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Assign 8249/3 for high-grade neuroendocrine tumor of the lung.

WHO Classification of Thoracic Tumors, 5th edition, defines two subtypes of neuroendocrine tumor of the lung, typical carcinoids (8240/3), and atypical carcinoids. WHO assigns typical carcinoid/neuroendocrine tumor grade 1 as 8240/3 and atypical carcinoid/neuroendocrine tumor grade 2 as 8249/3. They are regarded as low-grade and intermediate-grade, respectively. The preferred term for 8249/3 in ICD-O-3.2 is neuroendocrine tumor grade 2, with neuroendocrine tumor grade 3 as a related term. The Lung Solid Tumor Rules assign atypical carcinoid as 8249/3.

Do not report neuroendocrine tumorlet of lung and non-lung sites. Microcarcinoid and carcinoid tumors are reportable.

Tumorlet is a tumor of neuroendocrine differentiation, defined by size < 5 mm in diameter, mitotic count < 2 mitoses/2 mm², and absence of necrosis. Microcarcinoid is a designation for neuroendocrine tumors when they are less than 0.5 cm. in size. The term "tumorlet" is used in a number of other settings, referring to small tumors (usually < 0.5 cm), and does not necessarily mean carcinoid tumor.

The term microcarcinoid tumor is not equivalent to neuroendocrine tumorlet.

Report clear cell renal cell papillary tumor (CCRCPT), formerly classified as clear cell renal cell papillary carcinoma, and assign code 8323/3 until this new term and code (8323/1) have been adopted by standard setters.

The Kidney Solid Tumor Rules advise to code clear cell papillary renal cell carcinoma as 8323/3. WHO Classification of Tumors of the Urinary System and Male Genital Organs, 4th ed., has reclassified this histology as a /1. This change has not yet been implemented and it remains reportable.

WHO Classification of Urinary and Male Genital Tumors, 5th ed., has since reclassified clear cell papillary renal cell carcinoma as CCRCPT (8323/1). The name change was made because there have been no reports of metastatic events for this indolent tumor. The term clear cell renal cell papillary carcinoma is no longer recommended.

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

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 indicating 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.

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.

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

Abstract this case as a single primary. Code as 8044/3 (small cell carcinoma, hypercalcemic type) listed in the Other Sites Solid Tumor Rules, Table 13. Small cell carcinoma, large cell variant, is a subtype of small cell carcinoma, hypercalcemic type. This table does not include all possible histologies.

WHO Classification of Female Genital Tumors, 5th edition, states: Small cell carcinoma of the ovary, hypercalcemic type, is rare, accounting for < 1% of ovarian tumors. Small cell carcinomas, hypercalcemic type, are usually large, with a mean size of 15 cm (range: 6–26 cm). Large cells are present (in varying numbers) in half of these tumors, which are designated “small cell carcinoma, large cell subtype” if the large cells are predominant (which is rare).

Assign histology code 8054/3 for warty carcinoma. Assign 8051/3 for verrucous carcinoma and carcinoma cuniulatum. 

The WHO Classification of Urinary and Male Genital Tumors, 5th edition (2022) revised the terminology for squamous cell carcinoma groupings from "non-HPV-related" to "HPV-independent" and from "HPV-related to "HPV-associated". Warty carcinoma is defined as a "morphologically distinct HPV-associated verruciform neoplasm that shares histological features with a giant condyloma but has definitive cytological atypia and a malignant infiltrative architecture."  Verrucous carcinoma (including carcinoma cuniculatum) is defined as an HPV-independent squamous cell carcinoma, and is correctly coded to 8051/3.

The 2024 Solid Tumor Rules, Table 23, Penis and Scrotum Histologies will be updated to reflect this revised terminology and coding.

Do not report PanIN II. WHO Classification of Digestive Tumors, 5th edition, now categorizes PanIN into two categories, low grade (8148/0) and high grade (8148/2). PanIN grade I and PanIN grade II are categorized as PanIN low grade; PanIN grade III is categorized as PanIN high grade.

We will update the Reportability section of the manual.