Name

Langerhans cell sarcoma

ICD-O-3 Morphology

9756/3: Langerhans cell sarcoma
Effective 2001 and later

Reportable

for cases diagnosed 2001 and later

Primary Site(s)

See Module 7
Most common sites of involvement: skin and underlying tissue with multiorgan involvement, lymph nodes, lung, liver, spleen and bone

Grade

Not Applicable

Module Rule

None

Alternate Names

Dendritic/histiocytic sarcoma, Langerhans cell type
LCS
Malignant histiocytosis X

Definition

Langerhans cell (LC) sarcoma is a high-grade neoplasm with overtly malignant cytological features and the LC phenotype.

Abstractor Notes

Most occurrences are extranodal involving skin and bone.

Presentation is usually multifocal, high-stage disease (III-IV) in 44% of the patients.

Primarily nodal in 22% of cases.

Definitive Diagnostic Methods

Immunophenotyping

Genetics Data

None

Immunophenotyping

CD1a expression
CD68 expression
HLA-DR expression
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

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 (U.S. only)

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

Signs and Symptoms

Easy bruising or bleeding
Painful bone lesion
Pancytopenia
Skin rash
Weight loss

Diagnostic Exams

BRAF testing
CT (CAT) scan
Complete blood count (CBC)
MRI (magnetic resonance imaging)
PET (positron emission tomography) scan
Water deprivation test

Progression and Transformation

None

Epidemiology and Mortality

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

Sources

Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (Eds):
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Histiocytic and dendritic cell neoplasms
Pages: 473

International Classification of Diseases for Oncology, Third Edition, First Revision. Geneva: World Health Organization, 2013.
Section: ICD-O-3.1 (2011) Morphological Codes
Pages: http://codes.iarc.fr/codegroup/2

National Cancer Institute
Section: General Information About Langerhans Cell Histiocytosis (LCH)
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