Search Database ICD-O-3 Code Lists

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

Coding Manual: Hematopoietic Coding Manual (PDF)

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.

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.

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.

Definitive Diagnostic Methods

Immunophenotyping

Genetics Data

None

Immunophenotyping

CD1a+ (expression/positive)
CD68+ (expression/positive)
HLA-DR+ (expression/positive)
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-9 Codes

202.9 Other and unspecified malignant neoplasm of lymphoid and histiocytic tissue

Corresponding ICD-10 Codes

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
Blood chemistry studies
Bone marrow aspiration and biopsy
Bone scan
BRAF testing
CT (CAT) scan
Endoscopy
Immunophenotyping
Liver function test
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: 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, Second Revision. Geneva: World Health Organization, 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

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