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Oral Juvenile Xanthogranuloma: A Clinicopathological, Immunohistochemical and BRAF V600E Study of Five New Cases, with Literature Review.
| Content Provider | Europe PMC |
|---|---|
| Author | Mota, Carolina Peres Silva Cunha, John Lennon Magalhães, Maria Carolina Silva Versieux Israel, Mônica Simões Freire, Nathália de Almeida dos Santos, Emilia Rosaria Carvalho Canedo, Nathalie Henriques Silva Agostini, Michelle Abrahão, Aline Corrêa de Andrade, Bruno Augusto Benevenuto Romañach, Mário José de Sousa, Sílvia Ferreira |
| Abstract | Juvenile xanthogranuloma (JXG) is the most common form of non-Langerhans cell histiocytosis and oral mucosal involvement is exceedingly rare. Histiocytic disorders harbor activating mutations in MAPK pathway, including the report of BRAF V600E in JXG of extracutaneous site. However, no information is available for oral JXG. Herein, the clinicopathological and immunohistochemical features of five new oral JXG were evaluated in conjunction with literature review. Also, we assessed the BRAF V600E in oral samples. Five oral JXG were retrieved from pathology archives. Morphological and immunohistochemical analyses were performed. The BRAF V600E status was determined with TaqMan allele-specific qPCR. The series comprised of three female and two male patients, most of them adults, with a median age of 39 years (range 13–68 years). Clinically, the lesions appeared as asymptomatic solitary nodules, measuring until 2.5 cm, with more incident to the buccal mucosa. Morphologically, most of the cases presented classical histological features of JXG, with histiocytic cells consistent with the non-Langerhans cell immunophenotype. BRAF V600E was not detected in the cases tested. This is the first and largest published series of oral JXG affecting adults and a Brazilian population. The molecular pathogenesis of oral JXG remains unknown. Clinicians and pathologists must recognize JXG to avoid misdiagnoses with oral benign or malignant lesions. |
| Related Links | https://europepmc.org/backend/ptpmcrender.fcgi?accid=PMC9187812&blobtype=pdf |
| ISSN | 1936055X |
| Volume Number | 16 |
| DOI | 10.1007/s12105-021-01373-x |
| PubMed Central reference number | PMC9187812 |
| Issue Number | 2 |
| PubMed reference number | 34414559 |
| Journal | Head and Neck Pathology [Head Neck Pathol] |
| e-ISSN | 19360568 |
| Language | English |
| Publisher | Springer US |
| Publisher Date | 2021-08-19 |
| Publisher Place | New York |
| Access Restriction | Open |
| Rights License | © The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature 2021 |
| Subject Keyword | Juvenile xanthogranuloma non-Langerhans cell histiocytosis Histiocytic disorders Oral cavity BRAF V600E Mutation |
| Content Type | Text |
| Resource Type | Article |
| Subject | Otorhinolaryngology Pathology and Forensic Medicine Oncology |
