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Abstract two primaries using rule M4 of the Colon rules or rule M13 of Other Sites: 1. Invasive adenocarcinoma of distal rectum and 2. Paget disease of the anal mucosa / perianal skin (determine site of origin and code primary site accordingly). The rectum and the anus are separate sites and the histologies differ in each site.

The WHO Classification of Digestive System Tumors, 5th edition, states that in addition to secondary anal Paget disease arising from anal canal adenocarcinoma, or rarely, adenoma without documented invasive disease, secondary Paget cells may be contiguous with the underlying neoplasm or manifest at different at sites distinctly away from it (with skip lesions).  Document the details in the appropriate text fields.

Assign code 8742/2 (lentigo maligna) for “early lentiginous melanoma in situ.” ICD-O-3.2 lists the preferred term for 8742/2 as lentigo maligna (C44._).

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. 

Assign histology code 8254/3 (mixed invasive mucinous and non-mucinous adenocarcinoma) to this lung tumor using Lung Solid Tumor Rules, Rule H4. This is a new code/term approved by IARC/WHO for ICD-O. Rule H4 instructs one to code the histology when only one histology is present. In this case, the pathologist indicates the tumor is mixed mucinous and non-mucinous histologies. The non-mucinous carcinoma that is seen in this mixed histology may be identified as: Adenocarcinoma in situ, minimally invasive adenocarcinoma, or lepidic predominant adenocarcinoma. In this case it is lepidic predominant adenocarcinoma. Lepidic is a recognized histology in lung. It is not unusual for the pathologist to indicate mixed non-muncinous and mucinous adenocarcinoma AND also list the non-mucinous subytpe. It is important to capture both mucinous and non-mucinous histologies which drives treatment, etc. 

Assign 8077/2 for high-grade squamous intraepithelial lesion, VIN 3 in this case.  The WHO Classification of Female Genital Tumors, 5th edition, states that squamous intraepithelial lesions (SILs) of the vulva are also known as vulvar intraepithelial neoplasia, HPV-associated.  The term squamous cell carcinoma in situ is not recommended.

For the example provided: For 2023 cases and forward, if no part of the surgery was performed, code Surgery of Primary Site 2023 (NAACCR Item #1291) as code A000 or B000 (no surgical procedure of the primary site).  Code Reason for No Surgery of Primary Site (NAACCR Item #1340) as code 2 (surgery of the primary site was not recommended/performed because it was contraindicated due to patient risk factors (comorbid conditions, advanced age, progression of tumor prior to planned surgery, etc.).

In contrast, if any part of the surgery was performed, assign the Surgery of Primary Site 2023 (NAACCR Item #1291) code that best reflects the extent of the surgery performed.  Code Reason for No Surgery of Primary Site (NAACCR Item #1340) as code 0 (surgery of the primary site was performed).

Use text fields to record the details.

For cases prior to 2023, apply the same approach using Surgery of Primary Site (NAACCR Item #1290) instead of Surgery of Primary Site 2023 (NAACCR Item #1291).

Report as squamous cell carcinoma (8070/3) on the basis of “microscopic focus suspicious for superficial invasion.” "Severe dysplasia" is equivalent to "high grade dysplasia" in the Head and neck. As such, "severe squamous dysplasia" would be coded to 8077/2. However, in combination with the statement of "with microscopic focus suspicious for superficial invasion,” report as squamous cell carcinoma (8070/3) based on “microscopic focus suspicious for superficial invasion.” The 2023 SEER Manual instructs us to code the behavior as malignant (/3) if any portion of the primary tumor is invasive no matter how limited, i.e., microinvasion. Use text fields to record the details. 

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)

Do not report the case. The histology is 8825/0 based on the example provided and not reportable. Myofibroblastoma with sarcomatous transformation is a rare, benign condition, sometimes referred to as sarcomatous features. A malignant tumor would be referred to as a myofibroblastic sarcoma.

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.