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Do not report neuroendocrine tumorlet of lung and non-lung sites. Microcarcinoid and carcinoid tumors are reportable.

Tumorlet is a tumor of neuroendocrine differentiation, defined by size < 5 mm in diameter, mitotic count < 2 mitoses/2 mm², and absence of necrosis. Microcarcinoid is a designation for neuroendocrine tumors when they are less than 0.5 cm. in size. The term "tumorlet" is used in a number of other settings, referring to small tumors (usually < 0.5 cm), and does not necessarily mean carcinoid tumor.

The term microcarcinoid tumor is not equivalent to neuroendocrine tumorlet.

Report high grade serous intraepithelial neoplasm of the endometrium. 

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.

Use all information available in the medical record. EOD is a combination of the most precise clinical and pathological documentation of the extent of disease as instructed in the EOD 2018 General Instructions, Extent of Disease section.

EOD 2018 General Instructions, General Coding Instructions section advises to use the medical record documentation to assign EOD when there is a discrepancy between the T, N, M information and the documentation in the medical record. When there is doubt that the documentation in the medical record is complete, code the EOD corresponding to the physician staging. A discrepancy can exist within the medical record when the information in the chart is unclear, incomplete, or conflicting, for example, the TNM staging from pathology differs from the medical oncologist’s TNM staging.

In the scenario provided, use the medical oncologist stage information that takes into account imaging and exam findings. Based on the stage cT4 N3b, assign

EOD Primary Tumor: 400

Extensive skin involvement

WITHOUT a stated diagnosis of inflammatory carcinoma

WITH or WITHOUT dermal lymphatic filtration

EOD Regional Nodes: 600

Internal mammary node(s), ipsilateral, clinically apparent

(On imaging or clinical exam)

WITH axillary (level I, II, or III) lymph node(s), ipsilateral including infraclavicular

Assign code 550 (Bladder, NOS excluding mucosa) to EOD Primary Site based on invasion into the bladder with no mention of mucosa. EOD Primary Tumor for cervix, Note 1, instructions are to use the extension information to code primary tumor in preference to a statement of FIGO stage when both are available. TNM staging is closely related to FIGO stage, and the surgical findings of bladder invasion NOS in this case should be used in preference to the statement of T4.

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).

Stop at the first rule that applies which is M12. Per note 3: When it is unknown/not documented whether the patient had a recurrence, use date of diagnosis to compute the time interval. This means there are two primaries.

There is a genetic syndrome that causes multiple carcinoid tumors in the GI tract, per our GI expert, and they should be treated as new primaries per M12.

SINQ 20110046 describes a unique situation whereby the subject matter expert felt that the occurrence of multiple tumors was due to an unknown underlying condition driving the proliferation of neuroendocrine cells.

Assign code 8574/3 only when there is

A history of androgen-deprivation therapy or

No history of previous prostate adenocarcinoma

Prostate cancer with neuroendocrine differentiation (PCND) can present as untreated primary pathology (i.e., a new primary) or more commonly as a post ADT and androgen receptor inhibition resistance phenomenon.  PCND is either a newly diagnosed prostate cancer or a result of ADT indicated for treatment of other prostate cancers or other non-cancer diagnoses (e.g., benign prostatic hyperplasia) but not for the PCND diagnosis.

We will edit the notes to make them more clear.

Do not code Reblozyl (luspatercept) as treatment. Luspatercept is an ancillary drug approved to treat anemia associated with MDS but not the malignancy.

Assign histology as 9982/3 (myelodysplastic neoplasm with low blasts and SF3B1 mutation). This is a new term for 9982/3. WHO Classification of Hematolymphoid Tumors, 5th edition, defines myelodysplastic neoplasm (MDS) with low blasts and SF3B1 mutation (MDS-SF3B1) as a myeloid neoplasm with cytopenia and dysplasia characterized by SF3B1 mutation and often ring sideroblasts.