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This patient was diagnosed in 2016 with renal cell clear cell and was coded to that. In 2018, the patient's liver lesion was resected and pathology revealed epithelioid angiomyolipoma perivascular epithelioid cell tumor (PEComa) (8714/3), a new term as of 2018. This was compared to the kidney slides and it was determined to be metastatic PEComa from the kidney. The physician's note states: The patient had a nephrectomy for a kidney tumor in 2016, excision of cutaneous melanomas, and resection of liver mass in 2018. These three cases were sent in consultation. The diagnosis of cutaneous melanoma was confirmed by a dermatopathologist of our department, (a separate report had been already issued). The kidney tumor is poorly differentiated composed of sheets of discohesive cells with markedly pleomorphic cells with frequent giant and bizarre cells. Most of the cells have abundant eosinophilic to clear cytoplasm. The nuclei are enlarged and pleomorphic. Multinucleated cells are numerous. Some cells have markedly enlarged nucleoli. Multifocal tumor necrosis is noted. Extensive lymphovascular invasion is observed. There are foci at the periphery of the tumor consisting of a proliferation of spindle cells with entrapped adipocytes consistent with minor element of unusual angiomyolipoma (see block A18). The liver tumor has histologic features that are similar to the poorly differentiated component of the kidney tumor.

Procedure: Wedge, resection specimen, Laterality: Right, Tumor site: Right upper lobe, Tumor size: 1.0 cm in greatest dimension, Histologic type: Adenocarcinoma in-situ, mucinous, Histologic grade: N/A, Visceral pleura invasion: Not identified, Tumor extension: N/A, Margins: Uninvolved, Lymphocytosis.

The 2018 Solid Tumor (ST) Rules General Rules state: For those sites/histologies which have recognized biomarkers, the biomarkers frequently identify the histologic type. Currently there are clinical trials being conducted to determine whether these biomarkers can be used to identify multiple primaries. Follow the Multiple Primary Rules; do not code multiple primaries based on biomarkers.

Additionally, Biomarkers is at the top of the priority order to identify histology in several sections (it appears to be excluded from only Colon, Melanoma and Other sections). In the sections that include this rule, there is not much additional information on using biomarkers. Can you please provide further explanation for prioritizing biomarkers in the histology coding rules? For example, will the ST manual be updated when we need to look for specific biomarkers in a diagnosis?

5/15/17 Duodenal bulb, biopsy: Fragments of duodenal mucosa with well differentiated neuroendocrine tumor (carcinoid), extending to the edge of specimen and peptic duodenitis in the submitted tissue. No significant intraepithelial lymphocytosis.

Cytology is not listed in the Priority Order for Using Documentation to Identify Histology (Histology Coding Rules) in the Head and Neck schema. Other schemas do include cytology in the hierarchy below tissue from a biopsy or resection. Cytology is often less specific than histology, so one would expect cytology to be listed below tissue in this hierarchy. Was this an oversight? Or would cytology be equivalent to histology if it provided the most specific histology for the case?

The Solid Tumor Rules indicate bladder tumors that are urothelial carcinoma (8120) and small cell carcinoma (8041) are separate primaries per Rule M13 (Abstract multiple primaries when separate/non-contiguous tumors are on different rows in Table 2). These are distinctly different histologies and, presumably, one would want to capture the small cell carcinoma (or small cell carcinoma component) as this has a worse prognosis.

However, if a subsequent bladder tumor is composed of invasive urothelial carcinoma and small cell neuroendocrine carcinoma, the histology is coded as 8045/3 per Rule H4, but this is not abstracted as a multiple primary. The only M Rule that applies is Rule M18 (Abstract a single primary when tumors do not meet any of the above criteria). The mixed histology code 8045 is not included in Table 2, so none of the histology-based M Rules apply. Is the subsequent mixed invasive urothelial and small cell carcinoma tumor (8045/3) the same primary as a previously diagnosed invasive urothelial carcinoma (8120/3) when these tumors are diagnosed within three years?