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 Abstractor Notes

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. There is expression of one or more histiocytic markers without accessory/dendritic cell markers. Neoplastic proliferations associated with Acute monocytic leukemia are excluded.

Tumor mass of macrophage/mature tissue histiocytic cells occuring in any of several sites. Most common sites: lymph nodes (one third), skin (one third), intestinal tract and other extranodal sites.
Abstractor Notes
Histiocytic sarcoma typically presents in extranodal sites, most commonly intestinal tract, skin, and soft tissues. Others present with lymphadenopathy. Rarely, patients have a systemic presentation with multiple sites of involvement, sometimes referred to as "malignant histiocytosis" which was coded separately before 2010 as 9750/3. Patients may present with a solitary mass but systemic symptoms are relatively common, e.g. fever and weight loss. Skin manifestations may range from a benign-appearing rash to solitary lesions to numerous tumors on the trunk and extremities. Patients with intestinal lesions often present with intestinal obstruction. Hepatosplenomegaly and associated pancytopenia may occur. The bones may show lytic lesions. Histiocytic sarcoma is usually an aggressive neoplasm with a poor response to therapy although some exceptions have been reported. Most patients die of progressive disease reflecting the high clinical stage at presentation (stage III-IV) in the majority of patients.
Definitive Diagnostic Methods
Exclusion of B and T cell markers
Histological confirmation
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
No Transformations
Transformations from
No Transformations
Same Primaries
None
Corresponding ICD-9 Codes
200.0 Reticulosarcoma
202.3 Malignant histiocytosis
Corresponding ICD-10 Codes
C96.3 True histiocytic lymphoma
C96.1 Malignant histiocytosis
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
C96.A Histiocytic sarcoma
Signs and Symptoms
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None