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[Title] Increased expression of osteopontin gene in atypical teratoid/rhabdoid tumor of the central nervous system.

The atypical teratoid/rhabdoid tumor, primary to the central nervous system, is a highly malignant and aggressive neoplasm of infancy and childhood.

Although having distinct biological features and clinical outcomes, it is frequently misdiagnosed as primitive neuroectodermal tumor/medulloblastoma.

To further distinguish the underlying pathogenesis and to identify biological markers for clinical use, an atypical teratoid/rhabdoid tumor-derived cell line was established and its gene expression pattern analyzed in comparison to the human astrocyte SVG12 cell line and the human DAOY medulloblastoma cell line using a complementary DNA microarray method.

The osteopontin gene was found specifically upregulated in atypical teratoid/rhabdoid tumor cells.

This specificity was confirmed by immunohistochemistry in pathological sections of tissues from atypical teratoid/rhabdoid tumor patients.

Even though the role of osteopontin in the cytopathogenesis of atypical teratoid/rhabdoid tumor still needs to be determined, our data support that overexpressed osteopontin is a potential diagnostic marker for atypical teratoid/rhabdoid tumor.

[MeSH-major] Central Nervous System Neoplasms / pathology. Gene Expression Profiling. Rhabdoid Tumor / pathology. Sialoglycoproteins / genetics. Teratoma / pathology

[MeSH-minor] Cell Line. Cell Line, Tumor. Cluster Analysis. Gene Expression Regulation, Neoplastic / genetics. Humans. Immunohistochemistry. Medulloblastoma / genetics. Medulloblastoma / metabolism. Medulloblastoma / pathology. Nucleic Acid Hybridization / methods. Oligonucleotide Array Sequence Analysis. Osteopontin. Reverse Transcriptase Polymerase Chain Reaction

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(PMID = 15776015.001).

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

[Publication-country] United States

[Chemical-registry-number] 0 / SPP1 protein, human; 0 / Sialoglycoproteins; 106441-73-0 / Osteopontin

   

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

[Title] High-dose chemotherapy and autologous stem cell rescue for atypical teratoid/rhabdoid tumor of the central nervous system.

Atypical Teratoid/Rhabdoid tumors (AT/RT) of the central nervous system are rare but aggressive tumors of childhood.

Diagnosis was confirmed using molecular markers.

There are two long-term survivors (78 and 98 months from diagnosis).

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(PMID = 19936623.001).

[ISSN] 1573-7373

[Journal-full-title] Journal of neuro-oncology

[ISO-abbreviation] J. Neurooncol.

[Language] ENG

[Grant] United States / NCI NIH HHS / CA / R01 CA046274-18; United States / NCI NIH HHS / CA / R01 CA046274; United States / NCI NIH HHS / CA / CA46274; United States / NCI NIH HHS / CA / T32 CA108462; United States / NCI NIH HHS / CA / CA046274-18; United States / NCI NIH HHS / CA / T32 CA108462-01

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

[Publication-country] United States

[Other-IDs] NLM/ NIHMS184133; NLM/ PMC2880232

   

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

[Title] Atypical teratoid/rhabdoid tumor of the central nervous system in an 18-year-old patient.

OBJECTIVE: Atypical teratoid/rhabdoid tumors are aggressive neoplasms of the central nervous system occurring mainly in the early childhood and rarely in adults.

We described a case of this tumor in an 18-year-old male patient without previous medical history.

MATERIAL AND METHODS: The neoplasm was localized in the right frontotemporal area of the brain and was totally excised.

The histological and immunohistochemical features of the neoplasm were assessed, while sequencing analysis as well as interphase fluorescence in situ hybridization (FISH) were performed.

RESULTS: Histological and immunohistochemical analysis demonstrated atypical rhabdoid cells strongly and diffusely positive for EMA and Vimentin as well as focally immunoreactive for SMA and GFAP.

INI1 immunostaining demonstrated diffuse loss of nuclear INI1 expression in tumor cells.

Taken together, the results were consistent with a diagnosis of atypical teratoid/rhabdoid tumor (ATRT).

To our knowledge, this is the eighth case of an ATRT reported in an adult patient having genetic confirmation and the first one in which the tumor is, partly, localized in the right temporal area of the brain.

This unusual presentation underlines the necessity of considering this devastating neoplasm in the differential diagnosis of malignant brain tumors of young adults.

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(PMID = 19216214.001).

[ISSN] 0722-5091

[Journal-full-title] Clinical neuropathology

[ISO-abbreviation] Clin. Neuropathol.

[Language] ENG

[Grant] United States / NCI NIH HHS / CA / CA046274-17A2; United States / NCI NIH HHS / CA / R01 CA046274; United States / NCI NIH HHS / CA / CA 46274; United States / NCI NIH HHS / CA / R01 CA046274-17A2

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

[Publication-country] Germany

[Chemical-registry-number] 0 / Chromosomal Proteins, Non-Histone; 0 / DNA-Binding Proteins; 0 / SMARCB1 protein, human; 0 / Transcription Factors

[Other-IDs] NLM/ NIHMS113796; NLM/ PMC2712356

   

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

[Title] Atypical teratoid/rhabdoid tumor mimicking tuberculous meningitis.

Atypical teratoid/rhabdoid tumor of the central nervous system is a highly malignant neoplasm in infants and young children.

We report a 6 year-old girl with atypical teratoid/rhabdoid tumor.

Pathologic result revealed high grade atypical teratoid/rhabdoid tumor.

[MeSH-major] Rhabdoid Tumor / diagnosis. Teratoma / diagnosis. Tuberculosis, Meningeal / diagnosis

[MeSH-minor] Cerebrospinal Fluid. Child. Diagnosis, Differential. Female. Humans. Magnetic Resonance Imaging

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(PMID = 18451456.001).

[ISSN] 0019-6061

[Journal-full-title] Indian pediatrics

[ISO-abbreviation] Indian Pediatr

[Language] eng

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

[Publication-country] India

   

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

[Title] Neuroradiological findings in atypical teratoid/rhabdoid tumor of the central nervous system.

PURPOSE: To evaluate the computed tomography (CT) and magnetic resonance imaging (MRI) findings of atypical teratoid tumor/rhabdoid tumor (AT/RT) of the central nervous system (CNS).

MATERIAL AND METHODS: Twenty cases of CNS AT/RT have been found over the past 23 years in our hospital; these involving 11 boys and 9 girls whose mean age at diagnosis was 5.5 years.

The diagnosis mainly depends on the pathologic findings.

However, AT/RT should still remain in the differential diagnosis of brain tumors in young children, especially those located in the cerebellar hemisphere and with eccentric cysts.

[MeSH-major] Central Nervous System Neoplasms / pathology. Central Nervous System Neoplasms / radiography. Rhabdoid Tumor / pathology. Rhabdoid Tumor / radiography. Teratoma / pathology. Teratoma / radiography

[MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Diagnosis, Differential. Female. Humans. Infant. Magnetic Resonance Imaging. Male. Prognosis. Retrospective Studies. Survival Rate. Tomography, X-Ray Computed

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(PMID = 15841745.001).

[ISSN] 0284-1851

[Journal-full-title] Acta radiologica (Stockholm, Sweden : 1987)

[ISO-abbreviation] Acta Radiol

[Language] eng

[Publication-type] Journal Article

[Publication-country] Sweden

   

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

[Title] [Clinicopathologic and immunohistochemical study of atypical teratoid/rhabdoid tumor of central nervous system].

OBJECTIVE: To study the clinicopathologic features and differential diagnosis of atypical teratoid/rhabdoid tumor (AT/RT) occurring in the central nervous system.

RESULTS: Histologically, AT/RT was characterized by the presence of rhabdoid cells associated with various degrees of primitive neuroectodermal, epithelial or mesenchymal differentiation.

The tumor cells were positive for vimentin, CD99, epithelial membrane antigen, cytokeratin, glial fibrillary acidic protein, S-100 protein, neurofilament, desmin and smooth muscle actin.

CONCLUSIONS: AT/RT is a highly malignant tumor occurring in the central nervous system.

The tumor is characterized by a heterogeneous histologic and immunohistochemical phenotype.

It needs to be distinguished from a number of central nervous system tumors, including medulloblastoma, primitive neuroectodermal tumor, germ cell neoplasm and rhabdoid meningioma.

[MeSH-major] Brain Neoplasms / pathology. Rhabdoid Tumor / pathology. Teratoma / pathology

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(PMID = 17069697.001).

[ISSN] 0529-5807

[Journal-full-title] Zhonghua bing li xue za zhi = Chinese journal of pathology

[ISO-abbreviation] Zhonghua Bing Li Xue Za Zhi

[Language] chi

[Publication-type] Case Reports; English Abstract; Journal Article

[Publication-country] China

[Chemical-registry-number] 0 / Actins; 0 / Antigens, CD; 0 / CD99 protein, human; 0 / Cell Adhesion Molecules; 0 / Desmin; 0 / Glial Fibrillary Acidic Protein; 0 / Mucin-1; 0 / Neurofilament Proteins; 0 / S100 Proteins; 0 / Vimentin; 68238-35-7 / Keratins

   

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

[Title] Central nervous system atypical teratoid rhabdoid tumor: experience at the National Institute of Pediatrics, Mexico City.

OBJECTIVE: The purpose of this study is to present our experience with ten cases of Central nervous system atypical teratoid rhabdoid tumor (CNS/ATRT).

PATIENTS AND METHODS: A series of ten patients with CNS/ATRT, were diagnosed and treated between 1990 and 2005, at the National Institute of Pediatrics, in Mexico City.

The gender, age of presentation, clinical features, tumor localization, imaging studies, grade of tumor resection, complications, adjuvant therapy, and survival are presented.

RESULTS: The mean age at diagnosis was 37.8 months, seven cases were male, and their average clinical course was 1.3 months.

CONCLUSIONS: Preliminary results, show that in older children, we can improve their survival with the subtotal or total resection of the tumor and the addition of chemotherapy and radiotherapy.

[MeSH-major] Central Nervous System Neoplasms / pathology. Infratentorial Neoplasms / pathology. Rhabdoid Tumor / pathology. Supratentorial Neoplasms / pathology. Teratoma / pathology

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(PMID = 17876589.001).

[ISSN] 0256-7040

[Journal-full-title] Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery

[ISO-abbreviation] Childs Nerv Syst

[Language] eng

[Publication-type] Journal Article

[Publication-country] Germany

   

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

[Title] Atypical teratoid/rhabdoid tumor of the spine.

SUMMARY: Atypical teratoid/rhabdoid tumor (AT/RT) is a highly malignant central nervous system neoplasm usually seen in young children and infants.

[MeSH-major] Magnetic Resonance Imaging. Rhabdoid Tumor / pathology. Spinal Neoplasms / pathology. Teratoma / pathology

[MeSH-minor] Child. Diagnosis, Differential. Humans. Lumbar Vertebrae / pathology. Male. Thoracic Vertebrae / pathology

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(PMID = 17353344.001).

[ISSN] 0195-6108

[Journal-full-title] AJNR. American journal of neuroradiology

[ISO-abbreviation] AJNR Am J Neuroradiol

[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] Atypical teratoid/rhabdoid tumor of the central nervous system: clinicopathologic and immunohistochemical features of four cases.

BACKGROUND: Atypical teratoid/rhabdoid tumor (AT/RT) is a rare aggressive infantile neoplasm of uncertain origin.

Histopathologically, the tumors were composed of rhabdoid cells and undifferentiated small cells mixed with epithelial or mesenchymal components.

All of the patients died within a mean of 14 months due to tumor progression despite the chemotherapy.

Only one of our patients lived for 40 months after the diagnosis.

Morphologically, a large spectrum can be seen, like predominantly sarcoma in appearance, but immunohistochemistry is helpful in the correct diagnosis.

[MeSH-major] Brain Neoplasms / pathology. Rhabdoid Tumor / pathology. Teratoma / pathology

[MeSH-minor] Actins / analysis. Brain / pathology. Brain Chemistry. Child. Child, Preschool. Chromosomal Proteins, Non-Histone / analysis. DNA-Binding Proteins / analysis. Desmin / analysis. Diagnosis, Differential. Female. Glial Fibrillary Acidic Protein / analysis. Humans. Immunohistochemistry. Infant. Keratins / analysis. Male. Mucin-1 / analysis. S100 Proteins / analysis. SMARCB1 Protein. Synaptophysin / analysis. Transcription Factors / analysis. Vimentin / analysis

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[CommentIn] Childs Nerv Syst. 2009 Nov;25(11):1387; author reply 1389 [19636570.001]

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(PMID = 19212771.001).

[ISSN] 1433-0350

[Journal-full-title] Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery

[ISO-abbreviation] Childs Nerv Syst

[Language] eng

[Publication-type] Case Reports; Journal Article

[Publication-country] Germany

[Chemical-registry-number] 0 / Actins; 0 / Chromosomal Proteins, Non-Histone; 0 / DNA-Binding Proteins; 0 / Desmin; 0 / Glial Fibrillary Acidic Protein; 0 / Mucin-1; 0 / S100 Proteins; 0 / SMARCB1 Protein; 0 / SMARCB1 protein, human; 0 / Synaptophysin; 0 / Transcription Factors; 0 / Vimentin; 68238-35-7 / Keratins

   

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

[Title] Continuous remission of newly diagnosed and relapsed central nervous system atypical teratoid/rhabdoid tumor.

Atypical teratoid/rhabdoid tumors (AT/RT) are highly malignant lesions of childhood that carry a very poor prognosis.

AT/RT can occur in the central nervous system (CNS AT/RT) and disease in this location carries an even worse prognosis with a median survival of 7 months.

In spite of multiple treatment regimens consisting of maximal surgical resection (including second look surgery), radiation therapy (focal and craniospinal), and multi-agent intravenous, oral and intrathecal chemotherapy, with or without high-dose therapy and stem cell rescue, only seven long-term survivors of CNS AT/RT have been reported, all in patients with newly diagnosed disease.

We now report on four children, two with newly diagnosed CNS AT/RT and two with progressive disease after multi-agent chemotherapy who are long term survivors (median follow-up of 37 months) using a combination of surgery, radiation therapy, and intensive chemotherapy.

[MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Brain Neoplasms / therapy. Neoplasm Recurrence, Local / therapy. Rhabdoid Tumor / therapy. Teratoma / therapy

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(PMID = 15803379.001).

[ISSN] 0167-594X

[Journal-full-title] Journal of neuro-oncology

[ISO-abbreviation] J. Neurooncol.

[Language] eng

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

[Publication-country] United States

[Number-of-references] 37

   

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

[Title] Atypical teratoid/rhabdoid tumor of the central nervous system associated with congenital cataract.

Atypical teratoid /rhabdoid tumor (AT/RT) of the central nervous system is a rare but highly aggressive neoplasm that usually affects young children and infants and follows a rapidly fatal course.

[MeSH-major] Central Nervous System Neoplasms / diagnosis. Central Nervous System Neoplasms / pathology. Rhabdoid Tumor / diagnosis. Rhabdoid Tumor / pathology. Teratoma / diagnosis. Teratoma / pathology

[MeSH-minor] Cataract / complications. Cataract / congenital. Central Nervous System / pathology. Fatal Outcome. Humans. Infant. Male

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(PMID = 18723966.001).

[ISSN] 0377-4929

[Journal-full-title] Indian journal of pathology & microbiology

[ISO-abbreviation] Indian J Pathol Microbiol

[Language] eng

[Publication-type] Case Reports; Journal Article

[Publication-country] India

   

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

[Title] Childhood atypical teratoid rhabdoid tumor of the central nervous system: a meta-analysis of observational studies.

PURPOSE: Therapy for central nervous system (CNS) atypical teratoid rhabdoid tumor (ATRT) is controversial.

RESULTS: The median OS for patients treated with multiagent chemotherapy (n=79) was 17.3 months (range, 1.5-93 mo); unrelated to age at diagnosis, sex, tumor site, and extent of resection.

Patients (n=30) treated with intrathecal (IT) chemotherapy had significantly higher 2-year OS [64% (95% confidence interval, 46.5-82.0) vs. 17.3% (95% confidence interval, 5.4-29.3); P<0.0001] and lower prevalence of distant CNS metastasis compared with those without IT therapy (n=49) (20% vs. 59.2%; P=0.001).

CONCLUSIONS: Despite dismal OS, multimodal therapy can induce remission even in metastatic CNS ATRT with partial resection.

[MeSH-major] Brain Neoplasms / epidemiology. Rhabdoid Tumor / epidemiology. Teratoma / epidemiology

[MeSH-minor] Adolescent. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child. Child, Preschool. Cisplatin / administration & dosage. Combined Modality Therapy. Craniotomy. Cyclophosphamide / administration & dosage. Dactinomycin / administration & dosage. Doxorubicin / administration & dosage. Etoposide / administration & dosage. Fatal Outcome. Female. Humans. Infant. Infant, Newborn. Injections, Spinal. Kaplan-Meier Estimate. Male. Prognosis. Prospective Studies. Spinal Neoplasms / diagnosis. Spinal Neoplasms / drug therapy. Spinal Neoplasms / epidemiology. Spinal Neoplasms / radiotherapy. Spinal Neoplasms / surgery. Treatment Outcome. Vincristine / administration & dosage

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(PMID = 19707161.001).

[ISSN] 1536-3678

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

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

[Language] eng

[Publication-type] Case Reports; Journal Article; Meta-Analysis

[Publication-country] United States

[Chemical-registry-number] 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine; 6PLQ3CP4P3 / Etoposide; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; Q20Q21Q62J / Cisplatin

   

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

[Title] Efficacy and tolerability of intrathecal liposomal cytarabine for central nervous system embryonal tumors.

METHODS: We reviewed all patients at our institution treated with liposomal cytarabine for primary central nervous system (CNS) embryonal tumors-MB, primitive neuroectodermal tumor (PNET), and atypical teratoid rhabdoid tumor (ATRT).

RESULTS: A cohort of 17 patients were treated with liposomal cytarabine at diagnosis of CNS embryonal tumor (2 PNET, 3 ATRT) or relapse (12 MB [7 average-risk, 5 high-risk]); nine had leptomeningeal metastases.

All six evaluable patients with malignant cerebrospinal fluid (CSF) cytology and treated with at least two doses cleared their spinal fluid (mean 3 doses, range 1-5).

No patient developed malignant CSF cytology while receiving liposomal cytarabine.

All patients with neoplastic meningitis cleared malignant cells from their spinal fluid after treatment with IT liposomal cytarabine and systemic chemotherapy.

Our findings warrant a phase II trial of liposomal cytarabine in newly diagnosed or recurrent CNS embryonal tumors.

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(PMID = 27964690.001).

[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

[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] Successful outcome with tandem myeloablative chemotherapy and autologous peripheral blood stem cell transplants in a patient with atypical teratoid/rhabdoid tumor of the central nervous system.

Atypical teratoid rhabdoid tumors (ATRT) are highly malignant tumors of the central nervous system with a peak incidence in children less than 3 years of age.

We herein report the case of a 4-month-old boy with ATRT with partial resection of his tumor who achieved complete remission using tandem high-dose therapy followed by autologous peripheral blood stem cell re-infusions despite having biopsy proven disease at the time of starting the tandem regimens.

[MeSH-major] Central Nervous System Neoplasms / therapy. Myeloablative Agonists / therapeutic use. Peripheral Blood Stem Cell Transplantation / methods. Rhabdoid Tumor / therapy

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[Cites] J Clin Oncol. 2004 Jul 15;22(14):2877-84 [15254056.001]

[Cites] J Neurooncol. 2005 Dec;75(3):309-13 [16195799.001]

[Cites] J Neurooncol. 2005 Jan;71(1):33-8 [15719272.001]

[Cites] J Clin Oncol. 2004 Dec 15;22(24):4881-7 [15611503.001]

[Cites] J Neurooncol. 1998 Dec;40(3):265-75 [10066100.001]

[Cites] Pediatr Blood Cancer. 2004 Mar;42(3):254-60 [14752863.001]

[Cites] Med Pediatr Oncol. 2003 Mar;40(3):199-201 [12518355.001]

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[Cites] Pediatr Neurosurg. 2006;42(4):258-63 [16714870.001]

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[Cites] J Neurooncol. 2005 Mar;72(1):77-84 [15803379.001]

[Cites] Am J Surg Pathol. 2004 May;28(5):644-50 [15105654.001]

[Cites] J Pediatr Hematol Oncol. 1995 Feb;17(1):71-5 [7743242.001]

[Cites] J Neurosurg. 2005 Apr;102(3 Suppl):299-302 [15881754.001]

[Cites] Cancer. 2004 Jul 1;101(1):3-27 [15221985.001]

(PMID = 18317689.001).

[ISSN] 0167-594X

[Journal-full-title] Journal of neuro-oncology

[ISO-abbreviation] J. Neurooncol.

[Language] eng

[Publication-type] Case Reports; Journal Article

[Publication-country] United States

[Chemical-registry-number] 0 / Myeloablative Agonists

   

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

[Title] Atypical teratoid/rhabdoid tumour of the central nervous system in adult: case report.

Atypical teratoid/rhabdoid tumours (AT/RT) are aggressive neoplasms of the central nervous system occurring mainly in the paediatric population.

The neoplasm was localised in the left frontal region and was totally excised.

This unusual presentation underlines the necessity of considering this devastating neoplasm in the differential diagnosis of malignant brain tumours of adults.

[MeSH-major] Central Nervous System Neoplasms / pathology. Rhabdoid Tumor / pathology. Teratoma / pathology

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(PMID = 21070155.001).

[ISSN] 1360-046X

[Journal-full-title] British journal of neurosurgery

[ISO-abbreviation] Br J Neurosurg

[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] Impact of radiotherapy for pediatric CNS atypical teratoid/rhabdoid tumor (single institute experience).

PURPOSE: To assess outcomes and prognostic factors in radiotherapy of pediatric central nervous system atypical teratoid/rhabdoid tumor (AT/RT).

METHODS AND MATERIALS: Seventeen patients with central nervous system AT/RT were retrospectively reviewed after curative radiotherapy as primary or adjuvant therapy between January 1990 and December 2003.

The 3 longest-surviving patients were older, underwent gross tumor removal, and completed both craniospinal and focal boost irradiation.

[MeSH-major] Brain Neoplasms / radiotherapy. Rhabdoid Tumor / radiotherapy. Teratoma / radiotherapy

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[CommentIn] Int J Radiat Oncol Biol Phys. 2006 Jul 15;65(4):1273; author reply 1273-4 [16798419.001]

(PMID = 16406394.001).

[ISSN] 0360-3016

[Journal-full-title] International journal of radiation oncology, biology, physics

[ISO-abbreviation] Int. J. Radiat. Oncol. Biol. Phys.

[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] Molecular genetics of atypical teratoid/rhabdoid tumor.

Rhabdoid tumors are extremely aggressive malignancies that generally occur in infants and young children.

The most common locations are the kidney and central nervous system (atypical teratoid/rhabdoid tumor [RT]), although RTs can also arise in most soft-tissue sites.

Rhabdoid tumors in all anatomical locations have a similar molecular origin.

[MeSH-major] Central Nervous System Neoplasms / genetics. Chromosomes, Human, Pair 22. Kidney Neoplasms / genetics. Molecular Biology / methods. Rhabdoid Tumor / genetics

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(PMID = 16459991.001).

[ISSN] 1092-0684

[Journal-full-title] Neurosurgical focus

[ISO-abbreviation] Neurosurg Focus

[Language] eng

[Grant] United States / NCI NIH HHS / CA / CA46274

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

[Publication-country] United States

[Chemical-registry-number] 0 / Chromosomal Proteins, Non-Histone; 0 / DNA-Binding Proteins; 0 / SMARCB1 protein, human; 0 / Transcription Factors

[Number-of-references] 44

   

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

[Title] Primary intracranial extra-axial and supratentorial atypical rhabdoid tumor.

An atypical teratoid/rhabdoid tumor of the central nervous system is an aggressive infantile embryonal neoplasm, usually presenting as an infratentorial and intraparenchymatous lesion.

We report on magnetic resonance imaging findings of a 22-month-old boy with a biopsy-proven primary rhabdoid tumor, presenting as a single intracranial supratentorial extra-axial mass.

Based on the patient's age and imaging features (perfusion, diffusion magnetic resonance imaging, and magnetic resonance spectroscopy), a diagnosis of atypical teratoid/rhabdoid tumor was more accurate than diagnoses of meningioma and primitive neuroectodermal tumor.

Although this entity is relatively rare, it should be considered in the differential diagnosis of dural-based, space-occupying central nervous system lesions.

[MeSH-major] Brain Neoplasms / diagnosis. Brain Neoplasms / pathology. Rhabdoid Tumor / diagnosis. Rhabdoid Tumor / pathology. Supratentorial Neoplasms / diagnosis. Supratentorial Neoplasms / pathology

[MeSH-minor] Brain / metabolism. Brain / pathology. Brain / surgery. Brain Edema / pathology. Diagnosis, Differential. Diffusion Magnetic Resonance Imaging. Humans. Infant. Magnetic Resonance Imaging. Magnetic Resonance Spectroscopy. Male. Neoplasm Recurrence, Local. Treatment Outcome

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(PMID = 19931170.001).

[ISSN] 1873-5150

[Journal-full-title] Pediatric neurology

[ISO-abbreviation] Pediatr. Neurol.

[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] Primary atypical teratoid/rhabdoid tumor of the clival region. Case report.

An atypical teratoid/rhabdoid tumor of the central nervous system (CNS) is a rare, aggressive neoplasm found in infants and children that has similar characteristics to CNS primitive neuroectodermal tumors/medulloblastomas.

The authors present the case of a patient with an atypical teratoid/rhabdoid tumor and discuss the imaging, histopathological, immunohistochemical, and cytogenetic findings.

Tumor cells displayed positive reactions for vimentin, epithelial membrane antigen, and cytokeratin, and they displayed no reaction for glial fibrillary acidic protein, desmin, and actin.

The phenotype of an atypical teratoid/rhabdoid tumor appears heterogeneous when examined by histological, immunohistochemical, and genetic analysis.

The authors describe the case of a 4-year-old boy who harbored an atypical teratoid/rhabdoid tumor in the clivus, which appeared as a chordoma on neuroimages.

To their knowledge, this location of an atypical teratoid/rhabdoid tumor has not been described in the literature.

[MeSH-major] Cranial Fossa, Posterior. Rhabdoid Tumor / pathology. Skull Base Neoplasms / pathology. Teratoma / pathology

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(PMID = 17465367.001).

[ISSN] 0022-3085

[Journal-full-title] Journal of neurosurgery

[ISO-abbreviation] J. Neurosurg.

[Language] eng

[Publication-type] Case Reports; 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] Atypical teratoid/rhabdoid tumor in a patient with Beckwith-Wiedemann syndrome.

Here we describe a patient with BWS who developed a central nervous system atypical teratoid/rhabdoid tumor (AT/RT).

Due to the high propensity of these patients to develop childhood tumors, in addition to routine diagnostic tests, analysis of the tumor DNA using the Illumina Infinium whole-genome genotyping 550K Beadchip was performed to investigate a possible common underlying mechanism for his BWS and AT/RT.

The only alteration detected was monosomy 22, which was accompanied by a somatic mutation in the INI1 rhabdoid tumor gene.

[MeSH-major] Beckwith-Wiedemann Syndrome / genetics. Rhabdoid Tumor / complications. Teratoma / complications

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

(PMID = 17603804.001).

[ISSN] 1552-4833

[Journal-full-title] American journal of medical genetics. Part A

[ISO-abbreviation] Am. J. Med. Genet. A

[Language] eng

[Publication-type] Case Reports; 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] INI1 protein expression distinguishes atypical teratoid/rhabdoid tumor from choroid plexus carcinoma.

Central nervous system atypical teratoid/rhabdoid tumor (AT/RT) and choroid plexus carcinoma (CPC) are rare, highly malignant tumors that predominantly arise in infants and young children.

Overlapping clinical, histologic, ultrastructural, or immunophenotypic features may obscure the diagnosis in some cases.

AT/RT is characterized by deletions and/or mutations of the INI1 tumor-suppressor gene on chromosome band 22q11.2.

Negative staining of tumor cells resulting from inactivation of the INI1 gene is a consistent feature of AT/RT.

We examined 28 tumors with a submitted diagnosis of CPC.

[MeSH-major] Biomarkers, Tumor / metabolism. Carcinoma / metabolism. Choroid Plexus Neoplasms / metabolism. DNA-Binding Proteins / metabolism. Gene Expression Regulation, Neoplastic / physiology. Teratoma / metabolism

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(PMID = 15892296.001).

[ISSN] 0022-3069

[Journal-full-title] Journal of neuropathology and experimental neurology

[ISO-abbreviation] J. Neuropathol. Exp. Neurol.

[Language] eng

[Grant] United States / NCI NIH HHS / CA / CA46274; United States / NCI NIH HHS / CA / CA98543

[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.

[Publication-country] United States

[Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Chromosomal Proteins, Non-Histone; 0 / DNA-Binding Proteins; 0 / Mucin-1; 0 / SMARCB1 protein, human; 0 / Transcription Factors

   

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

[Title] Giant supratentorial atypical teratoid/rhabdoid tumor presentation: a case of a five-year-old child with favorable outcome and review of the literature.

Atypical teratoid/rhabdoid tumor of the central nervous system is a highly malignant neoplasm and that usually arises in the posterior fossa, survival from this is frequently poor.

We present a unique case in a 21-month-old girl who had an atypical teratoid/rhabdoid tumor with cystic components located in the right fronto-parietal lobe.

Two years later at the last follow-up visit, there was no evidence of a tumor relapse on MRI, and the examination was symptom free.

It is possible the favorable outcome of the patient resulted from a rapid diagnosis, prompt management, radical surgical intervention and aggressive chemotherapy.

[MeSH-major] Frontal Lobe / surgery. Parietal Lobe / surgery. Rhabdoid Tumor / surgery. Supratentorial Neoplasms / surgery. Teratoma / surgery

[MeSH-minor] Actins / analysis. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Biomarkers, Tumor / analysis. Chemotherapy, Adjuvant. Combined Modality Therapy. Diagnosis, Differential. Female. Follow-Up Studies. Glial Fibrillary Acidic Protein / analysis. Humans. Infant. Keratins / analysis. Magnetic Resonance Imaging. Microsurgery. Mitotic Index. Necrosis. Neurologic Examination. Vimentin / analysis

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[Copyright] Copyright (c) 2007 S. Karger AG, Basel.

(PMID = 17337931.001).

[ISSN] 1016-2291

[Journal-full-title] Pediatric neurosurgery

[ISO-abbreviation] Pediatr Neurosurg

[Language] eng

[Publication-type] Case Reports; Journal Article; Review

[Publication-country] Switzerland

[Chemical-registry-number] 0 / Actins; 0 / Biomarkers, Tumor; 0 / Glial Fibrillary Acidic Protein; 0 / Vimentin; 68238-35-7 / Keratins

[Number-of-references] 14

   

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

[Title] Elevation of plasma and cerebrospinal fluid osteopontin levels in patients with atypical teratoid/rhabdoid tumor.

Osteopontin, a cancer metastasis-associated gene, is specifically up-regulated in central nervous system (CNS) atypical teratoid/rhabdoid tumor (AT/RT), but its biological behavior in the progression of CNS AT/RT has never been studied.

The differences in osteopontin expression in plasma, CSF, and tumor samples in AT/RT and medulloblastoma correlated with survival differences.

[MeSH-major] Brain Neoplasms / metabolism. Rhabdoid Tumor / metabolism. Sialoglycoproteins. Teratoma / metabolism

[MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Enzyme-Linked Immunosorbent Assay. Humans. Infant. Infant, Newborn. Medulloblastoma / metabolism. Medulloblastoma / mortality. Medulloblastoma / pathology. Neoplasm Recurrence, Local. Osteopontin. Survival Rate

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(PMID = 15842057.001).

[ISSN] 0002-9173

[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

[Chemical-registry-number] 0 / SPP1 protein, human; 0 / Sialoglycoproteins; 106441-73-0 / Osteopontin

   

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

[Title] Atypical teratoid/rhabdoid tumors of the central nervous system.

Atypical teratoid/rhabdoid tumor (AT/RT) is a highly malignant central nervous system neoplasm that usually affects very young children and is typically deadly despite very aggressive treatment.

Considered rare, the tumor was not recognized as a distinct entity until the 80's, due to its similar features with other primitive tumors.

AT/RT is the first pediatric brain tumor for which a candidate tumor suppressor gene has been identified.

[MeSH-major] Central Nervous System Neoplasms / genetics. Central Nervous System Neoplasms / pathology. Genes, Tumor Suppressor. Rhabdoid Tumor / genetics. Rhabdoid Tumor / pathology. Teratoma / genetics. Teratoma / pathology

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(PMID = 16195799.001).

[ISSN] 0167-594X

[Journal-full-title] Journal of neuro-oncology

[ISO-abbreviation] J. Neurooncol.

[Language] eng

[Publication-type] Journal Article; Review

[Publication-country] United States

[Number-of-references] 19

   

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

[Title] Atypical teratoid/rhabdoid tumors of the central nervous system: management and outcomes.

OBJECT: Atypical teratoid/rhabdoid tumors (ATRTs) represent a relatively newly categorized neoplastic entity.

Signs and symptoms began, on average, a little more than 1 month before diagnosis and included the following: headache (36%), nausea and vomiting (46%), lethargy (18%), seizures (27%), cranial nerve findings (46%), ataxia (18%), long tract findings (18%), and hydrocephalus (46%).

Tumor location was cortical in four patients, in the pineal region in four, in the posterior fossa in two, and spinal in one.

In one patient disseminated disease was revealed on the initial imaging study; seven patients had disseminated tumor subsequently.

CONCLUSIONS: Atypical teratoid/rhabdoid tumors are malignant lesions with rapid progression.

[MeSH-major] Central Nervous System Neoplasms / therapy. Rhabdoid Tumor / therapy. Teratoma / therapy

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(PMID = 16048294.001).

[ISSN] 1092-0684

[Journal-full-title] Neurosurgical focus

[ISO-abbreviation] Neurosurg Focus

[Language] eng

[Publication-type] Comparative Study; 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] Atypical teratoid/rhabdoid tumor: the controversy behind radiation therapy.

To date, approximately 200 cases of atypical teratoid/rhabdoid tumor (AT/RT) of the central nervous system have been described in the literature.

This CNS tumor tends to present at an age of less than 3 years, and most patients succumb to their disease within 1 year of diagnosis.

Prior to the rise in utilization of immunohistochemical (IHC) testing in the late 1990s, this tumor was likely mistaken as medulloblastoma and treated as such.

A standardized and effective approach to treating this usually fatal tumor remains elusive, and the role of radiation therapy presents a particular dilemma as young patients with this disease may experience devastating late effects of therapy if they achieve a long-term survival.

Our review underscores the importance or enrolling patients in multi-institutional prospective studies to further investigate the value of radiation to treat this pediatric neoplasm.

[MeSH-major] Central Nervous System Neoplasms / radiotherapy. Radiotherapy / methods. Rhabdoid Tumor / radiotherapy. Teratoma / radiotherapy

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(PMID = 16855864.001).

[ISSN] 0167-594X

[Journal-full-title] Journal of neuro-oncology

[ISO-abbreviation] J. Neurooncol.

[Language] eng

[Publication-type] Journal Article; Review

[Publication-country] United States

[Chemical-registry-number] 0 / Antineoplastic Agents

[Number-of-references] 92

   

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

[Title] Primary diffuse cerebral leptomeningeal atypical teratoid rhabdoid tumor: report of the first case.

Atypical teratoid rhabdoid tumor (AT/RT) of the central nervous system has been recently described as a distinct clinicopathological entity with characteristic morphologic, immunophenotypic and molecular characteristics.

[MeSH-major] Central Nervous System Neoplasms / pathology. Meningeal Neoplasms / pathology. Rhabdoid Tumor / pathology

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(PMID = 20020178.001).

[ISSN] 1573-7373

[Journal-full-title] Journal of neuro-oncology

[ISO-abbreviation] J. Neurooncol.

[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] Giant lumbar paraspinal atypical teratoid/rhabdoid tumor in a child.

Atypical teratoid/rhabdoid tumor (AT/RT) is a highly aggressive and uncommon tumor of the central nervous system, primarily affecting young children.

[MeSH-major] Rhabdoid Tumor / pathology. Spinal Neoplasms / pathology. Teratoma / pathology

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(PMID = 20160373.001).

[ISSN] 1998-4138

[Journal-full-title] Journal of cancer research and therapeutics

[ISO-abbreviation] J Cancer Res Ther

[Language] eng

[Publication-type] Case Reports; Journal Article

[Publication-country] India

[Chemical-registry-number] 0 / Antineoplastic Agents

   

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

[Title] Ultrastructural evidence of ependymal differentiation in a genetically proven atypical teratoid/rhabdoid tumor.

INTRODUCTION: We describe a case of genetically proven atypical teratoid/rhabdoid tumor (ATRT), showing ultrastructural evidence of ependymal differentiation.

CONCLUSION: This finding supports the concept that ATRTs as the majority of central nervous system embryonal tumors may derive from an immature and pluripotent neuroectodermal cell capable of differentiating along multiple lineages.

[MeSH-major] Cerebral Ventricle Neoplasms / ultrastructure. Ependyma / ultrastructure. Rhabdoid Tumor / ultrastructure. Teratoma / ultrastructure

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(PMID = 19554334.001).

[ISSN] 1433-0350

[Journal-full-title] Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery

[ISO-abbreviation] Childs Nerv Syst

[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] Atypical teratoid/rhabdoid tumors of the central nervous system: imaging and clinical findings in 16 children.

AIM: To investigate the imaging and clinical findings of central nervous system (CNS) atypical teratoid/rhabdoid tumours (AT/RTs) in children.

MATERIALS AND METHODS: The computed tomography (CT) and magnetic resonance imaging (MRI) findings and clinical records of 16 children with CNS AT/RTs were retrospectively reviewed.

Tumour location, size, composition, enhancement pattern, peritumoural oedema, signal intensity (SI) on MRI and CT attenuation were evaluated.

Tumour location was infratentorial for 11 lesions and supratentorial for six lesions.

The mean diameter of the largest dimension for a tumour was 4 cm.

The tumour was mainly solid in 65% of cases, and solid and cystic or cystic and solid in 35% of cases.

In spite of a large tumour size, peritumoural oedema was minimal or mild except in four cases.

Rapid growth of the tumour was demonstrated in three cases.

Seven patients died from tumour progression, with a mean survival time of 8.4 months (range 2-12 months).

Rapid growth of the tumour was seen during the follow-up period.

[MeSH-major] Brain Neoplasms / diagnosis. Rhabdoid Tumor / diagnosis

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(PMID = 19185655.001).

[ISSN] 1365-229X

[Journal-full-title] Clinical radiology

[ISO-abbreviation] Clin Radiol

[Language] eng

[Publication-type] Journal Article; Multicenter Study

[Publication-country] England

   

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

[Title] Atypical teratoid/rhabdoid tumor in an infant conceived by in vitro fertilization.

BACKGROUND: Atypical teratoid/rhabdoid tumor (ATsRT) is a rare tumor and extremely aggressive embryonal neoplasm of the central nervous system.

[MeSH-major] Brain Neoplasms / pathology. Diseases in Twins / pathology. Fertilization in Vitro. Rhabdoid Tumor / pathology. Teratoma / pathology

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(PMID = 19937253.001).

[ISSN] 1433-0350

[Journal-full-title] Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery

[ISO-abbreviation] Childs Nerv Syst

[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] Diffusion-weighted imaging of an atypical teratoid/rhabdoid tumor of the cervical spine.

Spinal atypical teratoid/rhabdoid tumor (AT/RT) is a rare, aggressive malignant neoplasm of the central nervous system usually seen in young children and infants.

High signal on DWI and low apparent diffusion coefficients may represent high cellularity of the tumor.

These findings indicated a highly malignant tumor.

[MeSH-major] Cervical Vertebrae. Diffusion Magnetic Resonance Imaging / methods. Rhabdoid Tumor / pathology. Spinal Cord Neoplasms / pathology. Teratoma / pathology

[MeSH-minor] Child. Diagnosis, Differential. Humans. Immunohistochemistry. Male

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(PMID = 19783876.001).

[ISSN] 1880-2206

[Journal-full-title] Magnetic resonance in medical sciences : MRMS : an official journal of Japan Society of Magnetic Resonance in Medicine

[ISO-abbreviation] Magn Reson Med Sci

[Language] eng

[Publication-type] Case Reports; Journal Article

[Publication-country] Japan

   

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

[Title] Atypical teratoid/rhabdoid tumors: imaging findings of two cases and review of the literature.

Atypical teratoid/rhabdoid tumor (AT/RT) is a malignant embryonal central nervous system (CNS) tumor, manifesting in children, and composed of rhabdoid cells, with or without fields resembling a classical primitive neuroectodermal tumor (PNET), epithelial tissue and neoplastic mesenchyme.

Around 200 cases of CNS AT/RT have been documented in the literature.

Although the clinical and pathological findings have been defined in large series previously, and AT/RT has become increasingly recognized, awareness of typical AT/RT is important in making the correct diagnosis of this uncommon but probably underdiagnosed entity.

Neuroradiologists rarely mention AT/RT in their differential diagnosis and this paper presents two additional cases in which clinical and pathological findings are combined with neuroradiological presentation.

[MeSH-major] Brain Neoplasms / pathology. Magnetic Resonance Imaging. Rhabdoid Tumor / pathology. Teratoma / pathology

[MeSH-minor] Child. Diagnosis, Differential. Fatal Outcome. Female. Humans. Infant. Male. Tomography, X-Ray Computed

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(PMID = 18382974.001).

[ISSN] 1019-5149

[Journal-full-title] Turkish neurosurgery

[ISO-abbreviation] Turk Neurosurg

[Language] eng

[Publication-type] Case Reports; Journal Article; Review

[Publication-country] Turkey

[Number-of-references] 18

   

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

[Title] Predisposition to atypical teratoid/rhabdoid tumor due to an inherited INI1 mutation.

BACKGROUND: Germline mutations of the INI1 gene predispose children to the development of rhabdoid tumors.

PROCEDURE: We have identified a three-generation family in which two half-brothers were diagnosed with central nervous system atypical teratoid/rhabdoid tumors (AT/RT).

A maternal uncle died in childhood from a brain tumor and a malignant rhabdoid tumor of the kidney, and presumably carried the same germline mutation.

CONCLUSION: The identification of two unaffected carriers in a family segregating a germline mutation and rhabdoid tumor supports the hypothesis that there may be variable risks of development of rhabdoid tumor in the context of a germline mutation.

There may be a developmental window in which most rhabdoid tumors occur.

This family highlights the importance of mutation analysis in all patients with a suspected rhabdoid tumor.

[MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Chromosomal Proteins, Non-Histone / genetics. DNA-Binding Proteins / genetics. Genetic Predisposition to Disease. Rhabdoid Tumor / genetics. Teratoma / genetics. Transcription Factors / genetics

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[CommentIn] Pediatr Blood Cancer. 2006 Sep;47(3):343-4 [16609948.001]

[CommentIn] Pediatr Blood Cancer. 2006 Sep;47(3):235-7 [16304667.001]

(PMID = 16261613.001).

[ISSN] 1545-5009

[Journal-full-title] Pediatric blood & cancer

[ISO-abbreviation] Pediatr Blood Cancer

[Language] eng

[Grant] United States / NCI NIH HHS / CA / CA46274; United States / NCI NIH HHS / CA / CA98543

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

[Publication-country] United States

[Chemical-registry-number] 0 / Chromosomal Proteins, Non-Histone; 0 / DNA-Binding Proteins; 0 / SMARCB1 protein, human; 0 / Transcription Factors

   

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

[Title] Cyclin D1 is overexpressed in atypical teratoid/rhabdoid tumor with hSNF5/INI1 gene inactivation.

OBJECT: Although atypical teratoid/rhabdoid tumor (AT/RT) is known to generate through inactivation of the hSNF5/INI1 gene on chromosome 22q, the downstream molecular mechanism remains unclear.

Because of the presence of rhabdoid cells and the polyimmunophenotypic features, the diagnosis was revised to AT/RT in five (31%) tumors, namely, two E IIIs and one each of medulloblastoma, CPC and pineoblastoma.

[MeSH-major] Central Nervous System Neoplasms / genetics. Chromosomes, Human, Pair 22 / genetics. Cyclin D1 / metabolism. DNA-Binding Proteins / genetics. Rhabdoid Tumor / genetics. Teratoma / genetics. Transcription Factors / genetics

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(PMID = 15981100.001).

[ISSN] 0167-594X

[Journal-full-title] Journal of neuro-oncology

[ISO-abbreviation] J. Neurooncol.

[Language] eng

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

[Publication-country] United States

[Chemical-registry-number] 0 / Chromosomal Proteins, Non-Histone; 0 / DNA-Binding Proteins; 0 / SMARCB1 Protein; 0 / SMARCB1 protein, human; 0 / Transcription Factors; 136601-57-5 / Cyclin D1

   

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

[Title] Atypical teratoid rhabdoid tumor (AT/RT) in adults: review of four cases.

Atypical teratoid/rhabdoid (AT/RT) tumor is a rare, highly malignant tumor of the central nervous system (CNS) most commonly found in children less than 5 years of age.

In many instances, a reliable diagnosis is not possible without demonstrating the lack of nuclear INI1 protein expression by immunohistochemical methods.

In all cases, diagnosis during intraoperative consultation and preliminary diagnosis was different from the final diagnosis after immunohistochemical analysis.

Immunohistochemical staining showed that the tumor cells were positive for vimentin and reacted variably for keratin, epithelial membrane antigen (EMA), synaptophysin, neurofilament protein, CD34, and smooth muscle actin (SMA).

One patient is alive with no evidence of disease 17 years after the diagnosis.

In adult examples of AT/RT, the diagnosis requires a high index of suspicion, with early tissue diagnosis and a low threshold for investigation with INI1 immunohistochemistry to differentiate this entity from other morphologically similar tumors.

[MeSH-major] Brain Neoplasms / metabolism. Brain Neoplasms / pathology. Rhabdoid Tumor / metabolism. Rhabdoid Tumor / pathology

[MeSH-minor] Adult. Chromosomal Proteins, Non-Histone / metabolism. DNA-Binding Proteins / metabolism. Diagnosis, Differential. Female. Humans. Immunohistochemistry. Male. SMARCB1 Protein. Transcription Factors / metabolism

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(PMID = 18369529.001).

[ISSN] 0167-594X

[Journal-full-title] Journal of neuro-oncology

[ISO-abbreviation] J. Neurooncol.

[Language] eng

[Publication-type] Case Reports; Journal Article

[Publication-country] United States

[Chemical-registry-number] 0 / Chromosomal Proteins, Non-Histone; 0 / DNA-Binding Proteins; 0 / SMARCB1 Protein; 0 / SMARCB1 protein, human; 0 / Transcription Factors

   

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

[Title] Identification of CD133-positive radioresistant cells in atypical teratoid/rhabdoid tumor.

Atypical teratoid/rhabdoid tumor (AT/RT) is an extremely malignant neoplasm in the central nervous system (CNS) which occurs in infancy and childhood.

[MeSH-major] Antigens, CD / immunology. Glycoproteins / immunology. Peptides / immunology. Radiation Tolerance. Rhabdoid Tumor / diagnosis

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(PMID = 18509505.001).

[ISSN] 1932-6203

[Journal-full-title] PloS one

[ISO-abbreviation] PLoS ONE

[Language] eng

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

[Publication-country] United States

[Chemical-registry-number] 0 / AC133 antigen; 0 / Antigens, CD; 0 / Glycoproteins; 0 / Peptides; 0 / Proto-Oncogene Proteins c-bcl-2; 0 / RNA, Small Interfering

[Other-IDs] NLM/ PMC2396792

   

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

[Title] Atypical teratoid or rhabdoid tumors: improved outcome with high-dose chemotherapy.

PURPOSE: To retrospectively review an institutional experience in managing atypical teratoid/rhabdoid tumors (AT/RT) of the Central Nervous System with high-dose chemotherapy in infants and children less than 4 years old.

Tumor location was supratentorial in 3 cases, infratentorial in 3 cases, and multifocal in 2 patients.

RESULTS: At a median follow-up of 52 months, 4 patients are alive without evidence of tumor.

[MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Brain Neoplasms / drug therapy. Rhabdoid Tumor / drug therapy. Teratoma / drug therapy

[MeSH-minor] Child, Preschool. Cisplatin / administration & dosage. Combined Modality Therapy. Cranial Irradiation. Cyclophosphamide / administration & dosage. Etoposide / administration & dosage. Female. Humans. Infant. Male. Neoplasm Metastasis. Radiotherapy Dosage. Retrospective Studies. Survival Rate. Treatment Outcome. Vincristine / administration & dosage

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[ErratumIn] J Pediatr Hematol Oncol. 2011 Jul;33(5):400. Laura, Janzen [corrected to Janzen, Laura]

(PMID = 20495479.001).

[ISSN] 1536-3678

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

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

[Language] eng

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

[Publication-country] United States

[Chemical-registry-number] 5J49Q6B70F / Vincristine; 6PLQ3CP4P3 / Etoposide; 8N3DW7272P / Cyclophosphamide; Q20Q21Q62J / Cisplatin

   

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

[Title] Role of high-dose chemotherapy (HDCT) in treatment of atypical teratoid/rhabdoid tumors (AT/RTs).

Atypical teratoid/rhabdoid tumors (AT/RTs) of the CNS have been recently characterized as a distinct clinicopathologic entity with an unusually poor prognosis and with the highest incidence in the first 2 years of life.

It often arises in the posterior fossa and its distinctive immunohistochemical (negative stain for INI-1) and cytogenetic features (monosomy or deletion of chromosome 22) permit an adequate diagnosis in most of cases.

AT/RT of the CNS is a usually fatal disease virtually unresponsive to chemotherapy (CT) and radiotherapy (RT).

Rapid progression and CNS dissemination are commonly reported.

Whether combined regimens including high-dose CT are able to prolong survival or change the natural history of this tumor are under evaluation.

[MeSH-major] Antineoplastic Agents / therapeutic use. Central Nervous System Neoplasms / drug therapy. Rhabdoid Tumor / drug therapy. Teratoma / drug therapy

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(PMID = 20146222.001).

[ISSN] 1545-5017

[Journal-full-title] Pediatric blood & cancer

[ISO-abbreviation] Pediatr Blood Cancer

[Language] eng

[Publication-type] Journal Article; Review

[Publication-country] United States

[Chemical-registry-number] 0 / Antineoplastic Agents

[Number-of-references] 18

   

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

[Title] Antisense treatment of IGF-IR induces apoptosis and enhances chemosensitivity in central nervous system atypical teratoid/rhabdoid tumours cells.

Central nervous system (CNS) atypical teratoid/rhabdoid tumours (AT/RT) are among the paediatric malignant tumours with the worst prognosis and fatal outcome.

Moreover, we found IGF-I and IGF-II mRNA in BT-16 CNS AT/RT cells and IGF-II mRNA in BT-12 CNS AT/RT cells, and autophosphorylated IGF-IR in both cell lines, indicating the potential presence of an autocrine/paracrine IGF-I/II/IGF-IR loop in CNS AT/RT.

IGF-IR antisense oligonucleotide treatment of human CNS AT/RT cells resulted in significant down-regulation of IGF-IR mRNA and protein expression, induction of apoptosis, and chemosensitisation to doxorubicin and cisplatin.

These studies provide evidence for the influence of IGF-IR on cellular responses to chemotherapy and raise the possibility that curability of selected CNS AT/RT may be improved by pharmaceutical strategies directed towards the IGF-IR.

[MeSH-major] Apoptosis / drug effects. Central Nervous System Neoplasms / drug therapy. Oligoribonucleotides, Antisense / therapeutic use. Receptor, IGF Type 1 / drug effects. Rhabdoid Tumor / drug therapy. Teratoma / drug therapy

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(PMID = 17446062.001).

[ISSN] 0959-8049

[Journal-full-title] European journal of cancer (Oxford, England : 1990)

[ISO-abbreviation] Eur. 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 / Oligoribonucleotides, Antisense; 67763-96-6 / Insulin-Like Growth Factor I; 80168379AG / Doxorubicin; EC 2.7.10.1 / Receptor, IGF Type 1; Q20Q21Q62J / Cisplatin

   

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

[Title] CT and MR imaging in atypical teratoid/rhabdoid tumors of the central nervous system.

INTRODUCTION: Atypical teratoid/rhabdoid tumors (ATRT) are rare aggressive neoplasms of the CNS affecting predominantly very young children.

Heterogeneity on imaging, large size and high tumor stages are striking features reflecting the aggressive nature of this histopathological entity.

Although not present in the majority of children, a distinct and unusual pattern of a wavy band-like enhancement surrounding a central hypointensity was present in 12 of 32 children (38%) in whom contrast medium was used.

[MeSH-major] Brain Neoplasms / diagnosis. Magnetic Resonance Imaging. Rhabdoid Tumor / diagnosis. Spinal Cord Neoplasms / diagnosis. Teratoma / diagnosis. Tomography, X-Ray Computed

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(PMID = 18345534.001).

[ISSN] 0028-3940

[Journal-full-title] Neuroradiology

[ISO-abbreviation] Neuroradiology

[Language] eng

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

[Publication-country] Germany

   

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

[Title] Primary intracranial atypical teratoid/rhabdoid tumors of infancy and childhood: MRI features and patient outcomes.

BACKGROUND AND PURPOSE: Primary atypical teratoid/rhabdoid tumors (AT/RTs) are rare malignant intracranial neoplasms, usually occurring in young children.

The objectives of this study were to characterize the MR imaging features and locations of primary intracranial AT/RTs, to determine the frequency of disseminated disease in the central nervous system (CNS) at diagnosis and postoperatively, and to assess patient outcomes.

METHODS: The preoperative cranial MR images of 13 patients with AT/RTs were retrospectively reviewed for evaluation of lesion location, size, MR signal intensity and enhancement characteristics, and the presence of disseminated intracranial tumor.

Postoperative MR images of the head and spine for 17 patients were reviewed for the presence of locally recurrent or residual tumor and disseminated neoplasm.

A germ-line mutation of the hSNF5/INI1 tumor-suppressor gene was responsible for the simultaneous occurrence of an intracranial AT/RT and a malignant renal rhabdoid tumor in a 4-month-old patient.

Mean tumor sizes were 3.6 x 3.8 x 3.9 cm.

The fraction of tumor volume showing enhancement was greater than two thirds in 58%, between one third and two thirds in 33%, and less than one third in 9%.

Disseminated tumor in the leptomeninges was seen with MR imaging in 24% of patients at diagnosis/initial staging and occurred in another 35% from 4 months to 2.8 years (mean, 1.1 years) after surgery and earlier imaging examinations with negative findings.

Patients with MR imaging evidence of disseminated leptomeningeal tumor had a median survival rate of 16 months compared with 149 months for those without disseminated tumor (P < .004, logrank test).

Poor prognosis is associated with MR imaging evidence of disseminated leptomeningeal tumor.

[MeSH-major] Brain Neoplasms / diagnosis. Magnetic Resonance Imaging. Rhabdoid Tumor / diagnosis. Teratoma / diagnosis

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(PMID = 16687525.001).

[ISSN] 0195-6108

[Journal-full-title] AJNR. American journal of neuroradiology

[ISO-abbreviation] AJNR Am J Neuroradiol

[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] A child with atypical teratoid/rhabdoid tumour of the posterior cranial fossa.

Primary central nervous system (CNS) atypical teratoid/rhabdoid tumours (ATRT) are highly malignant neoplasms which usually present in infancy or early childhood.

Although ATRT may arise anywhere within the CNS, the majority (approximately two-thirds) arise in the cerebellum or posterior fossa, and the remainder in the cerebrum.

We described the imaging characteristics of CNS ATRT in the posterior cranial fossa of a 14-month-old boy.

[MeSH-major] Cranial Fossa, Posterior. Rhabdoid Tumor / diagnosis. Skull Base Neoplasms / diagnosis. Teratoma / diagnosis

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(PMID = 19122937.001).

[ISSN] 0037-5675

[Journal-full-title] Singapore medical journal

[ISO-abbreviation] Singapore Med J

[Language] eng

[Publication-type] Case Reports; Journal Article

[Publication-country] Singapore

   

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

[Title] Cerebral composite atypical teratoid/rhabdoid tumor and yolk sac tumor in the frontal lobe of an infant. Case report.

A 1-year-old male infant presented with a rare cerebral composite tumor consisting of atypical teratoid/rhabdoid tumor (AT/RT) with epithelial and mesenchymal components and yolk sac tumor (YST) with Schiller-Duval bodies.

Computed tomography and magnetic resonance imaging revealed a large, intra-axial solid tumor with a cyst in the left frontal lobe.

Total resection of the tumor was performed.

Histological examination showed two different main growth patterns: solid sheets of undifferentiated polygonal cells and a few rhabdoid cells with rosette structures and rhabdomyoblastic cells; and reticular or papillary structures with occasional Schiller-Duval bodies in a myxoid matrix.

Initial total resection of the tumor was subsequently followed by local recurrence, hydrocephalus, and spinal metastasis.

AT/RT is a recently established entity of the central nervous system.

[MeSH-major] Brain Neoplasms / pathology. Endodermal Sinus Tumor / pathology. Frontal Lobe / pathology. Rhabdoid Tumor / pathology. Teratoma / pathology

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(PMID = 15973067.001).

[ISSN] 0470-8105

[Journal-full-title] Neurologia medico-chirurgica

[ISO-abbreviation] Neurol. Med. Chir. (Tokyo)

[Language] eng

[Publication-type] Case Reports; Journal Article

[Publication-country] Japan

   

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

[Title] Atypical teratoid/rhabdoid tumor involving cerebrospinal fluid: a case report.

BACKGROUND: Atypical teratoid/rhabdoid tumor (AT/RT) is a rare, aggressive tumor of the central nervous system.

It is important for pathologists to recognize the cytomorphologic features of AT/RT in the CSF of patients with this tumor to help determine prognosis and disease progression.

[MeSH-major] Rhabdoid Tumor / cerebrospinal fluid. Rhabdoid Tumor / pathology. Spinal Neoplasms / cerebrospinal fluid. Spinal Neoplasms / pathology. Teratoma / cerebrospinal fluid. Teratoma / pathology

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(PMID = 21053577.001).

[ISSN] 0001-5547

[Journal-full-title] Acta cytologica

[ISO-abbreviation] Acta Cytol.

[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] Intensive induction chemotherapy followed by high dose chemotherapy with autologous hematopoietic progenitor cell rescue in young children newly diagnosed with central nervous system atypical teratoid rhabdoid tumors.

BACKGROUND: Central nervous system (CNS) atypical teratoid rhabdoid tumors (AT/RT) are rare tumors of childhood with a dismal prognosis.

Historically, surgery and standard dose chemotherapy have resulted in a median survival of 8.5 months from diagnosis.

METHODS: Thirteen children newly diagnosed with CNS AT/RT were treated with either the "Head Start I" (HS I) or "Head Start II" (HS II) regimens.

There are presently three event-free survivors 42+, 54+, and 67+ months following diagnosis; none received RT.

CONCLUSION: Three of seven children with CNS AT/RT treated on HS II have experienced long term remissions.

Long term survival can be achieved in a subset of young children with CNS AT/RT following resection with the use of multi-drug chemotherapy including high dose methotrexate and myeloablative chemotherapy without radiation therapy (RT).

[MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Brain Neoplasms / therapy. Hematopoietic Stem Cell Transplantation. Rhabdoid Tumor / therapy. Teratoma / therapy

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(PMID = 18381756.001).

[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] Immunohistochemical analysis of INI1 protein in malignant pediatric CNS tumors: Lack of INI1 in atypical teratoid/rhabdoid tumors and in a fraction of primitive neuroectodermal tumors without rhabdoid phenotype.

Immunohistochemical lack of nuclear INI1 protein expression has been recently described as characteristic finding in atypical teratoid/rhabdoid tumors (AT/RTs), and has been suggested as useful marker to distinguish AT/RTs from other malignant pediatric central nervous system (CNS) tumors.

In this study, we examined a large series of malignant pediatric CNS tumors to determine the immunohistochemical expression of INI1 protein in different malignant pediatric tumor entities.

Archival paraffin-embedded biopsy specimens of 289 malignant pediatric CNS tumors including medulloblastomas, supratentorial primitive neuroectodermal tumors, glioblastomas, anaplastic astrocytomas, anaplastic ependymomas, choroid plexus carcinomas, germ cell tumors, and AT/RTs were analyzed immunohistochemically for expression of nuclear INI1 protein.

Seventeen of the 26 tumors showed morphologically characteristic features of AT/RTs, whereas 9 embryonal tumors did not display rhabdoid features.

Tumors without rhabdoid phenotype but lack of INI1 showed an aggressive clinical course and poor response to conventional treatment regimens.

Furthermore, a certain number of embryonal tumors without rhabdoid features but lack of INI1 protein and aggressive biologic behavior can be detected.

We conclude that INI1 protein analysis should be routinely performed in all malignant pediatric embryonal CNS tumors to detect cases with lack of INI1 protein, because patients with these tumors are likely to benefit from intensified treatment.

[MeSH-major] Biomarkers, Tumor / analysis. Brain Neoplasms / metabolism. Chromosomal Proteins, Non-Histone / metabolism. DNA-Binding Proteins / metabolism. Neuroectodermal Tumors, Primitive / metabolism. Rhabdoid Tumor / metabolism. Teratoma / metabolism. Transcription Factors / metabolism

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(PMID = 17063089.001).

[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

[Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Chromosomal Proteins, Non-Histone; 0 / DNA-Binding Proteins; 0 / SMARCB1 protein, human; 0 / Transcription Factors

   

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

[Title] Successful multimodal therapies for a primary atypical teratoid/rhabdoid tumor in the cervical spine.

Atypical teratoid/rhabdoid tumor (AT/RT) occurring in the central nervous system is a high-grade malignant tumor, and its prognosis is poor for patients younger than 3 years of age.

At the age of nearly 3 years, she received radiation therapy to the local tumor bed and craniospinal axis.

The success of this treatment for the patient was that we could prevent tumor recurrence until she was able to receive radiotherapy.

[MeSH-major] Cervical Vertebrae / pathology. Rhabdoid Tumor / drug therapy. Spinal Neoplasms / drug therapy. Teratoma / drug therapy

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[Copyright] (c) 2008 S. Karger AG, Basel.

(PMID = 18703889.001).

[ISSN] 1423-0305

[Journal-full-title] Pediatric neurosurgery

[ISO-abbreviation] Pediatr Neurosurg

[Language] eng

[Publication-type] Case Reports; Journal Article; Review

[Publication-country] Switzerland

[Chemical-registry-number] 0 / Antineoplastic Agents

[Number-of-references] 22

   

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

[Title] Incidence of atypical teratoid/rhabdoid tumors in children: a population-based study by the Austrian Brain Tumor Registry, 1996-2006.

BACKGROUND: Atypical teratoid/rhabdoid tumors are highly malignant embryonal central nervous system (CNS) tumors that were defined as an entity in 1996.

As compared with other malignant CNS tumors, their biological behavior is particularly aggressive, but patients may benefit from an intensified treatment.

Atypical teratoid/rhabdoid tumors display a complex histomorphology, which renders them prone to misdiagnosis.

They occur predominantly in young children, with an estimated prevalence of 1% to 2% among all pediatric CNS tumors.

METHODS: A nation-wide survey of malignant high-grade CNS tumors (World Health Organization grade III/IV), diagnosed in children (aged birth to 14 years) from 1996 to 2006 was conducted by the Austrian Brain Tumor Registry.

A central histopathology review was performed including the assessment of SMARCB1 (INI1) protein status.

RESULTS: A total of 311 newly diagnosed, malignant CNS tumors were included.

Atypical teratoid/rhabdoid tumors constituted the sixth most common entity (6.1%), referring to an age-standardized incidence rate of 1.38 per 1,000,000 person-years in children.

Peak incidence was found in the birth to 2 years age group, where they were as common as CNS primitive neuroectodermal tumors and medulloblastomas.

A total of 47.4% of atypical teratoid/rhabdoid tumors were initially diagnosed, whereas 52.6% were retrospectively detected by the central review.

The 5-year survival of atypical teratoid/rhabdoid tumor patients was 39.5%, with 66.7% in the correctly diagnosed group versus 15.0% in the not recognized group (P = .0469).

CONCLUSIONS: Clinicians and pathologists should be aware of the high incidence of atypical teratoid/rhabdoid tumors in young children to optimize diagnostic and therapeutic management of patients with these tumors.

[MeSH-major] Brain Neoplasms / epidemiology. Rhabdoid Tumor / epidemiology. Teratoma / epidemiology

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[Copyright] Copyright © 2010 American Cancer Society.

(PMID = 20737418.001).

[ISSN] 0008-543X

[Journal-full-title] Cancer

[ISO-abbreviation] Cancer

[Language] eng

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

[Publication-country] United States

[Investigator] Azizi AA; Scarpatetti M; Ebetsberger G; Weis S; Jones N; Klein-Franke A; Sterlacci W; Jauk B; Kiefer A; Mueller G; Gruber-Moesenbacher U; Reiner-Concin A; Feichtinger H

   

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

[Title] Malignant rhabdoid tumor in a pregnant adult female: literature review of central nervous system rhabdoid tumors.

Rhabdoid tumors of the central nervous system are uncommon, aggressive childhood malignancies.

The 13 described adult cases comprise both primary CNS tumors and malignant transformation of previously existing gliomas, meningiomas, and astrocytomas.

Central nervous system rhabdoid lesions of adults have been diagnosed as primary malignant rhabdoid tumors, atypical teratoid/rhabdoid tumors, and more recently, rhabdoid glioblastomas.

Pathologic evaluation revealed histology, electron microscopy and immunohistochemistry consistent with the diagnosis of malignant rhabdoid tumor.

FISH studies were negative for the INI-1 genetic mutations and chromosome 22q deletion associated with childhood atypical rhabdoid/rhabdoid tumor in 75% of cases.

We briefly describe the characteristics and current understanding of rhabdoid tumors, and review the literature comparing the 12 other cases of central nervous system rhabdoid tumors in adults.

[MeSH-major] Brain Neoplasms / pathology. Pregnancy Complications, Neoplastic / pathology. Rhabdoid Tumor / pathology

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(PMID = 16132523.001).

[ISSN] 0167-594X

[Journal-full-title] Journal of neuro-oncology

[ISO-abbreviation] J. Neurooncol.

[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] Establishment of atypical-teratoid/rhabdoid tumor (AT/RT) cell cultures from disseminated CSF cells: a model to elucidate biology and potential targeted therapeutics.

Atypical teratoid/rhabdoid tumor (AT/RT) is a highly malignant central nervous system neoplasm that usually affects infants and young children.

In this report, we describe culture conditions that enabled the sustained growth of tumor cells obtained from the cerebrospinal fluid (CSF) of an infant with AT/RT.

These cells retained the morphological and biomarker characteristics of the original tumor.

In addition to previously described cell lines and xenograft models, continuous culture of CSF derived cells may also provide an effective way to study the biology of AT/RT and to identify potential targets for future therapeutics for this tumor.

[MeSH-major] Platelet Aggregation Inhibitors / therapeutic use. Rhabdoid Tumor / cerebrospinal fluid. Rhabdoid Tumor / drug therapy

[MeSH-minor] Actins / metabolism. Cell Proliferation. Chromosomal Proteins, Non-Histone / metabolism. DNA-Binding Proteins / metabolism. Dose-Response Relationship, Drug. Glial Fibrillary Acidic Protein / metabolism. Humans. Infant. Inhibitory Concentration 50. Male. Models, Biological. Nuclear Proteins / metabolism. Receptor Protein-Tyrosine Kinases / metabolism. SMARCB1 Protein. Transcription Factors / metabolism. Tumor Cells, Cultured / pathology. Vimentin / metabolism

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(PMID = 18651103.001).

[ISSN] 0167-594X

[Journal-full-title] Journal of neuro-oncology

[ISO-abbreviation] J. Neurooncol.

[Language] eng

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

[Publication-country] United States

[Chemical-registry-number] 0 / Actins; 0 / Chromosomal Proteins, Non-Histone; 0 / DNA-Binding Proteins; 0 / Glial Fibrillary Acidic Protein; 0 / Nuclear Proteins; 0 / Platelet Aggregation Inhibitors; 0 / SMARCB1 Protein; 0 / SMARCB1 protein, human; 0 / Transcription Factors; 0 / Vimentin; EC 2.7.10.1 / Receptor Protein-Tyrosine Kinases