| Report | Question ID | Question | Discussion | Answer | Year |
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20230060 | Histology--Urinary: How is histology coded for a diagnosis of bladder carcinoma with a mix of different urothelial carcinoma subtypes? See Discussion. |
The 10/2023 TURBT final diagnosis is “Urothelial carcinoma with mixed histologic appearances, see synoptic summary below for details.” The synoptic report includes, “Histologic Type Comment: Invasive carcinoma percentages: Micropapillary 60-70%, high grade or poorly differentiated urothelial 20-30%, squamous 10-20%.” The squamous component is stated to be “Urothelial carcinoma with squamous differentiation.” It appears there are two specific urothelial carcinoma subtypes to consider: Urothelial carcinoma, micropapillary variant (8131/3) and poorly differentiated carcinoma (8020/3). The squamous component would not be considered because there is no specific histology for “squamous differentiation.” The micropapillary component is the predominant histology (60-70%) in this case, and it does seem like this is important to capture. However, the WHO Blue Book indicates poorly differentiated carcinoma of the bladder has a poor prognosis. |
Code histology as urothelial carcinoma, NOS (8120/3). Our subject matter expert advises that WHO Classification of Urinary and Male Genital Tumors, 5th edition, does not recognize mixed urinary histologies; therefore, has not assigned an ICD-O code for urothelial mixed with multiple variants. Only pure variants are coded as they have a different prognosis from those that are mixed. According to WHO, invasive urothelial carcinoma is remarkable for its diversity of morphological appearances and a single lesion can display an admixture of conventional urothelial and various well-defined histological subtypes. |
2023 |
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20230029 | Primary Site--Skin: Are perianal skin primaries within 5 cm of the anus coded as perianal skin (C44.5) or anus (C21.0). See Discussion. |
ICD-O-3 tells us that perianal skin is C445 and we do not capture basal or squamous cell skin cancers in our registry. The AJCC manual stages perianal skin cancers within 5 cm of the anus with the anus chapter. We cannot AJCC stage them as an anus if we are not capturing them as C445. I realize we do not code a site in order to stage. We have been following the reportability rules and not capturing. Is this correct? I do not see this addressed in the new Other Sites Solid Tumor Rules. |
Code primary site based on the site of origin as determined by the physicians. If the physicians state the site of origin is anus, code anus; the same as with skin. As you state, squamous cell cancer of sites coded to C44 is not reportable. The AJCC instruction for physicians to stage perianal neoplasms within 5 cm of the anus using the Anus chapter does not change cancer registry instructions for coding primary site, nor does it affect cancer registry reportability instructions. |
2023 |
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20230017 | Solid Tumor Rules/Multiple Primaries--Rectum/Anal Canal: How many primaries are accessioned and how should histology be coded for a 2021 abdominoperineal resection showing invasive adenocarcinoma of distal rectum and associated Paget disease of the anal mucosa and perianal skin? See Discussion. |
The synoptic report calls this “Invasive adenocarcinoma with secondary Paget disease of anal mucosa and perianal skin.” The tumor size is listed as “2.1 x 1.7 x 0.7 cm, including associated advanced adenoma; size does not include the extent of the associated Paget disease, which extends for at least 2 cm distally.” Clinically this is called an incidentally discovered Paget’s disease. It is unclear if this is a collision tumor that should be abstracted as separate primaries, or if this is a single tumor with underlying Paget’s disease (similar to that described in Other Sites Rule H26). If this is a single rectal tumor, there does not appear to be an H rule for this scenario. |
Abstract two primaries using rule M4 of the Colon rules or rule M13 of Other Sites: 1. Invasive adenocarcinoma of distal rectum and 2. Paget disease of the anal mucosa / perianal skin (determine site of origin and code primary site accordingly). The rectum and the anus are separate sites and the histologies differ in each site. The WHO Classification of Digestive System Tumors, 5th edition, states that in addition to secondary anal Paget disease arising from anal canal adenocarcinoma, or rarely, adenoma without documented invasive disease, secondary Paget cells may be contiguous with the underlying neoplasm or manifest at different at sites distinctly away from it (with skip lesions). Document the details in the appropriate text fields. |
2023 |
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20230075 | EOD/Summary Stage--Eye: How is stage coded for a patient with extranodal non-Hodgkin lymphoma involving bilateral choroids (single focus, both sites) and no lymph node involvement? Since the eyes are a paired site, is this two separate extranodal sites? If so, there are no Summary Stage or EOD tumor codes that best fit this scenario. |
Assign as Stage IV as recommended by our expert hematological oncologist. This is a rare occurrence and this type of presentation does not fit the definition of intraocular extension. Stage IV is probably the best stage for this type of presentation, since there are two extranodal organs involved, even though they involve a bilateral site. EOD Primary Tumor: 700 SS: 7 (Distant) |
2023 | |
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20230023 | Solid Tumor Rules/Multiple Primaries—Brain and CNS: How many primaries are accessioned, and which M Rule applies, to a 2018 pituitary adenoma (8272/0) that was partially resected followed by a 2023 resection of residual disease proving pituitary adenoma/pituitary neuroendocrine tumor (8727/3)? See Discussion. |
The patient had residual tumor following the 2018 transsphenoidal resection and underwent an additional surgery after the residual tumor increased in size. Since pituitary adenoma/pituitary neuroendocrine tumor (PitNET) is a new malignant neoplasm for cases diagnosed 2023 and later, should this be a new primary per M5? Or do we disregard the change in behavior and apply rule M2 (single tumor is a single primary) for this scenario? |
This case does not fall into the standard rules. WHO criteria for diagnosing pituitary adenoma have recently changed (per 5th Ed WHO CNS book) and we will likely see more PitNET’ s than pituitary adenomas in the future. PitNET may be invasive or non-invasive but the likelihood of the pathologists providing this information is low. Since we don’t know if the 2018 adenoma was a PitNET based on current criteria or if it transformed to the malignant neoplasm, err on the side of caution and abstract a second primary per M5. This issue is new, and we’ve received numerous questions concerning pathologist reviewing older cases of pituitary adenoma and reclassifying them as PitNET using the new criteria. |
2023 |
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20230040 | First Course Treatment/Hormone Therapy--Prostate: Is Lupron first course therapy in a patient who initially elected active surveillance for prostate cancer and then consented to treatment with Lupron? See Discussion. |
in March, the patient with stage cT1c, Gleason grade 7, prostate cancer elected active surveillance. In April, the patient consented to treatment with Lupron. There was no evidence of disease progression. According to the rules on page 161 of the 2023 SEER manual, we think the answer is yes, but the reporting hospital states that this is second course therapy. |
Code Lupron as second course therapy and code active surveillance as first course therapy in this scenario. The 2023 SEER Manual states to code all treatment data items to 0 or 00 (Not done) when the physician opts for active surveillance, deferred therapy, expectant management, or watchful waiting. Assign code 2 to Treatment Status. Active surveillance is not the same as "refusing treatment." Active surveillance is a valid option offered to the patient. The patient chose this option and later changed their mind. This is not a refusal of recommended treatment. Document all the details in the appropriate treatment text fields. |
2023 |
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20230066 | Solid Tumor Rules/Histology--Lung: Table 3 in Lung Solid Tumor Rules, 2023 Update, lists neuroendocrine carcinoma, NOS 8246 as a specific subtype/variant for small cell carcinoma 8041/3. Should the table be updated? See Discussion. |
Small cell carcinoma is a specific type of neuroendocrine carcinoma for the lung. However, Table 3 lists neuroendocrine carcinoma, NOS as the more specific subtype/variant in Column 3. Using Lung Solid Tumor Rules, Rule H6, a diagnosis of poorly differentiated neuroendocrine carcinoma (small cell carcinoma)” would be coded as 8246, instead of 8041, because there are two histologies under consideration (an NOS and a subtype/variant in Table 3), and the rule tells us to code the subtype/variant. However, small cell carcinoma is more specific than the NOS diagnosis (neuroendocrine carcinoma, NOS). Should Table 3 be updated to reflect which histology is the NOS and which is the more specific? |
The Solid Tumor Rules for Lung have been updated for 2024. The row for Small cell carcinoma 8041/3 has been deleted and new separate rows have been added for Neuroendocrine carcinoma (NEC) 8246 and Neuroendocrine tumor, NOS (NET) 8240. This change is based on the WHO Classification of Thoracic Tumors, 5th edition, and current concepts. In addition, Table 3 now reflects that Small cell carcinoma/small cell neuroendocrine carcinoma 8041 (located in Column 3) is a subtype/variant of neuroendocrine carcinoma, NEC 8246 (Column 1). As a result, application of Rule H6 to a diagnosis of poorly differentiated neuroendocrine carcinoma (small cell carcinoma)” would be coded as 8041, instead of 8246. Please note: the 2024 updates may be used for cases diagnosed prior to 1/1/2024 unless otherwise noted in the rules. |
2023 |
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20230080 | Solid Tumor Rules/Histology--Brain and CNS: What is the histology code for low grade glioma? See Discussion. |
Patient has a 3 cm tumor in the temporal lobe of the brain. This was noted on MRI 12/2022. The radiologist states this is a low-grade glioma and recommends following with routine scans. No pathology or resection performed or planned. Patient has been followed with imaging every six months with stable disease. Low grade glioma is not currently listed in ICD-O-3.2 or the current Solid Tumor Rules. What histology should be assigned to the case? |
Assign 9380/1 for low grade glioma diagnosed 1/1/2018 forward and for low grade glioma diagnosed prior to 1/1/2018 assign code 8000/1 on the advice of our expert neuropathologists. The site/type combination of C71 _ and 9380/1 will flag histology/site/behavior edits which should be overridden. Low grade glioma is an umbrella term or non-specific diagnosis, primarily seen on radiologic reports such as CT scans and MRIs. Often, the patient is actively followed with scans and surgical intervention delayed or not recommended. WHO Classification of Central Nervous System Tumors, 5th edition, does not recognize this term and indicates that tissue diagnosis (including genetic testing) is needed to provide a specific diagnosis. Since biopsy of these “neoplasms” is not routinely done, a definitive diagnosis is not available. Literature searches yielded conflicting information with some stating low grade gliomas are malignant with an indolent clinical course while others felt they were benign. Until such time as WHO proposes a code for this neoplasm, our expert neuropathologists recommend coding glioma, NOS with borderline behavior 9380/1. |
2023 |
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20230052 | Reportability/Primary Site--Brain and CNS: What is the primary site of a meningioma arising from the jugular bulb/petrous aspect of the temporal bone? See Discussion. |
Example July 2022, Brain CT describes a mass appearing to be centered on the petrous aspect of the temporal bone with intracranial and extracranial extension. July 2022, Brain MRI describes an extra-axial mass centered in the right jugular bulb with intracranial and intraosseous extension as well as extension within the internal jugular vein. September 2022, Resection operative report surgical findings are of a calcified mass filling middle ear, abutting stapes and appearing to enter the stapes obturator foramen, debulked. Final diagnosis is right middle ear meningioma, WHO grade I of III. Is this a reportable intraosseous meningioma of the temporal bone/skull base, or a non-reportable meningioma arising in a meningocele within the middle ear? |
Do not report cases of meningioma originating in the jugular bulb or petrous aspect of temporal bone or middle ear. These are not intracranial locations. This is a non-reportable meningioma arising in a meningocele within the middle ear. The jugular bulb is the confluence of the lateral venous sinuses situated in the jugular fossa. The precise location of this structure within the temporal bone is variable.The jugular bulb, petrous aspect of temporal bone, and middle ear are not intracranial locations, and therefore meningiomas arising in these areas are not reportable. |
2023 |
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20230056 | Reportability/Histology--Heme and Lymphoid Neoplasms: What is the histology code for nodular lymphocyte predominant B cell lymphoma that is never called Hodgkin lymphoma? Is it acceptable to record the histology code for nodular lymphocyte predominant Hodgkin lymphoma, (9659/3)? See Discussion. |
Patient has a history of human immunodeficiency virus and diffuse large B cell lymphoma diagnosed in 2012, and is status/post systemic therapy and in remission since completing first course treatment. In 2022, the patient has imaging suspicious for recurrence. A biopsy of a deep left cervical lymph node showed atypical lymphoid infiltrate with the comment: “This is a challenging case. The constellation of findings is most in keeping with early / focal and subtle involvement by a nodular lymphocyte predominant B-cell lymphoma. We find no evidence of involvement by a diffuse large B-cell lymphoma.” The managing physician later states, “Cervical lymph node biopsy (06/2022) was consistent with nodular lymphocyte predominant B cell lymphoma.” |
According to the 5th edition WHO Blue Book for Hematopoietic Neoplasms, Beta Version, (not released yet), nodular lymphocyte predominant B-cell lymphoma is an alternate name for 9659/3. We will update the Heme database once the 5th edition is released in print. |
2023 |
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