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 Module 7
Most common primary site: skin and underlying tissue with multiorgan involvement, lymph nodes, lung, liver, spleen and bone

Help me code for diagnosis 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
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
CD68
HLA-DR
Langerin S100 protein vimentin
Treatments
Chemotherapy
Hormone
Radiation
Surgery
Transformations to
There are no known transformations
Transformations from
There are no known transformations
Same Primaries
There are no known same primaries
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
Diarrhea
Dyspnea
Easy bruising or bleeding
Edema
Fever
Hepatosplenomegaly
Painful bone lesion
Pancytopenia
Polydipsia
Polyuria
Skin rash
Weight loss
Diagnostic Exams
Bone scan
BRAF testing
CT (CAT) scan
Complete blood count (CBC)
Endoscopy
Liver function test
MRI (magnetic resonance imaging)
PET (positron emission tomography) scan
Ultrasound exam
Urinalysis
Water deprivation test
Recurrence and Metastases
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