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The pathologist noted this was a challenging brain neoplasm that did not easily fit into a specific WHO diagnostic classification. Multiple differential diagnoses were given including pilomyxoid astrocytoma, ganglioglioma and dysembryoplastic neuroepithelial tumor (DNET), but there were no definitive features characteristic of any of these tumors. In the Comment section following the Final Diagnosis, it further states: "In summary, the tumor appears to be a difficult to classify non-infiltrating glial/glioneuronal neoplasm without definitive high-grade features."

Table 3 (Specific Histologies, NOS, and Subtypes/Variants) lists Ewing sarcoma as a synonym for Peripheral primitive neuroectodermal tumor 9364. Presumably, this is to be used for the reportable malignant peripheral nerve tumors when diagnosed as pPNET or Ewing sarcoma. However, this patient has a type of central (or CNS) primitive neuroectodermal tumor (histology 9473). Table 3 does not list central primitive neuroectodermal tumor (PNET or CPNET) as a valid histology for CNS tumors.

While Table 3 does not list all the possible histologies for the CNS, it currently is not clear how one would arrive at the histology code for a CNS Ewing sarcoma family tumor with CIC alteration, as this is recognized as a new entity for primitive neuroectodermal tumors of the CNS (i.e., PNET, histology 9473) per multiple journal articles. Ewing sarcoma family tumors include both peripheral PNET and central PNET tumors, but to code this histology as a peripheral PNET (9364) in this case seems incorrect when the primary tumor is stated to be of central nervous system origin, not peripheral nervous system origin.

Example: Left breast total mastectomy final diagnosis is incidental microscopic findings suspicious for early Paget disease of the nipple. The diagnosis comment states: The left breast mastectomy shows mildly atypical cells within the nipple epidermis which are suspicious for early Paget disease of the nipple. Additional sampling of the left breast was performed, and no evidence of atypical hyperplasia, in situ carcinoma, or invasive carcinoma within the left breast tissue was identified.

Would this case be non-reportable using rationale similar to an early/evolving melanoma per SINQ 20180029?

The 2018 Breast Solid Tumor Rules Equivalent Terms and Definitions - Changes from 2007 Multiple Primaries/Histology Rules states: Mammary carcinoma is a synonym for carcinoma no special type (NST)/duct carcinoma not otherwise specified (NOS) 8500. It will no longer be coded as carcinoma NOS 8010. Should metastatic carcinomas of breast origin be 8500, or is code 8010 (carcinoma NOS) more applicable because histology coding from metastatic sites is not as reliable?

Lymphoma case diagnosed in 2021: The patient had first round of systemic therapy as documented in the treatment plan and a post-chemotherapy PET scan that showed residual disease. The patient then had a different combination of systemic therapy and still had some residual disease. The patient was given a third round of different combination of systemic therapy in preparation for stem cell transplant. According to the physician post-stem cell transplant note, the patient achieved complete remission.

Is the first course of therapy the first round of systemic therapy only or is it all the therapy given to achieve remission?  It seems like only the first round of systemic therapy is first course of therapy for both leukemia and lymphoma in the hematopoietic manual. I thought all treatment for all hematopoietic cases was first course until remission achieved or progression was evident.

For C220-C221 primaries, Surgery of Primary Site includes code A150 for Alcohol tumor destruction (percutaneous ethanol injection/intratumoral injection of alcohol/alcohol ablation). The SEER and STORE manuals also indicate that alcohol embolization should be coded as Other Therapy, code 1. We are trying to determine whether alcohol embolization should be coded under Surgery of Primary Site or Other Therapy.

There is no statement of mucinous or non-mucinous in this case, only adenocarcinoma in situ and an obsolete term bronchioloalveolar carcinoma (BAC) which used to be code 8250. However 8250 is now lepidic adenocarcinoma, and does not match this diagnosis.

Although the Histology Rules do include a general note indicating that the preferred term for BAC is now mucinous adenocarcinoma 8253, it is not listed as a synonym in Table 3. As a result it is unclear how to apply this statement in accordance with the H rules.

The ICD-O Histology Updates table also includes Bronchiolo-alveolar carcinoma, non-mucinous which seems to suggest that in order to apply histology code 8252 (non-mucinous) or 8253 (mucinous) one must also have a statement of mucinous or non-mucinous.