| Report | Question ID | Question | Discussion | Answer | Year |
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20250015 | Solid Tumor Rules/Behavior--Brain and CNS: Why was the Behavior of solitary fibrous tumor (SFT)/hemangiopericytoma, WHO Grade 1 changed from /0 to /1 in the 2025 Solid Tumor Rules (STR) updates? See Discussion. |
In previous STR versions and the ICD-O-3.2, SFT/hemangiopericytoma, WHO G1 is 8815/0 and only SFT/hemangiopericytoma, WHO G2 was 8815/1. However, Table 6 (Non-Malignant CNS, Specific Histologies, NOS, and Subtypes/Variants) was changed in the 2025 updates to indicate both G1 and G2 SFT/hemangiopericytoma are 8815/1. No date range was provided for this change in the STR and the behavior of this tumor was not updated by the standard setters in other references (i.e., ICD-O-3.2). The behavior of G1 SFT/hemangiopericytoma was not updated in the 2025 ICD-O-3.2 updates. If the ICD-O-3.2 was the source of this change, should this have been documented in the 2025 NAACCR Implementation Guidelines? However, the 2025 NAACCR Implementation Guidelines indicates, "There are no ICD-O-3 changes for 2025." Is this behavior change in 2025 Solid Tumor Rules updates an error? Should the behavior of SFT/hemangiopericytoma, WHO G1 remain /0? |
For cases diagnosed 2025 and later: Assign behavior /1 for solitary fibrous tumor unless stated to be malignant. A review by the Cancer PathCHART expert neuropathologists found behavior code /0 is incorrect and both solitary fibrous tumor grade 1 and grade 2 are coded as 8815/1. WHO Classification of Central Nervous System Tumors, 5th edition, assigns behavior as /1 and no longer recommends terms solitary fibrous tumor/hemagiopericytoma and hemagiopericytoma. The STR table is correct. Future updates to ICD-O should reflect this behavior. WHO Classification of Tumours, Central Nervous System Tumours, 5th ed. was reviewed by the CPC expert pathologists for implementation for cases diagnosed January 1, 2025. Reminder: Comparing the CPC Validity Status included in the 2024 CPC*Search to that included in the 2025 SMVL (that table that drives the edits) is incorrect. CNS Tumors were not reviewed for 2024 implementation, they were reviewed for 2025 implementation. There will be a 2025 CPC*Search and a /1 will be designated as a Valid. |
2025 |
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20240067 | Reportability/Ambiguous Terminology--Kidney: Is a clinical diagnosis of a right kidney lesion with a “75% chance of malignancy” reportable when no further information is available? See Discussion. |
The CT findings identified a right kidney rim-enhancing centrally cystic lesion most suggestive of clear cell renal cell carcinoma measuring 3.2 cm. The radiologist’s impression was “concerning for renal cell carcinoma.” The subsequent urologist’s consult states the right kidney lesion has a 75% chance of malignancy. The urologist discussed active surveillance, surgery, and ablation, and after discussion with the patient the plan was for active surveillance. No further information is available, and we are unable to follow up with the physician regarding this case. Should a lesion with a high percentage chance of malignancy (e.g., 75% chance) be considered a lesion “most likely” to be malignant? |
If you are unable to follow up with the physician, do not report this case until or unless more information becomes available. |
2024 |
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20240052 | Reportability/Histology--Heme & Lymphoid Neoplasms: Should a non-Langerhans cell histiocytosis involving the hypothalamus and pituitary gland be accessioned as a reportable, behavior /1, CNS neoplasm? See Discussion. |
Imaging identified a mass involving the hypothalamus and pituitary gland and excision of the mass proved “histiocytosis.” The case was extensively reviewed, and the physician notes this patient has a pituitary tumor that is a “non-Langerhans cell histiocytosis,” or a “non-LCH histiocytic neoplasm.” There is no histology for histiocytosis (NOS) or non-Langerhans cell histiocytosis. However, this does appear to be a non-malignant histiocytic neoplasm. If this were a Langerhans cell histiocytic neoplasm in the CNS it would be reportable. Should this non-Langerhans cell histiocytic neoplasm also be accessioned as a reportable CNS neoplasm? If so, how is the histology coded? |
Report this case as a pituitary tumor (8000/1) based on the information provided. This is the best choice as no specific histology code exists for this generic term “non-Langerhans cell histiocytosis” in ICD-O-3.2, WHO Classification of CNS Tumors, 5th ed., and WHO Classification of Tumors of Hematopoietic and Lymphoid Tissues, 4th ed. Be sure to double-check the behavior code of the tumor. Histiocytosis can be benign, borderline, or malignant. There was no mention of the behavior so we defaulted to uncertain behavior for this case. |
2024 |
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20240038 | Solid Tumor Rules/Multiple Primaries--Brain and CNS: How many primaries are accessioned, and what M Rule applies to a 2023 diagnosis of pituitary macroadenoma followed by a 2024 diagnosis of pituitary neuroendocrine tumor (PitNET) when the patient did not undergo surgery, but did undergo hormone therapy with Cabergoline? See Discussion. |
Malignant Central Nervous System (CNS) Rule M5 instructs us to abstract a single primary (as malignant) when a single tumor is originally diagnosed as non-malignant, the “First course treatment was active surveillance (no tumor resection),” and the subsequent resection pathology is malignant. This patient’s first course of treatment was not active surveillance. While the patient did not have first course tumor resection, the tumor was treated with Cabergoline. Should Rule M5 apply because there was no tumor resection? If so, should Rule M5 clearly state no tumor resection is the criteria (not active surveillance)? SINQ 20230023 does indicate a PitNET diagnosis following a diagnosis of pituitary adenoma does not fall into standard rules, but in the previous SINQ the first course treatment was a partial resection. It is unclear whether other types of treatment could result in a new malignant PitNET, following a previously treated non-malignant pituitary tumor. |
Abstract a single primary as 8272/3 (pituitary adenoma/PitNET) using the Malignant CNS and Peripheral Nerves Solid Tumor Rules, Rule M2, a single tumor is always a single tumor. Change the histology of the 2023 diagnosis to 8272/3. This scenario does not meet the criteria in the current rules for M5 in that it requires a resection as part of the criteria. Since the patient did not undergo resection for either diagnosis, the 2024 diagnosis may indicate recurrence or progression. A diagnosis of pituitary adenoma only is still coded 8272/0 (this code is still valid). A diagnosis of pituitary adenoma/PitNET, PitNET, or pituitary neuroendorine tumor is coded 8272/3. Cabergoline is used to treat prolactinoma or high levels of prolactin but does not impact the PitNET. |
2024 |
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20240007 | Histology--Brain and CNS: Provide clarification about the priority order of histology coding sources and an explanation of why the annotated histology lists are not the same as the WHO IARC ICD-O-3.2 Excel Table (adopted 1/1/2021). See Discussion. |
We have had multiple users unable to find the applicable histology in the ICD-O-3.2 (i.e., the site-specific table did not include the histology) because they were using the annotated histology list and could not find the complete list of related terms or synonyms for the histology code. For example, the ICD-O-3.2 lists Medulloblastoma, SHH-activated, NOS as a related term for 9471/3, but many users were unable to find this valid histology because they were using the annotated histology list, not the ICD-O-3.2. |
The NAACCR Annotated Histology List (AL) serves as an aid to registry software vendors for implementing annual histology changes. This file has been maintained by the Registry Plus team at CDC’s NPCR for several years and reflects modifications to ICD-O-3 implemented by North American cancer registries over time. Although this list is reviewed multiple times prior to posting, there is no guarantee of 100% accuracy. As such, the AL is not a substitute for referring to various standard-setter documents and implementation guidelines. In this instance, Medulloblastoma Desmoplastic SHH-activated and TP53-wildtype 9471 is across several resources: the Solid Tumor Rules, Malignant CNS and Peripheral Nerves module in Table 3, column 3 as a subtype/variant of Medulloblastoma NOS 9470; in the CNS WHO 5th Edition BB; and in the WHO IARC ICD-O-3.2 posted to ICD O 3 Coding Updates (naaccr.org). Although the exact related term of Medulloblastoma, SHH-activated, NOS is not listed, the NAACCR Implementation Guidelines for 2024 recommend checking the 2024 ICD-O-3 Update Table 1 or 2 to determine if the histology is listed. If the histology is not included in the update, then review ICD-O-3.2 and/or Hematopoietic and Lymphoid Database and/or Solid Tumor Rules (MP/H). The Cancer PathCHART initiative has been undertaken to address gaps such as this between standard setting resources. Having all the standard histology coding resources included in a single all-inclusive database enables alignment of morphology codes & terms included in the CPC*SMVL (Cancer PathCHART Site-Morphology Validation List), Solid Tumors Rules, ICD-O-3 Annual Updates, NAACCR Annotated Histology List as well as the WHO 5th edition Blue Books. Please see Cancer PathCHART - Tumor Site-Morphology Surveillance Standards Initiative for more information on the Cancer PathCHART initiative, and more specifically, see Transitioning the Annotated Histology List to Cancer PathCHART (naaccr.org). |
2024 |
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20240077 | 2024 SEER Manual/Primary Site--Retroperitoneum: What is the primary site code for a final diagnosis of endometrioid adenocarcinoma from a biopsy of a right retroperitoneal mass? See Discussion. |
An 80-year-old post-menopausal female (status post hysterectomy for benign reasons) presents with a retroperitoneal mass on imaging. The pre-operative imaging shows the cervix and uterus are absent. Patient undergoes a robotic left salpingo-oophorectomy with biopsy of the retroperitoneal mass. |
Code Primary Site to C480 (retroperitoneum). Endometrial tissue may "break away” from the uterus and implant throughout the pelvic and abdominal cavities. This can occur in patients who suffer from endometriosis. This tissue remains behind when surgical removal of the uterus is done. Common sites of implantation are colon, peritoneum, retroperitoneum, and bladder. These cells may become malignant. When the uterus is no longer present (patient had surgical removal), code the site where the carcinoma was identified. The site-morphology combination of C480 and 8380/3 was designated as an unlikely site-morphology combination by the Cancer PathCHART expert pathologist review, as this is a rare type of tumor. Assign a value of 1 in the Over-ride Site/Type [2030] data item in order to pass the Primary Site, Morphology-Type, Beh ICDO3, 2024 (SEER) edit. |
2024 |
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20240058 | Summary Stage 2018--Ovary: What is the summary stage for an ovarian primary in 2024, in which the ovary capsule was ruptured with surgical spill? See Discussion. |
In this case, the surgeon ruptured the ovarian tumor to drain it prior to removal causing the surgical spill. Regional lymph nodes are negative and there is no metastasis. The capsule was then noted as ruptured on pathology. Does it matter if the surgeon was the one who ruptured the capsule? Would the stage change if the surgeon intentionally ruptured the capsule to drain the tumor intraoperatively causing some surgical spill? The scenarios of an intentional and not intentional rupture are not specified in SEER Summary Stage 2018. |
Code SEER Summary Stage 2018 to Localized, Code 1. Per consult with AJCC and noted in the Primary Peritoneal Chapter in AJCC 8th edition, an intraoperative rupture is coded as a surgical spill. A capsule rupture is when the capsule ruptures prior to the surgery (Summary Stage Regional, Code 2). |
2024 |
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20240035 | Solid Tumor Rules--Urinary: The example used in Rule M15 of the Urinary Solid Tumor Rules refers to the same row in Table 3. Should the example say Table 2 since Table 3 is non-reportable urinary tumors. See Discussion. |
Rule M15 Abstract a single primary when synchronous, separate/non-contiguous tumors are on the same row in Table 2 in the Equivalent Terms and Definitions. Note: The same row means the tumors are • The same histology (same four-digit ICD-O code) OR • One is the preferred term (column 1) and the other is a synonym for the preferred term (column 2) OR • A NOS (column 1/column 2) and the other is a subtype/variant of that NOS (column 3) OR • A NOS histology in column 3 with an indented subtype/variant Example: TURBT shows invasive papillary urothelial carcinoma 8130/3 and CIS/in situ urothelial carcinoma 8120/2. Abstract a single primary. Papillary urothelial carcinoma and urothelial carcinoma are on the same row in Table 3. |
The example used in Rule M15 of the Urinary Solid Tumor Rules should refer to Table 2. We will update this in the next revision of the Rules. |
2024 |
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20240066 | Histology--Heme & Lymphoid Neoplasms: How should histology be coded for a pathologic diagnosis of “Follicular lymphoma, diffuse pattern grade 3A of 3, equivalent to diffuse large B cell lymphoma (germinal center cell type)” when later referenced clinically as follicular lymphoma grade 3A? See Discussion. |
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. |
Assign histology as DLBCL (9680/3) as supported by the WHO Classification of Hematolymphoid Tumors, 5th edition. It is consistent with how it would have been coded in the 4th edition. The Heme Manual and Database currently are based on the 4th edition. Physicians are using the 5th edition blue book, whereas the cancer registry field is not yet at this time. Regarding the Heme Manual and Database, this type of scenario is not covered because it is part of the 5th edition WHO Blue Book. The database cannot be updated until the 5th edition is approved for implementation (2026). |
2024 |
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20240019 | Solid Tumor Rules/Histology--Head and Neck, Other Sites: Do human papilloma virus (HPV) histologies that occur with subtype/variants of squamous cell carcinoma (SCC) in various sites apply only to sites in Solid Tumor Rules, Head and Neck, Table 5 and Other Sites, Table 23? See Discussion. |
The 2024 Solid Tumor Rules, Table 5: Tumors of the Oropharynx, Base of Tongue, Tonsils, Adenoids contain notes that say beginning 1/1/2022, keratinizing or non-keratinizing SCCs, HPV positive or HPV negative, are coded 8085 or 8086, respectively, for sites listed in the Head and Neck Solid Tumor Rules, Table 5 only. Table 5 introductory section also states for cases diagnosed 1/1/2023 forward: “When the diagnosis is a subtype/variant of squamous cell carcinoma and HPV status is also noted, code the subtype/variant.” This latter instruction is also included in Other Sites Table 23 (Penis and Scrotum Histologies) as a “Penis Coding Note.” Do these instructions ONLY apply to sites on those tables (and only to Penis or to Scrotum also in Table 23)? How should we code HPV-related keratinizing/non-keratinizing or other subtype/variant SCCs, for sites NOT on those tables, given the fact that only the more common histologies are listed in the Solid Tumor tables? For example, we recently reviewed a case with HPV-positive basaloid squamous cell carcinoma of the anus (C21.0). |
Code the specific histology as stated by the pathologist according to the site-specific instructions in the Solid Tumor Rules. When the histology provides a subtype/variant in addition to the HPV histology codes, code the subtype/variant as it is important to capture this histology as in the example provided. the instruction to code the subtype/variant over 8085 or 8086 applies to the following sites: oropharynx, cervix, vagina, vulva, anus, and penis. A note will be added indicating this in 2025. Per 2024 Cancer PathCHART expert pathologist review, morphology codes 8085/3 and/or 8086/3 are valid and applicable to head and neck, oropharynx, cervix, vagina, vulva, fallopian tube, anus, and penis (reference: Cancer PathCHART: Product Downloads and Timelines). Other coding resources will be updated to reflect these changes in 2025. |
2024 |
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