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Report optic nerve sheath meningioma arising in the intraorbital segment. The optic nerve contains four segments, of which intraorbital is one. The WHO Classification of Eye Tumors, 4th edition, defines meningioma as a neoplasm originating from the meningothelial cells of the optic nerve leptomeninges. According to the Table 3 of the Non-malignant Solid Tumor Rules, all portions of the optic are reportable and meningiomas arising in the dura/meninges of an intracranial nerve are coded to cerebral meninges C700.

The CPC Validity Status of the site morphology combinations of C569/8441/3 and C569/8441/2 was revised from Valid to Unlikely with the latest release of the Version v24A Edits Metafile. As a result, this site and morphology combination will now require an over-ride flag to be set.

Code as 8461/3 (high-grade serous carcinoma) or 8460/3 (low-grade serous carcinoma) if at all possible. Use 8441/3 (serous carcinoma, NOS) only if it cannot be distinguished as low grade or high grade. The codes for high-grade serous carcinoma and low-grade serous carcinoma are relatively new. High-grade serous carcinoma and low-grade serous carcinoma are very different tumors and pathologists should state whether it is high grade or low grade. Please make every attempt to use the newer codes. If unable to determine high gade versus low grade, assign 8441/3 and override the edit.

The files on the CPC website are currently being updated, and CPC*Search will be updated to reflect the changes sometime this Fall.

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).

Lymphoepithelial carcinoma is a subtype of SCC usually seen in skin or H&N sites and often associated with EBV. CPC SME review determined 8082/3 invalid for breast but did not recommend a substitute code. There were only 45 cases coded 8082 2001 to 2019. For this case, it's possible the lesion originated in the breast skin and progressed to breast tissue. SCC is a subtype of metaplastic breast carcinoma so one could argue it code be coded either 8575 or 8070. For this case, we recommend assigning 8500/3. Use text fields to record the details.

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

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.

Refer to the College of American Pathologists (CAP) protocols to determine how to interpret p16 staining results on pathology reports. Per the Vulva CAP Protocol, p16 positive is defined as diffuse or block-like expression. Since the pathology report states "block-like expression," code the histology as 8085/3 (invasive squamous cell carcinoma, HPV-associated).

Update February 2026, note added:

Report this case as smoldering myeloma (9732/3) based on the plasma cell 10% threshold and favorable cytogenetics and lack of organ dysfunction (9732/3). According to the College of American Pathologists Plasma Cell Malignancies Protocol, in order to code smoldering multiple myeloma, both criteria must be met:

• Serum monoclonal protein (IgG or IgA) ≥3gm/dL, or urinary monoclonal protein ≥ 500 mg per 24h and/or clonal bone marrow plasma cells 10-60%

• Absence of myeloma defining events or amyloidosis.

Note: This case was answered by our expert pathologist and applies to this case only. Registrars should not use the plasma cell threshold to determine reportability or histology. The diagnosis must come from the pathologist or the managing physician.

Assign Code 3 (Positive histology PLUS positive immunophenotyping or genetic testing) for

1. Cases with positive histology for the neoplasm being abstracted (including acceptable ambiguous terminology and provisional diagnosis), AND 

• Immunophenotyping, genetic testing, or JAK2 is listed in the Definitive Diagnosis in the Heme Database, AND
• Testing
  • Confirms the neoplasm OR
  • Identifies a more specific histology (not preceded by ambiguous terminology)
  • Peripheral blood smear followed by flow cytometry (most commonly done with CLL/SLL, 9823/3)

2. A not otherwise specified (NOS) histology diagnosed and not a provisional diagnosis, AND genetic/immunophenotyping was performed and positive

Refer to the current version of the Heme Manual for specific notes and examples when coding Diagnostic Confirmation.

Assign 8020/3 for SMARCA4- deficient malignant neoplasms of the esophagus.

The WHO Classification of Digestive System Tumors, 5th edition, lists undifferentiated carcinoma as 8020/3. Undifferentiated carcinoma of the esophagus is characterized by the frequent loss of SMARCA4 or SMARCA2 by immunohistochemistry.

SINQ 20200057 was updated in August 2025 and assigns code 8044/3 for Thoracic SMARCA4-deficient undifferentiated tumor (SMARCA4-UT). The 2025 Solid Tumor Manual includes SMARCA4-deficient or SMARCB1-deficient tumors for thoracic and sinonasal sites (8044/3).

Assigning histology to other individual sites should be on a case-by-case basis.