Complexity of diagnosing and treating langerhans cell sarcoma: A case report

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Published Oct 29, 2023
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Vol. 101 No. 7 (2023): Tunis med jul 2023

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Maha Charfi
Department of Hematology, Hedi Chaker Hospital of Sfax, Tunisia
https://orcid.org/0000-0002-4434-8119
Maissa Lajnaf
Department of Hematology, Hedi Chaker Hospital, Sfax, Tunisia
Faten Kallel
Department of Hematology, Hedi Chaker Hospital, Sfax, Tunisia
Ines Saguem
Department of Anatomopathology, Habib Bourguiba Hospital, Sfax, Tunisia
Olfa Kassar
Department of Hematology, Hedi Chaker Hospital, Sfax, Tunisia
Moez Elloumi
Department of Hematology, Hedi Chaker Hospital, Sfax, Tunisia

Abstract

Introduction: Langerhans cell sarcoma (LCS) is a very rare malignant tumor of Langerhans cells that may metastasize to many organs. The diagnosis of this tumor is difficult and its prognosis is poor.


Aim: To report the difficulty to diagnose LCS, and discuss therapeutic management of this rare entity.


Case presentation: We report a case of LCS in a 52-year-old man who presented with an axillar lymphadenopathy.  The diagnosis of nodular sclerosis type Hodgkin’s disease was established after histologic examination. The patient was treated with chemotherapy (ABVD regimen: Doxorubicin, Bleomycin, Vinblastine, Dacarbazine) and radiotherapy with a partial response. However, disease recurrence was observed and histological analysis confirmed the diagnosis of Langerhans cell sarcoma. A revision of the initial histological examination concluded to the diagnosis of sarcoma from the beginning. We chose the ESHAP (Etoposide, Methylprednisolone, Aracytine, Cisplatin) regimen and clinical improvement of LCS was obtained after 2 cycles but the patient had a fatal outcome and died by disease progression.


Conclusion: Because of its rarity, diagnosis is difficult and an optimal treatment strategy for this disease has not yet been identified. Polychemotherapy can be an effective modality for the treatment of LCS.

Keywords:

Hodgkin lymphoma, Langerhans cell sarcoma, Diagnosis, Chemotherapy

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References

  1. Uchida K, Kobayashi S, Inukai T, Noriki S, Imamura Y, Nakajima H, et al. Langerhans cell sarcoma emanating from the upper arm skin: successful treatment by MAID regimen. J Orthop Sci Off J Jpn Orthop Assoc. janv 2008;13(1):89‑93. https://doi: 10.1007/s00776-007-1184-4
  2. Howard JEF, Dwivedi RC, Masterson L, Jani P. Langerhans cell sarcoma: a systematic review. Cancer Treat Rev. avr 2015;41(4):320‑31. doi: 10.1016/j.ctrv.2015.02.011.
  3. Yoshimi A, Kumano K, Motokura T, Takazawa Y, Oota S, Chiba S, et al. ESHAP therapy effective in a patient with Langerhans cell sarcoma. Int J Hematol. juin 2008;87(5):532‑7. doi: 10.1007/s12185-008-0075-6.
  4. Nakamine H, Yamakawa M, Yoshino T, Fukumoto T, Enomoto Y, Matsumura I. Langerhans Cell Histiocytosis and Langerhans Cell Sarcoma: Current Understanding and Differential Diagnosis. J Clin Exp Hematop JCEH. 2016;56(2):109‑18. doi: 10.3960/jslrt.56.109.
  5. Aissa S, Knaz A, Aissa A, Abdessayed N, Hayouni A. Adult Langerhans’ cell histiocytosis with multisystem bone and lung involvement: About a case. Tunis Med. juill 2022;100(7):561‑3.
  6. Bohn OL, Ruiz-Argüelles G, Navarro L, Saldivar J, Sanchez-Sosa S. Cutaneous Langerhans cell sarcoma: a case report and review of the literature. Int J Hematol. févr 2007;85(2):116‑20. https://doi: 10.1532/IJH97.NA0604.
  7. Nakayama M, Takahashi K, Hori M, Okumura T, Saito M, Yamakawa M, et al. Langerhans cell sarcoma of the cervical lymph node: A case report and literature review. Auris Nasus Larynx. déc 2010;37(6):750‑3. . doi: 10.1016/j.anl.2010.04.007.
  8. Wang Y ni, Zhou X ge, Wang Z. Langerhans Cell Sarcoma in the Cervical Lymph Node: A Case Report and Literature Review. Acta Haematol. 2013;129(2):114‑20. https://doi: 10.1159/000342103
  9. Kaleem TA, Schild MH, Miller D, Jha A, Cortese C, Attia S, et al. Langerhan’s Cell Sarcoma: Two Case Reports. Rare Tumors. 31 mars 2016;8(1):17‑9. doi: 10.4081/rt.2016.6140.
  10. Kwong YL. Cutaneous Langerhans cell sarcoma relapsing systemically: Complete remission with the EPIG regimen. Ann Hematol. avr 2015;94(4):697‑9. https://doi:10.1007/s00277-014-2203-0