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Report this case of leukemia cutis and code to bone marrow (C421) and leukemia NOS (9800/3) based on the information provided. Update the abstract if new information becomes available.

Leukemia cutis is the rare infiltration of neoplastic leukocytes into the epidermis, dermis, or subcutis from an existing leukemia that results in clinically identifiable cutaneous lesions. Leukemia cutis may precede, follow, or occur concurrently with the diagnosis of systemic leukemia. It is an advanced phase of the leukemia having a poor prognosis that also strongly correlates with additional sites of extramedullary involvement. This can alter the appropriate treatment regimen for a patient. It is a type of "metastasis" or spread of the leukemia cells. The "conventional" definition for leukemia cutis is the infiltration of skin from a bone marrow primary. It is most often diagnosed via skin biopsy—punch, shave, etc., utilizing IHC/biomarker testing and is commonly associated with CMML and acute myeloid leukemia (AML). As such, it a reportable condition especially when preceding a confirmed systemic leukemia diagnosis. In this situation, the diagnosis date would be the date of the positive leukemia cutis skin bx—punch, shave, etc. The case should be coded to C421; 9800/3 Leukemia NOS until the official systemic leukemia diagnosis is rendered. If possible, follow back should be conducted to determine the specific systemic leukemia histology (CMML; AML) and the treatment received. If the leukemia cutis follows or occurs concurrently with the diagnosis of a systemic leukemia, it is NOT a separate primary but merely an advanced stage of the systemic leukemia diagnosis.

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).

Do not report this case of "adenocarcinoma spectrum lesions" based on the information provided in the absence of a more specific diagnosis. Do not report until/unless a definitive diagnosis of malignancy is made. "Adenocarcinoma spectrum lesion" covers a continuum of lung neoplasms from preinvasive lesions (atypical adenomatous hyperplasia and adenocarcinoma in situ) to invasive lesions (minimally invasive adenocarcinoma and invasive adenocarcinoma).

Should additional information become available, report the case and assign the histology code if a more specific histology is confirmed later. Use text fields to record the details.

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.

Assign histology as 8072 for non-keratinizing SCC, undifferentiated subtype.

 WHO Classification of Head and Neck Tumors, 5th edition assigns 8072/3 to squamous cell carcinoma, non-keratinizing, NOS in the nasopharynx. As the tumor exhibits a variety of architectural patterns and appearances histologically, they can be further classified as undifferentiated or differentiated subtypes. These subtypes do not change the histology code.

When there is neoadjuvant therapy followed by surgery, do not record the size from the pathologic specimen. Code the largest size of the tumor prior to neoadjuvant treatment; if unknown code size as 999. We will remove Coding Instruction 12.a.i in the next version of the manual.

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).

Abstract two primaries using Urinary Solid Tumor Rules, Rule M12. The histologies include non-invasive papillary urothelial carcinoma (8130/2) and non-invasive micropapillary urothelial carcinoma (8131/3). The two histology codes are listed as subtypes of Papillary urothelial (transitional cell) carcinoma in column 3 of Table 2.

WHO Classification of Urinary and Male Genital Tumors, 5th edition classifies micropapillary urothelial carcinoma as an aggressive subtype of urothelial carcinoma with carcinoma in situ present in more than half of all micropapillary carcinomas.

Rule 7 Note 3 of the Urinary Solid Tumor Rules states that there are no /2 subtypes for urothelial carcinoma with the exception of papillary urothelial carcinoma and applies to multiple occurrences of /2 urothelial carcinoma of the bladder.

Rule 8 applies to 8131/3 and 8120/3.

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.

Do not report angiomyxoma. ICD-O-3.2 assigns 8841/0 to this benign tumor. This includes superficial and deep (aggressive) angiomyxoma.