Langerhans Cell Tumor of the Spine: A Report of Two Clinical Cases
Main Article Content
Abstract
Langerhans cell histiocytosis (LCH) is a rare disorder characterized by the abnormal proliferation of Langerhans-like cells, with potential involvement of multiple organs. The skeletal system is the most commonly affected site, accounting for approximately 60–80% of cases, with spinal involvement reported in 6.5–25% of cases and associated with a high risk of severe neurological complications. Imaging plays a crucial role in the detection and characterization of lesions, with hallmark features including “vertebra plana,” lytic bone lesions with or without associated soft-tissue masses, and characteristic signal alterations on MRI. However, due to overlapping features with infectious and malignant conditions, histopathological biopsy remains the gold standard for definitive diagnosis.
Keywords
Langerhans cell histiocytosis, Bone lesions, Spinal lesions, Diagnostic imaging.
Article Details
References
2. Reisi, N., Raeissi, P., Harati Khalilabad, T., & Moafi, A. (2021). Unusual sites of bone involvement in Langerhans cell histiocytosis: a systematic review of the literature. Orphanet Journal of Rare Diseases, 16(1).
3. Khung S, Budzik JF, Amzallag-Bellenger E, Lambilliote A, Soto Ares G, Cotten A, Boutry N. Skeletal involvement in Langerhans cell histiocytosis. Insights Imaging. 2013 Oct;4(5):569-79.
4. Zhao M, Tang L, Sun S, Cui J, Chen H. Radiologic findings that aid in the reduction of misdiagnoses of Langerhans cell histiocytosis of the bone: a retrospective study. World J Surg Oncol. 2021; 19:146.
5. Lee SW, Kim H, Suh JK, Koh KN, Im HJ, Yoon HM, Seo JJ. Long-term clinical outcome of spinal Langerhans cell histiocytosis in children. Int J Hematol. 2017 Sep;106(3):441-449. doi: 10.1007/s12185-017-2252-y. Epub 2017 May 17. PMID: 28516403.
6. Choi JH, Lee JS, Lim GY, et al. Clinical and radiologic features of pediatric spinal LCH and surgical indications. Childs Nerv Syst. 2017;33(12):2147–2155.
7. Peng XS, Pan T, Chen LY, Huang G, Wang J. Langerhans’ cell histiocytosis of the spine in children with soft tissue extension and chemotherapy. Int Orthop. 2009 Jun;33(3):731-6. doi: 10.1007/s00264-008-0529-8. Epub 2008 Mar 13. PMID: 18338168; PMCID: PMC2903089.
8. Huang WD, Yang XH, Wu ZP, Huang Q, Xiao JR, Yang MS, Zhou ZH, Yan WJ, Song DW, Liu TL, Jia NY. Langerhans cell histiocytosis of spine: a comparative study of clinical, imaging features, and diagnosis in children, adolescents, and adults. Spine J. 2013 Sep;13(9):1108-17. doi: 10.1016/j.spinee.2013.03.013. Epub 2013 Apr 18. PMID: 23602327.
9. Héritier S, Barkaoui A, Miron J, Moshous D, Lambilliotte A, Mazingue F, et al. Incidence and risk factors for clinical neurodegenerative Langerhans cell histiocytosis: a longitudinal cohort study. British Journal of Haematology. 2018;183(4):608–617. doi:10.1111/bjh.15577.
10. Rodriguez-Galindo C, Allen CE. Langerhans cell histiocytosis. Blood. 2020;135(16):1319-1331.