Autopsy and Case Reports
https://app.periodikos.com.br/journal/autopsy/article/doi/10.4322/acr.2021.344
Autopsy and Case Reports
Clinical Case Report

Nodal Langerhans cell neoplasm: detailing the diagnostic quandaries

Zachariah Chowdhury; Juhi Varshney; Anil Singh; Satvik Khaddar

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Abstract

Langerhans cells, found in the supra-basal region of the mucous membranes in the epidermis of the skin, in lymph nodes and thymus, function as antigen-presenting cells within the histiocyte system. Tumors derived from Langerhans cells (LC) can be divided according to the degree of cytological atypia and clinical behavior into Langerhans cell histiocytosis (LCH) and Langerhans cell sarcoma (LCS). LCS is rare, and the nodal presentation is even rarer with challenging histological characteristics. LCS has a dismal overcome despite intensive chemotherapy. Herein, we report a case of a 29-year-old male who presented with generalized lymphadenopathy initially considered as a lymphoma. An outright definitive diagnosis could not be attained in the initial histomorphological and immunohistochemical evaluation, fraught with differential diagnoses. The key to decoding the precise neoplasm was a combination of the cytopathologic features, review of the histomorphology, and extensive immunohistochemical assessment in conjunction with the clinical and positron emission tomography (PET) scan findings. The best diagnosis proffered was a Langerhans cell histiocytosis progressing to Langerhans cell sarcoma. This case highlights the grey zone areas in LC neoplasms, the diagnostic conundrums encountered, the indispensable role of meticulous pathological analysis, and the importance of ancillary studies in hammering out the final diagnosis.

Keywords

histiocytosis, Langerhans cell, lymphoma, pleura, sarcoma

References

1 Giuliani M, Marinucci A, Melchiorri L, Zoccali G. Cutaneous Langerhans cell sarcoma: a case report. Int J Surg Surgical Proced. 2016;1(1):101-3. http://dx.doi.org/10.15344/2456-4443/2016/101.

2 Weiss LM, Jaffe R, Facchetti F. Tumors derived from Langerhans cells. In: Swerdlow SH, Campo E, Harris NL, et al. WHO classification of tumors of haematopoietic and lymphoid tissues. Lyon: IARC; 2017. p. 470-3.

3 Bonetti F, Knowles DM 2nd, Chilosi M, et al. A distinctive cutaneous malignant neoplasm expressing the Langerhans cell phenotype: synchronous occurrence with B-chronic lymphocytic leukemia. Cancer. 1985;55(10):2417-25. http://dx.doi.org/10.1002/1097-0142(19850515)55:10<2417::AID-CNCR2820551020>3.0.CO;2-9. PMid:3886125.

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. 2016;56(2):109-18. http://dx.doi.org/10.3960/jslrt.56.109. PMid:27980300.

5 Howard JEF, Dwivedi RC, Masterson L, Jani P. Langerhans cell sarcoma: a systematic review. Cancer Treat Rev. 2015;41(4):320-31. http://dx.doi.org/10.1016/j.ctrv.2015.02.011. PMid:25805533.

6 Zhang Y, Qu Z, Fang F. Langerhans cell sarcoma originating from left knee subcutaneous tissue: a case report and literature review. Oncol Lett. 2016;12(5):3687-94. http://dx.doi.org/10.3892/ol.2016.5194. PMid:27900055.

7 Kang D-W, Son H-J, Baek T-H, et al. Langerhans cell sarcoma arising in a lymph node: a case report and review of the literature. Korean J Pathol. 2011;45(1):101-5. http://dx.doi.org/10.4132/KoreanJPathol.2011.45.1.101.

8 Orii T, Takeda H, Kawata S, Maeda K, Yamakawa M. Differential immunophenotypic analysis of dendritic cell tumours. J Clin Pathol. 2010;63(6):497-503. http://dx.doi.org/10.1136/jcp.2009.067819. PMid:20439325.

9 Yi W, Chen WY, Yang TX, Lan JP, Liang WN. Langerhans cell sarcoma arising from antecedent langerhans cell histiocytosis: a case report. Medicine. 2019;98(10):e14531. http://dx.doi.org/10.1097/MD.0000000000014531. PMid:30855438.

10 Delabie J, De Wolf-Peeters C, De Vos R, et al. True histiocytic neoplasm of Langerhans’ cell type. J Pathol. 1991;163(3):217-23. http://dx.doi.org/10.1002/path.1711630306. PMid:1707450.

11 Lian YL, He HY, Liao SL, Yin LJ, Han ZH, Zheng J. Langerhans cell sarcoma of talus: report of a case. Zhonghua Bing Li Xue Za Zhi. 2006;35(11):697-8. PMid:17374221.

12 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. 2007;85(2):116-20. http://dx.doi.org/10.1532/IJH97.NA0604. PMid:17321988.

13 Yoshimi A, Kumano K, Motokura T, et al. ESHAP therapy effective in a patient with Langerhans cell sarcoma. Int J Hematol. 2008;87(5):532-7. http://dx.doi.org/10.1007/s12185-008-0075-6. PMid:18415657.

14 Uchida K, Kobayashi S, Inukai T, et al. Langerhans cell sarcoma emanating from the upper arm skin: successful treatment by MAID regimen. J Orthop Sci. 2008;13(1):89-93. http://dx.doi.org/10.1007/s00776-007-1184-4. PMid:18274862.

15 Lakshmaiah KC, Smitha CS, Lokanatha D, et al. Langerhans cell sarcoma: review of literature and a rare case report. J Mol Biomark Diagn. 2014;5(4):181. http://dx.doi.org/10.4172/2155-9929.1000181.

16 Li Y, Li B, Tian X, Li Z. Unusual cutaneous Langerhans cell sarcoma without extracutaneous involvement. Diagn Pathol. 2013;8(1):20. http://dx.doi.org/10.1186/1746-1596-8-20. PMid:23388086.

17 Wang C, Chen Y, Gao C, Yin J, Li H. Multifocal Langerhans cell sarcoma involving epidermis: a case report and review. Diagn Pathol. 2012;7(1):99. http://dx.doi.org/10.1186/1746-1596-7-99. PMid:22889043.

18 Ratei R, Hummel M, Anagnostopoulos I, et al. Common clonal origin of an acute B-lymphoblastic leukemia and a Langerhans’ cell sarcoma: evidence for hematopoietic plasticity. Haematologica. 2010;95(9):1461-6. http://dx.doi.org/10.3324/haematol.2009.021212. PMid:20421277.
 


Submitted date:
04/30/2021

Accepted date:
11/21/2021

Publication date:
12/10/2021

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