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Patient was diagnosed with carcinosarcoma of Mullerian origin on omental/pelvic biopsies in March 2021. First course treatment was neoadjuvant chemotherapy followed by July 2021 resection showing residual primary endometrial carcinosarcoma with cervical stromal invasion and involvement of bilateral tubes/ovaries, omentum, and mesenteric nodule. Additional findings included endometrium with background endometroid intraepithelial neoplasia (EIN).

1. 12/11/2020 Peripheral blood revealing what was thought to be chronic myelogenous leukemia BCR/ABL1 positive (9875/3). Patient was started on Hydrea while waiting for further tests on 12/12/2020.

2. 12/14/2020 Lymph node biopsy showed granulocytic sarcoma (9930/3), but flow cytometry states it is similar to that seen in the patient's peripheral blood and is consistent with nodal involvement by myeloblasts.

3. 12/15/2020 Bone marrow biopsy reads acute myeloid leukemia (9861/3), likely arising from BCR/ABL1 positive chronic myeloid leukemia. There is a note on this pathology from medical oncologist that says: This will dramatically change the course of his treatment, likely with a TKI.

4. 12/17/2020 Sprycel started. Patient was weaned off Hydrea.

According to Rule M3, abstract a single primary when a sarcoma is diagnosed simultaneously or after a leukemia of the same lineage. It lists 9930/3 when simultaneously (or after) with 9861/3. Technically, it was two days before, but I feel like I should and could count that as simultaneously because of Note 1 that says: These sarcomas are solid manifestations of the associated leukemia. For example, when acute myeloid leukemia and myeloid sarcoma are diagnosed simultaneously, the myeloid sarcoma is the result of myeloid cells migrating from the bone marrow or blood into tissue. It is part of the disease process for the acute leukemia. Also, the providers never mention granulocytic sarcoma

Based on that, I think that #2 & #3 above are the same primary, which would be acute myeloid leukemia (9861/3).

Per the hematopoietic database, 9875/3 transforms to 9861/3. Therefore, Rule M8 is confusing with the "only one" biopsy. Does this rule apply because the 9875/3 was from peripheral blood only? But peripheral blood is coded in Diagnostic Confirmation as histology.

Rule M9 reads: The two diagnoses are likely the result of an ongoing diagnostic work-up. The later diagnosis is usually based on all of the test results and correlated with any clinical information. Because that is truly what I think is happening here though that rule states there is no available documentation. If you do not have any documentation, how would you know you are dealing with a chronic and an acute diagnosis?

M10 does not apply.

According to Rule M11, abstract as multiple primaries when both a chronic and an acute neoplasm are diagnosed simultaneously or within 21 days and there is documentation of two biopsies. The chronic myelogenous leukemia only had peripheral blood and not a bone marrow, lymph node or tissue, but that is counted as positive histology in diagnostic confirmation, but I don't know if that is kept as a separate field/thought. I would not code a peripheral blood smear as with a surgical code or a surgical diagnostic and staging procedure code, so maybe that is what I should be thinking about and therefore would probably say Rule M8 and one primary.

Details

4/15/2018:  Right abdominal wall mass excision: infiltrating sebaceous carcinoma.  Noted to have a history of Muir-Torre/Lynch syndrome.

1/21/2019:  Two left upper back mass excisions and two lower back (laterality not specified) mass excisions: infiltrating sebaceous carcinomas

8/7/2019:  Excision of multiple sebaceous carcinomas from the right posterior back, left posterior thigh, left anterior abdominal wall, left anterior thigh, right scrotum, right lower abdominal fold, all positive for sebaceous carcinoma on pathology report

9/30/2020:  Right gluteal mass, left gluteal mass, back (NOS) excisions: sebaceous carcinomas.  

10/14/2020:  Right back excision: sebaceous carcinoma. Op note:  History of Lynch syndrome with multiple sebaceous carcinomas, recurrent back mass, site of prior mass resection.

10/18/2021:  Right thigh excision: sebaceous carcinoma

Proposed primaries using MP/H Other Sites Rules

#1:       4/15/2018:  C445-1

#2:       1/21/2019:  C445-2, separate from #1 per M8, same as 1/21/19 C445-9 per M18

#3:       8/7/2019:  C445-1, separate from #1 per M10, separate from #2 per M8

#4:       8/7/2019:  C447-2, separate from #1 & #3 per M8, separate from #2 per M12

#5:       8/7/2019:  C632, separate from #1 per M10, separate from #2-#4 per M11

#6:       9/30/2020:  C445-2, separate from #1 & #3 per M8, separate from #2, #4 & #5 per M10

#7:       9/30/2020:  C445-1, separate from #2, #4 & #6 per M8, separate from #1, #3 & #5 per M10; I do not think the back, NOS (C445-9) is a new primary per M18.

#8:       10/18/2021:  C447-1, separate from #2, #4 & #6 per M8, separate from #1, #3, #5 & #7 per M10

We are confused by SINQs 20180097, 20150001, and 20140051.  The latter two indicate that “well-differentiated neuroendocrine neoplasms” of the duodenum and appendix are reportable because they’re synonymous with neuroendocrine tumor (NET).  Yet 20180097 states “primary hepatic neuroendocrine neoplasm” is NOT reportable unless there is documentation that it’s being used as a synonym for Primary Hepatic Neuroendocrine Tumor (PHNET).  In addition, we see in the 2021 ICDO-3.2 update that only “poorly differentiated neuroendocrine neoplasm” is listed with behavior code /3 and noted to be reportable for 2021+ on the companion annotated histology list.  Does reportability of neuroendocrine neoplasms depend on primary site, differentiation terminology within the histology name, or something else?  Our casefinding staff is hoping for a general reportability guideline to follow when they come across “neuroendocrine neoplasms” NOS.  For example, we have a 2020 pathology report for a core biopsy of a soft tissue pelvic mass with final diagnosis of low grade neuroendocrine neoplasm; there is no further clarification as to whether it is felt to be primary or metastatic, and we have no other associated records for this patient in our central registry.

There is a discrepancy in the SEER and STORE manual definition of code 2 for Reason for No Surgery of Primary Site. STORE includes progression of tumor prior to planned surgery as part of the definition for code 2, but the SEER Manual does not. The progression statement is included in the SEER Manual (2018 and 2021) for Reason for No Radiation, but not for Reason for No Surgery.

12/3/2020 Pathology: AML: Blasts 40% of nucleated cells. CD45 positive, CD34 negative, CD 117+,

CD13 positive, CD33 positive in 59.6% and HLA-DR was dim and myeloperoxidase was dim.

Cytogenetics normal karyotype. The next generation sequencing detected IDH 2p.(R172K)c515>A.

Because this was AML NOS, we consulted with the physician. The physician stated the patient had AML with recurrent genetic abnormalities"and the basis for the diagnosis was the IDH-2 mutation identified on Next Generation Sequencing. We assigned 9896/3, based on the physician's interpretation of the pathology. This histology is being questioned.

ICD-O-3.2 lists microscopic thymoma as benign (8580/0) and thymoma, type A as malignant (8581/3).

January 2021: Left central neck node dissection for thyroid carcinoma with thymic tissue showing an incidental type A microscopic thymoma, described as a small (<0.2 cm) focus. Diagnosis comments further indicate this is morphologically consistent with a microscopic thymoma (type A).

Currently, First Course Therapy instruction for expectant management (also referred to as active surveillance, watchful waiting, etc.) instructs one to code 0 or 00 (not done) for all data items when the physician opts for expectant management.

We find that the treatment decisions can be driven by the patient, physician, or combination of both patient and physician depending on the options presented.