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

Abstract two primaries using the 2023 Solid Tumor Rules, Other Sites, Rule M19, as adenocarcinoma and pancreatic neuroendocrine tumors are two distinct histologies.  The WHO Classification of Digestive Tumors, 5th ed., Chapter 10-Tumors of the Pancreas, lists both epithelial tumors and neuroendocrine neoplasm as separate entities.  The Solid Tumor Rules histology-specific tables contain histologies that commonly occur in the 19 site-specific histology tables; therefore, not all histologies are listed in the rules. Further, the adenocarcinoma would be staged in the Pancreas Schema, while the neuroendocrine tumor would be staged in the NET Pancreas schema.

We will consider adding PanNETs to Table 11 in a future release of the Solid Tumor Rules.

Abstract a single primary using Solid Tumor Rules, Malignant CNS and Peripheral Nerves, Rule M10 based on the information provided.  Rule M10 applies as both non-contiguous tumors are of the same histology; i.e., on the same row in Table 3. As MPNST can arise in many sites, look for information about the precise location and tissue type in which the tumor arose. For example, if the tumors are stated to arise in soft tissue, follow the Multiple Primary Rules for Other Sites.

Both WHO Classification of Central Nervous System Tumors and WHO Classification of Soft Tissue and Bone Tumors state that MPNST is a malignant spindle cell tumor often arising from a peripheral nerve, from a pre-existing benign nerve sheath tumor, or in a patient with neurofibromatosis type 1 (NF1).

Future updates will move C470-C479 from CNS to other sites module.

For a primary endometrial or ovarian cancer, record the oophorectomy/BSO procedure using the appropriate Surgery of Primary Site code that includes oophorectomy/BSO when done as part of first course of treatment (surgical resection). If performed for hormone effect, also record in the Hematologic Transplant and Endocrine Procedures data item.  For other primary sites whose Surgery of Primary Site codes do not include oophorectomy/BSO, record it in the Hematologic Transplant and Endocrine Procedures data item when performed for hormone effect.  Document information in the appropriate text fields.

Candidates for risk-reducing BSO may include those with hereditary syndromes (such as BRCA mutations) or genes that carry a substantially increased lifetime risk of ovarian malignancy or hormone-sensitive cancers including estrogen-dependent cancers, like breast cancer, ovarian cancer and endometrial (uterine) cancer that rely on estrogen to develop and grow.

The rule order is the same as in the previous MP/H rules. Will keep as is for now.

Assign codes adenocarcinoma in adenomatous polyp (8210), adenocarcinoma in villous adenoma (8261), and (adenocarcinoma in tubulovillous adenocarcinoma (8263) using Other Sites Solid Tumor Rule H12 or Rule H27 as these are specific invasive histology codes. Rule H13 applies to histology codes associated with polyps but associated with a histology term/code other than adenocarcinoma.

The CPC ICD-O-3 Site Morphology Validation Lists (SMVLs) designate all tumor site-morphology combinations that are either valid or impossible as determined for the sites reviewed by the Cancer PathCHART initiative. These lists provide information on the Validity Status of specific tumor site and morphology combinations, similar to the way the ICD-O-3 SEER Site/Histology Validation List used to. However, the CPC SMVLs do not include information on the reportability of specific tumor site and morphology combinations. For tumor reportability, you will continue to use the Excel ICD-O-3.2 spreadsheets posted to the NAACCR ICD-O-3 Coding Updates website: https://www.naaccr.org/icdo3/, and the most recent SEER Manual and federal, state, local, and other standard setters' reportability requirements.

HGAP is listed as a separate classification and is not a subtype of the diffuse gliomas. WHO Classification of Tumors of the Central Nervous System, 5th edition, has two categories dealing with non-pediatric astrocytic tumors:

Adult-type diffuse gliomas

Circumscribed astrocytic tumors

HGAP falls into the second category as a result of updates to the 4th edition WHO classification in 2016 with advances in the role of molecular diagnostics with the 5th edition. All astrocytic tumors were previously grouped together whereas not all diffuse gliomas (astrocytic or not) are grouped together on the basis of growth pattern and behaviors, and shared IDH1 and IDH2 genetic status. The new classification separates astrocytomas that have a more circumscribed growth pattern, lack IDH gene alterations, and sometimes have BRAF mutations (i.e., pilocytic astrocytoma). The impact of molecular advances has driven classification changes as described in the 5th edition.

Review of site/histologiy combinations for CNS neoplasms is currently being performed by Cancer PathCHART experts. It's possible they will recommend HGAP be moved to a subtype/variant of astrocytoma, NOS. 

In sites other than breast (see Breast Solid Tumor Rules M8/M9), Paget disease with underlying invasive disease is a single primary and the underlying histology is coded. Primary Paget disease of the vulva is uncommon, and we cannot create rules for all possible situations in the Other Sites module. A GYN specific module is in development, and we will look into adding a Paget-related rule. It will differ because Paget in breast is a different situation while Paget in the vulva is always adenocarcinoma.

Paget disease of the vulva is an in-situ adenocarcinoma of vulvar skin with or without an underlying adenocarcinoma (WHO Classification of Female Genital Tumors, 5th ed.). When there is a statement of “underlying” adenocarcinoma, it is a single primary as with Breast Solid Tumor Rule M8.

Determine whether the lesion is on the mucosa or skin based on the pathology report, history and physical, and operative notes when available. The gross description of the pathology report should include information to help in determining whether the site of origin is epithelium (skin) or mucosa (lip).

Do not report the case when the site of origin cannot be determined between a reportable site and non-reportable site for this histology. This includes situations where the site of origin or the site with the greatest involvement is undetermined. In this case, you cannot confirm reportability.

Report LN II and LN III along with LIN II and LIN III and assign code 8520/2.

WHO Classification of Breast Tumors, 5th edition, lists lobular neoplasia as acceptable, related terminology for lobular carcinoma in situ.