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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.

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.

Rule M4 does not seem to apply because Note 1 defines clinically disease free to mean no evidence of recurrence in the same lung on follow-up. Patient had been disease free in the left lung after 09/2014 diagnosis. The 04/2019 diagnosis was in a different lung than the 4/2016 diagnosis.

The next applicable rule is either M11 or M14 depending on how we should compare the new 2019 tumor: to the most recent prior tumor in 2016 or to both prior tumors.

The patient underwent a partial mastectomy and sentinel lymph node biopsy, followed by an axillary lymph node dissection for the first right breast primary in 2011. The separate 2020 right breast primary was treated with a total mastectomy and removal of one involved axillary lymph node. The operative report only refers to this as a non-sentinel lymph node, with no mention of other axillary findings.

Cumulatively, this patient has undergone a modified radical mastectomy since there were likely no remaining axillary lymph nodes. If the Surgery of Primary Site data item is cumulative, does the order of surgeries matter?

It is unclear whether this question should be directed to SINQ (for coding in a SEER registry) or to CAnswer Forum because both have addressed similar surgery related questions in the past and and there is no guidance regarding this specific situation.

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

Example: Posterior fossa tumor resection final diagnosis was medulloblastoma, WHO Grade IV. The diagnosis comment notes the current tumor resection reveals large irregular reticulin-free nodules with streams of neoplastic cells in a fibrillary background in association with narrow reticulin-rich internodular strands of poorly differentiated neoplastic cells. Taken together, these findings are indicative of medulloblastoma with extensive nodularity. The diagnosis comment provided only one histology.

Per the 2018 Solid Tumor Manual, Malignant CNS, Priority Order for Using Documentation to Identify Histology instructions, an addendum or comment has priority over the final diagnosis. Although indicative is not listed on any ambiguous terminology list, is this an ambiguous diagnosis that must be ignored? Or does the diagnosis comment in this case provide a single, specific diagnosis of medulloblastoma with extensive nodularity?

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?

A patient had posterior cervical lymphadenopathy status post biopsy and subsequent lymph node dissection showed metastatic melanoma in 2018. Workup showed no skin lesions or primary site. Final diagnosis is melanoma of unknown primary (unknown if cutaneous or non-cutaneous). Should C760 be used as the primary site for this case since the histology codes of 8700-8790 are included in the Cervical Lymph Nodes and Unknown Primary Tumors of the Head and Neck schema in SEER*RSA?