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The article I read (https://www.ncbi.nlm.nih.gov/pubmed/?term=28984674) makes the distinction between mesophrenic adenocarcinoma and mesophrenic-like adenocarcinoma. The authors propose the term mesonephric-like Mullerian adenocarcinoma. So would this be coded as Mullerian adenocarcinoma?

Patient had a breast excision that proved a single tumor with no evidence of invasive carcinoma. The final diagnosis stated: Size (extent) of EPC DCIS: Spanning approximately 1.3 cm. The pathologist did not describe separate foci of DCIS; only one tumor comprised of both encapsulated papillary carcinoma and DCIS. The encapsulated papillary carcinoma was not described as invasive. The pathology noted: This case is best classified as EPC conventional DCIS. No conventional stromal invasion is identified. Solid Tumor Rule M2 confirms a single tumor is a single primary.

However, there does not appear to be an H Rule that instructs how to code histology. The Single Tumor: In Situ Only module, has only three H Rules and none of them apply to this case. The patient does not have Paget disease (H1), does not have a single histology (H2, there are multiple histologies present as DCIS and EPC are listed on different rows in Table 3) and does not have DCIS and LCIS (H3). How does one arrive at the correct histology for this case?

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?

We noticed this term was added to the most recent version of the Heme Database (DB) as an alternate name for chronic lymphocytic leukemia/small lymphocytic lymphoma; however we do not recall being notified that this was a new reportable term for code 9823 and the term was not included in the 2018 ICD-O-3 Histology updates. The Definition in the Heme DB for Chronic lymphocytic leukemia/small lymphocytic leukemia (CLL/SLL) includes information that the term was added in the 2016 WHO revision, thus would be reportable back to 2016, is that correct? In addition, the Definition seems to be describing it as a precursor condition to CLL and may never actually evolve into CLL, so it is unclear if this term should really be reportable.

Example: 09/08/2016 Onc Note: A/P: monoclonal B-cell lymphocytosis of undetermined significance (MBL): I reviewed with him the results of the bone marrow biopsy. Interestingly, there is no evidence of abnormal plasma cell population by flow cytometry and immunohistochemistry. Nevertheless, flow cytometry does demonstrate a very small population of abnormal and monoclonal B-cell lymphocyte population with immunophenotype consistent with CLL/SLL. Given the very low number of the abnormal B cells, this can be categorized as monoclonal B-cell lymphocytosis (MBL). I recommend surveillance visit in one year.

9/12/2017 Onc note: A/P: Monoclonal B-cell lymphocytosis of undetermined significance (MBL) and IgM MGUS. No symptoms concerning for active disease or progression. Explained that MBL is a very indolent process. Patients with CLL-phenotype MBL progress to CLL at a rate of ~1-2 percent per year. Follow-up in 1 year. Is this case reportable?

Glioblastoma multiforme is listed as a synonym for the preferred term glioblastoma, NOS (9440) per Table 3 Column 2. Therefore, it seems reasonable to assume that a diagnosis of glioblastoma, NOS would be a new primary if it followed a glial or astrocytic tumor. However, in general, the Solid Tumor Rules use the preferred terminology and/or indicate when a specific rule also includes any tumor diagnosed as a subtype/variant. Rule M6 does not explicitly include a diagnosis of glioblastoma, NOS or any of its subtypes/variants (e.g., glioblastoma IDH-mutant or gliosarcoma). Does Rule M6 apply to any diagnosis of glioblastoma, NOS and any of its synonyms or subtypes/variants?

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?

Example: Initial CT scan impression is large appendiceal mucocele with a moderate amount of right-sided abdominal ascites. Faint mural enhancement suggesting an underlying appendiceal neoplasm (mucinous adenoma or adenocarcinoma).

Appendectomy follows two days later with final diagnosis of high-grade appendiceal mucinous neoplasm, see comment. Histologic grade: Grade G2 of 4 (based on the CAP protocol) . . . Ascites fluid (ThinPrep(r) and cell block preparations): Mucin, fragments of debris, and macrophages. No diagnostic neoplastic cells are identified . . . Pathologic stage: pT4a, pNX, pM1a (AJCC 8th ed).

Diagnosis Comment states, We feel that there are areas of this tumor where the cytologic atypia is beyond what one would expect in low-grade appendiceal mucinous neoplasm. While mitotic figures are not strikingly increased, there are focal nuclear changes that would support classification of this tumor as high-grade appendiceal mucinous neoplasm.

Approximately two weeks later the patient has an Oncology assessment stating new diagnosis of T4a, NX, M1a, Stage IVA high-grade mucinous adenocarcinoma of the appendix with mucinous ascites. Patient has had an appendectomy but no further surgery so far. However, anecdotally, the best reported case series has been with surgical debulking followed by HIPEC chemotherapy In that instance I have recommended surgery with intraperitoneal chemotherapy.

Is this a reportable malignancy? If so, what is the best histology for the diagnosis?