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The only Other Sites H Rule related to extramammary Paget disease is included in the Multiple Tumors Abstracted as a Single Primary module. Rule H28 instructs one to code the histology of the underlying tumor when there is extramammary Paget disease and an underlying tumor of the anus, perianal region, or vulva. Therefore, a vulvar extramammary Paget disease with underlying adenocarcinoma is coded as adenocarcinoma (8140/3), and not extramammary Paget disease (8542/3).

However, there is no M Rule confirming extramammary Paget disease and the underlying adenocarcinoma are a single primary (i.e., multiple tumors abstracted as a single primary) making it difficult for one to use the Multiple Tumors Abstracted as a Single Primary H rules module.

We recognize this is a longstanding histology coding rule, but how are registrars supposed to arrive at Rule H28 when the M Rules must be applied first and do not instruct one to accession a single primary? Moreover, if this is to be a single primary (per rule M2), why is there no H Rule in the Single Tumor module?

Please comment on these specific scenarios.

1. A prostate biopsy is suspicious for adenocarcinoma, but the urologist is not considering this malignant, nor is the urologist treating for malignancy, but there is no clear statement from the physician that this is not reportable.  Should this case be abstracted?
2. A prostate biopsy is suspicious for adenocarcinoma, and there is a statement by the physician that this is not yet malignant. Should this case be abstracted?
3. Prostate MRI with PIRADS 4 or 5; however, the treating urologist does not call this malignant and is not treating patient for prostate cancer.  [Example: 2024 Prostate MRI - PI-RADS 4. Follow up with Urologist states patient to repeat Prostate MRI in 1 year and continue with yearly PSA levels and there is no mention of prostate cancer other than the PI-RADS score]. Should this case be abstracted?

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.

Should Note 2 state, "Beginning with cases diagnosed 1/1/2023 forward, diffuse leptomeningeal glioneuronal tumor is coded 9509/3? See the Malignant CNS rules." Currently the Note only states, "leptomeningeal glioneuronal tumor," but the histology that changed behavior is listed in both Table 6, Column 1 (Non-Malignant CNS) and Table 3 (Malignant CNS) as, "Diffuse leptomeningeal glioneuronal tumor."

2012 Total abdominal hysterectomy - bilateral salpingo-oophorectomy

Primary Site – Ovary, Right

Histology - Small-Cell Carcinoma (Hypercalcemic Type), Large-Cell Variant

2024 Total abdominal hysterectomy - bilateral salpingo-oophorectomy

Primary Site – Ovary, Left

Histology - Small-Cell Carcinoma (Hypercalcemic Type), Large-Cell Variant

Example 4:  Esophageal biopsy with diagnosis of “focal areas suspicious for adenocarcinoma in situ.” Diagnosis on partial esophagectomy specimen “with foci of high grade dysplasia; no invasive carcinoma identified.” Do not accession the case. The esophagectomy proved that the suspicious biopsy result was false.

Appendix E2 #32 of the SEER Manual states high grade dysplasia in site other than stomach, small intestines, and esophageal primary sites are not reportable. Does this mean high grade dysplasia is reportable for esophagus primaries?

Example: Patient has a 2023 brain MRI described as having a “new dural based nodule, statistically meningioma, along the left distal tentorial incisura.” All subsequent chart information is related to patient’s unrelated diagnosis of multiple sclerosis only.

Is the terminology “statistically” reportable ambiguous terminology in this context?

Based on the Solid Tumor Rules, we would make this two since it is over one year. According to a previous SINQ question 20110046, we are to code this as one primary. We see patients come back with multiple carcinoid tumors over the years and would like clarification.