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The Solid Tumor Rules indicate bladder tumors that are urothelial carcinoma (8120) and small cell carcinoma (8041) are separate primaries per Rule M13 (Abstract multiple primaries when separate/non-contiguous tumors are on different rows in Table 2). These are distinctly different histologies and, presumably, one would want to capture the small cell carcinoma (or small cell carcinoma component) as this has a worse prognosis.

However, if a subsequent bladder tumor is composed of invasive urothelial carcinoma and small cell neuroendocrine carcinoma, the histology is coded as 8045/3 per Rule H4, but this is not abstracted as a multiple primary. The only M Rule that applies is Rule M18 (Abstract a single primary when tumors do not meet any of the above criteria). The mixed histology code 8045 is not included in Table 2, so none of the histology-based M Rules apply. Is the subsequent mixed invasive urothelial and small cell carcinoma tumor (8045/3) the same primary as a previously diagnosed invasive urothelial carcinoma (8120/3) when these tumors are diagnosed within three years?

Rule M11 states: Abstract a single primary when there are urothelial carcinomas in multiple urinary organs, but neither the Rule nor the Notes describe the timing of these multiple urinary organ carcinomas. Timing requirements for other rules are clearly stated.

Does Rule M11 have a timing requirement or is it intended to apply to all urothelial carcinoma tumors regardless of timing (and not already qualifying for application of a previous M rule)?

7/8/16 Urinary bladder, biopsy: Non-invasive low grade papillary urothelial carcinoma. Muscularis propria (detrusor muscle) is not identified.

9/2/16 Urinary bladder, bladder tumor, transurethral resection: High grade papillary urothelial carcinoma. No definite invasion identified. Muscularis propria (detrusor muscle) is identified and not involved by tumor.

1/7/17 A\S\Bladder: Noninvasive low grade papillary urothelial carcinoma. Granulomatous cystitis, consistent with BCG (Bacillus Calmette-Guerin) treatment. Lamina propria is not involved with tumor. Detrusor muscle is not identified.

4/4/17 Dome: Papillary urothelial carcinoma, low grade. No evidence of invasion. Muscularis propria is not present.

Patient is clearly followed for at least a year but no further information until 19 months later, 11/14/18, when biopsy of lung indicates metastatic disease.

11/14/18 Lung, right lower lobe, mass, biopsy: Metastatic urothelial carcinoma. Immunohistochemical analysis results (CK7 positive, CK20 focally positive, P63 positive, GATA3 positive, TTF1 negative and NAPSIN-A negative) support the diagnosis

The Equivalent Terms and Definitions sections in the Solid Tumor Rules for these schemas identify which sites must have laterality coded, but there is no instruction for coding laterality when bilateral tumors are a single primary. The SEER Manual currently only indicates code 4 (bilateral) is seldom used (e.g., bilateral ovarian tumors, Wilms tumors, etc.) but does not indicate laterality code 4 should be used for CNS tumors. Is this note going to be updated or should a non-bilateral code be applied?

Example: MRI demonstrates multiple left-sided dural-based meningiomas including a 4.4 cm left posterior fossa meningioma, a 0.8 cm left frontal-parietal meningioma and a right posterior frontal meningioma. The large left posterior fossa meningioma was resected and proved atypical meningioma. Should the laterality be 4 (bilateral) as the patient had both left and right-sided meningiomas confirmed to be a single primary? Or should the laterality be coded as 2 (left) since only the large left-sided meningioma was proven to be a borderline tumor (atypical meningioma, 9539/1) and the others were benign?

The 2018 Solid Tumor (ST) Rules General Rules state: For those sites/histologies which have recognized biomarkers, the biomarkers frequently identify the histologic type. Currently there are clinical trials being conducted to determine whether these biomarkers can be used to identify multiple primaries. Follow the Multiple Primary Rules; do not code multiple primaries based on biomarkers.

Additionally, Biomarkers is at the top of the priority order to identify histology in several sections (it appears to be excluded from only Colon, Melanoma and Other sections). In the sections that include this rule, there is not much additional information on using biomarkers. Can you please provide further explanation for prioritizing biomarkers in the histology coding rules? For example, will the ST manual be updated when we need to look for specific biomarkers in a diagnosis?

The 2021 ICD-O-3.2 Update includes papillary microcarcinoma (8341/3) as the preferred term for thyroid primaries (C739). However, there are multiple SINQ entries instructing registrars not to use code 8341/3 for diagnoses of micropapillary carcinoma of the thyroid (including SINQ 20071076, 20081127, 20110027, 20150023, and 20180008).

SINQ 20150023 specifically indicates: Per the WHO Tumors of Endocrine Organs, for thyroid primaries/cancer only, the term micropapillary does not refer to a specific histologic type. It means that the papillary portion of the tumor is minimal or occult (1 cm or less in diameter) and was found incidentally. WHO does not recognize the code 8341 and classifies papillary microcarcinoma of the thyroid as a variant of papillary thyroid carcinoma and codes histology to 8260. If the primary is thyroid and the pathology states papillary microcarcinoma or micropapillary carcinoma, code 8260 is correct.

Does this clarification apply to cases diagnosed 2021 and later? If WHO feels the term micropapillary still does not refer to a specific histologic type for the thyroid, why is 8341/3 listed as a preferred term for this morphology/site combination? For cases 2021 and later, should a diagnosis of Incidental papillary thyroid microcarcinoma (3 mm) in left lower pole, be coded as 8341/3 per the ICD-O-3.2, or as 8260/3 per clarification in multiple SINQ entries?

This question was prompted from preparing SEER*Educate coding exercises. We will use the answer as a reference in the rationales.

According to the literature, CIC gene rearrangement sarcomas in young patients are soft tissue sarcomas with an aggressive clinical course and may have previously been grouped under the Ewing-like family of tumors or as undifferentiated round cell sarcomas. There is currently no guideline in the solid tumor rules for coding a CIC gene rearrangement sarcoma. However, coding the histology to 8800 (sarcoma, NOS) seems unlikely to capture the more aggressive nature of these tumors. Can a more specific histology be coded?

Following the existing Solid Tumor Rules Histology Rules, it would seem this is a single primary with histology 8045 (Combined small cell carcinoma) because there is no indication there are multiple prostate tumors and Table 2 states combined adenocarcinoma and small cell carcinoma is Combined small cell carcinoma (8045).

Conversely, while not an exact match to this case, SINQ 20190083 implies small cell carcinoma and adenocarcinoma of the prostate are separate primaries. In that SINQ case, the patient was simultaneously diagnosed with metastatic small cell carcinoma of the prostate on a liver biopsy and prostate adenocarcinoma on a prostate biopsy. There is no indication that patient had separate tumors in the prostate, however the SINQ instructs to code as separate primaries.

Would the previous SINQ logic apply to synchronous diagnoses in the prostate as well? Or does code 8045 apply to this situation?

Insulinoma, NOS is reportable for cases diagnosed 2021 and later. However, the diagnosis of insulinoma is most frequently made with clinical correlation of the patient's clinical syndrome and serum hormone levels. Despite a pathological diagnosis of NET, this will clinically be stated as insulinoma based on the functional type of tumor. At the largest facility in our area, all pathology reports with a diagnosis of insulinoma over the last year only provide a pathological Final Diagnosis of NET (either G1 or G2), but elsewhere specify, Functional Type: Pancreatic neuroendocrine tumor, functional. Correlation with Clinical Syndrome and Elevated Serum Levels of Hormone Product: Insulin-producing (Insulinoma).

For 2021 and later, it seems this should be accessioned as insulinoma (8151/3), but one cannot arrive at that histology using the current Other Sites (MP/H) H Rules. Following the existing rules, one would code the histology to NET, G1 or NET, G2 (8240 or 8249) per Rule H6. There are technically two specific histologies to consider: NET (either 8240 or 8249) and insulinoma, NOS (8151). Following the H Rules, Rule H6 instructs one to code the histology with the numerically higher ICD-O-3 code (8240 or 8249).

Coding this histology to NET (8240 or 8249) does not seem to reflect the most accurate classification of this tumor, but applying the current rules, this is the only histology that can be coded. There is no current guideline in the Other Sites schema or the ICD-O-3.2 Implementation Guidelines instructing us to ignore the pathological diagnosis of a NET for these tumors (even though insulinomas are NETs). The only SINQ that currently exists (SINQ 20150019) states the histology can be coded as either a NET or an insulinoma in these cases. How are registrars to consistently code histology for these tumors without a rule clarification?

This question was prompted from preparing SEER*Educate coding exercises. We will use the answer as a reference in the rationales.

11/01/2018, lung, left upper lobe, wedge resection: Invasive moderately differentiated adenocarcinoma, predominantly papillary subtype, with minor acinar and lepidic subtypes. Would this be 8260/3 since the acinar and lepidic subtypes are described as minor or would this be 8255/3 because there is papillary plus two other subtypes/variants described as subtypes?