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

Report this case as a pituitary tumor (8000/1) based on the information provided. This is the best choice as no specific histology code exists for this generic term “non-Langerhans cell histiocytosis” in ICD-O-3.2, WHO Classification of CNS Tumors, 5th ed., and WHO Classification of Tumors of Hematopoietic and Lymphoid Tissues, 4th ed.

Be sure to double-check the behavior code of the tumor. Histiocytosis can be benign, borderline, or malignant. There was no mention of the behavior so we defaulted to uncertain behavior for this case. 

Assign Primary Site code C504 based on the location in the upper outer quadrant of the right breast, 10 o’clock, as opposed to code C501, around the areolar complex. The 2024 SEER Manual Breast Coding Guidelines advise that C502 - C505 are generally preferred over C501 when there is no other way to determine the subsite.

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.

Assign code 550 (Bladder, NOS excluding mucosa) to EOD Primary Site based on invasion into the bladder with no mention of mucosa. EOD Primary Tumor for cervix, Note 1, instructions are to use the extension information to code primary tumor in preference to a statement of FIGO stage when both are available. TNM staging is closely related to FIGO stage, and the surgical findings of bladder invasion NOS in this case should be used in preference to the statement of T4.

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).

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.

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

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).

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