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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,

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?

Multilocular clear cell renal cell carcinoma is a specifc histologic type listed in the CAP cancer protocol for kidney, but not in the ICD-O-3 and it is not on the list of specific types of renal cell carcinomas in Table 1 of the kidney equivalent terms and definitions in the MP/H manual. There is a malignant multilocular cystic nephroma 8959 in Table 1, but I can't tell if this the same histology as what is stated in this path report.

Example: Right breast core biopsy found ductal carcinoma in situ in the upper outer quadrant. Subsequent resection has a final diagnosis of invasive mucinous carcinoma, grade 1, measuring approximately 7 mm, with close margins. See staging summary. Gross description mentions only the primary tumor with associated marker clip from previous biopsy.

Breast Cancer Staging Summary lists (testing and margins removed for brevity):

Procedure type: Lumpectomy.

Specimen laterality: Right.

Tumor size: 7mm.

Histologic type: Invasive mucinous carcinoma.

Histologic grade (Nottingham histologic score): Grade 1, (score 5/9).

Tumor focality: Single focus.

Lymph-vascular invasion: Not identified.

Treatment effect: No known therapy.

Ductal carcinoma in situ (DCIS): Present.

Architectural pattern: Cribriform.

Nuclear grade: Grade 1.

Necrosis: Not identified.

Calcifications: Not identified.

Estimated size/extent of DCIS: Spanning an area measuring 15mm.

Pathologic stage: pT1b, pNx. (AJCC 8th ed).

Distant metastasis: Not applicable.

Additional findings: Background lobular carcinoma in situ (LCIS), flat epithelial atypia (FEA), and atypical ductal hyperplasia (ADH).

The patient has two, separate, non-contiguous tumors. One tumor is a benign meningioma and the other is a malignant oligodendroglioma.

The original plan was not to treat the asymptomatic meningioma. However, after worsening symptoms, imaging and resection proved a separate left frontal lobe malignant tumor.

Rule M5 is the only M Rule in the Malignant CNS Multiple Primary Rules, Multiple Tumors module that addresses separate non-malignant and malignant tumors. This rule provides only two criteria to follow when a malignant tumor follows a non-malignant tumor. The first criteria (for non-malignant tumor followed by malignant tumor) states:

--Patient had a resection of the non-malignant tumor (not the same tumor) OR

--It is unknown/not documented if the patient had a resection.

This patient did not have a resection of the original, separate, non-malignant tumor, but the treatment plan was known to not include a resection. Should Rule M5 also apply to cases where the patient never had treatment planned for the separate non-malignant tumor?

SEER SINQ 20031051 applies to cases diagnosed before 2007 and advises:  Code histology as 9260/3, Ewing sarcoma. Ewing sarcoma is a specific histology on the continuum of primitive neuroectodermal tumors. Code Ewing sarcoma as it is more specific than PNET, NOS.

For tumors diagnosed 2007 or later, refer to the MP/H rules.

Note 1 states that this histology is treatment-related neuroendocrine prostatic carcinoma demonstrating complete neuroendocrine differentiation or partial neuroendocrine differentiation with adenocarcinoma after androgen-deprivation therapy (ADT). Conversely, Note 2 says to code 8574/3 only when there is no history of previous prostate adenocarcinoma or history of androgen-deprivation therapy.

The WHO Blue Book does confirm this is a treatment-related histology, so it seems we would only use this for an adenocarcinoma with neuroendocrine differentiation (or even possibly a mixed histology tumor with adenocarcinoma and small cell carcinoma components) if the patient had previous treatment.

If this histology is treatment-related, why would we use this code for a patient without a history of prostate adenocarcinoma or androgen-deprivation therapy? Should Note 2 be corrected?

Does this histology apply to a post-treatment diagnosis of mixed adenocarcinoma and small cell carcinoma? If yes, should this clarification be added?

Patient was diagnosed with essential thrombocytosis 9962/1 or 3 in 1998 (depending if ET was reportable in 1998), treated with Hydrea.

11-17-2022 Blood smear: CALR + myeloproliferative neoplasm, Most Consistent with Primary Myelofibrosis 9961/3 (Noted CALR and ASXL1 mutations).

The following abstractor note from 9661/3 is confusing: A diagnosis of "post essential thrombocythemia myelofibrosis" is a progression of essential thrombocythemia and would be the same primary.

Per Mayo consulting pathologist, the final diagnosis on this right lung biopsy is: SMARCA4-deficient malignant neoplasm (see Comment). Comment: Sections show a poorly-differentiated malignant neoplasm without any apparent glandular, squamous, or stromal differentiation. The tumor near totally replaces the underlying lung tissue without recognizable underlying alveolar parenchyma. Immunohistochemical stains performed at Mayo Clinic (Oscar keratin, INSM1, NUT, S100, desmin and BRG1 protein encoded by SMARCA4 gene) demonstrate that the malignant cells are positive for Oscar keratin (rare cells only), synaptophysin (weak/patchy) and p63 (focal) while negative for the remaining antibodies tested. Of note, SMARCA4 stain is negative in the tumor cells. Thus, this tumor can be categorized as a SMARCA4-deficient malignant neoplasm, which is known to be an aggressive malignancy, likely represent a SMARCA4-deficient thoracic sarcoma, a recently described entity. SMARCA4-deficient carcinomas in the lung have been reported to be mostly adenocarcinomas or squamous cell carcinomas, which would not fit for this case. Please refer to a paper published by our group (Sauter JL et al. Mod Pathol 2017;30:1422-32.