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The pathology report reads: Urinary bladder, tumor over right ureteral orifice, biopsy: Urinary bladder mucosa (urothelium) and submucosa (lamina propria), with papillary urothelial neoplasm of low malignant potential (previously known as papillary transitional cell carcinoma, grade 1 of 3), no invasion identified.

Rule PH28 confirms the more specific histologies are ignored if this is truly a low grade B-cell lymphoma (i.e., non-Hodgkin lymphoma, NOS) since both MALT lymphoma and LPL are more specific types of low grade B-cell lymphomas. This leaves only a diagnosis of low grade B-cell lymphoma with plasmacytic differentiation to consider.

SINQ 20130033 states a low grade B-cell lymphoma with plasmacytic differentiation should be coded as 9680/3 (diffuse large B-cell lymphoma (DLBCL)). However, DLBCL is a high grade B-cell lymphoma, not a low grade B-cell lymphoma.

If the pathologist classifies this as a non-specific low grade B-cell lymphoma, and clarifies that this may represent a more specific type of low grade B-cell lymphoma (MALT lymphoma or LPL), should the histology be coded to a high-grade lymphoma (DLBCL) or non-Hodgkin lymphoma, NOS?

Example: Patient has a left cerebral meningioma that is meningothelial meningioma (9531) and two right-sided cerebral meningiomas: one that is transitional meningioma (9537) and the other that is meningioma, transitional and angiomatous, WHO Grade I. If the histology for the mixed tumor is 9534 (angiomatous meningioma), then there are three primaries. If the histology is 9537 (transitional meningioma), then there are two primaries.

Per Table 6, angiomatous meningioma is 9534/0 and transitional meningioma is 9537/0. There is no mixed histology coding rule, or mixed histology meningioma code. There is also no default rule that would instruct registrars to code the numerically higher ICD-O code or to default to a meningioma (NOS) histology code.

There is no statement of mucinous or non-mucinous in this case, only adenocarcinoma in situ and an obsolete term bronchioloalveolar carcinoma (BAC) which used to be code 8250. However 8250 is now lepidic adenocarcinoma, and does not match this diagnosis.

Although the Histology Rules do include a general note indicating that the preferred term for BAC is now mucinous adenocarcinoma 8253, it is not listed as a synonym in Table 3. As a result it is unclear how to apply this statement in accordance with the H rules.

The ICD-O Histology Updates table also includes Bronchiolo-alveolar carcinoma, non-mucinous which seems to suggest that in order to apply histology code 8252 (non-mucinous) or 8253 (mucinous) one must also have a statement of mucinous or non-mucinous.

Example 1: Endometrial biopsy final diagnosis is high-grade serous adenocarcinoma. Should we code this endometrial primary with histology 8441 (serous adenocarcinoma) because C54.X topography code is not listed in the applicable 2018 ICD-O-3 codes Histology Update for the new morphology, or should we apply the new histology code 8461 (high-grade serous carcinoma)?

The NAACCR implementation guideline section 2.3 includes an important reminder that: Many of the new codes, terms, and behaviors listed in this update are site-specific and do not apply to all sites. Applicable C codes will be noted next to the term in bold font.

However, this is followed by the more ambiguous instruction for edits that appear to imply the combination with non-listed sites is possible: These site- and histology-specific combinations will not be added to the Impossible combination edit. However, if a site other than the one listed with the morphology code is assigned, the result will be an edit requiring review. This is Interfield Edit 25.

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?

The physicians do not use the Lugano system as we are instructed to stage chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) as lymphomas. I had always been instructed that this qualifies as "bone marrow involvement," or "diffuse disease," and therefore is a Stage IV. Our experts advise that there is not enough information to code it to bone marrow, but do not elaborate as to whether you can actually code Extent of Disease (EOD), SEER Summary Stage, and AJCC Staging?

The Solid Tumor Rules instruct us not to use differentiation for coding histology unless it is specifically listed in the table. The terminology non-small cell carcinoma with neuroendocrine differentiation is not in lung histology Table 2.

However, SINQ 20150033, prior to Solid Tumor rules, indicates this diagnosis should be coded to 8574 (adenocarcinoma/carcinoma with neuroendocrine differentiation).

This presentation appears to represent distinctly different histologies. However, because the 2018 histology diagnosis is not in the table and the prior SINQ appears to disagree with current instruction, it is not clear how to apply the M rules to this case. The outcome of the histology coding will affect the number of primaries reported in this case.