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Additional comments in this pathology report state "The entire case was sent in consultation to an ophthalmic pathologist. [Pathologist] assigns a conjunctival melanocytic intraepithelial neoplasia (C-MIN) score of 2-3 due to the upward pagetoid migration of small, dendritic melanocytes. A C-MIN score of 2-3 is between low-grade conjunctival melanocytic intraepithelial lesion (LG-CMIL; C-MIN 2) and high-grade conjunctival intraepithelial lesion (HG-CMIL; C-MIN 3). The older terminology for this lesion would be primary acquired melanosis (PAM) with mild to focally moderate atypia."

This term does not appear in the SEER Program Coding and Staging Manual (SPCSM), Appendix E1 of the SPCSM, or Solid Tumor Rules (specifically rule H3) . 

The WHO Classification of Hematopoietic Tumors (Blue Book), 5th edition states: “Rare cases of classic follicular lymphoma with cytological features of follicular lymphoma (FL) grade 3A can present with a prominent diffuse pattern. In the previous edition, such cases were defined as diffuse large B-cell lymphoma (DLBCL). Currently, it is uncertain whether such cases should be classified as FL or diffuse large B-cell lymphoma; and in such cases, individual treatment choices should be made in multidisciplinary conference settings taking into consideration clinical, laboratory, and imaging parameters. The presence of diffuse areas composed entirely or predominantly of large cells, however, warrants a diagnosis of diffuse large B-cell lymphoma.”

Our concern is that the Hematopoietic (Heme) Manual and Database do not provide instruction for coding this scenario. We hesitate to interpret the terms “equivalent to” as ambiguous because one could argue it is unambiguous. Barring this argument, the M and H rules would indicate this is a diagnosis of diffuse large B-cell lymphoma. However, the physician does not seem to agree with the pathologist.

The Solid Tumor Rules state that for cases diagnosed in 2022 and forward, p16 testing CAN be used to assign histology code 8085 (squamous cell carcinoma, HPV positive). The rules also state that for cases diagnosed prior to 1/1/2022, code 8085 MUST be based on ISH testing and not p16. ISH testing is not specifically addressed for 2022+ cases, but are we correct in assuming it can still be used as the basis for 8085?

Multiple CAnswer Forum posts and the AJCC 8th edition Head and Neck staging webinar indicate that the correct chapter/registry staging schema in this situation is determined ONLY by p16 results - not ISH testing, and therefore the Schema Discriminator 2 SSDI should be coded as 1 – p16 negative, regardless of ISH results.

While we understand that histology codes should not be changed based on staging criteria, there is a SEER/NAACCR edit, “Schema Discriminator 2, Head and Neck, Histology (NAACCR)” tag number N6802, that will not allow coding 8085 if Schema Discriminator 2 is coded as 1 (p16 negative). The edit does seem to be correctly enforcing the AJCC guidelines for choosing the staging schema, based on the sources noted above. Do the Solid Tumor or Site-Specific Data Items (SSDI) guidelines need to be modified for this situation?

There are multiple possible entries (rows) for a tumor with a neuroendocrine component and non-neuroendocrine component, but these rows do not specify which primary sites are applicable. Row 1 (Combined small cell carcinoma, 8045) seems applicable to a prostate primary, but not to a GI primary since GI primaries are now generally referred to as MiNENs (mixed neuroendocrine non-neuroendocrine tumors), but Table 2 does not provide any instructions regarding how to determine the difference between 8045 and 8154 (or 8244).

For SEER Workshop Case 03 (mixed prostate case), many users selected 8154 or 8244 as the mixed histology code per Table 2, but these histology codes are not listed as applicable in Table 3 (Prostate Histologies). Per the WHO Blue Books, these histologies are not listed as applicable to the prostate.

How are registrars to determine the correct mixed code without site designations, especially if they don't have access to the WHO Blue Book or to a pathologist who may be able to clarify the codes?

Example: 12/2023 Breast lumpectomy final diagnosis is Invasive ductal carcinoma, lymphoepithelioma-like carcinoma type. This is a single tumor with no in situ carcinoma present.

Lymphoepithelioma-like carcinoma is not listed as a subtype/variant or synonym for breast carcinoma in the Solid Tumor Rules histology tables.

Patient was diagnosed on imaging with enhancement along the right optic nerve intraorbital segment, displacing the optic nerve, most consistent with optic nerve sheath meningioma.

Extracranial meningiomas are rare, however SINQ 20230052 does contain an exception for reportability in a different head and neck site because it is not an intracranial location.

It is unclear if this portion of the meninges surrounding the intraorbital optic nerve is still “intracranial” and thus reportable.

1.  The 2022 and 2023 SEER Program Coding Manuals state under Histologic Type ICD-O-3: Beginning with cases diagnosed 01/01/2022 forward, p16 test results can be used to code squamous cell carcinoma, human papilloma virus (HPV) positive (8085) and squamous cell carcinoma, HPV negative (8086).

NAACCR 2023 Implementation Guidelines contain similar instructions on HPV histologies for cervix, vulva and vagina that are applicable back to 2022 (2021 for cervix).

The current Other Sites Solid Tumor Rules state on the Histology tables for anus, cervix, vagina, vulva, and penis and scrotum: "p16 is a valid test to determine HPV status and can be used to code HPV associated and HPV independent histologies." Since Other Sites Solid Tumor Rules apply to cases diagnosed 2023+, can p16 results only be used from 2023 onward, to code HPV-related histologies for primaries that fall under the Other Sites module? Or per the 2022 SEER Manual statement and NAACCR 2023 Implementation Guidelines, could a p16-confirmed HPV histology code also apply to a 2022 Other Sites case and if so, is that only for cervix, vulva, and vagina? Further complicating the matter are the 2024 ICD-O-3.2 update documents indicating these codes are valid 1/1/2024+ for the “Other Sites” penis and scrotum.

2.  Is using p16 testing for HPV-related histology codes ONLY allowed for sites in the Solid Tumor tables that contain the statements about p16 (Head & Neck Table 5, and the Other Sites tables noted above for anus, cervix, etc.)? Or could it apply to primary sites outside of those tables; for example, a 2022 pathology report from the ethmoid sinus C311 indicating an HPV-related histology based on p16 testing? The ICD-O-3 Annotated Histology lists include C310-C313 among the common site codes for 8085 and 8086. The Head and Neck Solid Tumor Rules “New for 2022” section and rule H1 Note 4 also mention that p16 can be used to code HPV histologies; these sections would seem to apply to all sites in that module, since only the more common histology codes are listed in the tables and if not, we are instructed to use ICD-O.

Example: Patient has a 04/2023 diagnosis of symptomatic anemia not responsive to Retacrit. Further testing includes diagnostic bone marrow biopsy 10/2023 proving MDS with low blasts and SF3B1 mutation, treated with Relozyl (Luspatercept).

There is no SEER*Rx listing for Reblozyl or Luspatercept. Per web search, Luspatercept, sold under the brand name Reblozyl, is a medication used for the treatment of anemia in beta thalassemia and myelodysplastic syndromes.

Is this non-cancer directed treatment since it is given to address the anemia rather than the MDS? If cancer-directed treatment, how should it be coded?

Pathologists are increasingly using the terms "myeloid stem cell disorder" and "myeloid stem cell neoplasm" to describe reportable myeloid neoplasms. If the pathologist uses these terms and indicates the differential diagnosis includes only reportable neoplasms such as myelodysplastic syndrome, myeloproliferative neoplasm, and acute myeloid leukemia (AML), should this be accessioned as a reportable primary?

Example: The 01/2023 peripheral blood shows high grade myeloid stem cell disorder, and the differential diagnosis includes chronic myelomonocytic leukemia(CMML) and AML. The patient refused further work-up and expired several days later. No additional information is available.