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Many of these terms are not specified in the codes and definitions in ICD-10-CM. This is because ICD-10-CM does not have the same granularity as ICD-O-3.2. There are a few sites where intraepithelial neoplasia II and/or III are mentioned.

Even though ICD-O-3.2 classifies these as /2 (in-situ), for the intraepithelial neoplasia that are listed in ICD-10-CM, Grade II is designated as benign, while Grade III is designated as in-situ. It is not clear if medical coding will change the Grade II to an in-situ code.

All the in-situ codes (except cervix) are included in the casefinding list. Grade III is included with the in-situ codes; however, there is no guarantee that medical coders will code them as in situ. High grades are coded as in-situ in ICD-10-CM.

For those where there is no specific intraepithelial neoplasia code, the benign codes will cover any benign lesion for that site. This would make for a lot of review using the codes for casefinding. Most of the benign codes were removed from the casefinding list a couple of years ago to make it more manageable. 

Use the casefinding list as a guide for these neoplasias. It is not the most definitive source due to the lack of specificity of ICD-10-CM. It is not possible to map every single histology to a specific code. It is also not known how medical coders across the U.S. are coding these neoplasias. For that reason, pathology should remain the foremost casefinding resource used.

The casefinding team will need to review the prepared list below and determine what codes to add. Any updates will be incorporated in the FY2025 updates (October 2024.)

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).

Do not report angiomyxoma. ICD-O-3.2 assigns 8841/0 to this benign tumor. This includes superficial and deep (aggressive) angiomyxoma.

We will add the same definition for teratoma with malignant transformation found in the ovary table: 9084/3 Teratoma with malignant transformation when a malignant (/3) histology arises in a benign teratoma.

Teratoma with malignant transformation and teratoma with somatic-type malignancy are synonoyms. The term teratoma with somatic-type malignancy is outdated and no longer recommended.

Code the specific histology as stated by the pathologist according to the site-specific instructions in the Solid Tumor Rules. When the histology provides a subtype/variant in addition to the HPV histology codes, code the subtype/variant as it is important to capture this histology as in the example provided. the instruction to code the subtype/variant over 8085 or 8086 applies to the following sites: oropharynx, cervix, vagina, vulva, anus, and penis. A note will be added indicatng this in 2025. 

Per 2024 Cancer PathCHART expert pathologist review, morphology codes 8085/3 and/or 8086/3 are valid and applicable to head and neck, oropharynx, cervix, vagina, vulva, fallopian tube, anus, and penis (reference: Cancer PathCHART: Product Downloads and Timelines). Other coding resources will be updated to reflect these changes in 2025.

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. 

Do not code Reblozyl (luspatercept) as treatment. Luspatercept is an ancillary drug approved to treat anemia associated with MDS but not the malignancy.

Assign code 8020/3 when the histologic type specifically includes the term of poorly differentiated, dedifferentiated, undifferentiated, or anaplastic undifferentiated carcinoma along with carcinoma as terms vary depending on the primary site. When the term poorly differentiated is included in the grade section only of the pathology report or only mentions poorly differentiated carcinoma without further substantiation from a pathology report as in examples 1 and 2, do not use code 8020/3.

The histology code 8020/3 and terms may be used for selected primary sites as included in the Solid Tumor Rules, WHO Classification of Tumors series (latest versions), and the Site/Morphology Validation List including

Nasal cavity

Nasopharynx

Salivary glands

Urinary sites

Colon, rectosigmoid, rectum

Esophagus

Stomach

Gallbladder and extrahepatic bile duct

Pancreas

Thyroid

Ovary

Uterine corpus

Vagina

Uterine cervix (also referred to as unclassifiable in WHO Classification of Female Genital Tumors, 5th ed.)

For sites other than those listed, if the diagnosis is poorly differentiated carcinoma, code 8010/3 and poorly differentiated in the grade field. 

Per 2024 Cancer PathCHART expert pathologist review, morphology codes 8085/3 and/or 8086/3 are valid and applicable to head and neck, oropharynx, cervix, vagina, vulva, fallopian tube, anus, and penis scrotum (reference: Cancer PathCHART: Product Downloads and Timelines). The Cancer PathCHART SMVL will be updated for C632, Scrotum, with the next release of the NAACCR Edits Metafile, currently scheduled for May 2024.

Assign histology codes 8085 and 8086 for the sites listed in the Solid Tumor Rules histology tables. The codes 8085 and 8086 are applicable for a small group of sites according to the year they became valid for implementation as follows.

Head and Neck

      Oropharynx, Base of Tongue, Tonsils, Adenoids (2022+)

Other Sites

      Cervix (2021+)

      Anus (2023+)

      Vagina (2023+)

      Vulva (2023+)

      Penis (2024+)

      Scrotum (2024+)

While ICD-O-3.2 and Cancer PathCHART list additional sites such as Accessory Sinuses, they have not yet been implemented in the U.S.

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.