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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?

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.

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.

Example: Operative report states, partial simple vulvectomy, anoscopy with normal-appearing clitoris, clitoral prepuce, bilateral labia majora, and labia minora. There is a 1.5 x 1 cm raised, hyperpigmented lesion which appears consistent with VIN 3 on the perineal body, just to the right of midline, and not touching the midline. It goes quite close to the anus but is not touching the anus.

Final diagnosis on resection is, Invasive squamous cell carcinoma arising in a background of high-grade squamous intraepithelial neoplasia (VIN III) with the following features: Location: perineum. Focal invasion arising in setting of 1 cm area of VIN III.

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?

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?