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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.

1. 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.

For cases diagnosed 2007 or later:

Assign code 8500 [duct carcinoma].

According to The World Health Organization (WHO), the term endometrioid carcinoma of the prostate is now called Prostate Duct Carcinoma.

Using Rule H11 (one type), code 8500 (duct carcinoma) for this rare type of tumor. Do not stop at Rule H10 because this is not acinar.

Refused [code 7] means this modality was specifically recommended by the physician and the patient refused. If two treatment alternatives were offered and surgery was refused, code Reason no surgery of primary site 1 [Surgery of the primary site was not performed because it was not part of the planned first-course treatment].

Refusal of surgery does not necessarily mean that all treatment was refused. Coding Surgery of Primary Site as "refused" does not affect the coding of Radiation, Chemotherapy, Hormone Therapy, etc.

Code as islet cell carcinoma [8150/3].

There are several cell types in the islets, and each produces a different hormone. The custom has been to name the tumors by their hormone production e.g. insulinoma, glucagonoma, etc. Occasional tumors do not produce any hormone (at least one that can be determined or measured). These tumors are called non-functioning endocrine tumors. Most of the endocrine tumors in the pancreas are islet cell tumors.

Revised December 2015

ELST is reportable. Code histology to adenocarcinoma (8140/3). Code primary site to inner ear (C301).

Endolymphatic sac tumors are rare non-metastasizing adenocarcinomas that originate in the endolymphatic sac of the inner ear (C301). They are slow growing and widely invade, and in later stages often destroy, the petrous bone. The WHO Classification assigns ICD-O-3 code 8140/3.

For cases diagnosed prior to 1/1/2010:Follow the instructions in the SEER manual on pages 1-4 to determine reportability.

Code the histology using all information available for the case. If the clinician reviews the case and states a particular histology based on his/her review, code that histology.

The clinician has access to all of the information available for this case. He/she uses his/her expertise to form a clinical diagnosis.

For cases diagnosed 1/1/10 and later, refer to the Hematopoietic and Lymphoid Neoplasm Case Reportability and Coding Manual and the Hematopoietic Database (Hematopoietic DB) provided by SEER on its website to research your question. If those resources do not adequately address your issue, submit a new question to SINQ.

Code the primary site C031 [Mandibular gingiva]. Code the histology 8033 [sarcomatoid carcinoma]. This tumor originated in the mandibular gingiva and invaded the bone (mandible) -- It did not originate in the bone. This type of tumor does not originate in bone.