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Assign code 8461/3 for high-grade papillary serous carcinoma.

Code as a localized, intracapsular tumor as ductal carcinoma in situ does not invade. Intraductal spread is describing the neoplasm spreading through the acinar/ductal cells in the prostate specimen. It is an in-situ type of spread and not invasive but almost always presents with an invasive tumor.

The NAACCR Annotated Histology List (AL) serves as an aid to registry software vendors for implementing annual histology changes. This file has been maintained by the Registry Plus team at CDC’s NPCR for several years and reflects modifications to ICD-O-3 implemented by North American cancer registries over time. Although this list is reviewed multiple times prior to posting, there is no guarantee of 100% accuracy. 

As such, the AL is not a substitute for referring to various standard-setter documents and implementation guidelines. In this instance, Medulloblastoma Desmoplastic SHH-activated and TP53-wildtype 9471 is across several resources: the Solid Tumor Rules, Malignant CNS and Peripheral Nerves module in Table 3, column 3  as a subtype/variant of Medulloblastoma NOS 9470; in the CNS WHO 5th Edition BB; and in the WHO IARC ICD-O-3.2 posted to  ICD O 3 Coding Updates (naaccr.org). Although the exact related term of Medulloblastoma, SHH-activated, NOS is not listed, the NAACCR Implementation Guidelines for 2024 recommend checking the 2024 ICD-O-3 Update Table 1 or 2 to determine if the histology is listed. If the histology is not included in the update, then review ICD-O-3.2 and/or Hematopoietic and Lymphoid Database and/or Solid Tumor Rules (MP/H).

The Cancer PathCHART initiative has been undertaken to address gaps such as this between standard setting resources. Having all the standard histology coding resources included in a single all-inclusive database enables alignment of morphology codes & terms included in the CPC*SMVL (Cancer PathCHART Site-Morphology Validation List), Solid Tumors Rules, ICD-O-3 Annual Updates, NAACCR Annotated Histology List as well as the WHO 5th edition Blue Books. Please see Cancer PathCHART - Tumor Site-Morphology Surveillance Standards Initiative for more information on the Cancer PathCHART initiative, and more specifically, see Transitioning the Annotated Histology List to Cancer PathCHART (naaccr.org).

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.

If you are unable to follow up with the physician, do not report this case until or unless more information becomes available.

The treatment with cryoablation is second course. Once the initial treatment plan is changed, everything after the change is no longer first course of treatment. If the cryoablation was not mentioned as part of the original treatment plan, it is second course.

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.

Use all information available in the medical record. EOD is a combination of the most precise clinical and pathological documentation of the extent of disease as instructed in the EOD 2018 General Instructions, Extent of Disease section.

EOD 2018 General Instructions, General Coding Instructions section advises to use the medical record documentation to assign EOD when there is a discrepancy between the T, N, M information and the documentation in the medical record. When there is doubt that the documentation in the medical record is complete, code the EOD corresponding to the physician staging. A discrepancy can exist within the medical record when the information in the chart is unclear, incomplete, or conflicting, for example, the TNM staging from pathology differs from the medical oncologist’s TNM staging.

In the scenario provided, use the medical oncologist stage information that takes into account imaging and exam findings. Based on the stage cT4 N3b, assign

EOD Primary Tumor: 400

Extensive skin involvement

WITHOUT a stated diagnosis of inflammatory carcinoma

WITH or WITHOUT dermal lymphatic filtration

EOD Regional Nodes: 600

Internal mammary node(s), ipsilateral, clinically apparent

(On imaging or clinical exam)

WITH axillary (level I, II, or III) lymph node(s), ipsilateral including infraclavicular

Please understand that AJCC sets the standards for TNM Staging and the Cancer PathCHART (CPC) initiative sets standards for the validity of site and morphology combinations. The statement in the AJCC manual “8240 and 8249 are excluded for topography code C160” means that these two histologies are not staged using the AJCC Staging System. As with numerous other reportable entities that are not staged by AJCC, the case is reportable and a Summary Stage should be assigned. Combinations of C160 with 8240 or 8249 are valid site/histology combinations for registry reporting and should not be discouraged from use if they correspond to the pathologist’s diagnosis. This goes for any other similar note in the AJCC manual. All CPC standards are enforced via the Primary Site, Morphology-Type, Beh ICDO3, 2024 (SEER) N7040 and Histologic Type ICDO3, Primary Site, Date of Diagnosis (NAACCR) N4911 data quality edits. Registrars can also look up the validity of site and morphology combinations using the CPC*Search tool:  https://seer.cancer.gov/cancerpathchart/search/tool/. 

It is important to remember the following.

• ALWAYS code the tumor histology stated by the pathologist/physician
• NEVER change the tumor histology to assign TNM
• Not all tumors or histologies can be staged per TNM
• Cases that cannot be assigned TNM are assigned a summary stage

Assign histology code, 8077/2 (squamous intraepithelial neoplasia, high grade) for LIN III and for LIN II. ICD-O-3.2 lists squamous intraepithelial neoplasia, grade II and grade III as 8077/2 indicating it is reportable. ICD-O-3.2 does not list every site-specific type of intraepithelial neoplasia. Check the SEER manual for reportable and non-reportable examples.