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Artikel ; Online: "Adult rhabdoid tumors-a riddle inside an enigma?"

Johann, Pascal D

Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc

2022 Band 35, Heft 12, Seite(n) 1757–1758

Mesh-Begriff(e)	Adult ; Humans ; Rhabdoid Tumor/genetics
Sprache	Englisch
Erscheinungsdatum	2022-09-21
Erscheinungsland	United States
Dokumenttyp	Editorial
ZDB-ID	645073-8
ISSN	1530-0285 ; 0893-3952
ISSN (online)	1530-0285
ISSN	0893-3952
DOI	10.1038/s41379-022-01144-1
Datenquelle	MEDical Literature Analysis and Retrieval System OnLINE

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Buch ; Dissertation / Habilitation: Isolation und immunologische Charakterisierung von Stroma-Zellen pädiatrischer Tumore

Johann, Pascal-David

2011

Verfasserangabe	vorgelegt von Pascal-David Johann
Sprache	Deutsch
Umfang	112 S., graph. Darst.
Erscheinungsland	Deutschland
Dokumenttyp	Buch ; Dissertation / Habilitation
Dissertation / Habilitation	Tübingen, Univ., Diss., 2011
HBZ-ID	HT017146533
Datenquelle	Katalog ZB MED Medizin, Gesundheit

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2012 D 136	bestellbar zur Ausleihe	Magazin

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Artikel: Renal Medullary Carcinomas Harbor a Distinct Methylation Phenotype and Display Aberrant Methylation of Genes Related to Early Nephrogenesis.

Fincke, Victoria E / Krulik, Mateja E / Joshi, Piyush / Frühwald, Michael C / Chen, Ying-Bei / Johann, Pascal D

Cancers

2022 Band 14, Heft 20

Abstract: Renal medullary carcinomas (RMC) are rare aggressive tumors of the kidneys, characterized by a loss of SMARCB1. Characteristically, these tumors arise in patients with sickle cell trait or other hemoglobinopathies. Recent characterization efforts have ... ...

Abstract	Renal medullary carcinomas (RMC) are rare aggressive tumors of the kidneys, characterized by a loss of SMARCB1. Characteristically, these tumors arise in patients with sickle cell trait or other hemoglobinopathies. Recent characterization efforts have unraveled oncogenic pathways that drive tumorigenesis. Among these, gene sets that characterize replicative stress and the innate immune response are upregulated in RMCs. Despite comprehensive genetic and transcriptomic characterizations, commonalities or differences to other SMARCB1 deficient entities so far have not been investigated. We analyzed the methylome of seven primary RMC and compared it to other SMARCB1 deficient entities such as rhabdoid tumors (RT) and epithelioid sarcomas using 850 K methylation arrays. Moreover, we evaluated the differential gene expression of RMC using RNA-sequencing in comparison to other rhabdoid tumors. In accordance with previous gene expression data, we found that RMCs separate from other SMARCB1 deficient entities, pointing to a potentially different cell of origin and a role of additional genetic aberrations that may drive tumorigenesis and thus alter the methylome when compared to rhabdoid tumors. In a focused analysis of genes that are important for nephrogenesis, we particularly detected genes that govern early nephrogenesis such as FOXI1 to be hypomethylated and expressed at high levels in RMC. Overall, our analyses underscore the fact that RMCs represent a separate entity with limited similarities to rhabdoid tumors, warranting specific treatment tailored to the aggressiveness of the disease.
Sprache	Englisch
Erscheinungsdatum	2022-10-14
Erscheinungsland	Switzerland
Dokumenttyp	Journal Article
ZDB-ID	2527080-1
ISSN	2072-6694
ISSN	2072-6694
DOI	10.3390/cancers14205044
Datenquelle	MEDical Literature Analysis and Retrieval System OnLINE

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Artikel: Current and Emerging Therapeutic Approaches for Extracranial Malignant Rhabdoid Tumors.

Nemes, Karolina / Johann, Pascal D / Tüchert, Stefanie / Melchior, Patrick / Vokuhl, Christian / Siebert, Reiner / Furtwängler, Rhoikos / Frühwald, Michael C

Cancer management and research

2022 Band 14, Seite(n) 479–498

Abstract: Extracranial malignant rhabdoid tumors (extracranial MRT) are rare, highly aggressive malignancies affecting mainly infants and children younger than 3 years. Common anatomic sites comprise the kidneys (RTK - rhabdoid tumor of kidney) and other soft ... ...

Abstract	Extracranial malignant rhabdoid tumors (extracranial MRT) are rare, highly aggressive malignancies affecting mainly infants and children younger than 3 years. Common anatomic sites comprise the kidneys (RTK - rhabdoid tumor of kidney) and other soft tissues (eMRT - extracranial, extrarenal malignant rhabdoid tumor). The genetic origin of these diseases is linked to biallelic pathogenic variants in the genes
Sprache	Englisch
Erscheinungsdatum	2022-02-09
Erscheinungsland	New Zealand
Dokumenttyp	Journal Article ; Review
ZDB-ID	2508013-1
ISSN	1179-1322
ISSN	1179-1322
DOI	10.2147/CMAR.S289544
Datenquelle	MEDical Literature Analysis and Retrieval System OnLINE

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Artikel ; Online: Cui Bono? Identifying Patient Groups That May Benefit From Granulocyte Transfusions in Pediatric Hematology and Oncology.

Johann, Pascal D / Wuchter, Patrick / Trojanova, Lenka / Sturm, Dominik / Lu, Kevin Hai-Ning / Kulozik, Andreas E / Kunz, Joachim B

Journal of pediatric hematology/oncology

2021 Band 44, Heft 7, Seite(n) e968–e975

Abstract: Introduction: Granulocyte transfusions have long been used to bridge the time to neutrophil recovery in patients with neutropenia and severe infection. Recent randomized controlled trials did not prove a beneficial effect of granulocyte transfusions, ... ...

Abstract	Introduction: Granulocyte transfusions have long been used to bridge the time to neutrophil recovery in patients with neutropenia and severe infection. Recent randomized controlled trials did not prove a beneficial effect of granulocyte transfusions, but were likely underpowered and suffered from very heterogeneous study populations. Methods: We retrospectively reviewed data of all patients treated with granulocyte transfusions at our pediatric center from 2004 to 2019. To identify parameters that predict the success of granulocyte transfusions, we stratified patients in 3 groups. Patients in group 1 cleared their infection, whereas patients in group 2 succumbed to an infection in neutropenia despite granulocyte transfusions. A third group included all patients who died of causes that were not related to infection. Results: We demonstrate that patients without respiratory or cardiocirculatory insufficiency are enriched in group 1 and more likely to benefit from granulocyte transfusions than patients who already require these intensive care measures. The effect of granulocyte transfusions correlates with the cell dose per body weight applied per time. With our standard twice weekly dosing, patients with a body weight below 40 kg are more likely to achieve a sufficient leukocyte increment and clear their infection in comparison to patients with a higher body weight. Discussion/conclusions: We suggest that future studies on the benefits of granulocyte transfusions stratify patients according to clinical risk factors that include the need for respiratory or cardiocirculatory support and strive for a sufficient dose density of granulocyte transfusions.
Mesh-Begriff(e)	Body Weight ; Child ; Granulocytes ; Hematology ; Humans ; Neutropenia/etiology ; Retrospective Studies
Sprache	Englisch
Erscheinungsdatum	2021-10-26
Erscheinungsland	United States
Dokumenttyp	Journal Article
ZDB-ID	1231152-2
ISSN	1536-3678 ; 1077-4114 ; 0192-8562
ISSN (online)	1536-3678
ISSN	1077-4114 ; 0192-8562
DOI	10.1097/MPH.0000000000002349
Datenquelle	MEDical Literature Analysis and Retrieval System OnLINE

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Artikel ; Online: Reduced histone H3 K27 trimethylation is encountered in about 50% of atypical teratoid/rhabdoid tumors (AT/RT) but is not associated with molecular subgroup status and outcome.

Hasselblatt, Martin / Johann, Pascal D / Kool, Marcel / Frühwald, Michael C

Acta neuropathologica

2017 Band 134, Heft 5, Seite(n) 817–818

Sprache	Englisch
Erscheinungsdatum	2017
Erscheinungsland	Germany
Dokumenttyp	Letter ; Research Support, Non-U.S. Gov't
ZDB-ID	1079-0
ISSN	1432-0533 ; 0001-6322
ISSN (online)	1432-0533
ISSN	0001-6322
DOI	10.1007/s00401-017-1766-y
Datenquelle	MEDical Literature Analysis and Retrieval System OnLINE

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Artikel ; Online: SMARCB1 loss interacts with neuronal differentiation state to block maturation and impact cell stability.

Parisian, Alison D / Koga, Tomoyuki / Miki, Shunichiro / Johann, Pascal D / Kool, Marcel / Crawford, John R / Furnari, Frank B

Genes & development

2020 Band 34, Heft 19-20, Seite(n) 1316–1329

Abstract: Atypical teratoid rhabdoid tumors (ATRTs) are challenging pediatric brain cancers that are predominantly associated with inactivation of the ... ...

Abstract	Atypical teratoid rhabdoid tumors (ATRTs) are challenging pediatric brain cancers that are predominantly associated with inactivation of the gene
Mesh-Begriff(e)	Brain Neoplasms/genetics ; Carcinogenesis/genetics ; Cell Differentiation/genetics ; Gene Deletion ; Gene Expression Profiling ; Gene Expression Regulation, Neoplastic/genetics ; Gene Knockdown Techniques ; Humans ; Induced Pluripotent Stem Cells ; Neurons/cytology ; Organoids/cytology ; Organoids/physiopathology ; Rhabdoid Tumor/genetics ; SMARCB1 Protein/genetics
Chemische Substanzen	SMARCB1 Protein ; SMARCB1 protein, human
Sprache	Englisch
Erscheinungsdatum	2020-09-10
Erscheinungsland	United States
Dokumenttyp	Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't
ZDB-ID	806684-x
ISSN	1549-5477 ; 0890-9369
ISSN (online)	1549-5477
ISSN	0890-9369
DOI	10.1101/gad.339978.120
Datenquelle	MEDical Literature Analysis and Retrieval System OnLINE

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Artikel ; Online: Transcriptional immunogenomic analysis reveals distinct immunological clusters in paediatric nervous system tumours.

Genome medicine

2023 Band 15, Heft 1, Seite(n) 67

Abstract: Background: Cancer immunotherapies including immune checkpoint inhibitors and Chimeric Antigen Receptor (CAR) T-cell therapy have shown variable response rates in paediatric patients highlighting the need to establish robust biomarkers for patient ... ...

Abstract	Background: Cancer immunotherapies including immune checkpoint inhibitors and Chimeric Antigen Receptor (CAR) T-cell therapy have shown variable response rates in paediatric patients highlighting the need to establish robust biomarkers for patient selection. While the tumour microenvironment in adults has been widely studied to delineate determinants of immune response, the immune composition of paediatric solid tumours remains relatively uncharacterized calling for investigations to identify potential immune biomarkers. Methods: To inform immunotherapy approaches in paediatric cancers with embryonal origin, we performed an immunogenomic analysis of RNA-seq data from 925 treatment-naïve paediatric nervous system tumours (pedNST) spanning 12 cancer types from three publicly available data sets. Results: Within pedNST, we uncovered four broad immune clusters: Paediatric Inflamed (10%), Myeloid Predominant (30%), Immune Neutral (43%) and Immune Desert (17%). We validated these clusters using immunohistochemistry, methylation immune inference and segmentation analysis of tissue images. We report shared biology of these immune clusters within and across cancer types, and characterization of specific immune cell frequencies as well as T- and B-cell repertoires. We found no associations between immune infiltration levels and tumour mutational burden, although molecular cancer entities were enriched within specific immune clusters. Conclusions: Given the heterogeneity of immune infiltration within pedNST, our findings suggest personalized immunogenomic profiling is needed to guide selection of immunotherapeutic strategies.
Mesh-Begriff(e)	Adult ; Humans ; Child ; Nervous System Neoplasms ; B-Lymphocytes ; Immune Checkpoint Inhibitors ; Immunotherapy ; Tumor Microenvironment/genetics
Chemische Substanzen	Immune Checkpoint Inhibitors
Sprache	Englisch
Erscheinungsdatum	2023-09-07
Erscheinungsland	England
Dokumenttyp	Journal Article ; Research Support, Non-U.S. Gov't ; Research Support, N.I.H., Extramural
ZDB-ID	2484394-5
ISSN	1756-994X ; 1756-994X
ISSN (online)	1756-994X
ISSN	1756-994X
DOI	10.1186/s13073-023-01219-x
Datenquelle	MEDical Literature Analysis and Retrieval System OnLINE

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Artikel ; Online: Recurrent atypical teratoid/rhabdoid tumors (AT/RT) reveal discrete features of progression on histology, epigenetics, copy number profiling, and transcriptomics.

Acta neuropathologica

2023 Band 146, Heft 3, Seite(n) 527–541

Abstract: Atypical teratoid/rhabdoid tumors (AT/RT) are the most common malignant brain tumors manifesting in infancy. They split into four molecular types. The major three (AT/RT-SHH, AT/RT-TYR, and AT/RT-MYC) all carry mutations in SMARCB1, the fourth ... ...

Abstract	Atypical teratoid/rhabdoid tumors (AT/RT) are the most common malignant brain tumors manifesting in infancy. They split into four molecular types. The major three (AT/RT-SHH, AT/RT-TYR, and AT/RT-MYC) all carry mutations in SMARCB1, the fourth quantitatively smaller type is characterized by SMARCA4 mutations (AT/RT-SMARCA4). Molecular characteristics of disease recurrence or metastatic spread, which go along with a particularly dismal outcome, are currently unclear. Here, we investigated tumor tissue from 26 patients affected by AT/RT to identify signatures of recurrences in comparison with matched primary tumor samples. Microscopically, AT/RT recurrences demonstrated a loss of architecture and significantly enhanced mitotic activity as compared to their related primary tumors. Based on DNA methylation profiling, primary tumor and related recurrence were grossly similar, but three out of 26 tumors belonged to a different molecular type or subtype after second surgery compared to related primary lesions. Copy number variations (CNVs) differed in six cases, showing novel gains on chromosome 1q or losses of chromosome 10 in recurrences as the most frequent alterations. To consolidate these observations, our cohort was combined with a data set of unmatched primary and recurrent AT/RT, which demonstrated chromosome 1q gain and 10 loss in 18% (n = 7) and 11% (n = 4) of the recurrences (n = 38) as compared to 7% (n = 3) and 0% (n = 0) in the primary tumors (n = 44), respectively. Similar to the observations made by DNA methylation profiling, RNA sequencing of our cohort revealed AT/RT primary tumors and matched recurrences clustering closely together. However, a number of genes showed significantly altered expression in AT/RT-SHH recurrences. Many of them are known tumor driving growth factors, involved in embryonal development and tumorigenesis, or are cell-cycle-associated. Overall, our work identifies subtle molecular changes that occur in the course of the disease and that may help define novel therapeutic targets for AT/RT recurrences.
Mesh-Begriff(e)	Child ; Child, Preschool ; Female ; Humans ; Infant ; Male ; Chromosomes, Human, Pair 1/genetics ; Chromosomes, Human, Pair 10/genetics ; Cohort Studies ; Dendritic Cells ; Disease Progression ; DNA Copy Number Variations/genetics ; DNA Methylation ; Epigenesis, Genetic ; Gene Expression Profiling ; Histology ; Mitosis ; Recurrence ; Rhabdoid Tumor/classification ; Rhabdoid Tumor/genetics ; Rhabdoid Tumor/immunology ; Rhabdoid Tumor/pathology ; Sequence Analysis, RNA ; Teratoma/classification ; Teratoma/genetics ; Teratoma/immunology ; Teratoma/pathology ; Transcription Factors/genetics ; Gene Expression Regulation, Neoplastic/genetics
Chemische Substanzen	Transcription Factors ; SMARCA4 protein, human (EC 3.6.1.-) ; SMARCB1 protein, human
Sprache	Englisch
Erscheinungsdatum	2023-07-14
Erscheinungsland	Germany
Dokumenttyp	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	1079-0
ISSN	1432-0533 ; 0001-6322
ISSN (online)	1432-0533
ISSN	0001-6322
DOI	10.1007/s00401-023-02608-7
Datenquelle	MEDical Literature Analysis and Retrieval System OnLINE

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Artikel: Outcome for Pediatric Adreno-Cortical Tumors Is Best Predicted by the COG Stage and Five-Item Microscopic Score-Report from the German MET Studies.

Kuhlen, Michaela / Kunstreich, Marina / Wudy, Stefan A / Holterhus, Paul-Martin / Lessel, Lienhard / Schneider, Dominik T / Brecht, Ines B / Schewe, Denis M / Seitz, Guido / Roecken, Christoph / Vokuhl, Christian / Johann, Pascal D / Frühwald, Michael C / Vorwerk, Peter / Redlich, Antje

Cancers

2022 Band 15, Heft 1

Abstract: Background: Adrenocortical tumors (ACTs) encompassing the adrenocortical adenoma (ACA), carcinoma (ACC), and tumors of undetermined malignant potential (ACx) are rare endocrine neoplasms with a poor prognosis. We report on pediatric ACT patients ... ...

Abstract	Background: Adrenocortical tumors (ACTs) encompassing the adrenocortical adenoma (ACA), carcinoma (ACC), and tumors of undetermined malignant potential (ACx) are rare endocrine neoplasms with a poor prognosis. We report on pediatric ACT patients registered with the Malignant Endocrine Tumor studies and explore the EXPeRT recommendations for management. Patients: Data from the ACT patients (<18 years) were analyzed. For the risk prediction, the patients were retrospectively assigned to the COG stages and the five-item score. Results: By December 2021, 161 patients with ACT (ACA n = 51, ACx n = 19, and ACC n = 91) had been reported (the median age at the diagnosis was 4.3 years with a range of 0.1−17.8), with lymph node and distant metastases in 10.7% and 18.9% of the patients with ACC/ACx. The mean follow-up was 4.5 years (with a range of 0−16.7). The three-year overall (OS) and event-free survival (EFS) rates were 65.5% and 50.6%. In the univariate analyses, the OS was impaired for patients aged ≥ 4 years (p = 0.001) with the initial biopsy (p = 0.016), tumor spillage (p = 0.028), incomplete tumor resection (p < 0.001), unfavorable histology (p = 0.047), and COG stages III/IV (p = 0.002). Multivariate analysis revealed COG stages III/IV and an unfavorable five-item score as independent negative prognostic factors for the EFS and OS. Conclusions: Age defines the clinical presentation and prognosis in pediatric ACTs. The outcome is best predicted by the COG stage and five-item score.
Sprache	Englisch
Erscheinungsdatum	2022-12-30
Erscheinungsland	Switzerland
Dokumenttyp	Journal Article
ZDB-ID	2527080-1
ISSN	2072-6694
ISSN	2072-6694
DOI	10.3390/cancers15010225
Datenquelle	MEDical Literature Analysis and Retrieval System OnLINE

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