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The Histology Coding Instructions give priority to the Final Diagnosis over the CAP protocol. However, when pathology reports are formatted using the CAP protocol, the presence of in situ carcinoma is generally only mentioned in the CAP protocol. Can the presence of in situ tumor mentioned only in the CAP protocol be used to apply rule H7 (Single Tumor: Invasive and In Situ Components Module)? Or are the rules in the Single Tumor: Invasive Only module used?

Example: Final diagnosis is invasive ductal carcinoma. CAP protocol mentions,

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?

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?

Mixed odontogenic tumor consistent with dentinoameloblastoma, 9.5 cm, See Note: Tumor involves maxillary bone including hard palate, alveolar ridges, nasal cavities and maxillary sinuses bilaterally and buccal soft tissue. Lymphovascular invasion not identified. Perineural invasion not identified. Margins: Tumor involves right posterior bone (alveolar) margin. All other margins negative. Note: This is a rare hybrid tumor showing features of ameloblastoma producing pre-dentin/osteodentin matrix. Submucosal tumor is seen in the nasal cavities and palate. A congo red stain shows that the acellular dentin-like matrix fluoresces similar to collagen after polarization. Immunohistochemistry shows that the tumor cells are diffusely and strongly positive for p63, focally positive for CK19, and negative for CK5/6, SOX10, S100 and calretinin.

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?

From a number of definitions reviewed, it seems unclear if it's part of the appendix or the cecum of the colon. For example: The cecum is usually located in the right iliac fossa. In the pole of the cecum, there is often the appearance of fusion of the three teniae coli around the appendix, giving rise to the tri-radiate fold (Mercedes Benz sign), but the anatomy can be variable. The most reliable landmarks of the cecum are the appendiceal orifice and ileocecal valve. The appendiceal orifice is usually an unimpressive slit, often crescentic in shape. The ileocecal valve is made up of the superior and inferior lips (usually not seen en face) and is the gateway leading into the terminal ileum. It is located on the prominent ileocecal fold encircling the cecum, between 3 and 5 cm distal to the cecal pole. (https://www.sciencedirect.com/science/article/pii/S2212097113701730)

Cytology is not listed in the Priority Order for Using Documentation to Identify Histology (Histology Coding Rules) in the Head and Neck schema. Other schemas do include cytology in the hierarchy below tissue from a biopsy or resection. Cytology is often less specific than histology, so one would expect cytology to be listed below tissue in this hierarchy. Was this an oversight? Or would cytology be equivalent to histology if it provided the most specific histology for the case?

The Solid Tumor Rules indicate bladder tumors that are urothelial carcinoma (8120) and small cell carcinoma (8041) are separate primaries per Rule M13 (Abstract multiple primaries when separate/non-contiguous tumors are on different rows in Table 2). These are distinctly different histologies and, presumably, one would want to capture the small cell carcinoma (or small cell carcinoma component) as this has a worse prognosis.

However, if a subsequent bladder tumor is composed of invasive urothelial carcinoma and small cell neuroendocrine carcinoma, the histology is coded as 8045/3 per Rule H4, but this is not abstracted as a multiple primary. The only M Rule that applies is Rule M18 (Abstract a single primary when tumors do not meet any of the above criteria). The mixed histology code 8045 is not included in Table 2, so none of the histology-based M Rules apply. Is the subsequent mixed invasive urothelial and small cell carcinoma tumor (8045/3) the same primary as a previously diagnosed invasive urothelial carcinoma (8120/3) when these tumors are diagnosed within three years?