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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?

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?

The SEER Manual states that Marriage is self-reported for the instruction in code 2, but it does not indicate if all other marital statuses are self-reported.

Examples: How is Marital Status reported for the following situations?

1. Patient with multiple tumors in the database, for the first tumor marital status is reported as married (code 2), for the subsequent tumor, marital status is reported as single (code 1).

2. Patient self- reports as single, but also has children.

3. Patient states they are in common law marriage, but our state is not a common law marriage state.

A recent molecular study of PNET tumors at NCI (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5139621) seems to indicate the discovery of four new CNS tumor entities, of which HGNET-BCOR is one. The article suggests that these are not primitive neuroectodermal tumors tumors (PNET), but something different.

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?

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,

This patient was diagnosed in 2016 with renal cell clear cell and was coded to that. In 2018, the patient's liver lesion was resected and pathology revealed epithelioid angiomyolipoma perivascular epithelioid cell tumor (PEComa) (8714/3), a new term as of 2018. This was compared to the kidney slides and it was determined to be metastatic PEComa from the kidney. The physician's note states: The patient had a nephrectomy for a kidney tumor in 2016, excision of cutaneous melanomas, and resection of liver mass in 2018. These three cases were sent in consultation. The diagnosis of cutaneous melanoma was confirmed by a dermatopathologist of our department, (a separate report had been already issued). The kidney tumor is poorly differentiated composed of sheets of discohesive cells with markedly pleomorphic cells with frequent giant and bizarre cells. Most of the cells have abundant eosinophilic to clear cytoplasm. The nuclei are enlarged and pleomorphic. Multinucleated cells are numerous. Some cells have markedly enlarged nucleoli. Multifocal tumor necrosis is noted. Extensive lymphovascular invasion is observed. There are foci at the periphery of the tumor consisting of a proliferation of spindle cells with entrapped adipocytes consistent with minor element of unusual angiomyolipoma (see block A18). The liver tumor has histologic features that are similar to the poorly differentiated component of the kidney tumor.