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12-19-19 Transurethral resection of bladder tumor pathology revealed high-grade papillary urothelial carcinoma with focal glandular and neuroendocrine features; Pathology Overread: High-grade papillary urothelial carcinoma with focal glandular and neuroendocrine differentiation. Carcinoma invades muscularis propria pT2.

Histology 8130

01/20/20 to 07/01/20, completed 6 cycles of gemcitabine/cisplatin.

07/30/20 Robotic radical cystoprostatectomy with bilateral pelvic lymph node dissection, open ileal conduit pathology revealed carcinosarcoma, invading perivesical fat, no lymphovascular invasion, negative margins. ypT3bN0M0 disease; Pathology Overread: Carcinosarcoma arising in association with high-grade papillary urothelial carcinoma.

Histology 8980/3 or is there another histology that should be used?

There is no specific ICD-O-3 code for a which resulted in abstractors assigning the malignant astrocytoma, NOS code. These lesions were previously called but we are seeing the new terminology more frequently.

Example: Patient is found to have a 9.4 cm GIST in the jejunum and 2 cm GIST in the stomach during resection, neither stated to be outright malignant. Similar to the instruction in SINQ 20190041, this case is coded as a malignant jejunal primary due to multifocal tumor. However, it is unclear how to account for the stomach tumor, or any other multifocal tumor for GIST, when coding EOD and Summary Stage.

Rule M4 does not seem to apply because Note 1 defines clinically disease free to mean no evidence of recurrence in the same lung on follow-up. Patient had been disease free in the left lung after 09/2014 diagnosis. The 04/2019 diagnosis was in a different lung than the 4/2016 diagnosis.

The next applicable rule is either M11 or M14 depending on how we should compare the new 2019 tumor: to the most recent prior tumor in 2016 or to both prior tumors.

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 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.

SINQ 20130140 indicates a histologic diagnosis that is characteristic of a specified malignancy is reportable because this is equivalent to the term, diagnostic of. Does the same logic apply to a clinical diagnosis that is strongly characteristic of a malignancy on imaging?

SINQ 20180104 indicates the term, almost certainly, is not a reportable ambiguous term. If a radiologist notes a mass was most certainly malignant, is this adequate to accession this as reportable? Is a clinically certain diagnosis equivalent to diagnostic of? Or are the modifiers almost and most irrelevant because the terms certainly and certain are not on the ambiguous terminology list?

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 radiologist noted the mass was most compatible with a chondroid lesion, which is not reportable on its own, but can the subsequent term be used to accession this as reportable if only one malignant etiology is provided by the radiologist? Or does the statement imply that this is only one of several possible etiologies?

First, reportability of clear cell papillary renal cell carcinoma changed from 8323/3 to 8323/1. Although it does not appear the standard-setters implemented this change, note of the conflict between the ICD-O-3.2 and the Solid Tumor Rules (STR) is not included in the Implementation Guidelines or STR. The current Note for clear cell papillary renal cell carcinoma (8323) was left in Table 1, so this presumably is still reportable. It would be helpful if the conflict with ICD-O-3.2 was addressed, especially since the existing Note refers to changes made back in 2016 (not 2018 or 2021).

Second, is the term sarcomatoid renal cell carcinoma still coded as a synonym for renal cell carcinoma (8312) because sarcomatoid is referring to a pattern of differentiation or 8318 (renal cell carcinoma, sarcomatoid)? The STR, Table 1, lists sarcomatoid renal cell carcinoma as 8312, but the ICD-O-3.2 lists this as 8318. The Note in Table 1 still indicates WHO/IARC and College of American Pathologists agree that sarcomatoid carcinoma is a pattern of differentiation, not a specific subtype, of renal cell carcinoma. This appears to conflict with WHO/IARC ICD-O-3.2 Coding Table as it provides a different, specific histology code for this malignancy. How can WHO/IARC classify this both a pattern of renal cell carcinoma and a separate, specific histology?

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