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Pathology report dated 10/17/2019: Final Diagnosis: Fallopian tubes and gonads, right and left, excision: Dysgenetic gonadal tissue with nests and tubules of atypical germ cells suspicious for gonadoblastoma and at least germ cell neoplasia in situ; and segments of fallopian tube (pending expert consultation).

Histologic Type: HPV-associated multiphenotypic carcinoma. Overall, the morphology, immunohistochemistry, and HPV testing results support the diagnosis of an HPV-related multiphenotypic carcinoma. This entity has been described in the sinonasal region, where it behaves more indolently than its other salivary gland carcinoma counterparts (e.g., adenoid cystic carcinoma), with local recurrence but rare metastases.

Patient had a vulvar biopsy with final diagnosis of Extramammary Paget neoplasm (intraepithelial glandular neoplasm). No invasion identified. We are unable to contact the pathologist or physician for clarification.

Although this terminology is not listed in the ICD-O-3, web search results refer to this as a possible synonym for Paget disease with associated VIN III, which is reportable.

For example, a patient has a 2.6 cm breast tumor on MRI; a core biopsy measuring 0.7 cm is positive for infiltrating duct carcinoma. Rule #1 states "Use the largest measurement of the primary tumor from physical exam, imaging, or other diagnostic procedures before any form of treatment." However, Rule #9 seems to imply that size from an "incisional biopsy" takes precedence over imaging, even though it is known to be less than the entire tumor in size.

The bone marrow biopsy proved bone marrow with blasts comprising 15-19%. Neither the pathologist nor the physician specifically diagnosed this as AML, calling this only high risk disease or early evolving AML prior to starting the patient on Vidaza.

No further information can be obtained from the pathologist or the physician for this case. Should this early evolving AML be accessioned as an additional primary per Rule M10, or is this the same MDS that is now high risk as the blast count is up to 19%, but has not yet reached the threshold of 20% blasts usually required for AML per the Hematopoietic and Lymphoid Neoplasm Database?

In Table 3 (Specific Histologies, NOS/ NST, and Subtypes/Variants), the entry for papillary carcinoma, NOS includes a change in column 3 of the 2018 Breast Solid Tumor Rules that conflicts with the H Rules. It is not accounted for in the change log. No explanation is offered as to why this change was made. This is a major change because encapsulated papillary carcinoma is frequently associated with carcinoma NST, and we have not been collecting these as such.

Encapsulated papillary carcinoma (8504) in column 3 now includes an indented entry, with invasive carcinoma, NST/invasive duct carcinoma 8504/3. However, most encapsulated papillary carcinomas are in situ or there is no definitive statement of invasive encapsulated papillary carcinoma, so when in situ and invasive tumors are present, we are instructed to code the invasive histology (which would be the invasive carcinoma (NST), 8500/3). How are registrars to arrive at the correct histology without a new H rule or a clarification regarding this update being documented in the change log?

Does the same change/addition apply to solid papillary carcinoma? These are often also associated with carcinoma, NST. Again, without an explanation regarding the change mentioned above, it is difficult to understand why the change was made.

This question was prompted from preparing SEER*Educate coding exercises. We will use the answer as a reference in the rationales.

Patient had a radical pharyngectomy showing a glossotonsillar sulcus tumor with high grade squamous cell carcinoma and adjacent high grade mucoepidermoid carcinoma. The pathologist commented, the tumor is composed of high grade mucoepidermoid carcinoma and high grade conventional-type squamous cell carcinoma that are immediately adjacent to one another. Given that the tumors are arising so close together and could represent a single neoplastic process with divergent morphologies, they are staged together.

Employing Solid Tumor Manual Rule M1 (single primary if unable to determine if there is a single or multiple tumors), it was determined that this should be reported as a single tumor because the pathologist referred to the case as both a tumor singular and tumors pleural. However, the Solid Tumor Manual Histology Rules for a Single Tumor do not appear to have an instruction for coding this histology combination.

The patient has a history of a minimally invasive endometrial adenocarcinoma that was low grade and confined to an endometrial polyp in 2001. The patient underwent a total abdominal hysterectomy/bilateral salpingo-oophorectomy (TAH/BSO) that entirely removed the tumor at that time.

Almost 18 years later, the patient had a left inguinal mass excision that was, Carcinoma of gynecologic origin, consistent with clear cell carcinoma. No other disease was found, the physician never indicated whether this was felt to be metastatic from the previous, low grade adenocarcinoma or not. It was only noted as, an unusual malignancy of the left lower quadrant and inguinal region of gynecologic origin. No further information was available in the medical record or from the physician on follow-up.

Although neither the Solid Tumor Rules nor the MPH Rules (still in use for the Other Sites schema) apply to metastasis, given the differences in histology and behavior of these two tumors (i.e., minimally invasive, low grade disease diagnosed in 2001 vs. higher grade, more aggressive tumor in 2019) should the current clear cell carcinoma of GYN origin really be the same primary as the 2001 endometrial adenocarcinoma?