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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?

Example: Operative report states, partial simple vulvectomy, anoscopy with normal-appearing clitoris, clitoral prepuce, bilateral labia majora, and labia minora. There is a 1.5 x 1 cm raised, hyperpigmented lesion which appears consistent with VIN 3 on the perineal body, just to the right of midline, and not touching the midline. It goes quite close to the anus but is not touching the anus.

Final diagnosis on resection is, Invasive squamous cell carcinoma arising in a background of high-grade squamous intraepithelial neoplasia (VIN III) with the following features: Location: perineum. Focal invasion arising in setting of 1 cm area of VIN III.

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.

A recent molecular study of PNET tumors at NCI (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5139621) seems to indicate the discovery of four new CNS tumor entities, of which HGNET-BCOR is one. The article suggests that these are not primitive neuroectodermal tumors tumors (PNET), but something different.

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.

Example 1: Physician states patient has an almost certain melanoma. Due to the patient's age, there is no plan to for any treatment or further workup. Almost certain is not listed as a reportable phrase, so typically we would not accession this case.

Example 2: Imaging states a diagnosis of renal cell carcinoma until proven otherwise. No additional workup is available at this facility. This terminology is also not seen on the ambiguous reportable terminology list but we are seeing it more often and wanted confirmation.

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.

According to this article, PRRT treatment lutetium Lu 177 dotatate was approved earlier this year by the United States Food and Drug Administration for adult use. PRRT is a nuclear medicine therapy that travels throughout the body looking for a certain receptor within neuroendocrine tumors. These include pancreatic and small neuroendocrine tumors in the gastrointestinal tract. Once absorbed into the tumor, the radioactive material starts to break down tumor cells, killing them. It is the first radioactive drug approved for the targeted treatment of gastroenteropancreatic neuroendocrine tumors.

(http://cinj.org/rutgers-cancer-institute-new-jersey-and-robert-wood-johnson-university-hospital-are-first-state ).

Immunostain results of the malignant cells show strong staining with p63 and negative staining with TTF-1 and Napsin. Rare cells stain with CK7. Findings are most compatible with squamous cell carcinoma. The patient is treated as if he has squamous cell carcinoma. The new histology coding rules say you cannot use ambiguous terms which modify the histology to code the histology. So is this 8010/3?