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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.

HL-7 e-path report, Final Diagnosis

High normocellular marrow with maturing trilineage hematopoiesis, multilineage dyspoiesis, compatible with MDS-MLD and involvement by plasma cell neoplasm/myeloma, IgA kappa positive, approximately 20-25% of total cellularity present. See comment.

Comments

Correlation with other relevant laboratory (amount and type of serum and urine paraprotein levels, renal function tests, serum calcium level, and anemia) and radiologic (lytic bone lesions) findings is recommended for complete interpretation.  Dyspoiesis of all lineages is seen and the findings are compatible with low grade myelodysplastic syndrome (MDS-MLD), assuming that other possible causes are excluded. Correlation with cytogenetic and molecular studies is recommended for complete characterization

Patient was initially diagnosed with a right lower lobe (RLL) lung adenocarcinoma in 2014 followed by subsequent right upper lobe (RUL) lung adenocarcinoma in 2016 (single primary). Both were treated with radiation and the nodules were seen as stable on surveillance. There was subsequent growth in the RUL nodule in 2019 and RLL nodule in 2020 as well as a new right middle lobe (RML) nodule in 2020. All left sided nodules were noted to be stable and/or ground glass opacities.

There was no documented diagnosis of malignancy in the left lung until June 2020 when the physician noted that if there was a response in the left lung to systemic treatment, then this was probably multifocal AIS. However, only one tumor in the left lung responded to treatment.

While it seems somewhat unlikely that only a single AIS in the contralateral lung should be metastasis from the right lung malignancy, it is difficult to apply the multiple tumors rules to this case.

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

Kidney, right interpolar neoplasm, partial nephrectomy: Oncocytic renal neoplasm of low malignant potential (ORNLMP).

Within part B, right interpolar kidney neoplasm, the neoplasm shows oncocytic features, with abundant granular eosinophilic cytoplasm and enlarged vesicular nuclei with prominent central nucleoli. The cells are arranged in small nests and tubules with hypocellular fibrous stroma identified within the background. Scattered binucleated cells are present, and rare cells with irregular nuclear membranes are present. No perinuclear halos or prominent cell membranes are present. Given the histologic features, the neoplasm is best classified as an oncocytic renal neoplasm of low malignant potential (ORNLMP).

Patient has a history of B-cell lymphoma with multimodality treatment in 2002. Lab work in 2015 showed multilineage dysplasia leading to a diagnosis of therapy-related myelodysplastic syndrome. Patient presents in 2020 for a bone marrow biopsy now showing low-grade MDS. The MDS appears to have the same multilineage dysplasia previously identified.

MDS, NOS is not listed in the Heme DB as a possible transformation of t-MDS, nor is it listed as a Same Primary for t-MDS. Likewise, t-MDS is not listed as a more specific myelodysplastic syndrome, a transformation of MDS NOS, or a Same Primary as MDS, NOS.

The first M rule that applies to this case is M15, and the Multiple Primaries Calculator indicates that the MDS, NOS should be a new primary.

The following cases were submitted to the central registry as separate primaries. First case submitted as C772 with histology coded 9591/3 (Lymphoma, NOS). Second case submitted as C162 with histology coded 9699/3 (MALT Lymphoma).

Sequence 01 - 5/2016, Excisional biopsy pancreatic tail lymph node: suspicious for malignant B-cell lymphoma. No treatment recommended or administered.

Sequence 02 - 2/2019, Stomach biopsy: MALT Lymphoma. Unknown if treatment was recommended or administered. Biopsy was only at this facility.

Using the Hematopoietic and Lymphoid Neoplasm Multiple Primaries/Histology rules, Rule M7 makes this a single primary. Note 4 instructs to change the histology of the initial abstract to the more specific histology (9699/3). If this is done, they would be multiple primaries per the exception within Rule M2. Should the histology on sequence 01 be changed to the MALT lymphoma and the cases would be multiple primaries or is this a single primary?

Patient has left salpingo-oophorectomy showing fallopian tube with focal high grade serous intraepithelial neoplasm.

In reviewing some journal articles, the term STIN is being used to describe both STIC and serous tubal intraepithelial lesion (STIL). We will likely continue to see this term used, so it would be nice to have some clarity.