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Table 1 of the 2018 Kidney Solid Tumor Rules (STR) lists succinate dehydrogenase-deficient renal cell carcinoma as histology code 8312, but in the ICD-O-3.2 Coding Table it is listed as histology code 8311.

No changes were made in the Kidney STR. As a result, the histology change described in the ICD-O-3.2 Coding Table conflicts with Table 1. Succinate dehydrogenase-deficient renal cell carcinoma (SDHD) is listed in Table 1 as a synonym for renal cell carcinoma, NOS (8312). However, the ICD-O-3.2 Coding Table lists this as a related term for histology code 8311/3. This related term was not discussed in the Implementation Guidelines, and no change was noted in the STR.

While it seems we should continue to follow the STR, without clarification as to why this histology change was not implemented in STR, achieving consistency will be problematic if registrars jump straight to the ICD-O-3.2 Coding Table to code histology for cases diagnosed 2021 and later. If this code cannot be used for cases diagnosed prior to 2021, should that 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.

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.

The ICD-O-3.2 Implementation Guidelines and ICD-O-3.2 Coding Table indicate that insulinoma, NOS has changed behavior from /0 to /3 for cases diagnosed 2021 and later. However, the ICD-O-3.2 Implementation Guidelines do not indicate whether this change applies to tumors described as above. Insulinomas are generally neuroendocrine tumors/neoplasms, so it seems any neuroendocrine tumor described as an insulinoma should be collected as 8151/3, but does that apply to an epithelioid tumor/neoplasm also described as insulinoma?

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

Path report reads: 7/15/2019 A. Endometrium, curettings: Serous carcinoma, high grade. B. Endometrial polyp, curettings: Serous carcinoma, high grade.

If coded to 8461/3, according to AJCC, this would not be an ideal code (since it is outdated). Also, endometrium is not included in the suggested site codes for 8461/3 according to the 8/22/2018 ICD-O-3 update.

Following the existing Solid Tumor Rules Histology Rules, it would seem this is a single primary with histology 8045 (Combined small cell carcinoma) because there is no indication there are multiple prostate tumors and Table 2 states combined adenocarcinoma and small cell carcinoma is Combined small cell carcinoma (8045).

Conversely, while not an exact match to this case, SINQ 20190083 implies small cell carcinoma and adenocarcinoma of the prostate are separate primaries. In that SINQ case, the patient was simultaneously diagnosed with metastatic small cell carcinoma of the prostate on a liver biopsy and prostate adenocarcinoma on a prostate biopsy. There is no indication that patient had separate tumors in the prostate, however the SINQ instructs to code as separate primaries.

Would the previous SINQ logic apply to synchronous diagnoses in the prostate as well? Or does code 8045 apply to this situation?

Recognizing the addition of the behavior requirement into this rule is an attempt to stop non-synchronous ductal carcinoma and lobular carcinomas from being accessioned as a single primary (SINQ 20200022), the issue with using behavior rather than timing is that now, synchronous separate/non-contiguous tumors that are invasive carcinoma NST (8500/3) and lobular carcinoma in situ (8520/2) (or vice versa) are separate primaries per M14.

Lobular and carcinoma, NST are separate rows in Table 3, so we cannot stop at M10 and code the mixed histology because there are two separate histologies with different behaviors. There is no rule that states we can just ignore the in situ tumors for the purpose of applying the M Rules. (We are instructed to ignore the in situ when coding histology only in certain circumstances.) The problem with Rule M10 appears to be related to timing.

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

According to the literature, PMH is a low-grade malignant vascular neoplasm of different tissue planes including skin and soft tissue. However, the references also state: PMH is a cutaneous tumor that behaves in an indolent fashion. There is no indication that this was a malignant diagnosis.

12/3/18 Foot, left skin lesion, punch biopsy: Superficial squamous epithelium demonstrating hyperkeratosis and fragments of keratin debris, no tumor seen.

Foot, left skin lesion, punch biopsy: Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma, see note.

NOTE: The submitted immunohistochemical slides were reviewed. Positive and negative controls reacted appropriately. The tumor cells demonstrate immunoreactivity to CK AE1/AE3 and CK7. The CD31 immunoreactivity described in the report cannot be confirmed as only the positive control is submitted for review. The tumor cells are negative for desmin, CD45, CD68, S-100, CD34, SMA, CD20, and HHV8. The proliferative index via Ki-67 is approximately 10%. The morphology (described below) and immunohistochemistry performed are compatible with a pseudomyogenic hemangioendothelioma.

12/4/18 Final Pathologic Diagnosis: Foot, left bone lesion, biopsy: Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma, see note.

Note: The patient's imaging findings were reviewed in conjunction with this case, revealing numerous lytic lesions of the tibia, fibula, talus, tarsal, metatarsal, and phalangeal bones. Additionally, as per the medical record, also reviewed in conjunction with this case, there are lesions of the skin. Thus, an extensive immunohistochemical panel was performed in an attempt to support the morphologic findings in this case, which were morphologically similar to the patient's skin biopsy. The tumor cells demonstrate strong immunoreactivity to pancytokeratin (CK AE1/AE3) and vimentin with moderate immunoreactivity to Fli-1. The tumor cells demonstrate weak immunoreactivity to epithelial membrane antigen. INI-1 is retained. There is focal immunoreactivity to CD31 although this is limited to the edges of the tissue fragments. The tumor cells are negative for HHV-8, CD34, smooth muscle actin, CK8/18, desmin, CD99, and Bcl-2. The combination of morphologic (see below for microscopic description) and immunohistochemical findings are consistent with pseudomyogenic hemangioendothelioma. Fresh tissue was submitted for karyotype analysis at the time of intraoperative consultation; however, it revealed only a normal appearing male karyotype. Thus, molecular confirmation was sought. The original slides and a paraffin block were submitted for FOSB rearrangement analysis, as pseudomyogenic hemangioendothelioma is known to have recurrent rearrangements with FOSB. Additional immunohistochemistry performed at (FACILITY) demonstrating immunoreactivity for ERG, supporting a vascular origin for this neoplasm. Fluorescence in situ hybridization demonstrated that 13% of the cells examined show FOSB rearrangement. While this FISH probe is for investigational purposes, the above findings support the diagnosis of pseudomyogenic hemangioendothelioma.

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.

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.