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Apply 2018 Malignant CNS Solid Tumor Rule M5 and abstract multiple primaries when there are multiple CNS tumors, one of which is malignant /3 and the other is non-malignant /0 or /1. According to Note 3, a non-malignant CNS tumor and a malignant CNS tumor are always multiple primaries (timing and primary sites are irrelevant). Prepare two abstracts; one for the non-malignant and another for the malignant tumor.

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.

Any treatment that destroys or modifies cancer tissue should be recorded as the appropriate type of treatment -- chemo, immuno, etc. Even if immunotherapy is given for symptoms/palliative treatment, it is likely to kill off tumor cells.

Answer revised July 2008

For cases diagnosed 2007 or later:

For the purpose of applying the Lung MP/H rules, the word "Nodule" can be used interchageably with "Tumor," "Mass," "Lesion" and "Neoplasm." HOWEVER, this does NOT apply to casefinding or staging.

This revision will be added to the next version of the MP/H rules. Sinq question 20071028 will be revised.

Answer revised 3/31/2022

Do not report cases based only on the TI-RADS category. The most recent information from ACR on TI-RADS indicates that neither TI-RADS 4 nor TI-RADS 5 is clearly defined as malignancy. TI-RADS 4 is "moderately suspicious" and TI-RADS 5 is "highly suspicious" but they do not specify what they are suspicious for. We need more information to determine reportability.

Angiocentric glioma is reportable. The best histology code currently available is 9380/1 [glioma, NOS; uncertain behavior].

According to the WHO Classification of Central Nervous System Tumours, Angiocentric glioma has a behavior of /1. WHO defines it as an epilepsy-associated stable or slowly growing cerebral tumour primarily affecting children and young adults; histopathologicaly characterized by an angiocentric pattern of growth, monomorphous bipolar cells and features of ependymal differentiation.

An extracranial schwannoma is not reportable. The schwannoma must arise on the intracranial part of the nerve to be reportable.

Always code primary site to the site of origin. Look for information about where the neoplasm originated. Primary site should always be coded to reflect the site of origin according to the medical opinion on the case. Always code the primary site based on where the tumor arose / site of origin. Site of origin may be indicated by terms such as "tumor arose from," "tumor originated in," or similar statements.

Refer to ICD-O-3.2 and ICD-O-3 for topographty codes that are associated with specific histologies whenthe medical documentation does not specify the primary site.

After consultation with our expert pathologist, the decision is report this case as a single primary. There was some confusion about how to apply the current MP/H rules to this pathology report given 1) the definition of M16 and M17 and 2) the likelihood for a single endometrial primary to present with several differentiations. According to our expert pathologist, "I would regard this case as a single endometrial primary with extensive endometrial involvement and several types of differentiation, all of which are seen in endometrial carcinomas."

Next, the Multiple Primary and Histology Coding Rules Manual is the correct source for coding histology for cases diagnosed 2007 or later.

The following steps are used to determine the histology code.

Open the Multiple Primary and Histology Coding Rules manual. For an endometrial primary, use the Other Sites Histo rules to determine the histology code because endometrium does not have site specific rules.

Start with the MULTIPLE TUMORS ABSTRACTED AS A SINGLE PRIMARY module, Rule H18. The rules are intended to be reviewed in consecutive order within the module from Rule H18 to Rule H31. You stop at the first rule that applies to the case you are processing.

Code the appropriate combination/mixed code (Table 2) when there are multiple specific histologies. GYN malignancies with multiple types of adenocarcinoma have histology coded to 8323/3 [mixed cell adenocarcinoma] per rule H30.

Adamantinomatous craniopharyngioma (9351/1) is a subtype of craniopharygioma. We will correct the Non-Malignant CNS Solid Tumor Rules in the next update.