Report | Question ID | Question | Discussion | Answer | Year |
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20240006 | Primary Site/Histology--Heme & Lymphoid Neoplasms: What are the correct primary site and histology for patient diagnosed with an oropharyngeal soft tissue mass revealing plasma cell neoplasm with 5-10% of marrow cellularity in 2022? See Discussion. |
Patient underwent excision of an oropharyngeal soft tissue mass revealing plasma cell neoplasm with extensive amyloid deposition. During work-up, bone marrow biopsy also revealed involvement by plasma cell neoplasm, with 5-10% of marrow cellularity. No amyloid seen in bone marrow. Patient was referred for radiation of the oropharyngeal mass. Per medical oncology qualifying best for the diagnosis of solitary extramedullary plasmacytoma with minimal marrow involvement. Decision made for observation by medical oncology in view of “minimal” bone marrow involvement. Question: Is rule M11 correct, and I abstract this case as a plasma cell myeloma, 9732/3, C421? |
Code as an oropharyngeal primary site and histology as solitary plasmacytoma (9734/3) based on consultation with our hematological expert. The WHO Classification of Hematopoietic and Lymphoid Tissues defines multiple myeloma as "bone marrow plasma cell percentage >60%." There are several other factors, but the bone marrow involvement is the key point for your case. The pathologist also states that the bone marrow is consistent with "plasma cell neoplasm," which by itself is not the same as multiple myeloma. This case has 5-10% involvement by plasma cell neoplasm. This does not meet the bone marrow qualifications for multiple myeloma and is consistent with the pathologist's statement that there is minimal bone marrow involvement. We will be updating the Hematopoietic and Lymphoid Neoplasms Database and Manual to clarify this (2025 updates). |
2024 |
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20081049 | Histology--Pancreas: What is the correct code for "non-secretory pancreatic endocrine tumor" with positive lymph nodes on excision indicating a malignant tumor? Pathologist indicated it was not an exocrine tumor. | Code as islet cell carcinoma [8150/3]. There are several cell types in the islets, and each produces a different hormone. The custom has been to name the tumors by their hormone production e.g. insulinoma, glucagonoma, etc. Occasional tumors do not produce any hormone (at least one that can be determined or measured). These tumors are called non-functioning endocrine tumors. Most of the endocrine tumors in the pancreas are islet cell tumors. |
2008 | |
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20200042 | Solid Tumor Rules (2018)/Histology--Brain and CNS: How is the histology coded when the diagnosis comment for a posterior fossa tumor resection states: Taken together, these findings are indicative of medulloblastoma with extensive nodularity? See Discussion. |
Example: Posterior fossa tumor resection final diagnosis was medulloblastoma, WHO Grade IV. The diagnosis comment notes the current tumor resection reveals large irregular reticulin-free nodules with streams of neoplastic cells in a fibrillary background in association with narrow reticulin-rich internodular strands of poorly differentiated neoplastic cells. Taken together, these findings are indicative of medulloblastoma with extensive nodularity. The diagnosis comment provided only one histology. Per the 2018 Solid Tumor Manual, Malignant CNS, Priority Order for Using Documentation to Identify Histology instructions, an addendum or comment has priority over the final diagnosis. Although indicative is not listed on any ambiguous terminology list, is this an ambiguous diagnosis that must be ignored? Or does the diagnosis comment in this case provide a single, specific diagnosis of medulloblastoma with extensive nodularity? |
Code as medulloblastoma, nodular (9471/3) based on the findings from both the comment and final diagnosis. |
2020 |
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20170076 | MP/H Rules/Histology--Brain and CNS: Is meningioma with atypical features coded as meningioma (9530/0) or atypical meningioma (9539/1)? See Discussion. |
Pathology report microscopic description: The tumor is a meningothelial neoplasm (EMA+; BCL-2 and CD34 negative) with prominent collagen deposition. Necrosis and prominent nucleoli are present; no other atypical features are seen. Mitoses are present, up to 2 per 10 high-powered fields. Final Diagnosis: Dura, bicoronal craniotomy (specimen A): Meningioma with atypical features. There is no rule in benign brain and CNS section of Multiple Primary/Histology (MP/H) Rules stating to code the most specific histologic term when the diagnosis is (something less specific, i.e., adenocarcinoma). This rule is in other site chapters of MP/H but appears missing in the benign brain and CNS section. |
Code as meningioma, NOS (9530/0). This lesion has some of the features of an atypical meningioma (necrosis and prominent nucleoli), but it does not fit the definition of atypical meningioma in WHO Classification of Tumors of the Central Nervous System. Use text fields to document the details. |
2017 |
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20190064 | Multiple Primaries--Heme & Lymphoid Neoplasms: Patient is diagnosed with myelodysplastic syndrome (MDS) with an early/evolving acute myeloid leukemia (AML) thought to be treatment related. Does rule M11 apply since there are two biopsies within 21 days, and therefore, two primaries, or one primary (9920/3)? See Discussion. |
Patient has a history of breast cancer and diffuse large B-cell lymphoma (DLBCL), both treated with chemotherapy and radiation. On 6/26/19, bone marrow biopsy: MDS with excess blasts-2 (18% dysplastic blasts) in a normocellular marrow (overall 40% cellularity) with trilineage dysplasia. Comment: least myelodysplastic syndrome with excess blasts-2. However, an early/evolving AML cannot be completely excluded. The findings likely represent therapy-related myeloid neoplasm. MD note on 7/15/19: Diagnosis: MDS, high grade borderline AML with complex karyotype secondary disease. Patient has high grade MDS which is bordering on AML transformation with 20% blasts by IHC and areas higher than this. This is likely secondary to the treatment she has received for her other cancers particularly pelvic radiation for her DLBCL. Given her very high IPSS score, it is likely she will eventually develop AML. No treatment given. On 7/15/19, bone marrow biopsy: Persistent acute leukemia in a marrow with trilineage dyspoiesis and 23% blasts. |
Code as one primary (9920/3). This case does not fit the rules very well, since it is a treatment-related neoplasm and involves a transformation of MDS to AML during the clinical workup. Per the abstractor notes for 9920/3, code 9920/3 when the physician comments that the neoplasm is treatment related. This can be for the MDS or the AML. Use text fields to document that it was first referred to as MDS and then transformed to AML. If you followed the rules strictly and coded this as two primaries (the MDS and AML), you would lose the information that this was treatment related, which is more important. |
2019 |
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20190063 | Solid Tumor Rules (2018)/Histology--Sarcoma: How is histology coded for a CIC gene rearrangement sarcoma? See Discussion. |
According to the literature, CIC gene rearrangement sarcomas in young patients are soft tissue sarcomas with an aggressive clinical course and may have previously been grouped under the Ewing-like family of tumors or as undifferentiated round cell sarcomas. There is currently no guideline in the solid tumor rules for coding a CIC gene rearrangement sarcoma. However, coding the histology to 8800 (sarcoma, NOS) seems unlikely to capture the more aggressive nature of these tumors. Can a more specific histology be coded? |
Code as undifferentiated round cell sarcoma (8803/3). The CIC rearrangement exists as a distinct molecular and clinical subset of small round cell tumors, and though similar, is felt to be a distinct entity from Ewing sarcoma. According to WHO Classification of Soft Tissues and Bone, 4th Edition, CID-DUX4 is a recurrent gene fusion associated with pediatric round cell undifferentiated soft tissue sarcoma (USTS). Although the genes involved in the fusion are different from those in Ewing sarcoma, the CIC-DUX4 protein has been shown to upregulate genes of the ETS family of genes thus providing a molecular link between Ewing sarcoma and round cell USTS. In contrast, there are strong arguments to suggest that Ewing-like sarcomas represent a separate and distinct entity. |
2019 |
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20081061 | Histology--Breast: What is the histology code for a 2007 diagnosis of basal-type breast carcinoma? | Code basal-type breast carcinoma to 8500/3 [Infiltrating duct carcinoma, NOS]. Basal-type breast carcinoma is a subtype of infiltrating duct carcinoma thought to have a poorer prognosis. There is no specific ICD-O-3 code for basal-type breast carcinoma. |
2008 | |
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20091082 | Behavior--Breast: How is this field coded for a case in which the final diagnosis reports DCIS, but the CAP protocol or microscopic findings show microinvasion? See Discussion. | 1. Path report for breast cancer has final diagnosis as 'DCIS' but the CAP protocol in the body of the report says 'microinvasion seen, T1mic.' 2. Path report says 'DCIS' in the final diagnosis and microinvasion is identified in the microscopic portion of the report, but it is not in CAP protocol format and not stated in the final diagnosis. |
Code both scenarios /3 [malignant (invasive)]. Information regarding behavior is not limited to the final diagnosis or the CAP protocol. See page 84 in the 2007 SEER manual: Code the behavior as malignant /3 if any portion of the primary tumor is invasive no matter how limited; i.e. microinvasion. |
2009 |
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20150033 | MP/H/Histology--Lung: Would you code a lung primary of "non-small cell carcinoma with neuroendocrine differentiation" to non-small cell carcinoma (8046/3) or carcinoma with neuroendocrine differentiation (8574/3)? See discussion. |
The pathology report states "Right mediastinal mass: poorly differentiated non-small cell carcinoma with neuroendocrine differentiation." This is the only histologic confirmation of this lung primary that is collected. |
Code carcinoma with neuroendocrine differentiation (8574/3). MP/H rule H7 applies: code the higher ICD-O-3 code. There is non-small cell lung carcinoma (8046/3) and a carcinoma, NOS with neuroendocrine differentiation present (8574/3). |
2015 |
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20190084 | Histology/Heme & Lymphoid Neoplasms: Should the histology be coded to chronic myeloid leukemia (CML), BCR-ABL1-positive (9875/3) regardless of the quantitative analysis percentage of BCR-ABL1 that was detected? See Discussion. |
Example: Bone marrow biopsy diagnosis is chronic myelogenous leukemia, chronic phase, and the RT-PCR test result proved, BCR-ABL1 p210 (Major Breakpoint) - Detected, 3.3659%. Even though the p210 fusion transcript was less than 5%, it was detected. The presence of BCR-ABL1 does define whether or not patients are treated with tyrosine kinase therapies. Therefore, it seems likely that the presence of any BCR-ABL1 would be captured using the more specific histology code 9875/3, instead of the non-specific CML, NOS histology code 9863/3. Are there minimum threshold requirements for these quantitative studies in order to code the histology to the more specific type of CML? |
Code chronic myeloid leukemia (CML) BCR-ABL1-positive as 9875/3. According to the WHO Classification of Tumors of Hematopoietic and Lymphoid Tissues, 4th edition, CML BCR-ABL1-positive is characterized by the chromosomal translocation t(9;22) which results in the formation of the Philadelphia (Ph) chromosome containing the BCR-ABL1 fusion gene. The diagnosis requires detection of the Ph chromosome and/or BCR-ABL1. If the mutation is detected, regardless of percentage, it is positive. Quantitative levels of BCR-ABL are used to monitor response to tyrosine kinase inhibitor therapy. |
2019 |