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Per rule PH11, "Code the primary site to the site of origin (lymph node region(s), tissue, or organ) and code the histology diffuse large B-cell lymphoma (DLBCL) (9680/3) when DLBCL and any other non-Hodgkin lymphoma are present in the same lymph node(s), lymph node region(s), organ(s), tissue(s) or bone marrow."

Should the focal diffuse large B cell lymphoma be ignored in this case and rule PH11 not be applied? To apply rule PH11, does the follicular lymphoma have to be NOS [9690/3] or does PH11 include all grades of follicular lymphoma [9695/3, 9691/3, 9698/3]?

This terminology is not specifically included in either alternate names list for myelodysplastic syndrome, NOS (9989/3) or refractory anemia with excess blasts (9983/3).

Example: Bone Marrow Biopsy, Final Diagnosis: Consistent with involvement by myelodysplastic syndrome with excess blasts-2 (MDS EB-2).

While we recognize the Heme DB has been the correct source for histology coding for heme and lymphoid neoplasms dating back to 2010, the ICD-O-3.2 Implementation Guidelines appear to provide incorrect coding instructions. Histologies 9670/3, 9728/3, 9729/3 and 9836/3 are listed in Table 3 - Deleted ICD-O codes in ICD-O-3.2.

While we recognize these histologies have been included in this Table because they have now been deleted, it is unclear whether the Comments regarding their use listed in the 4th column of the Table is correct. For each of these histologies, the comment states the histology listed in the 1st column (ICD-O-3/3.1) should be used prior to 2021. For example, for histology 9670/3, the comment states: Cases diagnosed prior to 1/1/2021 use code 9670/3. Cases diagnosed 1/1/2021 forward use code 9823/3. However, each of these histology codes have been obsolete for cases diagnosed 1/1/2010 and later. If registrars were following the Heme DB and Heme Manual instructions (the appropriate coding source for these neoplasms), these histologies would not have been used in a decade.

Should the Comments column in Table 3 be updated? Or should a Note follow the Table indicating registrars should not use these histology codes for cases diagnosed after 1/1/2010, and these histology codes have been deleted for cases diagnosed 1/1/2021? It seems misleading to indicate any of these are valid histology codes for a 2010-2020 diagnosis when the Heme DB confirms these histology codes only apply to cases diagnosed prior to 2010.

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.

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.

This patient has only nodal involvement for this EBV positive NK/T cell lymphoma. There is no extranodal involvement, specifically no nasal involvement. Additionally, the immunophenotyping does not match the immunophenotyping listed for histology 9719. Therefore, histology 9719 (Extranodal NK/T cell lymphoma, nasal type) does not seem applicable to this case. This patient is an adult, so histology 9724 (Systemic EBV-positive T-cell lymphoma of childhood) also does not seem to be a match. The most recent WHO Classification seems to classify these as 9702. Is this the appropriate histology for an EBV-positive nodal NK/T-cell lymphoma in an adult?

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.

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.

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?