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Table 19 is based on WHO 5th Ed Tumors of vulva and squamous cell variants, keratinizing and non-keratinizing, are no longer recommended and are excluded from the 5th Ed. HPV related terminology is now preferred for these neoplasms.

Per consultation with our GYN expert pathologist, based on the information provided, this is likely a single tumor that was not completely excised in the original biopsy. A new tumor in the same site would not appear within 8 months. If you cannot confirm two separate/non-contiguous tumors were present, abstract a single primary per M1. As for histology, the tumor showed both keratinizing and non-keratinizing features and HPV status is unclear. Per our expert, code to SCC 8070/3—keratinization or lack of does not change treatment or prognosis.

Even If there is proof of separate/non-contiguous tumors, our expert still feels this is a single primary coded to SCC 8070/3. Treatment does not differ by keratinization or HPV status. Coding two primaries would be incorrect and inflate incidence rates.

Per our expert, this is an unusual occurrence. The rules cover 85% of cases but there will always be situations that do not fit a rule. This case is an example of that.  A new GYN specific Solid Tumor Rules module is under development and a rule to address this situation could be included.

Assign as Stage IV as recommended by our expert hematological oncologist. This is a rare occurrence and this type of presentation does not fit the definition of intraocular extension. Stage IV is probably the best stage for this type of presentation, since there are two extranodal organs involved, even though they involve a bilateral site.

EOD Primary Tumor: 700

SS: 7 (Distant)

Assign histology as adenocarcinoma, HPV-associated (8483/3) as designated in Table 17, Uterine Cervix Histologies, of the Other Sites Solid Tumor Rules. The WHO Classification of Female Genital Tumors, 5th edition, states that p16 immunohistochemistry is an effective (yet flawed) indirect test for HR-HPV infection, in line with the STRs that state p16 is a valid test to determine HPV status and can be used to code HPV-associated and HPV-independent histologies. In this scenario, "negative for high-risk human papilloma virus (HR-HPV) on Pap smear" would be cytology-based, and may have missed cytologically malignant cells. A subsequent, more definitive biopsy was performed and was found to be strongly positive for p16, therefore, the tumor should be coded as 8483/3.

Report intracholecystic papillary neoplasm (ICPN) with high-grade dysplasia (8503/2) of the gallbladder.

High grade squamous dysplasia of the tongue is reportable as of 2021 and later as 8077/2.

NTRK-rearranged spindle cell neoplasm is a newly identified variant of sarcoma; however, WHO has not yet proposed a specific ICD-O code for this rare neoplasm. Code to spindle cell sarcoma (8801/3).

WHO defines NTRK-rearranged spindle cell neoplasm as an emerging group of molecularly defined rare soft tissue tumors that span a wide group of morphologies and histological grades, and are most often characterized by a spindle cell phenotype among other characteristics.

Solid pseudopapillary neoplasm of the pancreas is reportable for cases diagnosed in 2014 and later. Report solid pseudopapillary neoplasm of the pancreas (8452/3) as the guidance in SINQ 20140058 is still in effect.

The 4th and 5th editions of the WHO Classification of Tumors of the digestive system define solid pseudopapillary neoplasm of the pancreas as a low-grade malignant pancreatic tumor.

The patient's previous acinar adenocarcinoma in the right lower lobe of the lung in 2020 and squamous cell carcinoma in the right middle lobe of the lung in 2021 were correctly abstracted as two primaries per rule M8 as they are in different rows in Table 3. The NSCLC, RUL (8046) diagnosed in 2022 would not be abstracted as a third primary because NSCLC is a broad category which includes all histologies in Table 3 (except for small cell carcinoma/neuroendocrine tumors (NET Tumors) 8041 and all subtypes), and because it was diagnosed less than 3 years after the 2021 squamous cell carcinoma, RML (8070).

Do not report superficial angiomyxoma (8841/0) or aggressive angiomyxoma (8841/0).  WHO Classification of Female Genital Tumors, 5th edition, defines deep (aggressive) angiomyoma as a benign, infiltrative, myxoid spindle cell neoplasm that occurs in deep soft tissue of the pelviperineal region.

This is reportable as 9591/3, B-cell lymphoma, NOS.All diagnoses in the differential are all B-cell lymphomas. The pathologist knows it a B-cell lymphoma but has not determined the subtype. If at a later time a specific lymphoma is determined, update the histology code accordingly.