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Glioblastoma multiforme is listed as a synonym for the preferred term glioblastoma, NOS (9440) per Table 3 Column 2. Therefore, it seems reasonable to assume that a diagnosis of glioblastoma, NOS would be a new primary if it followed a glial or astrocytic tumor. However, in general, the Solid Tumor Rules use the preferred terminology and/or indicate when a specific rule also includes any tumor diagnosed as a subtype/variant. Rule M6 does not explicitly include a diagnosis of glioblastoma, NOS or any of its subtypes/variants (e.g., glioblastoma IDH-mutant or gliosarcoma). Does Rule M6 apply to any diagnosis of glioblastoma, NOS and any of its synonyms or subtypes/variants?

12/30/11 Bladder Biopsy: Diffuse carcinoma in situ of bladder, urothelial cancer at trigone (Stage T1)

1/30/2012 Transurethral resection of the bladder was non-papillary, urothelial carcinoma, focal invasion of lamina propria, staged T1

11/10/14, 9/28/15, 9/26/16, 10/19/17 all had positive bladder cytology of urothelial carcinoma

5/16/16 Left renal pelvis aspirate: positive for malignant cells, urothelial carcinoma

9/26/16 Left renal pelvis aspirate: positive for malignant cells, urothelial carcinoma

10/18/16-11/7/16 Bacillus Calmette-Guerin (BCG) x3 administered into the renal collecting system via ureteral catheter

The hospital registrar reports that the case was identified at the hospital because of the referral for chemotherapy for the metastatic GIST. The records from the out-of-state hospital mentioned a history of a small bowel resection in 2003 for a borderline tumor. The registrar went back through the hospital's old records and found the surgery was done for GIST of low malignant potential at her facility. The question is whether to report the case or not, and if reported, is 2003 the diagnosis date.

The rules say to change the behavior and backdate the diagnosiswhen a tumor is presumed benign and islater diagnosed as malignant. Another problem for this case is that the out-of-state hospital did not review the slides from the 2003 surgery.

It appears an argument could be made for both NLPHL (9659/3) and THRLBCL (9688/3). We favor coding NLPHL (9659/3) because the pathologist did specifically call this a Hodgkin lymphoma, and also specified that it only has a T-cell/histiocyte-rich large B-cell lymphoma-like pattern.

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?

This comment was added to the pathology report by the pathologist: A focus of neuroendocrine microtumor measured 350 micrometers, qualifying as a neuroendocrine microtumor. Focus was immunohistochemically positive for chromogranin and synaptophysin and negative for gastrin. The Ki-67/CD45 immunostain showed <1% positivity in microtumor.

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.