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Assign as Stage IV as recommended by our expert hematological oncologist. This is a rare occurrence and this type of presentation does not fit the definition of intraocular extension. Stage IV is probably the best stage for this type of presentation, since there are two extranodal organs involved, even though they involve a bilateral site.

EOD Primary Tumor: 700

SS: 7 (Distant)

Assign Laterality code 4 (Bilateral involvement at time of diagnosis, lateral origin unknown for a single primary) in the situations you describe. Skin of upper limb and shoulder and Skin of other and unspecific parts of the face are listed as paired organs in the table Sites for Which Laterality Must Be Recorded In the 2023 SEER Manual.

Yes. DFSP with fibrosarcomatous overgrowth and DFSP with fibrosarcomatous component Grade 2 are synonymous with fibrosarcomatous DFSP (8832/3).  Our expert pathologist also advises that DFSP with focal myxoid features is the same as DFSP, myxoid (8832/3).

Code Primary Site as Overlapping lesion of cervix uteri (C538). The 2023 SEER Program Coding and Staging Manual Primary Site Coding Instructions for Solid Tumors #4 says to code the last digit of the primary site code to ‘8’ when a single tumor overlaps an adjacent subsite(s) of an organ and the point of origin cannot be determined. This is also supported by the ICD-O-3, 3rd edition, note in the Topography section that states: In categories C00 to C809, neoplasms should be assigned to the subcategory that includes the point of origin of the tumor. A tumor that overlaps the boundaries of two or more subcategories and whose point of origin cannot be determined should be classified to subcategory ‘8.”

This is reportable as 9591/3, B-cell lymphoma, NOS.All diagnoses in the differential are all B-cell lymphomas. The pathologist knows it a B-cell lymphoma but has not determined the subtype. If at a later time a specific lymphoma is determined, update the histology code accordingly. 

Do not report superficial angiomyxoma (8841/0) or aggressive angiomyxoma (8841/0).  WHO Classification of Female Genital Tumors, 5th edition, defines deep (aggressive) angiomyoma as a benign, infiltrative, myxoid spindle cell neoplasm that occurs in deep soft tissue of the pelviperineal region.

Abstract two primaries using rule M4 of the Colon rules or rule M13 of Other Sites: 1. Invasive adenocarcinoma of distal rectum and 2. Paget disease of the anal mucosa / perianal skin (determine site of origin and code primary site accordingly). The rectum and the anus are separate sites and the histologies differ in each site.

The WHO Classification of Digestive System Tumors, 5th edition, states that in addition to secondary anal Paget disease arising from anal canal adenocarcinoma, or rarely, adenoma without documented invasive disease, secondary Paget cells may be contiguous with the underlying neoplasm or manifest at different at sites distinctly away from it (with skip lesions).  Document the details in the appropriate text fields.

Assign 000 for EOD Regional Nodes when you have a pathology only report with a localized tumor based on Breslow’s depth and/or Clark’s Level (per EOD and/or Summary Stage) and no information on regional lymph nodes or mets. When the tumor is noted to be regional or distant based on Breslow’s Depth and/or Clark’s based on the definitions in EOD and/or Summary Stage, do not assume that the nodes are negative and assign 999. Clarification will be added to the EOD manual.

Assign code 1 in Surgical Procedure of Other Site (NAACCR #1294) when a simple bilateral mastectomy is performed for a single tumor involving both breasts. This statement was inadvertently omitted from the STORE manual and will be added back in: For single primaries only, code removal of contralateral breast under the data item Surgical Procedure/Other Site (NAACCR Item #1294) or Surgical Procedure/Other Site at This Facility (NAACCR Item #674).

The information presented by NAACCR was intended to be consistent with what is in the SEER manual. It may have been misuderstood.

Code AML with monocytic differentiation as acute myeloid leukemia, NOS (9861/3) per consultation with our expert hematopathologist.

Acute monoblastic and monocytic leukemia (9891/3) and acute myelomonocytic leukemia (9867/3) are distinct entities according to the WHO. "AML with monocytic differentiation" is a descriptive diagnosis, whereas, "Acute monoblastic and monocytic leukemia" are specific diagnoses. In the WHO Classification of Tumours, Central nervous system tumours (4th Ed) in 2016, WHO began integrating information on molecular alterations that provide significant prognostic implications and/or a therapeutic target into the histology code/term itself. As a result it is also important to look at the molecular testing because acute myeloid leukemias can have different molecular mutations that could result in coding to a different histology code. In this case, there was no other information regarding additional immunophenotyping, so that is why AML, NOS was assigned.  Acute myeloid leukemia with monocytic differentiation has been added to the Hematopoietic and Lymphoid Neoplasm Database as an alternate name for 9861/3.