Report | Question ID | Question | Discussion | Answer | Year |
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20190043 | Diagnostic Confirmation: How is Diagnostic Confirmation coded for malignancies diagnosed by a FoundationOne Liquid biopsy/assay involving circulating tumor DNA in blood only? See Discussion. |
Example: FoundationAct assay of circulating tumor DNA in blood sample results: Tumor type = non-small cell lung carcinoma, NOS, with 3 genomic alterations identified: NRAS Q61H, IDH2 R140Q and TP53 V172F. The tumor was identified on imaging and the imaging findings were not clearly what one would expect to see with a SCLC. |
Code Diagnostic Confirmation as 7, Radiology and other imaging techniques without microscopic confirmation for this case. Results of a FoundationOne Liquid biopsy/assay are not specific enough to diagnose this lung malignancy. |
2019 |
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20190102 | Solid Tumor Rules/Histology--Head & Neck: What is the histology code of an external ear lesion when the dermatopathology report is the only available information (follow-up with the physician or pathologist is not possible) and the final diagnosis is malignant spindle cell neoplasm, most consistent with atypical fibroxanthoma? See Discussion. |
There are two histologies provided in the final diagnosis, malignant spindle cell neoplasm (8004/3) and atypical fibroxanthoma (8830/3). There is a definitive diagnosis of the non-specific histology, but the more specific histology is only described using ambiguous terminology. The external ear (C442) is included in the Head and Neck schema for diagnosis year 2018 and later. The Head and Neck Histology Rules indicate ambiguous terminology cannot be used to code a more specific histology. So ignoring the atypical fibroxanthoma, because it is modified by ambiguous terminology, we are left with a non-reportable site and histology combination (C442, 8004/3). Diagnoses of malignant atypical fibroxanthomas are regularly diagnosed using the syntax above in our area. Follow-up with the physician or pathologist is generally not possible as these cases are received from dermatopathology clinics only. The pathology report is the only information that will be received. If the reportable diagnosis of malignant atypical fibroxanthoma is ignored per the current Solid Tumor Rules, incidence cases will be lost. |
By definition, atypical fibroxanthoma (AFX) is a diagnosis of exclusion. Markers of specific differentiation must be negative. As written in your example, neither histology is reportable for skin. If possible, clarify the behavior of the AFX (8830/1) with the pathologist to determine reportability of the case. |
2019 |
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20190070 | Histology--Heme & Lymphoid Neoplasms: How is the histology coded for a when the pathologist notes the low grade B-cell lymphoma raises the possibilities of extranodal marginal zone lymphoma of mucosa associated tissue (MALT lymphoma) and lymphoplasmacytic lymphoma (LPL)? See Discussion. |
Rule PH28 confirms the more specific histologies are ignored if this is truly a low grade B-cell lymphoma (i.e., non-Hodgkin lymphoma, NOS) since both MALT lymphoma and LPL are more specific types of low grade B-cell lymphomas. This leaves only a diagnosis of low grade B-cell lymphoma with plasmacytic differentiation to consider. SINQ 20130033 states a low grade B-cell lymphoma with plasmacytic differentiation should be coded as 9680/3 (diffuse large B-cell lymphoma (DLBCL)). However, DLBCL is a high grade B-cell lymphoma, not a low grade B-cell lymphoma. If the pathologist classifies this as a non-specific low grade B-cell lymphoma, and clarifies that this may represent a more specific type of low grade B-cell lymphoma (MALT lymphoma or LPL), should the histology be coded to a high-grade lymphoma (DLBCL) or non-Hodgkin lymphoma, NOS? |
Code low grade B-cell lymphoma with plasmacytic differentiation as 9591/3 (Non-Hodgkin lymphoma, NOS). Plasmacytic differentiation is commonly seen with B-cell neoplasms. If further information identifies a more specific histology, the abstract can be updated to reflect the more specific histology. In the latest WHO Classification of Tumors of Hematopoietic and Lymphoid Tissues, 4th ed., there is confirmation that DLBCL is a high grade B-cell neoplasm. We will update the SINQ question. |
2019 |
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20190045 | Solid Tumor Rules (2018)/Multiple Primaries--Head & Neck: How many primaries are accessioned and what M Rule applies when a patient is diagnosed with a right lateral tongue (C023) tumor in 2016 that was verrucous carcinoma (8051), followed by a new left tongue border (C021) tumor in 2019 that was squamous cell carcinoma, NOS (8070)? See Discussion. |
According to the Multiple Primaries/Histology Rules in place at the time of the 2016 diagnosis, verrucous carcinoma was listed as a specific type of squamous carcinoma (Chart 1). However, in the current Solid Tumor Rules, verrucous carcinoma is not listed in Table 4 (Tumors of Oral Cavity and Mobile Tongue) either as a specific histology or as a specific subtype/variant of squamous carcinoma. The only subtype/variant listed for these sites is acantholytic squamous cell carcinoma (8075). Verrucous carcinoma is not listed in Table 4, making it unclear if it should be a different histology for these specified sites. However, verrucous carcinoma is listed as a specific subtype/variant of squamous carcinoma for other sites (e.g., Table 3). |
Accession a single primary based on the 2018 Head and Neck Solid Tumor Rule M13 as none of the other rules apply to the situation. Not all histology codes are contained in the tables in the Solid Tumor Rules as they list the more common histologies. Verrucous carcinoma is a subtype of squamous cell carcinoma according to Table 3 of the Rules. Solid Tumor rule tables are based on 4th Ed WHO Blue Books. Verrucous SCC is not included in oral cavity/mobile tongue chapter. |
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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20190010 | Reportability/Histology--Bladder: Is papillary urothelial neoplasm of low malignant potential (PUNLMP) (8130/1) reportable when also referred to as papillary transitional cell carcinoma, grade 1, no invasion (8130/2) previously? See Discussion. |
The pathology report reads: Urinary bladder, tumor over right ureteral orifice, biopsy: Urinary bladder mucosa (urothelium) and submucosa (lamina propria), with papillary urothelial neoplasm of low malignant potential (previously known as papillary transitional cell carcinoma, grade 1 of 3), no invasion identified. |
This case is not reportable. PUNLMP (8130/1) is the diagnosis stated by the pathologist for this case and PUNLMP is not reportable. The information in parentheses is informational in this case and does not change the pathologist's diagnosis. According to WHO Classification of Tumors of the Urinary System and Male Genital Organs, 4th edition, there is variation of architectural and cytological features between PUNLMP and papillary urothelial carcinoma, low grade, reflecting grading changes from an older classification system. |
2019 |
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20190076 | Primary Site/Brain and CNS: How is primary site coded when the ICD-O-3 provides a sub-site-associated morphology code and the only information available to code primary site for a particular diagnosis indicates a non-specific/not otherwise specified (NOS) site code? See Discussion. |
ICD-O-3 Rule H states to use the topography code provided when a topographic site is not stated in the diagnosis. This topography code should be ignored if the tumor arose in another site. For the following brain and central nervous system (CNS) examples, should the suggested sub-site codes be assigned based on the histology, or should the primary sites be coded as C719 (posterior fossa or suprasellar brain) since the only information available was a tumor in these non-specific sites? Example 1: Resection of a posterior fossa tumor proved medulloblastoma, WNT-activated. Although medulloblastoma has a site-associated code in the ICD-O-3 (C716, cerebellum), the only information available is that this was a posterior fossa tumor (C719). Example 2: Resection of a suprasellar brain tumor proved pineoblastoma. The pathologist labeled this as a brain tumor, suprasellar. Although pineoblastoma has a site-associated code in the ICD-O-3 (C753, pineal gland), the only information available is that this was a suprasellar brain tumor (C719). |
If possilbe, ask the physician(s) about the exact site of origin. If it is not possible to obtain more information, the information in the medical documentation takes priority over ICD-O-3 Rule H, even when that results in a less specific topography code. |
2019 |
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20190103 | Solid Tumor Rules/Multiple primaries--Brain and CNS: What M rule applies to a clinically diagnosed right-sided parietal meningioma undergoing active surveillance, followed by a left-sided frontal anaplastic oligodendroglioma? See Discussion. |
The patient has two, separate, non-contiguous tumors. One tumor is a benign meningioma and the other is a malignant oligodendroglioma. The original plan was not to treat the asymptomatic meningioma. However, after worsening symptoms, imaging and resection proved a separate left frontal lobe malignant tumor. Rule M5 is the only M Rule in the Malignant CNS Multiple Primary Rules, Multiple Tumors module that addresses separate non-malignant and malignant tumors. This rule provides only two criteria to follow when a malignant tumor follows a non-malignant tumor. The first criteria (for non-malignant tumor followed by malignant tumor) states: --Patient had a resection of the non-malignant tumor (not the same tumor) OR --It is unknown/not documented if the patient had a resection. This patient did not have a resection of the original, separate, non-malignant tumor, but the treatment plan was known to not include a resection. Should Rule M5 also apply to cases where the patient never had treatment planned for the separate non-malignant tumor? |
Apply 2018 Malignant CNS Solid Tumor Rule M5 and abstract multiple primaries when there are multiple CNS tumors, one of which is malignant /3 and the other is non-malignant /0 or /1. According to Note 3, a non-malignant CNS tumor and a malignant CNS tumor are always multiple primaries (timing and primary sites are irrelevant). Prepare two abstracts; one for the non-malignant and another for the malignant tumor. |
2019 |
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20190021 | Sequence Number Central--Brain and CNS: How is Sequence Number--Central coded for current/recent benign brain/CNS tumors when the patient has a history of an additional non-malignant CNS tumor diagnosed prior to 2004 (when these tumors became reportable to SEER)? See Discussion. |
We are confused by the SEER Program Coding and Staging Manual 2018 instruction that states: This sequence number counts all tumors that were reportable in the year they were diagnosed even if the tumors occurred before the registry existed or before the registry participated in the SEER Program. Does this rule apply to benign and borderline CNS tumors? Does this mean that any non-malignant CNS tumor diagnosed prior to 2004 should NOT be included in the sequencing (in the 60s range) if we were collecting non-malignant CNS per our State Registry reporting requirements prior to 2004? Example: Patient has a March 2017 diagnosis of right sided vestibular schwannoma (C724-1, 9560/0) and a prior history of left sided acoustic neuroma (c724-2, 9560/0) diagnosed in 1991. How should sequence be coded for each primary in our file? |
For your example, code the Sequence Number--Central as 61 for the 1991 diagnosis if this was a state registry requirement in 1991 and code 62 for the 2017 diagnosis. |
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 |