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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.

The article I read (https://www.ncbi.nlm.nih.gov/pubmed/?term=28984674) makes the distinction between mesophrenic adenocarcinoma and mesophrenic-like adenocarcinoma. The authors propose the term mesonephric-like Mullerian adenocarcinoma. So would this be coded as Mullerian adenocarcinoma?

10/27/2017 partial nephrectomy final path diagnosis: renal oncocytic neoplasm, favor hybrid oncocytic tumor. Comment:

Table 6 (Tumors of the Oropharynx, Base of Tongue, Tonsils, Adenoids) in the Head and Neck Equivalent Terms and Definitions lists both squamous cell carcinoma HPV-positive and squamous cell carcinoma HPV-negative as subtypes/variants of squamous cell carcinoma (the NOS histology, 8070). Squamous cell carcinoma HPV-positive and squamous cell carcinoma HPV-negative are also listed in the 2018 ICD-O-3 update table.

Previous clarification from the standard setters regarding the 2018 ICD-O-3 Update table indicated that histology codes 8085 and 8086 (HPV-positive and HPV-negative squamous cell carcinoma, respectively) included p16+ and p16- squamous cell carcinoma, respectively. Presumably, this clarification was made because p16 is a surrogate marker for HPV, and capturing whether a tumor is HPV-related or not has implications for staging for 2018 and later diagnoses. However, this clarification was not added to the 2018 ICD-O-3 Update table via errata, nor do the Head and Neck Equivalent Terms and Definitions or Histology Coding Rules address this.

Is a diagnosis of p16-positive squamous cell carcinoma equivalent to a diagnosis of squamous cell carcinoma HPV-positive (8085)? If so, will this clarification be added to the Head and Neck Solid Tumor Rules?

Pathology--Right breast, lumpectomy with needle localization: Encapsulated papillary carcinoma of the breast. A separate focus of ductal carcinoma in situ is present. Sentinel lymph node, right breast, biopsy: One lymph node, negative for malignancy. No metastatic carcinoma is seen on slides stained with immunostain for cytokeratin (AE1/AE3). Specimen laterality: Right. Tumor size: 1.2 cm. Histologic type: Encapsulated papillary carcinoma. Nuclear grade: Grade 1 (low). Mitotic rate: Score 1. Ductal carcinoma in situ (DCIS): DCIS is present. Estimated size (extent) of DCIS: 3 mm. Architectural patterns: Cribriform and papillary. Nuclear grade: grade 1 (low). Necrosis: Not identified. Margins: Margins uninvolved by encapsulated papillary carcinoma. Distance from closest margin: 8 mm, superior Margins uninvolved by DCIS. Distance from closest margin: 11 mm, superior Lymph nodes: Total number of lymph nodes examined (sentinel and nonsentinel): 1. Number of sentinel lymph nodes examined: 1. Number of lymph nodes with tumor cells: 0. Pathologic staging: Primary tumor: See comment. Regional lymph nodes: pN0(i-). Comment: In the WHO Classification of Tumours of the Breast (2012), it is stated that "there is no universal agreement on how to stage encapsulated papillary carcinomas. In the absence of conventional invasive carcinoma, the consensus of the WHO Working Group was that such lesions should be staged and managed as Tis disease."

Since high grade squamous intraepithelial lesion (HGSIL) is reportable for the vulva in 2018 (per SINQ 20130185) but VIN II-III is not reportable, we need to clarify this reporting format seen in our area.

Instruction number 1 under the Coding Multiple Histologies instructions states to code histology when the histology is described as subtype, type or variant. The general rules do indicate we can code the histology identified as type, but when applying the H Rules, it seems an argument could be made for either H1 or H3. H1 applies if you ignore the diagnosis of carcinoma and only code the histologic type: urothelial carcinoma. However, the rules do seem to imply that you take all histologies into account (e.g., code the subtype/variant when there is a not otherwise specified (NOS) and single subtype/variant). Following this logic, Rule H3 seems to be the only rule that fits, and one would code the subtype/variant urothelial carcinoma when the diagnosis is carcinoma NOS, histologic type: urothelial carcinoma.

The problem is that urothelial carcinoma is not a subtype/variant of carcinoma (NOS) per Table 2. The entry for Carcinoma NOS in Table 2 states, Subtypes of carcinoma NOS include adenocarcinoma and all subtypes/variants of adenocarcinoma. To some, urothelial carcinoma is a more specific type of carcinoma; however, urothelial carcinoma is not also listed as a subtype of carcinoma or of adenocarcinoma; only adenocarcinoma is categorized as a subtype of carcinoma. Consistently applying the rules becomes an issue when rules are interpreted in different ways. Should this Table be amended to include urothelial carcinoma as a subtype/variant of carcinoma NOS with the same caveat given to adenocarcinoma in Table 2?

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?