Malignant histiocytoses C96.8

Last updated on: 21.10.2022

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Definition
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Basically, the spectrum of malignant histiocytoses (MH) can be divided into:

  • primary malignant histiocytoses (manifest primarily in the skin)
  • and
  • secondary malignant histiocytoses (manifest primarily extracutaneously and secondarily affect the integument).

can be subdivided.

Classification
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Histology
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Individual reports have documented identical immunoglobulin gene rearrangements suggesting a common clonal origin for both histiocytic/dendritic cell sarcoma sarcoma and B-cell neoplasm. Langerhans cell sarcoma is characterized by pleomorphic cytology, a high mitotic rate, and characteristic immunohistochemical staining for langerin, S100, and CD1a.

Diagnosis
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Phenotypic histological analysis (cells express markers of macrophages and/or Langerhans cells). Malignancies with B-cell differentiation are still to be excluded, as is "sarcoma of interdigitating dendritic cells".

Note(s)
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With increasingly refined histological, immunological and molecular biological methods, it is now possible to assign many malignancies previously classified as malignant histiocytoses to T- or B-cell lymphomas. Especially anaplastic large cell CD30-positive T-cell lymphoma (historical name: atypical regressive histiocytosis) falls into this category. Severe cases of hemophagocytic lymphohistiocytosis (HLH) were also previously interpreted as malignant histiocytoses.

Literature
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  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. Tani M et al (1992) Malignant Langerhans cell tumour. Br J Dermatol 126:398-403.
  5. Mohanty P et al (2022) Unusual presentation of Langerhans cell sarcoma as bilateral submandibular gland swelling - A rare case report. Indian J Cancer 59:123-127.
  6. Nakamine H et al (2016) Langerhans cell histiocytosis and Langerhans cell sarcoma: Current Understanding and Differential Diagnosis. J Clin Exp Hematop 56:109-118.
  7. 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.

Outgoing links (1)

Langerhans cell-sarcoma;

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

Last updated on: 21.10.2022