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 of 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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20230002 | First Course Therapy/Surgery of Primary Site--Prostate: What is the correct surgical code for irreversible electroporation ablation of the prostate diagnosed in 2021? |
Assign code 17 for irreversible electroporation ablation of the prostate when there is no tissue submitted to pathology for a 2021 or 2022 case. Assign code A170 for a 2023 case. |
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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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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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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20230027 | Solid Tumor Rules/Multiple Primaries--Peripheral Nerves: How many primaries should be abstracted, and which M Rule applies, when a malignant peripheral nerve sheath tumor (MPNST) in the right arm (C471) is followed greater than one year later by a separate malignant peripheral nerve sheath tumor of the thoracic chest wall (C473)? See Discussion. |
Since the peripheral nerves are included in the Malignant CNS schema of the Solid Tumor Rules, neither the differences in subsite nor timing indicate these are separate primaries (Rule M10 indicates a single primary). However, these are separate MPNSTs in different sites and the tumors are not stated to be metastasis. Additionally, these are treated as separate primaries by the managing physician. While the malignant CNS tumors do not take timing into account, is this correct for these peripheral nerve tumors that are often treated similarly to soft tissue tumors? Should Rule M8 be updated to include tumors in different peripheral nerve subsites? |
Abstract a single primary using Solid Tumor Rules, Malignant CNS and Peripheral Nerves, Rule M10 based on the information provided. Rule M10 applies as both non-contiguous tumors are of the same histology; i.e., on the same row in Table 3. As MPNST can arise in many sites, look for information about the precise location and tissue type in which the tumor arose. For example, if the tumors are stated to arise in soft tissue, follow the Multiple Primary Rules for Other Sites. Both WHO Classification of Central Nervous System Tumors and WHO Classification of Soft Tissue and Bone Tumors state that MPNST is a malignant spindle cell tumor often arising from a peripheral nerve, from a pre-existing benign nerve sheath tumor, or in a patient with neurofibromatosis type 1 (NF1). Future updates will move C470-C479 from CNS to other sites module. |
2023 |
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20230064 | Primary Site--Cervix Uteri: When no other information is available regarding the origin of the tumor, can an overlapping cervical adenocarcinoma (C538, 8140/3) be coded to the endocervix (C530) based on the histology? See Discussion. |
Adenocarcinoma is a glandular tumor and the endocervix is generally the origin of glandular tissue for the cervix. However, if the only available information is pathology proving a single tumor overlapping the endocervix and exocervix, can we code the site to C530 instead of C538? Applying the current primary site coding instructions, primary site would be coded as C538 because there is no specific statement of the tumor origin; the primary site coding instructions state the tumor is coded to an overlapping site in the absence of a specific statement of origin and there is no existing SINQ confirming the site can be assumed to be the endocervix based on the histology. |
Code Primary Site as Overlapping lesion of cervix uteri (C538). The 2023 SEER Program Coding and Staging Manual Primary Site Coding Instructions for Solid Tumors #4 says to code the last digit of the primary site code to ‘8’ when a single tumor overlaps an adjacent subsite(s) of an organ and the point of origin cannot be determined. This is also supported by the ICD-O-3, 3rd edition, note in the Topography section that states: In categories C00 to C809, neoplasms should be assigned to the subcategory that includes the point of origin of the tumor. A tumor that overlaps the boundaries of two or more subcategories and whose point of origin cannot be determined should be classified to subcategory ‘8.” |
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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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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20230015 | Solid Tumor Rules/Multiple Primaries: Should two 2021 diagnoses be abstracted as two primaries? The patient has a history of thyroid cancer in 2008 with no evidence of recurrence/progression. In 2021, two abstracts were submitted with a diagnosis of C809, poorly differentiated malignant neoplasm and a C421, myeloproliferative disorder. See Discussion. |
2021-Right pleural fluid: Negative for carcinoma. 5/18/2021: Right iliac crest bone marrow core biopsy, aspirate smear, clot section and peripheral blood smear: Hypercellular bone marrow, morphological findings are suspicious for a myeloproliferative neoplasm. Flow Cytometry: Slight immunophenotypic abnormalities of the myeloid cells. No abnormal B cell, T cell, or NK cell populations identified. Normal female karyotype. KARYOTYPE: 46,XX[20]. Negative for deletion of 13q14.3 (D13S319) by FISH. Negative for deletion of 13q34 (LAMP1) by FISH. Negative for hyperdiploidy involving chromosome 9 by FISH. Negative for t(9;22)(q34;q11.2) by FISH. Negative for deletion of the EGR1 gene on 5q31 by FISH. Negative for monosomy 5 by FISH. Negative for deletion of 7q31 by FISH. Negative for monosomy 7 by FISH. Negative for deletion of 20q12 by FISH. Negative for trisomy of chromosome 8 by FISH. 6/4/21-Left adrenal; biopsy: poorly-differentiated malignant neoplasm with extensive necrosis. Immunohistochemical stains show the neoplastic cells to be negative for CK7, TTF-1 and p63. Negative CK7 and TTF-1 would argue against a lung primary. Correlation with clinical and radiological findings is advised. We are unable to contact the provider. |
Based on the diagnosis date for the unknown primary, use the 2007 MPH Other sites rules. Since the site codes differ for each primary, rule M11 applies, abstract two primaries. |
2023 |