| Report | Question ID | Question | Discussion | Answer | Year |
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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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20230022 | Solid Tumor Rules/Multiple Primaries: What M Rule of the updated Solid Tumor Rules, Other Sites, applies to a 2022 diagnosis of endometrial cancer, followed greater than one year later by a 2023 diagnosis of esophageal cancer with no interim evidence of tumor recurrence? See Discussion. |
These diagnoses were made greater than one year apart with a disease-free interval and M12 seems to be the first rule that applies. This rule does not specifically state the tumors diagnosed greater than 1 year apart must be in the same primary site but Note 1 could be interpreted as implying this. Note 1 states, “Clinically disease-free means that there was no evidence of recurrence in the same site on follow-up.” Does Other Sites Rule M12 (the timing rule) apply to tumors in different primary sites? It would be helpful if the notes specified this clarification, such as “Clinically disease-free means that there was no evidence of recurrence in the same site (same second and third character CXX.X) on follow-up.” |
Abstract multiple primaries using the Solid Tumor Rules, Other Sites, Rule M13. The topography differs at the second and third characters (C54.1 Endometrium; C15 Esophagus). Rule M12 refers to being disease-free vs. recurrence of a tumor, where Note 1 states that clinically disease-free means no evidence of recurrence in the same site on follow up. A note can be added to clarify that M12 applies to new tumors in the SAME site. |
2023 |
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20230028 | Histology--Vulva: How is the histology coded for vulvar intraepithelial neoplasia III (VIN III)/Squamous cell carcinoma in situ from a pathology report of the vulva, 8070/2 for squamous cell carcinoma in situ or 8077/2 for VIN III? The rules do not discuss this particular situation. |
Assign 8077/2 for high-grade squamous intraepithelial lesion, VIN 3 in this case. The WHO Classification of Female Genital Tumors, 5th edition, states that squamous intraepithelial lesions (SILs) of the vulva are also known as vulvar intraepithelial neoplasia, HPV-associated. The term squamous cell carcinoma in situ is not recommended. |
2023 | |
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20230032 | Reportability/Histology--Thyroid: Is a diagnosis of papillary carcinoma, follicular variant, encapsulated/well demarcated, non-invasive reportable? See Discussion. |
The final diagnosis for a left thyroid lobectomy was Papillary thyroid microcarcinoma, further stated to be Histologic Type: Papillary carcinoma, follicular variant, encapsulated/well demarcated, non-invasive. The diagnosis comment states there is a small follicular pattern papillary microcarcinoma. Is the designation of “non-invasive” for this papillary follicular tumor equivalent to a non-reportable diagnosis of Non-invasive follicular thyroid neoplasm with papillary-like nuclear features (NIFTP), 8349/1? Or should this be accessioned as either a reportable in situ (non-invasive) papillary follicular thyroid carcinoma or a papillary microcarcinoma per the diagnosis comment? |
Your case is equivalent to encapsulated follicular variant of papillary thyroid carcinoma, non-invasive (non-invasive EFVPTC) and is not reportable for cases diagnosed in 2021 or later even though it says "carcinoma." That is because the WHO assigned a behavior code of /1 to this entity (8349/1). NIFTP is assigned to the same histology and behavior code. |
2023 |
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20230078 | Primary Site/Heme & Lymphoid Neoplasms--CLL/SLL: Should the primary site be coded C421 (bone marrow) for a diagnosis of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) when the managing physician provides a Rai stage? See Discussion. |
The patient has adenopathy and a lymph node biopsy proved CLL/SLL. The patient underwent a peripheral blood smear, but the final diagnosis only indicated there is an abnormal CLL panel, positive for monoallelic or biallelic deletion of 13q. The pathologist noted a CLL related clone was detected, but there was no definitive diagnosis of CLL on the peripheral blood. No bone marrow biopsy was performed. However, the managing physician noted this was Rai Stage I CLL/SLL with adenopathy in the neck. The SSDI Manual notes, “Rai stage is only applicable for CLL, in which the bone marrow and/or peripheral blood are involved (primary site C421 for bone marrow, see Hematopoietic Manual, Module 3: PH 5, 6).” Should primary site default to C421 if the physician provides a Rai Stage in the absence of definitive peripheral blood or bone marrow involvement documented in the medical record? |
Assign primary site C421. The Site-Specific Data item (SSDI) Manual, Rai Classification section, states: Per confirmation from medical oncologists, Rai stage is only recorded for patients who have bone marrow and/or peripheral blood involvement. Per the Hematopoietic Rules, primary site would be C421 (See Hematopoietic Manual, Module 3: Rules PH 5, 6). A new code has been added to the 5 SSDIs (code 5) to use when primary site is not C421. |
2023 |
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20230077 | EOD 2018/ Primary Site/Heme & Lymphoid Neoplasms--CLL/SLL: How are Primary Site and Extent of Disease (EOD) Primary Tumor coded when a lymph node biopsy proved chronic lymphocytic leukemia (CLL), and the peripheral blood is involved with an “abnormal CD5-positive B-cell population”? See Discussion. |
The patient has adenopathy in multiple lymph node regions above and below the diaphragm and a lymph node biopsy pathology proved CLL/small lymphocytic lymphoma (SLL). Further work-up with peripheral blood proved an abnormal CD5-positive B-cell population comprising only a small percentage of the white blood cells (WBCs). The pathologist noted this neoplastic B-cell population comprises “3.5% of white blood cells and has an immunophenotype characteristic of CLL/SLL and is similar to the recent lymph node biopsy in this patient.” The managing physician indicated this was a Lugano Stage III SLL. The registrar coded the peripheral blood involvement in EOD Primary Tumor. If this small percentage of WBCs with an abnormal B-cell population is included in EOD Primary Tumor as peripheral blood involvement, then this would indicate peripheral blood/bone marrow involvement and primary site would need to be coded to C421 per Rule PH5. Rules PH5 and PH6 confirm primary site must be coded C421 if peripheral blood or bone marrow are involved. Is there a cutoff value for these abnormal B-cell populations in the peripheral blood? Or should these abnormal B-cell populations be ignored unless the pathologist states the abnormal B-cell population is consistent with CLL/SLL (not just immunophenotypically characteristic of CLL/SLL)? |
Primary site would be C421 based on Hematopoietic and Lymphoid Neoplasm Manual, Module 3, Rule PH 5. Assign EOD Primary Tumor to code 800 (peripheral blood involvement WITH other involvement). Per consultation with an expert hematologist oncologist, this is a Stage IV CLL/SLL since the peripheral blood is involved. There is no cutoff value for the abnormal B-cell populations in the peripheral blood when the cells are consistent with CLL/SLL. If the peripheral blood is involved, even only slightly, it is a Stage IV CLL/SLL. Our expert stated that the physician's staging was wrong (this is not a Lugano, Stage III). |
2023 |
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20230001 | Solid Tumor Rules/Multiple Primaries--Lung: How many primaries should be reported when two separate squamous cell carcinoma (SCC) tumors, one in the left upper lobe (LUL) and one in the right lower lobe (RLL), are diagnosed? The tumors are separated by an interval occurring right hilar lymph node biopsy proving metastatic pulmonary adenocarcinoma without a clear description of a corresponding interval occurring lung tumor. See Discussion. |
The patient was diagnosed with a biopsy-proven 12/2020 LUL SCC treated with radiation only, followed by a right hilar lymph node biopsy in 07/2022, that proved “metastatic pulmonary adenocarcinoma” per pathology and treated with radiation, followed by a biopsy-proven 12/2022 RLL SCC to be treated with immunotherapy only. The imaging never definitively identified a lung tumor that can be assumed to be a primary adenocarcinoma tumor. In 06/2022, a PET scan only described a “strongly PET positive Rt inferior hilar LN vs infrahilar pulmonary mass,” as well as the subsequently biopsy-proven SCC in the RLL (12/2022 SCC primary). The biopsy path indicates this was a right hilar lymph node metastasis and does not indicate this is an infrahilar pulmonary mass. No other PET positive pulmonary lesions were seen at the time. The oncologist’s assessment indicates the right hilar node was the only positive finding on the biopsy, and it was unclear if this right hilar node metastasis was from the left lung or if the primary was “not detectable.” The oncologist summarized this as a LUL lung lesion radiated for SCC, a right hilar lesion radiated for adenocarcinoma, and a RLL lung lesion on pathology found to be SCC. Should the interval occurring metastatic adenocarcinoma be accessioned as a separate lung, NOS primary based on the histology difference? While the Solid Tumor Rules do not apply to metastasis, the oncologist did treat these three malignancies separately and does not indicate the hilar lymph node metastasis was felt to be from either SCC primary. |
Abstract three primaries based on this scenario. 1 – 2020, SCC LUL lung 2 – 2022, Adenocarcinoma lung, described as metastatic pulmonary, based on biopsy of right hilar node (Rule M8) 3 – 2022, SCC RLL lung (Rule M11) |
2023 |
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20230011 | Solid Tumor Rules/Multiple Primaries--Prostate: How many primaries are accessioned when a 2023 liver biopsy diagnosed metastatic small cell carcinoma (SmCC) of the prostate following a 2018 radical prostatectomy treated diagnosis of prostatic adenocarcinoma? See Discussion. |
SINQs 20190083, 20180088, and 20130221 all indicate diagnoses of prostate adenocarcinoma, followed by a diagnosis of metastatic small cell carcinoma of the prostate are separate primaries because these are distinctly different histologies. Does this logic still apply for 2023 and later since Rule M4 was added to the Other Sites M Rules? Rule M4 states, “Abstract multiple primaries when the patient has a subsequent small cell carcinoma of the prostate more than 1 year following a diagnosis of acinar adenocarcinoma and/or subtype/variant of acinar adenocarcinoma of prostate.” This patient has a 2018 diagnosis of prostate adenocarcinoma treated with radical prostatectomy, followed by a 2023 diagnosis of metastatic small cell carcinoma of the prostate diagnosed on a liver metastasis core biopsy. Rule M4 does not indicate whether it applies to subsequent biopsy confirmed metastatic tumor only. When a diagnosis of small cell carcinoma follows a diagnosis of prostatic adenocarcinoma, it is almost always confirmed in metastatic sites rather than in the primary site. Does the logic in the referenced SINQs above still apply for Rule M4? |
Accession two primaries, adenocarcinoma (8140/3) of the prostate and SmCC (8041/3) of the prostate using Rule M4 of the current Other Sites Solid Tumor Rules. The guidance in the aforementioned SINQ entries still applies with the additional criteria of being diagnosed more than one year following the diagnosis of acinar adenocarcinoma, or subtype, of the prostate as stated in Rule M4 of the updated 2023 rules. Small cell carcinomas of the prostate are often diagnosed on follow-up TURP/biopsies; however, if a patient had a previous radical prostatectomy, the small cell carcinoma would be identified in a metstatic site and would still be a new prostate primary. This includes biopsy confirmed metastatic tumors only. It remains important to capture the two distinct histology types. |
2023 |
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20230019 | Solid Tumor Rules/Multiple Primaries--Pancreas: How many primaries, and what M Rule applies, when a pancreatectomy identified an invasive adenocarcinoma in one pancreatic head tumor, but multiple separate pancreatic neuroendocrine tumors (PanNETs), WHO grade 1, in the pancreatic body? See Discussion. |
There was a 3.5 cm invasive adenocarcinoma tumor in the pancreatic head. There were four separate, sized pancreatic neuroendocrine tumors measuring 0.9, 0.7, 0.5 and 0.2 cm in the pancreatic body. There are multiple tumors with distinctly different histologies. However, Table 11 (Pancreas Histologies) does not include any entries for neuroendocrine tumors of the pancreas (e.g., pancreatic NET, WHO grade 1, histology 8240). While it would seem Rule M19 should apply as they’re distinctly different histologies, because PanNETs are not included in Table 11, it is not clear which M Rule applies to these multiple tumors. If Rule M19 does not apply, we are left with Rule M21 (Abstract a single primary when there are multiple tumors that do not meet any of the above criteria). Are these separate tumors with distinctly different histologies really a single primary? Pancreatic neuroendocrine tumors are not an uncommon histology, is there a reason these were not included in Table 11? |
Abstract two primaries using the 2023 Solid Tumor Rules, Other Sites, Rule M19, as adenocarcinoma and pancreatic neuroendocrine tumors are two distinct histologies. The WHO Classification of Digestive Tumors, 5th ed., Chapter 10-Tumors of the Pancreas, lists both epithelial tumors and neuroendocrine neoplasm as separate entities. The Solid Tumor Rules histology-specific tables contain histologies that commonly occur in the 19 site-specific histology tables; therefore, not all histologies are listed in the rules. Further, the adenocarcinoma would be staged in the Pancreas Schema, while the neuroendocrine tumor would be staged in the NET Pancreas schema. We will consider adding PanNETs to Table 11 in a future release of the Solid Tumor Rules. |
2023 |
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20230063 | EOD 2018/EOD Regional Nodes--Melanoma: Can central cancer registries code Extent of Disease (EOD) Regional Nodes as 000 based on Breslow’s depth and/or Clark’s Level (per EOD and/or Summary Stage) from a melanoma pathology only report with a localized tumor and no information on regional lymph nodes or mets. See Discussion. |
Based on the EOD General instructions for accessible sites, the following three requirements must be met a. There is no mention of regional lymph node involvement in the physical examination, pre-treatment diagnostic testing, or surgical exploration; b. The patient has localized disease; c. The patient receives what would be the standard treatment to the primary site (treatment appropriate to the stage of disease as determined by the physician), or patient is offered usual treatment but refuses it. As a central registry, we receive a lot of melanoma path reports but never receive an abstract since the patients are seen at a dermatology office that does not report to the central registry. In these scenarios, we have both the diagnosis and wide excision or Mohs surgery from which we create a consolidated record. It is not often that lymph nodes are removed which indicates there were no palpable nodes. Since the Breslow’s and Clark’s level allow for summary staging, is it possible to have central registry guidelines that allow for coding lymph nodes other than 999? The path reports meet two of the three criteria. Is there any new literature that supports coding lymph nodes 000 based on a Clark’s level or Breslow measure providing the patient has a wide excision? |
Assign 000 for EOD Regional Nodes when you have a pathology only report with a localized tumor based on Breslow’s depth and/or Clark’s Level (per EOD and/or Summary Stage) and no information on regional lymph nodes or mets. When the tumor is noted to be regional or distant based on Breslow’s Depth and/or Clark’s based on the definitions in EOD and/or Summary Stage, do not assume that the nodes are negative and assign 999. Clarification will be added to the EOD manual. |
2023 |
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