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SEER Inquiry System - Report
Produced: 11/12/2025 10:18 AM
Question 20081126
Inquiry Details
References:
#1: 2007 SEER Manual, 2
#2: 2007 SEER Manual, C-961to C-964. Appendix C
Question:
MP/H Rules--Brain and CNS: Are stigmata of neurofibromatosis in the brain reportable neurofibromatosis lesions? See Discussion.
Discussion:
Reference: SINQ 20051108; SINQ 20061018 Three year old patient with history of neurofibromatosis 1. 3/05 MRI of the brain showed right optic nerve glioma. It also showed heterogeneous high t2 signal in the middle cerebellar peduncles and near the genu of the internal capsules bilaterally are stigmata of neurofibromatosis type I. 3/08 MRI showed new mass suspicious for glioma in the hypothalamus. Clinical diagnosis is benign glioma secondary to diagnosis of neurofibromatosis. How many primaries are to be accessioned for this patient? Should the matrix principle be invoked for the second glioma? Should the behavior code for the glioma be 0?
Answer:
For cases diagnosed 2007 through 2017
Accession NF (9540/1) when there is CNS tumor -- a glioma or some other intracranial/intraspinal tumor. Stigmata of NF are reportable when the stigmata themselves are reportable tumors. For example, glioma, or another intracranial/intraspinal tumor. Do not report sitgmata that are only termed "stigmata seen on MRI," for example, without other reportable terminology.
Do NOT accession NF (9540/1) when there is only peripheral nerve/nervous system involvement.
Accession the neurofibromatosis itself only once per patient. Accession any initial neoplasm in the CNS separately. Abstract and code any subsequent CNS neoplasms according to the multiple primary brain rules.
Accession three primaries for the case described above.
- Neurofibromatosis (C729 9540/1)
- Optic nerve glioma (C723 9421/3)--> see below.
- Hypothalamus glioma (C710 9380/0)
--> Optic nerve gliomas associated with NF are pilocytic astrocytomas. Code pilocytic astrocytoma as 9421/3 in North America.
For cases diagnosed 2018 or later
See the 2018 Solid Tumor Rules for Non-Malignant CNS tumors.
