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Code high grade urothelial carcinoma described as having "extensive sarcomatoid dedifferentiation" to sarcomatoid transitional cell carcinoma (8122/3).

High grade biliary intraepithelial neoplasia of the gallbladder is reportable. Assign code 8148/2. It is also known as biliary intraepithelial neoplasia grade 3, or BilIN-3.

You may code grade as follows.

Grade 1 – NET G1 (M8240/3)

Grade 2 – NET G2 (M8249/3)

Grade 1 – neuroendocrine carcinoma, low grade (M8240/3) or neuroendocrine carcinoma, well differentiation (M8240/3)

Grade 2 – neuroendocrine carcinoma, moderate differentiation (M8249/3)

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.

Default the coding of the Primary Site field for chordomas to the bone where the tumor began in the body if the primary site is not clearly stated to be soft tissue. Bone is often the primary site for chordomas.

Based on advice from pathologist consultants: This is one of those situations where we can be quite comfortable with a default, in this case to bone, not soft tissue. Chordoma is a tumor arising in the nucleus pulposis, presumably from remnants of notochord - thus its exclusive origin is in the sacrococcygeal region, spheno-occipital region, and vertebral bodies, otherwise known collectively as the axial skeleton. Any "chordoma" in soft tissue (with no relationship to axial skeleton) is probably a myxoid chondrosarcoma or parachordoma (extremely rare).

Rathke cleft cyst is not reportable. Cysts are not neoplastic. However, Rathke pouch tumor (C751, 9350/1) is a reportable neoplasm for cases diagnosed 2004 and later. The Rathke pouch is coded to the pituitary gland. Benign and borderline pituitary tumors have been reportable since 2004.

Mucinous borderline tumor, intestinal type, of the ovary is not reportable. The behavior is /1. There is no applicable histology code for this histology when it ocurs in the ovary. 

For cases diagnosed 1998-2003:

Code the EOD-Extension field to 65 [Pre-epiglottic tissues]. Extension to "pre-epiglottic space" is equivalent to extension to "pre-epiglottic tissue."

Code the Grade, Differentiation field to 9 [cell type not determined, not stated or not applicable]. For lymphomas, do not code the descriptions "high grade," "low grade," and "intermediate grade" in the Grade, Differentiation field. These terms refer to categories in the Working Formulation and not to histologic grade for lymphoma histologies.

Generally, for histologies other than Non-Hodgkin lymphoma, the Grade, Differentiation field is coded to 2 [low grade], 3 [intermediate grade] and 4 [high grade] for most cancers.

Conjuctival intraepithelial neoplasia grade III (CIN III) is reportable. Intraepithelial neoplasia, grade III, is listed in ICD-O-3 as /2. It is reportable for sites other than skin.