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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.

Scenario: Patient had a transurethral resection of the prostate on 8-29-19, positive for basal cell carcinoma with focal squamous differentiation involving approximately 50% of tissue (determined not to be mets by consult). On 11-14-19, the patient had a prostatectomy positive for residual basal cell carcinoma and a small focus of infiltrating prostatic adenocarcinoma. According to AJCC, 8th edition, page 724, basal cell carcinoma of the prostate is 8147/3 and we ignored the small focus of adenocarcinoma. The above scenario was reported as two primaries (8090/3 and 8140/3), but we are thinking it is one.

Since AIN I is not reportable and AIN II is not reportable until 2021, we are not sure if we can say the patient was disease free because there was no intervening reportable tumor (AIN III), or was never disease free because there was evidence of related disease (lower grade dysplasia).

The Breast EOD Regional Nodes notes contain new clarification regarding the clinical assessment vs. pathological assessment codes, but the new Note 2 does not specifically indicate an exception for neoadjuvant therapy. However, if the pre-treatment lymph node core biopsy proved cN1 disease, and the post-treatment resection proved ypN0 disease, should the clinical assessment code (code 150) have priority over any pathological assessment code (including 200) since the involved lymph node was only clinically positive and not pathologically positive? Should an exception be added to Note 2 to address cases where neoadjuvant therapy is given, but the clinical assessment is greater than the pathological assessment?

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?

4/28/21 PET: There is extensive widespread/multifocal hypermetabolic uptake within lymph nodes, skeleton, and spleen, compatible with malignancy. Differential diagnosis includes lymphoma and metastatic disease of indeterminate primary, with lymphoma favored.

4/28/21 Right retroperitoneal lymph node, needle core biopsy: Large B-cell lymphoma. See comment. Comment: The differential includes T-cell/histiocyte-rich large B-cell lymphoma and diffuse variant of nodular lymphocyte predominant Hodgkin lymphoma. It is challenging to distinguish these two on the needle core biopsy. An excisional biopsy is recommended for a definite diagnosis if clinically appropriate. ADDENDUM: B-Cell Lymphoma, FISH: negative. No rearrangement of MYC, BCL2 and BCL6 and no fusion of MYC and IGH.

5/14/21 Gastrohepatic lymph node, biopsy: Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) with T-cell/histiocyte rich diffuse large B-cell lymphoma-like transformation. Focal in situ follicular neoplasia.

6/3/21 Medical Oncologist: Biopsy confirms that patient has a nodular lymphocytic Hodgkin lymphoma which has transformed into a T-cell rich DLBCL. This variant of Hodgkin disease is a good prognostic histology which generally behaves indolently, like a low grade lymphoma.

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.

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).

Cervix, loop electrosurgical excision procedure: Cervix at transformation zone with stratified mucin-producing intraepithelial lesion (SMILE). SMILE is present at the ectocervical margin. An immunohistochemical stain* for p16 demonstrates strong, diffuse positivity in the lesional epithelium. A mucicarmine stain is also positive in the lesional epithelium, supporting the diagnosis of SMILE.

11/24/20 Adnexal mass, cyst fluid: Positive for malignant cells. Clusters of inhibin-positive, CK7-negative cells, consistent with adult granulosa cell tumor cells. Groups of inhibin-negative, CK7-positive epithelial cells consistent with serous borderline tumor cells. Peritoneal washing: Positive for malignant cells. Small groups of inhibin-positive, CK7-negative cells, consistent with adult granulosa cell tumor cells.

A. Left ovarian mass: Adult granulosa cell tumor (AGCT) of ovary (see note). pTNM Stage: pT1c3 pNX - Serous borderline tumor (SBT) of ovary (see note). pTNM Stage: pT1a pNX. Fallopian tube; unremarkable.

B. Right ovary: - Serous cystadenofibroma of ovary. Fallopian tube; unremarkable.

C. Left pelvic wall nodule: Fibro-calcified nodule, consistent with necrotic appendix epiploica.

D. Uterus (hysterectomy): Uterine leiomyomas. Endosalpingiosis of uterine serosa and paracervical tissue. Atrophic endometrium.

Note: The left ovarian mass is involved by a combined adult granulosa cell tumor and a serous borderline tumor. The AGCT mainly involves the thick-walled cystic area while the SBT the thin-walled cyst/s. The 2 neoplastic elements do, however, demonstrate areas of intimate and close intermingling. From the current literature, it appears that, based on FOXL2 mutation, the AGCT component of combined AGCT and ovarian epithelial tumors is either a true neoplastic processes or an AGCT- like proliferation morphologically indistinguishable from AGCT. To further evaluate the nature of the AGCT component, a FOXL2 analysis is in progress and an addendum will follow.