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At the central registry, we have accessioned this scenario as three primaries per Multiple Primaries/Histology (MP/H) Rule M10 (diagnosed more than 1 year apart), as the patient was stated to be disease free between each occurrence. However, the diagnosing/treating facility is not reporting these cases due to clinical statements of recurrent disease.

This is an example of a case type identified on casefinding audits conducted by our central registry in which we have learned SEER's expectation of MP/H rule application does not match hospital reporting. Can the 2018 version of the MP/H rules more clearly address how this type of clinically recurrent (multiple times) case should be handled?

For example, pathology states: Right axillary lymph node, excision: Diffuse large B-cell lymphoma (DLBCL) (see note). COMMENT: FISH studies were performed that were negative for BCL-6, c-Myc/IgH, CCND1/IgH and IgH/BCl-2 gene rearrangement, ruling out the most common forms of double-hit lymphoma. Flow cytometry studies demonstrated positivity for CD45, CD20, HLA-Dr, CD19, CD11c, CD22, CD30, CD38, CD79b, and FMC7. Low positivity was seen for CD5. No reactivity was seen for CD10, CD23, CD25, CD103 or CD123.

This specific histology (cribriform-morula variant of papillary thyroid carcinoma) is not found in the ICD-O and is not mentioned in the 2007 MP/H Manual. However, per a web search it appears that this is a distinct type of papillary thyroid carcinoma (http://erc.endocrinology-journals.org/content/24/4/R109.full).

Example: Right lobectomy shows thyroid epithelial neoplasm, pending consultation.

Consultation: Thyroid gland, right lobe: papillary thyroid carcinoma, favor cribriform-morula variant.

Consultation Comment: IHC stains argue against medullary carcinoma. The histologic features of growth patterns and cytologic atypia (with rare grooves and pseudoinclusions) and the immunohistochemical profile support a diagnosis of papillary thyroid carcinoma, favoring the cribriform-morula variant. It is important to note that a significant number of patients with this variant of papillary thyroid carcinoma have been associated with familial adenomatous polyposis syndrome.

This terminology is not specifically included in either alternate names list for myelodysplastic syndrome, NOS (9989/3) or refractory anemia with excess blasts (9983/3).

Example: Bone Marrow Biopsy, Final Diagnosis: Consistent with involvement by myelodysplastic syndrome with excess blasts-2 (MDS EB-2).

SEER Program Coding and Staging Manual 2016 states: Record size in specified order using a. The largest measurement of the primary tumor from physical exam, imaging, or other diagnostic procedures before any form of treatment. See Coding Instructions 7-9 below. b. The largest size from all information available within four months of the date of diagnosis, in the absence of disease progression when no treatment is administered. #7 Priority of imaging/radiographic techniques: Information on size from imaging/radiographic techniques can be used to code clinical size when there is no more specific size information from a biopsy or operative (surgical exploration) report. It should be taken as a lower priority, but over a physical exam.

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?