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9 - Grade/differentiation unknown, not stated, or not applicable
Dendritic/histiocytic sarcoma, Langerhans cell type
Malignant histiocytosis X
Langerhans cell sarcoma is a high-grade neoplasm with overly malignant cytologic features and the Langerhans cell phenotype.
Langerhans cell sarcoma (LCS) most commonly involves the skin and underlying tissue with multiorgan involvement that includes lymph nodes, lung, liver, spleen, and bone. LCS is rare and almost all reported cases are in adults. The median age is 39 years (range 10-72 years). A female predominance is described. Most occurrences are extranodal involving skin and bone. The presentation is multifocal, high-stage disease (III-IV) in 44%. Only 22% are primarily nodal. Hepatosplenomegaly is noted in 22% and pancytopenia in 11%. LCS is aggressive, with >50% mortality from progressive disease.
Definitive Diagnostic Methods
langerin S100 protein vimentin
Corresponding ICD-9 Codes
202.9 Other and unspecified malignant neoplasms
Corresponding ICD-10 Codes
C96.7 Other specified malignant neoplasms of lymphoid, hematopoietic
Corresponding ICD-10-CM Codes
(effective October 1, 2015 U.S. only)
C96.4 Sarcoma of dendritic cells (accessory cells)