Report | Question ID | Question | Discussion | Answer | Year |
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20180069 | Solid Tumor Rules (2018)/Behavior--Brain and CNS: The Behavior coding instructions in the Non-Malignant Central Nervous System (CNS) Equivalent Terms and Definitions section refer to Table 1 for help coding behavior when the other priority order instructions do not apply; however, the behavior cannot be reasonably determined using Table 1 alone for all WHO Grade I neoplasms. Should an additional default, such as the ICD-O-3 or Tables 5 and 6, be used to determine behavior? See Discussion. |
Similar to an issue previously submitted SINQ 20180063, Table 1 (WHO Grades of Select CNS Neoplasms) in the Non-Malignant CNS Equivalent Terms and Definitions section states WHO Grade I tumors are always non-malignant. However, this does not mean that the tumors listed in Table 1 as WHO Grade I are always benign (/0). Some tumors listed with a WHO Grade I have a behavior of /1 (borderline) per the ICD-O-3 and/or Tables 5 and 6. The Behavior coding instructions do not currently indicate these are the appropriate sources to use when the pathologist and/or physician do not comment on the behavior of these tumors. In our area, pathologists do not explicitly state the behavior for these tumors; the pathologist only assigns the WHO Grade. |
There is no way for us to know what behavior to assign WHO grade II tumors when the pathologist does not provide that information. Defaulting to either benign or malignant is incorrect. Please follow back with the pathologist to determine behavior. The behavior must be non-malignant, meaning /0 or /1, or the tumor is a WHO Grade 1, to be reportable as non-malignant CNS tumor. Refer to Table Instructions under Table 1, WHO Grades of Select CNS Neoplasms that says to use non-malignant CNS rules for all WHO Grade 1 tumors and to use the appropriate rules for WHO Grade 2 tumors Use ICD-O and all updates if not listed in Table 6 according to non-malignant CNS Histology Rule H3 (for single tumor) and Rule H8 (for multiple tumors) when only one histology is present. |
2018 |
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20200061 | Solid Tumor Rules (2018)/Histology--Bladder: A patient has high-grade papillary urothelial carcinoma with focal glandular and neuroendocrine differentiation followed by carcinosarcoma. Is this one or two primaries? See Discussion. |
12-19-19 Transurethral resection of bladder tumor pathology revealed high-grade papillary urothelial carcinoma with focal glandular and neuroendocrine features; Pathology Overread: High-grade papillary urothelial carcinoma with focal glandular and neuroendocrine differentiation. Carcinoma invades muscularis propria pT2. Histology 8130 01/20/20 to 07/01/20, completed 6 cycles of gemcitabine/cisplatin. 07/30/20 Robotic radical cystoprostatectomy with bilateral pelvic lymph node dissection, open ileal conduit pathology revealed carcinosarcoma, invading perivesical fat, no lymphovascular invasion, negative margins. ypT3bN0M0 disease; Pathology Overread: Carcinosarcoma arising in association with high-grade papillary urothelial carcinoma. Histology 8980/3 or is there another histology that should be used? |
The carcinosarcoma is a separate tumor, abstract a new primary per M13. Code this primary to 8980/3. Based on the information provided, the patient was first diagnosed with papillary urothelial carcinoma and received neo-adjuvant treatment for that specific histologic type. Subsequent resection identified carcinosarcoma arising within the papillary neoplasm. Carcinosarcoma is rare in bladder primaries and is not included in Table 2; however, it is a subtype/variant of sarcoma. |
2020 |
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20180054 | Solid Tumor Rules (2018)/Histology--Bladder: Under the Terms that are Not Equivalent or Equal section (Urinary Equivalent Terms and Definitions) it indicates noninvasive is not equivalent to papillary urothelial carcinoma and one should code the histology documented by the pathologist. However, many pathologists use Ta as both the description of the stage and the histology. Should this note be amended? See Discussion. |
The note in the Urinary Terms and Definition states, Both Ta and Tis tumors are technically noninvasive. Code the histology specified by the pathologist. While it is true that both Ta and Tis are technically noninvasive, the AJCC defines Ta specifically for, A pathologist's use of Ta does indicate the noninvasive carcinoma did arise from a papillary tumor. However, not all pathologists use terminology that, following the Urinary Solid Tumor Histology Coding Rules, will result in a histology coded to 8130, despite an AJCC-defined Ta (noninvasive papillary carcinoma) tumor having been diagnosed because the tumor projected from the wall on a stalk. In our region a number of pathologists provide the following types of diagnosis. Histologic type: Noninvasive. Histologic grade (WHO/ISUP 2016): High-grade. Tumor configuration: Papillary. The pathologist and/or physician may then stage this as Ta. How is the histology coded for these cases if the H Rules do not allow one to code the papillary and noninvasive Ta disease as not equivalent to noninvasive papillary carcinoma? Flat (in situ) urothelial carcinoma has an increased risk of invasive disease compared to the noninvasive papillary urothelial carcinomas. Will there be inconsistencies or a resulting impact to analysis of truly flat/in situ urothelial carcinoma vs. papillary urothelial carcinomas if the papillary tumors are not being coded as such? |
Per the April 2019 update: Noninvasive; papillary urothelial carcinoma; flat urothelial carcinoma Note: Noninvasive is not equivalent to either papillary urothelial or flat urothelial carcinoma. Both Ta and Tis tumors are technically noninvasive. Code the histology specified by the pathologist. |
2018 |
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20180057 | Solid Tumor Rules (2018)/Histology--Bladder: Which Solid Tumor H Rule applies when the patient has a single tumor removed by transurethral resection of bladder tumor and the final diagnosis is: Carcinoma of the bladder with the following features: Histologic type: Urothelial carcinoma? See Discussion. |
Instruction number 1 under the Coding Multiple Histologies instructions states to code histology when the histology is described as subtype, type or variant. The general rules do indicate we can code the histology identified as type, but when applying the H Rules, it seems an argument could be made for either H1 or H3. H1 applies if you ignore the diagnosis of carcinoma and only code the histologic type: urothelial carcinoma. However, the rules do seem to imply that you take all histologies into account (e.g., code the subtype/variant when there is a not otherwise specified (NOS) and single subtype/variant). Following this logic, Rule H3 seems to be the only rule that fits, and one would code the subtype/variant urothelial carcinoma when the diagnosis is carcinoma NOS, histologic type: urothelial carcinoma. The problem is that urothelial carcinoma is not a subtype/variant of carcinoma (NOS) per Table 2. The entry for Carcinoma NOS in Table 2 states, Subtypes of carcinoma NOS include adenocarcinoma and all subtypes/variants of adenocarcinoma. To some, urothelial carcinoma is a more specific type of carcinoma; however, urothelial carcinoma is not also listed as a subtype of carcinoma or of adenocarcinoma; only adenocarcinoma is categorized as a subtype of carcinoma. Consistently applying the rules becomes an issue when rules are interpreted in different ways. Should this Table be amended to include urothelial carcinoma as a subtype/variant of carcinoma NOS with the same caveat given to adenocarcinoma in Table 2? |
Code the most specific histology or subtype/variant. Urothelial carcinoma is more specific than carcinoma. See instruction #1 on page 29 of the April 2019 update. |
2018 |
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20200014 | Solid Tumor Rules (2018)/Histology--Brain and CNS: How are histology and primary site coded when a resection of a spine, designated intramedullary lesion, shows primary intramedullary melanocytoma? See Discussion. |
Patient has a resection labeled as: Spine, designated intramedullary lesion. The Final Diagnosis is: Melanocytic neoplasm with features most consistent with primary intramedullary melanocytoma. The Diagnosis Comment states: The overall immunophenotypic and morphologic impression is a primary central nervous system melanocytoma. The ICD-O-3 lists melanocytoma, NOS histology code as 8726/0, but does not provide a site-associated code. If the ICD-O-3 is used, the histology would be 8726/0 and the primary site presumably would be C720 since the tumor was specifically described as being intramedullary (i.e., within the spinal cord medulla). Table 6 (Solid Tumor Rules, Non-Malignant CNS Equivalent Terms and Definitions) does not list either an intramedullary melanocytoma or melanocytoma (NOS). However, Table 6 does include meningeal melanocytosis 8728/0 and meningeal melanocytoma 8728/1. If Table 6 is used and the histology is coded 8728/1, then the primary site would presumably be C701 per the ICD-O-3 site-associated listing for this histology (C709). |
Code primary site to spinal meninges (C701) and histology to meningeal melanocytoma (8728/1). According to the WHO Classification of Tumors of the Central Nervous System, 4th ed., primary melanocytic neoplasms of the central nervous system are diffuse or localized tumors that presumably arise from leptomeningeal melanocytes. Benign or intermediate grade lesions are termed melanocytomas. Meningeal melanocytoma is defined as a well-differentiated, solid, and non-infiltrative melanocytic neoplasm that arises from leptomeningeal melanocytes. Most arise in the extramedullary, intradural compartment at the cervical and thoracic spine though they can be dural-based or associated with nerve roots or spinal foramina. |
2020 |
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20200042 | Solid Tumor Rules (2018)/Histology--Brain and CNS: How is the histology coded when the diagnosis comment for a posterior fossa tumor resection states: Taken together, these findings are indicative of medulloblastoma with extensive nodularity? See Discussion. |
Example: Posterior fossa tumor resection final diagnosis was medulloblastoma, WHO Grade IV. The diagnosis comment notes the current tumor resection reveals large irregular reticulin-free nodules with streams of neoplastic cells in a fibrillary background in association with narrow reticulin-rich internodular strands of poorly differentiated neoplastic cells. Taken together, these findings are indicative of medulloblastoma with extensive nodularity. The diagnosis comment provided only one histology. Per the 2018 Solid Tumor Manual, Malignant CNS, Priority Order for Using Documentation to Identify Histology instructions, an addendum or comment has priority over the final diagnosis. Although indicative is not listed on any ambiguous terminology list, is this an ambiguous diagnosis that must be ignored? Or does the diagnosis comment in this case provide a single, specific diagnosis of medulloblastoma with extensive nodularity? |
Code as medulloblastoma, nodular (9471/3) based on the findings from both the comment and final diagnosis. |
2020 |
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20200078 | Solid Tumor Rules (2018)/Histology--Brain and CNS: Should the new malignant term pituitary blastoma be added to Table 3 of the 2018 Malignant Central Nervous System (CNS) and Peripheral Nerves Solid Tumor Rules? See Discussion. |
Pituitary blastoma was not added to Table 3 (Specific Histologies, NOS, and Subtypes/Variants) of the 2018 Malignant CNS and Peripheral Nerves Solid Tumor Rules as part of the December 2020 update. This is a new malignant CNS histology for 2021 and later. Not including this histology in Table 3 results in the registrars being required to check another source to correctly code this histology. If this histology cannot be used for cases diagnosed prior to 2021, should that diagnosis year clarification be included in the STR? This question was prompted from preparing SEER*Educate coding exercises. We will use the answer as a reference in the rationales. |
The Solid Tumor Malignant CNS tables do not list pituitary specific histologies at this time. Registrars will need to refer to ICD-O and/or updates until the decision to add malignant pituitary neoplasms is made. Pituitary blastoma is a rare tumor which occurs in children. |
2020 |
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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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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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20180043 | Solid Tumor Rules (2018)/Histology--Breast: Can the College of American Pathologists (CAP) protocol be used to determine whether in situ tumor is present for the purpose of determining which H Rule applies in the example presented? See Discussion. |
The Histology Coding Instructions give priority to the Final Diagnosis over the CAP protocol. However, when pathology reports are formatted using the CAP protocol, the presence of in situ carcinoma is generally only mentioned in the CAP protocol. Can the presence of in situ tumor mentioned only in the CAP protocol be used to apply rule H7 (Single Tumor: Invasive and In Situ Components Module)? Or are the rules in the Single Tumor: Invasive Only module used? Example: Final diagnosis is invasive ductal carcinoma. CAP protocol mentions, |
Apply Rule H12 of the 2018 Solid Tumor Rules for Breast Cancer, released April 2019. Remember the protocol is a checklist only and should not be used to code histology unless it is the only document available. |
2018 |