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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?

2021-Right pleural fluid:  Negative for carcinoma.

5/18/2021: Right iliac crest bone marrow core biopsy, aspirate smear, clot section and peripheral blood smear:  Hypercellular bone marrow, morphological findings are suspicious for a myeloproliferative neoplasm. Flow Cytometry: Slight immunophenotypic abnormalities of the myeloid cells. No abnormal B cell, T cell, or NK cell populations identified. Normal female karyotype. KARYOTYPE: 46,XX[20]. Negative for deletion of 13q14.3 (D13S319) by FISH. Negative for deletion of 13q34 (LAMP1) by FISH. Negative for hyperdiploidy involving chromosome 9 by FISH. Negative for t(9;22)(q34;q11.2) by FISH. Negative for deletion of the EGR1 gene on 5q31 by FISH. Negative for monosomy 5 by FISH. Negative for deletion of 7q31 by FISH. Negative for monosomy 7 by FISH. Negative for deletion of 20q12 by FISH. Negative for trisomy of chromosome 8 by FISH.

6/4/21-Left adrenal; biopsy: poorly-differentiated malignant neoplasm with extensive necrosis. Immunohistochemical stains show the neoplastic cells to be negative for CK7, TTF-1 and p63. Negative CK7 and TTF-1 would argue against a lung primary. Correlation with clinical and radiological findings is advised.

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These diagnoses were made greater than one year apart with a disease-free interval and M12 seems to be the first rule that applies. This rule does not specifically state the tumors diagnosed greater than 1 year apart must be in the same primary site but Note 1 could be interpreted as implying this. Note 1 states, “Clinically disease-free means that there was no evidence of recurrence in the same site on follow-up.”

Does Other Sites Rule M12 (the timing rule) apply to tumors in different primary sites? It would be helpful if the notes specified this clarification, such as “Clinically disease-free means that there was no evidence of recurrence in the same site (same second and third character CXX.X) on follow-up.”

The patient had residual tumor following the 2018 transsphenoidal resection and underwent an additional surgery after the residual tumor increased in size. Since pituitary adenoma/pituitary neuroendocrine tumor (PitNET) is a new malignant neoplasm for cases diagnosed 2023 and later, should this be a new primary per M5? Or do we disregard the change in behavior and apply rule M2 (single tumor is a single primary) for this scenario?

SEER Program Coding and Staging Manual indicates high grade dysplasia of esophagus, stomach, and small intestine are reportable. The ICD-O-3.2 does not include “high grade dysplasia” as equivalent to “high grade squamous dysplasia.”

If reportable, would high grade dysplasia (NOS) that originates in the stomach and small intestine default to 8148/2, while esophageal high grade dysplasia (NOS) default to 8077/2?

The patient was diagnosed with a left testis primary in the early 1980s that did not include a seminoma component per the information available. The slides were not available for review. In 2024, the patient was found to have a metastatic seminoma involving multiple pelvic lymph nodes and the prostate. The right testicular ultrasound was negative. The managing physician noted this was both a "relapsed seminoma" and a "Stage IIC seminoma."

Should the new diagnosis of metastatic seminoma be accessioned as a new primary based on the histology differences? Or is this situation similar to SINQ 20160073 in which this is a single primary even though the metastases are a distinctly different histology?

Example Case 1:  2022 mobile tongue tumor biopsy shows squamous cell carcinoma, basaloid non-keratinizing type.

Example Case 2:  2022 base of tongue mass biopsy shows squamous cell carcinoma, basaloid non-keratinizing type, p16 positive.

Table 5, Note 2 (Head and Neck Equivalent Terms and Definitions) instructs us to code non-keratinizing squamous cell carcinoma which is p16 positive to 8085 (Squamous cell carcinoma HPV-positive), ignoring the non-keratinizing subtype. Does p16 or HPV positivity also take priority over multiple subtypes (basaloid non-keratinizing type)?

This direction is also in SEER*RSA for Ovary. Researching a possible explanation for this, we found that LVI is an independent predictor of progression and survival in patients with primary epithelial ovarian cancer at early stage but not at advanced stage. However, studies also recommend that routine evaluation of LVI in ovarian cancer is highly recommended in daily practice.

In the summary staging manual pericolic fat is listed under regional direct extension with no mention of whether sub-serosal or supra-serosal. According to our report the pathologist must specify whether involvement of pericolonic fat is of subserosal or supraserosal fat. If involvement of pericolonic fat was not specified as such, this should be localized vs regional direct extension.

In this case, the lymphoma arose in the posterior orbit and the primary site was coded as C696 (orbit, NOS). The mass directly extended to at least one "adjacent" site, the lacrimal gland. Should SS2000 be coded to 1 (localized) or 5 (regional, NOS) when an ocular adnexal lymphoma arises in the posterior orbit and extends to involve the lacrimal gland? Although both the posterior orbit and the lacrimal gland are parts of the orbit, they have separate ICD-O-3 topography codes. Should extension to multiple sites within the orbit be classified as localized disease?

The issue is what constitutes "adjacent" structures for a tumor that arises in the orbit. In an article published by the Indian Journal of Opthamology it states, "According to the Ann-Arbor staging system, lymphoma confined to the orbit is designated as Stage I, involvement of adjacent structures (sinuses, tonsil and nose) makes it Stage II." Does SEER agree with this definition of "adjacent" structures? Or are the lacrimal gland, ciliary body, retina, conjunctiva and/or choroid "adjacent" structures for a lymphoma stated to arise in the posterior orbit?