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20230039 | Histology/Hematopoietic and Lymphoid Neoplasms--AML: What is the histology code for Acute Myelogenous Leukemia (AML) with monocytic differentiation, 9891/3: acute monoblastic and monocytic leukemia or 9867/3: Acute myelomonocytic leukemia? |
Code AML with monocytic differentiation as acute myeloid leukemia, NOS (9861/3) per consultation with our expert hematopathologist. Acute monoblastic and monocytic leukemia (9891/3) and acute myelomonocytic leukemia (9867/3) are distinct entities according to the WHO. "AML with monocytic differentiation" is a descriptive diagnosis, whereas, "Acute monoblastic and monocytic leukemia" are specific diagnoses. In the WHO Classification of Tumours, Central nervous system tumours (4th Ed) in 2016, WHO began integrating information on molecular alterations that provide significant prognostic implications and/or a therapeutic target into the histology code/term itself. As a result it is also important to look at the molecular testing because acute myeloid leukemias can have different molecular mutations that could result in coding to a different histology code. In this case, there was no other information regarding additional immunophenotyping, so that is why AML, NOS was assigned. Acute myeloid leukemia with monocytic differentiation has been added to the Hematopoietic and Lymphoid Neoplasm Database as an alternate name for 9861/3. |
2023 | |
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20230003 | SEER Manual/Reportability--Ambiguous Terminology: Please clarify the reportability and relevant date ranges of the following ambiguous terminology: almost certainly, most certainly, and malignant until proven otherwise. See Discussion. |
SINQ 20180104 indicates, in the absence of further info, the terms “almost certainly” and “until proven otherwise” are NOT reportable. There is no date range provided for this answer. SINQ 20200027 indicates, in the absence of further info, the term “most certainly” IS reportable. There is no date range provided for this answer. SEER Program Coding and Staging Manual 2022 indicates, in the absence of further info, the terms “until proven otherwise” and “most certainly” ARE reportable. Essentially, we are hoping for an update of SINQ 20180104 due to 2022 reportability change. Clarification to the equivalence of “almost certainly” and “most certainly” would also be helpful. |
Use the ambiguous terminology list as a guide in the absence of additional information after reviewing all available information and consulting the physician who diagnosed and/or staged the tumor. Equivalent to "Diagnostic for" malignancy or reportable diagnosis
Not Equivalent to "Diagnostic for" malignancy or reportable diagnosis
We will update SINQ 20180104. |
2023 |
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20230037 | Reportability/Histology--Gallbladder: Is intracholecystic papillary-tubular neoplasm (ICPN) with extensive high grade dysplasia of the gallbladder reportable? |
Report intracholecystic papillary neoplasm (ICPN) with high-grade dysplasia (8503/2) of the gallbladder. |
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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20230007 | SEER Manual/Reportability--Appendix: Is low-grade appendiceal mucinous neoplasm (LAMN) with peritoneal spread followed by evidence of extraperitoneal metastatic disease reportable prior to 2022? See Discussion. |
In 2021, the patient was diagnosed with a non-reportable appendiceal LAMN. Resection showed a tumor diffusely involving the appendix and perforating the visceral peritoneum, as well as extensive intraperitoneal metastasis. In 2023, a lung wedge resection revealed metastatic mucinous neoplasm involving lung parenchyma and pleura, consistent with metastasis of the known appendiceal primary. It is understood that intraperitoneal spread of an appendiceal LAMN does not make it reportable because the peritoneal disease is also non-invasive. Does extraperitoneal metastasis of an appendiceal LAMN diagnosed prior to 2022 make it invasive disease and therefore reportable? |
LAMN diagnosed prior to 1/1/2022 is not reportable even when it spreads or metastasizes according to our expert pathologist consultant. Spread of this neoplasm does not indicate malignancy. For this case to be reportable, the diagnosis must indicate “carcinoma” or “adenocarcinoma.” Pre-2022, LAMN is not reportable even when treated with surgery and chemotherapy. LAMN is reportable starting with cases diagnosed in 2022. |
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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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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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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20230006 | SEER Manual/First Course Treatment--Hematologic Transplant And Endocrine Procedures: How are Surgery of Primary Site and the Hematologic Transplant And Endocrine Procedures data items coded when patient has total abdominal hysterectomy and bilateral oophorectomy for an endometrial primary during the same procedure? Also, how would these data items be coded for a vaginal primary in a surgical scenario? See Discussion. |
The 2023 SEER Manual instructions contain a new note in Hematologic Transplant And Endocrine Procedure, Coding Instruction 6, regarding bilateral salpingo-oophorectomy (BSO) when performed for hormonal effect for breast, endometrial, vaginal, and other primary cancers. While we have observed BSO being performed for breast primaries, we do not recall ever seeing a statement for endometrial or vaginal primaries regarding a “BSO being done as hormonal manipulation” when scheduled either with or without a hysterectomy being performed simultaneously. As a result, we are not clear exactly when a BSO would be captured in the Hematologic Transplant And Endocrine Procedure field for these gynecologic primary sites. Also, if these types of procedures are Hematologic Transplant And Endocrine Procedures, are they also captured and coded in the Surgery of Primary Site codes that directly relate to those same organs? Does timing have any effect on the coding of either field? |
For a primary endometrial or ovarian cancer, record the oophorectomy/BSO procedure using the appropriate Surgery of Primary Site code that includes oophorectomy/BSO when done as part of first course of treatment (surgical resection). If performed for hormone effect, also record in the Hematologic Transplant and Endocrine Procedures data item. For other primary sites whose Surgery of Primary Site codes do not include oophorectomy/BSO, record it in the Hematologic Transplant and Endocrine Procedures data item when performed for hormone effect. Document information in the appropriate text fields. Candidates for risk-reducing BSO may include those with hereditary syndromes (such as BRCA mutations) or genes that carry a substantially increased lifetime risk of ovarian malignancy or hormone-sensitive cancers including estrogen-dependent cancers, like breast cancer, ovarian cancer and endometrial (uterine) cancer that rely on estrogen to develop and grow. |
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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