Search Database ICD-O-3 Code Lists

Name

Langerhans cell sarcoma (LCS)

ICD-O-3 Morphology

9756/3: Langerhans cell sarcoma
Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

See Module 7
Most common sites of involvement: skin and underlying tissue with multiorgan involvement, lymph nodes, lung, and bone.
Common metastatic sites are liver. Spleen involvement is common, but it due to infiltration and not the primary site.

See abstractor notes

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

Langerhans cell sarcoma (LCS) is part of the Langerhans cell and other dendritic cell neoplasms lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B11)

Most cases manifest in extranodal sites, such as skin, lung, bone, and soft tissue. Lung, liver, spleen, lymph nodes, and bone are affected in multiorgan disease, with widespread disease occurring in 45% of cases.

Immunophenotyping is required for diagnosis.

Diagnostic Confirmation

This histology can be determined by positive histology (including peripheral blood) with or without genetics and/or immunophenotyping. Review the Definitive Diagnostic Methods, Immunophenotyping and Genetics Data sections below, and the instructions in the Hematopoietic Manual for further guidance on assigning Diagnostic confirmation.

Module Rule

None

Alternate Names

Dendritic/histiocytic sarcoma, Langerhans cell type
Primary Langerhans cells sarcoma
Malignant histiocytosis of Langerhans phenotype
Secondary Langerhans cell sarcoma (following or associated with another hematological neoplasm)

Definition

Langerhans cell sarcoma (LCS) is an aggressive malignant neoplasm with a Langerhans cell immunophenotype and high-grade cytological features. (WHO 5th edition)

Definitive Diagnostic Methods

Histologic confirmation
Immunohistochemistry
Immunophenotyping

Genetics Data

MAPK pathway gene mutations (KRAS, BRAF p.V600E)

Immunophenotyping

CD1a+ (expression/positive)
CD68+ (expression/positive)
CD207 (langerin)+ (expression/positive)
Cyclin D1
HLA-DR
Langerin S100 protein vimentin

Treatments

Chemotherapy
Hormone therapy
Radiation therapy
Surgery

Transformations to

There are no known transformations

Transformations from

There are no known transformations

Same Primaries

Corresponding ICD-10 Codes (Cause of Death codes only)

C96.7 Other specified malignant neoplasms of lymphoid, hematopoietic, and related tissue
C96.9 Malignant neoplasms of lymphoid, hematopoietic, and related tissue, unspecified

Corresponding ICD-10-CM Codes (U.S. only)

C96.4 Sarcoma of dendritic cells (accessory cells) (effective October 01, 2015)

Signs and Symptoms

Diarrhea
Dyspnea
Easy bruising or bleeding
Edema
Fever
Hepatosplenomegaly
Painful bone lesion
Pancytopenia
Polydipsia
Polyuria
Skin rash
Weight loss

Diagnostic Exams

Biopsy
Bone marrow aspiration and biopsy
Bone scan
BRAF testing
CT (CAT) scan
Endoscopy
Immunophenotyping
Liver function test
Molecular analysis
PET (positron emission tomography) scan
Ultrasound exam
Urinalysis
Water deprivation test

Progression and Transformation

None

Epidemiology and Mortality

Age: 39 years median age (10-72 years age range)
Incidence: 0.02 per 1,000,000
Sex: female predominance
Survival: poor prognosis, >50% die of progressive disease

Sources

WHO Classification of Tumours Editorial Board. Haematolymphoid tumours. Lyon (France): International Agency for Research on Cancer; 2024. (WHO classification of tumours series, 5th ed.; vol. 11). https://publications.iarc.who.int/637.
Section: Histiocytic/dendritic cell neoplasms
Pages: Part A: 234-236

International Classification of Diseases for Oncology, 3rd edition (including revisions). Geneva: World Health Organization, 2001, 2011, 2020.
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577

PDQ® Pediatric Treatment Editorial Board. PDQ Langerhans Cell Histiocytosis Treatment. Bethesda, MD: National Cancer Institute. Updated <01/06/2025>. Available at: https://www.cancer.gov/types/langerhans/hp/langerhans-treatment-pdq. Accessed <02/28/25>. [PMID: 26389240]
Section: Langerhans Cell Histiocytosis Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/langerhans/hp/langerhans-treatment-pdq#section/_178%20or%20http://www.cancer.gov/types/langerhans/patient/langerhans-treatment-pdq
Glossary