Neurofibromatosis Type 1: Clinical and Molecular Characterization in a Romanian Cohort of 80 Patients

Background/Objectives: Neurofibromatosis type 1 (NF1) is an autosomal dominant tumor-predisposition disorder with age-dependent manifestations and marked clinical variability. This study characterized clinical observations and documented molecular findings in a Romanian regional referral cohort. Methods: We retrospectively analyzed 80 patients referred between 2017 and 2025 because of a family history of NF1 or suggestive clinical signs. Available next-generation sequencing (NGS) and multiplex ligation-dependent probe amplification (MLPA) results were reviewed, and variants were classified according to ACMG/AMP criteria. Results: Pathogenic or likely pathogenic NF1 variants were reported in 15/80 patients (18.75%): 13/66 children (19.7%) and 2/14 adults (14.3%). Five sequence variants and ten copy-number deletions were identified. Clinical observations included pigmentary, neurodevelopmental, skeletal, dysmorphic, and tumor-related findings. Testing availability and clinical documentation were heterogeneous. Conclusions: The detected molecular spectrum included both sequence and dosage variants. Uneven assay availability limits estimation of the incremental yield of combined testing. Complete diagnostic documentation and age-appropriate follow-up are important for interpreting molecular findings and supporting family counseling.

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Publication Details

Journal
Diagnostics
Published
2026-11-02
DOI
https://doi.org/10.3390/diagnostics16213618
Primary Topic
Neurofibromatosis and Schwannoma Cases
Type
article
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article

Neurofibromatosis Type 1: Clinical and Molecular Characterization in a Romanian Cohort of 80 Patients

Mihai Cucu, Magdalena Budișteanu, Raluca Ioana Teleanu, Florin Burada et al.
Diagnostics
Neurofibromatosis and Schwannoma Cases
article

Neurofibromatosis Type 1: Clinical and Molecular Characterization in a Romanian Cohort of 80 Patients

Mihai Cucu, Magdalena Budișteanu, Raluca Ioana Teleanu, Florin Burada, Eugenia Roza, I Gavrilă, Iancu Emil Pleşea, Rebecca-Cristiana Șerban, Ioana Streață, Cristian Gheonea, Renata Maria Văruț, Anca‐Lelia Riza
article en

Abstract

Background/Objectives: Neurofibromatosis type 1 (NF1) is an autosomal dominant tumor-predisposition disorder with age-dependent manifestations and marked clinical variability. This study characterized clinical observations and documented molecular findings in a Romanian regional referral cohort. Methods: We retrospectively analyzed 80 patients referred between 2017 and 2025 because of a family history of NF1 or suggestive clinical signs. Available next-generation sequencing (NGS) and multiplex ligation-dependent probe amplification (MLPA) results were reviewed, and variants were classified according to ACMG/AMP criteria. Results: Pathogenic or likely pathogenic NF1 variants were reported in 15/80 patients (18.75%): 13/66 children (19.7%) and 2/14 adults (14.3%). Five sequence variants and ten copy-number deletions were identified. Clinical observations included pigmentary, neurodevelopmental, skeletal, dysmorphic, and tumor-related findings. Testing availability and clinical documentation were heterogeneous. Conclusions: The detected molecular spectrum included both sequence and dosage variants. Uneven assay availability limits estimation of the incremental yield of combined testing. Complete diagnostic documentation and age-appropriate follow-up are important for interpreting molecular findings and supporting family counseling.

DiagnosticsVol. 16(21)
Carol Davila University of Medicine and Pharmacy (RO), University of Medicine and Pharmacy of Craiova (RO), Spitalul Clinic de Psihiatrie Alexandru Obregia (RO), University of Craiova (RO)
Openalex Percentile: Top 16%
Neurofibromatosis and Schwannoma Cases
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