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Bone marrow, right iliac crest (aspirate smear, touch preparation, clot section and core biopsy): Hypercellular marrow (40-50%) with plasma cell neoplasm (see Comment): " No evidence of metastatic carcinoma. " Adequate iron storage.

Comment: CBC data shows normocytic anemia. Flow cytometric analysis of bone marrow detects a kappa restricted plasma cell population that expresses CD138 and CD38. CD56 is positive. CD19 and CD20 are negative. T lymphocytes are immunophenotypically unremarkable. Polyclonal B lymphocytes are detected. Blast gate is not significantly increased. Immunohistochemical stains are performed on the biopsy core and clot section for greater sensitivity and further architectural assessment with adequate controls. CD138 positive plasma cells comprise > 70% of the total cellularity. AE1/AE3 is negative. Taken together, the morphologic and immunophenotypic findings are consistent with a diagnosis of plasma cell neoplasm. Trilineage hematopoietic activity as are seen.

The path report reads: COMMENT: There is multifocal involvement throughout both bowel segments which combined represent a subtotal colectomy procedure. There are at least 11 tumors, all of which are histologically similar. Given the unusual gross appearance, a representative portion of the largest mass (hepatic flexure) was forwarded to _____ for flow cytometric evaluation. There is chronic active colitis in the background suggestive of idiopathic inflammatory bowel disease, specifically ulcerative colitis. However, no dysplasia is seen in multiple random sections of grossly benign large bowel. ADDENDUM from expert gastroenterologist: The carcinomas are poorly differentiated without specific histologic features but are consistent with colon primaries. These findings are consistent with an MLH1-deficient carcinoma. Given the background chronic active colitis consistent with ulcerative colitis, this likely represents colitis-associated neoplasia which can be associated with multifocality.

Clinically, the patient was felt to have a pineocytoma (borderline tumor) on imaging, but the subsequent biopsy proved a pineal germinoma (malignant tumor). The Date of Diagnosis instructions state to code the month, day and year the tumor was first diagnosed, clinically or microscopically, by a recognized medical practitioner, but it does not indicate whether differences in behavior alter the diagnosis date.

For brain and central nervous system tumors, should the diagnosis date be the first date a tumor is SEER reportable? Or should the diagnosis date for those tumors ultimately proven to be malignant, be the date the malignancy was diagnosed?

The patient's right breast DCIS in 2007 was treated with bilateral mastectomies with negative lymph nodes and negative margins. The patient refused Tamoxifen at that time. In 2010 a right chest wall mass excision revealed infiltrating ductal carcinoma with negative axillary lymph nodes. The physician states this is a recurrence. Per MP/H rule M8 this invasive tumor must be abstracted as a new primary. Would the primary site of the 2010 tumor be coded to breast or chest wall given that the patient has a previous mastectomy?

A recent molecular study of PNET tumors at NCI (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5139621) seems to indicate the discovery of four new CNS tumor entities, of which HGNET-BCOR is one. The article suggests that these are not primitive neuroectodermal tumors tumors (PNET), but something different.

"Resection, periampullary duodenum: Gangliocytic paraganglioma, with metastasis to one large periduodunal lymph node. Six other small lymph nodes negative. COMMENT: The primary tumor in the duodenum is made up mainly endocrine cell component. This component appears to have metastasized to a periduodenal lymph node."

Mantle cell lymphoma diagnosed in 1997 which was treated with chemotherapy. Now in 2014 a 'relapse' of non-Hodgkin lymphoma. They do a biopsy of the pericardium, which is called low grade B cell non Hodgkin lymphoma. See comment. The comment says histochemical stains are reviewed and findings are consistent with involvement by a CD5 positive low grade B cell lymphoma. Lack of cyclin D1 and SOX-11 positivity as well as negative IGH-CCND1 FISH analysis essentially rule out mantle cell lymphoma. The morphologic and immunophenotypic features of this disorder are not specific for any lymphoma subtype. The differential includes CLL, marginal zone lymphoma, and lymphoplasmacytic lymphoma. If this is coded NHL, NOS (9591) it is the same primary as seq. 1 and would not be abstracted.

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.