Report | Question ID | Question | Discussion | Answer | Year |
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20200004 | Solid Tumor Rules (2018)/Multiple primaries--Lung: How are Primary Site and EOD Primary Tumor coded when a patient is diagnosed with four invasive tumors in the right lung that represent three separate primaries, but the not otherwise specified (NOS) tumor and one of the specific subtype/variants are in separate lobes? See Discussion. |
There are four invasive tumors in the right lung: Large cell undifferentiated carcinoma in the right lower lobe (8012/3, C343); Adenocarcinoma, acinar-predominant in the right lower lobe (8551/3, C343) that was 0.7 cm in size and limited to the lung; Mucinous adenocarcinoma in the right upper lobe (8253/3, C341) that was 0.9 cm and limited to the lung; Adenocarcinoma, NOS also in the right upper lobe (8140/3, C341) that was 1 cm and limited to the lung. The Lung M Rules confirm the large cell undifferentiated carcinoma is a separate primary from the three adenocarcinoma tumors (Rule M8). The acinar adenocarcinoma and mucinous adenocarcinoma tumors are separate primaries (Rule M6). The adenocarcinoma, NOS tumor is the same primary as both the acinar and mucinous are adenocarcinomas (Rule M7). How is Primary Site coded for both the acinar and mucinous adenocarcinomas if they represent multiple tumors reported as a single primary (when compared to the adenocarcinoma, NOS tumor)? Should the adenocarcinoma, NOS tumor also be included when coding EOD Primary Tumor for both the right lower lobe acinar adenocarcinoma and right upper lobe mucinous adenocarcinoma primaries? Further follow-up with the physician is not possible. |
Abstract three primaries using 2018 Lung Solid Tumor Rules, Rule M6 and M8 as these are multiple synchronous tumors. M6 (Subtypes in Column 3 of Table 3): Adenocarcinoma, acinar predominant: Primary Site: C343 (RLL) EOD Primary Tumor: 300 Mucinous adenocarcinoma Primary Site: C341 (RUL) EOD Primary Tumor: 300 M8 (Separate rows in Table 3): Large cell undifferentiated carcinoma: Primary Site: C343 (RLL) EOD Primary Tumor: 300 Note: The adenocarcinoma, NOS, along with the other subtypes, is on a different row than the large cell undifferentiated carcinoma and is already accounted for in Rule 6 as multiple synchronous tumors. Do not include the adenocarcinoma, NOS in EOD Primary Tumor for the reportable primaries. |
2020 |
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20200082 | Solid Tumor Rules (2018)/Histology--Corpus Uteri: How is histology coded for cases of carcinosarcoma/malignant mixed Mullerian (MMMT) tumor diagnosed 2021 and later? See Discussion. |
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. |
According to both the 4th and 5th Ed WHO GYN Tumors, carcinosarcoma (8980) is the preferred term and pathologists are encouraged to no longer use Mixed Mullerian Tumor (8950) in their diagnoses. WHO 4th Ed GYN now lists MMMT as synonym for carcinosarcoma. 8950/3 is no longer included in WHO 4th Ed. Until the the Other Sites Rules can be updated with histology tables to assist in coding, use the following to determine histology. Carcinosarcoma (8980/3) and MMMT (8950/3)
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2020 |
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20200005 | Multiple Primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned and what M rule applies when a patient is diagnosed with both plasmablastic lymphoma and at least one plasmacytoma? See Discussion. |
The patient was diagnosed with an EBV-positive plasmablastic lymphoma involving the left testis on radical orchiectomy in April 2019. In September 2019, a plasmacytoma was found on a right mandibular mass biopsy. Imaging at that time revealed diffuse disease involving the thoracic spine and sinus involvement. The patient then underwent a resection of the T8 spinal/epidural tumor that also proved plasmacytoma. Subsequently, the right mandibular mass and testis slides were reviewed (at an outside facility) and both were stated to be, The T8/epidural tumor pathology was not reviewed, so it is unclear if this is also assumed to be the same disease process as the right mandibular mass or still a separate, solitary plasmacytoma. Additionally, some chart notes indicate the patient has plasmablastic lymphoma with a secondary diagnosis of plasmacytoma, while other chart notes state this is stage IV plasmablastic lymphoma involving all documented sites. Although the plasmablastic lymphoma and at least the plasmacytoma of T8 have different ICD-O-3 histology codes, the physicians do seem to be treating this as a single disease process. |
Abstract multiple primaries using the Heme and Lymphoid Rule M15. The Multiple Primaries Calculator shows that the plasmablastic lymphoma (9735/3) and extraosseus plasmacytoma (9734/3) are separate primaries. We also checked with our expert pathologist who concurs as the spinal lesion was not reviewed to prove that it is plasmablastic lymphoma, therefore, the diagnosis as per pathology remains plasmacytoma. |
2020 |
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20200061 | Solid Tumor Rules (2018)/Histology--Bladder: A patient has high-grade papillary urothelial carcinoma with focal glandular and neuroendocrine differentiation followed by carcinosarcoma. Is this one or two primaries? See Discussion. |
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? |
The carcinosarcoma is a separate tumor, abstract a new primary per M13. Code this primary to 8980/3. Based on the information provided, the patient was first diagnosed with papillary urothelial carcinoma and received neo-adjuvant treatment for that specific histologic type. Subsequent resection identified carcinosarcoma arising within the papillary neoplasm. Carcinosarcoma is rare in bladder primaries and is not included in Table 2; however, it is a subtype/variant of sarcoma. |
2020 |
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20200085 | Solid Tumor Rules (2018)/Histology--Head and Neck: What is the histology of paraganglioma, NOS arising outside of the adrenal gland (for example, in the bladder) for cases diagnosed 1/1/2021 and later? See Discussion. |
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. |
Code the histology stated by the pathologist: paraganglioma, NOS 8680/3. |
2020 |
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20200087 | Solid Tumor Rules (2018)/Histology--Thyroid: What is the correct histology code for a micropapillary thyroid carcinoma for cases diagnosed 1/1/2021 and later? See Discussion. |
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. |
Continue to code micropapillary thyroid carcinoma to 8260/3 until instructed otherwise. This coding instruction is based on input from expert endocrine pathologists. This issue will be revisted based on the 4th Ed WHO Endocrine Tumors and updated if needed. |
2020 |
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20200073 | Solid Tumor Rules (2018)/Histology--Colon: Should the mixed adenoneuroendocrine carcinoma (MANEC) row in Table 1 include the still often used (yet older) terms of adenocarcinoma and carcinoid, adenocarcinoid, etc. for clarity? See Discussion. |
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. |
The next update to the Solid Tumor rules will include adding the following four terms to Colon Table 1 as synonyms for Mixed adenoneuroendocrine carcinoma 8244
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2020 |
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20200081 | Solid Tumor Rules (2018)/Histology--Pancreas: How is the histology coded, and what H Rule applies, for a 2021 diagnosis when the pathological diagnosis is neuroendocrine tumor (NET) G1 or NET G2, but clinically, the tumor is stated to be insulinoma? See Discussion. |
Insulinoma, NOS is reportable for cases diagnosed 2021 and later. However, the diagnosis of insulinoma is most frequently made with clinical correlation of the patient's clinical syndrome and serum hormone levels. Despite a pathological diagnosis of NET, this will clinically be stated as insulinoma based on the functional type of tumor. At the largest facility in our area, all pathology reports with a diagnosis of insulinoma over the last year only provide a pathological Final Diagnosis of NET (either G1 or G2), but elsewhere specify, Functional Type: Pancreatic neuroendocrine tumor, functional. Correlation with Clinical Syndrome and Elevated Serum Levels of Hormone Product: Insulin-producing (Insulinoma). For 2021 and later, it seems this should be accessioned as insulinoma (8151/3), but one cannot arrive at that histology using the current Other Sites (MP/H) H Rules. Following the existing rules, one would code the histology to NET, G1 or NET, G2 (8240 or 8249) per Rule H6. There are technically two specific histologies to consider: NET (either 8240 or 8249) and insulinoma, NOS (8151). Following the H Rules, Rule H6 instructs one to code the histology with the numerically higher ICD-O-3 code (8240 or 8249). Coding this histology to NET (8240 or 8249) does not seem to reflect the most accurate classification of this tumor, but applying the current rules, this is the only histology that can be coded. There is no current guideline in the Other Sites schema or the ICD-O-3.2 Implementation Guidelines instructing us to ignore the pathological diagnosis of a NET for these tumors (even though insulinomas are NETs). The only SINQ that currently exists (SINQ 20150019) states the histology can be coded as either a NET or an insulinoma in these cases. How are registrars to consistently code histology for these tumors without a rule clarification? This question was prompted from preparing SEER*Educate coding exercises. We will use the answer as a reference in the rationales. |
Code the tissue/pathology histology over the clinical diagnosis. Because of implementation timelines, a comprehensive revision to Other Sites rules will not be available 2022. A limited revision is planned and histology tables will be added for select sites. The General Instructions will also be revised for Other Sites. |
2020 |
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20200064 | Primary site--Heme &Lymphoid Neoplasms: What is the primary site of two extraosseous plasmacytomas, with positive pathology of right orbit and left lung. The patient's bone marrow biopsy, flow, and peripheral blood smear were negative. Is this coded as 9732/3, multiple myeloma (Primary Site and Histology Rule PH2) with the primary site as C809 (PH27)? Or is the primary site C421 since code 9732 says primary site is always C421, though bone marrow came back as negative? |
Assign the primary site to C421 since that is the only allowable primary site for plasma cell myeloma, even though the bone marrow was negative. According to the revised criteria from the WHO Blue Book for Hematopoietic and Lymphoid Neoplasms (2017), the presence of multiple plasmacytomas is plasma cell myeloma (9732/3). |
2020 | |
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20200049 | Summary Stage 2018/EOD 2018--Lymphoma Orbital Adnexa: What is the correct Summary Stage 2018 (SS2018) for the site/histology Orbit, NOS (C696), 9699/3? In SEER*RSA, Extent of Disease (EOD) Primary Tumor references code 7 (Distant), whereas SS2018 assigns code 2 (Regional)? See Discussion. |
We received an edit error in SEER*DMS on the following site/histology (Orbit, NOS (C696)/9699/3) that involved an incorrect staging code being assigned to SS2018. The staging language is identical in AJCC, EOD and SS2018. SEER*RSA notes that SS2018 should be coded distant, but in the SS2018 manual, this language is noted Regional. Staging language is: Orbital adnexal lymphoma AND extraorbital lymphoma extending beyond the orbit to adjacent structures--Bone, Brain, Maxillofacial sinuses |
To clear this edit of the derived Summary Stage (based on EOD) and the manually assigned Summary Stage (based on Summary Stage 2018), assign the manually assigned Summary Stage to 7. For this particular case, EOD Primary Tumor 700 (which is correct based on the information received) derives Distant; however, for Summary Stage 2018, this description is under Code 2 for Regional by direct extension. This is an error. For 2022, Summary Stage for Lymphoma Ocular Adnexa description under Code 2 (Regional by direct extension) will be moved to Distant. No changes will be done to EOD. |
2020 |