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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.

Abstract this case as a single primary. Code as 8044/3 (small cell carcinoma, hypercalcemic type) listed in the Other Sites Solid Tumor Rules, Table 13. Small cell carcinoma, large cell variant, is a subtype of small cell carcinoma, hypercalcemic type. This table does not include all possible histologies.

WHO Classification of Female Genital Tumors, 5th edition, states: Small cell carcinoma of the ovary, hypercalcemic type, is rare, accounting for < 1% of ovarian tumors. Small cell carcinomas, hypercalcemic type, are usually large, with a mean size of 15 cm (range: 6–26 cm). Large cells are present (in varying numbers) in half of these tumors, which are designated “small cell carcinoma, large cell subtype” if the large cells are predominant (which is rare).

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).

Report malignant spindle cell neoplasms consistent with atypical fibroxanthoma as directed by Appendix E.1 of the 2023 and 2024 versions of the SEER Manual using 8830/3 (fibroxanthoma, malignant).

We will update the answer in SINQ 20190102.  While the Other Sites Solid Tumor Rules address coding an NOS and specific histology sub-type/variant, this situation is not specifically addressed. We will also review the rules.

Change to current instructions. We will update this example in the next edition of the manual. The new example will instruct registrars to look for other descriptions of the patient’s race. When no other information is available, assign 07, Native Hawaiian, in Race 1 and assign 97, Pacific Islander, NOS in Race 2. Begin following this new instruction now.

Code histology as combined small cell carcinoma (8045) based on the Other Sites Solid Tumor Rules, May 2023 Update, Table 2, Mixed and Combination Codes, for this mixed histology prostate carcinoma consisting of adenocarcinoma and small cell neuroendocrine carcinoma regardless of treatment status. This is similar to SINQ 20200052 that applies to one tumor with mixed histologies.

1) Do not infer the percent of viable tumor if only percent of necrosis is provided. For the example, assign code 6 when Neoadjuvant therapy was completed and the treatment effect in the breast is stated only as “Present".

2) Do not use the residual cancer burden (RCB) score from the pathology report to code the Neoadjuvant Therapy--Treatment Effect field for breast cancer. We do not have a crosswalk from RCB to neoadjuvant Therapy--Treatment Effect.

RCB index is an accurate and reliable tool to assess patient prognosis. RCB is estimated from routine pathologic sections of the primary breast tumor site and the regional lymph nodes after the completion of neoadjuvant therapy. The data item Neoadjuvant Therapy--Treatment Effect records information on the primary tumor only.

Document information in a text field in both examples.

Your case is equivalent to encapsulated follicular variant of papillary thyroid carcinoma, non-invasive (non-invasive EFVPTC) and is not reportable for cases diagnosed in 2021 or later even though it says "carcinoma." That is because the WHO assigned a behavior code of /1 to this entity (8349/1). NIFTP is assigned to the same histology and behavior code.

Table 19 is based on WHO 5th Ed Tumors of vulva and squamous cell variants, keratinizing and non-keratinizing, are no longer recommended and are excluded from the 5th Ed. HPV related terminology is now preferred for these neoplasms.

Per consultation with our GYN expert pathologist, based on the information provided, this is likely a single tumor that was not completely excised in the original biopsy. A new tumor in the same site would not appear within 8 months. If you cannot confirm two separate/non-contiguous tumors were present, abstract a single primary per M1. As for histology, the tumor showed both keratinizing and non-keratinizing features and HPV status is unclear. Per our expert, code to SCC 8070/3—keratinization or lack of does not change treatment or prognosis.

Even If there is proof of separate/non-contiguous tumors, our expert still feels this is a single primary coded to SCC 8070/3. Treatment does not differ by keratinization or HPV status. Coding two primaries would be incorrect and inflate incidence rates.

Per our expert, this is an unusual occurrence. The rules cover 85% of cases but there will always be situations that do not fit a rule. This case is an example of that.  A new GYN specific Solid Tumor Rules module is under development and a rule to address this situation could be included.

Assign code 300 for EOD Prostate Pathologic Extension.  

In this scenario, the patient has a localized cancer confirmed by radical prostatectomy; the needle core biopsies likely removed all the cancer. Unlike prostate, other sites’ extension information is collected in EOD Primary Tumor, as seen commonly with breast tumors where the results from the surgical resection are recorded with tumor confined to primary site.