| Report | Question ID | Question | Discussion | Answer | Year |
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20230026 | Solid Tumor Rules/Multiple Primaries--Prostate: How many primaries should be abstracted, and which M rule applies when a patient is diagnosed with intraductal carcinoma of the prostate on biopsy followed by invasive adenocarcinoma on radical prostatectomy more than 60 days later? See Discussion. |
Example: A prostate core biopsy showed intraductal carcinoma in 09/2022, which is an in situ tumor. A core biopsy again showed intraductal carcinoma in 12/2022. The subsequent radical prostatectomy in 04/2023, revealed multiple foci of invasive prostate adenocarcinoma with extensive intraductal carcinoma. Per Solid Tumor Rules, Other Sites, Rule M3, acinar adenocarcinoma of the prostate is always a single primary. Note 4, this rule applies to subtype variants of acinar adenocarcinoma listed in Table 3, which has intraductal/ductal as a variant subtype of acinar adenocarcinoma. Does rule M3 apply to incidence cases (an invasive tumor following an in situ tumor)? |
Rule M1 applies because we don't know if there are separate tumors or separate foci within a single tumor. This is a single primary coded 8140/3. The prostate rules will be reviewed for an addition to cover this situation. |
2023 |
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20230020 | First Course Treatment/Reason for No Surgery of Primary Site: How should Reason for No Surgery of Primary Site be coded for cases when surgery was planned but aborted due to extent of disease seen during planned procedure? See Discussion. |
Lung abnormality on imaging prompted diagnosis on subsequent biopsy and clinical staging was documented as cT1b N0 M0. There was an attempt at resection, but the patient was found to have chest wall involvement and the procedure was aborted. How would Reason for No Surgery of Primary Site be coded in these types of scenarios when the surgery is aborted and the treatment plan changes due to the extension seen during surgery? |
For the example provided: For 2023 cases and forward, if no part of the surgery was performed, code Surgery of Primary Site 2023 (NAACCR Item #1291) as code A000 or B000 (no surgical procedure of the primary site). Code Reason for No Surgery of Primary Site (NAACCR Item #1340) as code 2 (surgery of the primary site was not recommended/performed because it was contraindicated due to patient risk factors (comorbid conditions, advanced age, progression of tumor prior to planned surgery, etc.). In contrast, if any part of the surgery was performed, assign the Surgery of Primary Site 2023 (NAACCR Item #1291) code that best reflects the extent of the surgery performed. Code Reason for No Surgery of Primary Site (NAACCR Item #1340) as code 0 (surgery of the primary site was performed). Use text fields to record the details. For cases prior to 2023, apply the same approach using Surgery of Primary Site (NAACCR Item #1290) instead of Surgery of Primary Site 2023 (NAACCR Item #1291). |
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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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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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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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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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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20230021 | Histology--Soft Tissue: How is histology coded for malignant neoplasm with neuroectodermal differentiation and TPR-NTRK1 gene rearrangement diagnosed on left shoulder excision? See Discussion. |
March 2022, left shoulder soft tissue mass excision shows a spindle cell tumor with outside consultation diagnosis of malignant neoplasm with neuroectodermal differentiation and TPR-NTRK1 gene rearrangement. Diagnosis comments indicate the findings most closely resemble the spectrum of kinase-rearranged mesenchymal neoplasms, such as lipofibromatosis-like neural tumor. However, the expression of SOX10 and mature melanocytic markers is unusual, and does not exclude melanocytic differentiation. Should this be classified as a peripheral neuroectodermal tumor (9364) or as an "NTRK-rearranged spindle cell neoplasm (emerging)" (8990) if there is a NTRK gene rearrangement? |
NTRK-rearranged spindle cell neoplasm is a newly identified variant of sarcoma; however, WHO has not yet proposed a specific ICD-O code for this rare neoplasm. Code to spindle cell sarcoma (8801/3). WHO defines NTRK-rearranged spindle cell neoplasm as an emerging group of molecularly defined rare soft tissue tumors that span a wide group of morphologies and histological grades, and are most often characterized by a spindle cell phenotype among other characteristics. |
2023 |
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20230065 | Solid Tumor Rules/Histology--Prostate: Is histology coded as 8045 (Combined small cell carcinoma) for a 2023 diagnosis of two-component carcinoma comprised of both acinar adenocarcinoma and small cell neuroendocrine carcinoma of the prostate? See Discussion. |
This patient does not have a previous diagnosis of prostate adenocarcinoma nor a previous history of androgen-deprivation therapy. Does the logic in the Other Sites Solid Tumor Rules (STRs) noted in SINQ 20200052 still apply? This SINQ confirms a diagnosis of mixed prostatic adenocarcinoma and small cell neuroendocrine carcinoma is 8045. This matches the STRs instructions for Rule H21 and Table 2 (Mixed and Combination Codes), row 1. Row 1 indicates a mixed small cell carcinoma and adenocarcinoma is combined small cell carcinoma (8045). For a patient without previous treatment, is this the correct mixed histology code? |
Code histology as combined small cell carcinoma (8045) based on the Other Sites Solid Tumor Rules, May 2023 Update, Table 2, Mixed and Combination Codes, for this mixed histology prostate carcinoma consisting of adenocarcinoma and small cell neuroendocrine carcinoma regardless of treatment status. This is similar to SINQ 20200052 that applies to one tumor with mixed histologies. |
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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