Report | Question ID | Question | Discussion | Answer | Year |
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20091053 | Multiple Primaries--Breast: How many primaries should be reported when a lobular carcinoma with positive margins is followed 8 years later by a lobular carcinoma near the previous lumpectomy site? See Discussion. |
Left breast invasive lobular ca diagnosed 3/00 and treated with a lumpectomy, but with multiple positive margins; she received no post operative radiation or other medical treatment (unknown why). 10/08 core biopsy of "an area of distortion" near the scar site is positive for invasive lobular ca. The radiologist states "compatible with recurrence at her previous lumpectomy site" on an x-ray report. One thought is that this should not be a new primary because the patient was never disease free (multiple positive margins) and the patient received incomplete treatment. Or should this be a new primary because the tumors are diagnosed more that 5 years apart? |
Abstract the 10/08 diagnosis as a new primary, per Breast rule M5. In spite of the positive margins and apparently incomplete treatment in 3/00, there is no mention of the presence of disease between 3/00 and 10/08 according to the information provided. |
2009 |
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20210002 | Multiple Primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned for a patient diagnosed with therapy-related myelodysplastic syndrome (t-MDS) (9920/3) in 2015 followed by a 2020 diagnosis of myelodysplastic syndrome, NOS (MDS, NOS) (9989/3)? See Discussion. |
Patient has a history of B-cell lymphoma with multimodality treatment in 2002. Lab work in 2015 showed multilineage dysplasia leading to a diagnosis of therapy-related myelodysplastic syndrome. Patient presents in 2020 for a bone marrow biopsy now showing low-grade MDS. The MDS appears to have the same multilineage dysplasia previously identified. MDS, NOS is not listed in the Heme DB as a possible transformation of t-MDS, nor is it listed as a Same Primary for t-MDS. Likewise, t-MDS is not listed as a more specific myelodysplastic syndrome, a transformation of MDS NOS, or a Same Primary as MDS, NOS. The first M rule that applies to this case is M15, and the Multiple Primaries Calculator indicates that the MDS, NOS should be a new primary. |
Abstract separate primaries using Rule M15 of the Hematopoietic and Lymphoid Neoplasms (Heme) Manual. The Heme Database states: Excluded from this category are progression of myeloproliferative neoplasms (MPNs) and evolution of primary MDS or primary MDS/MPN to acute myeloid leukemia (AML); in each of these latter cases evolution to AML is part of the natural history of the primary disease and it may be impossible to distinguish natural progression from therapy-related changes. There is no indication of transformation. |
2021 |
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20180087 | Solid Tumor Rules (2018)/Multiple Primaries--Brain: How many primaries are there and what M Rule applies when two tumors identified in the brain are pathologically proven to be glioblastoma, IDH-wild type and anaplastic astrocytoma per the pathology report final diagnosis, but the diagnosis comment and tumor board indicates multifocal glioblastoma is favored? See Discussion. |
The patient has one tumor each in the left parietal and left medial temporal lobe. The tumors were excised. The final diagnosis for the left parietal tumor is glioblastoma, IDH-wild type. he final diagnosis of the left medial temporal tumor is, at least anaplastic astrocytoma, WHO grade III; see comment. The comment states: There is a single focus of vascular hyperplasia, separate from neoplastic cells. No necrosis is identified. These findings on their own would warrant a diagnosis of anaplastic astrocytoma, WHO grade III. However, in the context of the patient's glioblastoma in the left parietal lobe, and imaging showing ring-enhancing lesions of the parietal and temporal lobes, this specimen is favored to be an un-sampled glioblastoma, WHO grade IV. The Solid Tumor Rules indicate we may no longer use terms like favor(s) to code the histology, leaving the final diagnosis as the priority source for coding histology per the Histology coding rules. The tumor board review confirmed that, despite the anaplastic astrocytoma on pathology, they felt strongly that this is a multifocal glioblastoma and not an anaplastic astrocytoma. Both the pathologist's comment and the tumor board's assessment indicate this patient does not have two primaries. However, the Solid Tumor Rules do not give priority to the tumor board's assessment over the pathology, and registrars are not to use ambiguous terms to code histology thus leaving the two histologies to consider. Per the Solid Tumor Rules, one tumor that is glioblastoma and one tumor that is anaplastic astrocytoma are multiple primaries per M11 (Abstract multiple primaries when separate, non-contiguous tumors are on different rows in Table 3 in the Equivalent Terms and Definitions. Timing is irrelevant). As a central registry, we cannot ask the pathologist or attending physician for clarification as suggested in Section 3 of the Malignant CNS and Peripheral Nerves Equivalent Terms and Definitions. We can only follow the current Solid Tumor Rules. In doing so, we would have to ignore both the pathologist's and tumor board's assessment that this patient has multifocal glioblastoma. Is there any concern that this will lead to over-reporting? |
Abstract separate primaries based on the two histology codes as these are separate tumors on different rows in Table 3 of the 2018 Solid Tumor Rules for Malignant CNS, Rule M11. The priority order for using documentation to identify histology for Malignant CNS is to use pathology/tissue from the resection over the tumor board. |
2018 |
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20190073 | Solid Tumor Rules (2018)/Multiple primaries--Lung: How many primaries should be reported for a patient with a March 2018 diagnosis of non-small cell carcinoma with neuroendocrine differentiation on lung biopsy (single left upper lobe tumor only) who also has a prior history of left lung squamous cell carcinoma in 2016 (treated with chemotherapy/radiation)? See Discussion. |
The Solid Tumor Rules instruct us not to use differentiation for coding histology unless it is specifically listed in the table. The terminology non-small cell carcinoma with neuroendocrine differentiation is not in lung histology Table 2. However, SINQ 20150033, prior to Solid Tumor rules, indicates this diagnosis should be coded to 8574 (adenocarcinoma/carcinoma with neuroendocrine differentiation). This presentation appears to represent distinctly different histologies. However, because the 2018 histology diagnosis is not in the table and the prior SINQ appears to disagree with current instruction, it is not clear how to apply the M rules to this case. The outcome of the histology coding will affect the number of primaries reported in this case. |
Abstract separate primaries according to the 2018 Lung Solid Tumor Rules. Lung Table 3 is not an exhaustive list of lung histologies and the H rules instruct you to use the tables, ICD-O and/or ICD-O updates. Per ICD-O-3, carcinoma with neuroendocrine differentiation is coded to 8574/3; whereas, squamous cell carcinoma is coded to 8070/3. These represent distinct histologies on different rows in Table 3. |
2019 |
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20180035 | Solid Tumor Rules (2018)/Multiple Primaries--Lung: How many primaries should be abstracted in this 2018 lung case? See Discussion. |
CT chest findings: 1. There is a dominant 1 cm. nodule in the left mid lung. 2. In addition, there is a new rather dominant bilobed nodule in the left lung base. 3. Distant metastases are not identified. Four months later, a doctor's note says routine follow-up visit status post Cyber Knife stereotactic body radiation therapy for synchronous early stage non-small cell carcinomas of the left upper and left lower lobes, both Stage IA. He is medically inoperable. This situation is described as a second primary tumor in AJCC8 page 438. However, by the 2018 Lung Solid Tumor rules, this would be a single primary, per rule M7. Is that correct? |
Abstract one primary per Rule M7. Follow the Lung Solid Tumor Rules to determine the number of primaries. The AJCC TNM manual is used for staging. Do not apply AJCC instructions to determine the number of primaries. |
2018 |
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20190098 | Solid Tumor Rules (2018)/Multiple primaries--Breast: How many primaries are there and how is histology coded for a breast primary showing encapsulated papillary carcinoma and Paget disease of the nipple? See Discussion. |
Patient has a 1.7 cm encapsulated papillary carcinoma staged as pTis located 2 cm from the nipple and Paget disease of the nipple on mastectomy pathology. There is no indication in Table 3: Specific Histologies, NOS/NST, and Subtypes/Variants that encapsulated papillary carcinoma is a subtype of ductal carcinoma. Rule M8 notes that if the histology of the underlying tumor is any histology OTHER THAN duct or subtypes of duct, one should continue through the rules. But if M9 applies to this case, then incidence reporting will be increased in comparison to prior years. |
Abstract multiple primaries when there is Paget disease (8540/3) and an underlying tumor that is not duct, in this case, encapsulated papillary carcinoma (8504/2) using Rule M9 of the 2018 Breast Solid Tumor Rules. |
2019 |
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20190037 | Solid Tumor Rules/Multiple Primaries--Breast: How many primaries should be abstracted for simultaneously diagnosed non-contiguous invasive duct carcinoma and mucinous carcinoma? Does rule M12 apply since the two histologies are on different rows of Table 3 of the Breast Solid Tumor Rules? See Discussion. |
Core biopsy of left breast at 2:00: Invasive ductal carcinoma, Nottingham score 6/9. Core biopsy of left breast at 4:00: Invasive mucinous carcinoma (variant of ductal carcinoma), Nottingham score 5/9. Post neo-adjuvant mastectomy: Main (largest tumor): Invasive ductal carcinoma, upper outer quadrant grade 2. Secondary tumor: mucinous carcinoma, grade 1 at 4:00. |
Abstract multiple primaries when separate, non-contiguous tumors are on different rows in Table 3 of the Breast Solid Tumor Rules. Use Rule M14 as each row in the table reflects a distinctly different histology, in this case, invasive ductal carcinoma (8500) and mucinous carcinoma (8480). |
2019 |
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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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20200005 | Multiple Primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned and what M rule applies when a patient is diagnosed with both plasmablastic lymphoma and at least one plasmacytoma? See Discussion. |
The patient was diagnosed with an EBV-positive plasmablastic lymphoma involving the left testis on radical orchiectomy in April 2019. In September 2019, a plasmacytoma was found on a right mandibular mass biopsy. Imaging at that time revealed diffuse disease involving the thoracic spine and sinus involvement. The patient then underwent a resection of the T8 spinal/epidural tumor that also proved plasmacytoma. Subsequently, the right mandibular mass and testis slides were reviewed (at an outside facility) and both were stated to be, The T8/epidural tumor pathology was not reviewed, so it is unclear if this is also assumed to be the same disease process as the right mandibular mass or still a separate, solitary plasmacytoma. Additionally, some chart notes indicate the patient has plasmablastic lymphoma with a secondary diagnosis of plasmacytoma, while other chart notes state this is stage IV plasmablastic lymphoma involving all documented sites. Although the plasmablastic lymphoma and at least the plasmacytoma of T8 have different ICD-O-3 histology codes, the physicians do seem to be treating this as a single disease process. |
Abstract multiple primaries using the Heme and Lymphoid Rule M15. The Multiple Primaries Calculator shows that the plasmablastic lymphoma (9735/3) and extraosseus plasmacytoma (9734/3) are separate primaries. We also checked with our expert pathologist who concurs as the spinal lesion was not reviewed to prove that it is plasmablastic lymphoma, therefore, the diagnosis as per pathology remains plasmacytoma. |
2020 |
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20200010 | Solid Tumor Rules (2018)/Histology--Head & Neck: How is histology coded for a glossotonsillar sulcus tumor with both squamous cell carcinoma and mucoepidermoid carcinoma? See Discussion. |
Patient had a radical pharyngectomy showing a glossotonsillar sulcus tumor with high grade squamous cell carcinoma and adjacent high grade mucoepidermoid carcinoma. The pathologist commented, the tumor is composed of high grade mucoepidermoid carcinoma and high grade conventional-type squamous cell carcinoma that are immediately adjacent to one another. Given that the tumors are arising so close together and could represent a single neoplastic process with divergent morphologies, they are staged together. Employing Solid Tumor Manual Rule M1 (single primary if unable to determine if there is a single or multiple tumors), it was determined that this should be reported as a single tumor because the pathologist referred to the case as both a tumor singular and tumors pleural. However, the Solid Tumor Manual Histology Rules for a Single Tumor do not appear to have an instruction for coding this histology combination. |
Abstract multiple primaries using 2018 Head and Neck Solid Tumor Rule M8 as these are separate tumors described as arising close together, and are on different rows in Table 3. Code histology separately as squamous cell carcinoma (8070/3) and mucoepidermoid carcinoma (8430/3). This appears to be a collision tumor. Collision tumors are counted as two individual tumors for the purpose of determining multiple primaries. Collision tumors were originally two separate tumors that arose in close proximity. As the tumors increased in size, they merged or overlapped each other. While more common in the colon, they can occur in other sites as well. |
2020 |