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We found that the term AML with recurrent genetic abnormalities, NOS"was incorrectly included as an alternate name with code 9896/3. We followed back with our expert hematopathologist and he stated that this should have been coded to 9861/3 (AML, NOS), for AML with recurrent genetic abnormalities, NOS. This alternate name has been added to 9861/3. (Note: The same alternate name has been removed from 9896/3).

IDH-2 is not listed as a genetic abnormality for any of the histologies listed in the database. It could be that this is a new genetic marker for one of the AML with recurrent genetic abnormalities that we are not aware of. Without further clarification on which histology the IDH-2 would indicate, you would have to default to 9861/3.

There are several histologies that are grouped as AML with recurrent genetic abnormalities."All of these have specific genetics listed as part of the ICD-O-3 histology name.

9865: Acute myeloid leukemia with t(6;9)(p23;q34.1) DEK-NUP214

9866: Acute promyelocytic leukemia with PML-RARA

9869: Acute myeloid leukemia with inv(3)(q21.3q26.2) or t(3;3)(q21.3;q26.2); GATA2, MECOM

9871: Acute myeloid leukemia with inv(16)(p13.1q22) or t(16;16)(p13.1;q22); CBFB-MYH11

9877: Acute myeloid leukemia with mutated NPM1 (2021+)

9878: Acute myeloid leukemia with biallelic mutation of CEBPA (2021+)

9879: Acute myeloid leukemia with mutated RUNX1 (2021+)

9896: Acute myeloid leukemia with t(8;21)(q22;q22.1); RUNX1-RUNX1T1

9897: Acute myeloid leukemia with t(9;11)(p21.3;q23.3); KMT2A-MLLT3

9911: Acute myeloid leukemia (megakaryoblastic) with t(1;22)(p13.3;q13.1); RBM15-MKL1

9912: Acute myeloid leukemia with BCR-ABL1 (2021)+

Of note, for the above histologies, since these are diagnosed solely based on genetics, diagnostic confirmation will always be 3. This instruction will be added to the Hematopoietic database for the 2022 update.

We do not recommend using the size from a core biopsy for clinical tumor size. A core biopsy does not necessarily obtain enough tissue to know the actual tumor size. Since there is imaging for this patient, it is preferable to record clinical tumor size from the imaging report in this case.

The instructions will be clarified in the next revision of the SEER manual.

We consulted with our expert hematopathologist who advised this is a single primary, Hodgkin lymphoma (9659/3).

The diagnosis from 5/14/2021 states NLPHL. It also states there is T-cell histiocyte rich large B-cell lymphoma-like transformation. The WHO Classification of Hematopoietic and Lymphoid Tissues demonstrates six different patterns to NLPHL, which are: A) 'classical' nodular, B) serpiginous/interconnected nodular, C) nodular with prominent extra-nodular LP cells, D) T-cell-rich nodular, E) diffuse with a T-cell-rich background, and F) diffuse, B-cell-rich pattern.

In this case, they are describing a NLPHL type E (diffuse with a T-cell rich background). The term used is "T-cell histiocyte rich large B-cell lymphoma-LIKE transformation. "Like" as used here means that it is like a transformation; if it was NLPHL transforming to T-cell histiocyte rich large B-cell lymphoma, it would not have the word "like" in the diagnosis. This is a variant of NLPHL and not an actual transformation to another lymphoma. Even though NLPHL can transform to T-cell histiocyte rich large B-cell lymphoma, it is not the case here since the word "like" appears in the diagnosis.

We will update the histology in the Hematopoietic and Lymphoid Neoplasm Database to include these additional patterns.

We consulted with an expert GYN pathologist. He confirmed our thinking that endometrial stripe or thickening does not represent clinical tumor size. We will add this to a future edition of the SEER manual for reference.

We consulted an expert genitourinary pathologist who advises that micropapillary serous borderline tumor of the testis is reportable. He states "it is the same neoplasm as in the ovary. It arises from tissue (tunica vaginalis) surrounding the testis so is a paratesticular neoplasm." 

 Please note: not all borderline tumors are reportable and this diagnosis is an exception because it is assigned /2 in ICD-O-3.2. It is reportable for cases diagnosed Jan 1, 2021 and later.

We confirmed with our expert pathologist consultant that lymphovascular invasion and lymphovascular space invasion are synonymous.

We are aware of the issue and have been in discussion with standard setters (SEER, NPCR, AJCC, and NAACCR). At this time, we recommend coding:

Histology: 8441/2

Extent of Disease (EOD) Primary Tumor: 000

Summary Stage: 0

AJCC Clin/Path T would be 88, since all in situ lesions are not applicable.

Edits will not allow you to have a 8441/2 with a T1. Also, EOD is not currently set up to derive the correct T value, unless you code 100.

The change to address the issue will take effect in 2021.

Vulvar intraepithelial neoplasia with no grade specified is not reportable. Reportability instructions have not changed. See page 11 in the SEER manual, http://seer.cancer.gov/manuals/2015/SPCSM_2015_maindoc.pdf

Venous angiomas (9122/0) are not reportable wherever they arise. The primary site for venous angioma arising in the cerebellum is C490.  The combination of 9122/0 and C490 is not reportable. Venous angioma is a venous abnormality, currently referred to as a developmental venous anomaly (DVA).

Vascular tumors of the CNS are reportable when they arise in the dura or parenchyma of the CNS and should be coded accordingly. The instructions in the CDC book regarding primary site coding are not the most current instructions.SEER assumed responsibility for brain and CNS reporting instructions in 2007.

The tumor in SINQ 20120034 is not reportable because it arises in a blood vessel. The cavernous hemangioma in SINQ 20081113 is reportable because the primary site is the white matter of the cerebral cortex.