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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.

Assign histology as squamous cell carcinoma, HPV positive (8085) for tonsil, NOS (C099) based on the positive HPV test. Codes 8085 and 8086 are valid for a select group of sites. The histology terms and codes that are valid for head and neck sites are included in the Head and Neck Solid Tumor Rules, Table 5 (oropharynx).

HPV detection tests that are used to identify HPV include DNA polymerase chain reaction (PCR), p16 (IHC), or DNA/RNA in situ hybridization. Assign the appropriate method of detection in the SEER data item, SEER Site-Specific Factor 1.  

Schema Discriminator 2 captures additional information needed to generate AJCC ID and Schema ID for some anatomic sites as stated in the SSDI Manual. For oropharyngeal cancer, a schema discriminator is used to discriminate between oropharyngeal tumors that are p16 positive, p16 negative, or p16 status unknown in order to assign the appropriate schema ID. Only the HPV p16 test can be used to assign Schema Discriminator 2. If another HPV test is performed, code 9. Override the edit for Schema Discriminator 2 when p16 is negative. Coding updates will be implemented in 2025.

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.

Assign 8249/3 for high-grade neuroendocrine tumor of the lung.

WHO Classification of Thoracic Tumors, 5th edition, defines two subtypes of neuroendocrine tumor of the lung, typical carcinoids (8240/3), and atypical carcinoids. WHO assigns typical carcinoid/neuroendocrine tumor grade 1 as 8240/3 and atypical carcinoid/neuroendocrine tumor grade 2 as 8249/3. They are regarded as low-grade and intermediate-grade, respectively. The preferred term for 8249/3 in ICD-O-3.2 is neuroendocrine tumor grade 2, with neuroendocrine tumor grade 3 as a related term. The Lung Solid Tumor Rules assign atypical carcinoid as 8249/3.

Report clear cell renal cell papillary tumor (CCRCPT), formerly classified as clear cell renal cell papillary carcinoma, and assign code 8323/3 until this new term and code (8323/1) have been adopted by standard setters.

The Kidney Solid Tumor Rules advise to code clear cell papillary renal cell carcinoma as 8323/3. WHO Classification of Tumors of the Urinary System and Male Genital Organs, 4th ed., has reclassified this histology as a /1. This change has not yet been implemented and it remains reportable.

WHO Classification of Urinary and Male Genital Tumors, 5th ed., has since reclassified clear cell papillary renal cell carcinoma as CCRCPT (8323/1). The name change was made because there have been no reports of metastatic events for this indolent tumor. The term clear cell renal cell papillary carcinoma is no longer recommended.

When pre-operative therapies are given as part of a clinical trial, code as neoadjuvant treatment in the Neoadjuvant Therapy data item when the intent is neoadjuvant and/or when surgical resection follows the clinical trial therapies. 

In the example, neoadjuvant chemotherapy was recommended in the treatment planning and the patient had the planned resection after neoadjuvant treatment. The treatment effect outcome is based on imaging that reported no mass and as documented by the physician, pathologist in this case as complete response to the neoadjuvant therapy based on the resection.

Use code 3 (limited systemic exposure) when treatment does not meet the definition of neoadjuvant therapy in the data item, Neoadjuvant Therapy. Limited exposure occurs when the patient receives some therapy prior to surgical resection, but the treatment is not enough to qualify for a full course of neoadjuvant therapy with the intent to impact extent of surgical resection or other outcomes. While this type of treatment may given as part of a clinical trial, it mostly refers to short term treatments such as hormone therapy.

When neoadjuvant therapy is given prior to surgical resection that is planned (intended) or performed to improve outcomes, use Code 1 or 2. Because a clinical trial is a type of research study that tests new methods of screening, prevention, diagnosis, or treatment of a disease, the treatment regimens likely will not be incorporated in recommended guidelines until all phases of the trial are completed and approved by the U.S. Food and Drug Administration. ClinicalTrials.gov is available to learn more about clinical studies around the world.

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.

A diagnosis of “tubulovillous adenoma with high grade dysplasia” in the duodenum is not equivalent to a diagnosis of “tubulovillous adenoma, high grade.”

Tubulovillous adenoma, high grade (8263/2) is not reportable as of 2022.

High grade dysplasia (glandular intraepithelial neoplasia, grade III) is reportable in the esophagus, stomach, and small intestine (8148/2).

Determine whether the lesion is on the mucosa or skin based on the pathology report, history and physical, and operative notes when available. The gross description of the pathology report should include information to help in determining whether the site of origin is epithelium (skin) or mucosa (lip).

Do not report the case when the site of origin cannot be determined between a reportable site and non-reportable site for this histology. This includes situations where the site of origin or the site with the greatest involvement is undetermined. In this case, you cannot confirm reportability.

The correct term is diffuse leptomeningeal glioneuronal tumor listed as a synonym in Column 2. 

We will add the term diffuse in Note 2, Column 1 with the 2025 updates. In the meantime, you can add "diffuse" to your pdf version until the update is published.