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20190067 | Reportability/Histology--Breast: Is a breast mastectomy showing mildly atypical cells within the nipple epidermis which are suspicious for Paget disease of the nipple a reportable malignancy? See Discussion. |
Example: Left breast total mastectomy final diagnosis is incidental microscopic findings suspicious for early Paget disease of the nipple. The diagnosis comment states: The left breast mastectomy shows mildly atypical cells within the nipple epidermis which are suspicious for early Paget disease of the nipple. Additional sampling of the left breast was performed, and no evidence of atypical hyperplasia, in situ carcinoma, or invasive carcinoma within the left breast tissue was identified. Would this case be non-reportable using rationale similar to an early/evolving melanoma per SINQ 20180029? |
Code as 8540/3, Paget disease, based on the use of reportable ambiguous terminology (suspicious) listed in the 2018 SEER Coding Manual. In addition, Rule H8 of the 2018 Breast Solid Tumor Rules says to code Paget disease (8540/3) when the diagnosis is exactly Paget disease when a new tumor with no underlying tumor and the pathology documents invasive or unknown behavior. When two ambiguous terms are used and one is on the reportable list (suspicious) and one is not (early), accept the reportable term and report the case. See #1.b.ii on page 12 in the SEER manual, https://seer.cancer.gov/manuals/2018/SPCSM_2018_maindoc.pdf |
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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20190105 | Histology--Brain and CNS: What morphology code should be assigned to a low-grade glial/glioneuronal neoplasm? See Discussion. |
Pathology Diagnosis: Left temporal lesion - Low grade glial/glioneuronal neoplasm BRAF mutant. Pathologist Comment: The histopathological appearance of this lesion does not allow for a definitive diagnosis. However, the low-grade appearance, fibrillary nature, immunohistochemical profile, and the presence of a BRAF V600E mutation allow this to be categorized as a low-grade glial or possibly glioneuronal tumor. Despite the lack of exact classification this neoplasm can be expected to behave in a very indolent manner consistent with a WHO grade I classification. |
Assign 9413/0 for glioneuronal neoplasm. We consulted with our expert neuropathologist about the histology "glioneuronal neoplasm." This term is relatively new and has not yet been recognized by WHO or assigned an ICD-O code. Until such time that WHO determines a code for this neoplasm, our expert instructed us to use 9413/0. Since this is not a recognized neoplasm it is not included in the solid tumor rules. |
2019 |
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20190097 | Solid Tumor Rules (2018)/Multiple primaries--Lung: How many primaries are there and what M rules apply for multiple lung histologies in the left lower lobe (LLL) and right upper lobe (RUL) of the lungs? See Discussion. |
There is one tumor in the left lung that is acinar adenocarcinoma, 8551/3, and two tumors in the right lung, one of which is 8551/3 and a separate one that is mucinous adenocarcinoma 8253/3. 3/21/18- left robotic video assisted thoracoscopy with left lower lobe lobectomy: 2.5 cm adenocarcinoma, acinar predominant, margins negative 11/3/18- right upper lobe lobectomy: invasive mucinous adenocarcinoma, 1.7 cm, invasive adenocarcinoma, acinar predominant, 0.6 cm, margins negative If you start by comparing the 8551/3 left lung tumor to the 8253/3 right lung tumor, M6 applies and these would be separate primaries (seq 01 and seq 02). How would we handle the third tumor, 8551/3, in the right lung? Seq 01: 3/21/18- left lung primary 8551/3 Seq 02: 11/3/18- right lung primary 8253/3 Is the right lung tumor 8551/3 a third primary, and if so, which M rule applies? I cannot find a rule that seems to fit completely. Rule M6 may apply if you were comparing the right 8551/3 tumor to the seq 02 8253/3 tumor. But how would you know to use the seq 02 histology code 8253/3 or seq 01 histology code 8551/3 for the comparison? I think M9 was designed for situations where you have multiple tumors involving both lungs but they didn't biopsy all of them. Is that correct? If so, then we would be able to bypass M9. Would M11 apply since we already took care of two of the tumors with rule M6? If M11 doesn't apply, it seems like you would get to M14. |
Abstract two primaries applying Rules M6 and M9 s follows. First, assign a histology for each tumor. --LLL adenocarcinoma, acinar predominant 8551/3 --RUL invasive mucinous adenocarcinoma 8253/3 --RUL invasive adenocarcinoma, acinar predominant 8551/3 For the RUL, this is two primaries according to Rule M6, to subtypes in Column 3 of the histology table. For the LLL and RUL, this represents the same primary as these are the same histology according to Rule M9. |
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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20190029 | Reportability--Testis: Is demarcated scar tissue with atrophic seminiferous tubules and cortical bone consistent with burnt-out germ cell tumor and no evidence of germ cell neoplasia in situ (GCNIS) reportable? See Discussion. |
The patient is a 34 year old who presented with testicular pain radiating into the abdomen approximately 1 month before orchiectomy in 2018. CT abdomen/pelvis: Multiple focal sclerotic bone lesions. Given the lack of change from July 2014, these are likely benign bone islands. No adenopathy mentioned. He has no prior history of germ cell tumor nor any surgery for any tumor/cancer before this. Pathology: Testis, left, radical orchiectomy: - Demarcated scar tissue (1.3 cm), with atrophic seminiferous tubules and cortical bone consistent with burnt-out germ cell tumor. No evidence of germ cell neoplasia in situ (GCNIS). - Margins are unremarkable. |
Burnt-out germ cell tumor (9080/1) is not reportable. According to WHO Classification of Urinary System and Male Genital Organ, regressed germ cell tumors are germ cell tumors that have undergone partial or complete regression leaving a generally well-delineated nodular focus of scar or fibrosis in the testis. |
2019 |
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20190001 | EOD 2018/Summary Stage 2018--Brain and CNS: What are the Extent of Disease (EOD) Primary Tumor, EOD Regional Nodes, and Summary Stage 2018 codes for intradural schwannoma of the lumbar spine (L2-L4)? See Discussion. |
Example: Patient diagnosed following a resection of a cystic mass at L2-4 that proved an intradural tumor excision with final diagnosis of schwannoma, WHO grade 1. Per new Solid Tumor Rules, the primary site in this case should be coded C476 (peripheral nerves of trunk, NOS) and histology is 9560/0 (schwannoma, NOS). However, there are currently no coding options in the Soft Tissue of Trunk and Extremities EOD schema relating to a benign tumor. Likewise there are no coding options in the Soft Tissue and Sarcoma Summary Stage 2018 schema relating to a benign tumor. How should EOD 2018 and Summary Stage 2018 be coded for reportable benign schwannomas of the spinal nerve roots? |
The instruction regarding C476 has been removed from the Solid Tumor rules. Benign and borderline neoplasms coded to C470-C479 are not reportable at this time. Assign C720 for an intradural schwannoma at L2-4. That should allow you to use the correct EOD and Summary Stage 2018 schemas. |
2019 |
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20190095 | Reportability/Histology--Thymus: Is a thymoma a malignancy if described as having separate tumor nodules within peri-thymic adipose tissue? See Discussion. |
Patient had a thymectomy including pericardial fat for a mediastinal mass found incidentally during lung screening. Final diagnosis is WHO B3 thymoma. Staging Summary lists transcapsular invasion: "Present, as separate tumor nodules within peri-thymic adipose tissue." Tumor extension is stated to be "Confined to thymus, including peri-thymic adipose tissue." The pathologist staged this resection as pT1a pNX with no mention of mets. Clinically, there are no noted metastatic sites and no further treatment is planned. |
Report this case as a malignant thymoma. Our expert pathologist consultant reviewed this case and in his opinion, the "separate tumor nodules within peri-thymic adipose tissue" fit registry reporting criteria for separate tumor nodules making this a malignant thymoma. |
2019 |
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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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20190066 | Solid Tumor Rules (2018)/Histology--Breast: How is the histology coded for a metastatic carcinoma, consistent with primary breast carcinoma, when no other pathology information is available? See Discussion. |
The 2018 Breast Solid Tumor Rules Equivalent Terms and Definitions - Changes from 2007 Multiple Primaries/Histology Rules states: Mammary carcinoma is a synonym for carcinoma no special type (NST)/duct carcinoma not otherwise specified (NOS) 8500. It will no longer be coded as carcinoma NOS 8010. Should metastatic carcinomas of breast origin be 8500, or is code 8010 (carcinoma NOS) more applicable because histology coding from metastatic sites is not as reliable? |
Code as 8500/3 as it is the only tissue available for this carcinoma associated with a breast primary. Breast carcinoma NST/NOS is now coded as 8500. |
2019 |
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