Report | Question ID | Question | Discussion | Answer | Year |
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20130172 | Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned and what is the histology for each if a bone marrow diagnosis reveals co-existent systemic mastocytosis and a lymphoplasmacytic neoplasm? See Discussion. | 11/7/12 Peripheral blood flow cytometry: small population of clonal CD5- CD10- B-cells consistent with a B-cell lymphoproliferative process.
1/16/13 Bone marrow final diagnosis: co-existent systemic mastocytosis and lymphoplasmacytic neoplasm.
B-cell component of lymphoplasmacytic neoplasm constitutes 20% of bone marrow cellularity and the plasma cell component approximately 20%. The differential diagnosis includes marginal zone lymphoma with plasmacytic differentiation and lymphoplasmacytic lymphoma.
Flow cytometry: kappa monotypic B-cells and plasma cells.
Comment: Co-existence of systemic mastocytosis and mature B-cell lymphoma meets the criteria for Systemic mastocytosis with Associated Clonal Hematological Non-Mast Cell Lineage Disease (SM-AHNMD).
From our physician's progress note: KIT-D816V-positive, CD117+/CD25+ /SM-AHNMD(40% of the nucleated cells as spindled mast cells) but also seemingly two distinct lymphoid neoplasms, a CD5-negative/CD10-negative B-cell lymphoproliferative neoplasm consistent with occupying another 20% of the nucleated marrow space, together with an IgG-kappa-restricted (non-reportable diagnosis) occupying another 20% of the nucleated marrow space (and an accompanying 2.0 g/dl M-spike without hypercalcemia or anemia). |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Under the Alternate Names section of the Heme DB, systemic mastocytosis with Associated Clonal Hematological Non-Mast Cell Lineage Disease (SM-AHNMD) is a synonym for systemic mastocytosis. Per Rule M2, this is one primary. Abstract a single primary when there is a single histology. Code the histology to 9741/3 [systemic mastocytosis].
Per the pathology report, the two diagnoses of systemic mastocytosis and mantle cell lymphoma meet the criteria for SM-AHNMD. The B-cell lymphoma is a symptom/marker of the AHNMD. In systemic mastocytosis with AHNMD, a myeloid or lymphatic malignancy is diagnosed with the SM. The prognosis is usually dominated by the non-mast cell malignancy.
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 |
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20130173 | Histology/Primary site--Heme & Lymphoid Neoplasms: How is the primary site and histology coded when a bladder biopsy reveals myeloid sarcoma and a simultaneously performed bone marrow biopsy demonstrates acute myeloid leukemia? See Discussion. | 12/22/11 Bladder biopsy: myeloid sarcoma,
12/22/11 Bone marrow biopsy: acute myeloid leukemia.
Presenting symptoms were urological with three month history of painful hematuria and hydronephrosis with solid mass of bladder.
Prior to biopsy hem/onc states bladder mass of unknown pathology. CBC revealed peripheral blasts and Auer rods -- presumed diagnosis of acute myeloid leukemia (AML). No statement from physician as to where disease originated. |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Per Rule M3, abstract a single primary when a sarcoma (myeloid sarcoma) is diagnosed either simultaneously or after a leukemia of the same lineage (acute myeloid leukemia). Per the notes for Rule M3, the sarcoma is a solid manifestation of the associate leukemia.
Per PH10, code the histology to 9861/3 [acute myeloid leukemia] and the primary site to C421 [bone marrow]. PH10 states one is to code the primary site bone marrow (C421) and code the histology acute myeloid leukemia, NOS (9861/3) or any of the specific AML histologies (9840/3, 9865/3-9867/3, 9869/3-9874/3, 9891/3, 9895/3-9898/3, 9910/3, 9911/3 and 9931/3) when the diagnosis is myeloid sarcoma (9930/3) AND there is a simultaneous or previous diagnosis of acute myeloid leukemia.
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 |
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20130174 | MP/H Rules/Histology--Breast: Given that the current MP/H rules do not recognize specific types of lobular carcinoma, should the histology for an invasive pleomorphic lobular carcinoma be coded to 8022/3 [pleomorphic carcinoma] or 8520/3 [lobular carcinoma]? See Discussion. | The MP/H rules do not seem to recognize specific types lobular carcinomas. As invasive pleomorphic lobular carcinoma is "a very rare and distinct morphological variant of invasive lobular carcinoma," (ncbi.nim.nih.gov). Is this histology best reflected in code 8022/3 [pleomorphic carcinoma] or 8520/3 [lobular carcinoma]? | Code the histology to 8520/3 [lobular carcinoma].
The 4th Edition of the WHO Classification of Tumors of the Breast now describes five variants of invasive lobular carcinoma. These variants are solid type, alveolar, pleomorphic, tubulolobular, and mixed-type. WHO has not yet proposed new ICD-O codes be assigned to these variants. The upcoming solid tumor (MP/H) revisions will include instructions on coding these variants. |
2013 |
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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 |
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20130177 | MP/H Rules/Histology--Bladder: What rule and histology code apply when a TURB final diagnosis is small cell neuroendocrine carcinoma and high grade urothelial carcinoma? See Discussion. | The patient has a 6 cm tumor arising in posterior-lateral bladder extending to prostate, obliterates seminal vesicle, and invades pelvic wall.
TURB final diagnosis: Small cell neuroendocrine carcinoma. High grade urothelial carcinoma involves 10% of tumor.
Following the current MP/H single tumor rules, it appears Rule H8 applies. Per Rule H8, code the numerically higher code of 8120. By following this rule, it does not seem the histology code fairly represents this tumor. |
There is currently no rule in the urinary site MP/H Rules for this combination of histologies. The best option is to code the histology to 8045/3 [mixed small cell carcinoma], a combination of small cell with other types of carcinoma. The presence of small cell carcinoma drives the treatment decisions for this case.
This issue will be addressed in the next revision of the MP/H Rules. |
2013 |
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20130178 | Reportability--Heme & Lymphoid Neoplasms: Is refractory iron deficiency anemia reportable? | Per Appendix F, refractory iron deficiency anemia is not reportable. It is not a clonal disorder and, therefore, is not malignant. Refractory iron deficiency anemia is a condition that is unresponsive to oral iron treatment. | 2013 | |
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20130179 | Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries and what is the histology for each primary if a diffuse large B-cell lymphoma [9680/3] and a focus of splenic marginal zone lymphoma [9689/3] occur in a splenectomy specimen? See Discussion. | Patient presents with a huge mass in the spleen with direct extension to gastric fundus.
12/1/12 Splenectomy: Macroscopic nodules compatible with diffuse large B-cell lymphoma [9680/3]. Further, in the white pulp there are changes compatible with focus of splenic marginal zone lymphoma [9689/3].
Under the Transformations To section in the Heme DB, splenic marginal zone lymphoma transforms to diffuse large B-cell lymphoma. |
Per Rule M4, this is a single primary. According to Rule M4, one is 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).
Per Rule PH11, code the histology to 9680/3 [diffuse large B-cell lymphoma] and the primary site to C422 [spleen]. According to PH11, one is to code the primary site to the site of origin, lymph node(s), lymph node region(s), tissue(s) or organ(s) and 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. |
2013 |
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20130180 | Histology--Pancreas: What is the difference between pancreatic endocrine neoplasm (PanNETs) [8240/3] and the new ICD-O-3 terms pancreatic endocrine tumor, benign [8150/0] and pancreatic endocrine tumor, malignant [8150/3]? See Discussion. | SEER Inquiry 20120035 discusses the reportability of pancreatic endocrine neoplasm (PanNETs) tumors. | The difference is that 8150 is for islet cell tumors. The preferred name was changed by WHO/IARC to reflect the current language used by pathologists to describe islet cell tumors [8150].
The 8240 histology code added the neuroendocrine tumor, grade 1, low or well differentiated terms to the carcinoid ICD-O name.
Islet cell tumors are more aggressive than the pancreatic NET tumors. Treatment and prognosis are determined by the histologic type. While the histology code 8150 is not new, the histology name has been updated. |
2013 |
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20130181 | Multiple Primaries--Heme & Lymphoid Neoplasms: Should Rule M4 or the Heme DB be used to determine whether diffuse large B-cell lymphoma of the large intestine and peripheral T-cell lymphoma of the bone marrow represents one or two primaries? See Discussion. | The Heme DB identifies these as new primaries:
10/12/12 Large intestine, biopsy: Diffuse large B-cell lymphoma.
10/12/12 Bone marrow biopsy: Peripheral T-cell lymphoma. |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Per Rule M15, accession two primaries. According to Rule M15, use the multiple primaries calculator to determine the number of primaries for all cases that do not meet the criteria of M1-M14. Per the calculator, this scenario represents two primaries.
Assuming the only area of involvement is the large intestine, code the histology to 9680/3 [diffuse Large B-Cell Lymphoma] and per Rule PH24 code the primary site to C189 [colon, NOS]. According to PH24, one is to code the primary site to the organ when lymphoma is present only in an organ.
Rule PH26 applies to the second primary. Assuming the only area of involvement is the bone marrow, code the histology to 9702/3 [peripheral T-cell lymphoma] and code the primary site to C421 [bone marrow]. According to PH26, one is to code the primary site to bone marrow (C421) when lymphoma is present only in the bone marrow.
Rule M4 does not apply for this case. Rule M4 applied when you have two or more types of non-Hodgkin lymphoma in the same anatomic location. That is not the case in this scenario.
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 |
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20130182 | Primary site--Head and Neck: How is primary site coded if a floor of mouth biopsy reveals microinvasive squamous cell carcinoma but the definitive resection of the tongue and floor of mouth unifocal lesion reveals only in situ squamous cell cancer? See Discussion. | Patient with overlapping lesion of tongue and floor of mouth. Initial biopsy of floor of mouth reveals microinvasive squamous cell cancer. Definitive resection reveals in situ squamous cell cancer. Pathology report states unifocal tumor. The tumor site on pathology report is documented as involving the tongue and floor of mouth.
Should the primary site be coded to floor of mouth because it is the site of invasive disease? Or is primary site C148 [overlapping sites of lip, oral cavity and pharynx] because invasion should not be used to determine primary site? |
Code the primary site to C068 [overlapping lesion of other and unspecified parts of the mouth]. Based on the information provided, this is a tumor described as a "book-leaf" lesion a lesion that overlaps the floor of the mouth and the underside of the tongue. | 2013 |