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2015 Rt kidney nephrectomy pathology states: congenital cellular mesoblastic nephroma, tumor sz 5.9cm, tumor limited to kidney, extension into pelvicalyceal system, margin not applicable, LVI negative. Per PubMed.gov: (In newborns) among the low-grade malignant tumors, congenital mesoblastic nephromas can be successfully treated with simple nephrectomy. Per ScienceDirect: ...currently thought that cellular mesoblastic nephroma is actually a renal variant of infantile fibrosarcoma.

The patient was incidentally diagnosed with cancer on a prophylactic mastectomy, so there are no positive imaging findings to correlate the number of tumors/masses. The final diagnosis was invasive tubular carcinoma, and referred to the CAP Protocol. The CAP notes: However, it does not specify whether the single contiguous focus also includes the in situ component. The CAP goes on to note DCIS was present: The gross description does not provide any indication of either a single or multiple tumors/masses/lesions, though it was referred to as "Lesion 1" in the gross description with no indication of other lesions.

The format of the CAP Protocol frequently does not specify whether the DCIS is a separate measured tumor, or if it is a component of the invasive tumor. This makes it difficult to determine whether the DCIS should be a separate primary when the invasive tumor is not also a type of ductal carcinoma. Per both the 2007 MP/H and 2018 Solid Tumor Rules, an invasive tubular carcinoma and a ductal carcinoma in situ would be multiple primaries if they were multiple tumors. Should we default to Rule M1: Abstract a single primary when it is not possible to determine if there is a single or multiple tumors? Or should we assume these are separate tumors because they were both sized, the focality only described a single invasive tumor, and the tumors are not both ductal carcinomas?

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?

02/18 RUL biopsy: Moderatley differentiated adenocacarcinoma with acinar predominant pattern

04/18 RUL lobectomy: 6.5cm poorly differentiated adenocarcinoma with solid growth pattern and 1.1 cm separate adenocarcinoma with lepidic predominant pattern

Synoptic report:

Procedure: Lobectomy

Specimen Laterality: Right Tumor

Tumor Site: Upper lobe

Histologic Type: Invasive adenocarcinoma, solid predominant

Tumor Size: 6.5 Centimeters (cm)

Tumor Focality: Synchronous primary tumors in same lobe

Lymph Nodes Number of Lymph Nodes Involved: 0

Number of Lymph Nodes Examined: 12

Nodal Stations Examined: 4R: Lower paratracheal; 8R: Para-esophageal (below carina); 10R: Hilar; 7: Subcarinal

Pathologic Stage Classification (pTNM, AJCC 8th Edition)

Primary Tumor (pT): pT3

Regional Lymph Nodes (pN): pN0

Pathology reads: 1. FNA left groin lymph node tissue (smears and cell block): B-cell lymphoma, low grade. The concurrent flow cytometry (3-FC-16-288) identifies a monoclonal B cell population with immunophenotype of CD10++, CD5-, CD23-, CD20++ and unusual CD19-. Overall findings favor follicular lymphoma. FNA Specimen Adequacy: Evaluation for specimen adequacy: Immediate cytology smear review for specimen adequacy was performed at the time of the FNA procedure by pathologist. Smears reviewed from 2 passes in one reading. The specimen was adequate cytological evaluation. Surg Path Final Report Special Studies Immunohistochemistry (CD45, MCK, CD20, CD3, CD10, Bcl6, MUM1 \T\ Ki67) was performed on block 1A to confirm the diagnosis. All controls show appropriate reaction. Lymphoma cells are positive for CD45, CD20, CD10 and weakly positive for bcl6(+) and MUM1(+/-), and negative for MCK. CD3 highlights few T lymphocytes. Ki67 labeling index is low, less than 10%. The immunoprofile supports above diagnosis. Chromosomal study for t(14;18) translocation will be performed, and an addendum report will follow. Flow Final Report Comment: The lymphoma appears to be derived from germinal centre B cells. Together with the findings from the lymph node biopsy (3-FN16-416), follicular lymphoma is favored. However, negative CD19 and CD22 are unusual.

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?

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?

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.

This will be a tough one for registrars to recognize as non-reportable since the terminology contains sarcoma, so we just want to double check.