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The 2021 AIN III is not a new primary. According to our GI pathology expert, findings of AIN I and/or AIN II following a diagnosis of AIN III indicates the patient was never NED and indicates persistent disease.

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The 2022 Solid Tumor Update will include a new note in the Terms & Definitions, Introduction section that will state: See the Non-malignant CNS rules when the primary site is optic nerve and the diagnosis is either optic glioma or pilocytic astrocytoma. The behavior is non-malignant and coded 9421/1.

The 5th bullet under Rule PH 22 was inadvertently omitted. A corrected version of the Heme manual will be posted soon. Thank you for identifying this omission.

In the meantime, please add the following to PH22:

Code the primary site to lymph nodes, NOS (C779) when lymph node(s) are involved but no primary site/particular lymph node region is identified.

The CPC Validity Status of the site morphology combinations of C569/8441/3 and C569/8441/2 was revised from Valid to Unlikely with the latest release of the Version v24A Edits Metafile. As a result, this site and morphology combination will now require an over-ride flag to be set.

Code as 8461/3 (high-grade serous carcinoma) or 8460/3 (low-grade serous carcinoma) if at all possible. Use 8441/3 (serous carcinoma, NOS) only if it cannot be distinguished as low grade or high grade. The codes for high-grade serous carcinoma and low-grade serous carcinoma are relatively new. High-grade serous carcinoma and low-grade serous carcinoma are very different tumors and pathologists should state whether it is high grade or low grade. Please make every attempt to use the newer codes. If unable to determine high gade versus low grade, assign 8441/3 and override the edit.

The files on the CPC website are currently being updated, and CPC*Search will be updated to reflect the changes sometime this Fall.

The Histology Task Force approved a 60 day time frame for ambiguous terminology.

The majority of cases are first identified by ambiguous terminology; for example, a patient has a mammogram that shows a lesion suspicious for cancer. That first indication of cancer prompts a work-up to either confirm or rule-out the cancer diagnosis.

The data item "Ambiguous terminology" is not intended to capture information on this routine method of detecting and diagnosing cancer. The 60 day time frame should keep these cases out of the ambiguous terminology data item.

The data item is intended to identify those cases where the cancer diagnosis is NOT confirmed during the work-up, but the case is still entered into the database. For example a patient who has a TRUS because of elevated PSA. The pathology from the TRUS says "Suspicious for adenocarcinoma of the prostate." The physician only documents that the patient is to return in 6 months for another PSA and TRUS. The registrar would enter this case into the data base because the word "suspicious" is on the ambiguous terminology list.

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 NAACCR Guidelines for ICD-O-3 Histology Code and Behavior Update Implementation, effective January 1, 2018, state: Currently in ICD-O-3, when a topography (C code) is listed in parentheses next to the morphology term, it indicates morphology is most common to that site. It may occur in other sites as well. Many of the new codes, terms, and behaviors listed in this update are site-specific and do not apply to all sites.

Please review the Comments to determine which histology codes are specific to sites. You may use sites not listed as the suggested site; however, it will generate an edit error for review and verification of the appropriate site.

The R-ICE treatment in this case is not part of the first course. Documentation of treatment failure and/or disease progression signifies the end of the first course of treatment.

The SEER rules changed in 1998 regarding what constitutes First Course of Cancer-Directed Therapy.

For cases diagnosed on or after 1/1/98: The First Course of Cancer-Directed Therapy fields will all be coded to 0 [None] for these types of cases. The documented disease progression would stop the timeframe for inclusion of any treatment to be part of first course of therapy.

The Solid Tumor Malignant CNS tables do not list pituitary specific histologies at this time. Registrars will need to refer to ICD-O and/or updates until the decision to add malignant pituitary neoplasms is made. Pituitary blastoma is a rare tumor which occurs in children.