| Report | Question ID | Question | Discussion | Answer | Cancer Site Category | Data Item Category | Year |
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20260017 | Multiple Primaries/Histology--Heme & Lymphoid Neoplasms: Should histology be coded as 9895 when the pathologist's diagnosis on the bone marrow biopsy is "consistent with transformation of the patient's known polycythemia vera to acute myeloid leukemia?" See Discussion. |
The patient has a known history of polycythemia vera (PV). The Hematopoietic and Lymphoid Neoplasms Database (Heme DB) entry for myelodysplasia-related acute myeloid leukemia ("acute myeloid leukemia post myelodysplastic-myeloproliferative neoplasm") states, "The pathologist must make the diagnosis of being myelodysplasia-related." However, it's unclear if the pathologist's statement of an acute myeloid leukemia (AML) "transforming from" counts as the AML being myelodysplasia-related since AML can arise/transform from PV. The Heme DB does indicate histology 9895 should not automatically be used just because the patient has a history of MPN. The bone marrow did not identify any of the specific cytogenetic or molecular abnormalities listed in the Heme DB. However, next generation sequencing identified mutations in BCOR, DNMT3A, IDH1, JAK2, EZH2. The managing physician's clinical diagnosis was "AML arising from polycythemia vera." |
Assign a second primary as Acute Myeloid Leukemia, NOS (9861/3), per Rule M10, the first being polycythemia vera (9950/3). The physician states “consistent with transformation of the patient's known polycythemia vera to acute myeloid leukemia.” This is not the same thing as “Acute myeloid leukemia with myelodysplasia-related changes.” The Heme DB states: Do not automatically assign this histology (Acute myeloid leukemia with myelodysplasia-related changes (9895/3) if patient has a history of a myeloproliferative neoplasm (Myelodysplastic Syndrome [MDS], or Myeloproliferative Neoplasm [MPN]). Note: Polycythemia Vera is a Myeloproliferative Neoplasm (MPN). The pathologist must make the diagnosis of being myelodysplasia-related. |
Heme & Lymphoid Neoplasms | Histology | 2026 |
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20081121 | Multiple primaries/Histology--Lymphoma: How many primaries should be abstracted and how should the histology field(s) be coded in this situation? How would the bone marrow involvement by only NHL be handled? Composite lymphoma (9596) as defined by SEER and ICD-O is NHL and HD in one node which fits the final impression on the removed cervical node. See Discussion. |
Patient presented with cervical, supraclavicular & superior mediastinal lymphadenopathy. A cervical node was excised for pathological review. The final impression on that node was Composite lymphoma characterized by (1) Nodular Lymphocyte Predominant Hodgkin Lymphoma [HD] (2) CLL/SLL [NHL]. Then, a bone marrow aspirate/bx was performed revealing CLL/SLL [NHL]. | For cases diagnosed prior to 1/1/2010:This is a single primary. The histology code is 9596/3 [composite Hodgkin and non-Hodgkin lymphoma]. According to the Single Versus Subsequent Primaries of Lymphatic and Hematopoietic Diseases table, 9596/3 followed by 9670/3 is one primary. For cases diagnosed 1/1/10 and later, refer to the Hematopoietic and Lymphoid Neoplasm Case Reportability and Coding Manual and the Hematopoietic Database (Hematopoietic DB) provided by SEER on its website to research your question. If those resources do not adequately address your issue, submit a new question to SINQ. |
Lymphoma | Histology | 2008 |
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20210058 | Multiple Primaries/Histology--Lymphoma: What is the histology code and how many primaries are there based on a gastrohepatic lymph node biopsy that shows: Nodular lymphocyte-predominant Hodgkin lymphoma with T-cell/histiocyte rich diffuse large B-cell lymphoma (DLBCL)-like transformation. If two primaries, what is the diagnosis date for each primary? See Discussion. |
4/28/21 PET: There is extensive widespread/multifocal hypermetabolic uptake within lymph nodes, skeleton, and spleen, compatible with malignancy. Differential diagnosis includes lymphoma and metastatic disease of indeterminate primary, with lymphoma favored. 4/28/21 Right retroperitoneal lymph node, needle core biopsy: Large B-cell lymphoma. See comment. Comment: The differential includes T-cell/histiocyte-rich large B-cell lymphoma and diffuse variant of nodular lymphocyte predominant Hodgkin lymphoma. It is challenging to distinguish these two on the needle core biopsy. An excisional biopsy is recommended for a definite diagnosis if clinically appropriate. ADDENDUM: B-Cell Lymphoma, FISH: negative. No rearrangement of MYC, BCL2 and BCL6 and no fusion of MYC and IGH. 5/14/21 Gastrohepatic lymph node, biopsy: Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) with T-cell/histiocyte rich diffuse large B-cell lymphoma-like transformation. Focal in situ follicular neoplasia. 6/3/21 Medical Oncologist: Biopsy confirms that patient has a nodular lymphocytic Hodgkin lymphoma which has transformed into a T-cell rich DLBCL. This variant of Hodgkin disease is a good prognostic histology which generally behaves indolently, like a low grade lymphoma. |
We consulted with our expert hematopathologist who advised this is a single primary, Hodgkin lymphoma (9659/3). The diagnosis from 5/14/2021 states NLPHL. It also states there is T-cell histiocyte rich large B-cell lymphoma-like transformation. The WHO Classification of Hematopoietic and Lymphoid Tissues demonstrates six different patterns to NLPHL, which are: A) 'classical' nodular, B) serpiginous/interconnected nodular, C) nodular with prominent extra-nodular LP cells, D) T-cell-rich nodular, E) diffuse with a T-cell-rich background, and F) diffuse, B-cell-rich pattern. In this case, they are describing a NLPHL type E (diffuse with a T-cell rich background). The term used is "T-cell histiocyte rich large B-cell lymphoma-LIKE transformation. "Like" as used here means that it is like a transformation; if it was NLPHL transforming to T-cell histiocyte rich large B-cell lymphoma, it would not have the word "like" in the diagnosis. This is a variant of NLPHL and not an actual transformation to another lymphoma. Even though NLPHL can transform to T-cell histiocyte rich large B-cell lymphoma, it is not the case here since the word "like" appears in the diagnosis. |
Lymphoma | Histology | 2021 |
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20130100 | Multiple primaries/Primary site--Heme & Lymphoid Neoplasms: How many primaries are there and how should I code the primary site(s)? See discussion. |
Patient had a hemicolectomy and a salpingo-oophorectomy and was found to have diffuse large B cell lymphoma in the colon (10 cm cecal mass), 3/16 regional lymph nodes involved with lymphoma. Fallopian tube showed involvement with diffuse large B Cell lymphoma.
Multiple primaries - Colon and fallopian tube?
One primary - Colon? Stage IV, or lymphoma from an unknown primary? Note: There were no other lymph nodes involved. |
Use Rule M2. Abstract as a single primary when there is a single histology.
When you have questions about how to code the primary site, start with the abstractor notes. If the answer isn't found there go to Module 7 (a specific module to help code primary site for lymphomas).
The abstractor notes for DLBCL in this case do not provide information you can use for this case. Go to Module 7 in the PH rules.
Use Rule PH25 Code the primary site to the organ when lymphoma is present in an organ and that organ’s regional lymph nodes. Code the primary site to colon (organ and regional lymph nodes involved). The fallopian tube is secondary involvement. As is common with lymphomas, there can be more than one organ involved. You can differentiate the primary site from the secondary site(s) because of the large colon mass with regional lymph node involvement. |
Heme & Lymphoid Neoplasms | Primary site | 2013 |
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20091002 | Multiplicity Counter--Ovary: Given the diffuse nature of ovarian cancer, should we count bilateral parenchymal involvment of ovaries as two tumors? See Discussion. |
Are peritoneal implants mets and not counted as separate tumors, even though they're not stated to be metastatic in the path report, and are not coded as distant mets? |
Code Multiplicity Counter to 02 [Two tumors present] for an epithelial ovarian primary involving both ovaries. Do not count the peritoneal implants; they are regional metastasis and not included in the multiplicity counter. An example like this will be added to the manual in the next revision. |
Ovary | N/A | 2009 |
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20160045 | Neoadjuvant treatment/Grade--Prostate: How should the grade/differentiation field be coded when hormone therapy is given prior to radiation for metastatic prostate cancer? Is hormone treatment "neoadjuvant treatment" in this situation? Per NCCN guidelines, neoadjuvant hormone therapy is strongly discouraged outside of a clinical trial for localized disease. However for metastatic disease, hormone is recommended (gold standard). See discussion. |
8/1/15 CT Exam showed enlarged prostate and left seminal vesicle with multiple enlarged pelvic LNs. Findings: suspicious for prostate cancer with invasion of seminal vesicle. Bone scan findings: positive bone mets in multiple sites. PSA 169.0 (elevated). Patient was started on casodex 8/12/15. A prostate biopsy was performed on 9/16/15 to confirm diagnosis, adenocarcinoma Gleason 4+5. Patient's treatment continued with radiation to bone. |
For cases diagnosed prior to 2018 Code the grade/differentiation field from the biopsy for this situation. According to experts consulted, hormone therapy does not alter the grade in this case and grade should be coded based on information after hormone therapy when that is the only grade information available. |
Prostate | N/A | 2016 |
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20150035 | Primary site--Anus/Anal Canal: What site do you code squamous cell carcinoma of the anal verge? |
Assign C211 for anal verge. Anal verge is defined as the lower (distal) end of the anal canal, junction between the skin of the anal canal and the perianal skin, http://www.seer.cancer.gov/manuals/2015/AppendixC/rectosigmoid/coding_guidelines.pdf |
N/A | Primary site | 2015 | |
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20190005 | Primary Site--Bladder: Does instruction #4 in the Urinary Sites Solid Tumor Rules Instructions for Coding Primary Site apply to a mix of in situ and invasive urothelial tumors? Instruction #4: Code Urinary System NOS C689 when there are multiple non-contiguous tumors in multiple organs within the urinary system. See Discussion. |
Example: Patient has multiple biopsies with final diagnosis of in situ papillary urothelial carcinoma in the prostatic urethra and invasive papillary urothelial carcinoma in the bladder. How should primary site be coded in this type of mixed in situ and invasive situation? |
Code Urinary System NOS C689 for this case since there are two separate urinary sites involved. Apply instruction #4 when there is a mix of in situ and invasive urothelial tumors. |
Bladder | Primary site | 2019 |
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20140059 | Primary site--Bladder: What is the primary site for bladder tumor biopsy: invasive adenocarcinoma, enteric type favor urachal origin, stage III |
Based on the information provided, code the primary site to urachus (C677). Primary adenocarcinoma of the bladder accounts for less than 1% of all bladder malignancies. Of these, 20–39% are urachal in origin. |
Bladder | Primary site | 2014 | |
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20110127 | Primary Site--Brain and CNS: Are meninges surrounding cranial nerves cranial meninges [C700] or a part of the specific nerve's sheath? Is the primary site for an optic nerve sheath meningioma coded to optic nerve [C723] or cranial meninges [C700]? |
Code the primary site to cranial meninges [C700]. |
Brain and CNS | Primary site | 2011 |
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