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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?

10/4/2018 Frozen Section Diagnosis: Brain tissue with atypical cells and inflammatory cells, defer to permanents for further evaluation. Note: Tissue for flow cytometry is submitted. Final Diagnosis: Preliminary Diagnosis: Brain Tumor, Biopsy: Neoplasm, suggestive of lymphoma (see comment). Comment: The tumor exhibits nuclear atypia and increased mitosis. The tumor cells are immunologically positive for LCA and with very high ki67 labeling index. GFAP and synaptophysin are not expressed by tumor cells. The above suggests a lympho-proliferative process. This case is forwarded to the hematopathology service of this department for further evaluation. The final diagnosis report will be issued by the hematopathologist as an addendum.

Supp Rpt Add Addendum Diagnosis: The brain biopsy showed brain tissue large lymphoid cell infiltrate. Additional immunohistochemical stains are performed. The large cells are positive for CD20, BCL2, BCL6 (subset), MUM1, and CD30, negative for CD3, CD5, and CD10. Staining for c-MYC is negative. Ki-67 positive large cells are approximately 18%. EBER is strongly positive by ISH. Diagnosis: Brain lesion, biopsy: EBV+ Diffuse Large B-cell Lymphoma. Addendum Comment: The concurrent flow cytometric study showed monoclonal lambda-positive B-cells without out CD5 and CD10 expression, consistent with B-cell lymphoma.

Patient was diagnosed with low-grade non-Hodgkin lymphoma in 2011, later classified as hairy cell leukemia-variant.

Right cervical node biopsy in 2020 proved HCL-v and a subsequent 2021 right tongue base biopsy showed DLBCL. The tongue base biopsy path includes the comment, patient has history of HCL-v, but the morphology and flow cytology features are different from the patient's previous right cervical node biopsy. This DLBCL likely represents a second de novo lymphoma, but cannot exclude an unusual transformation of the prior HCL-v.

Per Heme Rule M7, abstract a single primary when a more specific histology is diagnosed after an NOS if the Heme DB confirms the same primary. The histology code for HCL-v, 9591/3 is a non-specific code, but it seems like a specific histology. The Heme Calculator does say 9591 and 9680 are the same primary, but we are unsure if that is correct for this case of HCL-v followed by DLBCL.

There is a group of patients diagnosed within the past few years with ovarian cancers who are now enrolled in a clinical trial and are being screened as potential patients for a particular protocol. The screening for these particular cases is being done by a pathologist who has a particular interest in GYN pathology. As the pathologist is screening the cases, there are some which the pathologist is reclassifying as being fallopian tube primaries rather than ovarian primaries. This is apparently due to newly emerging findings and literature. The problem for me is that these cases have been entered into the registry as ovarian primaries, which was correct as of the time of the initial diagnosis. Should the abstracts remain as they were initially coded, since the diagnosis was ovarian cancer at the time they were diagnosed, or should these cases be updated to reflect the current pathologist's interpretation that these are fallopian tube primaries?

Patient is diagnosed July 2021 with high-grade serous carcinoma on ascites cytology. Tumor debulking total abdominal hysterectomy/bilateral salpingo-oophorectomy in August shows high-grade serous carcinoma involving the right ovary (capsule intact, right fallopian tube is negative), left ovary (capsule ruptured), and fallopian tube. Pathologist has chosen tumor site to be bilateral ovaries in the staging summary, with the left fallopian tube listed as “other tissue/organ involvement” along with uterus, peritoneum, and omentum. Additional findings in staging summary includes serous tubal intraepithelial carcinoma (STIC).

Our interpretation of SINQ 20210025 is that any case with both ovarian and tubal involvement would be coded as a fallopian tube primary if STIC is present, even when the pathologist is clearly calling the case ovarian. If this is correct, then the previous SINQ 20120093 may need to be updated with a date restriction reference since it would be in disagreement with this instruction.

If our interpretation is incorrect, then the STIC would be an additional primary per MP/H Rule M11.

Patient is diagnosed March 2021, with malignant pleural effusion, clinical impression supports either endometrial or tubo-ovarian primary and neoadjuvant chemotherapy is given. Subsequent total abdominal hysterectomy/bilateral salpingo-oophorectomy (TAH/BSO) in July, shows high-grade serous carcinoma involving the right fallopian tube and bilateral ovaries, as well as bilateral STIC. Summary Stage lists tumor site as right fallopian tube, with the serous tubal intraepithelial carcinoma (STIC) noted under “additional findings.”

Should the contralateral (left-sided) STIC be accessioned as an additional primary, per MP/H Rule M8, the since fallopian tubes are listed in Table 1 as Paired Organs with Laterality?

Lymphangioleiomyomatosis (LAM) became reportable (9174/3) for diagnoses 2023 and later. While this neoplasm was only added to the Lung schema in the Solid Tumor Rules manual, this is a mesenchymal neoplasm which may arise outside of the lung and the reportability change was not limited to LAM of the lung.

How should primary site be coded when a left pelvic lymph node dissection for an unrelated high-grade serous carcinoma of the right fallopian tube incidentally proved LAM in the pelvic lymph nodes? The pelvic lymph nodes were the only site of involvement; there was no evidence of lung involvement. As this is a mesenchymal tumor, should the primary site default to C499 (Soft tissue, NOS) according to the default primary site rule for sarcomas described in the SEER Manual? Or should the primary site be coded to C775 (Pelvic lymph nodes) as this was the only proven site of involvement?

SINQ 20000245 states that high grade or severe dysplasia in not synonymous with in situ disease. However, per page 109 in the 7th edition of AJCC Cancer Staging Manual, high grade dysplasia is the only term listed under Tis. A note on that page explains that "high-grade dysplasia includes all noninvasive neoplastic epithelia that was formerly called carcinoma in situ, a diagnosis that is no longer used for columnar mucosae anywhere in the gastrointestinal tract."

There has been considerable pressure from registrars at larger reporting facilities to re-address this issue. The pathologists at these facilities state that they are correctly documenting the presence of in situ disease when they use the term high grade dysplasia for gastrointestinal tract tumors. In their opinion, it is not necessary to add the term in situ in parentheses following the use of the term high grade dysplasia to clarify the behavior of these lesions in their pathology reports. If the term "carcinoma in situ" is no longer being used by many pathologists for sites in the gastrointestinal tract, won't this lead to underreporting of in situ disease for these sites unless the reportability guidelines are changed?