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Do not report superficial angiomyxoma (8841/0) or aggressive angiomyxoma (8841/0).  WHO Classification of Female Genital Tumors, 5th edition, defines deep (aggressive) angiomyoma as a benign, infiltrative, myxoid spindle cell neoplasm that occurs in deep soft tissue of the pelviperineal region.

Abstract a single primary when there are multiple tumors of carcinoma NST/duct and lobular using the current Breast Solid Tumor Rules, Rule M10, May 2023 Update, for cases diagnosed 01/01/2018 and forward in the examples provided.  The rule also notes to follow the H rules to determine the correct histology code when a mixture of behaviors is present in carcinoma, NST and lobular carcinoma. Rule M5 does not apply as the timeframe is less than 5 years in both examples.

The 2023 update for the Breast Solid Tumor Rules (released November 2022) states: The rules for determining single versus multiple primaries in tumors with carcinoma NST/duct and lobular carcinoma have been revised and now align with ICD-O-3.2.  Applicable Histology Rules have also been revised to reflect ICD-O-3.2 histology terminology and corresponding ICD-O codes.

Code the Date of First Surgical Procedure (NAACCR Item #1200) as the date the shave, punch, or elliptical biopsy was performed. This instruction applies to cutaneous lymphoma and Kaposi sarcoma as well. Beginning with cases diagnosed 2023 and after, shave, punch, or elliptical biopsies are coded as a surgical procedure regardless of margin status.

The instruction in the 2023 SEER Manual that states "shave or punch biopsies are most often diagnostic; code as a surgical procedure only when the entire tumor is removed and margins are free/gross disease is removed" has been deleted from the 2024 SEER Manual.  Refer also to the Appendix C Coding Guidelines for Kaposi Sarcoma of All Sites and Lymphoma for coding primary site.

Assign as Stage IV as recommended by our expert hematological oncologist. This is a rare occurrence and this type of presentation does not fit the definition of intraocular extension. Stage IV is probably the best stage for this type of presentation, since there are two extranodal organs involved, even though they involve a bilateral site.

EOD Primary Tumor: 700

SS: 7 (Distant)

Assign 8077/2 for high-grade squamous intraepithelial lesion, VIN 3 in this case.  The WHO Classification of Female Genital Tumors, 5th edition, states that squamous intraepithelial lesions (SILs) of the vulva are also known as vulvar intraepithelial neoplasia, HPV-associated.  The term squamous cell carcinoma in situ is not recommended.

Assign code 500 for EOD Primary Tumor and code 30 for EOD Mets. This will correctly derive the T4aM1b stage based on AJCC 8th edition.

Abstraction of peritoneal metastasis changed from the T category in the AJCC 7th edition to the M category in the 8th and 9th AJCC editions. As a result, for cases diagnosed in 2018 and later, peritoneal deposits in the right lower quadrant should be abstracted as EOD Primary Tumor code 500 and EOD Mets code 30. However, the EOD Primary Tumor code of 600 has not yet been updated to align with the 8th and 9th AJCC editions. The 2025 updates will correct for this via a conversion for cases diagnosed in 2018 and forward where EOD Primary Tumor = 600 and EOD Mets = 00 or 10 to EOD Primary Tumor = 500 and EOD Mets = 30. Effective immediately, abstract peritoneal deposits in the right lower quadrant as EOD Primary Tumor code 500 and EOD Mets code 30, even though you will still have the ability to assign EOD Primary Tumor code 600 in your abstraction software until the 2025 updates are deployed.

Abstract a single primary using Breast Solid Tumor Rules, Rule M18, as none of the previous rules apply.

Undifferentiated carcinoma is a malignant epithelial tumour lacking overt evidence of a specific line of differentiation. Dedifferentiated carcinoma is composed of an undifferentiated carcinoma and a differentiated component. Dedifferentiated carcinoma (8020/3) as a morphology is associated with cancer of the endometrium and ovary rather than the breast.  Breast cancer shows a broad spectrum of morphology with extensive variation in histological type and grade, related to the complexity of carcinogenesis.  This includes initial genetic changes in the cell of origin, subsequent genetic and epigenetic alterations, and reprogramming that occur at various stages of development along with interaction of other factors that influence the process of differentiation.  This scenario likely represents the process of phenotypic change of a carcinoma at a later stage, better known as transdifferentiation.

Abstract a single primary and assign code 9700/3 for MF.  According to our subject matter expert, this is all MF.  When MF progresses, there can be large cd30 positive T cells. This is not the same as anaplastic large cell lymphoma.

Code Lupron as second course therapy and code active surveillance as first course therapy in this scenario. The 2023 SEER Manual states to code all treatment data items to 0 or 00 (Not done) when the physician opts for active surveillance, deferred therapy, expectant management, or watchful waiting.  Assign code 2 to Treatment Status. 

Active surveillance is not the same as "refusing treatment."  Active surveillance is a valid option offered to the patient.  The patient chose this option and later changed their mind.  This is not a refusal of recommended treatment.

Document all the details in the appropriate treatment text fields.

Table 19 is based on WHO 5th Ed Tumors of vulva and squamous cell variants, keratinizing and non-keratinizing, are no longer recommended and are excluded from the 5th Ed. HPV related terminology is now preferred for these neoplasms.

Per consultation with our GYN expert pathologist, based on the information provided, this is likely a single tumor that was not completely excised in the original biopsy. A new tumor in the same site would not appear within 8 months. If you cannot confirm two separate/non-contiguous tumors were present, abstract a single primary per M1. As for histology, the tumor showed both keratinizing and non-keratinizing features and HPV status is unclear. Per our expert, code to SCC 8070/3—keratinization or lack of does not change treatment or prognosis.

Even If there is proof of separate/non-contiguous tumors, our expert still feels this is a single primary coded to SCC 8070/3. Treatment does not differ by keratinization or HPV status. Coding two primaries would be incorrect and inflate incidence rates.

Per our expert, this is an unusual occurrence. The rules cover 85% of cases but there will always be situations that do not fit a rule. This case is an example of that.  A new GYN specific Solid Tumor Rules module is under development and a rule to address this situation could be included.