Hematolymphoid neoplasms associated with rearrangements of PDGFRA, PDGFRB, and FGFR1.
OBJECTIVE
This session of the 2013 Society for Hematopathology/European Association for Haematopathology Workshop was dedicated to tumors currently included in the World Health Organization (WHO) classification category of myeloid and lymphoid neoplasms with eosinophilia and abnormalities of PDGFRA, PDGFRB, and FGFR1.


METHODS
We use the cases submitted to this session to review the clinicopathologic and genetic spectrum of these neoplasms, methods for their diagnosis, and issues related to the WHO classification terminology. Since many patients with these neoplasms have eosinophilia, we also briefly mention other causes of clonal eosinophilia.


RESULTS
These neoplasms are the result of gene fusions involving any one of these three tyrosine kinase genes. A variety of gene fusion partners have been found consistently for each category of neoplasms. Diagnoses of these neoplasms are often highly challenging and require a high index of suspicion and a multidisciplinary approach.


CONCLUSIONS
Early recognition of these neoplasms is important because patients with neoplasms associated with PDGFRA or PDGFRB fusions often respond to tyrosine kinase inhibitor therapy, whereas patients with neoplasms associated with FGFR1 fusions usually do not respond.
From the Division of Hematopathology, Department of Pathology and Laboratory Medicine, University of Miami/Sylvester Comprehensive Cancer Center, Miami, FL;
Francisco
F
Vega
Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston; and.
L Jeffrey
LJ
Medeiros
Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston; and.
Carlos E
CE
Bueso-Ramos
Departmento de Investigacion, Patologia y Laboratorio Clinico, Instituto Nacional de Enfermedades Neoplasicas, Lima, Peru.
Patricia
P
Arboleda
Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston; and Roberto.miranda@mdanderson.org.
Roberto N
RN
Miranda
D000818:Animals; D004802:Eosinophilia / Q000378:metabolism*; D006801:Humans; D009196:Myeloproliferative Disorders / Q000378:metabolism; D009369:Neoplasms / Q000378:metabolism*; D011505:Protein-Tyrosine Kinases / Q000378:metabolism*; D020796:Receptor, Platelet-Derived Growth Factor alpha / Q000378:metabolism*; D020797:Receptor, Platelet-Derived Growth Factor beta / Q000378:metabolism*
D011505:Protein-Tyrosine Kinases; D020796:Receptor, Platelet-Derived Growth Factor alpha; D020797:Receptor, Platelet-Derived Growth Factor beta
Eosinophilia; FGFR1; PDGFRA; PDGFRB; Tyrosine kinases