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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 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 histology as DLBCL (9680/3) as supported by the WHO Classification of Hematolymphoid Tumors, 5th edition. It is consistent with how it would have been coded in the 4th edition. The Heme Manual and Database currently are based on the 4th edition. Physicians are using the 5th edition blue book, whereas the cancer registry field is not yet at this time.

Regarding the Heme Manual and Database, this type of scenario is not covered because it is part of the 5th edition WHO Blue Book. The database cannot be updated until the 5th edition is approved for implementation (2026).

Report this case of leukemia cutis and code to bone marrow (C421) and leukemia NOS (9800/3) based on the information provided. Update the abstract if new information becomes available.

Leukemia cutis is the rare infiltration of neoplastic leukocytes into the epidermis, dermis, or subcutis from an existing leukemia that results in clinically identifiable cutaneous lesions. Leukemia cutis may precede, follow, or occur concurrently with the diagnosis of systemic leukemia. It is an advanced phase of the leukemia having a poor prognosis that also strongly correlates with additional sites of extramedullary involvement. This can alter the appropriate treatment regimen for a patient. It is a type of "metastasis" or spread of the leukemia cells. The "conventional" definition for leukemia cutis is the infiltration of skin from a bone marrow primary. It is most often diagnosed via skin biopsy—punch, shave, etc., utilizing IHC/biomarker testing and is commonly associated with CMML and acute myeloid leukemia (AML). As such, it a reportable condition especially when preceding a confirmed systemic leukemia diagnosis. In this situation, the diagnosis date would be the date of the positive leukemia cutis skin bx—punch, shave, etc. The case should be coded to C421; 9800/3 Leukemia NOS until the official systemic leukemia diagnosis is rendered. If possible, follow back should be conducted to determine the specific systemic leukemia histology (CMML; AML) and the treatment received. If the leukemia cutis follows or occurs concurrently with the diagnosis of a systemic leukemia, it is NOT a separate primary but merely an advanced stage of the systemic leukemia diagnosis.

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.

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.

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.

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.

Code as a localized, intracapsular tumor as ductal carcinoma in situ does not invade. Intraductal spread is describing the neoplasm spreading through the acinar/ductal cells in the prostate specimen. It is an in-situ type of spread and not invasive but almost always presents with an invasive tumor.