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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?

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.

CT chest findings: 1. There is a dominant 1 cm. nodule in the left mid lung. 2. In addition, there is a new rather dominant bilobed nodule in the left lung base. 3. Distant metastases are not identified. Four months later, a doctor's note says routine follow-up visit status post Cyber Knife stereotactic body radiation therapy for synchronous early stage non-small cell carcinomas of the left upper and left lower lobes, both Stage IA. He is medically inoperable. This situation is described as a second primary tumor in AJCC8 page 438. However, by the 2018 Lung Solid Tumor rules, this would be a single primary, per rule M7. Is that correct?

Spindled cell lesion of smooth muscle origin (desmin and SMA are positive, CD34, S100, pancytokeratin, Pax8, MDM2 and CDK4 are negative). Many of the cells have hyperchromatic, bizarre-shaped nuclei. Mitotic activity is inconspicuous. There are no areas of necrosis. The overall findings in this biopsy is best classified as a "STUMP"; however, a leiomyosarcoma cannot be excluded.

The patient has a history of prostate adenocarcinoma with lymph node metastases, status post prostatectomy and treatment by Lupron in 2014. The most recent prostate serum antigen measurement (April 2018) was normal. CT scan of the abdomen and pelvis revealed new hypodense liver lesions, a slightly enlarging lung right lower lobe nodule, and enlarging lobular mass in the prostatectomy bed. The core liver biopsy contains areas of metastatic tumor with a differential diagnosis on pathology of high-grade neuroendocrine carcinoma of the prostate (small cell type), which may have been seen in association with prostate adenocarcinoma, or metastatic small cell carcinoma of a different site.

Clinically, the physician impression is that this represents metastatic castration-resistant prostate cancer. The Solid Tumor Rules note that the Multiple Primary Rules are not used for tumor(s) described as metastases. However, SINQ 20130221 indicates that, at least historically, these would have been accessioned as multiple primaries (histology 8140 & 8041 per Rule M10). Does the previous SINQ note still apply to these types of cases, and if so how would one know to move beyond the initial note indicating metastases are not new primaries?

Instruction number 1 under the Coding Multiple Histologies instructions states to code histology when the histology is described as subtype, type or variant. The general rules do indicate we can code the histology identified as type, but when applying the H Rules, it seems an argument could be made for either H1 or H3. H1 applies if you ignore the diagnosis of carcinoma and only code the histologic type: urothelial carcinoma. However, the rules do seem to imply that you take all histologies into account (e.g., code the subtype/variant when there is a not otherwise specified (NOS) and single subtype/variant). Following this logic, Rule H3 seems to be the only rule that fits, and one would code the subtype/variant urothelial carcinoma when the diagnosis is carcinoma NOS, histologic type: urothelial carcinoma.

The problem is that urothelial carcinoma is not a subtype/variant of carcinoma (NOS) per Table 2. The entry for Carcinoma NOS in Table 2 states, Subtypes of carcinoma NOS include adenocarcinoma and all subtypes/variants of adenocarcinoma. To some, urothelial carcinoma is a more specific type of carcinoma; however, urothelial carcinoma is not also listed as a subtype of carcinoma or of adenocarcinoma; only adenocarcinoma is categorized as a subtype of carcinoma. Consistently applying the rules becomes an issue when rules are interpreted in different ways. Should this Table be amended to include urothelial carcinoma as a subtype/variant of carcinoma NOS with the same caveat given to adenocarcinoma in Table 2?

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?