Report | Question ID | Question | Discussion | Answer | Year |
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20190103 | Solid Tumor Rules/Multiple primaries--Brain and CNS: What M rule applies to a clinically diagnosed right-sided parietal meningioma undergoing active surveillance, followed by a left-sided frontal anaplastic oligodendroglioma? See Discussion. |
The patient has two, separate, non-contiguous tumors. One tumor is a benign meningioma and the other is a malignant oligodendroglioma. The original plan was not to treat the asymptomatic meningioma. However, after worsening symptoms, imaging and resection proved a separate left frontal lobe malignant tumor. Rule M5 is the only M Rule in the Malignant CNS Multiple Primary Rules, Multiple Tumors module that addresses separate non-malignant and malignant tumors. This rule provides only two criteria to follow when a malignant tumor follows a non-malignant tumor. The first criteria (for non-malignant tumor followed by malignant tumor) states: --Patient had a resection of the non-malignant tumor (not the same tumor) OR --It is unknown/not documented if the patient had a resection. This patient did not have a resection of the original, separate, non-malignant tumor, but the treatment plan was known to not include a resection. Should Rule M5 also apply to cases where the patient never had treatment planned for the separate non-malignant tumor? |
Apply 2018 Malignant CNS Solid Tumor Rule M5 and abstract multiple primaries when there are multiple CNS tumors, one of which is malignant /3 and the other is non-malignant /0 or /1. According to Note 3, a non-malignant CNS tumor and a malignant CNS tumor are always multiple primaries (timing and primary sites are irrelevant). Prepare two abstracts; one for the non-malignant and another for the malignant tumor. |
2019 |
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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 of 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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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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20230015 | Solid Tumor Rules/Multiple Primaries: Should two 2021 diagnoses be abstracted as two primaries? The patient has a history of thyroid cancer in 2008 with no evidence of recurrence/progression. In 2021, two abstracts were submitted with a diagnosis of C809, poorly differentiated malignant neoplasm and a C421, myeloproliferative disorder. See Discussion. |
2021-Right pleural fluid: Negative for carcinoma. 5/18/2021: Right iliac crest bone marrow core biopsy, aspirate smear, clot section and peripheral blood smear: Hypercellular bone marrow, morphological findings are suspicious for a myeloproliferative neoplasm. Flow Cytometry: Slight immunophenotypic abnormalities of the myeloid cells. No abnormal B cell, T cell, or NK cell populations identified. Normal female karyotype. KARYOTYPE: 46,XX[20]. Negative for deletion of 13q14.3 (D13S319) by FISH. Negative for deletion of 13q34 (LAMP1) by FISH. Negative for hyperdiploidy involving chromosome 9 by FISH. Negative for t(9;22)(q34;q11.2) by FISH. Negative for deletion of the EGR1 gene on 5q31 by FISH. Negative for monosomy 5 by FISH. Negative for deletion of 7q31 by FISH. Negative for monosomy 7 by FISH. Negative for deletion of 20q12 by FISH. Negative for trisomy of chromosome 8 by FISH. 6/4/21-Left adrenal; biopsy: poorly-differentiated malignant neoplasm with extensive necrosis. Immunohistochemical stains show the neoplastic cells to be negative for CK7, TTF-1 and p63. Negative CK7 and TTF-1 would argue against a lung primary. Correlation with clinical and radiological findings is advised. We are unable to contact the provider. |
Based on the diagnosis date for the unknown primary, use the 2007 MPH Other sites rules. Since the site codes differ for each primary, rule M11 applies, abstract two primaries. |
2023 |
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20240050 | Solid Tumor Rules/Multiple Primaries--Vulva: Why is there no M Rule in the Other Sites Multiple Primary Rules related to extramammary Paget disease of the vulva? See Discussion. |
The only Other Sites H Rule related to extramammary Paget disease is included in the Multiple Tumors Abstracted as a Single Primary module. Rule H28 instructs one to code the histology of the underlying tumor when there is extramammary Paget disease and an underlying tumor of the anus, perianal region, or vulva. Therefore, a vulvar extramammary Paget disease with underlying adenocarcinoma is coded as adenocarcinoma (8140/3), and not extramammary Paget disease (8542/3). However, there is no M Rule confirming extramammary Paget disease and the underlying adenocarcinoma are a single primary (i.e., multiple tumors abstracted as a single primary) making it difficult for one to use the Multiple Tumors Abstracted as a Single Primary H rules module. We recognize this is a longstanding histology coding rule, but how are registrars supposed to arrive at Rule H28 when the M Rules must be applied first and do not instruct one to accession a single primary? Moreover, if this is to be a single primary (per rule M2), why is there no H Rule in the Single Tumor module? |
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. |
2024 |
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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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20240033 | Solid Tumor Rules/Multiple Primaries--Stomach: Is a carcinoid tumor of the stomach diagnosed on 01/01/2023, on a patient who was followed up by Gastrointestinal (GI) and was found to have another stomach carcinoid on 02/01/2024, one primary or two? See Discussion. |
Based on the Solid Tumor Rules, we would make this two since it is over one year. According to a previous SINQ question 20110046, we are to code this as one primary. We see patients come back with multiple carcinoid tumors over the years and would like clarification. |
Stop at the first rule that applies which is M12. Per note 3: When it is unknown/not documented whether the patient had a recurrence, use date of diagnosis to compute the time interval. This means there are two primaries. There is a genetic syndrome that causes multiple carcinoid tumors in the GI tract, per our GI expert, and they should be treated as new primaries per M12. SINQ 20110046 describes a unique situation whereby the subject matter expert felt that the occurrence of multiple tumors was due to an unknown underlying condition driving the proliferation of neuroendocrine cells. |
2024 |
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20230017 | Solid Tumor Rules/Multiple Primaries--Rectum/Anal Canal: How many primaries are accessioned and how should histology be coded for a 2021 abdominoperineal resection showing invasive adenocarcinoma of distal rectum and associated Paget disease of the anal mucosa and perianal skin? See Discussion. |
The synoptic report calls this “Invasive adenocarcinoma with secondary Paget disease of anal mucosa and perianal skin.” The tumor size is listed as “2.1 x 1.7 x 0.7 cm, including associated advanced adenoma; size does not include the extent of the associated Paget disease, which extends for at least 2 cm distally.” Clinically this is called an incidentally discovered Paget’s disease. It is unclear if this is a collision tumor that should be abstracted as separate primaries, or if this is a single tumor with underlying Paget’s disease (similar to that described in Other Sites Rule H26). If this is a single rectal tumor, there does not appear to be an H rule for this scenario. |
Abstract two primaries using rule M4 of the Colon rules or rule M13 of Other Sites: 1. Invasive adenocarcinoma of distal rectum and 2. Paget disease of the anal mucosa / perianal skin (determine site of origin and code primary site accordingly). The rectum and the anus are separate sites and the histologies differ in each site. The WHO Classification of Digestive System Tumors, 5th edition, states that in addition to secondary anal Paget disease arising from anal canal adenocarcinoma, or rarely, adenoma without documented invasive disease, secondary Paget cells may be contiguous with the underlying neoplasm or manifest at different at sites distinctly away from it (with skip lesions). Document the details in the appropriate text fields. |
2023 |
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20230026 | Solid Tumor Rules/Multiple Primaries--Prostate: How many primaries should be abstracted, and which M rule applies when a patient is diagnosed with intraductal carcinoma of the prostate on biopsy followed by invasive adenocarcinoma on radical prostatectomy more than 60 days later? See Discussion. |
Example: A prostate core biopsy showed intraductal carcinoma in 09/2022, which is an in situ tumor. A core biopsy again showed intraductal carcinoma in 12/2022. The subsequent radical prostatectomy in 04/2023, revealed multiple foci of invasive prostate adenocarcinoma with extensive intraductal carcinoma. Per Solid Tumor Rules, Other Sites, Rule M3, acinar adenocarcinoma of the prostate is always a single primary. Note 4, this rule applies to subtype variants of acinar adenocarcinoma listed in Table 3, which has intraductal/ductal as a variant subtype of acinar adenocarcinoma. Does rule M3 apply to incidence cases (an invasive tumor following an in situ tumor)? |
Rule M1 applies because we don't know if there are separate tumors or separate foci within a single tumor. This is a single primary coded 8140/3. The prostate rules will be reviewed for an addition to cover this situation. |
2023 |
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20230012 | Solid Tumor Rules/Multiple Primaries--Prostate: How many primaries are accessioned when a 06/2022 diagnosis of prostate adenocarcinoma is followed less than one year later by a 01/2023 diagnosis of small cell carcinoma (SmCC)? See Discussion. |
Rule M4 was added to the Other Sites M Rules to address diagnoses of small cell carcinoma following prostate adenocarcinoma, but Rule M4 states the diagnoses must be greater than one year apart. In this situation, the diagnoses were less than one year apart and one must continue through the M Rules. The next M Rule that applies Rule M18: “Abstract multiple primaries when separate/non-contiguous tumors are on multiple rows in Table 2-21 in the Equivalent Terms and Definitions. Timing is irrelevant.” If one were to STOP at the first rule that applies, one would stop at Rule M18 which confirms the prostatic adenocarcinoma and small cell carcinoma are separate primaries, regardless of timing. If these are not to be accessioned as multiple primaries, does an Exception need to be added to M18? |
Assuming the smal cell is a seperate tumor, accession two primaries, adenocarcinoma (8140/3) of the prostate and SmCC (8041/3) of the prostate using Rule M18 of the current Other Sites Solid Tumor Rules. As these two tumors are less than a year apart, Rule M4 does not apply; however, Rule 18 does apply as these are two distinct histology types. It takes time for an acinar tumor to transform into the small cell and it is usually triggered by hormone and/or radiaiton treatment. |
2023 |