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.
Authors
- Mihai Cucu (ORCID: https://orcid.org/0000-0001-5847-6480)
- Magdalena Budișteanu (ORCID: https://orcid.org/0000-0003-2861-236X)
- Raluca Ioana Teleanu (ORCID: https://orcid.org/0000-0003-3038-6939)
- Florin Burada (ORCID: https://orcid.org/0000-0002-7132-0904)
- Eugenia Roza (ORCID: https://orcid.org/0000-0002-5061-7821)
- I Gavrilă
- Iancu Emil Pleşea (ORCID: https://orcid.org/0000-0003-2686-3878)
- Rebecca-Cristiana Șerban
- Ioana Streață
- Cristian Gheonea (ORCID: https://orcid.org/0009-0001-9234-6097)
- Renata Maria Văruț
- Anca‐Lelia Riza (ORCID: https://orcid.org/0000-0003-2073-8865)
Institutions
- 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)
Publication Details
- Journal
- Diagnostics
- Published
- 2026-11-02
- DOI
- https://doi.org/10.3390/diagnostics16213618
- Primary Topic
- Neurofibromatosis and Schwannoma Cases
- Type
- article
- Field-Weighted Citation Impact
- 0.00