Report | Question ID | Question | Discussion | Answer | Year |
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20190064 | Multiple Primaries--Heme & Lymphoid Neoplasms: Patient is diagnosed with myelodysplastic syndrome (MDS) with an early/evolving acute myeloid leukemia (AML) thought to be treatment related. Does rule M11 apply since there are two biopsies within 21 days, and therefore, two primaries, or one primary (9920/3)? See Discussion. |
Patient has a history of breast cancer and diffuse large B-cell lymphoma (DLBCL), both treated with chemotherapy and radiation. On 6/26/19, bone marrow biopsy: MDS with excess blasts-2 (18% dysplastic blasts) in a normocellular marrow (overall 40% cellularity) with trilineage dysplasia. Comment: least myelodysplastic syndrome with excess blasts-2. However, an early/evolving AML cannot be completely excluded. The findings likely represent therapy-related myeloid neoplasm. MD note on 7/15/19: Diagnosis: MDS, high grade borderline AML with complex karyotype secondary disease. Patient has high grade MDS which is bordering on AML transformation with 20% blasts by IHC and areas higher than this. This is likely secondary to the treatment she has received for her other cancers particularly pelvic radiation for her DLBCL. Given her very high IPSS score, it is likely she will eventually develop AML. No treatment given. On 7/15/19, bone marrow biopsy: Persistent acute leukemia in a marrow with trilineage dyspoiesis and 23% blasts. |
Code as one primary (9920/3). This case does not fit the rules very well, since it is a treatment-related neoplasm and involves a transformation of MDS to AML during the clinical workup. Per the abstractor notes for 9920/3, code 9920/3 when the physician comments that the neoplasm is treatment related. This can be for the MDS or the AML. Use text fields to document that it was first referred to as MDS and then transformed to AML. If you followed the rules strictly and coded this as two primaries (the MDS and AML), you would lose the information that this was treatment related, which is more important. |
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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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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20190061 | Solid Tumor Rules (2018)/Multiple primaries--Breast: How many primaries should be reported for a diagnosis of ductal carcinoma in situ (DCIS) on core biopsy of the right breast in 2016 with all treatment refused, followed by a 2019 large right breast mass ulcerating the skin and clinical diagnosis of invasive breast cancer (patient again refused all treatment)? See Discussion. |
The patient was never treated for the 2016 diagnosis, so the 2019 diagnosis is the same tumor that has progressed. Prior SINQ 20091096 for a similar case type cited multiple primaries per the 2007 Multiple Primaries/Histology Rules, Rule M8, the same rule as the current Solid Tumor rule M17, because this is to be reported as an incidence case. However, it seems like Solid Tumor Rule M3 would apply because a single tumor is a single primary, and behavior of the 2016 primary would then be updated from /2 to /3. It is unclear how one would advance to the Multiple Tumors module and apply M17 because there is really only a single tumor in this case. |
Since the first diagnosis is in situ, and the later diagnosis is invasive, the 2019 diagnosis is a new primary even though it may be the same non-treated tumor. For cases diagnosed 2018 and later, abstract multiple primaries according to the 2018 Breast Solid Tumor Rules, Rule M17 that states Abstract multiple primaries when an invasive tumor occurs more than 60 days after an in situ tumor in the same breast. Note 1: The rules are hierarchical. Only use this rule when none of the previous rules apply. Note 2: Abstract both the invasive and in situ tumors. Note 3: Abstract as multiple primaries even if physician states the invasive tumor is disease recurrence or progression. Note 4: This rule is based on long-term epidemiologic studies of recurrence intervals. The specialty medical experts (SMEs) reviewed and approved these rules. Many of the SMEs were also authors, co-authors, or editors of the AJCC Staging Manual. |
2019 |
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20190059 | Solid Tumor Rules/Histology--Lung: What is the histology code and what H Rule applies for a diagnosis of well differentiated adenocarcinoma in situ (bronchioloalveolar carcinoma)? See Discussion. |
There is no statement of mucinous or non-mucinous in this case, only adenocarcinoma in situ and an obsolete term bronchioloalveolar carcinoma (BAC) which used to be code 8250. However 8250 is now lepidic adenocarcinoma, and does not match this diagnosis. Although the Histology Rules do include a general note indicating that the preferred term for BAC is now mucinous adenocarcinoma 8253, it is not listed as a synonym in Table 3. As a result it is unclear how to apply this statement in accordance with the H rules. The ICD-O Histology Updates table also includes Bronchiolo-alveolar carcinoma, non-mucinous which seems to suggest that in order to apply histology code 8252 (non-mucinous) or 8253 (mucinous) one must also have a statement of mucinous or non-mucinous. |
Code adenocarcinoma in situ as 8140/2 using the 2018 Lung Solid Tumor Rules, Rule H4 as this single histology is listed as a synonym for adenocarcinoma (8140) in Table 3 . Bronchiolalveolar carcinoma, a synonym for adenocarcinoma in situ, is an obsolete term according to WHO Classification of Tumors of the Lung, Pleura, Thymus and Heart, 4th edition; however, some pathologists add in the no longer preferred term to the diagnosis. When stated as non-mucinous adenocarcinoma in situ, code as 8250/2 for lung only (Rule H2) and mucinous adenocarcinoma in situ as 8253/2 (Rule H1). Note: WHO published a corrected 4th Ed Lung blue book fixing the 8410 error. |
2019 |
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20190058 | Solid Tumor Rules (2018)/Histology--Cervix Uteri: What is the histology code and what H Rule applies for a diagnosis of papillary squamotransitional cell carcinoma of the cervix? See Discussion. |
It appears that the first Other Sites applicable rule is H16 (and Table 2) instructing the use of histology code 8323 (mixed cell adenocarcinoma). However, this really is not an adenocarcinoma tumor but is a mixed squamous and transitional cell carcinoma. The 2018 ICD-O-3 Histology Update Table provides a new term for a but does not indicate whether that new term would also include a papillary squamotransitional cell carcinoma of the cervix. |
Code papillary squamotransitional cell carcinoma (PSCC) as 8120/3 using the 2018 Other Sites Solid Tumor Rules, Rule H11. PSCC is a distinctive subcategory of squamous cell carcinoma of the uterine cervix. WHO Classification of Tumors of Female Reproductive Organs say that squamotransitional cell tumors show papillary architecture with fibrovascular cores lines by multilayered atypical epithelium. |
2019 |
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20190057 | Reportability/Histology--Penis: Are and (PeIN) equivalent to PeIN3 and thus reportable? See Discussion. |
Appendix E1 of the 2018 SEER manual references a similar diagnosis as being reportable for vulva and vagina only. However, the WHO Classification of Tumors of the Urinary System and Male Genital Organs (4th ed) does include high grade penile intraepithelial neoplasia as a synonym for 8077/2. |
Penile intraepithelial neoplasia, grade III (PeIN III) and squamous cell carcinoma in situ of the penis are reportable. If possible, query the physicians as to whether "high grade penile intraepithelial lesion" or are synonymous with one of the reportable terms. If no further information can be obtained, report the case as C609 8077/2, and use text fields to document the details. |
2019 |
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20190056 | Behavior--Breast: What is the behavior of a solid papillary carcinoma when a pathologist does not indicate it in the pathology report and follow-up with the pathologist to obtain clarification regarding the behavior is not possible? See Discussion. |
Example: Mastectomy specimen final diagnosis shows two foci of invasive ductal carcinoma including: Invasive ductal carcinoma, no special type, in association with solid papillary carcinoma (tumor #1, 1 cm, slices 6 and 7) and invasive ductal carcinoma, no special type (tumor #2, 1.2 cm, slices 9 and 10). Summary Staging outlines, Tumor #1: Histologic Type: Invasive ductal carcinoma, no special type, in association with solid papillary carcinoma. As well as, Tumor #2: Histologic type: Invasive ductal carcinoma, no special type. Additional findings include ductal carcinoma in situ (DCIS): presently approximately 3.3 cm, spanning slices 10-13. The behavior of the solid papillary carcinoma component will affect the provisional histology of the first tumor (8523/3) per Rule H17 vs. 8500/3 per Rule H7). Based on the response, we can determine whether this represents a single or multiple primaries (single primary per M13 vs. multiple primaries per M14). |
Review all sections of the pathology report carefully for any mention of invasion, or lack of invasion, pertaining to the solid papillary carcinoma. Per WHO 4th Ed Breast: If there is uncertainty that there is invasion, these lesions should be regarded as in situ. The distinction between in situ and invasive disease in solid papillary carcinoma is difficult. |
2019 |
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20190054 | Update to current manual/Solid Tumor Rules (2018)/Histology--Brain and CNS: Table 6 (Non-Malignant CNS Equivalent Terms and Definitions) lists as a subtype/variant of craniopharyngioma 9350/1. This is not a valid histology per the ICD-O-3 or the 2018 ICD-O-3 Update Table. Is this actually supposed to read, ? |
Adamantinomatous craniopharyngioma (9351/1) is a subtype of craniopharygioma. We will correct the Non-Malignant CNS Solid Tumor Rules in the next update. |
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 |