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20130088 | Grade--Heme & Lymphoid Neoplasms: Should Grade be coded to 5 [T-cell] or 9 [cell type not determined, not stated, not applicable] for anaplastic large cell lymphoma, NOS [9714/3]? See Discussion. | Under the Grade section in the Heme DB for anaplastic large cell lymphoma, NOS it indicates the following:
"Grade - Code grade specified by pathologist. If no grade specified, code 9."
There is no reference in the Grade section that we should look at the Abstractor Notes or a specific Module in the Heme DB for additional information. However, in the Abstractor Notes section it states, "Grade is T-cell (5) unless pathologist specifically designates as a B-cell (see G2 rule)." These two statements are conflicting. Which is the correct grade? |
Assign code 5 [T-cell] for anaplastic large cell lymphoma [9714/3] unless the pathologist specifies that the histology is a B-cell disease process. See Grade rule G2, Note 2.
In the Heme DB, there is a default value in the Grade field for histologies that do not have a grade specified. However, this particular histology does not default to code 9. There was an error in the Grade section of the 2010 and 2012 versions of Heme DB that has now been corrected in the latest release. |
2013 |
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20130035 | Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned and what rule applies when a subsequent diagnosis of diffuse large B-cell lymphoma (95%) and follicular lymphoma, grade 3 (5%) is made following an original diagnosis of low grade CD-10 positive B-cell lymphoma, most consistent with low grade follicular lymphoma (FL) ? See Discussion. |
In 2011, patient presented with a large mesenteric mass, numerous other smaller mesenteric lymph nodes, moderate retroperitoneal and extensive iliac chain adenopathy greater on right; small inguinal nodes are also present mostly on right side and splenomegaly per the CT scan. Abdominal pelvic mass needle biopsies showed low grade CD-10 positive B-cell lymphoma, most consistent with low grade follicular lymphoma (FL). The patient was treated with R-CVP with unknown response. In June 2012, patient presented again for laparoscopy and lymph node biopsy for stated recurrence of lymphoma found on CT scan. A large mass was seen in mesentery of bowel. Abdominal mass biopsy showed diffuse large B-cell lymphoma (DLBCL). Abdominal mass #2 excisional biopsy showed diffuse large B-cell lymphoma, 95%, and follicular lymphoma grade 3, 5%. The majority of the tumor is now DLBCL. |
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, diffuse large B-cell lymphoma diagnosed in 2011 per Rule M7. Note 4 for Rule M7 states to change the histology code on the original abstract to the more specific histology, diffuse large B-cell lymphoma in this case. There is no time restriction for rule M7. Apply rule PH11 and code the histology as 9680/3 [DLBCL] when both DLBCL and follicular lymphoma are present in the same lymph node(s). Ambiguous terminology is not used to code a more specific histologic type per the Heme Manual. The information submitted states only that this low grade B-cell lymphoma was "most consistent with follicular lymphoma." The term "consistent with" is an ambiguous term per SEER and cannot be used to code the histology of the 2011 neoplasm as follicular lymphoma. There was no subsequent clinical statement that this patient was diagnosed with follicular lymphoma in 2011. 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. Although the ambiguous terminology on the pathology report is not used to code the histology to follicular lymphoma, had there been a subsequent clinical statement that this patient had follicular lymphoma, the histology would be coded to follicular lymphoma [9690/3]. A diagnosis of follicular lymphoma followed by a diagnosis of DLBCL more than 21 days later is a new primary per rule M12. |
2013 |
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20130001 | Reportability--Brain and CNS: Are hemangioma, NOS (9120/0), cavernous hemangioma (9121/0) or venous hemangioma (9122/0) reportable when they arise in the brain or CNS?
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Hemangioma, NOS (9120/0) and cavernous hemangioma (9121/0) arising in the dura and parenchyma of the brain/CNS are reportable.
Venous angiomas (9122/0) are not reportable wherever they arise. The primary site for venous hemangioma arising in the brain is blood vessel (C490). The combination of 9122/0 and C490 is not reportable. This is a venous abnormality. Previously called venous angiomas, these are currently referred to as a developmental venous anomalies (DVA). |
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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20130203 | MP/H Rules/Multiple primaries--Brain and CNS: How many primaries are accessioned for a diagnosis of cerebral cavernous malformation disorder (CCM1) and MRI evidence of dozens of cavernous angiomas/malformations throughout the supra and infratentorium? See Discussion. | 9/9/11 IMP: Presymptomatic cerebral cavernous malformation disorder (CCM1).
9/9/11 Brain MRI: FINDINGS: Total of 14 foci. 2 largest in rt frontal lobe. In rt frontal lobe, total of 4 foci. Of remaining 10 small foci, 4 are in cerebellum, 1 in rightward pons, 1 in lt temporal lobe, 1 in lt occipital lobe, 1 in rt occipital lobe, 1 in posterior rt temporal lobe, & 1 in lt frontal lobe. Lesions in bilateral occipital lobes & lt temporal lobe are associated w/weighted signal suggestive of hemosiderin & are most c/w additional cavernous malformations. IMPRESSION: Just over a dozen scattered foci of gradient susceptibility throughout supra & infratentorium.
9/13/13 Brain MRI. Clinical diagnosis: Cerebral cavernous angiomas. FINDINGS: Approximately a dozen scattered foci. 2 largest in rt frontal lobe. Remaining small foci identified w/in cerebellum, rightward pons, rt occipital lobe, rt temporal lobe, & lt frontal lobe. Many are less conspicuous than in 2011 & a few that were present on prior study are not evident on current exam. This is likely due to differences in technique. IMPRESSION: Redemonstration of numerous scattered foci c/w cavernous malformations. |
This case is not reportable as is. The clinical diagnosis on the 9/13/13 MRI was "cerebral cavernous angiomas," but the final impression on the MRI was a re-demonstration of the numerous scattered foci consistent with cavernous malformations seen on the previous 9/9/11 MRI. There was no reportable statement of cavernous angioma. Cavernous malformation is not a reportable neoplasm; it has no valid ICD-O-3 code.
Vascular tumors of the CNS are reportable when they arise in the dura or parenchyma of the CNS. When they arise in blood vessels or bone, they are not reportable. Do not report vascular tumors when there is not enough information to determine whether they arise in the dura or parenchyma or elsewhere. |
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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20130055 | Primary site--Heme & Lymphoid Neoplasms: How is the primary site coded for a lymphoma with multifocal bone and epidural involvement but no lymph node involvement if the physician does not clearly state the primary site? See Discussion. | MRI Lumbar spine: Bony metastatic disease most evident at L5, L3 and T10. There is marrow tumor in the posterior elements of T12 and T10. The 14 mm epidural mass represents epidural tumor, likely metastatic, extending into the left intervertebral foramen at T12-L1.
PET scan: Hypermetabolic activity corresponding to epidural mass at the level of T12 and L1 concerning for malignancy. Other small areas of hypermetabolic activity in the left mandible and both femoral necks. There is no hypermetabolic activity corresponding to the areas of abnormal marrow edema in the vertebral bodies which enhanced on MRI scan in the lumbar and lower thoracic spine. No lymph nodes mentioned.
Biopsy epidural mass: Diffuse large B-cell lymphoma with a background of follicular lymphoma, consistent with a large cell transformation. Flow cytometry confirms a mixed large and small cell population of lymphoma (55% large cells).
T12/L1 Bone Biopsy: Bone and marrow with atypical paratrabecular lymphoid infiltrates, suspicious for involvement by follicular lymphoma. Negative for large cell lymphoma. |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Code the primary site of the diffuse large B-cell lymphoma [9680/3] to C809 [unknown primary site] per Rule PH27. The patient has involvement of multiple bones and an epidural mass with no evidence of nodal involvement. Code the primary site to unknown [C809] when multiple organs are involved without any lymph node involvement, even when there is no statement from the physician regarding primary site.
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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20130134 | Reportability--Heme & Lymphoid Neoplasms: According to the hematopoietic database, systemic mastocytosis is reportable; does that include INDOLENT systemic mastocytosis (which is not listed in the list of alternative names)? |
For cases diagnosed 2018 and forward, indolent systemic mastocytosis is not reportable (9741/1). Smoldering systemic mastocytosis is reportable (9741/3). |
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20130167 | Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are reported if a 2013 diagnosis of right leg skin nodules, consistent with plasmacytoma/plasma cell myeloma, follows a 3/20/07 biopsy diagnosis of multiple myeloma? |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph. Abstract this case as a single primary. Code the histology to 9732/2 [multiple myeloma]. Review the Abstractor Notes section in the Heme DB for multiple myeloma. It states that in multiple myeloma there is generalized bone marrow involvement and that extramedullary involvement is diagnostic of advanced disease. This is a case of advanced multiple myeloma. |
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20130111 | Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned if a 2008 diagnosis of extralymphatic follicular lymphoma in the breast is subsequently diagnosed in 2011 with ocular follicular lymphoma? See Discussion. | The patient was diagnosed with follicular lymphoma in the breast in 2008. Per notes, there was no evidence of disease again until 2011 when the patient presented with ocular lymphoma. The physician stated this was part of the same disease process as the prior breast diagnosis. The bone marrow was not involved in either case.
Is this a single primary (recurrence) of follicular lymphoma? Or are these multiple primaries because they arise in different extralymphatic sites? |
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Accession a single primary, follicular lymphoma [9690/3] of the breast diagnosed in 2008 per Rule M2.
Accession a single primary when there is a single histology. This is a recurrence of the patient's 2008 follicular lymphoma.
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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