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Report Produced: 02/21/2026 06:26 AM

Report Question ID Question Discussion Answer Year
20110132 Reportability/Histology--Heme & Lymphoid Neoplasms: Is a diagnosis of "small B-cell non-Hodgkin lymphoproliferative disorder" reportable? If so, how is the histology to be coded? See Discussion. The final diagnosis of a bone marrow biopsy dated 10/99/2010 was "small B-cell non-Hodgkin lymphoproliferative disorder." The differential diagnosis includes atypical small lymphocytic lymphoma/chronic lymphocytic leukemia and marginal zone lymphoma. Mantle cell lymphoma is very unlikely based on BCL1 negativity. Lymphoplasmacytic lymphoma is also excluded due to the absence of a plasma cell component (CD138 negative).

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

Yes. The term "small B-cell non-Hodgkin lymphoproliferative disorder" is reportable. Code the histology to 9591/3 [non-Hodgkin lymphoma, NOS] per Rule PH28. When there is a diagnosis of lymphoproliferative disorder and any lymphoma, code the lymphoma histology.

The information in the discussion is reflective of the difficulty in diagnosing hematopoietic and lymphoid neoplasms. The differential diagnosis indicates that a number of possible specific lymphoma/leukemia diagnoses that have been ruled out, which explains why the final diagnosis is non-Hodgkin, NOS.

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.

2011
20110031 Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned if patient initially diagnosed with granulocytic sarcoma on a vocal cord biopsy is subsequently diagnosed with acute myeloid leukemia more than 21 days later? See Discussion.

The patient has a history of refractory anemia with excess blasts diagnosed in 2008. A vocal cord biopsy performed on 6/2/2010 stated, "in view of a previous history of myelodysplastic syndrome this is indicative of transformation to acute leukemia" and consistent with granulocytic sarcoma. A bone marrow biopsy done on 7/19/2010 stated this was compatible with refractory anemia with excess blasts in transformation.

Granulocytic sarcoma is a solid manifestation of AML. When these diagnoses occur more than 21 days apart, are they separate primaries?

According to the WHO definition, this is acute myeloid leukemia complicating myelodysplasia. Which rule applies for this case?

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

This case should be accessioned as two primaries. The first is refractory anemia with excess blasts in 2008, and the second is AML June 2, 2010.

As for the disease occurring in 2010, granulocytic sarcoma does not transform into AML. Per the Abstractor Notes section in the Heme DB under the term "granulocytic sarcoma," it indicates that "Myeloid sarcoma (also known as granulocytic sarcoma) may occur de novo; it may precede or coincide with AML, or represent an acute blastic transformation of myelodysplastic syndromes." This means that when granulocytic/myeloid sarcoma is seen with AML, it represents a solid manifestation of the systemically involved AML. In other words, it is all the same disease process (coded to AML) if it occurs simultaneously.

In this case, when the physician gave a provisional diagnosis of "transformation to acute leukemia" it indicated he saw the solid deposits of myeloid cells on the vocal cord. Per Rule M3, AML and myeloid (granulocytic) sarcoma appearing simultaneously are a single primary coded to AML. When the patient has AML, solid myeloid deposits (myeloid sarcoma) may appear. This is a manifestation of the AML rather than a new primary. Rule PH10 states to code the histology to AML.

Under the Transformation section in the Heme DB for refractory anemia with excess blasts (a chronic neoplasm), it indicates this disease process does transform to acute myeloid leukemia, NOS (an acute neoplasm). In this case, the chronic and acute disease processes were diagnosed at different times. Per Rule M10, abstract as multiple primaries when a neoplasm is originally diagnosed in a chronic (less aggressive) phase AND second diagnosis of a blast or acute phase more than 21 days after the chronic diagnosis.

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.

2011
20110059

Histology: How do you code histology for "malignant myopericytoma"?

Report malignant myopericytoma as 8824/3 for cases diagnosed 2021 and later.

 2011
20110048 First course treatment--Heme & Lymphoid Neoplasms: How is a "donor lymphocyte infusion" that is used in the treatment of CLL coded?

Donor lymphocyte infusion (DLI) is coded as immunotherapy. The lymphocytes are donated by the same person who donated the original stem cell transplant. The lymphocyte infusion creates an immune response in which the T-cells are activated to attack the cancer cells.

See "Treatments" for CLL/SLL (9823/3)

 2011
20110008 MP/H Rules/Histology--Vulva: How is histology coded for VIN III with focal invasion? See Discussion. Per SINQ 20000442, the histology for CIN III with microinvasion is coded to 8077 [squamous intraepithelial neoplasia, grade III] per the matrix system rules, with a behavior code of /3 [malignant]. Coding the histology to 8077/3 per the matrix principle causes IF25_3 and MorphICDO3_P1 edits to fail. Flagging the first error resolves any reporting issue. How is the MorphICDO3_P1 edit resolved? Assign 8076/3 [squamous cell carcinoma, microinvasive] for VIN III with focal invasion. This applies to all terminologies listed under 8077/2. The SINQ question from 2000 will be retired. 2011
20110007 MP/H Rules/Multiple primaries--Bladder: How many primaries are to be abstracted and how are the histologies coded when a bladder resection demonstrates tumor with invasive small cell neuroendocrine carcinoma [8041/3], high grade papillary urothelial carcinoma in situ [8130/2], adenocarcinoma in situ [8140/2], and multifocal flat urothelial carcinoma in situ? See Discussion.

Are the areas of in situ tumor to be ignored or would MP/H Rule M9 apply?

Ignore the in situ histologies. This is a single primary. Code the histology to invasive small cell neuroendocrine carcinoma [8041/3]. 2011
20110079 MP/H Rules/Histology: In the MP/H Manual, where is the documentation indicating "focal" is not a term that can be used to code histology? See Discussion. Example: neuroendocrine carcinoma with focal squamous differentiation. For the purposes of the MP/H rules, the term "focal" is not used to indicate a more specific histology. Terms that may be used to indicate a more specific histology are listed in the relevant histology rules. For example, see Breast histology rule H3. Notice the terms listed in the note for this rule are "type, subtype, predominantly, with features of, major, with ___ differentiation, architecture or pattern." The term "focal" is not included. This concept will be clarified in future revisions to MP/H rules. 2011
20110042 MP/H Rules/Histology--Testis: How is histology coded when the initial biopsies of retroperitoneal mass demonstrated non-seminomatous germ cell tumor, but after neoadjuvant chemotherapy the final diagnosis on the radical orchiectomy specimen demonstrated mature teratoma, NOS (not stated to be malignant)? See Discussion. A large retroperitoneal mass was found on CT scan. A biopsy demonstrated non-seminomatous germ cell tumor. The biopsy was done at an outside facility. Neither the CT scan nor biopsy pathology report is available for review. Following neoadjuvant chemotherapy, the retroperitoneal mass decreased to 12 cm. Subsequently, the patient had a right radical orchiectomy. The final diagnosis per the pathology reports was a 3.5 cm mature teratoma (NOS, not stated to be "malignant") of right testicle. The patient then had resection of the retroperitoneal mass and biopsies. Pathology showed the "excision" specimen contained 6 benign lymph nodes and two of the "biopsy" specimens showed non-seminomatous germ cell neoplasm with IHC findings suggestive of a mix of embryonal carcinoma and a lesser component of yolk sac tumor.

This is a reportable case. Even though the pathology from the orchiectomy stated mature teratoma, NOS, the presence of lymph node metastases proves that this tumor is malignant. Code the histology as 9065/3 [germ cell tumor non-seminomatous].

The majority of germ cell tumors show the presence of multiple histologies. While the original tumor showed only mature teratoma, there were obviously yolk sac cells that were not detected on the sections taken from the primary tumor. Both teratoma and yolk sac are germ cell tumors. This explains why the pathologist gave you the diagnosis of germ cell tumor. The classification of "non-seminomatous" simply means that there was no seminomas present in the mixture of germ cell histologies.

2011
20110017

Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are reported if a patient originally diagnosed with CLL is subsequently diagnosed several months later on a bone marrow biopsy with Richter's syndrome that transformed into a large cell lymphoma? See Discussion.

Per reviewed resources, the described condition is rare. Should the histology remain CLL or be changed to large cell lymphoma?

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

This case is accessioned as two primaries per Rule M10 which states to abstract multiple primaries when a neoplasm is originally diagnosed as a chronic neoplasm and there is a second diagnosis of an acute neoplasm more than 21 days after the chronic diagnosis. The first primary is CLL [9823/3] and it is a chronic neoplasm. The second primary is diffuse large B-cell lymphoma (DLBCL) [9680/3] and it is an acute neoplasm.

Richter syndrome (RS) is a complication of B cell chronic lymphocytic leukemia (CLL) or hairy cell leukemia (HCL) in which the leukemia changes into DLBCL. There is also a less common variant in which the CLL changes into a Hodgkin lymphoma. Richter's transformation affects about 5% of CLL patients. Richter syndrome is listed under the Alternate Names section in the Heme DB for diffuse large B-cell lymphoma [9680/3].

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.

 2011
20110095 Reportability/Histology: Is the diagnosis "low-grade neuroendocrine neoplasm/carcinoid tumor with expression of gastrin (consistent with gastrinoma)" reportable with the histology code 8240/3 [carcinoid] or 8153/3 [malignant gastrinoma]? See Discussion. A carcinoid tumor (8240/3) is reportable but a gastrinoma, NOS (8153/1) is not.

Code histology to 8153/3 [malignant gastrinoma].

According to the WHO Classification of Tumors of the Digestive System, pages 64-65, carcinoid is a synonym for gastric neuroendocrine tumor (NET) and gastrinoma is synonymous with gastrin-producing NET. Gastrin-producing NET (gastrinoma) is coded 8153/3.

2011