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Your search keyword '"Hans Grossniklaus"' showing total 4 results
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4 results on '"Hans Grossniklaus"'

1. Differentiating MYCN-amplified RB1 wild-type retinoblastoma from biallelic RB1 mutant retinoblastoma using MR-based radiomics: a retrospective multicenter case–control study

Author

Christiaan M. de Bloeme, Robin W. Jansen, Liesbeth Cardoen, Sophia Göricke, Sabien van Elst, Jaime Lyn Jessen, Aparna Ramasubramanian, Alison H. Skalet, Audra K. Miller, Philippe Maeder, Ogul E. Uner, G. Baker Hubbard, Hans Grossniklaus, H. Culver Boldt, Kim E. Nichols, Rachel C. Brennan, Saugata Sen, Mériam Koob, Selma Sirin, Hervé J. Brisse, Paolo Galluzzi, Charlotte J. Dommering, Matthijs Cysouw, Ronald Boellaard, Josephine C. Dorsman, Annette C. Moll, Marcus C. de Jong, Pim de Graaf, and European Retinoblastoma Imaging Collaboration

Subjects
Retinoblastoma, MRI, radiomics, MYCN-amplification, Medicine, Science
Abstract

Abstract MYCN-amplified RB1 wild-type (MYCN amp RB1 +/+) retinoblastoma is a rare and aggressive subtype, often resistant to standard therapies. Identifying unique MRI features is crucial for diagnosing this subtype, as biopsy is not recommended. This study aimed to differentiate MYCN amp RB1 +/+ from the most prevalent RB1 -/- retinoblastoma using pretreatment MRI and radiomics. Ninety-eight unilateral retinoblastoma patients (19 MYCN cases and 79 matched controls) were included. Tumors on T2-weighted MR images were manually delineated and validated by experienced radiologists. Radiomics analysis extracted 120 features per tumor. Several combinations of feature selection methods, oversampling techniques and machine learning (ML) classifiers were evaluated in a repeated fivefold cross-validation machine learning pipeline to yield the best-performing prediction model for MYCN. The best model used univariate feature selection, data oversampling (duplicating MYCN cases), and logistic regression classifier, achieving a mean AUC of 0.78 (SD 0.12). SHAP analysis highlighted lower sphericity, higher flatness, and greater gray-level heterogeneity as predictive for MYCN amp RB1 +/+ status, yielding an AUC of 0.81 (SD 0.11). This study shows the potential of MRI-based radiomics to distinguish MYCN amp RB1 +/+ and RB1 -/- retinoblastoma subtypes.

Published
2024
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2. Isolated hemangiopericytoma of the conjunctiva

Author

William Edwards, Caroline Craven, Joseph Ryan Turner, Hans Grossniklaus, and Jill Wells

Subjects
Conjunctiva, Hemangiopericytoma, Tumor, Ophthalmology, RE1-994
Abstract

Purpose: To report a unique presentation of hemangiopericytoma and discuss the clinical course, pathological features, and management of this tumor. Observations: An otherwise healthy 54-year-old Caucasian female presented with a painless conjunctival mass. The lesion gradually enlarged over a three-week period and was unresponsive to corticosteroid treatment. The mass was surgically removed, and histopathologic findings were consistent with hemangiopericytoma. Conclusions and importance: Conjunctival hemangiopericytoma should be considered in patients with conjunctival lesions unresponsive to medical management. Surgical excision is diagnostic and therapeutic and is the strongest predictor of clinical course. Incompletely excised lesions are at a greater risk of local recurrence and subsequent metastasis. Given the neoplasm's malignant potential, patients should be followed in the outpatient setting.

Published
2022
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3. MRI Features for Identifying MYCN-amplified RB1 Wild-type Retinoblastoma

Author

Robin W. Jansen, Christiaan M. de Bloeme, Liesbeth Cardoen, Sophia Göricke, Sabien van Elst, Jaime Lyn Jessen, Aparna Ramasubramanian, Alison H. Skalet, Audra K. Miller, Philippe Maeder, Ogul E. Uner, G. Baker Hubbard, Hans Grossniklaus, H. Culver Boldt, Kim E. Nichols, Rachel C. Brennan, Saugata Sen, Selma Sirin, Hervé J. Brisse, Paolo Galluzzi, Charlotte J. Dommering, Jonas A. Castelijns, Paul van der Valk, Ronald Boellaard, Josephine Dorsman, Annette C. Moll, Marcus C. de Jong, Pim de Graaf, University of Zurich, Radiology and nuclear medicine, Human genetics, CCA - Cancer biology and immunology, Pathology, Amsterdam Neuroscience - Neuroinfection & -inflammation, Amsterdam Neuroscience - Brain Imaging, CCA - Imaging and biomarkers, Amsterdam Reproduction & Development (AR&D), Ophthalmology, ACS - Diabetes & metabolism, APH - Health Behaviors & Chronic Diseases, APH - Quality of Care, CCA - Cancer Treatment and quality of life, and AII - Cancer immunology

Subjects
10036 Medical Clinic, Radiology, Nuclear Medicine and imaging, 610 Medicine & health
Abstract

Background MYCN-amplified RB1 wild-type (MYCNARB1+/+) retinoblastoma is a rare but clinically important subtype of retinoblastoma due to its aggressive character and relative resistance to typical therapeutic approaches. Because biopsy is not indicated in retinoblastoma, specific MRI features might be valuable to identify children with this genetic subtype. Purpose To define the MRI phenotype of MYCNARB1+/+ retinoblastoma and evaluate the ability of qualitative MRI features to help identify this specific genetic subtype. Materials and Methods In this retrospective, multicenter, case-control study, MRI scans in children with MYCNARB1+/+ retinoblastoma and age-matched children with RB1-/- subtype retinoblastoma were included (case-control ratio, 1:4; scans acquired from June 2001 to February 2021; scans collected from May 2018 to October 2021). Patients with histopathologically confirmed unilateral retinoblastoma, genetic testing (RB1/MYCN status), and MRI scans were included. Associations between radiologist-scored imaging features and diagnosis were assessed with the Fisher exact test or Fisher-Freeman-Halton test, and Bonferroni-corrected P values were calculated. Results A total of 110 patients from 10 retinoblastoma referral centers were included: 22 children with MYCNARB1+/+ retinoblastoma and 88 control children with RB1-/- retinoblastoma. Children in the MYCNARB1+/+ group had a median age of 7.0 months (IQR, 5.0-9.0 months) (13 boys), while children in the RB1-/- group had a median age of 9.0 months (IQR, 4.6-13.4 months) (46 boys). MYCNARB1+/+ retinoblastomas were typically peripherally located (in 10 of 17 children; specificity, 97%; P < .001) and exhibited plaque or pleomorphic shape (in 20 of 22 children; specificity, 51%; P = .011) with irregular margins (in 16 of 22 children; specificity, 70%; P = .008) and extensive retina folding with vitreous enclosure (specificity, 94%; P < .001). MYCNARB1+/+ retinoblastomas showed peritumoral hemorrhage (in 17 of 21 children; specificity, 88%; P < .001), subretinal hemorrhage with a fluid-fluid level (in eight of 22 children; specificity, 95%; P = .005), and strong anterior chamber enhancement (in 13 of 21 children; specificity, 80%; P = .008). Conclusion MYCNARB1+/+ retinoblastomas show distinct MRI features that could enable early identification of these tumors. This may improve patient selection for tailored treatment in the future. © RSNA, 2023 Supplemental material is available for this article. See also the editorial by Rollins in this issue.

Published
2023
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