Search Database ICD-O-3 Code Lists

Name

Myeloid/lymphoid neoplasms with PDGFRA rearrangement

ICD-O-3 Morphology

9965/3: Myeloid and lymphoid neoplasms with PDGFRA rearrangement
Effective 2010 and later

Reportable

for cases diagnosed 2010 and later

Primary Site(s)

C421
Primary site must be bone marrow (C421). Blood and bone marrow always involved.

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

(This code is effective for cases diagnosed 2010 and later. For cases diagnosed prior to 2010 see code 9960/3.)

Myeloid/lymphoid neoplasms with PDGFRA rearrangement is part of the Myeloid/lymphoid neoplasms lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B8)

The peripheral blood and bone marrow are invariably involved. The lungs, heart, central and peripheral nervous system, skin, and gastrointestinal tract are among other frequently involved organs.

Diagnostic Confirmation

This histology can only be determined by positive genetics, diagnostic confirmation will always be 3.

Module Rule

C96.9 Malignant neoplasms of lymphoid, hematopoietic, and related tissue, unspecified

Alternate Names

None

Definition

Myeloid/lymphoid neoplasm with PDGFRA rearrangement includes myeloid and lymphoid neoplasms and is frequently associated with eosinophilia. The majority of cases are characterized by cytogenetically cryptic deletion of 4q12, resulting in formation of the FIP1L1::PDGFRA fusion gene, although other partner genes have been described. (WHO 5th edition)

Definitive Diagnostic Methods

Cytogenetics
Genetic testing

Genetics Data

None

Immunophenotyping

Immunophenotyping not relevant, diagnosed via genetic analysis

Treatments

Chemotherapy
Other 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)

C96.7 Other specified malignant neoplasms of lymphoid, hematopoietic, and related tissue
C96.9 Malignant neoplasms of lymphoid, hematopoietic, and related tissue, unspecified

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

C96.Z Other specified malignant neoplasms of lymphoid, hematopoietic, and related tissue (effective October 01, 2015)
C96.9 Malignant neoplasms of lymphoid, hematopoietic, and related tissue, unspecified (effective October 01, 2015)

Signs and Symptoms

Cardiomyopathy
Endomyocardial fibrosis
Fatigue
Pruritus
Serum tryptase increased
Splenomegaly
Vitamin B12 serum levels markedly elevated

Diagnostic Exams

Bone marrow aspiration and biopsy
Cytogenetic analysis
Flow cytometry
Immunophenotyping
Molecular analysis
Peripheral blood smear

Progression and Transformation

None

Epidemiology and Mortality

Age: 25-55 years (median age onset 40s)
Sex: male dominance

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: Myeloid/lymphoid neoplasms with eosinophilia and tyrosine kinase gene fusions
Pages: Part A: 185-187

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
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