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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).

12-19-19 Transurethral resection of bladder tumor pathology revealed high-grade papillary urothelial carcinoma with focal glandular and neuroendocrine features; Pathology Overread: High-grade papillary urothelial carcinoma with focal glandular and neuroendocrine differentiation. Carcinoma invades muscularis propria pT2.

Histology 8130

01/20/20 to 07/01/20, completed 6 cycles of gemcitabine/cisplatin.

07/30/20 Robotic radical cystoprostatectomy with bilateral pelvic lymph node dissection, open ileal conduit pathology revealed carcinosarcoma, invading perivesical fat, no lymphovascular invasion, negative margins. ypT3bN0M0 disease; Pathology Overread: Carcinosarcoma arising in association with high-grade papillary urothelial carcinoma.

Histology 8980/3 or is there another histology that should be used?

7/8/16 Urinary bladder, biopsy: Non-invasive low grade papillary urothelial carcinoma. Muscularis propria (detrusor muscle) is not identified.

9/2/16 Urinary bladder, bladder tumor, transurethral resection: High grade papillary urothelial carcinoma. No definite invasion identified. Muscularis propria (detrusor muscle) is identified and not involved by tumor.

1/7/17 A\S\Bladder: Noninvasive low grade papillary urothelial carcinoma. Granulomatous cystitis, consistent with BCG (Bacillus Calmette-Guerin) treatment. Lamina propria is not involved with tumor. Detrusor muscle is not identified.

4/4/17 Dome: Papillary urothelial carcinoma, low grade. No evidence of invasion. Muscularis propria is not present.

Patient is clearly followed for at least a year but no further information until 19 months later, 11/14/18, when biopsy of lung indicates metastatic disease.

11/14/18 Lung, right lower lobe, mass, biopsy: Metastatic urothelial carcinoma. Immunohistochemical analysis results (CK7 positive, CK20 focally positive, P63 positive, GATA3 positive, TTF1 negative and NAPSIN-A negative) support the diagnosis

The Hematopoietic and Lymphoid Neoplasms Database (Heme DB) definition for systemic mastocytosis with an associated hematological neoplasm (SM-AHN, 9741/3) states SM-AHN is a variant of systemic mastocytosis that arises with a myeloid disease of non-mast cell lineage (e.g., MDS, MPN, etc.) and that,

However, SINQ 20130172 conflicts with the Heme DB stating the systemic mastocytosis and the associated hematological neoplasm are a single primary coded to a single histology (9741/3) per Rule M2.

Per Mayo consulting pathologist, the final diagnosis on this right lung biopsy is: SMARCA4-deficient malignant neoplasm (see Comment). Comment: Sections show a poorly-differentiated malignant neoplasm without any apparent glandular, squamous, or stromal differentiation. The tumor near totally replaces the underlying lung tissue without recognizable underlying alveolar parenchyma. Immunohistochemical stains performed at Mayo Clinic (Oscar keratin, INSM1, NUT, S100, desmin and BRG1 protein encoded by SMARCA4 gene) demonstrate that the malignant cells are positive for Oscar keratin (rare cells only), synaptophysin (weak/patchy) and p63 (focal) while negative for the remaining antibodies tested. Of note, SMARCA4 stain is negative in the tumor cells. Thus, this tumor can be categorized as a SMARCA4-deficient malignant neoplasm, which is known to be an aggressive malignancy, likely represent a SMARCA4-deficient thoracic sarcoma, a recently described entity. SMARCA4-deficient carcinomas in the lung have been reported to be mostly adenocarcinomas or squamous cell carcinomas, which would not fit for this case. Please refer to a paper published by our group (Sauter JL et al. Mod Pathol 2017;30:1422-32.

It is unclear if code 300 is appropriate, since technically the fissure is comprised of pleura, involvement of the fissure appears to imply a tumor that is no longer localized.

An argument could be made for code 400, since the term traverses could be interpreted as crossing into adjacent lobe, however the lower lobe is not mentioned in this scan.

Table 4 (Changes in reportable terminology), 2021 ICD-O-3.2 Update, does confirm that the term malignant no longer needs to be used to describe a paraganglioma, but Table 4 includes the histology for extra-adrenal paraganglioma, NOS (8693/3) as the new preferred term for paraganglioma. Paraganglioma, NOS is histology code 8680/3. Which code is correct?

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

Example: An MRI Spine showed a large expansile mass arising from the sella turcica and extending into the suprasellar cistern, but the radiologist only noted: The leading differential considerations include pituitary macroadenoma or a large suprasellar base meningioma. The patient was subsequently pathologically diagnosed with a pituitary adenoma. It is unclear if the diagnosis date should be coded to the MRI date.

There are two existing SINQ questions regarding the term consider. SINQ 20061094 confirms a diagnosis that is considered to be is reportable because it is unambiguous, but SINQ 20081033 states the phrase malignancy is highly considered is not a reportable ambiguous term.

How should we interpret differential considerations? If differential considerations is equivalent to a differential diagnosis, then this patient was clinically diagnosed on imaging. However, if differential considerations is not reportable, then there was no diagnosis prior to the resection.

10/4/2018 Frozen Section Diagnosis: Brain tissue with atypical cells and inflammatory cells, defer to permanents for further evaluation. Note: Tissue for flow cytometry is submitted. Final Diagnosis: Preliminary Diagnosis: Brain Tumor, Biopsy: Neoplasm, suggestive of lymphoma (see comment). Comment: The tumor exhibits nuclear atypia and increased mitosis. The tumor cells are immunologically positive for LCA and with very high ki67 labeling index. GFAP and synaptophysin are not expressed by tumor cells. The above suggests a lympho-proliferative process. This case is forwarded to the hematopathology service of this department for further evaluation. The final diagnosis report will be issued by the hematopathologist as an addendum.

Supp Rpt Add Addendum Diagnosis: The brain biopsy showed brain tissue large lymphoid cell infiltrate. Additional immunohistochemical stains are performed. The large cells are positive for CD20, BCL2, BCL6 (subset), MUM1, and CD30, negative for CD3, CD5, and CD10. Staining for c-MYC is negative. Ki-67 positive large cells are approximately 18%. EBER is strongly positive by ISH. Diagnosis: Brain lesion, biopsy: EBV+ Diffuse Large B-cell Lymphoma. Addendum Comment: The concurrent flow cytometric study showed monoclonal lambda-positive B-cells without out CD5 and CD10 expression, consistent with B-cell lymphoma.

11/18/2019 Left 1st Digit/Thumb Biopsy: Atypical Melanocytic Proliferation consistent with Early Acral Lentiginous Melanoma in situ. Margins Positive. (Not a reportable diagnosis for 2019.)

12/5/2019 Left 1st Digit Shave Biopsies: Malignant Melanoma in situ. Margins Positive.

1/15/2020 Started Aldara (treatment plan: use for ~3 months then Mohs/excision, but due to COVID could not get resection until 7/2020).

7/29/2020 Left Thumb Excision: Residual Melanoma in situ. Margins Positive. Treatment Plan: re-excision.

8/6/2020 Left Thumb Re-Excision: Atypical Lentiginous Melanocytic Proliferation at the 12-2 margin may represent the advancing edge of melanoma in situ. (8/19/2020 Plan to treat the 12-2 margin as positive with in situ; plan for re-excision).

8/20/2020 Left Thumb Re-Excision & Left Nail Plate Excision: Malignant Acral Lentiginous Melanoma with extensive melanoma in situ. Breslow 1.3mm. Margins Positive. Nail plate & bed epithelium with hemorrhage and a mild increase in melanocyte density likely represent melanoma in situ.

9/4/2020 Left thumb partial amputation & Left axillary Sentinel Lymph Node Excision: Residual Malignant Melanoma in situ. 0/3 sentinel nodes positive.