Name

Therapy-related myeloid neoplasms

ICD-O-3 Morphology

9920/3: Therapy related myeloid neoplasm
Effective 2001 and later

Reportable

for cases diagnosed 2001 and later

Primary Site(s)

C421
Primary site must be bone marrow (C421)

Grade

Not Applicable

Module Rule

None

Alternate Names

Secondary MDS
t-AML
t-AML/t-MDS
t-MDS
t-MDS/MPN
Therapy-related acute myeloid leukemia, alkylating agent-related
Therapy-related acute myeloid leukemia, epipodophyllotoxin-related
Therapy-related acute myeloid leukemia, NOS
Therapy-related acute promyelocytic leukemia (APL)
Therapy-related myelodysplastic syndrome
Therapy-related myelodysplastic/myeloproliferative neoplasm

Definition

Therapy-related myeloid neoplasms (t-MNs) include therapy-related cases of acute myeloid leukemia (t-AML), myelodysplastic syndromes (t-MDS), and myelodysplastic/myeloproliferative neoplasms (t-MDS/MPN) that occur as a late complication of cytotoxic chemotherapy and/or radiation therapy administered for a prior neoplastic or non-neoplastic disorder.

Although cases may be diagnosed morphologically as t-MDS, t-MDS/MPN, or t-AML according to the number of blasts in the blood and/or bone marrow, these t-MNs are best grouped together as a unique clinical syndrome distinguished by prior iatrogenic exposure to mutagenic agents.

Excluded from this category are progression of MPNs and evolution of primary MDS or primary MDS/MPN to AML; in each of these latter cases evolution to AML is part of the natural history of the primary disease and it may be impossible to distinguish natural progression from therapy-related changes.

Abstractor Notes

For cases abstracted prior to 2010, this code was only used for therapy-related Acute myeloid leukemia (t-AML).

DO NOT code therapy-related myeloid neoplasm simply because the patient has a history of radiation therapy or chemotherapy. There must be a physician's statement that says this is a therapy-related neoplasm (acute myeloid leukemia, MDS, MPN, or MDS/MPN.)

If a specific myeloid neoplasm that is described with a different specific histology term is also stated to be therapy related, code 9920/3 to capture the fact that this disease was therapy related. Document the other specific histology term in the text part of the abstract.

Definitive Diagnostic Methods

Bone marrow biopsy
Genetic testing
Immunophenotyping

Genetics Data

del(3p)
del (13q)
del(20q)
del(11q)
FLT3
Gain of chormosome 8
IDH1/2
Loss of chromosomes 5q, 7, 17, 17p, 18, 21
NRAS
PTPN11
TET2
TP53 loss

Immunophenotyping

CD13 expression
CD33 expression
CD34 positive
MPO expression

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Immunotherapy
Radiation therapy

Transformations from

None

Corresponding ICD-9 Codes

205.0 Acute myeloid leukemia

Corresponding ICD-10 Codes

C92.0 Acute myeloid leukemia

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

C92.0 Acute myeloblastic leukemia (effective October 01, 2015)

Signs and Symptoms

Easy bruising or bleeding
Shortness of breath
Weakness
Weight loss or loss of appetite

Progression and Transformation

None

Epidemiology and Mortality

Incidence: 10-20% of all cases of AML, MDS and MDS/MPN
Survival: 5 year survival less than 10%

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: Acute myeloid leukemia and related precursor neoplasms
Pages: 153-155

International Classification of Diseases for Oncology, Third Edition, First Revision. Geneva: World Health Organization, 2013. (http://codes.iarc.fr/codegroup/2)
Section: ICD-O-3.1 (2011) Morphological Codes
Pages: http://codes.iarc.fr/codegroup/2

National Cancer Institute
Section: General Information About Acute Myeloid Leukemia
Pages: https://www.cancer.gov/types/leukemia/hp/adult-aml-treatment-pdq
Glossary