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20190053 | Solid Tumor Rules (2018)/Histology--Brain and CNS: What is the histology code for a central nervous system (CNS) Ewing sarcoma family tumor with CIC alteration of the right parietal lobe? See Discussion. |
Table 3 (Specific Histologies, NOS, and Subtypes/Variants) lists Ewing sarcoma as a synonym for Peripheral primitive neuroectodermal tumor 9364. Presumably, this is to be used for the reportable malignant peripheral nerve tumors when diagnosed as pPNET or Ewing sarcoma. However, this patient has a type of central (or CNS) primitive neuroectodermal tumor (histology 9473). Table 3 does not list central primitive neuroectodermal tumor (PNET or CPNET) as a valid histology for CNS tumors. While Table 3 does not list all the possible histologies for the CNS, it currently is not clear how one would arrive at the histology code for a CNS Ewing sarcoma family tumor with CIC alteration, as this is recognized as a new entity for primitive neuroectodermal tumors of the CNS (i.e., PNET, histology 9473) per multiple journal articles. Ewing sarcoma family tumors include both peripheral PNET and central PNET tumors, but to code this histology as a peripheral PNET (9364) in this case seems incorrect when the primary tumor is stated to be of central nervous system origin, not peripheral nervous system origin. |
Code as 9364/3. WHO Classification of Tumors of the CNS, 4th edition, refers to Ewing sarcoma/peripheral primitive neuroectodermal tumor as a tumor of neuroectodermal origin involving the CNS either as a primary dural neoplasm or by direct extension from contiguous bone or soft tissues (such as skull, vertebra, or paraspinal soft tissue). |
2019 |
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20190086 | EOD 2018/EOD Primary Tumor--Melanoma: The code and level translations in the Note 4 of Extent of Disease (EOD) Primary Tumor for Melanoma Skin seem incorrect. Please advise. * Code 000: In situ * Code 100: Level I (should be level II) (< 0.75 mm Breslow's Depth) * Code 200: Level II (should be level III) (0.76 mm to 1.50 mm Breslow's Depth) * Code 300: Level III (should be level IV) (> 1.50 mm Breslow's Depth) |
Please see the corrected levels below for the note. Note 4: If a Breslow's depth is given in the pathology report and there is no other indication of involvement, the following guidelines may be used (Note: If a physician documents a different Clark's Level than provided by these guidelines, go with the physician's Clark Level) Code 000: Level I (In situ) Code 100: Level II (< 0.75 mm Breslow's Depth) Code 200: Level III (0.76 mm to 1.50 mm Breslow's Depth) Code 300: Level IV (> 1.50 mm Breslow's Depth) Thank you for bringing this to our attention. |
2019 | |
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20190033 | Update to current manual/Neoadjuvant therapy/Pathologic tumor size--Breast: When a patient with invasive breast cancer is started on neoadjuvant therapy and at surgery is found to have only residual in-situ disease, do we record the size of the in-situ tumor for Pathologic Tumor Size? See Discussion. |
I understand that we are to record the Clinical Tumor Size in Tumor Size Summary because of the neoadjuvant therapy, but the SEER manual does not address what to record in the Pathologic Tumor Size after neoadjuvant therapy. Would we record 999 or the size of the in-situ tumor in the Pathologic Tumor Size field? Will there ever be a new data item added or changes to this current data item? By recording the Patholigic Tumor Size this way, there currently will not be any way to compare tumor size clinically versus after neoadjuvant therapy and assessing the response. |
Note: this is an update to the 2018 SEER manual. Assign 999 in Pathologic Tumor Size when neoadjuvant therapy has been administered. We can explore the possibility of another data item in the future. |
2019 |
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20190063 | Solid Tumor Rules (2018)/Histology--Sarcoma: How is histology coded for a CIC gene rearrangement sarcoma? See Discussion. |
According to the literature, CIC gene rearrangement sarcomas in young patients are soft tissue sarcomas with an aggressive clinical course and may have previously been grouped under the Ewing-like family of tumors or as undifferentiated round cell sarcomas. There is currently no guideline in the solid tumor rules for coding a CIC gene rearrangement sarcoma. However, coding the histology to 8800 (sarcoma, NOS) seems unlikely to capture the more aggressive nature of these tumors. Can a more specific histology be coded? |
Code as undifferentiated round cell sarcoma (8803/3). The CIC rearrangement exists as a distinct molecular and clinical subset of small round cell tumors, and though similar, is felt to be a distinct entity from Ewing sarcoma. According to WHO Classification of Soft Tissues and Bone, 4th Edition, CID-DUX4 is a recurrent gene fusion associated with pediatric round cell undifferentiated soft tissue sarcoma (USTS). Although the genes involved in the fusion are different from those in Ewing sarcoma, the CIC-DUX4 protein has been shown to upregulate genes of the ETS family of genes thus providing a molecular link between Ewing sarcoma and round cell USTS. In contrast, there are strong arguments to suggest that Ewing-like sarcomas represent a separate and distinct entity. |
2019 |
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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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20190002 | Histology/Behavior--Brain and CNS: How should Histology and Behavior be coded for a polymorphous low-grade neuroepithelial tumor of the young (PLNTY) arising in the brain? |
Updated answer Assign code 9413/0. |
2019 | |
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20190050 | Reportability/Melanoma: Is evolving melanoma reportable with a Clark's level and Breslow's thickness are cited in the pathology report? See Discussion. |
How do we interpret the reportability of the following: The histological and immunohistochemical findings are most consistent with an early-evolving malignant melanoma, superficial spreading type, with Clark's level II and maximal Breslow thickness 0.33 mm, arising in association with an atypical nevus. Since a Clark's level and Breslow's thickness are included, is this reportable? Is this really an evolving melanoma? |
As of 01/01/2021, early or evolving melanoma in situ, or any other early or evolving melanoma, is reportable. |
2019 |
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20190108 | Primary site--Breast: how is subsite coded for a breast cancer when it is described as central portion between 1-3:00 or central portion at 12:00? |
See the SEER coding guidelines for breast, https://seer.cancer.gov/manuals/2018/AppendixC/Coding_Guidelines_Breast_2018.pdf Generally, codes C502 - C505 are preferred over C501. C501 would be preferred over C508. Apply these general guidelines when there is no other way to determine the subsite using the available medical documentation. Table 1, Primary Site codes, in the breast solid tumor rules also provide helpful information for coding site. |
2019 | |
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20190079 | Reportability/Histology--Pancreas: Is mucinous cystic neoplasm of pancreas reportable? |
Non-invasive mucinous cystic neoplasm (MCN) of the pancreas with low or intermediate grade dysplasia is NOT reportable. Non-invasive mucinous cystic neoplasm (MCN) of the pancreas with high grade dysplasia is reportable. For neoplasms of the pancreas, the term MCN with high grade dysplasia replaces the term mucinous cystadenocarcinoma, non-invasive. |
2019 | |
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20190094 | Reportability/Heme & Lymphoid Neoplasms--Skin: Is elephantiasis nostras verrucosa (ENV) reportable as a lymphoma? See Discussion. |
The autopsy report indicated a diagnosis of: Skin: Hyperkeratosis and pseudoepitheliomatous hyperplasia as well as reactive angioendotheliomatosis indicating Elephantiasis Nostras Verrucosa. |
Elephantiasis nostras verrucosa (ENV) is not reportable. ENV is a rare form of chronic lymphedema caused by any number of conditions including neoplasms, trauma, radiation treatment, congestive heart failure, obesity, hypothyroidism, chronic venous stasis, and parasitic infection. |
2019 |
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