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According to the literature, CIC gene rearrangement sarcomas in young patients are soft tissue sarcomas with an aggressive clinical course and may have previously been grouped under the Ewing-like family of tumors or as undifferentiated round cell sarcomas. There is currently no guideline in the solid tumor rules for coding a CIC gene rearrangement sarcoma. However, coding the histology to 8800 (sarcoma, NOS) seems unlikely to capture the more aggressive nature of these tumors. Can a more specific histology be coded?

Left frontal mass biopsy diagnosis comment states: Given the synaptophysin and patchy CD34 staining of these cells, the possibility of ganglioglioma and pleomorphic xanthoastrocytoma is raised. Astroblastoma and ependymoma were considered given the perivascular pseudorosettes, however GFAP staining is quite limited against these tumors. Reticulin stain shows limited perivascular reticulin staining however. Nevertheless, the necrosis, mitotic activity and elevated mitotic activity would point to a malignant neoplasm. Given the neural and limited GFAP staining, a generic classification of neuroglial is provided.

This is the only available information. Further clarification or discussion with the physician or pathologist is not possible. Therefore, is this diagnosis of neuroglial tumor equivalent to that described in SINQ 20091037?

The patient was never treated for the 2016 diagnosis, so the 2019 diagnosis is the same tumor that has progressed. Prior SINQ 20091096 for a similar case type cited multiple primaries per the 2007 Multiple Primaries/Histology Rules, Rule M8, the same rule as the current Solid Tumor rule M17, because this is to be reported as an incidence case.

However, it seems like Solid Tumor Rule M3 would apply because a single tumor is a single primary, and behavior of the 2016 primary would then be updated from /2 to /3. It is unclear how one would advance to the Multiple Tumors module and apply M17 because there is really only a single tumor in this case.

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.

It appears that the first Other Sites applicable rule is H16 (and Table 2) instructing the use of histology code 8323 (mixed cell adenocarcinoma). However, this really is not an adenocarcinoma tumor but is a mixed squamous and transitional cell carcinoma. The 2018 ICD-O-3 Histology Update Table provides a new term for a but does not indicate whether that new term would also include a papillary squamotransitional cell carcinoma of the cervix.

Appendix E1 of the 2018 SEER manual references a similar diagnosis as being reportable for vulva and vagina only. However, the WHO Classification of Tumors of the Urinary System and Male Genital Organs (4th ed) does include high grade penile intraepithelial neoplasia as a synonym for 8077/2.

Example: Mastectomy specimen final diagnosis shows two foci of invasive ductal carcinoma including: Invasive ductal carcinoma, no special type, in association with solid papillary carcinoma (tumor #1, 1 cm, slices 6 and 7) and invasive ductal carcinoma, no special type (tumor #2, 1.2 cm, slices 9 and 10).

Summary Staging outlines, Tumor #1: Histologic Type: Invasive ductal carcinoma, no special type, in association with solid papillary carcinoma. As well as, Tumor #2: Histologic type: Invasive ductal carcinoma, no special type. Additional findings include ductal carcinoma in situ (DCIS): presently approximately 3.3 cm, spanning slices 10-13.

The behavior of the solid papillary carcinoma component will affect the provisional histology of the first tumor (8523/3) per Rule H17 vs. 8500/3 per Rule H7). Based on the response, we can determine whether this represents a single or multiple primaries (single primary per M13 vs. multiple primaries per M14).

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.

Head and Neck Multiple Primary Rule M8 appears to be the first rule that applies to this case and instructs the user to abstract multiple primaries when separate/non-contiguous tumors are on different rows in the appropriate site table (Tables 1-9) in the Equivalent Terms and Definitions. Table 1 (tumors of the nasal cavity) shows Non-keratinizing squamous cell carcinoma and squamous cell carcinoma on different rows making the 2019 case a new primary. Is this correct?