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Lung cancer, 2022

12/1/2022 PET/CT showed destructive hypermetabolic bone lesions in right frontal and left posterior calvarium. Left posterior calvarium lesion involves portions of left parietal and temporal bones w/invasion of mastoid air cells.

1/4/2023 MRI Brain showed large destructive mass involving left posterior temporal calvarium that extends into left mastoid region and may invade left distal transverse sinus.

2/8/2023 Radiation Oncology follow-up note: MD states there are extensive calvarium metastasis with the left parietal lesion invading the brain causing edema and MS-like changes.

2/13/23 Radiation Oncology Final Letter- Patient was treated with 1 EBRT fraction aimed at brain/skull before enrolling in hospice.

Imaging identified a mass involving the hypothalamus and pituitary gland and excision of the mass proved “histiocytosis.” The case was extensively reviewed, and the physician notes this patient has a pituitary tumor that is a “non-Langerhans cell histiocytosis,” or a “non-LCH histiocytic neoplasm.”

There is no histology for histiocytosis (NOS) or non-Langerhans cell histiocytosis. However, this does appear to be a non-malignant histiocytic neoplasm. If this were a Langerhans cell histiocytic neoplasm in the CNS it would be reportable. Should this non-Langerhans cell histiocytic neoplasm also be accessioned as a reportable CNS neoplasm? If so, how is the histology coded?

Patient was diagnosed on imaging with enhancement along the right optic nerve intraorbital segment, displacing the optic nerve, most consistent with optic nerve sheath meningioma.

Extracranial meningiomas are rare, however SINQ 20230052 does contain an exception for reportability in a different head and neck site because it is not an intracranial location.

It is unclear if this portion of the meninges surrounding the intraorbital optic nerve is still “intracranial” and thus reportable.

Patient was seen in 2009 at Hospital A for bone pain and was found to have metastatic adenocarcinoma. A paraffin block specimen was sent to BioThernostics for THEROS CancerTYPE ID Molecular Cancer Classification Tests. The results came back with a 94% likelihood that the urinary bladder was the primary site. No scans were done on the abdomen or pelvis.

The patient was then sent to Hospital B for radiation to the bones and chemotherapy (Carboplatin and Taxol). The patient died within 6 months.

According to Hospital A, the primary site is bladder based on the molecular study report. Hospital B says this is an unknown primary. Which is correct? Do we take primary site from these tests, even when no clinical correlation is documented?

The only Other Sites H Rule related to extramammary Paget disease is included in the Multiple Tumors Abstracted as a Single Primary module. Rule H28 instructs one to code the histology of the underlying tumor when there is extramammary Paget disease and an underlying tumor of the anus, perianal region, or vulva. Therefore, a vulvar extramammary Paget disease with underlying adenocarcinoma is coded as adenocarcinoma (8140/3), and not extramammary Paget disease (8542/3).

However, there is no M Rule confirming extramammary Paget disease and the underlying adenocarcinoma are a single primary (i.e., multiple tumors abstracted as a single primary) making it difficult for one to use the Multiple Tumors Abstracted as a Single Primary H rules module.

We recognize this is a longstanding histology coding rule, but how are registrars supposed to arrive at Rule H28 when the M Rules must be applied first and do not instruct one to accession a single primary? Moreover, if this is to be a single primary (per rule M2), why is there no H Rule in the Single Tumor module?

The STR states that p16 can be used to code HPV-associated and HPV-independent histologies for selected sites depending on diagnosis year but contains no instructions about how to interpret p16 staining results on pathology reports. These are often stated in various ways in our area, depending on the pathology lab and different pathologists. The SSDI Manual and SEER Coding and Staging Manual each have some instructions and code definitions for p16, including:

- Code 0 for p16 expression of weak intensity or limited distribution

- Code 0: p16 Negative; Nonreactive

- Code 1: p16 Positive; Diffuse, Strong reactivity

- IHC for p16 expression is a surrogate marker for HPV infection

Example: 2023 squamous cell carcinoma of the vulva, partial vulvectomy; pathology states vulvar intraepithelial neoplasia-3, p16 immunohistochemistry demonstrates block-like expression, which supports the diagnosis. The next path report states invasive squamous cell carcinoma, stain for p16 is strong and diffuse in the lesion, supporting the above diagnosis. Neither path report specifically states "HPV-related," so are p16 "expression" and "strong and diffuse" staining enough to code the histology as 8085/3 for this case?

Table 19: Vulva Histologies of the Other Sites Solid Tumor Rules does not include entries for either keratinizing or nonkeratinizing squamous cell carcinoma in the “Squamous cell carcinoma, NOS” row. However, these are two distinctly different histologies per the ICD-O-3.2.

All other Solid Tumor Rules schemas include an M Rule instructing one to abstract multiple primaries when separate/non-contiguous tumors are two or more different subtypes/variants in Column 3 of the Specific Histologies, NOS, and Subtype/Variants Table for the schema (e.g., Rule M6 for Lung). The timing of these tumors is stated to be irrelevant. The Notes confirm the tumors may be subtypes/variants of the same or different NOS histologies and tumors in column 3 are all distinctly different histologies (even if they are in the same row). However, the 2023 Other Sites schema appears to be missing this rule.

Should these distinctly different histologies be accessioned as separate primaries? Is an M Rule missing from the Other Sites schema to address distinctly different histologies?