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Do not report angiomyxoma. ICD-O-3.2 assigns 8841/0 to this benign tumor. This includes superficial and deep (aggressive) angiomyxoma.

Assign the highest code with 51 as the highest and 10 the lowest when there is more than one test performed with differing results.

The SEER Manual will be updated in the 2025 release. SEER*RSA will be updated in a future release, version 3.3 in 2026, as version 3.2 has been updated for 2025.

Please understand that AJCC sets the standards for TNM Staging and the Cancer PathCHART (CPC) initiative sets standards for the validity of site and morphology combinations. The statement in the AJCC manual “8240 and 8249 are excluded for topography code C160” means that these two histologies are not staged using the AJCC Staging System. As with numerous other reportable entities that are not staged by AJCC, the case is reportable and a Summary Stage should be assigned. Combinations of C160 with 8240 or 8249 are valid site/histology combinations for registry reporting and should not be discouraged from use if they correspond to the pathologist’s diagnosis. This goes for any other similar note in the AJCC manual. All CPC standards are enforced via the Primary Site, Morphology-Type, Beh ICDO3, 2024 (SEER) N7040 and Histologic Type ICDO3, Primary Site, Date of Diagnosis (NAACCR) N4911 data quality edits. Registrars can also look up the validity of site and morphology combinations using the CPC*Search tool:  https://seer.cancer.gov/cancerpathchart/search/tool/. 

It is important to remember the following.

• ALWAYS code the tumor histology stated by the pathologist/physician
• NEVER change the tumor histology to assign TNM
• Not all tumors or histologies can be staged per TNM
• Cases that cannot be assigned TNM are assigned a summary stage

Report malignant spindle cell neoplasms consistent with atypical fibroxanthoma as directed by Appendix E.1 of the 2023 and 2024 versions of the SEER Manual using 8830/3 (fibroxanthoma, malignant).

We will update the answer in SINQ 20190102.  While the Other Sites Solid Tumor Rules address coding an NOS and specific histology sub-type/variant, this situation is not specifically addressed. We will also review the rules.

Many of these terms are not specified in the codes and definitions in ICD-10-CM. This is because ICD-10-CM does not have the same granularity as ICD-O-3.2. There are a few sites where intraepithelial neoplasia II and/or III are mentioned.

Even though ICD-O-3.2 classifies these as /2 (in-situ), for the intraepithelial neoplasia that are listed in ICD-10-CM, Grade II is designated as benign, while Grade III is designated as in-situ. It is not clear if medical coding will change the Grade II to an in-situ code.

All the in-situ codes (except cervix) are included in the casefinding list. Grade III is included with the in-situ codes; however, there is no guarantee that medical coders will code them as in situ. High grades are coded as in-situ in ICD-10-CM.

For those where there is no specific intraepithelial neoplasia code, the benign codes will cover any benign lesion for that site. This would make for a lot of review using the codes for casefinding. Most of the benign codes were removed from the casefinding list a couple of years ago to make it more manageable. 

Use the casefinding list as a guide for these neoplasias. It is not the most definitive source due to the lack of specificity of ICD-10-CM. It is not possible to map every single histology to a specific code. It is also not known how medical coders across the U.S. are coding these neoplasias. For that reason, pathology should remain the foremost casefinding resource used.

The casefinding team will need to review the prepared list below and determine what codes to add. Any updates will be incorporated in the FY2025 updates (October 2024.)

Assign code 01 (White) for Race 1 when described as Indigenous-Latino/a or Indigenous-Latinx. Indigenous-Latinx is an umbrella term for Indigenous migrants to the United States from Latin America including South and Central America, the Caribbean, and Mexico (for example, Maya, Mixteco, Purépecha, Taino, Zapoteco, etc.). Latin America is listed in Appendix D of the 2025 SEER Manual as White.

Assign code 6 (Spanish, NOS; Hispanic, NOS; Latino, NOS) for Spanish Surname or Origin for Indigenous-Latino/a or Indigenous-Latinx in the absence of more specific information. Code 6 description includes the statement, There is evidence, other than surname or birth surname (maiden name), that the person is Hispanic but he/she cannot be assigned to any of the categories 1-5.

Assign code 03 (American Indian or Alaska Native) when described as First Nations. First Nations usually refers to Indigenous peoples for ethnic groups who are the original or earliest known inhabitants of an area. The term ‘First Nations’ can be applied to individuals, but technically refers only to those who have Indian status under Canadian law as part of a recognized community. Within Canada, the term First Nations is generally used for Indigenous peoples other than Inuit and Métis. Outside Canada, the term can refer to Indigenous Australians, U.S. tribes within the Pacific Northwest, as well as supporters of the Cascadian independence movement. 

Code the primary site to pelvic lymph nodes (C775) as it is the only site involved with this extrapulmonary lymphangioleiomyomatosis (E-LAM).

Do not assign a behavior code of /2 to these cases unless you have a way to flag them so that they are not reported to the standard setters as in situ cases. Work with your state central registry to ensure that these cases are not unintentionally included in state case submission.

Report the case when the differential diagnosis includes only reportable neoplasms in the absence of additional information. We are unable to provide general instructions for provisional diagnoses as each situation will need to be reviewed and assessed individually when no further work-up information is available.

Assign myeloid leukemia, NOS (9860/3) to the case described in the example. Assign a generic histology code because a specific histology code cannot be assigned when there are several differential diagnoses. Since the differential diagnoses include a chronic and an acute leukemia, code as myeloid leukemia, NOS since it is not clear if this is chronic or acute.