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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20210071 | Solid Tumor Rules (2018/2021)/Histology--Breast: How is histology coded for a diagnosis of invasive mammary neuroendocrine tumor (NET), grade 2/3? See Discussion. |
Table 3 (Breast Equivalent Terms and Definitions) lists “Neuroendocrine tumor, well-differentiated” of the breast as histology 8246/3. There is no entry for a grade 2 neuroendocrine tumor of the breast in Table 3. The pathologist did not indicate the neuroendocrine tumor was poorly differentiated (or it would otherwise be a small cell carcinoma). The pathologist noted “By current WHO criteria, this tumor is characteristic of a mammary neuroendocrine tumor, grade 2. These invasive tumors have similar prognostic and predictive features of invasive ductal carcinoma of the same grade and stage.” |
Assign code 8249/3, neuroendocrine tumor, grade 2 based on the pathologist statement of mammary neuroendocrine tumor grade 2. According to WHO Classification of Tumors of the Breast, 5th edition, neuroendocrine tumor (NET) is an invasive tumor characterized by low/intermediate grade. If the histology term is not listed in the Solid Tumor rules, the instructions state to also check ICD-O and updates. Per ICD-O, NET, grade 2 is coded 8249/3. Breast Table 3 will be updated for 2023. |
2021 |
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20210062 | Histology/Reportability--Heme and Lymphoid Neoplasms: Is a case that is compatible with low grade myelodysplastic syndrome with multilineage dysplasia (MDS-MLD) reportable, and if so, is the histology plasma cell myeloma or myelodysplastic syndrome (MDS)? See Discussion. |
HL-7 e-path report, Final Diagnosis High normocellular marrow with maturing trilineage hematopoiesis, multilineage dyspoiesis, compatible with MDS-MLD and involvement by plasma cell neoplasm/myeloma, IgA kappa positive, approximately 20-25% of total cellularity present. See comment. Comments Correlation with other relevant laboratory (amount and type of serum and urine paraprotein levels, renal function tests, serum calcium level, and anemia) and radiologic (lytic bone lesions) findings is recommended for complete interpretation. Dyspoiesis of all lineages is seen and the findings are compatible with low grade myelodysplastic syndrome (MDS-MLD), assuming that other possible causes are excluded. Correlation with cytogenetic and molecular studies is recommended for complete characterization |
This case is reportable. Assign MDS, NOS (9989/3) based on the information provided for this case. “Compatible with” can be used for reportability; however, it cannot be used for assigning histology. There is no confirmed diagnosis of plasma cell myeloma/neoplasm; the comment specifically addresses the need for further evaluation of this case. |
2021 |
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20210011 | Primary site: Is C720 the correct primary site for a diagnosis of a paraspinal neuroblastoma on autopsy in a nine month old with Noonan syndrome? See Discussion. |
Autopsy/Pathology Report (2020) excerpts External Examination Nervous System: There is an 8.5 cm mass located in the right thoracic paraspinal area. Final Anatomic Diagnosis Clinical History: Paraspinal mass suspicious for neuroblastic tumor (detected by imaging studies) Nervous System: Right thoracic paraspinal neuroblastoma, poorly differentiated |
Assign primary site code C473 for this case based on the information provided (peripheral nerves and autonomic nervous system of thorax). From our expert pathologist consultant: The origin of neuroblastomas is generally in the adrenal medulla or one of the sympathetic ganglia on either side of the vertebral column (although they have been reported in many other locations given the migration of the neural crest cells embryologically). |
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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20210045 | Update to Current Manual/Neoadjuvant Treatment: What codes should be used for Neoadjuvant Therapy--Clinical Response and Neoadjuvant Therapy--Treatment Effect when the neoadjuvant therapy is still in progress at the time the case is initially abstracted as with rapid reporting. There is no code for neoadjuvant therapy still in progress and code 9 generates an edit for Neoadjuvant Therapy--Clinical Response. |
Assign code 8 for Neoadjuvant Therapy--Clinical Response and assign a code 9 for Neoadjuvant Therapy--Treatment Effect when the treatment is still in progress. Revise these codes after the treatment has been completed. We will update the manual to include these instructions. |
2021 | |
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20210018 | Reportability/Histology--Head & Neck: Is carcinoma cuniculatum of the hard palate diagnosed in 2017 reportable? Was this rare variant of squamous cell carcinoma (SCC) missed in Casefinding? If reportable, what is the histology code? |
Carcinoma cuniculatum of the hard palate is reportable. Code to SCC, NOS (8070/3). Use text fields to record the details. While WHO recognizes carcinoma cuniculatum to be a new variant of oral cancer, it has not proposed a new ICD-O code for this neoplasm. |
2021 | |
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20210010 | Reportability--Head & Neck: Is chondrosarcoma, grade 1 reportable for cases diagnosed 01/01/2021 and later? See Discussion. |
Neither the ICD-O-3.2 Implementation Guidelines nor the ICD-O-3.2 Coding Guidelines (including Tables 1-7) address reportability changes for chondrosarcoma grade 1. In the Solid Tumor Rules Manual, Head and Neck Equivalent Terms and Definitions, Table 7 (Tumors of Odontogenic and Maxillofacial Bone (Mandible, Maxilla)), Chrondrosarcoma grade 2/3 (9220/3) is included as a subtype/variant for sarcomas in these sites, but it does not address chrondrosarcoma, grade 1. The ICD-O-3.2 Coding Table lists Chondrosarcoma, grade 1 as morphology code 9222/1. If Chondrosarcoma, grade 1 is no longer a reportable tumor for cases diagnosed 01/01/2021 and later, why wasn't this reportability change included in the ICD-O-3.2 Implementation Guidelines? If the standard setters chose not to include this reportability change, shouldn't Table 7 also indicate that all chondrosarcomas (NOS, grade 1, grade 2 or grade 3) are reportable for cases diagnosed 2018 and later? How are registrars to make reportability and histology coding decisions for chondrosarcomas when neither source provides clear instructions regarding these tumors? |
Chrondrosarcoma, grade 1 (9222/1) is not reportable according to the Reportability section in the 2021 SEER Manual. The histology (9222/1) is listed in ICD-O-3.2 as a synonym for atypical cartilaginous tumor (preferred term). In general, the tables do not include non-reportable terms and codes. Registrars should refer to their standard setter (to whom they submit data) for reportable neoplasms. Currently, /0 and /1 neoplasms are reportable for central nervous system sites only. ICD-O-3.2 includes all neoplasms but that does not mean they are reportable. If a facility collects non-malignant neoplasms, use the corresponding ICD-O code in 3.2. |
2021 |
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20210076 | Reportability/Brain and CNS: Is a 2021 case of ecchordosis physaliphora (lesion within the prepontine cistern) on brain MRI reportable? |
Ecchordosis physaliphora is not reportable. |
2021 | |
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20210047 | Summary Stage 2018/EOD 2018--Colon: Does the 2018 SEER Summary Staging Manual, Digestive System Sites, Distinguishing In Situ and Localized Tumors for the Digestive System, #1. b., Exception, include in situ plus intramucosal carcinoma (involvement of the lamina propria and may involve but not penetrate through the muscularis mucosa) (penetration through the muscularis mucosa is behavior code 3.)? This seems to be in conflict with Extent of Disease (EOD) 2018. See Discussion. |
We are preparing to send our hospitals a reminder that the behavior changes from 2 to 3 at the bottom of the basement membrane, and the T category changes from Tis to T1 at the bottom of the mucosa for colon and rectum carcinomas. We are confused by the wording of the Exception. Distinguishing In Situ and Localized Tumors for the Digestive System 1.b. If the tumor has penetrated the basement membrane to invade the lamina propria, in which case it is localized and assigned Summary Stage 1 (localized) and for invasion of the lamina propria Exception: Code 0 (behavior code 2) includes cancer cells confined within the glandular basement membrane (intraepithelial); includes in situ plus intramucosal carcinoma (involvement of the lamina propria and may involve but not penetrate through the muscularis mucosa) (penetration through the muscularis mucosa is behavior code 3.) The text following (intraepithelial) is unclear. The question is: Does the text include in situ plus intramucosal carcinoma (involvement of the lamina propria and may involve but not penetrate through the muscularis mucosa) (penetration through the muscularis mucosa is behavior code 3.) mean the following: Code 0 (behavior code 2) includes in situ plus intramucosal carcinoma. In situ plus intramucosal carcinoma is involvement of the lamina propria, which may involve (but not penetrate through) the muscularis mucosae. Penetration through the muscularis mucosa is behavior 3. If that is what the text above means, then it seems that the 2018 SEER Summary Stage Manual is saying colorectal tumors reported as: adenocarcinoma in situ, at least intramucosal adenocarcinoma in situ, high grade dysplasia/intramucosal adenocarcinoma in situ, focally intramucosal at the margin are to be coded behavior 2 and SEER Summary stage In situ (0) like the intraepithelial carcinoma tumors. However, it conflicts with the EOD Data for Colon and Rectum, Note 2, and SINQ 20210006. The text for both EOD Data for Colon and Rectum and SINQ 20210006 is clear. According to them, the above bulleted adenocarcarcinoma examples are coded SEER Summary Stage localized (1) and behavior 3. SINQ 20210006 states that: For purposes of Summary Stage, intramucosal carcinoma is a localized lesion So, intramucosal carcinoma is coded SEER Summary Stage 1 (localized) and (behavior code 3). According to the text for EOD Primary Tumor, Colon and Rectum, Note 2 below, intramucosal, NOS involvement is invasive. Note 2: Code 050 (behavior code 3) includes the following: Intramucosal, NOS Lamina propria Mucosa, NOS Confined to, but not through the muscularis mucosa Thank you for your help clarifying the 2018 SEER Summary Manual Exception text above. |
For purposes of Summary Stage, intramucosal, NOS is a localized lesion. Intramucosal carcinoma is coded SEER Summary Stage 1 (localized) and (behavior code 3). The involvement of the following are assigned localized in Summary Stage and assigned a behavior code of 3. Intramucosal, NOS Lamina propria Mucosa, NOS Confined to, but not through the muscularis mucosa The Exception you cite may need to be reworded. We will review for the next version of the Summary Stage manual. |
2021 |