Case Report
Year 2025,
Volume: 38 Issue: 2, 182 - 185, 30.05.2025
Abstract
References
- Rodriguez-Galindo C, Allen CE. Langerhans cell histiocytosis. Blood 2020;135:1319-31. doi: 10.1182/ blood.201.900.0934.
- Grois N, Pötschger U, Prosch H, et al. Risk factors for diabetes insipidus in langerhans cell histiocytosis. Pediatr Blood Cancer 2006;46:228-33. doi: 10.1002/pbc.20425.
- Ni M, Yang X. Langerhans’ cell histiocytosis of the temporal bone: A case report. Exp Ther Med 2017;13:1051-3. doi:10.3892/etm.2017.4072.
- Zheng H, Xia Z, Cao W, et al. Pediatric Langerhans cell histiocytosis of the temporal bone: clinical and imaging studies of 27 cases. World J Surg Oncol 2018;16:1-7. doi:10.1186/ s12957.018.1366-x.
- Modest MC, Garcia JJ, Arndt CS, Carlson ML. Langerhans cell histiocytosis of the temporal bone: a review of 29 cases at a single center. Laryngoscope 2016;126:1899-904. doi: 10.1002/ lary.25773.
- Döring K, Ivanyi P, Lanfermann H, Warnecke A, Giesemann A. Langerhans cell histiocytosis involving the temporal bone with destruction and subsequent reossification of the bony labyrinth boundaries. Rofo 2024;196:1063-67. doi: 10.1055/a- 2254-5536.
- Allen A, Matrova E, Ozgen B, Redleaf M, Emmadi R, Saran N. Langerhans’ cell histiocytosis of the temporal bone in an adult with central diabetes insipidus. Radiol Case Rep 2019;14:847- 50. doi:10.1016/j.radcr.2019.03.040.
- Allen CE, Ladisch S, McClain KL. How I treat Langerhans cell histiocytosis. Blood 2015;126:26-35. doi: 10.1182/ blood-2014-12-569301.
- Ginat DT, Johnson DN, Cipriani NA. Langerhans cell histiocytosis of the temporal bone. Head Neck Pathol 2016;10:209-12. doi:10.1007/s12105.015.0629-x.
- Berres M-L, Allen CE, Merad M. Pathological consequence of misguided dendritic cell differentiation in histiocytic diseases. Adv Immunol 2013;120:127-61. doi:10.1016/B978-0-12- 417028-5.00005-3.
- Héritier S, Emile J-F, Barkaoui M-A, et al. BRAF mutation correlates with high-risk Langerhans cell histiocytosis and increased resistance to first-line therapy. J Clin Oncol 2016;34:3023-30. doi:10.1200/JCO.2015.65.9508.
- Haroche J, Cohen-Aubart F, Rollins BJ, et al. Histiocytoses: emerging neoplasia behind inflammation. Lancet Oncol 2017;18:e113-e25. doi: 10.1016/S1470-2045(17)30031-1.
- Mosnier I, Rondini-Gilli E, Crosara PTB, et al. Langerhans’ cell histiocytosis of the labyrinth in adults. Otol Neurotol 2004;25:27-32. doi: 10.1097/00129.492.200401000-00006.
- Zimny A, Neska-Matuszewska M, Bladowska J, Sąsiadek MJ. Intracranial lesions with low signal intensity on T2-weighted MR images–review of pathologies. Pol J Radiol 2015;80:40. doi: 10.12659/PJR.892146.
- Li Y, Umbach DM, Li L. Putative genomic characteristics of BRAF V600K versus V600E cutaneous melanoma. Melanoma Res 2017;27:527-35. doi: 10.1097/CMR.000.000.0000000388.
- Morimoto A, Shimazaki C, Takahashi S, et al. Therapeutic outcome of multifocal Langerhans cell histiocytosis in adults treated with the Special C regimen formulated by the Japan LCH Study Group. Int J Hematol 2013;97:103-8. doi: 10.1007/ s12185.012.1245-0.
- Heisig A, Sörensen J, Zimmermann S-Y, et al. Vemurafenib in Langerhans cell histiocytosis: report of a pediatric patient and review of the literature. Oncotarget 2018;9:22236. doi: 10.18632/oncotarget.25277.
- Sachdev R, Shyama J. Co-existent Langerhans cell histiocytosis and Rosai–Dorfman disease: a diagnostic rarity. Cytopathology 2008;19. doi: 10.1111/j.1365 2303.2006.00428.x.
Successful Treatment of Temporal Bone Langerhans Cell Histiocytosis with Oral Steroids: A Case Report
Abstract
Langerhans cell histiocytosis (LCH) is a rare disease; treatment recommendations are based on organ involvement and the extent
of the disease. We present a patient with a solitary lesion of LCH with BRAF-V600K mutation which was successfully managed
with systemic steroids and followed up for a long time. A 39-year-old man presented with persistent right otitis media with effusion
despite treatment. LCH was diagnosed by punch biopsy, and temporal computed tomography (CT) showed an invasive, enhancing
soft tissue mass extending from the malleolus to the epitympanic recess. Due to the patient’s young age and the localization of the
lesion, radiation and surgery were not considered. The patient was treated with systemic methylprednisolone. The patient’s symptoms
resolved after treatment and the tumor regressed completely. The case had a good prognosis in the 5-year period.
This is the first report of a LCH case with the BRAF-V600K mutation, highlighting a unique aspect of the disease. The successful
therapy of the temporal bone LCH with systemic steroids alone, underscores the potential effect of this treatment for the management
of unifocal lesions in critical locations. Further studies and case reports are needed to expand our understanding of LCH and explore
optimal therapeutic approaches for different mutation subtypes.
References
- Rodriguez-Galindo C, Allen CE. Langerhans cell histiocytosis. Blood 2020;135:1319-31. doi: 10.1182/ blood.201.900.0934.
- Grois N, Pötschger U, Prosch H, et al. Risk factors for diabetes insipidus in langerhans cell histiocytosis. Pediatr Blood Cancer 2006;46:228-33. doi: 10.1002/pbc.20425.
- Ni M, Yang X. Langerhans’ cell histiocytosis of the temporal bone: A case report. Exp Ther Med 2017;13:1051-3. doi:10.3892/etm.2017.4072.
- Zheng H, Xia Z, Cao W, et al. Pediatric Langerhans cell histiocytosis of the temporal bone: clinical and imaging studies of 27 cases. World J Surg Oncol 2018;16:1-7. doi:10.1186/ s12957.018.1366-x.
- Modest MC, Garcia JJ, Arndt CS, Carlson ML. Langerhans cell histiocytosis of the temporal bone: a review of 29 cases at a single center. Laryngoscope 2016;126:1899-904. doi: 10.1002/ lary.25773.
- Döring K, Ivanyi P, Lanfermann H, Warnecke A, Giesemann A. Langerhans cell histiocytosis involving the temporal bone with destruction and subsequent reossification of the bony labyrinth boundaries. Rofo 2024;196:1063-67. doi: 10.1055/a- 2254-5536.
- Allen A, Matrova E, Ozgen B, Redleaf M, Emmadi R, Saran N. Langerhans’ cell histiocytosis of the temporal bone in an adult with central diabetes insipidus. Radiol Case Rep 2019;14:847- 50. doi:10.1016/j.radcr.2019.03.040.
- Allen CE, Ladisch S, McClain KL. How I treat Langerhans cell histiocytosis. Blood 2015;126:26-35. doi: 10.1182/ blood-2014-12-569301.
- Ginat DT, Johnson DN, Cipriani NA. Langerhans cell histiocytosis of the temporal bone. Head Neck Pathol 2016;10:209-12. doi:10.1007/s12105.015.0629-x.
- Berres M-L, Allen CE, Merad M. Pathological consequence of misguided dendritic cell differentiation in histiocytic diseases. Adv Immunol 2013;120:127-61. doi:10.1016/B978-0-12- 417028-5.00005-3.
- Héritier S, Emile J-F, Barkaoui M-A, et al. BRAF mutation correlates with high-risk Langerhans cell histiocytosis and increased resistance to first-line therapy. J Clin Oncol 2016;34:3023-30. doi:10.1200/JCO.2015.65.9508.
- Haroche J, Cohen-Aubart F, Rollins BJ, et al. Histiocytoses: emerging neoplasia behind inflammation. Lancet Oncol 2017;18:e113-e25. doi: 10.1016/S1470-2045(17)30031-1.
- Mosnier I, Rondini-Gilli E, Crosara PTB, et al. Langerhans’ cell histiocytosis of the labyrinth in adults. Otol Neurotol 2004;25:27-32. doi: 10.1097/00129.492.200401000-00006.
- Zimny A, Neska-Matuszewska M, Bladowska J, Sąsiadek MJ. Intracranial lesions with low signal intensity on T2-weighted MR images–review of pathologies. Pol J Radiol 2015;80:40. doi: 10.12659/PJR.892146.
- Li Y, Umbach DM, Li L. Putative genomic characteristics of BRAF V600K versus V600E cutaneous melanoma. Melanoma Res 2017;27:527-35. doi: 10.1097/CMR.000.000.0000000388.
- Morimoto A, Shimazaki C, Takahashi S, et al. Therapeutic outcome of multifocal Langerhans cell histiocytosis in adults treated with the Special C regimen formulated by the Japan LCH Study Group. Int J Hematol 2013;97:103-8. doi: 10.1007/ s12185.012.1245-0.
- Heisig A, Sörensen J, Zimmermann S-Y, et al. Vemurafenib in Langerhans cell histiocytosis: report of a pediatric patient and review of the literature. Oncotarget 2018;9:22236. doi: 10.18632/oncotarget.25277.
- Sachdev R, Shyama J. Co-existent Langerhans cell histiocytosis and Rosai–Dorfman disease: a diagnostic rarity. Cytopathology 2008;19. doi: 10.1111/j.1365 2303.2006.00428.x.
There are 18 citations in total.
Details
| Primary Language | English |
|---|---|
| Subjects | Surgery (Other) |
| Journal Section | Case Reports |
| Authors | |
| Publication Date | May 30, 2025 |
| Submission Date | January 8, 2025 |
| Acceptance Date | February 27, 2025 |
| Published in Issue | Year 2025 Volume: 38 Issue: 2 |
