journal-full-title] Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery

[ISO-abbreviation] Otolaryngol Head Neck Surg

[Language] eng

[Publication-type] Journal Article

[Publication-country] United States

[Chemical-registry-number] 0 / Antineoplastic Agents

   

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[Title] [Soft tissue sarcomas: update on molecular data].

Soft tissue sarcomas are rare and may be a source of problems for diagnosis and treatment.

Detection of these disorders may help in diagnosis and in determining prognosis.

Detection of specific translocation is recommended in synovial sarcoma, alveolar rhabdomyosarcoma or PNET diagnosis because of therapeutic consequences; in case of rarer histologic type (low grade fibromyxoid sarcoma, clear cell sarcoma, infantile fibrosarcoma...), it may confirm the diagnosis.

In some cases, some translocations have a prognostic value (alveolar rhabdomyosarcoma) whereas it is discussed in others (synovial sarcoma).

The techniques used to detect these translocations are very sensitive so it may be used to detect microscopical metastasis (bone marrow metastasis of alveolar rhabdomyosarcoma for example).

Detection of MDM2 and CDK4 genes amplifications (FISH or quantitative PCR) may be sometimes useful in well differentiated and dedifferentiated liposarcomas diagnosis.

Mutation detection of KIT or PDGFRA may help in GIST diagnosis and type of mutation is predictive of response to treatment.

[MeSH-major] Sarcoma / genetics. Soft Tissue Neoplasms / genetics

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(PMID = 16442827.001).

[ISSN] 1278-3218

[Journal-full-title] Cancer radiothérapie : journal de la Société française de radiothérapie oncologique

[ISO-abbreviation] Cancer Radiother

[Language] fre

[Publication-type] English Abstract; Journal Article; Review

[Publication-country] France

[Number-of-references] 35

   

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[Title] PDK-1/AKT pathway as a novel therapeutic target in rhabdomyosarcoma cells using OSU-03012 compound.

Rhabdomyosarcoma (RMS) is the most common paediatric soft-tissue sarcoma including two major subtypes, alveolar rhabdomyosarcoma (ARMS) and embryonal rhabdomyosarcoma (ERMS).

We examined phosphorylation levels of AKT using ARMS and ERMS tissue microarray and immunohistochemistry staining.

[MeSH-major] Antineoplastic Agents / pharmacology. Enzyme Inhibitors / pharmacology. Oncogene Protein v-akt / metabolism. Protein-Serine-Threonine Kinases / metabolism. Pyrazoles / pharmacology. Rhabdomyosarcoma / drug therapy. Rhabdomyosarcoma / metabolism. Sulfonamides / pharmacology

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[Cites] Cancer Res. 2004 Jun 15;64(12):4309-18 [15205346.001]

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(PMID = 17848913.001).

[ISSN] 0007-0920

[Journal-full-title] British journal of cancer

[ISO-abbreviation] Br. J. Cancer

[Language] eng

[Publication-type] Journal Article; Research Support, Non-U.S. Gov't

[Publication-country] England

[Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Chromones; 0 / Enzyme Inhibitors; 0 / Morpholines; 0 / OSU 03012; 0 / Pyrazoles; 0 / Sulfonamides; 154447-36-6 / 2-(4-morpholinyl)-8-phenyl-4H-1-benzopyran-4-one; EC 2.7.11.1 / Oncogene Protein v-akt; EC 2.7.11.1 / Protein-Serine-Threonine Kinases; EC 2.7.11.2 / pyruvate dehydrogenase (acetyl-transferring) kinase

[Other-IDs] NLM/ PMC2360380

   

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[Title] Activation of the hedgehog pathway confers a poor prognosis in embryonal and fusion gene-negative alveolar rhabdomyosarcoma.

Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children and comprises two major histological subtypes: alveolar rhabdomyosarcoma (ARMS) and embryonal rhabdomyosarcoma (ERMS).

[MeSH-major] Hedgehog Proteins / physiology. Rhabdomyosarcoma, Alveolar / metabolism. Rhabdomyosarcoma, Embryonal / metabolism. Signal Transduction / physiology

[MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Female. Gene Fusion. Humans. Infant. Kruppel-Like Transcription Factors / analysis. Male. Myogenic Regulatory Factor 5 / analysis. Nerve Tissue Proteins / analysis. Prognosis. Receptors, Cell Surface / analysis. Transcription Factors / analysis

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(PMID = 20818440.001).

[ISSN] 1476-5594

[Journal-full-title] Oncogene

[ISO-abbreviation] Oncogene

[Language] eng

[Publication-type] Journal Article; Research Support, Non-U.S. Gov't

[Publication-country] England

[Chemical-registry-number] 0 / GLI1 protein, human; 0 / GLI3 protein, human; 0 / Hedgehog Proteins; 0 / Kruppel-Like Transcription Factors; 0 / MYF5 protein, human; 0 / Myogenic Regulatory Factor 5; 0 / Nerve Tissue Proteins; 0 / Receptors, Cell Surface; 0 / Transcription Factors; 0 / patched receptors

   

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[Title] Alveolar rhabdomyosarcoma originating between the fourth and fifth metatarsal--case report and literature review.

We report a case of alveolar rhabdomyosarcoma arising between the fourth and fifth metatarsal.

Plain radiographs and MRI showed a soft tissue mass displacing the fourth metatarsal.

Percutaneous biopsy revealed an alveolar rhabdomyosarcoma.

[MeSH-major] Metatarsus. Rhabdomyosarcoma, Alveolar / diagnosis

[MeSH-minor] Adolescent. Biopsy. Diagnosis, Differential. Follow-Up Studies. Humans. Magnetic Resonance Imaging. Male. Positron-Emission Tomography

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[Copyright] Copyright (c) 2010 European Foot and Ankle Society. Published by Elsevier Ltd. All rights reserved.

(PMID = 20654999.001).

[ISSN] 1460-9584

[Journal-full-title] Foot and ankle surgery : official journal of the European Society of Foot and Ankle Surgeons

[ISO-abbreviation] Foot Ankle Surg

[Language] eng

[Publication-type] Case Reports; Journal Article

[Publication-country] France

   

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[Title] Rhabdomyosarcoma: new windows of opportunity.

Rhabdomyosarcoma is a highly malignant, small blue cell tumor characterized by muscle differentiation.

Adequate biopsy to obtain sufficient tissue for accurate diagnosis and molecular characterization is critical.

Patients must be assessed for tumor extent; the Intergroup Rhabdomyosarcoma Study (IRS) clinical group and Staging system is universally applied in North America.

Local control relies on complete surgical excision when possible; those whose tumors are not completely excised and those with alveolar histology tumors require local irradiation to maximize local control.

[MeSH-major] Rhabdomyosarcoma. Soft Tissue Neoplasms

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(PMID = 16079319.001).

[ISSN] 1083-7159

[Journal-full-title] The oncologist

[ISO-abbreviation] Oncologist

[Language] eng

[Publication-type] Journal Article; Review

[Publication-country] United States

[Number-of-references] 99

   

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[Title] Examination of gene fusion status in archival samples of alveolar rhabdomyosarcoma entered on the Intergroup Rhabdomyosarcoma Study-III trial: a report from the Children's Oncology Group.

Alveolar rhabdomyosarcoma (ARMS) is a soft tissue cancer in which chromosomal translocations generate PAX3-FKHR and PAX7-FKHR gene fusions.

We applied this approach to a convenience sample of 78 formalin-fixed, paraffin-embedded ARMS tumors from the Intergroup Rhabdomyosarcoma Study (IRS)-III clinical trial and obtained satisfactory results in 59 (76%) cases.

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(PMID = 16645206.001).

[ISSN] 1525-1578

[Journal-full-title] The Journal of molecular diagnostics : JMD

[ISO-abbreviation] J Mol Diagn

[Language] ENG

[Grant] United States / NCI NIH HHS / CA / CA24507; United States / NCI NIH HHS / CA / CA89461; United States / NCI NIH HHS / CA / CA98543; United States / NCI NIH HHS / CA / U10 CA098413; United States / NCI NIH HHS / CA / U10 CA098543; United States / NCI NIH HHS / CA / CA98413; United States / NCI NIH HHS / CA / U10 CA024507; United States / NCI NIH HHS / CA / R01 CA089461

[Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't

[Publication-country] United States

[Chemical-registry-number] 0 / FOXO1 protein, human; 0 / Forkhead Transcription Factors; 0 / Neoplasm Proteins; 0 / PAX3 protein, human; 0 / PAX7 Transcription Factor; 0 / PAX7 protein, human; 0 / Paired Box Transcription Factors; 0 / RNA, Messenger

[Other-IDs] NLM/ PMC1867584

   

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[Title] Trends in childhood rhabdomyosarcoma incidence and survival in the United States, 1975-2005.

BACKGROUND: Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children and adolescents aged<20 years; its etiology remains largely unknown.

It is believed that embryonal (ERMS) and alveolar rhabdomyosarcoma (ARMS), the most common subtypes, arise through distinct biologic mechanisms.

This trend may have been attributable in part to shifts in diagnosis, because a significant negative trend in RMS, not otherwise specified was observed concurrently.

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[Copyright] Copyright (c) 2009 American Cancer Society.

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(PMID = 19536876.001).

[ISSN] 0008-543X

[Journal-full-title] Cancer

[ISO-abbreviation] Cancer

[Language] ENG

[Grant] United States / NCI NIH HHS / CA / T32 CA099936-07; United States / NCI NIH HHS / CA / T32 CA099936-06S1; United States / NCI NIH HHS / CA / CA099936-07; United States / NCI NIH HHS / CA / T32 CA099936; United States / NCI NIH HHS / CA / CA099936-06S1

[Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't

[Publication-country] United States

[Other-IDs] NLM/ NIHMS218300; NLM/ PMC2953716

   

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[Title] Molecular classification of soft tissue sarcomas and its clinical applications.

Sarcomas are a heterogeneous group of tumors that are traditionally classified according to the morphology and type of tissue that they resemble, such as rhabdomyosarcoma, which resembles skeletal muscle.

These specific genetic alterations are an important adjunct to standard morphological and immunohistochemical diagnoses, and in some cases have a prognostic value, e. g., Ewing family tumors, synovial sarcoma, and alveolar rhabdomyosarcoma.

[MeSH-major] Sarcoma / classification. Sarcoma / genetics. Soft Tissue Neoplasms / classification. Soft Tissue Neoplasms / genetics

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(PMID = 20490332.001).

[ISSN] 1936-2625

[Journal-full-title] International journal of clinical and experimental pathology

[ISO-abbreviation] Int J Clin Exp Pathol

[Language] eng

[Publication-type] Journal Article; Review

[Publication-country] United States

[Number-of-references] 45

[Other-IDs] NLM/ PMC2872748

[Keywords] NOTNLM ; Sarcomas / molecular classification / molecular genetics / soft tissue tumors / specific genetic translocation

   

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[Title] [Statistical study of the evolution over ten years of the clinical and therapeutic approach in childhood soft tissue sarcoma].

Soft tissue sarcoma has a primitive mesenchymal origin and represents a heterogeneous group of malignant entities with a continuous rising frequency in the age range below 18.

Rhabdomyosarcoma (RMS) constitutes 5.8% from the whole amount of pediatric solid tumors, taking the fourth place after CNS tumors, neuroblastoma, and Wilms tumors.

The other category of non-rhabdomyosarcoma tumors in children and teenagers represents 3% of the solid malignancies under 18 years old.

We included in the study a well-known category of soft tissue tumors called of uncertain malignancy.

Based on histological examination 19 cases (32.75%) were of rhabdomyosarcoma type with following subtypes: alveolar--7 patients, embryonic-- 9 cases, fusiform - 2 cases, bothrioid--1 case), 8 cases were undifferentiated soft tissue sarcomas and one patient had a tumor of pleiomorphic type; 13 children (22.41%) had non-rhabdomyosarcoma soft tissue sarcomas: 6 fibrosarcomas, 2 synovial sarcomas, 1 leiomyosarcoma, 1 Kaposi sarcoma, 1 case of malignant peripheral nerve sheath tumor, 1 case of angioma tumor, one liposarcoma; 16 cases were included in soft tissue tumors of uncertain origin (fibromatosis--6 cases, fibrous histiocytoma--4 cases, hamartoma--cases, myoblastoma--1 case, fibro-xanthoma--1 case, hemangioendothelioma--1 case); 1 PPNET (Askin tumor).

CONCLUSIONS: The continuously augmented incidence of soft tissue sarcoma in young ages and the advanced stages initial presentations of tumors require a much more serious and rhythmic survey at general practitioner level.

[MeSH-major] Sarcoma / pathology. Sarcoma / therapy. Soft Tissue Neoplasms / pathology. Soft Tissue Neoplasms / therapy

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(PMID = 17983168.001).

[ISSN] 0048-7848

[Journal-full-title] Revista medico-chirurgicală̆ a Societă̆ţ̜ii de Medici ş̧i Naturaliş̧ti din Iaş̧i

[ISO-abbreviation] Rev Med Chir Soc Med Nat Iasi

[Language] rum

[Publication-type] English Abstract; Journal Article

[Publication-country] Romania

   

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[Title] A human recombinant autoantibody-based immunotoxin specific for the fetal acetylcholine receptor inhibits rhabdomyosarcoma growth in vitro and in a murine transplantation model.

Rhabdomyosarcoma (RMS) is the most common malignant soft tissue tumor in children and is highly resistant to all forms of treatment currently available once metastasis or relapse has commenced.

While scFv35-ETA had no damaging effect on fAChR-negative control cell lines, it killed human embryonic and alveolar RMS cell lines in vitro and delayed RMS development in a murine transplantation model.

[MeSH-major] ADP Ribose Transferases / administration & dosage. Autoantibodies / immunology. Bacterial Toxins / administration & dosage. Exotoxins / administration & dosage. Immunotoxins / administration & dosage. Receptors, Nicotinic / immunology. Recombinant Fusion Proteins / administration & dosage. Rhabdomyosarcoma / drug therapy. Single-Chain Antibodies / immunology. Virulence Factors / administration & dosage

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(PMID = 20204062.001).

[ISSN] 1110-7251

[Journal-full-title] Journal of biomedicine & biotechnology

[ISO-abbreviation] J. Biomed. Biotechnol.

[Language] eng

[Grant] United Kingdom / Wellcome Trust / / 084655; United Kingdom / Medical Research Council / / G0701521

[Publication-type] Journal Article; Research Support, Non-U.S. Gov't

[Publication-country] United States

[Chemical-registry-number] 0 / Autoantibodies; 0 / Bacterial Toxins; 0 / CHRNG protein, human; 0 / Exotoxins; 0 / Immunotoxins; 0 / Receptors, Nicotinic; 0 / Recombinant Fusion Proteins; 0 / Single-Chain Antibodies; 0 / Virulence Factors; EC 2.4.2.- / ADP Ribose Transferases; EC 2.4.2.31 / toxA protein, Pseudomonas aeruginosa

[Other-IDs] NLM/ PMC2829619

   

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[Title] Childhood rhabdomyosarcoma: new insight on biology and treatment.

Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma of childhood.

The two most common histologic variants are the embryonal and alveolar subtypes.

Recent studies have pointed to a possible mesenchymal stem cell as the progenitor for alveolar RMS.

[MeSH-major] Rhabdomyosarcoma, Alveolar / diagnosis. Rhabdomyosarcoma, Alveolar / genetics. Rhabdomyosarcoma, Alveolar / therapy. Rhabdomyosarcoma, Embryonal / diagnosis. Rhabdomyosarcoma, Embryonal / genetics. Rhabdomyosarcoma, Embryonal / therapy. Soft Tissue Neoplasms / diagnosis. Soft Tissue Neoplasms / genetics. Soft Tissue Neoplasms / therapy

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(PMID = 20820958.001).

[ISSN] 1534-6269

[Journal-full-title] Current oncology reports

[ISO-abbreviation] Curr Oncol Rep

[Language] eng

[Publication-type] Journal Article; Review

[Publication-country] United States

[Chemical-registry-number] 0 / Protein Kinase Inhibitors; EC 2.7.1.1 / TOR Serine-Threonine Kinases; W36ZG6FT64 / Sirolimus

   

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[Title] Treatment outcome of rhabdomyosarcoma in Hong Kong Chinese children.

OBJECTIVES: To review the treatment outcome of rhabdomyosarcoma in Hong Kong Chinese children.

PATIENTS: Consecutive cases of rhabdomyosarcoma diagnosed and treated by the Department of Paediatrics and Adolescent Medicine of Queen Mary Hospital between 1989 and 2005.

Each patient was staged and treated according to the Intergroup Rhabdomyosarcoma Study guidelines.

The median age at diagnosis was 6 (range, 0.5-17) years.

Primary sites of rhabdomyosarcoma included: the head and neck (n=8; 6 classified as cranial parameningeal), genitourinary (3), extremity (3), pelvis (3), and trunk (2).

Thirteen (68%) had embryonal and six (32%) had alveolar histology.

Two, 2, 9, and 6 were classified as belonging to Intergroup Rhabdomyosarcoma Study groups 1, 2, 3, and 4, respectively.

In non-metastatic cases (Intergroup Rhabdomyosarcoma Study groups 1-3), the 5-year overall survival rate was 66% (95% confidence interval, 39-93%) and in metastatic cases (group 4) it was 17% (0-46%).

CONCLUSIONS: Treatment outcome of rhabdomyosarcoma in this cohort of Chinese children was less favourable than that reported in international studies.

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(PMID = 18382018.001).

[ISSN] 1024-2708

[Journal-full-title] Hong Kong medical journal = Xianggang yi xue za zhi

[ISO-abbreviation] Hong Kong Med J

[Language] ENG

[Publication-type] Journal Article

[Publication-country] China

   

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[Title] Sentinel node biopsy in pediatric soft tissue sarcomas of extremities.

Our experience with sentinel node biopsy in soft tissue sarcomas of extremities in children is reported.

The diagnosis was rhabdomyosarcoma in 5 and other soft tissue sarcomas in 12: Ewing/PNET sarcoma 6, epithelioid sarcoma 1, malignant peripheral-nerve-sheath tumor 1, undifferentiated sarcoma 1, myxoid liposarcoma 2, adult-type fibrosarcoma 1.

Nodes were positive for metastasis in two patients with alveolar rhabdomyosarcoma and undifferentiated sarcoma.

No further lymph node metastases were recognized either at diagnosis or during the follow-up (6-78 months).

It could be an alternative to aggressive or random biopsies for extremity rhabdomyosarcoma and it can contribute to define those non-rhabdomyosarcoma soft tissue sarcomas that spread to regional nodes.

[MeSH-major] Extremities. Rhabdomyosarcoma / pathology

[MeSH-minor] Adolescent. Child. Child, Preschool. Female. Humans. Male. Neoplasm Metastasis / pathology. Sentinel Lymph Node Biopsy. Soft Tissue Neoplasms / pathology. Young Adult

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(PMID = 18819127.001).

[ISSN] 1545-5017

[Journal-full-title] Pediatric blood & cancer

[ISO-abbreviation] Pediatr Blood Cancer

[Language] eng

[Publication-type] Evaluation Studies; Journal Article; Research Support, Non-U.S. Gov't

[Publication-country] United States

   

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[Title] [Metastatic alveolar soft tissue sarcoma of the central nervous system: a clinicopathological analysis of four cases].

BACKGROUND AND OBJECTIVE: Metastatic alveolar soft tissue sarcoma (ASTS) of the central nervous system is rare and is easy to be misdiagnosed as other primary tumors of central nervous system.

This study was to analyze the clinical and pathological features of four patients with ASTS of the central nervous system and to clarify their differential diagnosis as well as prognosis.

The tumor cells had clear or eosinophilic cytoplasm and prominent nucleoli, arranged in alveolar structures, which were surrounded by delicate blood sinuses.

CONCLUSION: ASTS of the central nervous system was mostly metastatic and should be differentiated from other CNS tumors such as meningioma, melonocytic tumor, rhabdomyosarcoma and paraganglioma.

[MeSH-major] Cranial Fossa, Posterior. Sarcoma, Alveolar Soft Part / pathology. Sarcoma, Alveolar Soft Part / secondary. Skull Base Neoplasms / pathology. Skull Base Neoplasms / secondary

[MeSH-minor] Actins / metabolism. Adult. Desmin / metabolism. Diagnosis, Differential. Female. Follow-Up Studies. Humans. Male. Meningeal Neoplasms / diagnosis. Meningioma / diagnosis. Neoplasm Recurrence, Local. Paraganglioma / diagnosis. Prognosis. Rhabdomyosarcoma / diagnosis. S100 Proteins / metabolism. Survival Rate

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(PMID = 19895745.001).

[Journal-full-title] Ai zheng = Aizheng = Chinese journal of cancer

[ISO-abbreviation] Ai Zheng

[Language] chi

[Publication-type] English Abstract; Journal Article

[Publication-country] China

[Chemical-registry-number] 0 / Actins; 0 / Desmin; 0 / S100 Proteins

   

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RESULTS: Nine children were included aged 0.9-3.8 years (embryonal RMS in six, and alveolar, undifferentiated or nonclassified in one each).

[MeSH-major] Protons / therapeutic use. Rhabdomyosarcoma / radiotherapy. Soft Tissue Neoplasms / radiotherapy

[MeSH-minor] Adolescent. Age Factors. Child, Preschool. Combined Modality Therapy. Feasibility Studies. Female. Head and Neck Neoplasms / radiotherapy. Humans. Infant. Infant, Newborn. Male. Meningeal Neoplasms / radiotherapy. Orbital Neoplasms / radiotherapy. Prospective Studies. Prostatic Neoplasms / radiotherapy. Radiotherapy Planning, Computer-Assisted. Rhabdomyosarcoma, Alveolar / radiotherapy. Rhabdomyosarcoma, Embryonal / radiotherapy. Survival Analysis. Treatment Outcome

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(PMID = 17072523.001).

[ISSN] 0179-7158

[Journal-full-title] Strahlentherapie und Onkologie : Organ der Deutschen Röntgengesellschaft ... [et al]

[ISO-abbreviation] Strahlenther Onkol

[Language] ger

[Publication-type] Comparative Study; English Abstract; Evaluation Studies; Journal Article

[Publication-country] Germany

[Chemical-registry-number] 0 / Protons

   

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[Title] Effects of standard chemotherapy on tumor growth and regulation of multidrug resistance genes and proteins in childhood rhabdomyosarcoma.

The prognosis of rhabdomyosarcoma (RMS) in advanced stages is still sobering.

The aim of this study was to investigate the development of multidrug resistance in cell lines and in xenografts of alveolar and embryonal RMS treated according to the German Soft Tissue Sarcoma Study (CWS).

Alveolar and embryonal RMS cell lines were treated with Vincristine, Topotecan, Carboplatin, Actinomycin D, or Ifosfamide.

Nude mice (NMRI nu/nu, n = 10 per group) underwent xenotransplantation of human embryonal or alveolar RMS.

In the cell lines, an up-regulation of MDR-1 gene was found in alveolar rhabdomyosarcoma.

In embryonal rhabdomyosarcoma, an up-regulation of LRP and MRP was found.

Standard chemotherapy of alveolar rhabdomyosarcoma resulted in a significant reduction of tumor growth (P < 0.05) in all groups.

In embryonal rhabdomyosarcoma strongest effects were found after treatment with Ifosfamide, Vincristine and Carboplatin (P < 0.05).

RT-PCR revealed a MDR1-dependent mechanism in alveolar rhabdomyosarcoma.

In embryonal rhabdomyosarcoma, MDR1 occurred to a lower degree.

[MeSH-major] Drug Resistance, Neoplasm / drug effects. Gene Expression Regulation, Neoplastic / drug effects. Genes, MDR / genetics. Multidrug Resistance-Associated Proteins / drug effects. Rhabdomyosarcoma, Alveolar / drug therapy. Rhabdomyosarcoma, Embryonal / drug therapy. Soft Tissue Neoplasms / drug therapy

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(PMID = 17211591.001).

[ISSN] 0179-0358

[Journal-full-title] Pediatric surgery international

[ISO-abbreviation] Pediatr. Surg. Int.

[Language] eng

[Publication-type] Journal Article; Research Support, Non-U.S. Gov't

[Publication-country] Germany

[Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Multidrug Resistance-Associated Proteins; 0 / Neoplasm Proteins

   

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[Title] Sclerosing pseudovascular rhabdomyosarcoma-immunohistochemical, ultrastructural, and genetic findings indicating a distinct subtype of rhabdomyosarcoma.

Sclerosing (pseudovascular) rhabdomyosarcoma in adults has been described as a rare variant of rhabdomyosarcoma characterized by extensive hyaline fibrosis and pseudovascular growth patterns.

We describe another case of this rhabdomyosarcoma subtype including ultrastructural and genetic findings-the lesion presented in a 62-year-old male patient in the left lower leg.

The tumor was located within the deep soft tissue with maximum diameter of 11.8 cm and skin ulceration.

Reciprocal translocations t(1;13) and t(2;13)(q35;q14) which are characteristic of alveolar rhabdomyosarcoma could be excluded.

These findings, while showing a relation to other rhabdomyosarcoma subtypes, represent a relatively circumscribed genetic defect pattern in sclerosing (pseudovascular) rhabdomyosarcoma that is somewhat different from patterns described in most other rhabdomyosarcoma subtypes.

Further studies should concentrate on the identification of genes especially on chromosomal region 10q22 to elucidate more aspects in the pathogenesis of this rhabdomyosarcoma subtype.

[MeSH-major] Chromosome Aberrations. Rhabdomyosarcoma / pathology. Soft Tissue Neoplasms / pathology

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(PMID = 17016719.001).

[ISSN] 0945-6317

[Journal-full-title] Virchows Archiv : an international journal of pathology

[ISO-abbreviation] Virchows Arch.

[Language] eng

[Publication-type] Case Reports; Journal Article

[Publication-country] Germany

   

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[Title] Advanced alveolar rhabdomyosarcoma of the uterus: a case report.

Alveolar rhabdomyosarcoma is an uncommon malignant soft tissue tumour rarely found in the female genital tract and carries a very poor prognosis especially in adults.

The cervical, uterine and omental biopsies were all diagnosed alveolar rhabdomyosarcoma.

While the optimal management of this rare tumour is unknown, early recognition and diagnosis, and a prompt multimodality treatment approach of surgery, chemotherapy and radiotherapy offers the best chance of cure.

[MeSH-major] Omentum / pathology. Peritoneal Neoplasms / pathology. Rhabdomyosarcoma, Alveolar / pathology. Uterine Neoplasms / pathology

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(PMID = 20687274.001).

[ISSN] 1118-4841

[Journal-full-title] African journal of reproductive health

[ISO-abbreviation] Afr J Reprod Health

[Language] eng

[Publication-type] Case Reports; Journal Article

[Publication-country] Nigeria

[Chemical-registry-number] 0 / Oncogene Proteins, Fusion; 0 / PAX3-FKHR fusion protein, human

   

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[Title] A metastatic alveolar rhabdomyosarcoma of the hand.

[MeSH-major] Bone Neoplasms / secondary. Fingers. Rhabdomyosarcoma, Alveolar / secondary. Soft Tissue Neoplasms / pathology

[MeSH-minor] Adolescent. Diagnostic Errors. Fatal Outcome. Humans. Magnetic Resonance Imaging. Male. Myoma / diagnosis. Positron-Emission Tomography

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(PMID = 16200631.001).

[ISSN] 1545-5009

[Journal-full-title] Pediatric blood & cancer

[ISO-abbreviation] Pediatr Blood Cancer

[Language] eng

[Publication-type] Case Reports; Letter

[Publication-country] United States

   

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[Title] The CXCR4-SDF1alpha axis is a critical mediator of rhabdomyosarcoma metastatic signaling induced by bone marrow stroma.

Rhabdomyosarcoma (RMS) is the most common malignant soft-tissue tumor of childhood.

CXCR4 was expressed in RMS cell lines and primary tumors, with higher expression in alveolar subtype RMS.

[MeSH-major] Chemokine CXCL12 / metabolism. Neoplasm Invasiveness / physiopathology. Receptors, CXCR4 / metabolism. Rhabdomyosarcoma / pathology. Signal Transduction / physiology

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(PMID = 17768666.001).

[ISSN] 0262-0898

[Journal-full-title] Clinical & experimental metastasis

[ISO-abbreviation] Clin. Exp. Metastasis

[Language] eng

[Publication-type] Journal Article; Research Support, Non-U.S. Gov't

[Publication-country] Netherlands

[Chemical-registry-number] 0 / CXCR4 protein, human; 0 / Chemokine CXCL12; 0 / Culture Media, Conditioned; 0 / Receptors, CXCR4

   

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[Title] Alveolar rhabdomyosarcoma of the extremity and nodal metastasis: Is the in-transit lymphatic system at risk?

Alveolar rhabdomyosarcoma (RMS) of the extremity is not infrequently associated with regional node metastasis.

In this report we describe two patients with alveolar RMS of the lower extremity with inguinal metastasis at presentation.

The in-transit lymphatics can be a site of failure in children with alveolar RMS of the extremity and nodal involvement.

[MeSH-major] Lymphatic Metastasis / physiopathology. Rhabdomyosarcoma, Alveolar / secondary. Soft Tissue Neoplasms / pathology

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[Copyright] (c) 2009 Wiley-Liss, Inc.

(PMID = 19711439.001).

[ISSN] 1545-5017

[Journal-full-title] Pediatric blood & cancer

[ISO-abbreviation] Pediatr Blood Cancer

[Language] eng

[Publication-type] Case Reports; Journal Article

[Publication-country] United States

[Chemical-registry-number] 0 / Benzenesulfonates; 0 / Phenylurea Compounds; 0 / Pyridines; 1CC1JFE158 / Dactinomycin; 24R60NVC41 / cositecan; 25X51I8RD4 / Niacinamide; 5J49Q6B70F / Vincristine; 6PLQ3CP4P3 / Etoposide; 7673326042 / irinotecan; 7GR28W0FJI / Dacarbazine; 85622-93-1 / temozolomide; 8N3DW7272P / Cyclophosphamide; 9ZOQ3TZI87 / sorafenib; UM20QQM95Y / Ifosfamide; XT3Z54Z28A / Camptothecin

   

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[Title] Expression of insulin-like growth factor pathway proteins in rhabdomyosarcoma: IGF-2 expression is associated with translocation-negative tumors.

Recent studies have shown a significant involvement of insulin-like growth factor (IGF) signaling components in the pathogenesis of rhabdomyosarcoma (RMS).

The present study utilized immunohistochemistry to determine the expression patterns of IGF1, IGF2, IGF binding protein 2 (IGFBP2), IGF receptor 1 (IGF1R), and IGF receptor 2 (IGF2R) in 24 embryonal RMS (ERMS) and 8 alveolar RMS (ARMS).

[MeSH-major] Insulin-Like Growth Factor II / metabolism. Rhabdomyosarcoma / metabolism. Soft Tissue Neoplasms / metabolism. Translocation, Genetic / genetics

[MeSH-minor] Biomarkers, Tumor / metabolism. Cell Line, Tumor. Fluorescent Antibody Technique, Direct. Forkhead Transcription Factors / genetics. Forkhead Transcription Factors / metabolism. Gene Expression Profiling. Gene Expression Regulation, Neoplastic / genetics. Humans. Paired Box Transcription Factors / genetics. Paired Box Transcription Factors / metabolism. RNA, Messenger / metabolism. RNA, Neoplasm / analysis. Tissue Array Analysis

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(PMID = 18788888.001).

[ISSN] 1093-5266

[Journal-full-title] Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society

[ISO-abbreviation] Pediatr. Dev. Pathol.

[Language] eng

[Publication-type] Journal Article; Research Support, Non-U.S. Gov't

[Publication-country] United States

[Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Forkhead Transcription Factors; 0 / PAX3 protein, human; 0 / Paired Box Transcription Factors; 0 / RNA, Messenger; 0 / RNA, Neoplasm; 67763-97-7 / Insulin-Like Growth Factor II

   

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Rhabdomyosarcoma is the most common soft tissue tumor of childhood, frequently presenting in the head and neck, genitourinary tract, or extremities.

We present a case of rhabdomyosarcoma in which an 18-year-old woman presented with abrupt onset unilateral hearing loss, tinnitus, dysarthria, dysphagia, and a new painless red bump on the palate.

With an alveolar subtype and older age, both predictors of poor prognosis, early recognition of disease of these symptoms is vital.

[MeSH-major] Deglutition Disorders / etiology. Dysarthria / etiology. Hearing Loss, Sensorineural / etiology. Palatal Neoplasms / diagnosis. Rhabdomyosarcoma, Alveolar / diagnosis. Tinnitus / etiology

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(PMID = 19687710.001).

[ISSN] 1535-1815

[Journal-full-title] Pediatric emergency care

[ISO-abbreviation] Pediatr Emerg Care

[Language] eng

[Publication-type] Case Reports; Journal Article

[Publication-country] United States

   

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[Title] Clinico-epidemiological analysis of orofacial rhabdomyosarcoma in a Nigerian population.

Rhabdomyosarcoma (RMS) is a malignant soft tissue neoplasm, with varying degrees of striated muscle cell differentiation and a relative predilection for the head and neck region.

Embryonal and alveolar subtypes were mainly found in the maxilla.

[MeSH-major] Facial Neoplasms / epidemiology. Head and Neck Neoplasms / epidemiology. Mouth Neoplasms / epidemiology. Neoplasms, Multiple Primary / epidemiology. Rhabdomyosarcoma / epidemiology

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(PMID = 19153030.001).

[ISSN] 1399-0020

[Journal-full-title] International journal of oral and maxillofacial surgery

[ISO-abbreviation] Int J Oral Maxillofac Surg

[Language] eng

[Publication-type] Journal Article

[Publication-country] Denmark

   

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[Title] Alveolar rhabdomyosarcoma of the hand in a 2 year-old child.

[MeSH-major] Hand / surgery. Rhabdomyosarcoma, Alveolar / surgery. Soft Tissue Neoplasms / surgery

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(PMID = 17049698.001).

[ISSN] 1753-1934

[Journal-full-title] The Journal of hand surgery, European volume

[ISO-abbreviation] J Hand Surg Eur Vol

[Language] eng

[Publication-type] Case Reports; Letter

[Publication-country] Scotland