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Pituitary blastoma was not added to Table 3 (Specific Histologies, NOS, and Subtypes/Variants) of the 2018 Malignant CNS and Peripheral Nerves Solid Tumor Rules as part of the December 2020 update. This is a new malignant CNS histology for 2021 and later. Not including this histology in Table 3 results in the registrars being required to check another source to correctly code this histology. If this histology cannot be used for cases diagnosed prior to 2021, should that diagnosis year clarification be included in the STR?

This question was prompted from preparing SEER*Educate coding exercises. We will use the answer as a reference in the rationales.

The patient has a history of a minimally invasive endometrial adenocarcinoma that was low grade and confined to an endometrial polyp in 2001. The patient underwent a total abdominal hysterectomy/bilateral salpingo-oophorectomy (TAH/BSO) that entirely removed the tumor at that time.

Almost 18 years later, the patient had a left inguinal mass excision that was, Carcinoma of gynecologic origin, consistent with clear cell carcinoma. No other disease was found, the physician never indicated whether this was felt to be metastatic from the previous, low grade adenocarcinoma or not. It was only noted as, an unusual malignancy of the left lower quadrant and inguinal region of gynecologic origin. No further information was available in the medical record or from the physician on follow-up.

Although neither the Solid Tumor Rules nor the MPH Rules (still in use for the Other Sites schema) apply to metastasis, given the differences in histology and behavior of these two tumors (i.e., minimally invasive, low grade disease diagnosed in 2001 vs. higher grade, more aggressive tumor in 2019) should the current clear cell carcinoma of GYN origin really be the same primary as the 2001 endometrial adenocarcinoma?

Is this histologic terminology synonymous with 8071/2 Differentiated-type vulvar intraepithelial neoplasia?

Per the 7/20/2018 updates to the 2018 ICD-O-3 Histology list, the reportability flag was changed from N to Y for Differentiated-type vulvar intraepithelial neoplasia as well as Differentiated penile intraepithelial neoplasia, both 8071/2. It appears that both SINQ 20180020 and the second half of SINQ 20160069 are no longer valid and should be deleted.

Patient had a vulvar biopsy with final diagnosis of Extramammary Paget neoplasm (intraepithelial glandular neoplasm). No invasion identified. We are unable to contact the pathologist or physician for clarification.

Although this terminology is not listed in the ICD-O-3, web search results refer to this as a possible synonym for Paget disease with associated VIN III, which is reportable.

There is no specific ICD-O-3 code for a which resulted in abstractors assigning the malignant astrocytoma, NOS code. These lesions were previously called but we are seeing the new terminology more frequently.

10/4/2018 Frozen Section Diagnosis: Brain tissue with atypical cells and inflammatory cells, defer to permanents for further evaluation. Note: Tissue for flow cytometry is submitted. Final Diagnosis: Preliminary Diagnosis: Brain Tumor, Biopsy: Neoplasm, suggestive of lymphoma (see comment). Comment: The tumor exhibits nuclear atypia and increased mitosis. The tumor cells are immunologically positive for LCA and with very high ki67 labeling index. GFAP and synaptophysin are not expressed by tumor cells. The above suggests a lympho-proliferative process. This case is forwarded to the hematopathology service of this department for further evaluation. The final diagnosis report will be issued by the hematopathologist as an addendum.

Supp Rpt Add Addendum Diagnosis: The brain biopsy showed brain tissue large lymphoid cell infiltrate. Additional immunohistochemical stains are performed. The large cells are positive for CD20, BCL2, BCL6 (subset), MUM1, and CD30, negative for CD3, CD5, and CD10. Staining for c-MYC is negative. Ki-67 positive large cells are approximately 18%. EBER is strongly positive by ISH. Diagnosis: Brain lesion, biopsy: EBV+ Diffuse Large B-cell Lymphoma. Addendum Comment: The concurrent flow cytometric study showed monoclonal lambda-positive B-cells without out CD5 and CD10 expression, consistent with B-cell lymphoma.

The Hematopoietic and Lymphoid Neoplasms Database (Heme DB) definition for systemic mastocytosis with an associated hematological neoplasm (SM-AHN, 9741/3) states SM-AHN is a variant of systemic mastocytosis that arises with a myeloid disease of non-mast cell lineage (e.g., MDS, MPN, etc.) and that,

However, SINQ 20130172 conflicts with the Heme DB stating the systemic mastocytosis and the associated hematological neoplasm are a single primary coded to a single histology (9741/3) per Rule M2.

Rule H13 states to code the histology to metaplastic carcinoma when there is metaplastic carcinoma (or a subtype/variant) and invasive carcinoma NST. This rule makes no mention of lobular carcinoma. However, in Table 3, Note 2 for metaplastic carcinoma (8575) states metaplastic carcinoma, NOS and subtypes are almost always mixed with invasive mammary carcinoma, NST and at times lobular carcinoma. These tumors should be coded to metaplastic regardless of percent invasive mammary carcinoma or lobular carcinoma present.

While Table 2 (the mixed histology code table) does include an entry for metaplastic carcinoma AND carcinoma NST OR lobular carcinoma, it is unclear why lobular carcinoma has not been added to Rule H13 as well.

If a single tumor has metaplastic plus lobular carcinoma, Rule H13 does not apply and one has to continue through the rules. Unfortunately, the next rule registrars would be tempted to use is Rule H18: Code the histology that comprises greater than 50% of tumor when two histologies are on different rows in Table 3. This Rule does not state it does NOT apply to metaplastic carcinoma (only mucinous). So, if for some reason the lobular was greater than 50%, the incorrect histology would be coded (unless the registrar happened to remember Note 2 in the metaplastic carcinoma entry in Table 3).

This question was prompted from preparing SEER*Educate coding exercises. We will use the answer as a reference in the rationales.

Histologic Type: HPV-associated multiphenotypic carcinoma. Overall, the morphology, immunohistochemistry, and HPV testing results support the diagnosis of an HPV-related multiphenotypic carcinoma. This entity has been described in the sinonasal region, where it behaves more indolently than its other salivary gland carcinoma counterparts (e.g., adenoid cystic carcinoma), with local recurrence but rare metastases.

Is an M rule needed to address multiple tumors and Note 2 in Table 3? Does Note 2 in Table 3 apply when multiple tumors exist and one tumor contains only ductal carcinoma?

The M Rules currently confirm that a metaplastic carcinoma (whether it is involved with ductal or lobular) and a separate ductal carcinoma are separate primaries because these histologies are on different rows in Table 3 (separate primaries per M14). There is no specific rule regarding metaplastic carcinomas in the Multiple Tumors (M Rules) module, so presumably, the presence of a separate ductal carcinoma is not lumped into Note 2 in Table 3 for metaplastic carcinoma.

However, the note is confusing when there are multiple tumors involved because it appears to the registrars there are two options for coding the histology. To some registrars, the rules indicate it does not matter if the tumor is predominantly ductal carcinoma as long as some percentage of metaplastic carcinoma is present, code histology to metaplastic carcinoma. For other registrars, the presence of solely a ductal carcinoma in a second tumor is a separate primary from the separate metaplastic carcinoma.

The M rules and Note 2 need to clarify this issue to promote consistency.

This question was prompted from preparing SEER*Educate coding exercises. We will use the answer as a reference in the rationales.