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The Hematopoietic and Lymphoid Neoplasms Database (Heme DB) definition for systemic mastocytosis with an associated hematological neoplasm (SM-AHN, 9741/3) states SM-AHN is a variant of systemic mastocytosis that arises with a myeloid disease of non-mast cell lineage (e.g., MDS, MPN, etc.) and that,

However, SINQ 20130172 conflicts with the Heme DB stating the systemic mastocytosis and the associated hematological neoplasm are a single primary coded to a single histology (9741/3) per Rule M2.

The Heme DB identifies these as new primaries:

10/12/12 Large intestine, biopsy: Diffuse large B-cell lymphoma.

10/12/12 Bone marrow biopsy: Peripheral T-cell lymphoma.

The Heme DB originally stated the Definitive Diagnostic Method is coded to 8 [clinical diagnosis only] while an updated version stated it can coded as a clinical diagnosis or it can be based on the results of a bone marrow biopsy or a genetic test. The Abstractor Note section specifies this is a diagnosis of exclusion. According to a recent Web-based training seminar, the JAK-2 diagnosis would be coded 5 [positive laboratory test/marker study]. Doesn't the Definitive Diagnostic Method of a clinical diagnosis/diagnosis of exclusion mean that the diagnostic confirmation of essential thrombocythemia will always be coded as 8 [clinical diagnosis only]? Many people use code 3 for positive bone marrow biopsy and genetics (JAK-2), but the bone marrow is usually reported as only borderline or is stated to be abnormal for a person's age.

The Histology Coding Instructions give priority to the Final Diagnosis over the CAP protocol. However, when pathology reports are formatted using the CAP protocol, the presence of in situ carcinoma is generally only mentioned in the CAP protocol. Can the presence of in situ tumor mentioned only in the CAP protocol be used to apply rule H7 (Single Tumor: Invasive and In Situ Components Module)? Or are the rules in the Single Tumor: Invasive Only module used?

Example: Final diagnosis is invasive ductal carcinoma. CAP protocol mentions,

The ICD-O-3.2 Coding Table includes Mullerian mixed tumor as the preferred term for histology code 8950 (previously malignant mixed Mullerian tumor/MMMT). This table also includes carcinosarcoma, NOS as the preferred term for histology code 8980. Neither the ICD-O-3.2 Coding Table nor the Implementation Guidelines address the long-standing issue of coding histology for diagnoses of carcinosarcoma/malignant mixed Mullerian tumor.

These endometrial primaries are frequently diagnosed as both carcinosarcoma and MMMT. The questions regarding histology coding for carcinosarcoma and carcinosarcoma/MMMT of the endometrium date back to before the Multiple Primaries/Histology Rules, with at least three SINQ entries instructing registrars not to use code 8950/3 (MMMT) for diagnoses of MMMT. SINQ has instructed registrars that MMMT is a synonym for carcinosarcoma and these tumors should be coded to 8980 (carcinosarcoma), not to 8950 (MMMT). The most recent SINQ is partly inconsistent with the others, indicating 8950 can be used if the tumor is only described as MMMT. The other SINQ entries state carcinosarcoma should be used as it is the preferred term for MMMT. (See SINQ 20061008, 20100009, 20180071.)

The most recent SINQ (20180071) specifically indicates: According to the WHO Classification of Tumors of Female Reproductive Organs, 4th edition, MMMT (8950/3) is now a synonym for carcinosarcoma (8980/3) even though it has a separate ICD-O code. The ICD-O code for MMMT is no longer in the WHO book. However, MMMT is in the ICD-O-3.2 Coding Table and is not stated to be obsolete or a synonym. Which is correct, the clarification in the SINQ or the 2021 ICD-O-3.2 Coding Table?

For a 2021 diagnosis of carcinosarcoma/malignant mixed Mullerian tumor, how should registrars code the histology? Follow the previous SINQ entries and Rule H17 to code the histology to 8980 when the diagnosis includes both carcinosarcoma and MMMT? Do these previous SINQ entries still apply to cases diagnosed 2021 and later?

This question was prompted from preparing SEER*Educate coding exercises. We will use the answer as a reference in the rationales.

The ICD-O-3.2 Coding Table includes hybrid oncocytic chromophobe tumor as a related term for histology code 8317 (Renal cell carcinoma, chromophobe type). However, this related term is not discussed in the implementation guidelines as being a new term/reportable tumor. The Solid Tumor Rules do not indicate a hybrid oncocytic chromophobe tumor is reportable; however, if a registrar only looked at the ICD-O-3.2 Coding Table, it may seem as though this histology should be collected.

The term hybrid oncocytic chromophobe tumor was not included in the Solid Tumor Rules as a subtype/variant of RCC, or as an equivalent term for chromophobe RCC. There is a SINQ (20180047) that states not to report renal hybrid oncocytic tumor, despite the fact these tumors exhibit mixed features of both oncocytoma and chromophobe RCC. For cases diagnosed 2021 and later, should the clarification in the SINQ apply? Or should the ICD-O-3.2 Coding Table be used which indicates this is a reportable diagnosis? If the standard setters decided not to implement use of hybrid oncocytic chromophobe tumor for 2021, can clarification be added to the Solid Tumor Rules or Implementation Guidelines?

This question was prompted from preparing SEER*Educate coding exercises. We will use the answer as a reference in the rationales.

The ICD-O-3.2 Implementation Guidelines and ICD-O-3.2 Coding Table indicate that insulinoma, NOS has changed behavior from /0 to /3 for cases diagnosed 2021 and later. However, the ICD-O-3.2 Implementation Guidelines do not indicate whether this change applies to tumors described as above. Insulinomas are generally neuroendocrine tumors/neoplasms, so it seems any neuroendocrine tumor described as an insulinoma should be collected as 8151/3, but does that apply to an epithelioid tumor/neoplasm also described as insulinoma?

This question was prompted from preparing SEER*Educate coding exercises. We will use the answer as a reference in the rationales.

The ICD-O-3.2 indicates histology 8051 only applies to diagnoses of condylomatous carcinoma and warty carcinoma made prior to 2018. For penis cases diagnosed 2018 and later, these neoplasms should be coded as 8054. This is consistent with SINQ 20200003.

However, a new Table was added to the Other Sites schema in the 2024 Solid Tumor Rules update. Table 23 lists “Verrucous carcinoma / carcinoma cuniculatum / Warty carcinoma” as histology 8051. While verrucous carcinoma is still listed under histology 8051 in the ICD-O-3.2, warty carcinoma is not.

Does Table 23 need to be updated? Or is this an error in both the ICD-O-3.2 and SINQ 20200003?

The Lymphatic and Hematopoietic Diseases folding table states that this should be one primary, but is this true when they are both extralymphatic in origin?

The MP/H Equivalent Terms and Definitions for Colon indicate that type, subtype, predominantly, with features of, major, or with ___ differentiation are all equivalent in terms of coding histology. However, this is not indicated in the General Instructions (e.g., Histologic Type ICD-O-3 or General Instructions Histology Coding Rules). It also is not included as a Note under the Rules where one would expect to use these terms, for example, Rule H7. Is this an oversight or error in the Manual?

In this case, Rule H7 seems to be the first (and most appropriate) rule that applies to this mixed histology tumor. However, the specific histology terms that an invasive tumor may be identified as, are only listed under Rule H13. Can these same terms be used when applying rules for which they are not specifically noted? It would seem logical to use the equivalent histology terms to code a mixed histology tumor identified as a subtype or with features, etc., despite the fact that the specific terms are not listed under Rule H7.