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Patient had a total abdominal hysterectomy-bilateral salpingo-oophorectomy performed in January 2023 with final diagnosis of myxoid and epithelioid leiomyosarcoma.

Diagnosis comment states: The tumor is 15 cm per report. It grows in nests and poorly formed interanastomosing trabeculae and cords that are separated by abundant myxoid background. The cells have an epithelioid morphology with eosinophilic cytoplasm, large nuclei, and very prominent nucleoli. The mitotic activity is overall low ranging from 1 to 3/10 HPFs.

Immunohistochemical stains performed at the outside hospital showed diffuse positivity for SMA, desmin, caldesmon, and PR. They are negative for CD10, claudin-4, calretinin, HBM45, MART1 (rare weakly positive cells), PANCK, and SOX10. This immunohistochemical profile supports a smooth muscle derivation of this neoplasm. As this tumor is extensively myxoid, diagnostic criteria differ from the spindle cell leiomyosarcoma.

Per Solid Tumor Rules Other Sites, Table 16: Uterine Corpus Histologies, Epithelioid Leiomyosarcoma (8891/3) and Myxoid Leiomyosarcoma (8896/3) are both subtypes of Sarcoma, NOS (8800/3).  Per Rule H21, use a combination code when there are multiple specific histologies AND the combination is listed in Table 2 OR there are coding instructions for the combination in the applicable histology Tables 3-21 OR you receive a combination code from Ask A SEER Registrar.  Since there is no combination listed in Table 2 and there is no instruction for the combination in Table 16, how should the histology be coded for this tumor?

March 2022, left shoulder soft tissue mass excision shows a spindle cell tumor with outside consultation diagnosis of malignant neoplasm with neuroectodermal differentiation and TPR-NTRK1 gene rearrangement. Diagnosis comments indicate the findings most closely resemble the spectrum of kinase-rearranged mesenchymal neoplasms, such as lipofibromatosis-like neural tumor. However, the expression of SOX10 and mature melanocytic markers is unusual, and does not exclude melanocytic differentiation.

Should this be classified as a peripheral neuroectodermal tumor (9364) or as an "NTRK-rearranged spindle cell neoplasm (emerging)" (8990) if there is a NTRK gene rearrangement?

Can you provide clarification on Rectum primary surgical code 40 Pull through WITH sphincter preservation (colo-anal anastomosis)? Would this be code 30 or 40 due to the colorectal anastomosis? 

Example:  A prostate core biopsy showed intraductal carcinoma in 09/2022, which is an in situ tumor.  A core biopsy again showed intraductal carcinoma in 12/2022.  The subsequent radical prostatectomy in 04/2023, revealed multiple foci of invasive prostate adenocarcinoma with extensive intraductal carcinoma. 

Per Solid Tumor Rules, Other Sites, Rule M3, acinar adenocarcinoma of the prostate is always a single primary.  Note 4, this rule applies to subtype variants of acinar adenocarcinoma listed in Table 3, which has intraductal/ductal as a variant subtype of acinar adenocarcinoma. Does rule M3 apply to incidence cases (an invasive tumor following an in situ tumor)?

While SEER*Rx does indicate infliximab should be coded as biological response modifier (BRM)/Immunotherapy, the manufacturer website for this medication indicates it is given for: Crohn’s disease, ulcerative colitis, rheumatoid arthritis, psoriatic arthritis, ankylosing spondylitis, and plaque psoriasis. In addition, SEER*Rx does not indicate which primary sites this treatment may be given for. If it is indeed cancer directed treatment, can the typical primary sites be added for clarity?

Case example: Patient is diagnosed with colorectal cancer and also has an existing diagnosis of Crohn’s disease; received surgery and FOLFOX6, as well as infliximab. There was no statement of what disease the infliximab was given to treat.  

The AJCC 8th edition confirms that the identification of psammomatous calcifications within a cervical lymph node is metastatic disease.

Example: Patient had a thyroid lobectomy and level VI neck node excision in August 2022. The final diagnosis is multifocal papillary carcinoma of the thyroid, as well as rare psammomatous calcifications only in the resected node. The pathologist notes that “psammoma bodies only” in lymph nodes is not well defined, and while indolent, they do indicate capacity for lymphatic spread and are pN1a.

Should thyroid primaries with cervical node psammomatous calcifications get captured in EOD Regional Nodes category as it is in the AJCC pN staging?

Since the peripheral nerves are included in the Malignant CNS schema of the Solid Tumor Rules, neither the differences in subsite nor timing indicate these are separate primaries (Rule M10 indicates a single primary). However, these are separate MPNSTs in different sites and the tumors are not stated to be metastasis. Additionally, these are treated as separate primaries by the managing physician.

While the malignant CNS tumors do not take timing into account, is this correct for these peripheral nerve tumors that are often treated similarly to soft tissue tumors? Should Rule M8 be updated to include tumors in different peripheral nerve subsites?

The resection pathology report final diagnosis indicates this is both mixed mucinous and non-mucinous with a lepidic predominant component. The pathologist notes this is “Lepidic: 75%. Acinar: 25%.”  The percentage of the mucinous component is not documented.

Rule H1, Note 1, states “When mucinous carcinoma is mixed with another histology, such as adenocarcinoma and mucinous carcinoma, code mucinous ONLY when mucinous is documented to be greater than 50% of the tumor.” While mixed invasive mucinous and non-mucinous carcinoma is included in Table 2 (Combination/Mixed Histology Codes) without a required percentage, it is unclear whether one should move past Rule H7 and use Rule H8 to code this combination histology code. Rule H7 would instruct one to code the histology to lepidic adenocarcinoma (adenocarcinoma, lepidic predominant) based on the percentage of the lepidic component in the tumor. However, this does not address the mixed mucinous and non-mucinous diagnosis. Which H Rule and histology apply to this case?

Patient has a 3 cm tumor in the temporal lobe of the brain. This was noted on MRI 12/2022. The radiologist states this is a low-grade glioma and recommends following with routine scans. No pathology or resection performed or planned. Patient has been followed with imaging every six months with stable disease. Low grade glioma is not currently listed in ICD-O-3.2 or the current Solid Tumor Rules. What histology should be assigned to the case?

In the examples below, are these a single or multiple primaries?

Example 1:

Tumor 1:  C509/left breast, 8520/2 (in situ lobular carcinoma), dx date-01/10/2019

Tumor 2:  C509/ left breast, 8500/3 (carcinoma NST), dx date-08/19/2021

Example 2:

Tumor 1:  C509, right breast, 8520/2, dx date 06/26/2014

Tumor 2:  C508, right breast, 8500/3, dx date-05/23/2019

There seems to be some conflicting info on this. In the 2020 Breast Rules there was a note add to the revision history. “M10 Same behavior requirement re-added.” Which is not in the rules now, nor was it noted to the revision changes in the last two change logs.

Inquiry 20200070 would seem to indicate that this is multiple primaries, but that contrasts with 20230010 which would seem to indicate a single primary, and an ASK A SEER Registrar question that we received a response to. I don’t see a scenario where rule M17, an invasive tumor DX more than 60 days after an in situ tumor would come into play.

If behavior no longer applies to rule M10, at what point did that change get made? Please advise.