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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 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.

Change to current instructions. We will update this example in the next edition of the manual. The new example will instruct registrars to look for other descriptions of the patient’s race. When no other information is available, assign 07, Native Hawaiian, in Race 1 and assign 97, Pacific Islander, NOS in Race 2. Begin following this new instruction now.

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.

Report this case of leukemia cutis and code to bone marrow (C421) and leukemia NOS (9800/3) based on the information provided. Update the abstract if new information becomes available.

Leukemia cutis is the rare infiltration of neoplastic leukocytes into the epidermis, dermis, or subcutis from an existing leukemia that results in clinically identifiable cutaneous lesions. Leukemia cutis may precede, follow, or occur concurrently with the diagnosis of systemic leukemia. It is an advanced phase of the leukemia having a poor prognosis that also strongly correlates with additional sites of extramedullary involvement. This can alter the appropriate treatment regimen for a patient. It is a type of "metastasis" or spread of the leukemia cells. The "conventional" definition for leukemia cutis is the infiltration of skin from a bone marrow primary. It is most often diagnosed via skin biopsy—punch, shave, etc., utilizing IHC/biomarker testing and is commonly associated with CMML and acute myeloid leukemia (AML). As such, it a reportable condition especially when preceding a confirmed systemic leukemia diagnosis. In this situation, the diagnosis date would be the date of the positive leukemia cutis skin bx—punch, shave, etc. The case should be coded to C421; 9800/3 Leukemia NOS until the official systemic leukemia diagnosis is rendered. If possible, follow back should be conducted to determine the specific systemic leukemia histology (CMML; AML) and the treatment received. If the leukemia cutis follows or occurs concurrently with the diagnosis of a systemic leukemia, it is NOT a separate primary but merely an advanced stage of the systemic leukemia diagnosis.

High grade dysplasia of the esophagus is reportable. The example will be corrected in the next edition of the SEER manual.

Report smoldering WM (9761/3) using the Hematopoietic and Lymphoid Neoplasms Manual and Database (Table B9). Smoldering WM is defined as a poorly described asymptomatic disorder with a high risk of progressing to symptomatic WM requiring treatment. The term “smoldering” refers to the process meaning it is progressing, perhaps slowly, or even at a slower pace than might be expected. Smoldering WM resembles smoldering MM.

Report ovarian mucinous borderline tumor with foci of multifocal intraepithelial carcinoma. The foci of intraepithelial carcinoma makes this reportable. See the list of synonyms for in situ in the SEER Manual, Behavior Code data item.

Reportability for EIN became effective in 2021. 

1. Do not report atypical glandular epithelium. Atypical glandular epithelium, also referred to as atypical glandular cells (AGC), refers to abnormal looking cells that may be found in the tissue lining the inside of the endometrium or the cervix. While not malignant (in situ or invasive), they can be associated with a range of lesions in the female reproductive system.

2. Do not report isthmic-type mucosa with focal severe atypia. The NCI data dictionary defines atypia as an abnormality in cells in tissue. Report the case when further defined as atypical hyperplasia.

3. Do not report simple hyperplasia without atypia. WHO Classification of Tumors online, Female Genital Tumors (5th ed.), defines endometrial hyperplasia without atypia as a proliferation of endometrial glands of irregular size and shape without significant atypia. There is no ICD-O code for this term. Simple endometrial hyperplasia without atypia is an acceptable related term for endometrial hyperplasia without atypia. Pathology has priority over a physician statement.

4. Report EIN/CAH or focal EIN/CAH (8380/2) based on the biopsy. WHO Classification of Tumors online, Female Genital Tumors (5th ed.), defines EAH/EIN as a simultaneous change of epithelial cytology and an increased number of endometrial glands in a defined region. The preferred term is atypical hyperplasia of the endometrium; terms not recommended include complex atypical endometrial hyperplasia; simple atypical endometrial hyperplasia; endometrial intraepithelial neoplasia.

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.