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Patient has a resection labeled as: Spine, designated intramedullary lesion. The Final Diagnosis is: Melanocytic neoplasm with features most consistent with primary intramedullary melanocytoma. The Diagnosis Comment states: The overall immunophenotypic and morphologic impression is a primary central nervous system melanocytoma.

The ICD-O-3 lists melanocytoma, NOS histology code as 8726/0, but does not provide a site-associated code. If the ICD-O-3 is used, the histology would be 8726/0 and the primary site presumably would be C720 since the tumor was specifically described as being intramedullary (i.e., within the spinal cord medulla).

Table 6 (Solid Tumor Rules, Non-Malignant CNS Equivalent Terms and Definitions) does not list either an intramedullary melanocytoma or melanocytoma (NOS). However, Table 6 does include meningeal melanocytosis 8728/0 and meningeal melanocytoma 8728/1. If Table 6 is used and the histology is coded 8728/1, then the primary site would presumably be C701 per the ICD-O-3 site-associated listing for this histology (C709).

Example: MRI found an intradural, extra-axial mass at T12/L1 with possible intramedullary component. Resection of the intradural intramedullary and extramedullary spinal cord tumor found a capillary hemangioma pathologically. The microscopic description on the path report describes a tumor with extensive vascularity involving the dura.

Should we equate the statement of capillary to mean the tumor is arising in a blood vessel as we do for venous hemangioma (non-reportable per SINQ 20130001)? Or should it be reportable as C700, 9131/0 because it is described as involving the dura (intradural, intramedullary and extramedullary)?

Example: Posterior fossa tumor resection final diagnosis was medulloblastoma, WHO Grade IV. The diagnosis comment notes the current tumor resection reveals large irregular reticulin-free nodules with streams of neoplastic cells in a fibrillary background in association with narrow reticulin-rich internodular strands of poorly differentiated neoplastic cells. Taken together, these findings are indicative of medulloblastoma with extensive nodularity. The diagnosis comment provided only one histology.

Per the 2018 Solid Tumor Manual, Malignant CNS, Priority Order for Using Documentation to Identify Histology instructions, an addendum or comment has priority over the final diagnosis. Although indicative is not listed on any ambiguous terminology list, is this an ambiguous diagnosis that must be ignored? Or does the diagnosis comment in this case provide a single, specific diagnosis of medulloblastoma with extensive nodularity?

For example, a patient has a 2.6 cm breast tumor on MRI; a core biopsy measuring 0.7 cm is positive for infiltrating duct carcinoma. Rule #1 states "Use the largest measurement of the primary tumor from physical exam, imaging, or other diagnostic procedures before any form of treatment." However, Rule #9 seems to imply that size from an "incisional biopsy" takes precedence over imaging, even though it is known to be less than the entire tumor in size.

Patient had a radical pharyngectomy showing a glossotonsillar sulcus tumor with high grade squamous cell carcinoma and adjacent high grade mucoepidermoid carcinoma. The pathologist commented, the tumor is composed of high grade mucoepidermoid carcinoma and high grade conventional-type squamous cell carcinoma that are immediately adjacent to one another. Given that the tumors are arising so close together and could represent a single neoplastic process with divergent morphologies, they are staged together.

Employing Solid Tumor Manual Rule M1 (single primary if unable to determine if there is a single or multiple tumors), it was determined that this should be reported as a single tumor because the pathologist referred to the case as both a tumor singular and tumors pleural. However, the Solid Tumor Manual Histology Rules for a Single Tumor do not appear to have an instruction for coding this histology combination.

There is no specific ICD-O-3 code for a which resulted in abstractors assigning the malignant astrocytoma, NOS code. These lesions were previously called but we are seeing the new terminology more frequently.

Patient had a vulvar biopsy with final diagnosis of Extramammary Paget neoplasm (intraepithelial glandular neoplasm). No invasion identified. We are unable to contact the pathologist or physician for clarification.

Although this terminology is not listed in the ICD-O-3, web search results refer to this as a possible synonym for Paget disease with associated VIN III, which is reportable.

Lip has not been added to any of the site-specific histology tables, nor has any other instruction been provided for coding tumors in this site.

Coding histology for lip primaries is difficult because registrars do not know where to look first. The Solid Tumor Rules indicate one should use the tables first, but then do not inform registrars what table to use for a lip primary (i.e., a specific table, any table, no table).

This question was prompted from preparing SEER*Educate coding exercises. We will use the answer as a reference in the rationales.

Per the site validation list: https://seer.cancer.gov/icd-o-3/sitetype.icdo3.20190618.pdf#search=site%20validation, ill-defined sites (ILL-DEFINED C760-C768) does not include 8070- Squamous cell carcinoma as a valid histology. Therefore when a Cervical Lymph Node and Unknown Primary Tumor of the Head and Neck is submitted with a C760 and 8070/3, it requires an override be set.