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Do not code Reblozyl (luspatercept) as treatment. Luspatercept is an ancillary drug approved to treat anemia associated with MDS but not the malignancy.

Report optic nerve sheath meningioma arising in the intraorbital segment. The optic nerve contains four segments, of which intraorbital is one. The WHO Classification of Eye Tumors, 4th edition, defines meningioma as a neoplasm originating from the meningothelial cells of the optic nerve leptomeninges. According to the Table 3 of the Non-malignant Solid Tumor Rules, all portions of the optic are reportable and meningiomas arising in the dura/meninges of an intracranial nerve are coded to cerebral meninges C700.

The CPC ICD-O-3 Site Morphology Validation Lists (SMVLs) designate all tumor site-morphology combinations that are either valid or impossible as determined for the sites reviewed by the Cancer PathCHART initiative. These lists provide information on the Validity Status of specific tumor site and morphology combinations, similar to the way the ICD-O-3 SEER Site/Histology Validation List used to. However, the CPC SMVLs do not include information on the reportability of specific tumor site and morphology combinations. For tumor reportability, you will continue to use the Excel ICD-O-3.2 spreadsheets posted to the NAACCR ICD-O-3 Coding Updates website: https://www.naaccr.org/icdo3/, and the most recent SEER Manual and federal, state, local, and other standard setters' reportability requirements.

Answer updated September 2025:

Abstract a single primary as primary myelofibrosis (9961/3). ET was not reportable in 1998.

Assign code 01 (White) for Hispanic when there is no additional information. It is listed in the 2024 SEER Manual, Race Coding Instruction 6.b.i. and in Appendix D for code 01. We will remove Hispanic from the list in Appendix D under code 99 in the next version of the manual.

The example used in Rule M15 of the Urinary Solid Tumor Rules should refer to Table 2. We will update this in the next revision of 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 8249/3 for high-grade neuroendocrine tumor of the lung.

WHO Classification of Thoracic Tumors, 5th edition, defines two subtypes of neuroendocrine tumor of the lung, typical carcinoids (8240/3), and atypical carcinoids. WHO assigns typical carcinoid/neuroendocrine tumor grade 1 as 8240/3 and atypical carcinoid/neuroendocrine tumor grade 2 as 8249/3. They are regarded as low-grade and intermediate-grade, respectively. The preferred term for 8249/3 in ICD-O-3.2 is neuroendocrine tumor grade 2, with neuroendocrine tumor grade 3 as a related term. The Lung Solid Tumor Rules assign atypical carcinoid as 8249/3.

Assign histology as squamous cell carcinoma, HPV positive (8085) for tonsil, NOS (C099) based on the positive HPV test. Codes 8085 and 8086 are valid for a select group of sites. The histology terms and codes that are valid for head and neck sites are included in the Head and Neck Solid Tumor Rules, Table 5 (oropharynx).

HPV detection tests that are used to identify HPV include DNA polymerase chain reaction (PCR), p16 (IHC), or DNA/RNA in situ hybridization. Assign the appropriate method of detection in the SEER data item, SEER Site-Specific Factor 1.  

Schema Discriminator 2 captures additional information needed to generate AJCC ID and Schema ID for some anatomic sites as stated in the SSDI Manual. For oropharyngeal cancer, a schema discriminator is used to discriminate between oropharyngeal tumors that are p16 positive, p16 negative, or p16 status unknown in order to assign the appropriate schema ID. Only the HPV p16 test can be used to assign Schema Discriminator 2. If another HPV test is performed, code 9. Override the edit for Schema Discriminator 2 when p16 is negative. Coding updates will be implemented in 2025.

Sites may be added to certain combinations when indicated by ClinCORE review for Cancer PathCHART. Please note some sites were added in the 2024 update as a result of PathCHART review. A newly-formed Solid Tumor Editorial Board and its subgroups are currently working to evaluate the Solid Tumor Manual and make recommendations on ways to improve the structure and formatting of the manual and its content.

Follow the rules and instructions in the Other Sites STRs when assigning combination histology codes.

Histology Coding Rules

Use the Histology Coding Rules when assigning combination codes.

Coding Histology Information

Use this section that includes the mixed histology (Table 2) and site-specific histology tables (Tables 3-23) for one or more histologies within a single tumor. Do not use this section in place of the Histology Coding Rules.

While site-specific histology tables, based on current WHO Classification of Tumors books, have been added to Other Sites STRs, not all site groups have individual histology tables; coding may require the use of ICD-O and updates. The histology tables in Other Sites STRs include additional coding instructions and notes to assign the correct ICD-O code when appropriate.

The tables are not meant to be all-inclusive; rather they are intended to address difficult coding situations to facilitate the assignment of the correct histology code.

Table 2: Mixed and Combination Codes Instructions

Once you have identified the histology terms and have been instructed to use Table 2 by the Histology Coding Rules, compare the terms in the diagnosis to the terms in Column 1. When the terms match, use the combination code listed in Column 2. Use adenocarcinoma mixed subtypes 8255 as a “last resort” code.