Question: 20200007

Status


Final

Category


#1:   Multiple primaries
#2:   Heme & Lymphoid Neoplasms
#3:  


References


Source 1:   Hematopoietic and Lymphoid Neoplasms Manual and Database
pgs:  
Notes:   Effective with cases diagnosed 1/1/2010 and forward
Source 2:   WHO Class Hem & Lymph Tumors
pgs:   69
Notes:   Revised 4th edition


Question


Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned when a patient is simultaneously diagnosed with systemic mastocytosis and chronic myelomonocytic leukemia (CMML-0) on a single bone marrow biopsy? See Discussion.
 



Discussion


The Hematopoietic and Lymphoid Neoplasms Database (Heme DB) definition for systemic mastocytosis with an associated hematological neoplasm (SM-AHN, 9741/3) states SM-AHN is a variant of systemic mastocytosis that arises with a myeloid disease of non-mast cell lineage (e.g., MDS, MPN, etc.) and that, The AHN should be abstracted as a second primary.

However, SINQ 20130172 conflicts with the Heme DB stating the systemic mastocytosis and the associated hematological neoplasm are a single primary coded to a single histology (9741/3) per Rule M2.



Answer


Abstract a single primary when the diagnosis is systemic mastocytosis with an associated clonal hematogoical non-mast cell lineage disease (SM-AHNMD) (9741/3).  However, if the patient has a previous history of myelodysplastic syndrome, myeloproliferative neoplasm, myelodysplastic/myeloproliferative neoplasm or acute leukemia, abstract the SM-AHNMD as a second primary as stated in the Heme DB. 

SINQ 20130172 represents a single primary as there is no mention of a prior history of myelodysplastic syndrome, myeloproliferative neoplasm, myelodysplastic/myeloproliferative neoplasm or acute leukemia. 



History




Last Updated


01/17/2020

Date Finalized


02/20/2020