Report | Question ID | Question | Discussion | Answer | Year |
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20230070 | Solid Tumor Rules/Multiple Primaries--Breast: How many primaries should be accessioned for a diagnosis of invasive carcinoma of the left breast (8500/3) in 2020 followed by a 2023 diagnosis of dedifferentiated carcinoma in the left breast (8020/3)? See Discussion. |
The WHO Blue Books do not include dedifferentiated carcinoma as a valid histology for the breast. However, there is known to be progression of ductal carcinoma that is essentially dedifferentiation of an estrogen receptor, progesterone receptor, and HER2 breast carcinoma to a triple negative "dedifferentiated" carcinoma which it appears this patient has. Whether we should accession this as a separate 8020/3 primary per M14 is unclear and the Solid Tumor Manual does not address this scenario. |
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. |
2023 |
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20230009 | Solid Tumor Rules/Multiple Primaries--Vulva: How many primaries are accessioned when a 2023 diagnosis of keratinizing squamous cell carcinoma (SCC) (8071/3) of the vulva follows a previous diagnosis of nonkeratinizing SCC (8072/3) of the vulva and the timing rule (M12) does not apply? See Discussion. |
Table 19: Vulva Histologies of the Other Sites Solid Tumor Rules does not include entries for either keratinizing or nonkeratinizing squamous cell carcinoma in the “Squamous cell carcinoma, NOS” row. However, these are two distinctly different histologies per the ICD-O-3.2. All other Solid Tumor Rules schemas include an M Rule instructing one to abstract multiple primaries when separate/non-contiguous tumors are two or more different subtypes/variants in Column 3 of the Specific Histologies, NOS, and Subtype/Variants Table for the schema (e.g., Rule M6 for Lung). The timing of these tumors is stated to be irrelevant. The Notes confirm the tumors may be subtypes/variants of the same or different NOS histologies and tumors in column 3 are all distinctly different histologies (even if they are in the same row). However, the 2023 Other Sites schema appears to be missing this rule. Should these distinctly different histologies be accessioned as separate primaries? Is an M Rule missing from the Other Sites schema to address distinctly different histologies? |
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. |
2023 |
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20230034 | Update to Current Manual/Surgery of Primary Site 2023--Melanoma: Considering the 2023 melanoma surgery codes for punch biopsy NOS (B220) and shave biopsy NOS (B230), how is Date of First Surgical Procedure coded when the punch or shave biopsy is not excisional? See Discussion. |
Now that there are specific surgery codes for shave and punch biopsies, are these biopsies always the Date of First Surgical Procedure (NAACCR Item #1200)? Or should we still be applying the Surgery of Primary Site 2023 instruction in the 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? Example: On 01/01/2023, patient has a frontal scalp shave biopsy showing melanoma, margins involved. On 02/01/2023, frontal scalp excision shows residual melanoma. Surgery code is assigned B520 (shave followed by wide excision). How is Date of First Surgical Procedure coded now that there is an additional surgery code for the shave biopsy? |
Code the Date of First Surgical Procedure as 01/01/2023 in the example provided where the shave biopsy is followed by wide excision. Beginning in 2023, significant changes were made in that shave, punch, and elliptical biopsies are coded as surgical procedure regardless of margin status. Appendix C Skin Surgery Codes state that an incisional biopsy would be a needle or core biopsy of the primary tumor. Please see Appendix M: Case Studies for Coding Melanoma in STORE v23, Case study 2: Shave Biopsy followed by WLE (page 412), for an explicit example of how to code your example case. We will clarify this in the upcoming release of the SEER manual, |
2023 |
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20230064 | Primary Site--Cervix Uteri: When no other information is available regarding the origin of the tumor, can an overlapping cervical adenocarcinoma (C538, 8140/3) be coded to the endocervix (C530) based on the histology? See Discussion. |
Adenocarcinoma is a glandular tumor and the endocervix is generally the origin of glandular tissue for the cervix. However, if the only available information is pathology proving a single tumor overlapping the endocervix and exocervix, can we code the site to C530 instead of C538? Applying the current primary site coding instructions, primary site would be coded as C538 because there is no specific statement of the tumor origin; the primary site coding instructions state the tumor is coded to an overlapping site in the absence of a specific statement of origin and there is no existing SINQ confirming the site can be assumed to be the endocervix based on the histology. |
Code Primary Site as Overlapping lesion of cervix uteri (C538). The 2023 SEER Program Coding and Staging Manual Primary Site Coding Instructions for Solid Tumors #4 says to code the last digit of the primary site code to ‘8’ when a single tumor overlaps an adjacent subsite(s) of an organ and the point of origin cannot be determined. This is also supported by the ICD-O-3, 3rd edition, note in the Topography section that states: In categories C00 to C809, neoplasms should be assigned to the subcategory that includes the point of origin of the tumor. A tumor that overlaps the boundaries of two or more subcategories and whose point of origin cannot be determined should be classified to subcategory ‘8.” |
2023 |
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20230051 | First Course Treatment/Surgical Margins of the Primary Site--Melanoma: Is margin status positive or negative when the lesion “approximates” margins? This was noted in the pathology report comment on a malignant melanoma in-situ shave biopsy. Follow-up with physicians is not possible in this situation. |
Assign margin status as “positive” when stated as approximates margins as recommended by our expert pathologists. Approximating means coming right up to inked margin without the margin transecting the tumor. |
2023 | |
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20230075 | EOD/Summary Stage--Eye: How is stage coded for a patient with extranodal non-Hodgkin lymphoma involving bilateral choroids (single focus, both sites) and no lymph node involvement? Since the eyes are a paired site, is this two separate extranodal sites? If so, there are no Summary Stage or EOD tumor codes that best fit this scenario. |
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) |
2023 | |
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20230036 | Reportability/Histology--Vulva: Is angiomyxoma (8841/1), such as aggressive angiomyxoma of vulva diagnosed in 2022, reportable? |
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. |
2023 | |
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20230056 | Reportability/Histology--Heme and Lymphoid Neoplasms: What is the histology code for nodular lymphocyte predominant B cell lymphoma that is never called Hodgkin lymphoma? Is it acceptable to record the histology code for nodular lymphocyte predominant Hodgkin lymphoma, (9659/3)? See Discussion. |
Patient has a history of human immunodeficiency virus and diffuse large B cell lymphoma diagnosed in 2012, and is status/post systemic therapy and in remission since completing first course treatment. In 2022, the patient has imaging suspicious for recurrence. A biopsy of a deep left cervical lymph node showed atypical lymphoid infiltrate with the comment: “This is a challenging case. The constellation of findings is most in keeping with early / focal and subtle involvement by a nodular lymphocyte predominant B-cell lymphoma. We find no evidence of involvement by a diffuse large B-cell lymphoma.” The managing physician later states, “Cervical lymph node biopsy (06/2022) was consistent with nodular lymphocyte predominant B cell lymphoma.” |
According to the 5th edition WHO Blue Book for Hematopoietic Neoplasms, Beta Version, (not released yet), nodular lymphocyte predominant B-cell lymphoma is an alternate name for 9659/3. We will update the Heme database once the 5th edition is released in print. |
2023 |
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20230016 | Solid Tumor Rules/Histology--Brain: How is histology coded for an anaplastic glioneuronal tumor, BRAF p.V600E mutant, WHO Grade III, diagnosed following a right temporal lobe resection in 2021? See Discussion. |
The patient has a history of ganglioglioma, WHO grade I, involving the deep right parietal lobe diagnosed on resection in 07/2012. Tumor recurrence in 2017 was treated with radiation. The patient then had right temporal tumor biopsy and resection 06/2021 with final diagnosis of anaplastic glioneuronal tumor, BRAF p.V600E mutant, WHO Grade III. Pathologist notes that the tumor demonstrates a ganglioglioma with frequent mitoses and possible vascular proliferation. Subsequent consult findings support an anaplastic glioneuronal tumor, compatible with progression of the patient's ganglioglioma that is post-irradiation. However, the pleomorphic and epithelioid areas are also reminiscent of pleomorphic xanthoastrocytoma, which may occur in combination with ganglion cell components. There is no related SINQ to code this histology. |
Assign histology as 9505/3. WHO Classification of Central Nervous System (CNS) Tumors describe ganglioglioma as a well-diffferentiated and slow-growing glioneuronal neoplasm. While WHO does not recognize the histology/behavior combination 9505/3, the 2021 CNS Solid Tumor Rules identify non-malignant tumors that have the potential of transforming to a malignant tumor (new primary). Ganglioglioma (9505/1) is listed with the transformed histology and instructs us to code as anaplastic ganglioglioma (9505/3). |
2023 |
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20230007 | SEER Manual/Reportability--Appendix: Is low-grade appendiceal mucinous neoplasm (LAMN) with peritoneal spread followed by evidence of extraperitoneal metastatic disease reportable prior to 2022? See Discussion. |
In 2021, the patient was diagnosed with a non-reportable appendiceal LAMN. Resection showed a tumor diffusely involving the appendix and perforating the visceral peritoneum, as well as extensive intraperitoneal metastasis. In 2023, a lung wedge resection revealed metastatic mucinous neoplasm involving lung parenchyma and pleura, consistent with metastasis of the known appendiceal primary. It is understood that intraperitoneal spread of an appendiceal LAMN does not make it reportable because the peritoneal disease is also non-invasive. Does extraperitoneal metastasis of an appendiceal LAMN diagnosed prior to 2022 make it invasive disease and therefore reportable? |
LAMN diagnosed prior to 1/1/2022 is not reportable even when it spreads or metastasizes according to our expert pathologist consultant. Spread of this neoplasm does not indicate malignancy. For this case to be reportable, the diagnosis must indicate “carcinoma” or “adenocarcinoma.” Pre-2022, LAMN is not reportable even when treated with surgery and chemotherapy. LAMN is reportable starting with cases diagnosed in 2022. |
2023 |