Report | Question ID | Question | Discussion | Answer | Year |
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20200052 | Solid Tumor Rules (2018)/Histology--Prostate: How is the histology coded for a diagnosis of mixed prostatic adenocarcinoma (5%) and small cell neuroendocrine carcinoma (95%) from a transurethral resection of the prostate? See Discussion. |
Following the existing Solid Tumor Rules Histology Rules, it would seem this is a single primary with histology 8045 (Combined small cell carcinoma) because there is no indication there are multiple prostate tumors and Table 2 states combined adenocarcinoma and small cell carcinoma is Combined small cell carcinoma (8045). Conversely, while not an exact match to this case, SINQ 20190083 implies small cell carcinoma and adenocarcinoma of the prostate are separate primaries. In that SINQ case, the patient was simultaneously diagnosed with metastatic small cell carcinoma of the prostate on a liver biopsy and prostate adenocarcinoma on a prostate biopsy. There is no indication that patient had separate tumors in the prostate, however the SINQ instructs to code as separate primaries. Would the previous SINQ logic apply to synchronous diagnoses in the prostate as well? Or does code 8045 apply to this situation? |
Assign histology code 8045 for combined small cell carcinoma as this represents one tumor with mixed histologies using the 2018 Other Sites Solid Tumor Rules, Rule H16. |
2020 |
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20100024 | Histology: How is this field coded for a perivascular epithelioid cell neoplasm (PEComa) of uncertain malignant potential that is malignant based on the presence of metastases? See Discussion. |
In 11/2006 the patient had surgery for a 6cm mass in the RUQ arising in the falciform ligament. The pathologic final diagnosis was: Perivascular epithelioid cell neoplasm (PEComa) of uncertain malignant potential. In 10/2009 a liver biopsy showed metastatic perivascular epithelioid cell neoplasm. |
Assign histology code 8005/3 [malignant clear cell tumor]. According to our expert pathology consultant, this is the best histology code available at this time for the occasional tumor which is designated as malignant. The appearance of metastatic disease clearly defines this case as malignant. |
2010 |
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20240066 | Histology--Heme & Lymphoid Neoplasms: How should histology be coded for a pathologic diagnosis of “Follicular lymphoma, diffuse pattern grade 3A of 3, equivalent to diffuse large B cell lymphoma (germinal center cell type)” when later referenced clinically as follicular lymphoma grade 3A? See Discussion. |
The WHO Classification of Hematopoietic Tumors (Blue Book), 5th edition states: “Rare cases of classic follicular lymphoma with cytological features of follicular lymphoma (FL) grade 3A can present with a prominent diffuse pattern. In the previous edition, such cases were defined as diffuse large B-cell lymphoma (DLBCL). Currently, it is uncertain whether such cases should be classified as FL or diffuse large B-cell lymphoma; and in such cases, individual treatment choices should be made in multidisciplinary conference settings taking into consideration clinical, laboratory, and imaging parameters. The presence of diffuse areas composed entirely or predominantly of large cells, however, warrants a diagnosis of diffuse large B-cell lymphoma.” Our concern is that the Hematopoietic (Heme) Manual and Database do not provide instruction for coding this scenario. We hesitate to interpret the terms “equivalent to” as ambiguous because one could argue it is unambiguous. Barring this argument, the M and H rules would indicate this is a diagnosis of diffuse large B-cell lymphoma. However, the physician does not seem to agree with the pathologist. |
Assign histology as DLBCL (9680/3) as supported by the WHO Classification of Hematolymphoid Tumors, 5th edition. It is consistent with how it would have been coded in the 4th edition. The Heme Manual and Database currently are based on the 4th edition. Physicians are using the 5th edition blue book, whereas the cancer registry field is not yet at this time. Regarding the Heme Manual and Database, this type of scenario is not covered because it is part of the 5th edition WHO Blue Book. The database cannot be updated until the 5th edition is approved for implementation (2026). |
2024 |
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20230071 | Solid Tumor Rules/Histology--Cervix: How is histology coded for a 2023 endocervical adenocarcinoma negative for high-risk human papilloma virus (HR-HPV) on Pap smear and strongly positive for p16 on biopsy? See Discussion. |
The Solid Tumor Rules indicate p16 is a valid test to determine HPV status and can be used to code HPV-associated/-independent. In this case, we do not know whether the HR-HPV test was done on cytologically malignant cells, or on benign cervical cells. It may be impossible to tell unless 100% of the cytology specimen is malignant, but we will not have access to that information. Also, HR-HPV testing is routine on Pap smears, so this testing does not mean the tumor cells specifically harbor HPV. |
Assign histology as adenocarcinoma, HPV-associated (8483/3) as designated in Table 17, Uterine Cervix Histologies, of the Other Sites Solid Tumor Rules. The WHO Classification of Female Genital Tumors, 5th edition, states that p16 immunohistochemistry is an effective (yet flawed) indirect test for HR-HPV infection, in line with the STRs that state p16 is a valid test to determine HPV status and can be used to code HPV-associated and HPV-independent histologies. In this scenario, "negative for high-risk human papilloma virus (HR-HPV) on Pap smear" would be cytology-based, and may have missed cytologically malignant cells. A subsequent, more definitive biopsy was performed and was found to be strongly positive for p16, therefore, the tumor should be coded as 8483/3. |
2023 |
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20240061 | Heme & Lymphoid Neoplasms/Histology--Myelodysplastic Neoplasm: What is the histology code for myelodysplastic neoplasm with low blasts and SF3B1 mutation with primary site bone marrow (C421) diagnosed in 2023? |
Assign histology as 9982/3 (myelodysplastic neoplasm with low blasts and SF3B1 mutation). This is a new term for 9982/3. WHO Classification of Hematolymphoid Tumors, 5th edition, defines myelodysplastic neoplasm (MDS) with low blasts and SF3B1 mutation (MDS-SF3B1) as a myeloid neoplasm with cytopenia and dysplasia characterized by SF3B1 mutation and often ring sideroblasts. |
2024 | |
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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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20240074 | Solid Tumor Rules/Histology--Head & Neck: How is histology coded for nasopharyngeal non-keratinizing squamous cell carcinoma, undifferentiated type? See Discussion. |
Example: Patient had a 2023 nasopharyngeal mass biopsy showing “Nasopharyngeal non-keratinizing squamous cell carcinoma, undifferentiated type.” The Head and Neck Solid Tumor Rules (STRs) do not include an H Rule that instructs us how to code histology when there are two subtypes/variants present for a head and neck primary, nor does the STR define undifferentiated carcinoma as a subtype/variant for 8072. The WHO Classification of Head and Neck Tumors states non-keratinizing nasopharyngeal carcinoma (non-keratinizing squamous cell carcinoma (SCC) is the most common subtype for nasopharyngeal ca, but that non-keratinizing can be subdivided into undifferentiated and differentiated subtypes. Should histology be 8020 (undifferentiated carcinoma) or 8072 (non-keratinizing SCC)? |
Assign histology as 8072 for non-keratinizing SCC, undifferentiated subtype. WHO Classification of Head and Neck Tumors, 5th edition assigns 8072/3 to squamous cell carcinoma, non-keratinizing, NOS in the nasopharynx. As the tumor exhibits a variety of architectural patterns and appearances histologically, they can be further classified as undifferentiated or differentiated subtypes. These subtypes do not change the histology code. |
2024 |
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20160049 | Grade/Sarcoma--Breast: Is the correct grade for high grade angiosarcoma of the breast a code 3 or 4? The breast usually uses a three grade system but sarcoma is not a typical histologic type of the breast. |
Assign grade code 4 using the sarcoma table. Nottingham or Bloom-Richardson (BR) Score/Grade does not apply to angiosarcomas. This is a good question and points out needed clarification of the grade rules. |
2016 | |
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20180103 | Histology/Grade--Small intestine: For a 2017 diagnosis, is the grade/differentiation field coded 1 or 9 when the diagnosis is well-differentiated neuroendocrine tumor (NET) (carcinoid)? It seems as though the term well-differentiated defines type of neuroendocrine tumor so they can diagnosis the carcinoid. See Discussion. |
5/15/17 Duodenal bulb, biopsy: Fragments of duodenal mucosa with well differentiated neuroendocrine tumor (carcinoid), extending to the edge of specimen and peptic duodenitis in the submitted tissue. No significant intraepithelial lymphocytosis. |
Assign grade code 1 for well-differentiated NET (8240/3). Well-differentiated is synonymous with NET, grade 1, according to WHO Classification of Tumors of the Digestive System. |
2018 |
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20230057 | EOD (2018)/EOD Regional Nodes--Thyroid: How is Extent of Disease (EOD) Regional Nodes coded for thyroid primary with cervical lymph nodes containing psammomatous calcifications (psammoma bodies) but negative for metastatic tumor cells? See Discussion. |
The AJCC 8th edition confirms that the identification of psammomatous calcifications within a cervical lymph node is metastatic disease. Example: Patient had a thyroid lobectomy and level VI neck node excision in August 2022. The final diagnosis is multifocal papillary carcinoma of the thyroid, as well as rare psammomatous calcifications only in the resected node. The pathologist notes that “psammoma bodies only” in lymph nodes is not well defined, and while indolent, they do indicate capacity for lymphatic spread and are pN1a. Should thyroid primaries with cervical node psammomatous calcifications get captured in EOD Regional Nodes category as it is in the AJCC pN staging? |
Assign EOD Regional Nodes code 300 for Psammoma bodies within a cervical lymph node that are microscopically confirmed. A clarifying note for the Thyroid Schema will be included in the 2025 EOD updates. |
2023 |