Report | Question ID | Question | Discussion | Answer | Year |
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20230068 | Solid Tumor Rules/Histology--Thyroid: What is the histology code for a diagnosis of poorly differentiated thyroid carcinoma arising in a background of solid papillary thyroid carcinoma? See Discussion. |
Patient had a hemithyroidectomy with the final diagnosis above. There does not appear to be an Other Sites H rule or table that addresses this combination of histologies for thyroid primaries. |
Code to poorly differentiated thyroid carcinoma, 8337/3. In this case the tumor is comrpised of two difffernat thyroid histologies: poorly differentiated carcinoma 8337/3 and papillary thyroid carcinoma 8260/3. WHO does not have a code for this combination. Per our endocrine pathology expert, the poorly differentiated carcinoma is the more agressive histology and will determine treatment and progrnosis. |
2023 |
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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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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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20230018 | SEER Manual/First Course Treatment--Chemotherapy: Does the First Course of Treatment end when subcategories change for treatments such as hormone therapy or immunotherapy or is that instruction specific to chemotherapy? See Discussion. |
Treatment for estrogen receptor positive (ER+) breast cancer started with tamoxifen (non-steroidal estrogen subcategory) and switched to letrozole (non-steroidal aromatase inhibitor subcategory). Patient being treated with immunotherapy, Avastin (cytostatic agent-antiangiogenesis agent subcategory), and then changed to Atezolizumab (monoclonal antibody subcategory). Is Atezolizumab a new course of therapy because it is a different subcategory? |
A change in the subcategory for a hormone drug does not indicate the end of First Course of Treatment because different hormone therapies generally achieve the same result. For example, some forms of breast cancer are estrogen-dependent and the various subcategories of hormone drugs used to treat them, such as gonadotropin-releasing factor agonists, aromatase inhibitors and estrogen antagonists, all achieve the same result - to block estradiol effects in these tumors. Similarly, a change in immunotherapy is not a new course of treatment. The instruction in the SEER Manual is specific to chemotherapy. Chemotherapy is the only systemic treatment for which a change in the subcategory of a drug indicates the end of First Course of Treatment, due to the fact that different chemical agents damage cancer cells in different ways and at different phases in the cell cycle. |
2023 |
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20230070 | Solid Tumor Rules/Multiple Primaries--Breast: How many primaries should be accessioned for a diagnosis of invasive carcinoma of the left breast (8500/3) in 2020 followed by a 2023 diagnosis of dedifferentiated carcinoma in the left breast (8020/3)? See Discussion. |
The WHO Blue Books do not include dedifferentiated carcinoma as a valid histology for the breast. However, there is known to be progression of ductal carcinoma that is essentially dedifferentiation of an estrogen receptor, progesterone receptor, and HER2 breast carcinoma to a triple negative "dedifferentiated" carcinoma which it appears this patient has. Whether we should accession this as a separate 8020/3 primary per M14 is unclear and the Solid Tumor Manual does not address this scenario. |
Abstract a single primary using Breast Solid Tumor Rules, Rule M18, as none of the previous rules apply. Undifferentiated carcinoma is a malignant epithelial tumour lacking overt evidence of a specific line of differentiation. Dedifferentiated carcinoma is composed of an undifferentiated carcinoma and a differentiated component. Dedifferentiated carcinoma (8020/3) as a morphology is associated with cancer of the endometrium and ovary rather than the breast. Breast cancer shows a broad spectrum of morphology with extensive variation in histological type and grade, related to the complexity of carcinogenesis. This includes initial genetic changes in the cell of origin, subsequent genetic and epigenetic alterations, and reprogramming that occur at various stages of development along with interaction of other factors that influence the process of differentiation. This scenario likely represents the process of phenotypic change of a carcinoma at a later stage, better known as transdifferentiation. |
2023 |
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20230076 | Solid Tumor Rules/Histology--Prostate: How is histology coded and what rule applies to a diagnosis of “prostatic adenocarcinoma with neuroendocrine differentiation” with reference to the Comment: Immunohistochemical findings are consistent with amphicrine carcinoma for a patient with no prior androgen-deprivation therapy. See Discussion. |
The case in question represents an adenocarcinoma with neuroendocrine differentiation that arises in the absence of androgen-deprivation therapy. A 2023 journal article states, “We show that amphicrine prostate cancer is a unique entity and differs in clinical and molecular features from high-grade neuroendocrine carcinomas of the prostate. Our study highlights the need to recognize AMPC as a unique molecularly defined subgroup of prostate cancer.” Should we be coding this with histology 8140 (Adenocarcinoma, NOS) because we have no specific code for an amphicrine carcinoma? Should we code this as 8045 (Mixed small cell carcinoma) because this is possibly the only way to capture both the adenocarcinoma and neuroendocrine components in a patient without previous treatment? Our concern about using histology code 8574 (Adenocarcinoma with neuroendocrine differentiation) is that, while a valid histology code, this might confound the data if researchers are trying to separate the truly treatment-related tumors from other histologies captured under 8574. |
Assign 8140/3 (adenocarcinoma, NOS). WHO has not yet recognized the variant amphicrine prostate carcinoma and have not proposed an ICD-O code for this neoplasm. Document information in a related text field. |
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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20230030 | Primary site: Is there a physician priority list for coding primary site? For example, the surgeon states during a pancreatectomy that the primary is in body while the pathologist states in their synopitc report that primary is neck; neither is in agreement, or neither is available for confirmation. |
As a general rule, the surgeon is usually in a better position to determine the site of origin compared to the pathologist. The surgeon sees the tumor in its anatomic location, while the pathologist is often using information given to him/her by the surgeon and looking at a specimen removed from the anatomic landmarks. However, when a pathologist is looking at an entire organ, such as the pancreas, he/she may be able to pinpoint the site of origin within that organ. In the case of pancreas body vs. neck, the neck is a thin section of the pancreas located between the head and the body. It may be a matter of opinion whether a tumor is located in the "body" vs. the "neck." In the situation you describe, we would give preference to the surgeon and assign the code for body of pancreas, C251. |
2023 | |
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20230016 | Solid Tumor Rules/Histology--Brain: How is histology coded for an anaplastic glioneuronal tumor, BRAF p.V600E mutant, WHO Grade III, diagnosed following a right temporal lobe resection in 2021? See Discussion. |
The patient has a history of ganglioglioma, WHO grade I, involving the deep right parietal lobe diagnosed on resection in 07/2012. Tumor recurrence in 2017 was treated with radiation. The patient then had right temporal tumor biopsy and resection 06/2021 with final diagnosis of anaplastic glioneuronal tumor, BRAF p.V600E mutant, WHO Grade III. Pathologist notes that the tumor demonstrates a ganglioglioma with frequent mitoses and possible vascular proliferation. Subsequent consult findings support an anaplastic glioneuronal tumor, compatible with progression of the patient's ganglioglioma that is post-irradiation. However, the pleomorphic and epithelioid areas are also reminiscent of pleomorphic xanthoastrocytoma, which may occur in combination with ganglion cell components. There is no related SINQ to code this histology. |
Assign histology as 9505/3. WHO Classification of Central Nervous System (CNS) Tumors describe ganglioglioma as a well-diffferentiated and slow-growing glioneuronal neoplasm. While WHO does not recognize the histology/behavior combination 9505/3, the 2021 CNS Solid Tumor Rules identify non-malignant tumors that have the potential of transforming to a malignant tumor (new primary). Ganglioglioma (9505/1) is listed with the transformed histology and instructs us to code as anaplastic ganglioglioma (9505/3). |
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 |