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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?

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?

The patient has one tumor each in the left parietal and left medial temporal lobe. The tumors were excised. The final diagnosis for the left parietal tumor is glioblastoma, IDH-wild type. he final diagnosis of the left medial temporal tumor is, at least anaplastic astrocytoma, WHO grade III; see comment. The comment states: There is a single focus of vascular hyperplasia, separate from neoplastic cells. No necrosis is identified. These findings on their own would warrant a diagnosis of anaplastic astrocytoma, WHO grade III. However, in the context of the patient's glioblastoma in the left parietal lobe, and imaging showing ring-enhancing lesions of the parietal and temporal lobes, this specimen is favored to be an un-sampled glioblastoma, WHO grade IV. The Solid Tumor Rules indicate we may no longer use terms like favor(s) to code the histology, leaving the final diagnosis as the priority source for coding histology per the Histology coding rules.

The tumor board review confirmed that, despite the anaplastic astrocytoma on pathology, they felt strongly that this is a multifocal glioblastoma and not an anaplastic astrocytoma. Both the pathologist's comment and the tumor board's assessment indicate this patient does not have two primaries. However, the Solid Tumor Rules do not give priority to the tumor board's assessment over the pathology, and registrars are not to use ambiguous terms to code histology thus leaving the two histologies to consider. Per the Solid Tumor Rules, one tumor that is glioblastoma and one tumor that is anaplastic astrocytoma are multiple primaries per M11 (Abstract multiple primaries when separate, non-contiguous tumors are on different rows in Table 3 in the Equivalent Terms and Definitions. Timing is irrelevant).

As a central registry, we cannot ask the pathologist or attending physician for clarification as suggested in Section 3 of the Malignant CNS and Peripheral Nerves Equivalent Terms and Definitions. We can only follow the current Solid Tumor Rules. In doing so, we would have to ignore both the pathologist's and tumor board's assessment that this patient has multifocal glioblastoma. Is there any concern that this will lead to over-reporting?

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?

Is Rule H8 a duplicate of Rule H7? Rule H7 instructs one to use Table 2 when there are multiple histologies and the combination is listed in Table 2 (or a combination code was received from Ask a SEER Registrar). Rule H8 states to code adenocarcinoma with mixed subtypes (8255) when there are multiple adenocarcinoma subtypes OR any combination of histologies which are not listed in Table 2. However, both conditions for Rule H8 are already included in Table 2 (the last row). How would one ever move past Rule H7 if all the conditions for both Rules H7 and H8 are covered first under Rule H7?

Example: A resection pathology report proves invasive adenocarcinoma, acinar, solid and papillary types. Rule H7 seems to be the first H Rule that applies as there are multiple histologies (identified using a reportable term: type) AND the combination is listed in Table 2. The last row of Table 2 instructs one to code Adenocarcinoma with mixed subtypes (8255) when there are at least two of the subtypes/variants of adenocarcinoma listed in Column 1 (Required Terms). In this case, there were three subtypes/variants that are listed in Column 1 (acinar, solid and papillary). However, Rule H8 also instructs one to, Which rule applies here, Rule H7 or Rule H8?

It appears an argument could be made for both NLPHL (9659/3) and THRLBCL (9688/3). We favor coding NLPHL (9659/3) because the pathologist did specifically call this a Hodgkin lymphoma, and also specified that it only has a T-cell/histiocyte-rich large B-cell lymphoma-like pattern.

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.

Similar to an issue previously submitted SINQ 20180063, Table 1 (WHO Grades of Select CNS Neoplasms) in the Non-Malignant CNS Equivalent Terms and Definitions section states WHO Grade I tumors are always non-malignant. However, this does not mean that the tumors listed in Table 1 as WHO Grade I are always benign (/0). Some tumors listed with a WHO Grade I have a behavior of /1 (borderline) per the ICD-O-3 and/or Tables 5 and 6. The Behavior coding instructions do not currently indicate these are the appropriate sources to use when the pathologist and/or physician do not comment on the behavior of these tumors. In our area, pathologists do not explicitly state the behavior for these tumors; the pathologist only assigns the WHO Grade.

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?