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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.

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.

For cases diagnosed 1998-2003:

Code EOD-Lymph Nodes fields as follows for the examples given:

1) 9 [Unknown; not stated] for a "hilar mass"

2) 2 [Mediastinal] for a "mediastinal mass"

3) 2 [Mediastinal] for "enlarged" or "greater than 1 cm," if used to describe any of the named lymph nodes listed under code 2 in the EOD-Lymph Nodes field.

For cases diagnosed prior to 2018

Assign grade code 2 for "differentiating" retroperitoneal neuroblastoma. The rationale of our expert pathologist advisor is that "it leaves the grade 1 category open (since a "well differentiated neuroblastoma" is actually called ganglioneuroblastoma), and it also avoids putting "differentiating" into what is usually a well differentiated category."

Additionally, assign grade code 3 to a poorly differentiated retroperitoneal neuroblastoma and grade code 4 to an undifferentiated retroperitoneal neuroblastoma.

For cases diagnosed 2018 and later

Follow the instructions for coding grade in SEER*RSA

Assign histology code 8140/3, adenocarcinoma, based on the final diagnosis. The neuroendocrine component in this case is not another histology, nor is it a more specific adenocarcinoma. "Component" is not one of the words that we use to indicate a more specific histology.

Code histology to 8010/3.

NUT carcinoma is identified by the NUTM1 gene rearrangement.  

NUT midline carcinomas (NMC) are lethal and morphologically indistinguishable from other poorly diff carcinomas. They are epithelial tumors which can range from undifferentiated carcinomas to carcinomas with prominent squamous differentiation.

A new proposed ICD-O-3 code has been suggested for NUT tumors but it is not yet approved for implementation. Do not use the new code until it is approved for use in the United States.

For tumors diagnosed prior to 2007:

Assign code 8560 [adenosquamous carcinoma]. According to our pathologist consultant, the mix of adenocarcinoma and squamous carcinoma is adenosquamous carcinoma. Adenosquamous tumors are rare, but known, representing 3-4% of pancreatic carcinomas.

For tumors diagnosed 2007 or later, refer to the MP/H rules. If there are still questions about how this type of tumor should be coded, submit a new question to SINQ and include the difficulties you are encountering in applying the MP/H rules.