Histiocytic sarcoma

Name
Histiocytic sarcoma
ICD-O-2 Morphology
9723/3
Effective 1992 - 2000
ICD-O-3 Morphology
9755/3
Effective 2001 and later
Reportable
for cases diagnosed 1992 and later
Primary Site(s)
See Module 7
Most common sites of involvement: lymph nodes, skin, intestinal tract, soft tissue

Help me code for diagnosis year :

Grade
9 - Grade/differentiation unknown, not stated, or not applicable
Module Rule
None
Alternate Names
Histiocytic medullary reticulosis [OBS]
Malignant histiocytosis [see code 9751/3]
Monocytic sarcoma
True histiocytic lymphoma
True histiocytic sarcoma
Definition
A malignant proliferation of cells showing
*Morphologic and immunophenotypic features similar to those of mature tissue histiocytes.
*Expression of one or more histiocytic markers without accessory/dendritic cell markers.
*Neoplastic proliferations associated with Acute monocytic leukemia are excluded.
*Mass of macrophage/mature tissue histiocytic cells occurring in any of several sites.
Abstractor Notes
Rarely, systemic presentation with multiple sites of involvement may be seen, and is sometimes referred to as "malignant histiocytosis".

This is a very aggressive neoplasm with a usually poor response to therapy.
Definitive Diagnostic Methods
Genetic testing
Histologic confirmation
Immunophenotyping
Genetics Data
Clonal IgH negative
TCR rearrangements are lacking
Immunophenotyping
Absence of markers from non-histiocytic cells
CD45+
CD45RO+
CD68+
CD163+
HLA-DR+
Lysozyme
Treatments
Chemotherapy
Hormone
Radiation
Stem cell transplant
Surgery
Transformations to
There are no known transformations
Transformations from
There are no known transformations
Corresponding ICD-9 Codes
200.0 Reticulosarcoma
202.3 Malignant histiocytosis
Corresponding ICD-10 Codes
C96.3 True histiocytic lymphoma
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
C96.A Histiocytic sarcoma
Signs and Symptoms
Pancytopenia
Skin lesions (solitary and innumerable)
Weight loss
Diagnostic Exams
BRAF testing
Complete blood count (CBC)
CT (CAT) scan
MRI (magnetic resonance imaging)
PET (positron emission tomography) scan
Water deprivation test
Progression and Transformation
Most patients die of progressive disease reflecting the high clinical stage at presentation (stage III-IV) in the majority of patients
Epidemiology and Mortality
Age: 52 years median age (infancy to elderly age range)
Incidence: rare, limited number of reported cases
Sex: male predominance
Survival: generally poor, 60-80% of patients die from progressive disease