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Code AML with monocytic differentiation as acute myeloid leukemia, NOS (9861/3) per consultation with our expert hematopathologist.

Acute monoblastic and monocytic leukemia (9891/3) and acute myelomonocytic leukemia (9867/3) are distinct entities according to the WHO. "AML with monocytic differentiation" is a descriptive diagnosis, whereas, "Acute monoblastic and monocytic leukemia" are specific diagnoses. In the WHO Classification of Tumours, Central nervous system tumours (4th Ed) in 2016, WHO began integrating information on molecular alterations that provide significant prognostic implications and/or a therapeutic target into the histology code/term itself. As a result it is also important to look at the molecular testing because acute myeloid leukemias can have different molecular mutations that could result in coding to a different histology code. In this case, there was no other information regarding additional immunophenotyping, so that is why AML, NOS was assigned.  Acute myeloid leukemia with monocytic differentiation has been added to the Hematopoietic and Lymphoid Neoplasm Database as an alternate name for 9861/3.

Do not report serous borderline tumor–micropapillary variant of the ovary (8460/2, C569) as borderline ovarian tumors are not reportable. This applies to cases 2018 and later.

Do report serous borderline tumor–micropapillary variant of the testis as stated in SINQ 20220032. It is reportable for cases diagnosed Jan 1, 2021 and later.

Solid pseudopapillary neoplasm of the pancreas is reportable for cases diagnosed in 2014 and later. Report solid pseudopapillary neoplasm of the pancreas (8452/3) as the guidance in SINQ 20140058 is still in effect.

The 4th and 5th editions of the WHO Classification of Tumors of the digestive system define solid pseudopapillary neoplasm of the pancreas as a low-grade malignant pancreatic tumor.

Assign 8077/2 for high-grade squamous intraepithelial lesion, VIN 3 in this case.  The WHO Classification of Female Genital Tumors, 5th edition, states that squamous intraepithelial lesions (SILs) of the vulva are also known as vulvar intraepithelial neoplasia, HPV-associated.  The term squamous cell carcinoma in situ is not recommended.

Assign primary site C421.

The Site-Specific Data item (SSDI) Manual, Rai Classification section, states: Per confirmation from medical oncologists, Rai stage is only recorded for patients who have bone marrow and/or peripheral blood involvement. Per the Hematopoietic Rules, primary site would be C421 (See Hematopoietic Manual, Module 3: Rules PH 5, 6). A new code has been added to the 5 SSDIs (code 5) to use when primary site is not C421.

Report as squamous cell carcinoma (8070/3) on the basis of “microscopic focus suspicious for superficial invasion.” "Severe dysplasia" is equivalent to "high grade dysplasia" in the Head and neck. As such, "severe squamous dysplasia" would be coded to 8077/2. However, in combination with the statement of "with microscopic focus suspicious for superficial invasion,” report as squamous cell carcinoma (8070/3) based on “microscopic focus suspicious for superficial invasion.” The 2023 SEER Manual instructs us to code the behavior as malignant (/3) if any portion of the primary tumor is invasive no matter how limited, i.e., microinvasion. Use text fields to record the details. 

Table 19 is based on WHO 5th Ed Tumors of vulva and squamous cell variants, keratinizing and non-keratinizing, are no longer recommended and are excluded from the 5th Ed. HPV related terminology is now preferred for these neoplasms.

Per consultation with our GYN expert pathologist, based on the information provided, this is likely a single tumor that was not completely excised in the original biopsy. A new tumor in the same site would not appear within 8 months. If you cannot confirm two separate/non-contiguous tumors were present, abstract a single primary per M1. As for histology, the tumor showed both keratinizing and non-keratinizing features and HPV status is unclear. Per our expert, code to SCC 8070/3—keratinization or lack of does not change treatment or prognosis.

Even If there is proof of separate/non-contiguous tumors, our expert still feels this is a single primary coded to SCC 8070/3. Treatment does not differ by keratinization or HPV status. Coding two primaries would be incorrect and inflate incidence rates.

Per our expert, this is an unusual occurrence. The rules cover 85% of cases but there will always be situations that do not fit a rule. This case is an example of that.  A new GYN specific Solid Tumor Rules module is under development and a rule to address this situation could be included.

The patient's previous acinar adenocarcinoma in the right lower lobe of the lung in 2020 and squamous cell carcinoma in the right middle lobe of the lung in 2021 were correctly abstracted as two primaries per rule M8 as they are in different rows in Table 3. The NSCLC, RUL (8046) diagnosed in 2022 would not be abstracted as a third primary because NSCLC is a broad category which includes all histologies in Table 3 (except for small cell carcinoma/neuroendocrine tumors (NET Tumors) 8041 and all subtypes), and because it was diagnosed less than 3 years after the 2021 squamous cell carcinoma, RML (8070).

Assign EOD Regional Nodes code 300 for Psammoma bodies within a cervical lymph node that are microscopically confirmed. A clarifying note for the Thyroid Schema will be included in the 2025 EOD updates.

Do not report superficial angiomyxoma (8841/0) or aggressive angiomyxoma (8841/0).  WHO Classification of Female Genital Tumors, 5th edition, defines deep (aggressive) angiomyoma as a benign, infiltrative, myxoid spindle cell neoplasm that occurs in deep soft tissue of the pelviperineal region.