Search Database ICD-O-3 Code Lists

Name

Myeloid sarcoma

ICD-O-3 Morphology

9930/3: Myeloid sarcoma
Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

See Module 7
Primary site cannot be bone marrow (C421). Most common sites of involvement: skin, lymph nodes, gastrointestinal tract, bone, soft tissue and testis.

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

Myeloid Sarcoma is part of the Acute myeloid leukemia (AML) lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B6)

Almost any site in the body can be involved. The most frequently affected sites are the skin, soft tissue, lymph nodes, gastrointestinal tract, bones, and head and neck region; less frequently involved sites include the reproductive organs, brain, and lungs.

If myeloid sarcoma (9930/3) and an acute myeloid leukemia occur during the same clinical workup, this is one primary, the leukemia.

If the myeloid sarcoma occurs after the diagnosis of the leukemia, that is a manifestation of the leukemia and is the same primary.

See Multiple Primary Rule M3

If an AML and myeloid sarcoma (9930/3) occur during the same clinical workup, this is one primary, the leukemia.

If the myeloid sarcoma occurs after the diagnosis of the leukemia, that is a manifestation of the leukemia and is the same primary.

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.

Module Rule

None

Alternate Names

Chloroma

Definition

Myeloid sarcoma is a tumor mass involving any anatomical site other than bone marrow (i.e. extramedullary) that effaces tissue architecture and is composed of myeloid blasts, with or without maturation. (WHO 5th edition)

Definitive Diagnostic Methods

Genetic testing
Histologic confirmation
Immunophenotyping

Genetics Data

DNA methylation (DNMT3A, IDH1/2, TET2)
Other gene function (NPM1)
RAS pathway (BRAF, KRAS, NRAS, PTPN11)
Signaling gene mutation (FLT3-TKD, FLT3ITD, KIT)
Tumor suppression (TP53, WTI)

Immunophenotyping

CD13+ (expression/positive)
CD33+ (expression/positive)
CD43+ (expression/positive)
CD45+ (expression/positive)
CD68+ (expression/positive)

Treatments

Chemotherapy
Radiation therapy

Transformations to

There are no known transformations

Transformations from

There are no known transformations

Same Primaries

Corresponding ICD-10 Codes (Cause of Death codes only)

C92.3 Myeloid sarcoma

Corresponding ICD-10-CM Codes (U.S. only)

C92.3 Myeloid sarcoma (effective October 01, 2015 - September 30, 2024)
C92.30 Myeloid sarcoma not having achieved remission (effective October 01, 2024)
C92.31 Myeloid sarcoma, in remission (effective October 01, 2024)
C92.32 Myeloid sarcoma, in relapse (effective October 01, 2024)

Signs and Symptoms

Easy bruising or bleeding
Fatigue
Fever
Petechiae
Shortness of breath
Weakness
Weight loss or loss of appetite

Diagnostic Exams

Bone marrow aspiration and biopsy
CT (CAT) scan
Cytogenetic analysis
Immunophenotyping
Lumbar puncture
Molecular analysis
Peripheral blood smear
Physical exam and history

Progression and Transformation

None

Epidemiology and Mortality

Age: 56 years median (1 month to 89 years range)
Sex: male predominance
Survival: higher probability or prolonged survival for patients who have allogeneic or autologous BM transplantation

Sources

WHO Classification of Tumours Editorial Board. Haematolymphoid tumours. Lyon (France): International Agency for Research on Cancer; 2024. (WHO classification of tumours series, 5th ed.; vol. 11). https://publications.iarc.who.int/637.
Section: Acute myeloid leukemia
Pages: Part A: 165-168

International Classification of Diseases for Oncology, 3rd edition (including revisions). Geneva: World Health Organization, 2001, 2011, 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

PDQ® Adult Treatment Editorial Board. PDQ Acute Myeloid Leukemia Treatment. Bethesda, MD: National Cancer Institute. Updated <03/06/2024>. Available at: https://www.cancer.gov/types/leukemia/hp/adult-aml-treatment-pdq. Accessed <02/06/2025>. [PMID: 26389432]
Section: Acute Myeloid Leukemia Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/leukemia/hp/adult-aml-treatment-pdq
Glossary