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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 Primary Site code C504 based on the location in the upper outer quadrant of the right breast, 10 o’clock, as opposed to code C501, around the areolar complex. The 2024 SEER Manual Breast Coding Guidelines advise that C502 - C505 are generally preferred over C501 when there is no other way to determine the subsite.

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).

Abstract a single primary for this scenario based on Urinary Sites STRs. 

10/2017 and 12/2017 bladder diagnoses:  Single primary (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). This interval is not indicative of recurrence as there is no clinically disease free period on follow-up.

Use the Multiple Primary Rules as written to determine whether a subsequent tumor is a new primary or a recurrence as stated in the General Instructions. The only exception is when a pathologist compares slides from the subsequent tumor to the “original” tumor and documents the subsequent tumor is a recurrence of the previous primary. Never code multiple primaries based only on a physician’s statement of “recurrence” or “recurrent.”

 12/2017 (bladder) and 4/2018 diagnoses (renal pelvis):  Single primary (Rule M11:  Abstract a single primary when there are urothelial carcinomas in multiple urinary organs; behavior is irrelevant.)

Assign code 8020/3 when the histologic type specifically includes the term of poorly differentiated, dedifferentiated, undifferentiated, or anaplastic undifferentiated carcinoma along with carcinoma as terms vary depending on the primary site. When the term poorly differentiated is included in the grade section only of the pathology report or only mentions poorly differentiated carcinoma without further substantiation from a pathology report as in examples 1 and 2, do not use code 8020/3.

The histology code 8020/3 and terms may be used for selected primary sites as included in the Solid Tumor Rules, WHO Classification of Tumors series (latest versions), and the Site/Morphology Validation List including

Nasal cavity

Nasopharynx

Salivary glands

Urinary sites

Colon, rectosigmoid, rectum

Esophagus

Stomach

Gallbladder and extrahepatic bile duct

Pancreas

Thyroid

Ovary

Uterine corpus

Vagina

Uterine cervix (also referred to as unclassifiable in WHO Classification of Female Genital Tumors, 5th ed.)

For sites other than those listed, if the diagnosis is poorly differentiated carcinoma, code 8010/3 and poorly differentiated in the grade field. 

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.

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 scrotum (reference: Cancer PathCHART: Product Downloads and Timelines). The Cancer PathCHART SMVL will be updated for C632, Scrotum, with the next release of the NAACCR Edits Metafile, currently scheduled for May 2024.

Assign histology codes 8085 and 8086 for the sites listed in the Solid Tumor Rules histology tables. The codes 8085 and 8086 are applicable for a small group of sites according to the year they became valid for implementation as follows.

Head and Neck

      Oropharynx, Base of Tongue, Tonsils, Adenoids (2022+)

Other Sites

      Cervix (2021+)

      Anus (2023+)

      Vagina (2023+)

      Vulva (2023+)

      Penis (2024+)

      Scrotum (2024+)

While ICD-O-3.2 and Cancer PathCHART list additional sites such as Accessory Sinuses, they have not yet been implemented in the U.S.

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.

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.

Report these high grade dysplasia of the following organs as stated below.

Stomach: Assign code 8148/2 glandular intraepithelial neoplasia, high grade using the Other Sites Solid Tumor Rules, Table 6: Stomach Histologies and as described in the WHO Classification of Digestive Tumors, 5th edition.

Small intestine and Esophagus: Assign code 8148/2 glandular intraepithelial neoplasia, high grade, using the Other Sites Solid Tumor Rules, Other Sites Histology Rules, Rule H4/H26. The following note is listed for both of these rules.

Note: This list may not include all reportable neoplasms for 8148/2. See SEER Program Coding and Staging Manual or STORE manual for reportable neoplasms

The Other Sites Solid Tumor Rules, Table 5: Esophagus Histologies and Table 7: Small Intestine and Ampulla of Vater Histologies will be updated to reflect this code as time permits.