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Do not report this case of "adenocarcinoma spectrum lesions" based on the information provided in the absence of a more specific diagnosis. Do not report until/unless a definitive diagnosis of malignancy is made. "Adenocarcinoma spectrum lesion" covers a continuum of lung neoplasms from preinvasive lesions (atypical adenomatous hyperplasia and adenocarcinoma in situ) to invasive lesions (minimally invasive adenocarcinoma and invasive adenocarcinoma).

Should additional information become available, report the case and assign the histology code if a more specific histology is confirmed later. Use text fields to record the details.

Abstract two separate primaries, ET (9962/3) and primary myelofibrosis (9961/3) using the current Hematopoietic and Lymphoid Neoplasms (Heme) Manual and Database (DB), Rule M15, use the Heme DB Multiple Primaries Calculator. Also refer to the example in Rule M15. In 1998, though the ET was not reportable (9962/1), the patient was treated with chemotherapy as a malignant neoplasm (9962/3). The Calculator instructs us to code separate primaries for these two histologies.

ET may evolve into a secondary myelofibrosis, also known as post-essential thrombocythemia-myelofibrosis (post-ET MF). The diagnosis must be stated as post-ET MF; this would be a single primary.

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 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.

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.

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.

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.

Abstract two primaries using Urinary Solid Tumor Rules, Rule M12. The histologies include non-invasive papillary urothelial carcinoma (8130/2) and non-invasive micropapillary urothelial carcinoma (8131/3). The two histology codes are listed as subtypes of Papillary urothelial (transitional cell) carcinoma in column 3 of Table 2.

WHO Classification of Urinary and Male Genital Tumors, 5th edition classifies micropapillary urothelial carcinoma as an aggressive subtype of urothelial carcinoma with carcinoma in situ present in more than half of all micropapillary carcinomas.

Rule 7 Note 3 of the Urinary Solid Tumor Rules states that there are no /2 subtypes for urothelial carcinoma with the exception of papillary urothelial carcinoma and applies to multiple occurrences of /2 urothelial carcinoma of the bladder.

Rule 8 applies to 8131/3 and 8120/3.

High grade squamous dysplasia (8077/2) is reportable for head and neck sites for cases diagnosed as of 01/01/2021. High grade glandular intraepithelial neoplasia / glandular intraepithelial neoplasia grade III (8148/2) and high grade squamous intraepithelial neoplasia / squamous intraepithelial neoplasia grade III (8077/2) are reportable for head and neck sites for cases diagnosed as of 01/01/2001. Refer to other standard setters’ criteria for reportability as appropriate.