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Report Produced: 12/27/2025 06:00 AM

Report Question ID Question Discussion Answer Year
20130215

Reportability--Heme & Lymphoid Neoplasms: Is hemophagocytic lymphohistiocytosis synonymous with an EBV-associated lymphoproliferative disorder in children reportable? See Discussion.

Pathology report states: Prominent T-cell infiltrate with frequent immunoblast-like cells. COMMENT: Findings consistent with an acute EBV-associated hemophagocytic process. In addition, there is a prominent CD8 + T-cell infiltrate with many large, activated forms. This T-cell process may represent an EBV-associated lymphoproliferative disorder in children.

EBV-associated lymphoproliferative disorder in children is listed in the Heme database. However, throughout multiple admissions, the oncologist states the diagnosis as "hemophagocytic lymphohistiocytosis". Are the two the same condition?

The patient is being treated with Etoposide.

Per Appendix F, do not report this case based on the information provided.

The oncologist likely used the pathology report and clinical factors to determine the diagnosis of hemophagocytic lymphohistiocytosis, which is not reportable. Hemophagocytic lymphohistiocytosis is caused by an over stimulated immune system (infection, etc.). This clinical syndrome is associated with a variety of underlying conditions. To be reportable, it must state "fulminant hemophagocytic syndrome" (in a child) to be reportable (9724/3).

The pathology report for this case is not definitive. It states that the process "may" represent the EBV-associated lymphoproliferative disorder in children.

Follow back on this case to confirm reportability if possible.

 2013
20130122 Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned when an excisional biopsy of a chest wall nodule shows diffuse large B-cell lymphoma (40%) and follicular lymphoma, grade 3A (60%)? See Discussion.

The patient presented with a right chest wall nodule. The PET scan showed widespread disease: subcutaneous nodule/mass in the left scalp and right chest wall; large right paraspinal mass; soft tissue density likely a second early paraspinal mass at the right costovertebral junction; right paravertebral mass; and abnormal bony foci in the right humeral head, right iliac crest, right acetabulum and right femur. The physical exam showed 2 cm left supraclavicular lymphadenopathy and a firm 3 cm mass in the right chest wall. Lungs were clear. Abdomen showed no masses or ascites, and no palpable hepatosplenomegaly.

Chest wall nodule excisional biopsy pathology: Lymph node and adjacent soft tissue: Malignant lymphoma with components: 1. Diffuse large B-cell lymphoma (40%). 2. Follicular lymphoma, grade 3A (60%). Pathology report note states the diffuse large cell lymphoma is probably arising from the follicular center cell lymphoma.

Should this be a single primary? There is no mention of cutaneous lymphoma.

Accession a single primary per Rule M4. Code histology to 9680/3 [diffuse large B-cell lymphoma] per Rule PH11.

Per Rule M4, accession a single primary when two or more non-Hodgkin lymphomas are present in the same lymph node or organ.

Per Rule PH11 code the histology to diffuse large B-cell lymphoma (DLBCL) (9680/3) when DLBCL and any other non-Hodgkin lymphoma are present in the same lymph node(s), lymph node region(s), organ(s), tissue(s) or bone marrow.

SEER*Educate provides training on how to use the Heme Manual and DB. If you are unsure how to arrive at the answer in this SINQ question, refer to SEER*Educate to practice coding hematopoietic and lymphoid neoplasms. Review the step-by-step instructions provided for each case scenario to learn how to use the application and manual to arrive at the answer provided. https://educate.fhcrc.org/LandingPage.aspx.

2013
20130129 Histology--Heme & Lymphoid Neoplasms: How is histology coded for a diagnosis of composite lymphoma (follicular lymphoma and small lymphocytic lymphoma, BCL-2 positive)?

For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.

Code the histology to 9823/3 [chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL)] per Rule PH15. Code the histology to the non-Hodgkin lymphoma (NHL) with the numerically highest ICD-O-3 code when two or more NHLs are present in the same present in the same lymph node(s) or lymph node region(s), tissue(s), organ(s), or bone marrow. Both follicular lymphoma [9690/3] and SLL [9823/3] are types of NHL. Therefore, the histology is coded to 9823/3.

This composite histology represents a single primary per Rule M4. The rule states to abstract a single primary when two or more types of non-Hodgkin lymphoma are simultaneously present in the same anatomic location(s), such as the same lymph node or lymph node region(s), the same organ(s), and/or the same tissue(s).

SEER*Educate provides training on how to use the Heme Manual and DB. If you are unsure how to arrive at the answer in this SINQ question, refer to SEER*Educate to practice coding hematopoietic and lymphoid neoplasms. Review the step-by-step instructions provided for each case scenario to learn how to use the application and manual to arrive at the answer provided. https://educate.fhcrc.org/LandingPage.aspx.

2013
20130094 MP/H Rules/Multiple primaries--Lung: How many primaries are accessioned and which M rule applies for a 2010 diagnosis of clear cell adenocarcinoma of the left upper lobe lung followed by a 2012 diagnosis of adenosquamous carcinoma of lung origin without evidence of a primary lung tumor? See Discussion.

Patient was diagnosed with T1 N0 M0 adenocarcinoma with prominent clear cell features [8310/3] in the LUL on 08/05/2010. The patient underwent a lobectomy only.

On 10/09/2012 the patient underwent an iliac bone biopsy showing non-small cell carcinoma with glandular and squamous features [8560/3]. Clinically, the physician is calling this stage IV adenosquamous carcinoma of lung origin involving lymph nodes, spleen and bones. There were no FDG avid pulmonary nodules found. There was no pathologic comparison to the prior lung tumor.

Should the 2012 diagnosis be a new primary because the histology is different from the 2010 diagnosis? Or should this be one primary because there appears to be only metastatic disease with no new primary lung tumor identified in 2012? The choice of one primary seems supported by the fact that the 2012 tumor showed glandular and squamous features, and the 2010 tumor also showed glandular and clear cell (NOS) features. The clear cell could have been a clear cell squamous cell carcinoma. The original tumor was not re-examined.

Accession a single primary, clear cell adenocarcinoma [8310/3] of the left upper lobe lung [C341] diagnosed on 08/05/2010.

The MP/H Rules do not apply to the 2012 diagnosis because only metastatic sites were examined and there was no re-examination of the original 2010 tumor. Therefore, the disease process in 2012 is assumed to be metastatic from the lung primary diagnosed in 2010.

2013
20130021 Histology--Heme & Lymphoid Neoplasms: When will the follicular lymphoma, grade 1 code [9695/3] ever be used? See Discussion. The Abstractor Notes currently do not explain the histologic classification of follicular lymphoma [FL]. Frequently, FL grade 1 and 2 are not being separated and are described as "low grade" or "grade 1-2" in the pathology final diagnosis. The correct histology code would be 9691/3 [FL, grade 2] for these cases. Apparently, per the 2008 WHO Classification, grade 1 and grade 2 are being grouped together as grade 1-2 due to the minimal difference in patient outcome. If these histologies are grouped together, will histology code 9695/3 [FL, grade 1] ever be used? Should the Heme Database explain the classifications of follicular lymphoma grade 1, 2, and 3?

When the latest WHO classification for heme neoplasms was written in 2008, there was a lot of controversy about whether or not the FL grading system was useful or not. A number of papers have been written stating that grades 1 and 2 do not have a statistically different survival or transformation rate. Given that the controversy had not been settled by those in the clinical world, the WHO recommended analyzing grades 1 and 2 together. They did not, however, remove either grade 1 or 2 from their classification. When the WHO intend to change their classification (have both grades classified under one histology number), they omit one code from their book (make it obsolete) and change the definition for the other code. The 2008 WHO book did not make either ICD-O-3 code obsolete. Therefore, we continue to collect the cases as designated by the pathologist. If the controversy is settled before the next WHO classification, you may see changes in the codes.

Additionally, since the 2008 WHO book was written, there have been some clinical papers challenging the designation of grade 3. They contend that grade 3 can be mistaken for low-grade.

The grades for follicular lymphoma are based on the number of centroblasts per high powered field (HPF). The number of centroblasts for grade 1 is 0-5; for grade 2 is 6-15, for grade 3a and 3b is >15 centroblasts. 3a has centrocytes and 3b has no centrocytes.

2013
20130069 Reportability--Heme & Lymphoid Neoplasms: Is chronic myeloproliferative neoplasm reportable? See Discussion. The Heme DB indicates myeloproliferative neoplasm is reportable, but does not indicate whether chronic myeloproliferative neoplasm is. Does the word "chronic" make this non-reportable?

For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.

Chronic myeloproliferative neoplasm is reportable. The preferred term is myelodysplastic/myeloproliferative neoplasm, unclassifiable (MPN). Chronic myeloproliferative neoplasm is listed in the Heme DB under the Alternate Names section for this neoplasm.

The term chronic does not affect the reportability of this neoplasm. The newer terms are myeloproliferative neoplasm or myeloproliferative disorder and chronic is not used in most diagnoses.

SEER*Educate provides training on how to use the Heme Manual and DB. If you are unsure how to arrive at the answer in this SINQ question, refer to SEER*Educate to practice coding hematopoietic and lymphoid neoplasms. Review the step-by-step instructions provided for each case scenario to learn how to use the application and manual to arrive at the answer provided. https://educate.fhcrc.org/LandingPage.aspx.

2013
20130031

Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned when a plasmacytoma of the intervertebral disc is diagnosed in 2010 followed by a diagnosis of immature plasma cell myeloma by a right hip biopsy in 2011? See Discussion.

The patient was diagnosed with intervertebral disc plasmacytoma and had radiation therapy to the pelvic bones in 2010. In 2011 (more than 21 days later) a right hip biopsy revealed immature plasma cell myeloma. There is clinical documentation that this is progression into myeloma. Per the Heme DB (Primary Site(s) and Definition sections) and Rule PH30, in the Heme Manual, the primary site is coded to C421 [bone marrow] and the histology is coded 9732/3 [plasma cell myeloma] when there is a clinical diagnosis of multiple myeloma and/or there is no documentation of a bone marrow biopsy or the results are unknown. This patient did have a bone marrow biopsy that indicates there are an increased plasma cells present; plasma cells represent less than 10%. The skeletal survey and bone scan did not reveal any further lesions.

Is this progression of disease because there is only one lesion in the right hip 8 months after the diagnosis of plasmacytoma? Or is this a second primary based on the right hip biopsy that showed plasma cell myeloma and the physician's documentation of disease progression? Plasmacytomas are usually single lesions. Would this disease process have multiple lesions if they are diagnosed at different times?

For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.

This case is accessioned as two primaries: Plasmacytoma diagnosed in 2010 and plasma cell myeloma diagnosed in 2011 per Rule M10. The patient has a diagnosis of a solitary plasmacytoma (chronic neoplasm) followed by a diagnosis of plasma cell myeloma (acute neoplasm) diagnosed greater than 21 days later.

The physician is calling this a progression to plasma cell myeloma even though the bone marrow has less than 10% plasma cells, take this statement as progression or a clinical diagnosis of plasma cell myeloma.

SEER*Educate provides training on how to use the Heme Manual and DB. If you are unsure how to arrive at the answer in this SINQ question, refer to SEER*Educate to practice coding hematopoietic and lymphoid neoplasms. Review the step-by-step instructions provided for each case scenario to learn how to use the application and manual to arrive at the answer provided. https://educate.fhcrc.org/LandingPage.aspx.

 2013
20130176

Reportability--Ovary: Is an adult granulosa cell tumor of the right adnexa reportable if the left adnexa, diaphragm and paratubal tissue are reported to be consistent with metastasis? See discussion.

Per the pathology report: Right adnexa: adult granulosa cell tumor. Left adnexa: Foci of metastatic granulosa cell tumor in paratubal tissue. Diaphragm smears: consistent with metastatic granulosa cell tumor. Comment: The morphology and immunoprofile of the cellular aggregates in the paratubal soft tissue are consistent with metastatic granulosa cell tumor.

Based on the information provided, this case of adult granulosa cell tumor is malignant and reportable. According to our expert pathologist consultant, "though granulosa cell tumor NOS/ adult NOS is 8620/1, the presence of peritoneal implants or metastases, and/or lymph node metastases indicates the tumor is malignant, and it should be coded /3."

Note that the presence of implants or metastases does not indicate malignancy in the case of low malignant potential ovarian epithelial tumors. Our path expert explains "in contrast, by convention the behavior of borderline/LMP ovarian epithelial tumors is determined by the ovarian primary, and is /1, even though there may be peritoneal implants/metastases, or metastatic disease in lymph nodes. The treatment may vary in these circumstances, but to my knowledge the decision as to the tumor designation remains based on the primary tumor."

2013
20130060 Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned for a diagnosis of bilateral extranodal orbital lymphoma when the same histology is present in both orbits?

For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.

This case should be accessioned as a single primary lymphoma of bilateral orbits per Rule M2. Abstract a single primary when there is a single histology. Both orbits showed the same histology. Note 1 for Rule M2 states bilateral involvement of lymph nodes and/or organs is a single primary.

SEER*Educate provides training on how to use the Heme Manual and DB. If you are unsure how to arrive at the answer in this SINQ question, refer to SEER*Educate to practice coding hematopoietic and lymphoid neoplasms. Review the step-by-step instructions provided for each case scenario to learn how to use the application and manual to arrive at the answer provided. https://educate.fhcrc.org/LandingPage.aspx.

2013
20130157

Primary Site--Heme & Lymphoid Neoplasms: What primary site code should be assigned and what rule justifies that code?

Scenario: Pleural effusion, underwent thoracentesis. Pleural fluid unexpectedly showed Large B-Cell Lymphoma. Extensive workup including CT & PET was done and all findings were within normal limits. No evidence of lymphoma was seen and no palpable adenopathy was found. The only indication of lymphoma was the malignant pleural effusion.

Code to pleura, C384.

Per the Hematopoietic database, Diffuse Large B-Cell Lymphoma can originate in the pleural cavity.

2013