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The 2021 ICD-O-3.2 Update includes papillary microcarcinoma (8341/3) as the preferred term for thyroid primaries (C739). However, there are multiple SINQ entries instructing registrars not to use code 8341/3 for diagnoses of micropapillary carcinoma of the thyroid (including SINQ 20071076, 20081127, 20110027, 20150023, and 20180008).

SINQ 20150023 specifically indicates: Per the WHO Tumors of Endocrine Organs, for thyroid primaries/cancer only, the term micropapillary does not refer to a specific histologic type. It means that the papillary portion of the tumor is minimal or occult (1 cm or less in diameter) and was found incidentally. WHO does not recognize the code 8341 and classifies papillary microcarcinoma of the thyroid as a variant of papillary thyroid carcinoma and codes histology to 8260. If the primary is thyroid and the pathology states papillary microcarcinoma or micropapillary carcinoma, code 8260 is correct.

Does this clarification apply to cases diagnosed 2021 and later? If WHO feels the term micropapillary still does not refer to a specific histologic type for the thyroid, why is 8341/3 listed as a preferred term for this morphology/site combination? For cases 2021 and later, should a diagnosis of Incidental papillary thyroid microcarcinoma (3 mm) in left lower pole, be coded as 8341/3 per the ICD-O-3.2, or as 8260/3 per clarification in multiple SINQ entries?

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

According to the literature, PMH is a low-grade malignant vascular neoplasm of different tissue planes including skin and soft tissue. However, the references also state: PMH is a cutaneous tumor that behaves in an indolent fashion. There is no indication that this was a malignant diagnosis.

12/3/18 Foot, left skin lesion, punch biopsy: Superficial squamous epithelium demonstrating hyperkeratosis and fragments of keratin debris, no tumor seen.

Foot, left skin lesion, punch biopsy: Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma, see note.

NOTE: The submitted immunohistochemical slides were reviewed. Positive and negative controls reacted appropriately. The tumor cells demonstrate immunoreactivity to CK AE1/AE3 and CK7. The CD31 immunoreactivity described in the report cannot be confirmed as only the positive control is submitted for review. The tumor cells are negative for desmin, CD45, CD68, S-100, CD34, SMA, CD20, and HHV8. The proliferative index via Ki-67 is approximately 10%. The morphology (described below) and immunohistochemistry performed are compatible with a pseudomyogenic hemangioendothelioma.

12/4/18 Final Pathologic Diagnosis: Foot, left bone lesion, biopsy: Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma, see note.

Note: The patient's imaging findings were reviewed in conjunction with this case, revealing numerous lytic lesions of the tibia, fibula, talus, tarsal, metatarsal, and phalangeal bones. Additionally, as per the medical record, also reviewed in conjunction with this case, there are lesions of the skin. Thus, an extensive immunohistochemical panel was performed in an attempt to support the morphologic findings in this case, which were morphologically similar to the patient's skin biopsy. The tumor cells demonstrate strong immunoreactivity to pancytokeratin (CK AE1/AE3) and vimentin with moderate immunoreactivity to Fli-1. The tumor cells demonstrate weak immunoreactivity to epithelial membrane antigen. INI-1 is retained. There is focal immunoreactivity to CD31 although this is limited to the edges of the tissue fragments. The tumor cells are negative for HHV-8, CD34, smooth muscle actin, CK8/18, desmin, CD99, and Bcl-2. The combination of morphologic (see below for microscopic description) and immunohistochemical findings are consistent with pseudomyogenic hemangioendothelioma. Fresh tissue was submitted for karyotype analysis at the time of intraoperative consultation; however, it revealed only a normal appearing male karyotype. Thus, molecular confirmation was sought. The original slides and a paraffin block were submitted for FOSB rearrangement analysis, as pseudomyogenic hemangioendothelioma is known to have recurrent rearrangements with FOSB. Additional immunohistochemistry performed at (FACILITY) demonstrating immunoreactivity for ERG, supporting a vascular origin for this neoplasm. Fluorescence in situ hybridization demonstrated that 13% of the cells examined show FOSB rearrangement. While this FISH probe is for investigational purposes, the above findings support the diagnosis of pseudomyogenic hemangioendothelioma.

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.

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.

Should histology be coded as paraganglioma, NOS (8680/3) or as extra-adrenal paraganglioma, NOS (8693/3) for a diagnosis of paraganglioma in the bladder? Does the pathologist have to specifically diagnose the tumor as extra-adrenal paraganglioma, NOS to use histology code 8693/3? Or, does any diagnosis of paraganglioma (NOS) arising outside of the adrenal gland, carotid body, middle ear, or aortic body (the specified sites for other types of paragangliomas) qualify as an extra-adrenal paraganglioma, NOS?

The ICD-O-3.2 Implementation Guidelines (Tables 6 and 7) provide an associated site of C755 for histology 8680/3 (paraganglioma, NOS), but no associated site code is provided for histology 8693/3 (extra-adrenal paraganglioma, NOS). If the preferred site for paraganglioma, NOS is the paraganglia, would a paraganglioma in the bladder be an extra-adrenal paraganglioma?

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

Race information is provided in the Centers for Medicare and Medicaid Services (CMS) linkage results. Oftentimes it matches what is coded in the database, but other times it does not.

In situations where the CMS (or other) linkage provides a race value that differs from the coded Patient set, are we to ignore the CMS stated race given the SEER Manual instructions indicating self-reported race has priority or should we add the different Race values from linkages as an additional race (ex. Race 02)?

Is this histologic terminology synonymous with 8071/2 Differentiated-type vulvar intraepithelial neoplasia?

Per the 7/20/2018 updates to the 2018 ICD-O-3 Histology list, the reportability flag was changed from N to Y for Differentiated-type vulvar intraepithelial neoplasia as well as Differentiated penile intraepithelial neoplasia, both 8071/2. It appears that both SINQ 20180020 and the second half of SINQ 20160069 are no longer valid and should be deleted.

If the term discontinuous foci means separate tumors, then rule M14 would apply making these multiple reportable tumors.

The Terms and Definitions Introduction discusses how these are older terms, but pathologists may still use them. In our region, pathologists do, in fact, still use these terms. Can these terms be added to Table 1? For registrars who do not reference the Introduction every time they code histology but go directly to Table 1, coding consistency would likely improve if such terms were added in the Table.

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