| Report | Question ID | Question | Discussion | Answer | Year |
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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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20210075 | Reportability: What American College of Radiology Reporting and Data Systems (RADS) can be used to determine reportability? See Discussion. |
LI-RADS (liver), PI-RADS (prostate), and TI-RADS (thyroid) can be used to determine reportability. BI-RADS (breast) and Lung-RADS cannot be used to determine reportability. Can these systems below to determine reportability? C-RADS (from CT colonography) NI-RADS (head & neck) O-RADS (ovarian-adnexal) |
The following cancer cases are reportable unless there is information to the contrary. –Liver cases with an LI-RADS category LR-4 (reportable since 2021) or LR-5 (reportable since 2016) –Prostate cases with a PI-RADS category 4 or 5 (reportable since 2017) The following are not reportable without additional information. –Breast cases designated BI-RADS 4, 4A, 4B, 4C or BI-RADS 5 –Lung cases designated Lung-RADS 4A," 4B, or 4X –Liver cases based only on an LI-RADS category of LR-3 –Colon cases with only C-RADS information (C-RADS category C4 is not reportable by itself) –Head and Neck cases with only NI-RADS information (NI-RADS category 3 is not reportable by itself) –Ovarian or fallopian tube cases with only O-RADS information (none of the O-RADS categories are reportable without additional information) –Thyroid cases with only TI-RADS information (none of the TI-RADS categories are reportable without additional information) |
2021 |
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20210027 | Reportability--Heme and Lympoid Neoplasms--Polycythemia vera: Is secondary polycythemia vera reportable? See Discussion. |
A physician stated the patient likely had secondary polycythemia vera due to cardiac and pulmonary conditions but that a polycythemia vera could not be ruled out. A JAK2 was ordered that was positive for JAK2 V617F mutation. The patient was treated with hydrea. According to SEER SINQ 20120049, secondary polycythemia vera is not reportable. However, in this case, the patient was positive for JAK2 V617F mutation. Therefore, is this reportable? We looked for guidance in the Hematopoietic and Lymphoid Neoplasms Database and found it confusing that secondary polycythemia vera was not mentioned or discussed under polycythemia vera in the database. The only thing we could find was secondary polycythemia NOS that was discussed under polycythemia. |
Abstract as a new primary for polycythemia vera, 9950/3. JAK2 is commonly used to assess suspected polycythemia vera and in this case, the mutation is positive for V617F. Based on the JAK2 results, this looks like a true polycythemia vera and not a secondary polycythemia. |
2021 |
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20210058 | Multiple Primaries/Histology--Lymphoma: What is the histology code and how many primaries are there based on a gastrohepatic lymph node biopsy that shows: Nodular lymphocyte-predominant Hodgkin lymphoma with T-cell/histiocyte rich diffuse large B-cell lymphoma (DLBCL)-like transformation. If two primaries, what is the diagnosis date for each primary? See Discussion. |
4/28/21 PET: There is extensive widespread/multifocal hypermetabolic uptake within lymph nodes, skeleton, and spleen, compatible with malignancy. Differential diagnosis includes lymphoma and metastatic disease of indeterminate primary, with lymphoma favored. 4/28/21 Right retroperitoneal lymph node, needle core biopsy: Large B-cell lymphoma. See comment. Comment: The differential includes T-cell/histiocyte-rich large B-cell lymphoma and diffuse variant of nodular lymphocyte predominant Hodgkin lymphoma. It is challenging to distinguish these two on the needle core biopsy. An excisional biopsy is recommended for a definite diagnosis if clinically appropriate. ADDENDUM: B-Cell Lymphoma, FISH: negative. No rearrangement of MYC, BCL2 and BCL6 and no fusion of MYC and IGH. 5/14/21 Gastrohepatic lymph node, biopsy: Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) with T-cell/histiocyte rich diffuse large B-cell lymphoma-like transformation. Focal in situ follicular neoplasia. 6/3/21 Medical Oncologist: Biopsy confirms that patient has a nodular lymphocytic Hodgkin lymphoma which has transformed into a T-cell rich DLBCL. This variant of Hodgkin disease is a good prognostic histology which generally behaves indolently, like a low grade lymphoma. |
We consulted with our expert hematopathologist who advised this is a single primary, Hodgkin lymphoma (9659/3). The diagnosis from 5/14/2021 states NLPHL. It also states there is T-cell histiocyte rich large B-cell lymphoma-like transformation. The WHO Classification of Hematopoietic and Lymphoid Tissues demonstrates six different patterns to NLPHL, which are: A) 'classical' nodular, B) serpiginous/interconnected nodular, C) nodular with prominent extra-nodular LP cells, D) T-cell-rich nodular, E) diffuse with a T-cell-rich background, and F) diffuse, B-cell-rich pattern. In this case, they are describing a NLPHL type E (diffuse with a T-cell rich background). The term used is "T-cell histiocyte rich large B-cell lymphoma-LIKE transformation. "Like" as used here means that it is like a transformation; if it was NLPHL transforming to T-cell histiocyte rich large B-cell lymphoma, it would not have the word "like" in the diagnosis. This is a variant of NLPHL and not an actual transformation to another lymphoma. Even though NLPHL can transform to T-cell histiocyte rich large B-cell lymphoma, it is not the case here since the word "like" appears in the diagnosis. We will update the histology in the Hematopoietic and Lymphoid Neoplasm Database to include these additional patterns. |
2021 |
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20210014 | Solid Tumor Rules (2018, 2021)/Multiple Primaries--Lung: How many primaries should be reported for a 4/2019 diagnosis of left upper lobe (LUL) adenosquamous carcinoma (left lingula mass biopsy: adenosquamous carcinoma; LUL lung biopsy: pulmonary adenocarcinoma, stated to be a collision tumor and single primary per the Tumor Board), treated with radiation followed by an enlarging LUL mass in 7/2020 found to be squamous cell carcinoma? See Discussion. |
The physician stated the prior LUL adenosquamous carcinoma was PD-L1 negative and the LUL squamous cell carcinoma is PD-L1 positive and is calling it a new primary. 5/22-7/3/19 6000x30 IMRT Photons LUL lung Chemo refused Not a Surg candidate 10/01/2019 CT Chest: IMP: In comparison to CT chest 03/06/2019 and PET/CT 03/21/2019, left lingular mass has mildly decreased in size. Left apical anterior and posterior lung lesions more anterior lesion appears slightly increased in size, the other slight decreased in size, with adjacent areas of atelectasis and scarring. 06/23/2020 CT Chest: MP: In comparison to CT chest 10/1/2019, left lingular mass has increased in size concerning for increasing tumor with adjacent thicker focal pleural thickening involving the chest wall, concerning for possible chest wall invasion. Left apical anterior and posterior lung lesions appears more solid in appearance, representing known adeno CA, given that the appearance has changed, is concerning for residual tumor. 07/06/2020 PET: Hypermetabolic lingular mass and peripheral nodularity has increased in size and FDG avidity on the prior PET/CT. Left apical nodular opacity is difficult to separate from fairly uniform mild left apical pleural hypermetabolism which may be treatment related and/or neoplastic. |
Abstract two primaries: 8560 and 8140 using rule M6. One of the original tumors with adenosquamous now shows only residual SCC following XRT. PD-L-1 is not used to determine multiple primaries. Assuming three tumors (the post-XRT SCC is not a new tumor but residual from one of the adenosquamous tumors) there are two primaries: 8560 and 8140 per M6. For collision tumors, each histology identified in the tumor is used to determine multiple primaries. |
2021 |
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20200033 | Solid Tumor Rules (2018)/Multiple primaries--Breast: How many primary tumors should be abstracted for a 2018 breast excision with a final diagnosis of invasive mucinous adenocarcinoma (0.7 cm) with ductal carcinoma in situ (DCIS) present as discontinuous foci, spanning 12 cm? See Discussion. |
If the term discontinuous foci means separate tumors, then rule M14 would apply making these multiple reportable tumors. |
Abstract two primaries, invasive mucinous and DCIS, using 2018 Solid Tumor Rules for Breast, M14, as the discontinuous foci are separate tumors in this example and the histologies are on different rows of Table 3 of the rules. |
2020 |
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20200064 | Primary site--Heme &Lymphoid Neoplasms: What is the primary site of two extraosseous plasmacytomas, with positive pathology of right orbit and left lung. The patient's bone marrow biopsy, flow, and peripheral blood smear were negative. Is this coded as 9732/3, multiple myeloma (Primary Site and Histology Rule PH2) with the primary site as C809 (PH27)? Or is the primary site C421 since code 9732 says primary site is always C421, though bone marrow came back as negative? |
Assign the primary site to C421 since that is the only allowable primary site for plasma cell myeloma, even though the bone marrow was negative. According to the revised criteria from the WHO Blue Book for Hematopoietic and Lymphoid Neoplasms (2017), the presence of multiple plasmacytomas is plasma cell myeloma (9732/3). |
2020 | |
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20200036 | Reportability--Skin: Is malignant proliferative trichilemmal tumor (PTT) reportable, and if so, do we apply the matrix rule and code it to 8103/3? A literature search reveals these do exist, but are extremely rare. |
Malignant PTT (8103/3) of the skin is not reportable. A neoplasm originating in the skin with histology coded to 8103 is not reportable. See 1.b.i. on page 7 in the 2018 SEER manual for a complete list, https://seer.cancer.gov/manuals/2018/SPCSM_2018_maindoc.pdf |
2020 | |
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20200030 | Solid Tumor Rules/Multiple primaries--Lung: How many primaries should be accessioned for the following patient scenario? 1) 09/2014 Left upper lobe (LUL), unifocal, localized acinar adenocarcinoma (8550/3) treated with lobectomy. 2) 04/2016 Right lower lobe (RLL), unifocal, localized acinar adenocarcinoma (8550/3) treated with wedge resection. 3) 04/2019 (within 3 years, but masked full date) Left lower lobe (LLL), unifocal, non-small cell carcinoma (8046/3) with brain metastasis. See Discussion. |
Rule M4 does not seem to apply because Note 1 defines clinically disease free to mean no evidence of recurrence in the same lung on follow-up. Patient had been disease free in the left lung after 09/2014 diagnosis. The 04/2019 diagnosis was in a different lung than the 4/2016 diagnosis. The next applicable rule is either M11 or M14 depending on how we should compare the new 2019 tumor: to the most recent prior tumor in 2016 or to both prior tumors. |
Abstract three primary tumors according to the 2018 Solid Tumor Rules as follows : 2014: LUL, single primary using M2 2016: RLL, multiple primary; abstract second primary using M11 (different lung) 2019: LLL, multiple primary after reapplying rules using M4 when comparing to the same lung in 2014. Abstract this tumor as it has been more than three years and it appears the patient had no clinical evidence of disease in the left lung until 2019. |
2020 |
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20200059 | Reportability--Kidney: Is Bosniak 4 cystic lesion of right kidney reportable, and would the first CT date be the date of diagnosis? See Discussion. |
CT a/p read by radiologist shows: "Bosniak 4 cystic lesion of right kidney." Follow-up MRI a month later reads "right kidney cystic lesion with enhancing mural nodule concerning for cystic renal cell carcinoma (RCC)." Urologist consult used the same wording of "Bosniak 4 cystic lesion" and "concerning for renal cell carcinoma." Treatment discussed but due to patient health status recommended repeat imaging. Repeat CT few months later reads: "cystic right renal lesion with enhancing nodule similar to most recent prior and suspicious for cystic RCC." Though "suspicious for cystic RCC" per latest imaging is reportable, Bosniak 4 is "clearly malignancy, ~100% malignant" by definition, so is the case actually reportable with the first CT a/p date as date of diagnosis? |
2023 Bosniak 4 is defined as "clearly malignant cystic mass." The case is reportable as of the first date it is diagnosed as a Bosniak 4 lesion unless further workup (especially biopsy or resection) disproves the CT findings. https://radiopaedia.org/articles/bosniak-classification-system-of-renal-cystic-masses?lang=us |
2020 |
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