| Report | Question ID | Question | Discussion | Answer | Year |
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20230010 | Solid Tumor Rules/Multiple Primaries--Breast: How many primaries are accessioned when a 2020 diagnosis of invasive ductal carcinoma treated by lumpectomy is followed by a 2023 diagnosis of invasive lobular carcinoma treated by mastectomy? See Discussion. |
Historically, multiple invasive ductal and lobular carcinomas diagnosed within 5 years were abstracted as a single primary. However, it is not clear if Rule M10 or M14 applies to this situation per the 2023 Solid Tumor Rules updates. Rule M10 addresses multiple tumors of carcinoma of no special type (NST)/duct and lobular, but there is no timing criteria mentioned. Does M10 apply to cases diagnosed synchronously, or metachronously, or at least within 5 years? Should Rule M10 include a Note instructing registrars to accession a single primary for the scenario in question? If timing matters for Rule M10, then the next rule that applies is M14. Rule M14 instructs one to abstract multiple primaries when separate/non-contiguous tumors are on different rows in Table 3, and carcinoma NST/duct and lobular carcinoma are on separate rows in Table 3. |
Abstract a single primary using the Breast Solid Tumor Rules, Rule M10, assuming the tumors are in the same breast. This rule is specific to multiple tumors of carcinoma NST/duct and lobular. Timing is not a factor in this rule. As stated in ‘New for 2023,’ 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. Tumors occurring more than five years apart are multiple primaries and would have been caught at Rule M5. Thus, rule M10 pertains to tumors occuring less than five years apart. |
2023 |
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20230011 | Solid Tumor Rules/Multiple Primaries--Prostate: How many primaries are accessioned when a 2023 liver biopsy diagnosed metastatic small cell carcinoma (SmCC) of the prostate following a 2018 radical prostatectomy treated diagnosis of prostatic adenocarcinoma? See Discussion. |
SINQs 20190083, 20180088, and 20130221 all indicate diagnoses of prostate adenocarcinoma, followed by a diagnosis of metastatic small cell carcinoma of the prostate are separate primaries because these are distinctly different histologies. Does this logic still apply for 2023 and later since Rule M4 was added to the Other Sites M Rules? Rule M4 states, “Abstract multiple primaries when the patient has a subsequent small cell carcinoma of the prostate more than 1 year following a diagnosis of acinar adenocarcinoma and/or subtype/variant of acinar adenocarcinoma of prostate.” This patient has a 2018 diagnosis of prostate adenocarcinoma treated with radical prostatectomy, followed by a 2023 diagnosis of metastatic small cell carcinoma of the prostate diagnosed on a liver metastasis core biopsy. Rule M4 does not indicate whether it applies to subsequent biopsy confirmed metastatic tumor only. When a diagnosis of small cell carcinoma follows a diagnosis of prostatic adenocarcinoma, it is almost always confirmed in metastatic sites rather than in the primary site. Does the logic in the referenced SINQs above still apply for Rule M4? |
Accession two primaries, adenocarcinoma (8140/3) of the prostate and SmCC (8041/3) of the prostate using Rule M4 of the current Other Sites Solid Tumor Rules. The guidance in the aforementioned SINQ entries still applies with the additional criteria of being diagnosed more than one year following the diagnosis of acinar adenocarcinoma, or subtype, of the prostate as stated in Rule M4 of the updated 2023 rules. Small cell carcinomas of the prostate are often diagnosed on follow-up TURP/biopsies; however, if a patient had a previous radical prostatectomy, the small cell carcinoma would be identified in a metstatic site and would still be a new prostate primary. This includes biopsy confirmed metastatic tumors only. It remains important to capture the two distinct histology types. |
2023 |
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20230021 | Histology--Soft Tissue: How is histology coded for malignant neoplasm with neuroectodermal differentiation and TPR-NTRK1 gene rearrangement diagnosed on left shoulder excision? See Discussion. |
March 2022, left shoulder soft tissue mass excision shows a spindle cell tumor with outside consultation diagnosis of malignant neoplasm with neuroectodermal differentiation and TPR-NTRK1 gene rearrangement. Diagnosis comments indicate the findings most closely resemble the spectrum of kinase-rearranged mesenchymal neoplasms, such as lipofibromatosis-like neural tumor. However, the expression of SOX10 and mature melanocytic markers is unusual, and does not exclude melanocytic differentiation. Should this be classified as a peripheral neuroectodermal tumor (9364) or as an "NTRK-rearranged spindle cell neoplasm (emerging)" (8990) if there is a NTRK gene rearrangement? |
NTRK-rearranged spindle cell neoplasm is a newly identified variant of sarcoma; however, WHO has not yet proposed a specific ICD-O code for this rare neoplasm. Code to spindle cell sarcoma (8801/3). WHO defines NTRK-rearranged spindle cell neoplasm as an emerging group of molecularly defined rare soft tissue tumors that span a wide group of morphologies and histological grades, and are most often characterized by a spindle cell phenotype among other characteristics. |
2023 |
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20230022 | Solid Tumor Rules/Multiple Primaries: What M Rule of the updated Solid Tumor Rules, Other Sites, applies to a 2022 diagnosis of endometrial cancer, followed greater than one year later by a 2023 diagnosis of esophageal cancer with no interim evidence of tumor recurrence? See Discussion. |
These diagnoses were made greater than one year apart with a disease-free interval and M12 seems to be the first rule that applies. This rule does not specifically state the tumors diagnosed greater than 1 year apart must be in the same primary site but Note 1 could be interpreted as implying this. Note 1 states, “Clinically disease-free means that there was no evidence of recurrence in the same site on follow-up.” Does Other Sites Rule M12 (the timing rule) apply to tumors in different primary sites? It would be helpful if the notes specified this clarification, such as “Clinically disease-free means that there was no evidence of recurrence in the same site (same second and third character CXX.X) on follow-up.” |
Abstract multiple primaries using the Solid Tumor Rules, Other Sites, Rule M13. The topography differs at the second and third characters (C54.1 Endometrium; C15 Esophagus). Rule M12 refers to being disease-free vs. recurrence of a tumor, where Note 1 states that clinically disease-free means no evidence of recurrence in the same site on follow up. A note can be added to clarify that M12 applies to new tumors in the SAME site. |
2023 |
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20230049 | Update to Current Manual/Surgery of Primary Site 2023--Skin: Regarding the 2023 skin surgery codes for punch biopsy NOS (B220) and shave biopsy NOS (B230), how is Date of First Surgical Procedure coded for cutaneous lymphoma and Kaposi sarcoma 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? We are aware of the instruction for melanoma cases outlined in SINQ 20230034; however, it is unclear if this should also apply to cutaneous lymphomas and Kaposi sarcomas, or if the intent of the procedure is used for these specific types of skin cases that typically present with multifocal involvement. Example 1: Patient is diagnosed March 2023 with primary cutaneous T-cell lymphoma presenting as pink, tan patches on the trunk. Punch biopsy diagnosed CTCL and treatment was given via narrow band UVB phototherapy. Example 2: Patient is diagnosed February 2023 with Kaposi sarcoma presenting as widespread violaceous macules, papules, plaques on the torso, bilateral extremities, and abdomen. Punch biopsy diagnosed Kaposi sarcoma. |
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. |
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 |
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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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20230038 | Histology/Heme & Lymphoid Neoplasms--Mycosis Fungoides: What is the histology code for lymphomatoid papulosis that transforms initially to mycosis fungoides (MF)/cutaneous T-cell lymphoma (CTCL) and subsequently to CTCL with large cell transformation, and is it a new primary? See Discussion. |
Disease History 2018 - Lymphomatoid papulosis (non-reportable) 2020 - Transform to CTCL (and called Mycosis Fungoides specifically) (CTCL/MF same primary) 2021 - Transform to CTCL with large cell transformation |
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. |
2023 |
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20230065 | Solid Tumor Rules/Histology--Prostate: Is histology coded as 8045 (Combined small cell carcinoma) for a 2023 diagnosis of two-component carcinoma comprised of both acinar adenocarcinoma and small cell neuroendocrine carcinoma of the prostate? See Discussion. |
This patient does not have a previous diagnosis of prostate adenocarcinoma nor a previous history of androgen-deprivation therapy. Does the logic in the Other Sites Solid Tumor Rules (STRs) noted in SINQ 20200052 still apply? This SINQ confirms a diagnosis of mixed prostatic adenocarcinoma and small cell neuroendocrine carcinoma is 8045. This matches the STRs instructions for Rule H21 and Table 2 (Mixed and Combination Codes), row 1. Row 1 indicates a mixed small cell carcinoma and adenocarcinoma is combined small cell carcinoma (8045). For a patient without previous treatment, is this the correct mixed histology code? |
Code histology as combined small cell carcinoma (8045) based on the Other Sites Solid Tumor Rules, May 2023 Update, Table 2, Mixed and Combination Codes, for this mixed histology prostate carcinoma consisting of adenocarcinoma and small cell neuroendocrine carcinoma regardless of treatment status. This is similar to SINQ 20200052 that applies to one tumor with mixed histologies. |
2023 |
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20230023 | Solid Tumor Rules/Multiple Primaries—Brain and CNS: How many primaries are accessioned, and which M Rule applies, to a 2018 pituitary adenoma (8272/0) that was partially resected followed by a 2023 resection of residual disease proving pituitary adenoma/pituitary neuroendocrine tumor (8727/3)? See Discussion. |
The patient had residual tumor following the 2018 transsphenoidal resection and underwent an additional surgery after the residual tumor increased in size. Since pituitary adenoma/pituitary neuroendocrine tumor (PitNET) is a new malignant neoplasm for cases diagnosed 2023 and later, should this be a new primary per M5? Or do we disregard the change in behavior and apply rule M2 (single tumor is a single primary) for this scenario? |
This case does not fall into the standard rules. WHO criteria for diagnosing pituitary adenoma have recently changed (per 5th Ed WHO CNS book) and we will likely see more PitNET’ s than pituitary adenomas in the future. PitNET may be invasive or non-invasive but the likelihood of the pathologists providing this information is low. Since we don’t know if the 2018 adenoma was a PitNET based on current criteria or if it transformed to the malignant neoplasm, err on the side of caution and abstract a second primary per M5. This issue is new, and we’ve received numerous questions concerning pathologist reviewing older cases of pituitary adenoma and reclassifying them as PitNET using the new criteria. |
2023 |
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