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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.

Report VIN II. The 2024 SEER Manual lists this as a separate diagnosis in the Reportability section under Malignant Histologies 1.a.x. 

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. 

For each of your scenarios, the medical record information indicates that the case is not reportable based on physician opinion. Do not abstract these cases.

Remember that the ambiguous terms list is to be used as a last resort. The ideal way to approach abstracting situations when the medical record is not clear is to follow up with the physician. If the physician is not available, the medical record, and any other pertinent reports (e.g., pathology, etc.) should be read closely for the required information. See page 19 in the SEER Manual, https://seer.cancer.gov/manuals/2024/SPCSM_2024_MainDoc.pdf

Lymphoepithelial carcinoma is a subtype of SCC usually seen in skin or H&N sites and often associated with EBV. CPC SME review determined 8082/3 invalid for breast but did not recommend a substitute code. There were only 45 cases coded 8082 2001 to 2019. For this case, it's possible the lesion originated in the breast skin and progressed to breast tissue. SCC is a subtype of metaplastic breast carcinoma so one could argue it code be coded either 8575 or 8070. For this case, we recommend assigning 8500/3. Use text fields to record the details.

Sites may be added to certain combinations when indicated by ClinCORE review for Cancer PathCHART. Please note some sites were added in the 2024 update as a result of PathCHART review. A newly-formed Solid Tumor Editorial Board and its subgroups are currently working to evaluate the Solid Tumor Manual and make recommendations on ways to improve the structure and formatting of the manual and its content.

Follow the rules and instructions in the Other Sites STRs when assigning combination histology codes.

Histology Coding Rules

Use the Histology Coding Rules when assigning combination codes.

Coding Histology Information

Use this section that includes the mixed histology (Table 2) and site-specific histology tables (Tables 3-23) for one or more histologies within a single tumor. Do not use this section in place of the Histology Coding Rules.

While site-specific histology tables, based on current WHO Classification of Tumors books, have been added to Other Sites STRs, not all site groups have individual histology tables; coding may require the use of ICD-O and updates. The histology tables in Other Sites STRs include additional coding instructions and notes to assign the correct ICD-O code when appropriate.

The tables are not meant to be all-inclusive; rather they are intended to address difficult coding situations to facilitate the assignment of the correct histology code.

Table 2: Mixed and Combination Codes Instructions

Once you have identified the histology terms and have been instructed to use Table 2 by the Histology Coding Rules, compare the terms in the diagnosis to the terms in Column 1. When the terms match, use the combination code listed in Column 2. Use adenocarcinoma mixed subtypes 8255 as a “last resort” code.

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.

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.

Report LN II and LN III along with LIN II and LIN III and assign code 8520/2.

WHO Classification of Breast Tumors, 5th edition, lists lobular neoplasia as acceptable, related terminology for lobular carcinoma in situ. 

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.