SEER Inquiry System - Report
Produced: 11/23/2024 8:44 PM
Question 20160028
Inquiry Details
References:
#1: WHO Class Soft Tissue & Bone, 306-307. 2013
#2: ICD-O-3, 95
Question:
MP/H/Histology--Sarcoma: How should Ewing Sarcoma/primitive neuroectodermal tumor (PNET) be coded for a 2012 case? See Discussion.
Discussion:
SEER SINQ 20031051 applies to cases diagnosed before 2007 and advises: Code histology as 9260/3, Ewing sarcoma. Ewing sarcoma is a specific histology on the continuum of primitive neuroectodermal tumors. Code Ewing sarcoma as it is more specific than PNET, NOS.
For tumors diagnosed 2007 or later, refer to the MP/H rules.
Answer:
Apply 2007 MP/H rule H6 and assign the numerically higher ICD-O-3 code that reflects PNET (9364/3).
According to the WHO Tumors of Soft Tissue and Bone, though Ewing sarcoma ICD-O-3 code is 9260/3, Ewing sarcoma with a higher degree of neuroectodermal differentiation present is classically termed peripheral neuroectodermal tumors (PNET). WHO does not offer guidance how to classify tumors stated to be Ewing sarcoma PNET.
Histology code 9364/3 is assigned for a Ewing/PNET that arises outside of the brain/CNS. Peripheral neuroectodermal tumor (PNET) and peripheral primitive neuroectodermal tumor (PPNET) are Ewing family tumors.
Histology code 9473/3 (PNET, primitive neuroectodermal tumor, central primitive neuroectodermal tumor, or supratentorial PNET) is only used for tumors arising inside the brain/CNS.