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Table 19 is based on WHO 5th Ed Tumors of vulva and squamous cell variants, keratinizing and non-keratinizing, are no longer recommended and are excluded from the 5th Ed. HPV related terminology is now preferred for these neoplasms.

Per consultation with our GYN expert pathologist, based on the information provided, this is likely a single tumor that was not completely excised in the original biopsy. A new tumor in the same site would not appear within 8 months. If you cannot confirm two separate/non-contiguous tumors were present, abstract a single primary per M1. As for histology, the tumor showed both keratinizing and non-keratinizing features and HPV status is unclear. Per our expert, code to SCC 8070/3—keratinization or lack of does not change treatment or prognosis.

Even If there is proof of separate/non-contiguous tumors, our expert still feels this is a single primary coded to SCC 8070/3. Treatment does not differ by keratinization or HPV status. Coding two primaries would be incorrect and inflate incidence rates.

Per our expert, this is an unusual occurrence. The rules cover 85% of cases but there will always be situations that do not fit a rule. This case is an example of that.  A new GYN specific Solid Tumor Rules module is under development and a rule to address this situation could be included.

Abstract a single primary using Solid Tumor Rules, Malignant CNS and Peripheral Nerves, Rule M10 based on the information provided.  Rule M10 applies as both non-contiguous tumors are of the same histology; i.e., on the same row in Table 3. As MPNST can arise in many sites, look for information about the precise location and tissue type in which the tumor arose. For example, if the tumors are stated to arise in soft tissue, follow the Multiple Primary Rules for Other Sites.

Both WHO Classification of Central Nervous System Tumors and WHO Classification of Soft Tissue and Bone Tumors state that MPNST is a malignant spindle cell tumor often arising from a peripheral nerve, from a pre-existing benign nerve sheath tumor, or in a patient with neurofibromatosis type 1 (NF1).

Future updates will move C470-C479 from CNS to other sites module.

Abstract a single primary and assign code 9700/3 for MF.  According to our subject matter expert, this is all MF.  When MF progresses, there can be large cd30 positive T cells. This is not the same as anaplastic large cell lymphoma.

Based on the diagnosis date for the unknown primary, use the 2007 MPH Other sites rules. Since the site codes differ for each primary, rule M11 applies, abstract two primaries.

For a primary endometrial or ovarian cancer, record the oophorectomy/BSO procedure using the appropriate Surgery of Primary Site code that includes oophorectomy/BSO when done as part of first course of treatment (surgical resection). If performed for hormone effect, also record in the Hematologic Transplant and Endocrine Procedures data item.  For other primary sites whose Surgery of Primary Site codes do not include oophorectomy/BSO, record it in the Hematologic Transplant and Endocrine Procedures data item when performed for hormone effect.  Document information in the appropriate text fields.

Candidates for risk-reducing BSO may include those with hereditary syndromes (such as BRCA mutations) or genes that carry a substantially increased lifetime risk of ovarian malignancy or hormone-sensitive cancers including estrogen-dependent cancers, like breast cancer, ovarian cancer and endometrial (uterine) cancer that rely on estrogen to develop and grow.

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.

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).

Report as squamous cell carcinoma (8070/3) on the basis of “microscopic focus suspicious for superficial invasion.” "Severe dysplasia" is equivalent to "high grade dysplasia" in the Head and neck. As such, "severe squamous dysplasia" would be coded to 8077/2. However, in combination with the statement of "with microscopic focus suspicious for superficial invasion,” report as squamous cell carcinoma (8070/3) based on “microscopic focus suspicious for superficial invasion.” The 2023 SEER Manual instructs us to code the behavior as malignant (/3) if any portion of the primary tumor is invasive no matter how limited, i.e., microinvasion. Use text fields to record the details. 

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.

Do not report the case. The histology is 8825/0 based on the example provided and not reportable. Myofibroblastoma with sarcomatous transformation is a rare, benign condition, sometimes referred to as sarcomatous features. A malignant tumor would be referred to as a myofibroblastic sarcoma.