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SEER Inquiry System - Report
Produced: 12/11/2023 12:23 AM
Question 20220006
Inquiry Details
References:
WHO Class CNS Tumors. 5th edition
Question:
Histology/Brain and CNS: How is histology coded for a 2021 diagnosis of “neuroepithelial tumor with PATZ1-EWSR1 fusion, not elsewhere classified” found during a right thalamic mass resection? See Discussion.
Discussion:
Patient has a remote history of a right thalamic mass status-post two resections; reported as malignant oligodendroglioma (pathology not received) and chemo/radiation therapy, who recently presented with persistent headaches. Imaging revealed a 3.4 cm heterogeneous lobulated right thalamic mass with coarse calcifications and a probable cystic component.
Pathologist indicates the histologic and immunophenotypic features of this neoplasm are that of relatively circumscribed neuroepithelial tumor without high grade features (mitotic activity, microvascular proliferation, necrosis). Molecularly this neoplasm is characterized by a PATZ1-EWSR1 fusion, which has recently been proposed to be a distinct neuroepithelial tumor entity with a broad histological spectrum.
Answer:
Assign 8000/1. Neuroepithelial tumor with PATZ1-EWSR1 fusion, not elsewhere classified, is not recognized as a distinct entity at this time. It is not listed in ICD-O-3.2 or in the 5th edition of the WHO CNS classification.
