Name
Histiocytic sarcoma
ICD-O-2 Morphology
9723/3: True histiocytic sarcoma
Effective
1992 - 2000
ICD-O-3 Morphology
9755/3: Histiocytic sarcoma
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
Coding Manual:
Hematopoietic Coding Manual (PDF)
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.
This is a very aggressive neoplasm with a usually poor response to therapy.
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
Histiocytic medullary reticulosis [OBS]
Malignant histiocytosis
Monocytic sarcoma
True histiocytic lymphoma
True histiocytic sarcoma
Definition
Histiocytic sarcoma is a malignant proliferation of cells showing morphological and immunophenotypic features of mature tissue histiocytes. Neoplastic proliferations associated with acute monocytic leukemia are excluded.
Definitive Diagnostic Methods
Genetic testing
Histologic confirmation
Immunophenotyping
Genetics Data
Clonal Ig rearrangements
TCR rearrangements are lacking
Immunophenotyping
Absence of markers from non-histiocytic cells
CD4+ (expression/positive)
CD15+ (expression/positive)
CD45+ (expression/positive)
CD45RO+ (expression/positive)
CD68 (KP1 and PGM1)+ (expression/positive)
CD163+ (expression/positive)
HLA-DR+ (expression/positive)
Lysozyme+ (expression/positive)
Treatments
Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
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
202.3 Malignant histiocytosis
Corresponding ICD-10 Codes
C96.3 True histiocytic lymphoma
Corresponding ICD-10-CM Codes (U.S. only)
C96.A Histiocytic sarcoma (effective October 01, 2015)
Signs and Symptoms
Fever
Hepatosplenomegaly
Lymphadenopathy
Pancytopenia
Skin lesions (solitary and innumerable)
Weight loss
Diagnostic Exams
Biopsy
Blood chemistry studies
Bone marrow aspiration and biopsy
Bone scan
BRAF testing
CT (CAT) scan
Endoscopy
Flow cytometry
Immunophenotyping
Liver function test
Neurological exam
PET (positron emission tomography) scan
Ultrasound exam
Urinalysis
Water deprivation test
Progression and Transformation
Most patients die of progressive involvement reflecting the high clinical stage at presentation (stage III-IV) in the majority of patients
Epidemiology and Mortality
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: 468-470
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Histiocytic and dendritic cell neoplasms
Pages: 468-470
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
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 Adult Non-Hodgkin Lymphoma (NHL)
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq
Section: General Information About Adult Non-Hodgkin Lymphoma (NHL)
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq