Langerhans cell-sarcoma C96.4

Langerhans cell sarcoma (LCS) is an extremely rare malignant neoplasm arising from Langerhans cells (LCs) (see also under histiocytoses).

Fewer than 70 cases have been described in the international literature (Guo Y et al. 2022).

The etiology is unknown. Prolonged immunosuppression, viral infections (e.g., Epstein-Barr virus), and previous hematologic disorders have been discussed (Guo Y et al. 2022).

The most common sites where tumor masses spread are: skin, lymph nodes and bones in locoregional cases and lymph nodes, lungs, liver, spleen and bones in disseminated disease.

Due to its rarity, standard treatment protocols for Langerhans cell sarcoma are not well established. Isolated success has been achieved with BRAF V600E inhibitors (Subbiah V et al. 2013).

In contrast to (benign) Langerhans cell histiocytosis (LCH), Langerhans cell sarcoma has an aggressive clinical course (Furmanczyk PS et al 2012).

1. Furmanczyk PS et al (2012) Langerhans cell sarcoma in a patient with hairy cell leukemia: common clonal origin indicated by identical immunoglobulin gene rearrangements. J Cutan Pathol 39:644-650.
2. Guo Y et al. (2022) Epstein-Barr virus-positive Langerhans cell sarcoma: Is There a Link? A Case Report. Front Oncol 11:769310
3. Misery L (2003) Malignant Langerhans cell tumor: a case with a favorable outcome associated with the absence of blood dendritic cell proliferation. J Am Acad Dermatol 49: 527-529
4. Nakamine H et al (2016) Langerhans cell histiocytosis and Langerhans cell sarcoma: current understanding and differential diagnosis. J Clin Exp Hematop 56:109-118.
5. Subbiah V et al (2013) Killing two birds with one stone: BRAF V600E inhibitor therapy for hairy cell leukemia and Langerhans/dendritic cell sarcoma. Ann Hematol 92:1149.