Report | Question ID | Question | Discussion | Answer | Year |
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20230021 | Histology--Soft Tissue: How is histology coded for malignant neoplasm with neuroectodermal differentiation and TPR-NTRK1 gene rearrangement diagnosed on left shoulder excision? See Discussion. |
March 2022, left shoulder soft tissue mass excision shows a spindle cell tumor with outside consultation diagnosis of malignant neoplasm with neuroectodermal differentiation and TPR-NTRK1 gene rearrangement. Diagnosis comments indicate the findings most closely resemble the spectrum of kinase-rearranged mesenchymal neoplasms, such as lipofibromatosis-like neural tumor. However, the expression of SOX10 and mature melanocytic markers is unusual, and does not exclude melanocytic differentiation. Should this be classified as a peripheral neuroectodermal tumor (9364) or as an "NTRK-rearranged spindle cell neoplasm (emerging)" (8990) if there is a NTRK gene rearrangement? |
NTRK-rearranged spindle cell neoplasm is a newly identified variant of sarcoma; however, WHO has not yet proposed a specific ICD-O code for this rare neoplasm. Code to spindle cell sarcoma (8801/3). WHO defines NTRK-rearranged spindle cell neoplasm as an emerging group of molecularly defined rare soft tissue tumors that span a wide group of morphologies and histological grades, and are most often characterized by a spindle cell phenotype among other characteristics. |
2023 |
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20170044 | Histology--Sarcoma: What is the histology code for epithelioid angiosarcoma? |
Assign 9120/3 for epithelioid angiosarcoma. |
2017 | |
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20200003 | Histology--Penis: What is the histology code of a glans penis primary with the final diagnosis squamous cell carcinoma, verrucous type? See Discussion. |
Penile mass excision shows final diagnosis of squamous cell carcinoma, verrucous type. Subsequent partial penectomy has a final diagnosis of squamous cell carcinoma, verrucous type and the summary cancer data lists Both the final diagnosis and summary cancer data indicate a histology code of 8051/3 (squamous cell carcinoma, verrucous type / verrucous carcinoma). However, this site and histology combination triggers edit IFN4911. Edit documentation indicates that for sites C600-C609 (all penile sites) use histology code 8051 and do not use 8054. Review of the 2018 ICD-O-3 Histology Updates table does not indicate these terms are synonymous. |
Code squamous cell carcinoma, verrucous type of the penis as verrucous carcinoma (8051/3). In WHO Classification of Tumors of the Male Urinary System and Male Genital Organs, 4th edition, tumors of the penis, verrucous carcinoma is described as an extremely differentiated keratinizing papillomatous and acanthotic neoplasm; it accounts for 2-3% of penile squamous cell carcinomas. The coding of condylomatous carcinoma and warty carcinoma changed from 8051/3 to 8054/3 in 2018 for penile sites only in the 2018 ICD-O-3 New Codes, Behaviors, and Terms-Updated 8/22/18. Override the edit until the edit issue is explored. |
2020 |
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20180015 | Histology--Ovary: What is the correct ICD-O-3 histology code for sertoliform endometrioid carcinoma of the ovary? |
Assign 8380/3. Sertoliform endometrioid carcinoma is a variant of endometrioid carcinoma according to the WHO Classification of Tumors of Female Reproductive Organs, 4th edition. There is no specific ICD-O-3 code for this variant. |
2018 | |
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20031187 | Histology--Lymphoma: What code is used to represent the histology "monomorphic post-transplant lymphoproliferative disorder [diffuse large B-cell lymphoma]"? See Description. |
A 14 year old with a cadaver kidney transplant in 1994 for membranous glomerulonephritis presented in 6/26/03 with a right cervical LN with biopsy showing "lymph node involved by monomorphic post-transplant lymphoproliferative disorder (diffuse large B-cell lymphoma). Staging was done including a bone marrow which was negative, CSF negative. The oncologist on the case reduced the immunosuppression drugs with the final outcome being no sign of the lymphoma. | For cases diagnosed prior to 1/1/2010:Code 9680/36 [Diffuse large B-cell lymphoma]. This post-transplant lymphoproliferative disorder was diffuse large B-cell lymphoma. According to the World Health Organization, there are two types of post-transplant lymphoproliferative disorder. "Regular" post transplant lymphoproliferative disorder is not a neoplasm and is therefore not reportable to a cancer registry. The second type (sometimes called Hodgkin-like PTLD) is classified as a B-cell lymphoma, which means that it IS reportable.
For cases diagnosed 2010 forward, refer to the Hematopoietic and Lymphoid Neoplasm Case Reportability and Coding Manual and the Hematopoietic Database (Hematopoietic DB) provided by SEER on its website to research your question. If those resources do not adequately address your issue, submit a new question to SINQ. |
2003 |
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20220030 | Histology--Lung: Is it acceptable to code histology as 8042/3 for a 2020 lung primary when the pathology report states only "oat cell carcinoma?" See Discussion. |
In the old 2007 Multiple Primaries/Histology rules, Lung Equivalent Terms and Definitions section, oat cell carcinoma (8042) was listed as one of the obsolete terms that was no longer recognized for small cell carcinoma. That note is not in the current 2018 Solid Tumor Manual lung chapter, and ICDO-3.2 lists oat cell carcinoma as the preferred term for code 8042/3. Would rule H4, Note 2 apply -- only one histology present, if not listed in Table 3 use ICD-O and all updates, to code oat cell carcinoma as 8042/3? |
While oat cell carcinoma is an outdated term, if that is all the pathology report states, code histology as 8042/3. Yes, Rule H4 applies: the diagnosis was a single histology. H4 instructs you to refer to the solid tumor H table, and if the term is not found there, check ICD-O and ICD-O updates. All possible histologic types that could occur in the lung may not be included in the table. |
2022 |
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20200057 | Histology--Lung: Is there a better code for SMARCA4-deficient malignant neoplasms than 8000/3 that could be used especially given its aggressive nature? This term is not included in the Lung Solid Tumor Rules or ICD-O-3.1 and 3.2. See Discussion. |
Per Mayo consulting pathologist, the final diagnosis on this right lung biopsy is: SMARCA4-deficient malignant neoplasm (see Comment). Comment: Sections show a poorly-differentiated malignant neoplasm without any apparent glandular, squamous, or stromal differentiation. The tumor near totally replaces the underlying lung tissue without recognizable underlying alveolar parenchyma. Immunohistochemical stains performed at Mayo Clinic (Oscar keratin, INSM1, NUT, S100, desmin and BRG1 protein encoded by SMARCA4 gene) demonstrate that the malignant cells are positive for Oscar keratin (rare cells only), synaptophysin (weak/patchy) and p63 (focal) while negative for the remaining antibodies tested. Of note, SMARCA4 stain is negative in the tumor cells. Thus, this tumor can be categorized as a SMARCA4-deficient malignant neoplasm, which is known to be an aggressive malignancy, likely represent a SMARCA4-deficient thoracic sarcoma, a recently described entity. SMARCA4-deficient carcinomas in the lung have been reported to be mostly adenocarcinomas or squamous cell carcinomas, which would not fit for this case. Please refer to a paper published by our group (Sauter JL et al. Mod Pathol 2017;30:1422-32. |
Assign code 8020/3. SMARCA4-deficient malignant neoplasms are newly identified. WHO has not proposed an ICD-O code as of yet. Our pathology experts suggest coding to undifferentiated carcinoma until they are better classified. |
2020 |
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20180101 | Histology--Kidney: What is the histology code for renal cell clear cell of the kidney with subsequent epithelioid angiomyolipoma PEComa of the liver stated to be metastatic? Case originaly diagnosed in 2016. See discussion. |
This patient was diagnosed in 2016 with renal cell clear cell and was coded to that. In 2018, the patient's liver lesion was resected and pathology revealed epithelioid angiomyolipoma perivascular epithelioid cell tumor (PEComa) (8714/3), a new term as of 2018. This was compared to the kidney slides and it was determined to be metastatic PEComa from the kidney. The physician's note states: The patient had a nephrectomy for a kidney tumor in 2016, excision of cutaneous melanomas, and resection of liver mass in 2018. These three cases were sent in consultation. The diagnosis of cutaneous melanoma was confirmed by a dermatopathologist of our department, (a separate report had been already issued). The kidney tumor is poorly differentiated composed of sheets of discohesive cells with markedly pleomorphic cells with frequent giant and bizarre cells. Most of the cells have abundant eosinophilic to clear cytoplasm. The nuclei are enlarged and pleomorphic. Multinucleated cells are numerous. Some cells have markedly enlarged nucleoli. Multifocal tumor necrosis is noted. Extensive lymphovascular invasion is observed. There are foci at the periphery of the tumor consisting of a proliferation of spindle cells with entrapped adipocytes consistent with minor element of unusual angiomyolipoma (see block A18). The liver tumor has histologic features that are similar to the poorly differentiated component of the kidney tumor. |
Revise the histology code for the 2016 diagnosis based on the review of slides performed in 2018. When new information becomes available, the information in the abstract can be updated. PEComa is a synonym for epithelioid angiomyolipoma (8860/1). These tumors can be malignant with local recurrence and or mets. For a pre-2018 diagnosis, code histology to 8860/3 using the ICD-O-3 Rule F, aka: Matrix principle. |
2018 |
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20230059 | Histology--Heme and Lymphoid Neoplasms: How is histology coded for a diagnosis stated as MDS/AML (myelodysplastic syndrome/acute myeloid leukemia) per the international consensus classification (ICC)? See Discussion. |
The final diagnosis on bone marrow biopsy was high grade myeloid stem cell neoplasm, 17% blasts by differential count. The pathologist further states that this could be classified as “MDS with increased blasts (MDS-IB2) per the WHO 5th edition classification, or MDS/AML per the international consensus classification (ICC).” FISH and cytogenetics revealed a loss of 7q, but no other AML-related genetic abnormalities. The physician confirms the patient has MDS/AML. |
Updated Answer July 2024 Code histology as myelodysplastic neoplasm with increased blasts (9983/3) based on the WHO Classification of Hematolymphoid Tumors, 5th edition, Beta version 2. WHO lists MDS with increased blasts-2 (MDS-IB2) as a subtype of 9983/3. Terms coded to 9983/3 include
When differences exist between WHO and ICC, assign the histology based on the WHO Classification. |
2023 |
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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 |