Langerhans cell sarcoma

Name
Langerhans cell sarcoma
ICD-O-3 Morphology
9756/3
Effective 2001 and later
Reportable
for cases diagnosed 2001 and later
Primary Site(s)
See Abstractor Notes

Help me code for dx year :

Grade
9 - Grade/differentiation unknown, not stated, or not applicable
Module Rule
None
Alternate Names
Dendritic/histiocytic sarcoma, Langerhans cell type
LCS
Malignant histiocytosis X
Definition
Langerhans cell sarcoma is a high-grade neoplasm with overly malignant cytologic features and the Langerhans cell phenotype.
Abstractor Notes
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
None
Genetics Data
None
Immunophenotyping
CD1a
CD68
HLA-DR
langerin S100 protein vimentin
Treatments
Chemotherapy
Hormone
Radiation
Surgery
Transformations to
None
Transformations from
None
Same Primaries
None
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)
Signs and Symptoms
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None