Search Database ICD-O-3 Code Lists

Name

Acute myeloid leukemia with CBFB::MYH11 fusion (ICD-O-4: 98710/3)

ICD-O-3 Morphology

9871/3: Acute myeloid leukemia with inv(16)(p13.1q22) or t(16;16) (p13.1;q22), CBFB/MYH11
Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

C421
Primary site must be bone marrow (C421)

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

Acute myeloid leukemia with CBFB: MYH11 fusion 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)

The blood and bone marrow are nearly always involved. Some patients may present with myeloid sarcoma.

If this leukemia 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.

See Multiple Primary Rule M3

Diagnostic Confirmation

This histology must be coded as diagnostic confirmation code 3 until the 2027 updates are implemented in your software. Once the software updates are implemented: Assign code 1 for histological confirmation WITH positive genetics

Module Rule

See abstractor notes

Alternate Names

Acute myeloid leukemia, CBF-beta::MYH11 (ICD-O-4: 98710/3)
Acute myeloid leukemia, inv(16)(p13;1q22) (ICD-O-4: 98710/3)
Acute myeloid leukemia with CBFB rearrangement (ICD-O-4: 98710/3)
Acute myeloid leukemia with inv(16)(p13.1q22) (ICD-O-4: 98710/3)
Acute myeloid leukemia with t(16;16)(p13.1;q22) (ICD-O-4: 98710/3)
Acute myeloid leukemia with abnormal marrow eosinophils (ICD-O-4: 98710/3)
Acute myelomonocytic leukemia with abnormal eosinophils (ICD-O-4: 98710/3)
FAB M4Eo (ICD-O-4: 98710/3)

Definition

Acute myeloid leukemia (AML) with CBFB::MYH11 fusion is characterized by fusion of the core-binding factor beta subunit gene (CBFB) and the myosin heavy chain 11 gene (MYH11). (WHO 5th edition)

Definitive Diagnostic Methods

Cytogenetics
Genetic testing

Genetics Data

CBFB rearrangement (inv(16)(p13.1q22) or t(16;16)(p13.1;q22))

Immunophenotyping

Not applicable, genetics required for diagnosis

Treatments

Chemotherapy

Transformations to

None

Transformations from

Same Primaries

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

C92.0 Acute myeloid leukemia

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

C92.0 Acute myeloblastic leukemia (effective October 01, 2015 - September 30, 2024)
C92.00 Acute myeloblastic leukemia not having achieved remission (effective October 01, 2024)
C92.01 Acute myeloblastic leukemia, in remission (effective October 01, 2024)
C92.02 Acute myeloblastic leukemia, 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
Flow cytometry
Immunophenotyping
Lumbar puncture
Molecular analysis
Peripheral blood smear
Physical exam and history

Progression and Transformation

Longer complete remissions when treated with high dose cytarabine in the consolidation phase
Older patients and those with KIT mutations have higher risk of relapse and worse survival

Epidemiology and Mortality

Age: predominantly younger patients
Incidence: 5-8% of all cases of AML
Survival: longer complete remissions possible when treated with high dose cytarabine in the consolidation phase

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: 118-120

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
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