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Patient diagnosed with multinodular and vacuolating neuronal tumor of the cerebrum. My research shows: Multinodular and vacuolating neuronal tumor of the cerebrum is a recently reported benign, mixed glial neuronal lesion that is included in the 2016 updated World Health Organization classification of brain neoplasms as a unique cytoarchitectural pattern of gangliocytoma. There is no code in ICD-O-3 for it, so do I report it and use 9492/0 or not ?

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.

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?

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.

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?

According to this article, PRRT treatment lutetium Lu 177 dotatate was approved earlier this year by the United States Food and Drug Administration for adult use. PRRT is a nuclear medicine therapy that travels throughout the body looking for a certain receptor within neuroendocrine tumors. These include pancreatic and small neuroendocrine tumors in the gastrointestinal tract. Once absorbed into the tumor, the radioactive material starts to break down tumor cells, killing them. It is the first radioactive drug approved for the targeted treatment of gastroenteropancreatic neuroendocrine tumors.

(http://cinj.org/rutgers-cancer-institute-new-jersey-and-robert-wood-johnson-university-hospital-are-first-state ).