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Similar to an issue previously submitted SINQ 20180063, Table 1 (WHO Grades of Select CNS Neoplasms) in the Non-Malignant CNS Equivalent Terms and Definitions section states WHO Grade I tumors are always non-malignant. However, this does not mean that the tumors listed in Table 1 as WHO Grade I are always benign (/0). Some tumors listed with a WHO Grade I have a behavior of /1 (borderline) per the ICD-O-3 and/or Tables 5 and 6. The Behavior coding instructions do not currently indicate these are the appropriate sources to use when the pathologist and/or physician do not comment on the behavior of these tumors. In our area, pathologists do not explicitly state the behavior for these tumors; the pathologist only assigns the WHO Grade.

An endometrial cancer was diagnosed in 2018. The endometrial biopsy showed malignant mixed mullerian tumor (MMMT/Carcinosarcoma). The total abdominal hysterectomy/bilateral salpingo-oophorectomy showed Endometrial Carcinosarcoma (50% serous carcinoma, 50% high grade sarcoma with rhabdomyoblastic differentiation) with invasion of 100% of the myometrium and involvement of the uterine serosa. I am not finding this in the Solid Tumor Rules or the site-specific ICD-O-3 code lists.

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?

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?

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?

Patient had a breast excision that proved a single tumor with no evidence of invasive carcinoma. The final diagnosis stated: Size (extent) of EPC DCIS: Spanning approximately 1.3 cm. The pathologist did not describe separate foci of DCIS; only one tumor comprised of both encapsulated papillary carcinoma and DCIS. The encapsulated papillary carcinoma was not described as invasive. The pathology noted: This case is best classified as EPC conventional DCIS. No conventional stromal invasion is identified. Solid Tumor Rule M2 confirms a single tumor is a single primary.

However, there does not appear to be an H Rule that instructs how to code histology. The Single Tumor: In Situ Only module, has only three H Rules and none of them apply to this case. The patient does not have Paget disease (H1), does not have a single histology (H2, there are multiple histologies present as DCIS and EPC are listed on different rows in Table 3) and does not have DCIS and LCIS (H3). How does one arrive at the correct histology for this case?

The right breast ultrasound shows a 1.4 cm mass at 8 o'clock and a separate mass .6 cm at 7 o'clock (site code for both C50.5). Pathology report: Right 8 o'clock core needle biopsy fragments of intracystic noninvasive papillary carcinoma (8504/2), right 7 o'clock core needle biopsy fragments of intracystic noninvasive papillary carcinoma (8504/2). Then, another facility performs a right breast lumpectomy (operative note not available). Outside Facility: Right breast lumpectomy pathology shows invasive ductal carcinoma .6cm (8500/3) multifocal DCIS .5cm greatest dimension tumor site right breast NOS.

Should we use Rule M12-Abstract multiple primaries when separate/non-contiguous tumors are on different rows in Table 3 in the Equivalent Terms and Definitions. Timing is irrelevant. Note: Each row in the table is a distinctly different histology. So would this be two primaries C50.5 (8504/2) and C50.9 (8500/3)?

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?