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The behavior code for hemangioblastoma can be coded to /3 when a pathologist indicates that the behavior is malignant. The behavior code should be based on a pathologist's opinion. It is usually not possible for a radiologist or patient care physician to make this determination clinically.

The histologic appearance of hemangioblastoma may resemble metastatic renal cell carcinoma; therefore, one will often see renal cell carcinoma listed as a possible diagnosis. This does not indicate that the hemangioblastoma is malignant. Do not code the behavior as /3 based on a differential diagnosis of renal cell carcinoma.

The WHO Classification of CNS tumours lists glioblastoma with primitive neuroectodermal tumor component as a subtype of glioblastoma and assigns 9440/3. Also referred to as glioblastoma with a primitive neuronal component.

The Solid Tumor Rules for Lung rule H4 applies. Per Table 3, page 12, third column on adenocarcinoma row, adenocarcinoma, acinar predominant is coded to 8551/3.

The Solid Tumor Rules for Lung have been updated for 2024. The row for Small cell carcinoma 8041/3 has been deleted and new separate rows have been added for Neuroendocrine carcinoma (NEC) 8246 and Neuroendocrine tumor, NOS (NET) 8240. This change is based on the WHO Classification of Thoracic Tumors, 5th edition, and current concepts. In addition, Table 3 now reflects that Small cell carcinoma/small cell neuroendocrine carcinoma 8041 (located in Column 3) is a subtype/variant of neuroendocrine carcinoma, NEC 8246 (Column 1). As a result, application of Rule H6 to a diagnosis of poorly differentiated neuroendocrine carcinoma (small cell carcinoma)” would be coded as 8041, instead of 8246. Please note: the 2024 updates may be used for cases diagnosed prior to 1/1/2024 unless otherwise noted in the rules.

The Solid Tumor Malignant CNS tables do not list pituitary specific histologies at this time. Registrars will need to refer to ICD-O and/or updates until the decision to add malignant pituitary neoplasms is made. Pituitary blastoma is a rare tumor which occurs in children.

The SEER rules changed in 1998 regarding what constitutes First Course of Cancer-Directed Therapy.

For cases diagnosed on or after 1/1/98: The First Course of Cancer-Directed Therapy fields will all be coded to 0 [None] for these types of cases. The documented disease progression would stop the timeframe for inclusion of any treatment to be part of first course of therapy.

The R-ICE treatment in this case is not part of the first course. Documentation of treatment failure and/or disease progression signifies the end of the first course of treatment.

The NAACCR Guidelines for ICD-O-3 Histology Code and Behavior Update Implementation, effective January 1, 2018, state: Currently in ICD-O-3, when a topography (C code) is listed in parentheses next to the morphology term, it indicates morphology is most common to that site. It may occur in other sites as well. Many of the new codes, terms, and behaviors listed in this update are site-specific and do not apply to all sites.

Please review the Comments to determine which histology codes are specific to sites. You may use sites not listed as the suggested site; however, it will generate an edit error for review and verification of the appropriate site.

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).

The Histology Task Force approved a 60 day time frame for ambiguous terminology.

The majority of cases are first identified by ambiguous terminology; for example, a patient has a mammogram that shows a lesion suspicious for cancer. That first indication of cancer prompts a work-up to either confirm or rule-out the cancer diagnosis.

The data item "Ambiguous terminology" is not intended to capture information on this routine method of detecting and diagnosing cancer. The 60 day time frame should keep these cases out of the ambiguous terminology data item.

The data item is intended to identify those cases where the cancer diagnosis is NOT confirmed during the work-up, but the case is still entered into the database. For example a patient who has a TRUS because of elevated PSA. The pathology from the TRUS says "Suspicious for adenocarcinoma of the prostate." The physician only documents that the patient is to return in 6 months for another PSA and TRUS. The registrar would enter this case into the data base because the word "suspicious" is on the ambiguous terminology list.