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[Title] Defining the cooperative genetic changes that temporally drive alveolar rhabdomyosarcoma.

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

Despite advances in therapy, patients with a histologic variant of RMS known as alveolar (aRMS) have a 5-year survival rate of <30%.

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(PMID = 19047133.001).

[ISSN] 1538-7445

[Journal-full-title] Cancer research

[ISO-abbreviation] Cancer Res.

[Language] ENG

[Grant] United States / NICHD NIH HHS / HD / HD043494-06; United States / NICHD NIH HHS / HD / 5K12-HD043494; United States / NCI NIH HHS / CA / CA094184-07; United States / NICHD NIH HHS / HD / K12 HD043494-05; United States / NCI NIH HHS / CA / R01-CA94184; United States / NCI NIH HHS / CA / R01 CA094184; United States / NICHD NIH HHS / HD / K12 HD043494; United States / NICHD NIH HHS / HD / K12 HD043494-06; United States / NCI NIH HHS / CA / R01 CA094184-07; United States / NICHD NIH HHS / HD / HD043494-05

[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 / Cyclin-Dependent Kinase Inhibitor p16; 0 / Oncogene Proteins, Fusion; 0 / PAX3-FKHR fusion protein, human

[Other-IDs] NLM/ NIHMS73714; NLM/ PMC2593800

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Phosphorylation profiles of protein kinases in alveolar and embryonal rhabdomyosarcoma.

Rhabdomyosarcoma is the most common pediatric soft-tissue sarcoma, which includes two major subtypes, alveolar and embryonal rhabdomyosarcoma.

However, the oncogenic process of rhabdomyosarcoma involves multi-stages of signaling protein dysregulation characterized by prolonged activation of tyrosine and serine/threonine kinases.

To better understand this protein dysregulation, we evaluated the phosphorylation profiles of multiple tyrosine and serine/threonine kinases to identify whether these protein kinases are activated in rhabdomyosarcoma.

We applied immunohistochemistry with phospho-specific antibodies to examine phosphorylation levels of selected receptor and non-receptor tyrosine kinases, mammalian target of rapamycin (mTOR), p70S6K, and protein kinase C (PKC) isozymes on alveolar and embryonal rhabdomyosarcoma tissue microarray slides.

Our results showed that the phosphorylation levels of these kinases are elevated in some rhabdomyosarcoma tissues compared to normal tissues.

Phosphorylation levels of receptor and non-receptor tyrosine kinases are elevated between 26 and 68% in alveolar rhabdomyosarcoma and between 24 and 71% in embryonal rhabdomyosarcoma, respectively, compared to normal tissues.

In addition, phosphorylation levels of mTOR and its downstream targets, p70S6K, S6, and 4EBP1, are increased between 50 and 72% in both subtypes of rhabdomyosarcoma.

Further, phosphorylation levels of PKCalpha, PKCdelta, PKCtheta, and PKCzeta/lambda are upregulated between 57 and 69% in alveolar rhabdomyosarcoma and between 43 and 72% in embryonal rhabdomyosarcoma.

This is the first report to create a phosphorylation profile of tyrosine and serine/threonine kinases involved in the mTOR and PKC pathways of alveolar and embryonal rhabdomyosarcoma.

[MeSH-major] Protein-Serine-Threonine Kinases / analysis. Protein-Tyrosine Kinases / analysis. Rhabdomyosarcoma, Alveolar / enzymology. Rhabdomyosarcoma, Embryonal / enzymology

[MeSH-minor] Adaptor Proteins, Signal Transducing / analysis. Adolescent. Child. Child, Preschool. Female. Humans. Immunohistochemistry. Infant. Infant, Newborn. Isoenzymes / analysis. Male. Neoplasm Staging. Phosphoproteins / analysis. Phosphorylation. Pilot Projects. Protein Kinase C / analysis. Protein Kinases / analysis. Receptor Protein-Tyrosine Kinases / analysis. Ribosomal Protein S6 / analysis. Ribosomal Protein S6 Kinases, 70-kDa / analysis. TOR Serine-Threonine Kinases. Tissue Array Analysis

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(PMID = 17585318.001).

[ISSN] 0893-3952

[Journal-full-title] Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc

[ISO-abbreviation] Mod. Pathol.

[Language] eng

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

[Publication-country] United States

[Chemical-registry-number] 0 / Adaptor Proteins, Signal Transducing; 0 / EIF4EBP1 protein, human; 0 / Isoenzymes; 0 / Phosphoproteins; 0 / Ribosomal Protein S6; EC 2.7.- / Protein Kinases; EC 2.7.1.1 / MTOR protein, human; EC 2.7.1.1 / TOR Serine-Threonine Kinases; EC 2.7.10.1 / Protein-Tyrosine Kinases; EC 2.7.10.1 / Receptor Protein-Tyrosine Kinases; EC 2.7.11.1 / Protein-Serine-Threonine Kinases; EC 2.7.11.1 / Ribosomal Protein S6 Kinases, 70-kDa; EC 2.7.11.13 / Protein Kinase C

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[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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[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Childhood rhabdomyosarcoma in Ibadan, Nigeria: 1984-2003.

BACKGROUND: Rhabdomyosarcoma is the most common soft tissue sarcoma in children under 15 years of age.

AIM: To describe the pattern of childhood rhabdomyosarcoma in Ibadan with respect to demography, morphology and tumour site.

All histologically confirmed cases of rhabdomyosarcoma in children under 15 years of age seen at the University College Hospital Ibadan between 1984 and 2003 were included.

Other subtypes were alveolar (13.2%), pleomorphic (4.4%) and rhabdomyosarcoma 'not otherwise specified' (20.9%).

The majority (50.6%) of tumours were in the head and neck region and the common primary sites were soft tissue of the head, face (24.2%) and orbit (14.3%).

Other sites included soft tissue of the pelvis (11.0%), genito-urinary tract (9.9%) and abdomen (9.9%).

CONCLUSION: The pattern of rhabdomyosarcoma in Nigeria is similar to that in the United States and Europe, except for the rarity of parameningeal sites and extremities.

There is a need for larger descriptive studies on childhood rhabdomyosarcoma in Africa.

[MeSH-major] Rhabdomyosarcoma / epidemiology. Soft Tissue Neoplasms / epidemiology

[MeSH-minor] Adolescent. Age Distribution. Child. Child, Preschool. Female. Head and Neck Neoplasms / epidemiology. Humans. Infant. Infant, Newborn. Male. Nigeria / epidemiology. Registries. Retrospective Studies. Rhabdomyosarcoma, Alveolar / epidemiology. Rhabdomyosarcoma, Alveolar / pathology. Rhabdomyosarcoma, Embryonal / epidemiology. Rhabdomyosarcoma, Embryonal / pathology

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(PMID = 17132301.001).

[ISSN] 0272-4936

[Journal-full-title] Annals of tropical paediatrics

[ISO-abbreviation] Ann Trop Paediatr

[Language] eng

[Publication-type] Journal Article

[Publication-country] England

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Expression and genomic status of EGFR and ErbB-2 in alveolar and embryonal rhabdomyosarcoma.

EGFR and ErbB-2 expression has been observed in rhabdomyosarcoma cell lines but not analyzed systematically in rhabdomyosarcoma tumors.

Tissue microarray sections representing 66 rhabdomyosarcoma tumors (34 embryonal rhabdomyosarcoma, 32 alveolar rhabdomyosarcoma) were surveyed by immunohistochemistry using antibodies specific for EGFR and ErbB-2.

Expression of ErbB-2 was identified in 22/66 (33%) cases and tended to be more frequent in the alveolar subtype (13/32, 41%, vs 9/34, 26%, P=0.30).

Coexpression of EGFR and ErbB-2 was identified in eight tumors, of which six were embryonal rhabdomyosarcoma.

Furthermore, analysis of 11 additional rhabdomyosarcoma tumors (six alveolar; five embryonal) revealed no evidence of mutations in EGFR exons 18, 19, 20, and 21.

In summary, expression of EGFR and/or ErbB-2 is detected in a sizeable subset of rhabdomyosarcoma tumors without evidence of EGFR or ErbB-2 amplification or mutations in the EGFR tyrosine kinase domain.

[MeSH-major] Receptor, Epidermal Growth Factor / metabolism. Receptor, ErbB-2 / metabolism. Rhabdomyosarcoma, Alveolar / metabolism. Rhabdomyosarcoma, Embryonal / metabolism. Soft Tissue Neoplasms / metabolism

[MeSH-minor] Adolescent. Adult. Child. Child, Preschool. DNA Mutational Analysis. DNA, Neoplasm / analysis. Female. Gene Dosage. Humans. Immunoenzyme Techniques. In Situ Hybridization, Fluorescence. Infant. Male. Tissue Array Analysis

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[Copyright] Published online 26 May 2006.

(PMID = 16729016.001).

[ISSN] 0893-3952

[Journal-full-title] Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc

[ISO-abbreviation] Mod. Pathol.

[Language] eng

[Grant] United States / NCI NIH HHS / CA / CA-023099

[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 / DNA, Neoplasm; EC 2.7.10.1 / Receptor, Epidermal Growth Factor; EC 2.7.10.1 / Receptor, ErbB-2

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] [Diagnosis of micrometastases of alveolar rhabdomyosarcoma].

Alveolar rhabgomyosarcoma is a highly malignant, small blue cell pediatric soft tissue tumor.

Identification of micrometastases in alveolar rhabdomyosarcoma is important because the poor prognosis associated with this subgroup necessitates a modified therapeutic regimen.

Since the obtained lymph node specimen can be very small; rhabdomyosarcoma cells are not easily detected using conventional histological methods.

To assess the value of myogenin staining in the diagnosis of micrometastases in alveolar rhabdomyosarcoma, the authors examined 36 lymph nodes from children bearing this tumor.

The PAX3/7-FKHR gene fusion that resulted from chromosomal translocation in alveolar rhabdomyosarcoma provided potential molecular diagnostic markers.

Thirty-six lymph nodes were examined and of them 17 lymph nodes had PAX3/7-FKHR fusion transcripts of alveolar rhadomyosarcoma cells.

The study demonstrates that molecular RT-PCR detection of micrometastases is the most sensitive method for diagnosing alveolar rhabdomyosarcoma.

[MeSH-major] Biomarkers, Tumor / biosynthesis. Eye Proteins / biosynthesis. Forkhead Transcription Factors / biosynthesis. Homeodomain Proteins / biosynthesis. Oncogene Proteins, Fusion / biosynthesis. Rhabdomyosarcoma, Alveolar / diagnosis. Rhabdomyosarcoma, Alveolar / metabolism. Soft Tissue Neoplasms / diagnosis. Soft Tissue Neoplasms / metabolism. Transcription Factors / biosynthesis

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(PMID = 18540440.001).

[ISSN] 0004-1955

[Journal-full-title] Arkhiv patologii

[ISO-abbreviation] Arkh. Patol.

[Language] rus

[Publication-type] English Abstract; Journal Article

[Publication-country] Russia (Federation)

[Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Eye Proteins; 0 / FOXO1 protein, human; 0 / Forkhead Transcription Factors; 0 / Homeodomain Proteins; 0 / Oncogene Proteins, Fusion; 0 / RAX protein, human; 0 / RNA, Neoplasm; 0 / Transcription Factors

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Differential expression of Snail1 transcription factor and Snail1-related genes in alveolar and embryonal rhabdomyosarcoma subtypes.

Rhabdomyosarcoma (RMS) represents the most common sarcoma of soft tissue among children.

Two main RMS subtypes are alveolar (ARMS) and embryonal (ERMS).

The major goal of this study was to find differentially expressed genes between RMS subtypes that could explain higher metastatic potential in ARMS and would be useful for the differential diagnosis.

Using RQ-PCR analysis we compared expression of Snail1 and Snail-related genes among 7 ARMS and 8 ERMS patients' samples obtained from the primary tumors and among 2 alveolar and 2 embryonal cell lines.

Our results show that Snail1 is highly expressed both in ARMS patients' samples and the alveolar cell lines.

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(PMID = 21478114.001).

[ISSN] 1897-5631

[Journal-full-title] Folia histochemica et cytobiologica

[ISO-abbreviation] Folia Histochem. Cytobiol.

[Language] ENG

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

[Publication-country] Poland

[Chemical-registry-number] 0 / Transcription Factors; 0 / beta Catenin; 0 / snail family transcription factors; EC 3.4.24.24 / Matrix Metalloproteinase 2; EC 3.4.24.35 / Matrix Metalloproteinase 9

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Assessing the use of FDG-PET in the detection of regional and metastatic nodes in alveolar rhabdomyosarcoma of extremities.

Alveolar rhabdomyosarcoma (ARS) accounts for 20% to 30% of childhood rhabdomyosarcoma and is known to have a worse prognosis than embryonal rhabdomyosarcoma.

Metastatic disease is more frequent in patients with alveolar tumors and these children with metastatic disease fare poorly, with a 5-year survival between 20% and 30%.

The value of FDG in patients with soft tissue sarcomas has been demonstrated in several series, but none specifically in ARS.

All the 3 patients we present had focally increased tracer uptake in nodal stations on a pretherapy PET performed at diagnosis.

Tissue confirmation available in 2 patients was negative in 1 patient and positive for metastatic nodal spread in the other.

[MeSH-major] Extremities / pathology. Fluorodeoxyglucose F18. Lymphoma, Non-Hodgkin / diagnosis. Positron-Emission Tomography / methods. Rhabdomyosarcoma, Alveolar / diagnosis. Soft Tissue Neoplasms / diagnosis

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(PMID = 16825990.001).

[ISSN] 1077-4114

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

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

[Language] eng

[Publication-type] Case Reports; Journal Article

[Publication-country] United States

[Chemical-registry-number] 0Z5B2CJX4D / Fluorodeoxyglucose F18

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[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

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[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

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Adult sclerosing rhabdomyosarcoma: cytogenetic link with embryonal rhabdomyosarcoma.

Rhabdomyosarcomas are classified into three well-defined categories: embryonal, alveolar and pleomorphic rhabdomyosarcoma.

Recently, seven cases of an unusual adult type of rhabdomyosarcoma with a prominent hyaline sclerosis have been described.

We report the hitherto unreported cytogenetic changes of an adult sclerosing rhabdomyosarcoma.

A 79-year-old woman underwent an amputation for a rapidly growing soft tissue mass in the anterior compartment of the right lower leg.

Using fluorescent in situ hybridization (FISH) analysis, the tumor cells were negative for FOXO1A-disrupting translocations specific for alveolar rhabdomyosarcoma.

The chromosomal composition of malignant cells resembled the pattern of numerical changes frequently observed in embryonal rhabdomyosarcoma, suggesting a close relationship of an adult sclerosing rhabdomyosarcoma with this entity.

[MeSH-major] Rhabdomyosarcoma / genetics. Rhabdomyosarcoma, Embryonal / genetics. Translocation, Genetic

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[Cites] Med Pediatr Oncol. 2001 Feb;36(2):259-67 [11452933.001]

[Cites] Hum Mol Genet. 1996 Jan;5(1):15-21 [8789435.001]

[Cites] Cancer Res. 1993 Nov 1;53(21):5108-12 [8221646.001]

[Cites] Mod Pathol. 2000 Sep;13(9):988-93 [11007039.001]

[Cites] Virchows Arch. 2000 Apr;436(4):305-11 [10834531.001]

[Cites] Cancer Genet Cytogenet. 1999 Mar;109(2):161-5 [10087953.001]

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[Cites] Genes Chromosomes Cancer. 2000 Apr;27(4):337-44 [10719362.001]

(PMID = 15660283.001).

[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

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Metastatic alveolar rhabdomyosarcoma in multiple endocrine neoplasia type 2A.

Rhabdomyosarcoma (RMS), the most common pediatric soft tissue sarcoma, accounts for 3% of childhood malignancies.

We describe a previously unreported association of MEN-2A with metastatic alveolar RMS and review the literature on associated hereditary cancer predisposition syndromes and current therapeutic options.

The diagnosis of RMS should prompt consideration of screening for familial genetic syndromes in certain patients.

[MeSH-major] Multiple Endocrine Neoplasia Type 2a / pathology. Rhabdomyosarcoma, Alveolar / secondary

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(PMID = 20533522.001).

[ISSN] 1545-5017

[Journal-full-title] Pediatric blood & cancer

[ISO-abbreviation] Pediatr Blood Cancer

[Language] eng

[Grant] United States / NCI NIH HHS / CA / T32 CA082086

[Publication-type] Case Reports; Journal Article

[Publication-country] United States

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Rhabdomyosarcoma of the extremities in adults.

Eight patients with an average age of 32.6 years (range: 21 to 75) who were treated for rhabdomyosarcomas on the extremities between 1991 and 2002 in a regional centre for the treatment of soft tissue sarcomas were studied retrospectively.

Histologically there were four alveolar, two pleomorphic, one embryonal and one anaplastic subtype.

Extremity rhabdomyosarcoma is a highly malignant tumour and our results are poor compared to the reported results in children.

[MeSH-major] Extremities. Rhabdomyosarcoma / therapy. Soft Tissue Neoplasms / therapy

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(PMID = 16768266.001).

[ISSN] 0001-6462

[Journal-full-title] Acta orthopaedica Belgica

[ISO-abbreviation] Acta Orthop Belg

[Language] eng

[Publication-type] Journal Article

[Publication-country] Belgium

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Rhabdomyosarcoma an ubiquitous pediatric tumour.

Rhabdomyosarcoma is the most common soft tissue tumor in children.

It occurs everywhere and its prognosis depends on the location and its histological type--embryonic or alveolar.

The new immunohistochemical markers desmin and myogenin in combination with molecular biological detection of specific translocations in alveolar rhabdomyosarcoma improved diagnostic capacities.

[MeSH-major] Rhabdomyosarcoma

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(PMID = 18727437.001).

[ISSN] 0004-1955

[Journal-full-title] Arkhiv patologii

[ISO-abbreviation] Arkh. Patol.

[Language] eng

[Publication-type] Lectures

[Publication-country] Russia (Federation)

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[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

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Fusions involving PAX and FOX genes in the molecular pathogenesis of alveolar rhabdomyosarcoma: recent advances.

Rhabdomyosarcoma is the most frequent soft tissue sarcoma in the pediatric population.

Two main histopathologic variants have been described, embryonal (ERMS) and alveolar (ARMS), which demonstrate clinical and genetic differences.

This review highlights recent advances in numerous areas of biomedical investigation that are providing new insights into the biology, molecular pathology, and translational science of ARMS: the identification of downstream targets of PAX3-FKHR and collaborating events in the process of tumorigenesis and metastasis; generation of animal models based on the gene fusion and collaborating events; development of new assays for diagnosis, prognosis, and detection of minimal disseminated disease; and exploration of immune recognition of this tumor and the fusion protein.

[MeSH-major] Oncogene Proteins, Fusion / genetics. Paired Box Transcription Factors / genetics. Rhabdomyosarcoma, Alveolar / pathology

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(PMID = 17311532.001).

[ISSN] 1566-5240

[Journal-full-title] Current molecular medicine

[ISO-abbreviation] Curr. Mol. Med.

[Language] eng

[Grant] United States / NCI NIH HHS / CA / CA64202; United States / NCI NIH HHS / CA / CA87812; United States / NCI NIH HHS / CA / CA89461

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

[Publication-country] Netherlands

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

[Number-of-references] 101

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Alterations of RB1 gene in embryonal and alveolar rhabdomyosarcoma: special reference to utility of pRB immunoreactivity in differential diagnosis of rhabdomyosarcoma subtype.

PURPOSE: Rhabdomyosarcoma (RMS), which is the most common pediatric soft tissue sarcoma, is classified into two major histologic subtypes, embryonal RMS (ERMS) and alveolar RMS (ARMS).

In addition, immunohistochemical pRB LI may have the potential to be a useful ancillary tool in the differential diagnosis of RMS subtypes.

[MeSH-major] Retinoblastoma Protein / biosynthesis. Retinoblastoma Protein / genetics. Rhabdomyosarcoma, Alveolar / diagnosis. Rhabdomyosarcoma, Alveolar / genetics. Rhabdomyosarcoma, Embryonal / diagnosis. Rhabdomyosarcoma, Embryonal / genetics

[MeSH-minor] Base Sequence. Diagnosis, Differential. Gene Expression. Humans. Immunohistochemistry. Mutation. Reverse Transcriptase Polymerase Chain Reaction

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(PMID = 18386058.001).

[ISSN] 0171-5216

[Journal-full-title] Journal of cancer research and clinical oncology

[ISO-abbreviation] J. Cancer Res. Clin. Oncol.

[Language] eng

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

[Publication-country] Germany

[Chemical-registry-number] 0 / Retinoblastoma Protein

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[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

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Unusual leptomeningeal dissemination in a child with extracranial metastatic alveolar rhabdomyosarcoma.

The authors describe a 6-year-old boy diagnosed with alveolar rhabdomyosarcoma located in the thigh, with distal metastases to lungs, bones, and bone marrow.

This case demonstrates the rapidity with which leptomeningeal spread of extracranial metastatic alveolar rhabdomyosarcoma can occur and underscores the importance of diagnostic lumbar puncture and brain radiological investigations at diagnosis, even when the tumors are not in the parameningeal location.

[MeSH-major] Bone Marrow Neoplasms / secondary. Brain Neoplasms / secondary. Lung Neoplasms / secondary. Meningeal Neoplasms / secondary. Rhabdomyosarcoma, Alveolar / secondary. Soft Tissue Neoplasms / pathology

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(PMID = 19657999.001).

[ISSN] 1521-0669

[Journal-full-title] Pediatric hematology and oncology

[ISO-abbreviation] Pediatr Hematol Oncol

[Language] eng

[Publication-type] Case Reports; Journal Article

[Publication-country] England

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[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

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Alveolar rhabdomyosarcoma on the external ear: a case report.

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

[MeSH-major] Ear Neoplasms / pathology. Rhabdomyosarcoma, Alveolar / pathology. Skin Neoplasms / pathology

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(PMID = 19208077.001).

[ISSN] 1600-0560

[Journal-full-title] Journal of cutaneous pathology

[ISO-abbreviation] J. Cutan. Pathol.

[Language] eng

[Publication-type] Case Reports; Journal Article

[Publication-country] Denmark

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Pax3-fkhr and pax7-fkhr fusion genes impact outcome of alveolar rhabdomyosarcoma in children.

Rhabdomyosarcoma is a highly malignant embryonic tumor of childhood.

In addition, fusion gene analysis is a helpful tool in differential diagnosis of poorly differentiated soft tissue tumors.

[MeSH-major] Forkhead Transcription Factors / genetics. PAX7 Transcription Factor / genetics. Paired Box Transcription Factors / genetics. Rhabdomyosarcoma, Alveolar / diagnosis. Rhabdomyosarcoma, Alveolar / genetics

[MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Female. Gene Fusion. Head and Neck Neoplasms / diagnosis. Head and Neck Neoplasms / genetics. Head and Neck Neoplasms / pathology. Humans. Infant. Male. Neoplasm Staging. Prognosis. Retrospective Studies. Survival Analysis. Translocation, Genetic. Urinary Bladder Neoplasms / diagnosis. Urinary Bladder Neoplasms / genetics. Urinary Bladder Neoplasms / pathology. Urogenital Neoplasms / diagnosis. Urogenital Neoplasms / genetics. Urogenital Neoplasms / pathology

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(PMID = 17613043.001).

[ISSN] 1551-3815

[Journal-full-title] Fetal and pediatric pathology

[ISO-abbreviation] Fetal Pediatr Pathol

[Language] eng

[Publication-type] Journal Article; Multicenter Study

[Publication-country] United States

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

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Credentialing a preclinical mouse model of alveolar rhabdomyosarcoma.

The highly aggressive muscle cancer alveolar rhabdomyosarcoma (ARMS) is one of the most common soft tissue sarcoma of childhood, yet the outcome for the unresectable and metastatic disease is dismal and unchanged for nearly three decades.

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(PMID = 19339268.001).

[ISSN] 1538-7445

[Journal-full-title] Cancer research

[ISO-abbreviation] Cancer Res.

[Language] ENG

[Grant] United States / NCI NIH HHS / CA / R01 CA074907-06; United States / NCI NIH HHS / CA / CA090438-06; United States / NCI NIH HHS / CA / CA064202-07; United States / NCI NIH HHS / CA / CA064202-13; United States / NCI NIH HHS / CA / R01 CA064202-08S1; United States / NCI NIH HHS / CA / P30CA54174; United States / NCI NIH HHS / CA / R01 CA064202-11; United States / NCI NIH HHS / CA / CA064202-08S1; United States / NCI NIH HHS / CA / R01 CA074907-09; United States / NCI NIH HHS / CA / K08 CA090438; United States / NCI NIH HHS / CA / CA074907-11; United States / NCI NIH HHS / CA / CA64202; United States / NCI NIH HHS / CA / R01 CA064202-06; United States / NCI NIH HHS / CA / R01 CA074907; United States / NCI NIH HHS / CA / CA064202-08; United States / NCI NIH HHS / CA / CA074907-07; United States / NCI NIH HHS / CA / R01 CA064202-14; United States / NCI NIH HHS / CA / R01 CA064202-13; United States / NCI NIH HHS / CA / R01 CA064202-07; United States / NCI NIH HHS / CA / R29 CA074907; United States / NCI NIH HHS / CA / CA074907-08; United States / NCI NIH HHS / CA / R01 CA064202-09; United States / NCI NIH HHS / CA / R01 CA064202; United States / NCI NIH HHS / CA / CA074907; United States / NCI NIH HHS / CA / CA074907-10A1; United States / NCI NIH HHS / CA / CA064202-11; United States / NCI NIH HHS / CA / CA074907-09; United States / NCI NIH HHS / CA / CA074907-06; United States / NCI NIH HHS / CA / R01 CA064202-08; United States / NCI NIH HHS / CA / R01 CA074907-11; United States / NCI NIH HHS / CA / CA064202-14; United States / NCI NIH HHS / CA / CA064202-10A1; United States / NCI NIH HHS / CA / R01 CA074907-07; United States / NCI NIH HHS / CA / P30 CA054174; United States / NCI NIH HHS / CA / CA064202-12; United States / NCI NIH HHS / CA / R01 CA074907-08; United States / NCI NIH HHS / CA / CA064202-06; United States / NCI NIH HHS / CA / R01 CA064202-12; United States / NCI NIH HHS / CA / K08 CA090438-06; United States / NCI NIH HHS / CA / R01 CA064202-10A1; United States / NCI NIH HHS / CA / CA064202-09; United States / NCI NIH HHS / CA / R01 CA074907-10A1

[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 / Cdkn2a protein, mouse; 0 / Cyclin-Dependent Kinase Inhibitor p16; 0 / Forkhead Transcription Factors; 0 / Foxo1 protein, mouse; 0 / Oncogene Proteins, Fusion; 0 / PAX3-FKHR fusion protein, human; 0 / Paired Box Transcription Factors; 0 / Tumor Suppressor Protein p53; 138016-91-8 / Pax3 protein, mouse

[Other-IDs] NLM/ NIHMS96027; NLM/ PMC2789740

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[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

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[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

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Synchronous bifocal alveolar rhabdomyosarcoma: a case report.

We report a case of bifocal rhabdomyosarcoma involving the hand and thigh in an 11-year-old female.

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

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(PMID = 16799004.001).

[ISSN] 0301-620X

[Journal-full-title] The Journal of bone and joint surgery. British volume

[ISO-abbreviation] J Bone Joint Surg Br

[Language] eng

[Publication-type] Case Reports; Journal Article

[Publication-country] England

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] The Fem1a gene is downregulated in Rhabdomyosarcoma.

Rhabdomyosarcoma (RMS) is the most common soft tissue neoplasm of children, and those metastatic at presentation have a poor prognosis.

We find that the human homolog, FEM1A, is downregulated in all of 8 different human RMS cell lines, including those derived from embryonal and alveolar RMS.

[MeSH-major] Cell Cycle Proteins / genetics. Down-Regulation / genetics. Gene Expression Regulation, Neoplastic / genetics. Rhabdomyosarcoma / genetics

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[Copyright] Copyright 2005 S. Karger AG, Basel.

(PMID = 16254458.001).

[ISSN] 1010-4283

[Journal-full-title] Tumour biology : the journal of the International Society for Oncodevelopmental Biology and Medicine

[ISO-abbreviation] Tumour Biol.

[Language] eng

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

[Publication-country] United States

[Chemical-registry-number] 0 / Cell Cycle Proteins; 0 / FEM1A protein, human; 0 / Fem1a protein, mouse

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[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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[Cites] Am J Pathol. 2001 Nov;159(5):1925-32 [11696453.001]

[Cites] Cancer. 1988 Nov 1;62(9):2060-5 [3167818.001]

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[Cites] Laryngoscope. 2005 Jan;115(1):48-50 [15630365.001]

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[Cites] Virchows Arch. 2005 Jan;446(1):64-7 [15660283.001]

(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

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[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

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Prevalence and clinical impact of anaplasia in childhood rhabdomyosarcoma : a report from the Soft Tissue Sarcoma Committee of the Children's Oncology Group.

BACKGROUND: Anapalsia is rare in childhood rhabdomyosarcoma and has not been included in the International Classification of Rhabdomyosarcoma (ICR).

A recent review of cases from the Soft Tissue Sarcoma Committee of the Children's Oncology Group (COG) suggests that anaplasia might be more common than previously reported and may impact clinical outcome.

METHODS: The prevalence of anaplasia (focal or diffuse) was prospectively assessed in 546 eligible cases who were registered in an Intergroup Rhabdomyosarcoma Study Group (IRSG) or COG therapeutic trial from 1995 through 1998.

Regardless of its distribution (focal or diffuse), on univariate analysis the presence of anaplasia negatively influenced the failure-free survival rate (63% vs 77% at 5 years) and overall survival (68% vs 82% at 5 years) rates in patients with embryonal rhabdomyosarcoma.

Anaplasia did not affect outcome in patients with alveolar tumors.

CONCLUSIONS: The incidence of anaplasia in patients with rhabdomyosarcoma is higher than previously described and may be of prognostic significance in children with intermediate-risk embryonal rhabdomyosarcoma.

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[Copyright] (c) 2008 American Cancer Society

[Cites] J Clin Oncol. 2001 Jun 15;19(12):3091-102 [11408506.001]

[Cites] Genes Chromosomes Cancer. 2002 Mar;33(3):310-21 [11807989.001]

[Cites] J Pediatr Hematol Oncol. 2001 May;23(4):215-20 [11846299.001]

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[Cites] Nat Genet. 1994 May;7(1):91-7 [8075648.001]

[Cites] J Clin Oncol. 1995 Mar;13(3):610-30 [7884423.001]

[Cites] Am J Pathol. 1996 May;148(5):1577-89 [8623926.001]

[Cites] Cancer. 1995 Sep 15;76(6):1073-85 [8625211.001]

[Cites] Am J Surg Pathol. 1996 Aug;20(8):909-20 [8712292.001]

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[Cites] Pediatr Dev Pathol. 1998 Nov-Dec;1(6):550-61 [9724344.001]

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[Cites] Clin Cancer Res. 2007 Nov 15;13(22 Pt 1):6593-602 [18006759.001]

[ErratumIn] Cancer. 2009 Jun 15;115(12):2806

(PMID = 18985676.001).

[ISSN] 0008-543X

[Journal-full-title] Cancer

[ISO-abbreviation] Cancer

[Language] ENG

[Grant] United States / NCI NIH HHS / CA / U10 CA098543-06; United States / NCI NIH HHS / CA / U10 CA098413-06; United States / NCI NIH HHS / CA / U10 CA098413; None / None / / U10 CA024507-25; United States / NCI NIH HHS / CA / U10 CA024507-26; United States / NCI NIH HHS / CA / U10 CA098543; None / None / / U10 CA098413-06; None / None / / U10 CA024507-26; United States / NCI NIH HHS / CA / U10 CA024507-25; None / None / / U10 CA098543-06; 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/ NIHMS107192; NLM/ PMC2727712

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[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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[Cites] J Clin Oncol. 1995 Mar;13(3):610-30 [7884423.001]

[Cites] Cancer. 1995 Sep 15;76(6):1073-85 [8625211.001]

[Cites] Diagn Mol Pathol. 1997 Apr;6(2):91-7 [9098647.001]

[Cites] JAMA. 1995 Feb 15;273(7):553-7 [7530783.001]

[Cites] Oncogene. 2001 Sep 10;20(40):5736-46 [11607823.001]

[Cites] J Clin Oncol. 2002 Jun 1;20(11):2672-9 [12039929.001]

[Cites] EMBO J. 1993 Dec;12(12):4481-7 [8223458.001]

[Cites] Cancer. 1958 Jan-Feb;11(1):181-99 [13500314.001]

(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

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[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

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Rhabdomyosarcoma of the urinary bladder in adults: predilection for alveolar morphology with anaplasia and significant morphologic overlap with small cell carcinoma.

Rhabdomyosarcoma (RMS) represents the most common malignant soft tissue tumor in children and adolescents with the urinary bladder representing a frequent site.

We report the clinicopathologic features of 5 bladder neoplasms with rhabdomyosarcomatous differentiation in adults and emphasize the differential diagnosis in the adult setting.

Three cases were of the alveolar subtype (1 admixed with embryonal histology) and 2 were RMS, not further classified.

In conclusion, (1) RMS of the urinary bladder in adults more commonly presents as a primitive round blue cell neoplasm that has significant morphologic and immunohistochemical overlap with small cell carcinoma of the bladder. (2) Although RMS in children generally have a botryoid embryonal histology with favorable outcome, bladder RMS in adults frequently demonstrates alveolar or unclassified histology, commonly with anaplasia, and have a uniformly aggressive clinical course.

[MeSH-major] Carcinoma, Small Cell / pathology. Rhabdomyosarcoma, Alveolar / pathology. Urinary Bladder Neoplasms / pathology

[MeSH-minor] Adult. Aged. Aged, 80 and over. Anaplasia. Biomarkers, Tumor / analysis. Cell Nucleus / pathology. Combined Modality Therapy. Desmin / analysis. Diagnosis, Differential. Fatal Outcome. Female. Humans. Male. Middle Aged. MyoD Protein / analysis. Myogenin / analysis. Synaptophysin / analysis

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(PMID = 18469707.001).

[ISSN] 1532-0979

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

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

[Language] eng

[Publication-type] Case Reports; Journal Article

[Publication-country] United States

[Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Desmin; 0 / MyoD Protein; 0 / MyoD1 myogenic differentiation protein; 0 / Myogenin; 0 / Synaptophysin

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] A case of alveolar rhabdomyosarcoma of the ethmoid sinus invading the orbit in an adult.

PURPOSE: A case study and literature review of alveolar rhabdomyosarcoma (RMS) in an adult.

A computed tomography (CT) scan revealed a destructive soft-tissue mass in the left ethmoid sinus with invasion of the left orbit and compression of the medial rectus muscle.

Endoscopic intranasal biopsy revealed alveolar RMS.

RESULTS: Immunohistochemical testing was positive for desmin, S-100, and smooth muscle actin (SMA), supporting the diagnosis of RMS.

CONCLUSIONS: Although rarely found in adults, RMS should be considered in the differential diagnosis of orbital tumors.

Immunohistochemical analysis plays an important role in the definitive diagnosis of RMS.

[MeSH-major] Ethmoid Sinus. Orbital Neoplasms / pathology. Paranasal Sinus Neoplasms / pathology. Rhabdomyosarcoma, Alveolar / pathology

[MeSH-minor] Biopsy. Diagnosis, Differential. Humans. Magnetic Resonance Imaging. Male. Middle Aged. Neoplasm Invasiveness. Tomography, X-Ray Computed

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[Cites] Cancer. 2001 Feb 15;91(4):794-803 [11241248.001]

[Cites] Virchows Arch Pathol Anat Physiol Klin Med. 1962;335:329-44 [14488094.001]

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[Cites] J Clin Oncol. 2001 Jun 15;19(12):3091-102 [11408506.001]

(PMID = 16768194.001).

[ISSN] 1011-8942

[Journal-full-title] Korean journal of ophthalmology : KJO

[ISO-abbreviation] Korean J Ophthalmol

[Language] eng

[Publication-type] Case Reports; Journal Article

[Publication-country] Korea (South)

[Other-IDs] NLM/ PMC2908821

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Validation of met as a therapeutic target in alveolar and embryonal rhabdomyosarcoma.

Rhabdomyosarcoma (RMS) is a highly malignant soft-tissue tumor of childhood deriving from skeletal muscle cells.

RMS can be classified in two major histologic subtypes: embryonal (ERMS) and alveolar (ARMS), the latter being characterized by the PAX3/7-FKHR translocation.

This property was lost in low serum but addition of hepatocyte growth factor/scatter factor (HGF/SF) rescued soft-agar growth.

Only Met(+/+) MEFs acquired anchorage-independent growth whereas PAX3-FKHR-transduced Met(D/D) cells were unable to form colonies in soft agar.

[MeSH-major] Proto-Oncogene Proteins / antagonists & inhibitors. Proto-Oncogene Proteins / physiology. Receptors, Growth Factor / antagonists & inhibitors. Receptors, Growth Factor / physiology. Rhabdomyosarcoma, Alveolar / therapy. Rhabdomyosarcoma, Embryonal / therapy

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(PMID = 16651427.001).

[ISSN] 0008-5472

[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 / FOXO1 protein, human; 0 / Forkhead Transcription Factors; 0 / Oncogene Proteins, Fusion; 0 / PAX3 protein, human; 0 / Paired Box Transcription Factors; 0 / Proto-Oncogene Proteins; 0 / RNA, Small Interfering; 0 / Receptors, Growth Factor; 67256-21-7 / Hepatocyte Growth Factor; EC 2.7.10.1 / MET protein, human; EC 2.7.10.1 / Proto-Oncogene Proteins c-met

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Incidence and outcomes of extremity soft-tissue sarcomas in children.

The median age at diagnosis was 12 y, but most patients were ≥10 y of age (72%, n = 842).

Most tumors were non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) (79%, n = 879).

The most common tissue of origin was muscle (43%, n = 474).

Most rhabdomyosarcoma (RMS) (n = 220) were alveolar type (n = 140).

Multivariate analysis identified RMS (HR 2.20, P < 0.001), nerve and muscle (not synovial sarcoma) tissue of origin (HR 2.26, P = 0.002, and HR 1.59, P = 0.036), regional or distant disease (HR 1.65, P = 0.011, and HR 5.96, P < 0.001, respectively), and lack of surgical intervention (HR 2.20, P < 0.001) as independent predictors of poor outcome.

[MeSH-major] Extremities. Sarcoma / epidemiology. Soft Tissue Neoplasms / epidemiology

[MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Female. Humans. Incidence. Infant. Infant, Newborn. Male. Multivariate Analysis. Rhabdomyosarcoma / epidemiology. Rhabdomyosarcoma / mortality. SEER Program

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[Copyright] Copyright © 2010 Elsevier Inc. All rights reserved.

(PMID = 20638678.001).

[ISSN] 1095-8673

[Journal-full-title] The Journal of surgical research

[ISO-abbreviation] J. Surg. Res.

[Language] eng

[Publication-type] Journal Article

[Publication-country] United States

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Complete second look operation and radiotherapy in locally advanced non-alveolar rhabdomyosarcoma in children: A report from the AIEOP soft tissue sarcoma committee.

BACKGROUND: To evaluate the effect of radiotherapy (RT) in association with complete second look operation, histologically confirmed, on outcome of patients with IRS Gr.III non-alveolar RMS.

Although the limited number of patients does not allow statistically significant conclusions, our experience suggests that RT may have a positive influence on local control for completely resected non-alveolar RMS.

[MeSH-major] Rhabdomyosarcoma / radiotherapy. Rhabdomyosarcoma / surgery. Second-Look Surgery

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[Copyright] (c) 2008 Wiley-Liss, Inc.

(PMID = 18668515.001).

[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

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Novel genes implicated in embryonal, alveolar, and pleomorphic rhabdomyosarcoma: a cytogenetic and molecular analysis of primary tumors.

Rhabdomyosarcoma, the most common pediatric soft tissue sarcoma, likely results from deregulation of the skeletal myogenesis program.

Using a combined approach of spectral karyotyping, array-based comparative genomic hybridization (CGH), and expression analysis, we examined 10 primary RMS tumors, including embryonal, alveolar, and the rare adult pleomorphic variant, to explore the involvement of different genes and genetic pathways in RMS tumorigenesis.

[MeSH-major] Biomarkers, Tumor / metabolism. Genetic Predisposition to Disease. Neoplasms / genetics. Rhabdomyosarcoma, Alveolar / genetics. Rhabdomyosarcoma, Alveolar / metabolism. Rhabdomyosarcoma, Embryonal / embryology. Rhabdomyosarcoma, Embryonal / genetics

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(PMID = 16790082.001).

[ISSN] 1476-5586

[Journal-full-title] Neoplasia (New York, N.Y.)

[ISO-abbreviation] Neoplasia

[Language] eng

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

[Publication-country] Canada

[Chemical-registry-number] 0 / Biomarkers, Tumor

[Other-IDs] NLM/ PMC1592451

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Primary ovarian rhabdomyosarcoma in children.

Although rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children, primary rhabdomyosarcomas of the ovary are extremely rare, with only eight well-documented pediatric cases previously reported in the literature.

We present two additional cases: an alveolar RMS originating in the right ovary with metastatic spread to the splenic flexure of the colon and to both lungs in a 13-year-old African American girl, and an embryonal RMS arising in the right ovary of a 6-year-old Caucasian girl with pre-operative intra-abdominal rupture and a malignant right pleural effusion.

[MeSH-major] Ovarian Neoplasms / diagnosis. Rhabdomyosarcoma / diagnosis

[MeSH-minor] Adolescent. Biopsy, Needle. Chemotherapy, Adjuvant. Child. Diagnosis, Differential. Female. Follow-Up Studies. Humans. Laparoscopy. Ovariectomy / methods. Tomography, X-Ray Computed

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[Cites] Cancer. 1997 Sep 15;80(6):1165-70 [9305719.001]

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(PMID = 18004572.001).

[ISSN] 0179-0358

[Journal-full-title] Pediatric surgery international

[ISO-abbreviation] Pediatr. Surg. Int.

[Language] eng

[Publication-type] Case Reports; Journal Article

[Publication-country] Germany

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Clinicopathologic features of rhabdomyosarcoma of gynecologic origin in adults.

Rhabdomyosarcoma (RMS) is the most common soft tissue tumor found in children.

Of the remaining 4 tumors, 2 were of alveolar (vulva) and 2 of pleomorphic (uterus, 1; fallopian tube, 1) histologic subtype.

[MeSH-major] Genital Neoplasms, Female / pathology. Rhabdomyosarcoma / pathology

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[CommentIn] Am J Surg Pathol. 2008 Jan;32(1):174 [18162788.001]

(PMID = 17325479.001).

[ISSN] 0147-5185

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

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

[Language] eng

[Publication-type] Journal Article

[Publication-country] United States

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[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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[CommentIn] NIH Guide Grants Contracts. 2011 Jun 17;:NOT-OD-11-084 [21714172.001]

[RetractionIn] Bois PR, Izeradjene K, Houghton PJ, Cleveland JL, Houghton JA, Grosveldz GC. J Cell Biol. 2007 May 7;177(3):563 [17485494.001]

[CommentIn] Fed Regist. 2011 Jun 9;76(111):33763-33764 [27737203.001]

(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

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

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

Here, we identify such a fusion protein breakpoint epitope in the PAX-FKHR fusion protein created by the t(2;13) translocation present in 80% of cases of alveolar rhabdomyosarcoma, a highly aggressive pediatric soft-tissue sarcoma.

These human peptide-specific CTL lyse human HLA-B7+ rhabdomyosarcoma tumor cells.

This epitope-enhanced peptide may serve as a candidate cancer vaccine for HLA-B7+ patients with alveolar rhabdomyosarcoma.

[MeSH-major] Epitopes / immunology. Forkhead Transcription Factors / immunology. Immunotherapy, Adoptive / methods. Oncogene Proteins, Fusion / immunology. Paired Box Transcription Factors / immunology. Rhabdomyosarcoma, Alveolar / immunology. T-Lymphocytes, Cytotoxic / immunology

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(PMID = 16452243.001).

[ISSN] 0008-5472

[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 / Epitopes; 0 / Forkhead Transcription Factors; 0 / HLA-B7 Antigen; 0 / Oncogene Proteins, Fusion; 0 / Paired Box Transcription Factors

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[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

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Current concepts and future perspectives in retroperitoneal soft-tissue sarcoma management.

Retroperitoneal soft-tissue sarcomas are complex, heterogeneous cancers requiring expert multidisciplinary care.

Pediatric subtypes mainly comprise extraskeletal Ewing sarcoma/pPNET and alveolar rhabdomyosarcoma.

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(PMID = 19671034.001).

[ISSN] 1744-8328

[Journal-full-title] Expert review of anticancer therapy

[ISO-abbreviation] Expert Rev Anticancer Ther

[Language] eng

[Publication-type] Journal Article; Review

[Publication-country] England

[Chemical-registry-number] 0 / Antineoplastic Agents

[Number-of-references] 93

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Alveolar rhabdomyosarcoma of the uterine cervix. A case report confirmed by FKHR break-apart rearrangement using a fluorescence in situ hybridization probe on paraffin-embedded tissues.

Alveolar rhabdomyosarcoma (ARMS) is a very rare malignant soft tissue tumor found in the uterine cervix.

The literature has been reviewed; the histologic differential diagnosis of this rare lesion has been discussed and FISH has been suggested to be useful in differentiating ARMS from other poorly differentiated round cell tumors.

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

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International Agency for Research on Cancer - Screening Group. diagnostics - Histopathology and cytopathology of the uterine cervix - digital atlas .

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(PMID = 18580325.001).

[ISSN] 1538-7151

[Journal-full-title] International journal of gynecological pathology : official journal of the International Society of Gynecological Pathologists

[ISO-abbreviation] Int. J. Gynecol. Pathol.

[Language] eng

[Publication-type] Case Reports; Journal Article

[Publication-country] United States

[Chemical-registry-number] 0 / FOXO1 protein, human; 0 / Forkhead Transcription Factors

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Correlation between histology and PAX/FKHR fusion status in alveolar rhabdomyosarcoma: a report from the Children's Oncology Group.

At the molecular level, alveolar rhabdomyosarcomas (ARMS) are characterized by 3 mutually exclusive PAX/FKHR conditions: PAX3/FKHR fusion (present in 60% of cases), PAX7/FKHR fusion (present in 20%), and PAX/FKHR fusion-negativity (present in 20%).

We evaluated cytohistologic parameters such as microcyst formation, solid foci, differentiation, giant cell formation, anaplasia, nuclear grade, mitosis/karyorrhexis index, rosette formation, geographic necrosis, presence and amount of rhabdomyoblastic differentiation, and the presence of foci resembling embryonal rhabdomyosarcoma.

Of these features, only totally solid alveolar architecture reached significance (P=0.00014), with 7 of 16 PAX/FKHR-negative cases lacking this feature, compared with 0 of 36 PAX3/FKHR cases and 2/13 PAX7/FKHR cases.

These preliminary results indicate that in general, only totally solid alveolar architecture in ARMS may predict the absence of a PAX/FKHR fusion.

[MeSH-major] Oncogene Proteins, Fusion / genetics. Rhabdomyosarcoma, Alveolar / genetics. Rhabdomyosarcoma, Alveolar / pathology

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(PMID = 17527077.001).

[ISSN] 0147-5185

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

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

[Language] eng

[Grant] United States / NCI NIH HHS / CA / CA 98543; United States / NCI NIH HHS / CA / CA24507; United States / NCI NIH HHS / CA / CA72989; United States / NCI NIH HHS / CA / CA81659; United States / NCI NIH HHS / CA / CA89461; United States / NCI NIH HHS / CA / CA98413; United States / NCI NIH HHS / CA / CA98543

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

[Publication-country] United States

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

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] PAX3-FOXO1 fusion gene in rhabdomyosarcoma.

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

The predominant histologic variants of this disease are termed embryonal (eRMS) and alveolar (aRMS), based on their appearance under light microscopy.

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(PMID = 18457914.001).

[ISSN] 1872-7980

[Journal-full-title] Cancer letters

[ISO-abbreviation] Cancer Lett.

[Language] ENG

[Grant] United States / NICHD NIH HHS / HD / HD043494-05; United States / NICHD NIH HHS / HD / K12 HD043494; United States / NICHD NIH HHS / HD / 5K12-HD043494; United States / NICHD NIH HHS / HD / K12 HD043494-05

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

[Publication-country] Ireland

[Chemical-registry-number] 0 / CXCL12 protein, human; 0 / Chemokine CXCL12; 0 / FOXO1 protein, human; 0 / Forkhead Transcription Factors; 0 / Oncogene Proteins, Fusion; 0 / PAX3-FOXO1A fusion protein, human; 0 / Paired Box Transcription Factors; EC 2.7.10.1 / FLT1 protein, human; EC 2.7.10.1 / Vascular Endothelial Growth Factor Receptor-1

[Number-of-references] 59

[Other-IDs] NLM/ NIHMS72264; NLM/ PMC2575376

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[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

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[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

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[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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[Cites] Cancer. 2003 Aug 1;98(3):571-80 [12879475.001]

[Cites] Cancer. 1995 Sep 15;76(6):1073-85 [8625211.001]

[Cites] Virchows Arch. 2006 Nov;449(5):572-8 [17016719.001]

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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

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[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

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[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

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Clinical presentation, treatment, and outcome of alveolar soft part sarcoma in children, adolescents, and young adults.

PURPOSE: Alveolar soft part sarcoma is a rare soft tissue neoplasm that can affect children and adolescents.

METHODS: After institutional review board approval, we examined the records of all patients younger than 25 years old who received treatment at our institution for alveolar soft part sarcoma in the past 30 years.

Younger patients tended to have Intergroup Rhabdomyosarcoma Study group I disease.

CONCLUSIONS: Achievement of complete microscopic resection is critical in localized alveolar soft part sarcoma, but incomplete excision and misdiagnosis are often encountered.

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(PMID = 16410131.001).

[ISSN] 1531-5037

[Journal-full-title] Journal of pediatric surgery

[ISO-abbreviation] J. Pediatr. Surg.

[Language] eng

[Publication-type] Journal Article

[Publication-country] United States

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Assessment of response to induction therapy and its influence on 5-year failure-free survival in group III rhabdomyosarcoma: the Intergroup Rhabdomyosarcoma Study-IV experience--a report from the Soft Tissue Sarcoma Committee of the Children's Oncology Group.

For Intergroup Rhabdomyosarcoma Study (IRS) IV patients with group III rhabdomyosarcoma, we assessed whether reported response assessed by anatomic imaging at week 8 predicted FFS.

In these patients, response did not influence FFS except for those with alveolar histology.

[MeSH-major] Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Neoadjuvant Therapy. Rhabdomyosarcoma / drug therapy. Soft Tissue Neoplasms / drug therapy

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(PMID = 17971587.001).

[ISSN] 1527-7755

[Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology

[ISO-abbreviation] J. Clin. Oncol.

[Language] eng

[Grant] United States / NCI NIH HHS / CA / CA 98543; United States / NCI NIH HHS / CA / U10 CA098413; United States / NCI NIH HHS / CA / U10 CA24507

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

[Publication-country] United States

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] First case report of bilateral metastatic intratesticular rhabdomyosarcoma.

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

We present a case of an 18-year-old boy with metastatic alveolar RMS to the testis and the first reported case of bilateral testicular involvement.

[MeSH-major] Lung Neoplasms / pathology. Lymph Nodes / pathology. Rhabdomyosarcoma / secondary. Rhabdomyosarcoma / therapy. Testicular Neoplasms / secondary. Testicular Neoplasms / therapy

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[Copyright] Copyright (c) 2010 Elsevier Inc. All rights reserved.

(PMID = 19819527.001).

[ISSN] 1527-9995

[Journal-full-title] Urology

[ISO-abbreviation] Urology

[Language] eng

[Publication-type] Case Reports; Journal Article

[Publication-country] United States

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[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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[CommentIn] Pediatr Blood Cancer. 2010 Oct;55(4):597-8 [20589627.001]

(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

   

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[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