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SEER Inquiry System - Report
Produced: 09/13/2025 9:35 AM
Question 20180102
Inquiry Details
References:
Solid Tumor Rules, 17. Malignant CNS, 10/12/2018
Question:
Solid Tumor Rules 2018/Histology--Brain and CNS: What code should be used for high grade neuroepithelial tumor with BCOR Alteration? See Discussion
Discussion:
A recent molecular study of PNET tumors at NCI (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5139621) seems to indicate the discovery of four new CNS tumor entities, of which HGNET-BCOR is one. The article suggests that these are not primitive neuroectodermal tumors tumors (PNET), but something different.
Answer:
This question was reviewed by an expert neuropathologist. He recommends coding these tumors to malignant tumor, clear cell type 8005/3.
He states: these tumors are extremely rare. In summary, CNS HGNET-BCOR represents a rare tumor occurring in young patients with dismal prognosis. Whether CNS HGNET-BCOR should be classified among the category of "embryonal tumors" or within the category of "mesenchymal, nonmeningothelial tumors" remains to be clarified. Because CNS HGNET-BCOR share pathologic features and characteristic BCOR-ITD with clear cell sarcoma of the kidney, these tumors may represent local variants of the same entity.
