Report | Question ID | Question | Discussion | Answer | Year |
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20190104 | Histology--Corpus uteri: Is 8020/3 used for a predominantly dedifferentiated carcinoma with focal well-differentiated endometrioid adenocarcinoma diagnosed in 2018? See Discussion. |
After a little research, it appears as though Endometrial Dedifferentiated carcinoma is a relatively new term and is set to be included in ICD-O-3.2: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577 If you look at the link on that page for All Additions, Changes, and Revisions to the ICD-O-3, 1st Revision for ICDO-3.2, there is 8020/3 Dedifferentiated carcinoma. Currently, 8020/3 is Carcinoma, undifferentiated, NOS. For 2018 diagnosis, would you use 8020/3 for a predominantly dedifferentiated carcinoma with focal well-differentiated endometrioid adenocarcinoma as stated in the pathology: Uterus, bilateral ovaries and fallopian tubes; supracervical hysterectomy/BSO: Predominantly dedifferentiated carcinoma with focal well-differentiated endometrioid adenocarcinoma in the endometrium, FIGO grade 1 Portion of omentum, omental/anterior abdominal wall/ round ligament/uterine/small bowel mesenteric tumor nodules all involved by dedifferentiated carcinoma. Synoptic reads as follows: Histological Type: Endometrioid carcinoma, NOS Dedifferentiated carcinoma predominantly Histological Grade: Endometrioid carcinoma, FIGO grade 1. |
Assign code 8380/3 for endometrioid carcinoma, NOS as this is listed as the histological type in the synoptic report. |
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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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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20190085 | Primary site/Histology: Are the 2018 ICD-O Histology Update topography codes intended to specify the most common sites for these new codes and can the histology be coded if they occur in other sites? See Discussion. |
Example 1: Endometrial biopsy final diagnosis is high-grade serous adenocarcinoma. Should we code this endometrial primary with histology 8441 (serous adenocarcinoma) because C54.X topography code is not listed in the applicable 2018 ICD-O-3 codes Histology Update for the new morphology, or should we apply the new histology code 8461 (high-grade serous carcinoma)? The NAACCR implementation guideline section 2.3 includes an important reminder that: Many of the new codes, terms, and behaviors listed in this update are site-specific and do not apply to all sites. Applicable C codes will be noted next to the term in bold font. However, this is followed by the more ambiguous instruction for edits that appear to imply the combination with non-listed sites is possible: These site- and histology-specific combinations will not be added to the Impossible combination edit. However, if a site other than the one listed with the morphology code is assigned, the result will be an edit requiring review. This is Interfield Edit 25. |
The NAACCR Guidelines for ICD-O-3 Histology Code and Behavior Update Implementation, effective January 1, 2018, state: Currently in ICD-O-3, when a topography (C code) is listed in parentheses next to the morphology term, it indicates morphology is most common to that site. It may occur in other sites as well. Many of the new codes, terms, and behaviors listed in this update are site-specific and do not apply to all sites. Please review the Comments to determine which histology codes are specific to sites. You may use sites not listed as the suggested site; however, it will generate an edit error for review and verification of the appropriate site. |
2019 |
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20190017 | Reportability--Heme & Lymphoid Neoplasms: The term indolent systemic mastocytosis is listed in the 2018 ICD-O-3 Histology Update table with borderline behavior (9741/1). However, smoldering systemic mastocytosis is listed in the Hematopoietic and Lymphoid Database (Heme DB) as an alternate name for histology 9741/3. Are smoldering systemic mastocytosis and indolent systemic mastocytosis synonymous? If so, should smoldering systemic mastocytosis also be removed from the Heme DB alternate names listing? See Discussion. |
In addition to the issue mentioned above, there is a SINQ answer that conflicts with the 2018 ICD-O-3 Histology Update table. SINQ 20130134 indicates indolent systemic mastocytosis is reportable for cases diagnosed 2010 and forward. There is no date restriction indicating the SINQ note applies only for cases diagnosed 2010-2017. Since indolent systemic mastocytosis was changed to borderline (9741/1) for diagnosis year 2018+, should the diagnosis year range be updated for this SINQ answer? |
Smoldering systemic mastocytosis is reportable, 9741/3. Indolent systemic mastocytosis is not reportable as of cases diagnosed 2018, 9741/1. Smoldering systemic mastocytosis and indolent systemic mastocytosis are not synonymous. Smoldering differs from indolent based on diagnostic criteria and burden of disease; indolent is low whereas smoldering is high burden of disease that can progress to aggressive systemic mastocytosis or mast cell leukemia. We will update SINQ 20130134. |
2019 |
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20190062 | Solid Tumor Rules (2018)/Histology--Brain: How is histology coded for a left frontal lobe mass when the final diagnosis is malignant neuroglial tumor and the diagnosis comment describes multiple possible histologies? See Discussion. |
Left frontal mass biopsy diagnosis comment states: Given the synaptophysin and patchy CD34 staining of these cells, the possibility of ganglioglioma and pleomorphic xanthoastrocytoma is raised. Astroblastoma and ependymoma were considered given the perivascular pseudorosettes, however GFAP staining is quite limited against these tumors. Reticulin stain shows limited perivascular reticulin staining however. Nevertheless, the necrosis, mitotic activity and elevated mitotic activity would point to a malignant neoplasm. Given the neural and limited GFAP staining, a generic classification of neuroglial is provided. This is the only available information. Further clarification or discussion with the physician or pathologist is not possible. Therefore, is this diagnosis of neuroglial tumor equivalent to that described in SINQ 20091037? |
Code to 8000/3. Use text fields to record the details. The WHO Revised 4th Ed CNS Tumors includes a chapter for "Neuronal and mixed neuronal-glial tumors. This chapter lists 13 histologies in this category. Glioneuronal NOS is not listed. Do not assign 9505 because ambiguous terminology was used AND because of the numerous possible histologies discussed for this diagnosis. |
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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20190084 | Histology/Heme & Lymphoid Neoplasms: Should the histology be coded to chronic myeloid leukemia (CML), BCR-ABL1-positive (9875/3) regardless of the quantitative analysis percentage of BCR-ABL1 that was detected? See Discussion. |
Example: Bone marrow biopsy diagnosis is chronic myelogenous leukemia, chronic phase, and the RT-PCR test result proved, BCR-ABL1 p210 (Major Breakpoint) - Detected, 3.3659%. Even though the p210 fusion transcript was less than 5%, it was detected. The presence of BCR-ABL1 does define whether or not patients are treated with tyrosine kinase therapies. Therefore, it seems likely that the presence of any BCR-ABL1 would be captured using the more specific histology code 9875/3, instead of the non-specific CML, NOS histology code 9863/3. Are there minimum threshold requirements for these quantitative studies in order to code the histology to the more specific type of CML? |
Code chronic myeloid leukemia (CML) BCR-ABL1-positive as 9875/3. According to the WHO Classification of Tumors of Hematopoietic and Lymphoid Tissues, 4th edition, CML BCR-ABL1-positive is characterized by the chromosomal translocation t(9;22) which results in the formation of the Philadelphia (Ph) chromosome containing the BCR-ABL1 fusion gene. The diagnosis requires detection of the Ph chromosome and/or BCR-ABL1. If the mutation is detected, regardless of percentage, it is positive. Quantitative levels of BCR-ABL are used to monitor response to tyrosine kinase inhibitor therapy. |
2019 |
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20190026 | Solid Tumor Rules (2018)/Multiple primaries--Bladder: Does Rule M11 in the 04/2019 Solid Tumor Rules Urinary update apply to synchronous/simultaneous tumors only or to multiple tumors with any timing? See Discussion. |
Rule M11 states: Abstract a single primary when there are urothelial carcinomas in multiple urinary organs, but neither the Rule nor the Notes describe the timing of these multiple urinary organ carcinomas. Timing requirements for other rules are clearly stated. Does Rule M11 have a timing requirement or is it intended to apply to all urothelial carcinoma tumors regardless of timing (and not already qualifying for application of a previous M rule)? |
The revised Urinary Solid Tumor Rules 2018 Rule M11, updated April 2019, removed the requirement of synchronous. This applies to urothelial carcinoma (8120) and its corresponding subtypes, regardless of behavior, that occur in more than one urinary site in a patient's lifetime. See change log for the April 2019 update to urinary rules.This is the same M/PH rule for multiple sites. Timing does not factor in to this rule. |
2019 |