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Article ; 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 Volume 35, Issue 12, Page(s) 1757–1758

MeSH term(s)	Adult ; Humans ; Rhabdoid Tumor/genetics
Language	English
Publishing date	2022-09-21
Publishing country	United States
Document type	Editorial
ZDB-ID	645073-8
ISSN	1530-0285 ; 0893-3952
ISSN (online)	1530-0285
ISSN	0893-3952
DOI	10.1038/s41379-022-01144-1
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Zs.A 2450: Show issues

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Book ; Thesis: Isolation und immunologische Charakterisierung von Stroma-Zellen pädiatrischer Tumore

Johann, Pascal-David

2011

Author's details	vorgelegt von Pascal-David Johann
Language	German
Size	112 S., graph. Darst.
Publishing country	Germany
Document type	Book ; Thesis
Thesis / German Habilitation thesis	Tübingen, Univ., Diss., 2011
HBZ-ID	HT017146533
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Article: 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 Volume 14, Issue 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.
Language	English
Publishing date	2022-10-14
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	2527080-1
ISSN	2072-6694
ISSN	2072-6694
DOI	10.3390/cancers14205044
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Article: 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 Volume 14, Page(s) 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
Language	English
Publishing date	2022-02-09
Publishing country	New Zealand
Document type	Journal Article ; Review
ZDB-ID	2508013-1
ISSN	1179-1322
ISSN	1179-1322
DOI	10.2147/CMAR.S289544
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Article ; 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 Volume 44, Issue 7, Page(s) 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 term(s)	Body Weight ; Child ; Granulocytes ; Hematology ; Humans ; Neutropenia/etiology ; Retrospective Studies
Language	English
Publishing date	2021-10-26
Publishing country	United States
Document type	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
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Article ; 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 Volume 134, Issue 5, Page(s) 817–818

Language	English
Publishing date	2017
Publishing country	Germany
Document type	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
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Article ; 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 Volume 34, Issue 19-20, Page(s) 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 term(s)	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
Chemical Substances	SMARCB1 Protein ; SMARCB1 protein, human
Language	English
Publishing date	2020-09-10
Publishing country	United States
Document type	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
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Zs.A 2292: Show issues			Location: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular Jg. 1995 - 2021: Lesesall (2.OG) ab Jg. 2022: Lesesaal (EG)
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Article: 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 Volume 15, Issue 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.
Language	English
Publishing date	2022-12-30
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	2527080-1
ISSN	2072-6694
ISSN	2072-6694
DOI	10.3390/cancers15010225
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Article ; Online: Histopathological patterns in atypical teratoid/rhabdoid tumors are related to molecular subgroup.

Zin, Francesca / Cotter, Jennifer A / Haberler, Christine / Dottermusch, Matthias / Neumann, Julia / Schüller, Ulrich / Schweizer, Leonille / Thomas, Christian / Nemes, Karolina / Johann, Pascal D / Kool, Marcel / Frühwald, Michael C / Paulus, Werner / Judkins, Alexander / Hasselblatt, Martin

Brain pathology (Zurich, Switzerland)

2021 Volume 31, Issue 5, Page(s) e12967

Abstract: Atypical teratoid/rhabdoid tumor (AT/RT) is a highly malignant tumor that may not only contain rhabdoid tumor cells but also poorly differentiated small-round-blue cells as well as areas with mesenchymal or epithelial differentiation. Little is known on ... ...

Abstract	Atypical teratoid/rhabdoid tumor (AT/RT) is a highly malignant tumor that may not only contain rhabdoid tumor cells but also poorly differentiated small-round-blue cells as well as areas with mesenchymal or epithelial differentiation. Little is known on factors associated with histopathological diversity. Recent studies demonstrated three molecular subgroups of AT/RT, namely ATRT-TYR, ATRT-SHH, and ATRT-MYC. We thus aimed to investigate if morphological patterns might be related to molecular subgroup status. Hematoxylin-eosin stained sections of 114 AT/RT with known molecular subgroup status were digitalized and independently categorized by nine blinded observers into four morphological categories, that is, "rhabdoid," "small-round-blue," "epithelial," and "mesenchymal." The series comprised 48 ATRT-SHH, 40 ATRT-TYR, and 26 ATRT-MYC tumors. Inter-observer agreement was moderate but significant (Fleiss' kappa = 0.47; 95% C.I. 0.41-0.53; p < 0.001) and there was a highly significant overall association between morphological categories and molecular subgroups for each of the nine observers (p < 0.0001). Specifically, the category "epithelial" was found to be over-represented in ATRT-TYR (p < 0.000001) and the category "small-round-blue" to be over-represented in ATRT-SHH (p < 0.01). The majority of ATRT-MYC was categorized as "mesenchymal" or "rhabdoid," but this association was less compelling. The specificity of the category "epithelial" for ATRT-TYR was highest and accounted for 97% (range: 88-99%) whereas sensitivity was low [49% (range: 35%-63%)]. In line with these findings, cytokeratin-positivity was highly overrepresented in ATRT-TYR. In conclusion, morphological features of AT/RT might reflect molecular alterations and may also provide a first hint on molecular subgroup status, which will need to be confirmed by DNA methylation profiling.
MeSH term(s)	Adolescent ; Adult ; Biomarkers, Tumor/genetics ; Central Nervous System Neoplasms/genetics ; Central Nervous System Neoplasms/pathology ; Child ; DNA Methylation/genetics ; Female ; Humans ; Male ; Neoplasms, Neuroepithelial/genetics ; Neoplasms, Neuroepithelial/pathology ; Rhabdoid Tumor/pathology ; SMARCB1 Protein/genetics ; Teratoma/pathology ; Young Adult
Chemical Substances	Biomarkers, Tumor ; SMARCB1 Protein
Language	English
Publishing date	2021-05-03
Publishing country	Switzerland
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	1051484-3
ISSN	1750-3639 ; 1015-6305
ISSN (online)	1750-3639
ISSN	1015-6305
DOI	10.1111/bpa.12967
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Article ; Online: Transcriptional immunogenomic analysis reveals distinct immunological clusters in paediatric nervous system tumours.

Genome medicine

2023 Volume 15, Issue 1, Page(s) 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 term(s)	Adult ; Humans ; Child ; Nervous System Neoplasms ; B-Lymphocytes ; Immune Checkpoint Inhibitors ; Immunotherapy ; Tumor Microenvironment/genetics
Chemical Substances	Immune Checkpoint Inhibitors
Language	English
Publishing date	2023-09-07
Publishing country	England
Document type	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
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