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Patient has a resection labeled as: Spine, designated intramedullary lesion. The Final Diagnosis is: Melanocytic neoplasm with features most consistent with primary intramedullary melanocytoma. The Diagnosis Comment states: The overall immunophenotypic and morphologic impression is a primary central nervous system melanocytoma.

The ICD-O-3 lists melanocytoma, NOS histology code as 8726/0, but does not provide a site-associated code. If the ICD-O-3 is used, the histology would be 8726/0 and the primary site presumably would be C720 since the tumor was specifically described as being intramedullary (i.e., within the spinal cord medulla).

Table 6 (Solid Tumor Rules, Non-Malignant CNS Equivalent Terms and Definitions) does not list either an intramedullary melanocytoma or melanocytoma (NOS). However, Table 6 does include meningeal melanocytosis 8728/0 and meningeal melanocytoma 8728/1. If Table 6 is used and the histology is coded 8728/1, then the primary site would presumably be C701 per the ICD-O-3 site-associated listing for this histology (C709).

Patient has a remote history of a right thalamic mass status-post two resections; reported as malignant oligodendroglioma (pathology not received) and chemo/radiation therapy, who recently presented with persistent headaches. Imaging revealed a 3.4 cm heterogeneous lobulated right thalamic mass with coarse calcifications and a probable cystic component.

Pathologist indicates the histologic and immunophenotypic features of this neoplasm are that of relatively circumscribed neuroepithelial tumor without high grade features (mitotic activity, microvascular proliferation, necrosis). Molecularly this neoplasm is characterized by a PATZ1-EWSR1 fusion, which has recently been proposed to be a distinct neuroepithelial tumor entity with a broad histological spectrum.

Patient has a perihilar bile duct primary with a microscopic focus of invasive moderately differentiated adenocarcinoma arising in a large papilloma. The MP/H Rules do not address adenocarcinomas arising in a papilloma, only adenocarcinomas arising in an adenoma (or polyp). Should the histology be coded as 8140 for the invasive adenocarcinoma component? Or should the matrix principle be applied and the histology coded as a malignant glandular papilloma (8260/3)?

Patient has a history of human immunodeficiency virus and diffuse large B cell lymphoma diagnosed in 2012, and is status/post systemic therapy and in remission since completing first course treatment. In 2022, the patient has imaging suspicious for recurrence. A biopsy of a deep left cervical lymph node showed atypical lymphoid infiltrate with the comment: “This is a challenging case. The constellation of findings is most in keeping with early / focal and subtle involvement by a nodular lymphocyte predominant B-cell lymphoma. We find no evidence of involvement by a diffuse large B-cell lymphoma.” The managing physician later states, “Cervical lymph node biopsy (06/2022) was consistent with nodular lymphocyte predominant B cell lymphoma.”

Patient has a history of follicular cell non-Hodgkin lymphoma dating back to the 1990s. The patient was treated with chemotherapy and bone marrow transplantation, radiation and rituximab. The patient had no evidence of recurrence. In April 2010 a lesion appeared on the side of the scalp above the left ear with a diagnosis of "B-cell lymphoma with prominent large cell component, compatible with primary cutaneous follicle center cell lymphoma." The oncology diagnosis is "primary cutaneous follicle center lymphoma."

Would the Multiple Primaries Calculator be used in this case?

Patient has a history of diffuse large B-cell lymphoma involving multiple lymph node regions (site C778) with bone marrow involvement diagnosed in 2003 and a history of follicular lymphoma confined to the thyroid and neck lymph nodes diagnosed in 2009. In 2010 the patient was diagnosed with follicular lymphoma in the inguinal and abdominal lymph nodes.

The 2003 diagnosis of DLBCL and the 2009 diagnosis of follicular lymphoma are the same primary according to the 2009 rules, the Single Versus Subsequent Primaries Table.

What rule is used to determine whether the 2010 diagnosis of follicular lymphoma represents a new primary? Which histologies are compared using the rules: the 2010 follicular lymphoma diagnosis to the 2009 follicular diagnosis or the 2010 follicular lymphoma diagnosis to the 2003 DLBCL diagnosis?

Patient has a history of chronic lymphocytic leukemia (CLL). February 2011 abdomen/pelvis x-ray showed development of bilateral renal masses. April 2011 PET scan showed intense areas of hypermetabolic activity corresponding to known bilateral renal masses, new hypermetabolic liver lesions, as well as left upper retroperitoneal lymphadenopathy. All findings are worrisome for malignancy. March 2011 right kidney mass biopsy was positive for diffuse large B-cell lymphoma (DLBCL). Bone marrow biopsy was negative for lymphoma.

Patient has a history of breast cancer and diffuse large B-cell lymphoma (DLBCL), both treated with chemotherapy and radiation.

On 6/26/19, bone marrow biopsy: MDS with excess blasts-2 (18% dysplastic blasts) in a normocellular marrow (overall 40% cellularity) with trilineage dysplasia. Comment: least myelodysplastic syndrome with excess blasts-2. However, an early/evolving AML cannot be completely excluded. The findings likely represent therapy-related myeloid neoplasm.

MD note on 7/15/19: Diagnosis: MDS, high grade borderline AML with complex karyotype secondary disease. Patient has high grade MDS which is bordering on AML transformation with 20% blasts by IHC and areas higher than this. This is likely secondary to the treatment she has received for her other cancers particularly pelvic radiation for her DLBCL. Given her very high IPSS score, it is likely she will eventually develop AML. No treatment given.

On 7/15/19, bone marrow biopsy: Persistent acute leukemia in a marrow with trilineage dyspoiesis and 23% blasts.

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.

Patient has a history of AIN I/low-grade squamous intraepithelial lesion (LSIL) dating back to at least 2015, was diagnosed with AIN II-III in 12/2019, and then diagnosed again with AIN II-III in 08/2021. There is no indication of treatment or a disease-free interval for this patient.

SINQ 20210015, while not an exact match to this case, implies there is no clear disease-free interval for these AIN diagnoses, so it is the same non-reportable neoplasm diagnosed prior to reportability (12/2019). However, there was a diagnosis of a reportable neoplasm in 2021, so it also seems possible this would be accessioned as a reportable tumor based on a diagnosis of reportable tumor diagnosis in 2021.

With the reportability changes for these intraepithelial neoplasia II/II-III tumors, these situations will arise more frequently.