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The patient underwent a gross total resection of the 2005 glioblastoma multiforme (9440/3). The patient was subsequently diagnosed with a 2024 diagnosis of astrocytoma, IDH-mutant, WHO grade 4 (9445/3).

Should Rule M13 apply to the new 2024 diagnosis and a new primary be accessioned because astrocytoma, IDH-mutant, WHO grade 4 is listed on a different row than glioblastoma? It is unclear whether histology 9445 should be classified as being on a different row because it is also listed as a subtype/variant for glioblastoma in Table 3. Table 3 lists histology 9445 as both “Astrocytoma, IDH-mutant, WHO grade 4” and as “Glioblastoma IDH-mutant.”

There was a 3.5 cm invasive adenocarcinoma tumor in the pancreatic head. There were four separate, sized pancreatic neuroendocrine tumors measuring 0.9, 0.7, 0.5 and 0.2 cm in the pancreatic body. There are multiple tumors with distinctly different histologies. However, Table 11 (Pancreas Histologies) does not include any entries for neuroendocrine tumors of the pancreas (e.g., pancreatic NET, WHO grade 1, histology 8240). While it would seem Rule M19 should apply as they’re distinctly different histologies, because PanNETs are not included in Table 11, it is not clear which M Rule applies to these multiple tumors.

If Rule M19 does not apply, we are left with Rule M21 (Abstract a single primary when there are multiple tumors that do not meet any of the above criteria). Are these separate tumors with distinctly different histologies really a single primary? Pancreatic neuroendocrine tumors are not an uncommon histology, is there a reason these were not included in Table 11?

The synoptic report calls this “Invasive adenocarcinoma with secondary Paget disease of anal mucosa and perianal skin.” The tumor size is listed as “2.1 x 1.7 x 0.7 cm, including associated advanced adenoma; size does not include the extent of the associated Paget disease, which extends for at least 2 cm distally.”  Clinically this is called an incidentally discovered Paget’s disease.

It is unclear if this is a collision tumor that should be abstracted as separate primaries, or if this is a single tumor with underlying Paget’s disease (similar to that described in Other Sites Rule H26). If this is a single rectal tumor, there does not appear to be an H rule for this scenario.

Head and Neck Multiple Primary Rule M8 appears to be the first rule that applies to this case and instructs the user to abstract multiple primaries when separate/non-contiguous tumors are on different rows in the appropriate site table (Tables 1-9) in the Equivalent Terms and Definitions. Table 1 (tumors of the nasal cavity) shows Non-keratinizing squamous cell carcinoma and squamous cell carcinoma on different rows making the 2019 case a new primary. Is this correct?

There is one tumor in the left lung that is acinar adenocarcinoma, 8551/3, and two tumors in the right lung, one of which is 8551/3 and a separate one that is mucinous adenocarcinoma 8253/3.

3/21/18- left robotic video assisted thoracoscopy with left lower lobe lobectomy: 2.5 cm adenocarcinoma, acinar predominant, margins negative

11/3/18- right upper lobe lobectomy: invasive mucinous adenocarcinoma, 1.7 cm, invasive adenocarcinoma, acinar predominant, 0.6 cm, margins negative

If you start by comparing the 8551/3 left lung tumor to the 8253/3 right lung tumor, M6 applies and these would be separate primaries (seq 01 and seq 02). How would we handle the third tumor, 8551/3, in the right lung? Seq 01: 3/21/18- left lung primary 8551/3 Seq 02: 11/3/18- right lung primary 8253/3 Is the right lung tumor 8551/3 a third primary, and if so, which M rule applies? I cannot find a rule that seems to fit completely. Rule M6 may apply if you were comparing the right 8551/3 tumor to the seq 02 8253/3 tumor. But how would you know to use the seq 02 histology code 8253/3 or seq 01 histology code 8551/3 for the comparison? I think M9 was designed for situations where you have multiple tumors involving both lungs but they didn't biopsy all of them. Is that correct? If so, then we would be able to bypass M9. Would M11 apply since we already took care of two of the tumors with rule M6? If M11 doesn't apply, it seems like you would get to M14.

The following cases were submitted to the central registry as separate primaries. First case submitted as C772 with histology coded 9591/3 (Lymphoma, NOS). Second case submitted as C162 with histology coded 9699/3 (MALT Lymphoma).

Sequence 01 - 5/2016, Excisional biopsy pancreatic tail lymph node: suspicious for malignant B-cell lymphoma. No treatment recommended or administered.

Sequence 02 - 2/2019, Stomach biopsy: MALT Lymphoma. Unknown if treatment was recommended or administered. Biopsy was only at this facility.

Using the Hematopoietic and Lymphoid Neoplasm Multiple Primaries/Histology rules, Rule M7 makes this a single primary. Note 4 instructs to change the histology of the initial abstract to the more specific histology (9699/3). If this is done, they would be multiple primaries per the exception within Rule M2. Should the histology on sequence 01 be changed to the MALT lymphoma and the cases would be multiple primaries or is this a single primary?

The left thyroid lobectomy/isthmusectomy returned a diagnosis of sclerosing mucoepidermoid carcinoma with eosinophina, 6.5 cm, replacing nearly the entire left lobe of the thyroid.

The patient has a completion thyroidectomy of the right lobe and returned the diagnosis of papillary thyroid carcinoma, 0.5 mm, in maximum dimension.

The endocrinologist describes it as "co-exsisting" and states the tumor is iodine non-avid.

Rule M4 does not seem to apply because Note 1 defines clinically disease free to mean no evidence of recurrence in the same lung on follow-up. Patient had been disease free in the left lung after 09/2014 diagnosis. The 04/2019 diagnosis was in a different lung than the 4/2016 diagnosis.

The next applicable rule is either M11 or M14 depending on how we should compare the new 2019 tumor: to the most recent prior tumor in 2016 or to both prior tumors.

There are four invasive tumors in the right lung:

Large cell undifferentiated carcinoma in the right lower lobe (8012/3, C343);

Adenocarcinoma, acinar-predominant in the right lower lobe (8551/3, C343) that was 0.7 cm in size and limited to the lung;

Mucinous adenocarcinoma in the right upper lobe (8253/3, C341) that was 0.9 cm and limited to the lung;

Adenocarcinoma, NOS also in the right upper lobe (8140/3, C341) that was 1 cm and limited to the lung.

The Lung M Rules confirm the large cell undifferentiated carcinoma is a separate primary from the three adenocarcinoma tumors (Rule M8). The acinar adenocarcinoma and mucinous adenocarcinoma tumors are separate primaries (Rule M6). The adenocarcinoma, NOS tumor is the same primary as both the acinar and mucinous are adenocarcinomas (Rule M7).

How is Primary Site coded for both the acinar and mucinous adenocarcinomas if they represent multiple tumors reported as a single primary (when compared to the adenocarcinoma, NOS tumor)?

Should the adenocarcinoma, NOS tumor also be included when coding EOD Primary Tumor for both the right lower lobe acinar adenocarcinoma and right upper lobe mucinous adenocarcinoma primaries? Further follow-up with the physician is not possible.