Search Database ICD-O-3 Code Lists

Name

Myeloid/lymphoid neoplasms with FGFR1 rearrangement

ICD-O-3 Morphology

9967/3: Myeloid and lymphoid neoplasms with FGFR1 abnormalities
Effective 2010 and later

Reportable

for cases diagnosed 2010 and later

Primary Site(s)

C421
Primary site must be bone marrow (C421)

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 FGFR1 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)

There is no established tyrosine kinase inhibitor therapy for MPN with FGFR1 rearrangement.

The most common sites of involvement include the bone marrow, peripheral blood, lymph nodes, liver, and spleen. Other extramedullary sites may be involved.

Diagnostic Confirmation

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

Module Rule

None

Alternate Names

None

Definition

This is a heterogenous group of neoplasms with FGFR1 rearrangement, arising from a pluripotent hematopoietic stem cell that may manifest as chronic myeloid neoplasms or blast-phase disease of B-cell, T-cell, myeloid, or mixed-phenotype origin, typically with associated eosinophilia. The phenotype may change throughout the disease course. (WHO 5th edition)

Definitive Diagnostic Methods

Cytogenetics
Genetic testing

Genetics Data

FGFR1 at8p11.2
Tyrosine kinase FGFR1 fusion gene

Immunophenotyping

Immunophenotyping not relevant, diagnosed via genetic analysis

Treatments

Hematologic Transplant and/or Endocrine Procedures

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

Fever
Hypermetabolism
Lymph node enlargement
Night sweats
Splenomegaly
Weight loss

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: 32 years median age (3-84 age range)
Sex: moderate male predominance
Survival: currently poor, specific therapy still being developed

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: 191-193

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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