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In a situation like this with one area of metastatic involvement and an unknown primary, if there is no further information, we advise that the metastasis are "regional" until/unless proven otherwise. With this in mind, code the Mets at Diagnosis fields as 0, including the Mets at Diagnosis--Distant Lymph Node(s). This case should continue to be worked up to identify the primary site. If a primary site is identified later, update the abstract accordingly. In the meantime, use text fields to describe the situation.

Assign code 500 for EOD Primary Tumor for now.

We have confirmed with AJCC that "invasion of" but not "through" the visceral peritoneum maps to a T2 and not T3. Involvement of the visceral peritoneum for Summary Stage is Regional and does not make a distinction between "invasion of" or "invasion through." Any involvement of the visceral peritoneum is regional.

To correct this situation would require a new code, which would derive a T2/RE. That code will be added to the updates for 2023.

Code 500 will derive the appropriate Summary Stage of 2 (Regional). We are aware that this will derive the incorrect T; however, there is no work around at this time that will derive the correct T and Summary Stage, so we are defaulting to deriving the correct Summary Stage.

Assign code 8249/3, neuroendocrine tumor, grade 2 based on the pathologist statement of mammary neuroendocrine tumor grade 2.  According to WHO Classification of Tumors of the Breast, 5th edition, neuroendocrine tumor (NET) is an invasive tumor characterized by low/intermediate grade.

If the histology term is not listed in the Solid Tumor rules, the instructions state to also check ICD-O and updates. Per ICD-O, NET, grade 2 is coded 8249/3. Breast Table 3 will be updated for 2023.

Abstract this case as a single primary and code histology as carcinosarcoma (8980/3). The carcinosarcoma is intermixed with the EIN making this a single primary coded to the invasive histology. EIN is a precursor of endometrial carcinoma in the WHO Classification of Female Genital Tumors, 5th edition. Carcinosarcoma of the uterus is described in the literature as an aggressive variant of endometrial carcinoma characterized by unusual histologic features including discrete malignant epithelial and mesenchymal components (carcinoma and sarcoma).

Assign the neoadjuvant therapy data items as if the patient had neoadjuvant therapy.  Neoadjuvant Therapy data item would be coded either code 1 or 2 depending on whether the chemotherapy was completed or not. In this case, they are a surgical candidate by having the chemotherapy with the plan from the beginning to evaluate the chemotherapy after X cycles to see if surgery can be performed. After the patient is evaluated, update the abstract as needed.

The following cancer cases are reportable unless there is information to the contrary.

–Liver cases with an LI-RADS category LR-4 (reportable since 2021) or LR-5 (reportable since 2016)

–Prostate cases with a PI-RADS category 4 or 5 (reportable since 2017)

The following are not reportable without additional information.

–Breast cases designated BI-RADS 4, 4A, 4B, 4C or BI-RADS 5

–Lung cases designated Lung-RADS 4A," 4B, or 4X

–Liver cases based only on an LI-RADS category of LR-3

–Colon cases with only C-RADS information (C-RADS category C4 is not reportable by itself)

–Head and Neck cases with only NI-RADS information (NI-RADS category 3 is not reportable by itself)

–Ovarian or fallopian tube cases with only O-RADS information (none of the O-RADS categories are reportable without additional information)

–Thyroid cases with only TI-RADS information (none of the TI-RADS categories are reportable without additional information)

Ecchordosis physaliphora is not reportable.

Assign code 3 (Progressive disease (PD)(per managing/treating physician statement) for Neoadjuvant Treatment--Clinical Response and code 7 (Neoadjuvant therapy completed and planned surgical resection not performed) for Neoadjuvant Treatment--Treatment Effect. These are the best choices under the circumstances. Use text fields to record the details.

Assign the number of primaries following the Other Sites Solid Tumor Rules.  Based on sites, laterality and or timing there are 8 primaries.  This is similar to SINQ 20061112 that advised to follow the Multiple Primaries/Histology rules for sebaceous carcinoma.  According to the WHO Classification of Skin Tumors, 5th edition, there is a 30-40% risk of local tumor recurrence, and 20-25% risk of distant metastasis.  In only one instance did a physician refer this as a recurrence in the available notes. 

Beginning with cases diagnosed 1/1/2022, the term "configuration" can be used to code histology for urinary sites only. At the request of the AJCC urinary experts, the instructions were changed to allow configuration to be used to code histology.