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Per Rule M8, this is a single primary as there are multiple urothelial tumors as outlined in Table 1 (papillary urothelial carcinoma [8130] and sarcomatoid urothelial carcinoma [8122]) simultaneously present in multiple urinary organs (bladder, ureter and renal pelvis). As Rule M8 indicates these are a single primary, despite the histologies differing at the third digit (8130 vs 8122), then Rule H14 (Code the histology of the most invasive tumor) seems to be the most applicable histology rule. Following Rule H14 (in the Text version of the MP/H Rules), the histology would be coded as 8122 (sarcomatoid urothelial carcinoma) since the renal pelvis tumor was the most invasive tumor present.

However, in both the Matrix and Flowchart versions of the MP/H Rules, Rule H14 contains a note (missing from the Text version) that states that this rule should only be used when the first three numbers of the histology codes are identical (This is a single primary). Rule M8 clearly tells us these are a single primary, despite the differences at the third digit of the histology. Further defaulting to Rule H15 (Code the numerically higher histology code) in this case would ignore the histology of the tumor with the worse prognosis (the most invasive tumor). Was this note included in the Matrix and Flowchart versions in error?

Pathology shows: Right liver lobe, partial hepatectomy " intraductal papillary neoplasm with high grade dysplasia.

The pathologist noted this was a challenging brain neoplasm that did not easily fit into a specific WHO diagnostic classification. Multiple differential diagnoses were given including pilomyxoid astrocytoma, ganglioglioma and dysembryoplastic neuroepithelial tumor (DNET), but there were no definitive features characteristic of any of these tumors. In the Comment section following the Final Diagnosis, it further states: "In summary, the tumor appears to be a difficult to classify non-infiltrating glial/glioneuronal neoplasm without definitive high-grade features."

CT-guided liver biopsy, diagnosis: Metastatic neuroendocrine carcinoma. Diagnosis Comment: Cytology of the tumor appears to be low to intermediate grade.

Would this case be coded as an atypical carcinoid tumor (8249/3) based on SINQ 20170033 and the statement of intermediate grade; or should this be 8240/3 (neuroendocrine tumor) per SINQ 20160023 because it is a metastatic site? More clarification is needed on when to code 8249/3 or 8240/3 for a neuroendocrine carcinoma or neoplasm seen in a metastatic specimen only when there is specified grade.

Patient has an April 2009 biopsy proven diagnosis of cholangiocarcinoma with a single liver mass in segment 4 that was treated with TACE and systemic chemotherapy. The treated lesion was stated to be stable in subsequent scans performed between 2010 and late 2015.

December 2015 imaging identified a new mass in the left hepatic lobe consistent with cholangiocarcinoma. Is the 2015 tumor a new primary?

In auditing files for expected (but not received) abstracts due from facilities, we've observed these types of cases not being consistently reported as multiple primaries.

Rectal mass biopsy final diagnosis: High grade neuroendocrine tumor (WHO Grade 3). Neither SINQ 20170033 nor 20160023 address coding histology or grade for neuroendocrine tumors that are designated as high grade and/or WHO grade 3.

Pathology report microscopic description: The tumor is a meningothelial neoplasm (EMA+; BCL-2 and CD34 negative) with prominent collagen deposition. Necrosis and prominent nucleoli are present; no other atypical features are seen. Mitoses are present, up to 2 per 10 high-powered fields. Final Diagnosis: Dura, bicoronal craniotomy (specimen A): Meningioma with atypical features.

There is no rule in benign brain and CNS section of Multiple Primary/Histology (MP/H) Rules stating to code the most specific histologic term when the diagnosis is (something less specific, i.e., adenocarcinoma). This rule is in other site chapters of MP/H but appears missing in the benign brain and CNS section.