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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. |
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20130124 | Reportability--Heme & Lymphoid Neoplasms: Is Rosai-Dorfman's syndrome (histiocytosis) a reportable malignant condition? | Rosai-Dorfman disease is not reportable. Rosai-Dorfman disease is a rare non-neoplastic disease. This disease can mimic lymphoma and extranodal involvement is frequent. | 2013 | |
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20130189 | Reportability--Brain and CNS: Are the terms 'mass' and 'lesion' reportable terms for accessioning brain and CNS primaries? See Discussion. |
With respect to reportability, the SEER Manual mentions 'tumor' and 'neoplasm,' but not 'mass' or 'lesion.' The SEER MP/H Manual states tumor, mass, lesion and neoplasm are equivalent terms for determining multiple primaries, but does this apply to reportability? If not, what is the distinction? |
'Mass' and 'lesion' are not reportable terms for benign/borderline brain and CNS tumors. Reportable terms for benign/borderline brain and CNS primaries are 'tumor' and 'neoplasm.' These terms appear in the ICD-O-3. 'Lesion' and 'mass' do not appear in the ICD-O-3. Do not use the MP/H Manual to determine reportability; page 2 of the SEER Manual is the correct source for reportability instructions. |
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20130222 | MP/H Rules/Histology--Bladder: How is the histology coded for a single bladder tumor showing invasive urothelial carcinoma with extensive divergent differentiation including small cell carcinoma, micropapillary carcinoma, and squamous cell carcinoma features? See Discussion. | MP/H rules seem to lead to Rule H8 which indicates that one use the numerically higher ICD-O-3 code. If one applies Rule H8, the histology is coded to 8131/3 [micropapillary urothelial carcinoma]. That would ignore the small cell carcinoma, which seems prognostically more significant. | Code the histology to 8045/3 [mixed small cell carcinoma], a combination of small cell with other types of carcinoma. There is currently no rule in the urinary site MP/H Rules for this combination of histologies. This will be included in the next revision of the MP/H Rules. | 2013 |
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20130201 | Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are reported for a patient with a 6/5/12 RUL biopsy that is positive for MALT lymphoma and a 6/7/12 cervical lymph node biopsy that is positive for follicular lymphoma? | For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Per Rule M15, abstract two primaries for this case. According to M15, use the Heme DB Multiple Primaries Calculator to determine the number of primaries for all cases that do not meet the criteria of M1-M14. The result is two primaries, MALT lymphoma [9699/3] and follicular cell lymphoma [9690/3].
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. |
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20130028 | Primary site--CLL/SLL: How is the primary site coded and what rule applies when no bone marrow biopsy is performed on a patient diagnosed with chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) which was based on the results of an axillary biopsy, positive peripheral blood and a CT scan showing multiple lymph nodes involved above and below the diaphragm? See Discussion | The physician staged this as Stage 0 CLL/SLL. Should the primary site be coded to lymph nodes if the MD stated this was leukemia? | For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Code the primary site to C421 [bone marrow] per Rule PH5. Code the primary site to the bone marrow when the peripheral blood is involved, even if no bone marrow biopsy is performed.
According to the notes for Rule PH5, CLL always has peripheral blood involvement (PH5 Note 1). CLL/SLL may also have involvement of lymph node regions in later stages (PH5, Note 2). For this patient a bone marrow biopsy was not performed but he had extensive lymph node and peripheral blood involvement. Therefore, the primary site is coded to C421. In addition, the physician's documentation specifies this patient has Stage 0 disease which indicates this disease process is being classified as leukemia (CLL).
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. |
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20130136 | Multiple primaries--Heme & Lymphoid Neoplasms: If a neoplasm is listed under the Transformations section in the Heme DB, is this always a new primary? See Discussion. | Where are the instructions for coding transformations? When a disease is listed under the transformations, the Multiple Primaries Calculator states it is a new primary. Is this a new primary when the physician calls it a transformation?
For example, patient was diagnosed in 2000 with chronic lymphocytic leukemia (CLL). A biopsy of a stomach mass on 4/26/12 was positive for diffuse large B-cell lymphoma. DLBCL is listed under the Transformations To section in the Heme DB for CLL. Is this a new primary because it is a transformation? |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Transformations do not always indicate a multiple primary is to be reported. Always apply the M Rules to determine the number of primaries. Refer to Rules M8-M13 in the Heme Manual address to determine the number of reportable primaries when chronic and acute neoplasms (transformations) are indicated on a case. Do not use the MP Calculator to determine the number of primaries unless the M Rules direct you to use it.
This case should be accessioned as two primaries, chronic lymphocytic leukemia [9823/3] diagnosed in 2000, and diffuse large B-cell lymphoma [9680/3] diagnosed on 04/26/2012 per Rule M10. Abstract a new primary when a neoplasm is originally diagnosed as a chronic (less aggressive) neoplasm (CLL) and there is a second diagnosis of an acute neoplasm (DLBCL) more than 21 days later.
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. |
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20130165 | MP/H Rules/Multiple primaries--Thyroid: How many primaries are reported and what is histology for the papillary carcinomas if a Classical cytomorphology with a follicular architecture is on the right and a Columnar cell cytomorphology with a follicular and papillary architecture is on the left? See Discussion. |
The answer seems to hinge on whether or not the two tumors differ at the third digit of histology. Can we code the histology based on the terms listed for variant or architecture? |
This is a single thyroid primary. The tumors are both papillary carcinoma with follicular architecture for the most part. Apply Rule M6 and abstract a single primary. | 2013 |
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20130073 | Reportability--Brain and CNS: Is Rosai-Dorfman disease a neoplastic reportable disease process if it occurs in the brain? See Discussion. |
The pathology report diagnosis is: Cranium, right temporal area, resection of intradural, extra-axial mass: Severe acute and chronic inflammation, histiocytic reaction, and proliferative fibrosis. See comment. Comment: Among potential alternative considerations are an infectious process, or non-infectious inflammatory CNS lesions such as inflammatory pseudotumor, Rosai-Dorfman disease, plasma cell granuloma, idiopathic hypertrophic pachymeningitis, and inflammatory myofibroblastic tumor. The clinicians discuss this and review other chart information and conclude the patient has a clinical diagnosis of Rosai-Dorfman disease. This is a rare disorder characterized by proliferation of histiocytes. |
This case is not reportable. Rosai-Dorfman disease is not listed in the ICD-O-3. To be reportable, a neoplasm must be listed in the ICD-O-3 and originate in a reportable brain/CNS site. |
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20130192 | MP/H Rules/Histology--Pleura: How is histology coded when the pathology report final diagnosis is "malignant neoplasm, compatible with malignant mesothelioma" if the COMMENT section of the pathology report indicates the tumor has a mixed epithelial and sarcomatoid pattern? See Discussion. | This case was discussed with a pathologist who feels the correct histology should be biphasic mesothelioma (9053/3) because there are both epithelial and sarcomatoid components to this tumor. However, applying the current MP/H Rules, the histology is coded to 9050/3 (mesothelioma, NOS) because the term "pattern" cannot be used to code a more specific histologic type for invasive tumors. If this truly is a biphasic mesothelioma, that data is lost for researchers because the current MP/H Rules fail to capture this information. Should the term pattern be used to code the more specific histology in this case? | Code the histology to malignant mesothelioma, NOS [9050/3]. Apply the MP/H Rules as written until they are revised. The word "pattern" and other terms will be reconsidered for the next iteration of the rules. | 2013 |
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