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We previously downloaded the 8/22/2018 ICD-O-3 histology update tables which included the note, not reportable for 2018, for both of these terms (with an updated histology 8071/2). SINQ 20180020 confirms differentiated penile and vulvar intraepithelial neoplasia are NOT reportable for 2018 (as does 20160069). However, when looking at the 8/22/2018 ICD-O-3 histology update table today, the not reportable for 2018 comment has been removed and it appears these two terms are reportable. Which is correct?

Example: Patient has left thigh skin excision with final diagnosis of atypical smooth muscle cell proliferation, inked peripheral margin is involved and inked deep margin is free of disease in the sections examined. See Comment.

Diagnosis comment states: The terminology regarding this lesion is controversial. Lesions with identical features are designated as leiomyosarcoma in the dermatopathology literature, whereas, the preferred classification in the soft tissue pathology is atypical intradermal smooth muscle neoplasm. Although the lesion appears predominantly dermal based, since the margin is involved, the lesion cannot be entirely evaluated, and therefore the final designation is deferred to the findings in the excisional specimen. (This slide was read by bone and soft tissue pathologist.)

There has been no excision of this tumor and, as a central registry, we have no access to the pathologist for clarification.

Is this skin case reportable based on the dermatopathology interpretation when further documentation is not available?

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?

Patient is diagnosed with invasive ductal carcinoma (IDC), right breast, receives HT, radiation therapy, and surgery. The same patient is diagnosed with LCIS, left breast one month later--recommend surveillance only (no surgery). Is the HT for the left breast coded at all? I think for COC/NCCN, we do not, but for SEER what would I do? Treatment in the SEER Manual 2018 states, "Code the treatment on each abstract when a patient has multiple primaries and the treatment given for one primary also affects/treats another primary." The example include bladder/prostate and ovarian/cervix. It also states, "Code the treatments only for the site that is affected when a patient has multiple primaries and the treatment affects only one of the primaries." The example includes colon/tonsil. Breast LCIS treatment appears complicated. Per NCCN guidelines, this condition no longer has recommendations, however it appears as though they still state that if a core biopsy is done and is LCIS, follow up should be ultrasound or surgical excision. Nowhere does it state hormone is recommended.

Example: Small intestine wedge resection with GIST, 1.8 cm in mid small intestine, single nodule. Stomach nodule biopsy: GIST, 0.3 cm. Pathology report comment section indicates the gastric GIST is not staged due to the small size and incidental nature.

Patient has a history of breast cancer and diffuse large B-cell lymphoma (DLBCL), both treated with chemotherapy and radiation.

On 6/26/19, bone marrow biopsy: MDS with excess blasts-2 (18% dysplastic blasts) in a normocellular marrow (overall 40% cellularity) with trilineage dysplasia. Comment: least myelodysplastic syndrome with excess blasts-2. However, an early/evolving AML cannot be completely excluded. The findings likely represent therapy-related myeloid neoplasm.

MD note on 7/15/19: Diagnosis: MDS, high grade borderline AML with complex karyotype secondary disease. Patient has high grade MDS which is bordering on AML transformation with 20% blasts by IHC and areas higher than this. This is likely secondary to the treatment she has received for her other cancers particularly pelvic radiation for her DLBCL. Given her very high IPSS score, it is likely she will eventually develop AML. No treatment given.

On 7/15/19, bone marrow biopsy: Persistent acute leukemia in a marrow with trilineage dyspoiesis and 23% blasts.

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 are two histologies provided in the final diagnosis, malignant spindle cell neoplasm (8004/3) and atypical fibroxanthoma (8830/3). There is a definitive diagnosis of the non-specific histology, but the more specific histology is only described using ambiguous terminology.

The external ear (C442) is included in the Head and Neck schema for diagnosis year 2018 and later. The Head and Neck Histology Rules indicate ambiguous terminology cannot be used to code a more specific histology. So ignoring the atypical fibroxanthoma, because it is modified by ambiguous terminology, we are left with a non-reportable site and histology combination (C442, 8004/3).

Diagnoses of malignant atypical fibroxanthomas are regularly diagnosed using the syntax above in our area. Follow-up with the physician or pathologist is generally not possible as these cases are received from dermatopathology clinics only. The pathology report is the only information that will be received. If the reportable diagnosis of malignant atypical fibroxanthoma is ignored per the current Solid Tumor Rules, incidence cases will be lost.

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.