Diagnosis and Treatment of Langerhans Cell Sarcoma: A Rare Case Report

Langerhans cell sarcoma (LCS) is a rare high-grade malignant tumor consisting of Langerhans cells and characterized by aggressive course and high mortality. The incidence rate is 2 cases per 100 million population. As opposed to Langerhans cell histiocytosis (LCH), LCS is marked by pronounced cell atypia and tendency towards rapid metastasis. Clinical manifestations vary depending on the lesion location and involve skin, lymph nodes, bones, lungs, liver, spleen, and bone marrow. Due to the rarity of this disease, there are no standardized treatment protocols. The approaches used in LCH therapy are not effective in the treatment of LCS. The present paper reports a case of LCS with intensified chemotherapy regimens similar to those used in highly aggressive lymphomas including subsequent maintenance therapy which is commonly administered to high-risk LCH patients. This therapeutic strategy resulted in the complete LCS remission which has sustained for over 10 years.

• Timur Teimurazovich Valiev NN Blokhin National Medical Cancer Research Center, 23 Kashirskoye sh., Moscow, Russian Federation, 115522 ; Russian Medical Academy of Postgraduate Education, 2/1 Barrikadnaya ul., Moscow, Russian Federation, 125993 ; IM Sechenov First Moscow State Medical University, 8 korp. 2 Trubetskaya ul., Moscow, Russian Federation, 119991 https://orcid.org/0000-0002-1469-2365 (unauthenticated)
• A.M. Kovrigina National Research Center for Hematology, 4 Novyi Zykovskii pr-d, Moscow, Russian Federation, 125167 https://orcid.org/0000-0002-1082-8659 (unauthenticated)
• G.A. Radjabova Russian Medical Academy of Postgraduate Education, 2/1 Barrikadnaya ul., Moscow, Russian Federation, 125993 https://orcid.org/0009-0008-8297-3629 (unauthenticated)

1. Валиев Т.Т., Гурьева О.Д., Павловская А.И. Редкие варианты гистиоцитарных опухолей: особенности диагностики и выбора тактики лечения (обзор литературы). Онкогематология. 2022;17(1):53–64. doi: 10.17650/1818-8346-2022-17-1-53-64. [Valiev T.T., Gurieva O.D., Pavlovskaya A.I. Rare variants of histiocytic tumors: peculiarities of diagnosis and choice of treatment (literature review). Oncohematology. 2022;17(1):53–64. doi: 10.17650/1818-8346-2022-17-1-53-64. (In Russ)] DOI: https://doi.org/10.17650/1818-8346-2022-17-1-53-64
2. Yang X, Guo S, Xu Q, Tao J. Langerhans cell sarcoma in the skull: A very rare case report. Asian J Surg. 2024:S1015-9584(24)02644-7. doi: 10.1016/j.asjsur.2024.11.041. DOI: https://doi.org/10.1016/j.asjsur.2024.11.041
3. Dezzani A, Punziano C, Berti E, Bonometti A. Langerhans cell sarcoma is a clinically, biologically, and prognostically heterogeneous “malignant” histiocytosis: a systematic review of 88 cases from the literature. Virchows Arch. 2025;487(6):1195–207. doi: 10.1007/s00428-025-04230-2. DOI: https://doi.org/10.1007/s00428-025-04230-2
4. Katsuragawa H, Yamada Y, Ishida Y, et al. A case of Langerhans cell sarcoma on the scalp: Whole-exome sequencing reveals a role of ultraviolet in the pathogenesis. Pathol Int. 2020;70(11):881–7. doi: 10.1111/pin.13007. DOI: https://doi.org/10.1111/pin.13007
5. Karai LJ, Sanik E, Ricotti CA, et al. Langerhans cell sarcoma with lineage infidelity/plasticity: a diagnostic challenge and insight into the pathobiology of the disease. Am J Dermatopathol. 2015;37(11):854–61. doi: 10.1097/DAD.0000000000000318. DOI: https://doi.org/10.1097/DAD.0000000000000318
6. Howard JE, Dwivedi RC, Masterson L, Jani P. Langerhans cell sarcoma: a systematic review. Cancer Treat Rev. 2015;41(4):320–31. doi: 10.1016/j.ctrv.2015.02.011.
7. Zwerdling T, Won E, Shane L, et al. Langerhans cell sarcoma: case report and review of world literature. J Pediatr Hematol Oncol. 2014;36(6):419–25. doi: 10.1097/MPH.0000000000000196. DOI: https://doi.org/10.1097/MPH.0000000000000196
8. Wei Y, Hong H, Huang H. Primary Langerhans cell sarcoma in the urinary bladder: Case report and literature review. Front Oncol. 2023;13:1118222. doi: 10.3389/fonc.2023.1118222. DOI: https://doi.org/10.3389/fonc.2023.1118222
9. Hamaguchi K, Hashimoto A, Fujimi A, et al. Langerhans cell sarcoma developing acute myeloid leukemia after achieving complete response by THP-COP. Rinsho Ketsueki. 2015;56(12):2456–61. doi: 10.11406/rinketsu.56.2456.
10. Nakamine H, Yamakawa M, Yoshino T, et al. Langerhans Cell Histiocytosis and Langerhans Cell Sarcoma: Current Understanding and Differential Diagnosis. J Clin Exp Hematop. 2016;56(2):109–18. doi: 10.3960/jslrt.56.109. DOI: https://doi.org/10.3960/jslrt.56.109
11. Çalli AO, Morgül Y, Alacacioglu I, et al. Langerhans Cell Sarcoma of the Axillary Lymph Node: A Case Report and Review of the Literature. Turk J Haematol. 2013;30(2):198–203. doi: 10.5152/tjh.2011.98. DOI: https://doi.org/10.5152/tjh.2011.98
12. Nakayama M, Takahashi K, Hori M, et al. Langerhans cell sarcoma of the cervical lymph node: a case report and literature review. Auris Nasus Larynx. 2010;37(6):750–3. doi: 10.1016/j.anl.2010.04.007. DOI: https://doi.org/10.1016/j.anl.2010.04.007
13. Howard JE, Dwivedi RC, Masterson L, Jani P. Langerhans cell sarcoma: a systematic review. Cancer Treat Rev. 2015;41(4):320–31. doi: 10.1016/j.ctrv.2015.02.011. DOI: https://doi.org/10.1016/j.ctrv.2015.02.011
14. Барях Е.А., Кременецкая А.М., Кравченко С.К. и др. Новый короткий высокоинтенсивный протокол терапии Беркиттоподобной лимфомы взрослых БПЛ-М-04: промежуточные результаты. Гематология и трансфузиология. 2006;51(6):3–11. [Baryakh E.A., Kremenetskaya A.M., Kravchenko S.K., et al. A new short intensive protocol BL-M-04 for therapy of Burkitt lymphoma in adults: Intermediate results. Gematologiya i transfuziologiya. 2006;51(6):3–11. (In Russ)]
15. Yi W, Chen WY, Yang TX, et al. Langerhans cell sarcoma arising from antecedent langerhans cell histiocytosis: A case report. Medicine (Baltimore). 2019;98(10):e14531. doi: 10.1097/MD.0000000000014531. DOI: https://doi.org/10.1097/MD.0000000000014531
16. Keklik M, Sivgin S, Kontas O, et al. Langerhans cell sarcoma of the nasopharynx: a rare case. Scott Med J. 2013;58(4):e17–20. doi: 10.1177/0036933013508046. DOI: https://doi.org/10.1177/0036933013508046
17. Allen CE, Merad M, McClain KL. Langerhans-Cell Histiocytosis. N Engl J Med. 2018;379(9):856–68. doi: 10.1056/NEJMra1607548. DOI: https://doi.org/10.1056/NEJMra1607548
18. Chakraborty R, Abdel-Wahab O, Durham BH. MAP-Kinase-Driven Hematopoietic Neoplasms: A Decade of Progress in the Molecular Age. Cold Spring Harb Perspect Med. 2021;11(5):a034892. doi: 10.1101/cshperspect.a034892. DOI: https://doi.org/10.1101/cshperspect.a034892
19. Zanwar S, Ravindran A, Abeykoon JP, et al. Prolonged remission with pembrolizumab and radiation therapy in a patient with multisystem Langerhans cell sarcoma. Haematologica. 2022;107(9):2276–9. doi: 10.3324/haematol.2022.280948. DOI: https://doi.org/10.3324/haematol.2022.280948