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In the September 2021 Urinary Sites Solid Tumor Rules update, the term configuration was removed from the “DO NOT CODE histology when described as” list. However, it was not added as a term that can be used to code the more specific histology for urinary tumors.

Can configuration be used to code the more specific histology 8130 (papillary urothelial carcinoma) when the diagnosis is urothelial carcinoma, tumor configuration: papillary?

7-03-97 PE: Past history significant for cavernous hemangiomas. Has had radiation and was on high-dose steroids in early 1996. Patient reports subsequent MRI done and neurologist gave "clean bill of health."

1-26-99 MRI BRAIN. Clinical information: history of intracranial cavernous hemangiomas. Comparison with prior brain MRI in 12/15/95. IMP: Upper medullary, right parieto-occipital, left frontal cavernous hemangiomas without interval change in size as compared to 12/15/95.

1-25-06 MRI BRAIN. Clinical info: history of prior radiation for cavernous angiomas. Comparison made with prior exam on 1/26/99. Impression: Multiple, variable sized cavernous angiomas within medulla, pontomedullary junction, midbrain, & cerebral hemispheres. Dominant lesion centered within posterior pontomedullary junction. FINDINGS: 8mm lesion in posterior pontomedullary junction. 2mm lesion within right paracentral portion of medulla. Several less than 5mm lesions noted within brain stem bilateral. Two, less than 1-2mm, areas within right inferior aspect of right and left cerebellar hemispheres. 1cm lesion centered within white matter within right posterior parietal/occipital region. Several small, less than 1-2mm, lesion within surrounding white matter. 3rd dominant lesion within left frontal lobe equal 6mm. Several 1-2mm foci of susceptibility artifact within subcortical white matter of high right and left cerebral hemispheres consistent with small cavernous angiomas.

Patient is diagnosed July 2021 with high-grade serous carcinoma on ascites cytology. Tumor debulking total abdominal hysterectomy/bilateral salpingo-oophorectomy in August shows high-grade serous carcinoma involving the right ovary (capsule intact, right fallopian tube is negative), left ovary (capsule ruptured), and fallopian tube. Pathologist has chosen tumor site to be bilateral ovaries in the staging summary, with the left fallopian tube listed as “other tissue/organ involvement” along with uterus, peritoneum, and omentum. Additional findings in staging summary includes serous tubal intraepithelial carcinoma (STIC).

Our interpretation of SINQ 20210025 is that any case with both ovarian and tubal involvement would be coded as a fallopian tube primary if STIC is present, even when the pathologist is clearly calling the case ovarian. If this is correct, then the previous SINQ 20120093 may need to be updated with a date restriction reference since it would be in disagreement with this instruction.

If our interpretation is incorrect, then the STIC would be an additional primary per MP/H Rule M11.

SINQ 20091103 states that prostate biopsies showing “highly suspicious for, but not diagnostic of adenocarcinoma, suggest another biopsy” are NOT reportable. However, SINQ 20071056 states that “atypical glands suspicious for adenocarcinoma with insufficient atypia to establish a definitive diagnosis of malignancy” is reportable. This appears to be an issue of semantics with no clearly outlined method to determine reportability of such cases.

We have two recent cases with similar semantic issues and want to know whether they are reportable.

1) Prostate biopsy with “atypical small acinar proliferation, highly suspicious for adenocarcinoma, with quality/quantity insufficient for outright diagnosis of cancer.”

2) Prostate biopsy with “atypical small acinar proliferation highly suspicious for adenocarcinoma but due to the small size of focus, findings are not definitively diagnostic.”

Example 1: 5/2013 diagnosis of HCC in segment 4B (single tumor), treated with microwave ablation in 7/2013. CT scan in 11/2017 with new 23mm hypodensity in liver segment 4 suspicious for recurrent disease. Clinical assessment in 1/2018: New enlarging lesion in liver most consistent with progression of HCC. Treated with RFA in 2/2018. Is the 2018 occurrence a new primary as imaging stated this was a new lesion?

Example 2: 7/2017 diagnosis of HCC in right liver; 2.5 cm lesion in segment 5/6 with a couple of satellites and 12mm lesion in segment 6, treated with Y90 radioembolization. Follow-up note in 11/2017: complete response of treated cluster of lesions in segment 5/6 and lesion in segment 6, increase in size of caudate lesion not amenable for treatment (this lesion was stated to be indeterminate on 7/2017 imaging). Caudate lesion finally stated as LI-RADS5 on 3/2018 imaging and was treated with chemoembolization 6/2018. 7/2018 and 10/2018 Follow-up imaging states LR-TR nonviable lesion in caudate lobe. 8/2019 CT shows caudate lobe with arterial enhancement, new compared to prior imaging, LR-TR viable. MD note states patient has small local HCC recurrence in segment 1 (caudate lobe) with plan to repeat TACE. Is this 8/2019 HCC a new primary as the patient was disease free for greater than 1 year, or is it the same tumor and a single primary?

For example, pathology states: Right axillary lymph node, excision: Diffuse large B-cell lymphoma (DLBCL) (see note). COMMENT: FISH studies were performed that were negative for BCL-6, c-Myc/IgH, CCND1/IgH and IgH/BCl-2 gene rearrangement, ruling out the most common forms of double-hit lymphoma. Flow cytometry studies demonstrated positivity for CD45, CD20, HLA-Dr, CD19, CD11c, CD22, CD30, CD38, CD79b, and FMC7. Low positivity was seen for CD5. No reactivity was seen for CD10, CD23, CD25, CD103 or CD123.

The patient is a 34 year old who presented with testicular pain radiating into the abdomen approximately 1 month before orchiectomy in 2018. CT abdomen/pelvis: Multiple focal sclerotic bone lesions. Given the lack of change from July 2014, these are likely benign bone islands. No adenopathy mentioned. He has no prior history of germ cell tumor nor any surgery for any tumor/cancer before this. Pathology: Testis, left, radical orchiectomy: - Demarcated scar tissue (1.3 cm), with atrophic seminiferous tubules and cortical bone consistent with burnt-out germ cell tumor. No evidence of germ cell neoplasia in situ (GCNIS). - Margins are unremarkable.

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.