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Report Produced: 03/01/2026 16:49 PM

Report Question ID Question Discussion Answer Year
20110023 Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are reported in a patient with a November 2009 diagnosis of refractory anemia and a 10/25/2010 biopsy diagnosis of refractory anemia with excess blasts type 2 with ringed sideroblasts that the clinician indicates actually demonstrates progression to AML? See Discussion.

Refractory anemia, NOS diagnosed in November 2009. The diagnosis on a bone marrow biopsy performed on 10/25/10 is myelodysplastic syndrome - refractory anemia with excess blasts type 2 with ringed sideroblasts. Per the medical oncologist, in the 12/16/10 clinic note it states, "Pt underwent bone marrow biopsy on 10/25/10 and ultimately this marrow demonstrates progression to AML.

When applying the Hematopoietic Rules, the refractory anemia, NOS and the myelodysplastic syndrome - refractory anemia with excess blasts type 2 with ringed sideroblasts is the same primary. However, the refractory anemia NOS and the AML are multiple primaries.

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

First, note that myelodysplastic syndrome (MDS) is a term that includes a number of diseases. Refractory anemia, NOS and refractory anemia with ringed sideroblasts are types of MDS. These two diseases are an NOS and a more specific disease, which is accessioned as one primary per Rule M7.

Next, assess the change from refractory anemia to AML. In checking the Heme DB, AML is listed under transformations for refractory anemia with ringed sideroblasts. This patient has a chronic disease (refractory anemia with ringed sideroblasts) and an acute disease (AML). 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
20110103 MP/H Rules/Histology/Ambiguous terminology: Can synonyms of listed terms, such as "variety" for the list termed "type," be used to code a more specific histology? See Discussion. The list of terms denoting a more specific histology does not include "variety." During MP/H training sessions there was an emphasis placed on only using terms listed to code a more specific histology. However, the results of an audit indicated that because "variety" is a synonym for "type" it could be used to code a more specific histology. Are synonyms of listed terms to be used to code histology? No. Synonyms of listed words used in the MP/H rules (e.g., "variety" for the listed term "type") cannot be used to designate a more specific histology. 2011
20110091 MP/H Rules/Histology--Bladder: How is this field coded for a patient with ureter specimen with "high grade urothelial carcinoma with adenocarcinoma differentiation" and a TURB specimen with "urothelial ca, high grade, a biphasic pattern with cautery-distorted urothelial carcinoma and adenocarcinoma"?

According to the MP/H rules, code histology to 8120/3 [urothelial carcinoma] for cases diagnosed 2007 or later. The term "glandular differentiation" is equivalent to adenocarcinoma differentiation. 8120/3 [urothelial carcinoma] would be the best way to code a "biphasic pattern with cautery-distorted urothelial carcinoma and adenocarcinoma" according to a pathologist consultant.

The steps used to arrive at this decision are as follows:

Go to the Urinary Histo rules found in the Multiple Primary and Histology Coding Rules Manual.

Start at the MULTIPLE TUMORS ABSTRACTED AS A SINGLE PRIMARY module, rule H9. Code the histology to 8120 [transitional cell/urothelial carcinoma] when there is transitional cell carcinoma with glandular differentiation.

2011
20110004 MP/H Rules/Histology--Breast: Which MP/H rule applies when coding the histology field for a tumor described as a "metaplastic carcinoma, adenosquamous and spindle cell type"? See Discussion.

Per path comment: "The neoplasm is composed of adenosquamous carcinoma which merges with spindle cell carcinoma. The cystic component shows a mixed squamous and ductal epithelial lining which shows cytologic atypia and mitotic activity and can be seen to merge with invasive carcinoma. The features suggest the possibility that the tumor may have arisen from a sclerosing and cystic papilloma with squamous metaplasia, although a clearly benign component is not evident."

Would MP/H rule H19 apply based on the pathology report comment resulting in histology for the case being coded to 8255 [adenocarcinoma with mixed subtypes]? Or, would MP/H rule H14 apply based on the final diagnosis resulting in histology for the case being coded to 8575 [metaplastic carcinoma] because adenosquamous and spindle cell are not specific types of metaplastic carcinoma?

This is a metaplastic carcinoma as stated in the path diagnosis. Rule H14 applies. Assign code 8575/3. According to the WHO Classification, metaplastic carcinoma is a general term for a group of neoplasms characterized by a mixture of adenocarcinoma with dominant areas of spindle cell, squamous, and/or mesenchymal differentiation.

Use the Multiple Primary and Histology Coding Rules Manual for cases diagnosed 2007 or later to determine the histology for this case. Code histology to 8575/3 [metaplastic carcinoma] as stated in the pathology diagnosis.

Open the Multiple Primary and Histology Coding Rules manual. Choose one of the three formats (i.e., flowchart, matrix or text) under the Breast Histo rules determine histology for the case.

Go to the SINGLE TUMOR: INVASIVE CARCINOMA ONLY module. The rules are intended to be reviewed in consecutive order within the module from Rule H10 to Rule H19. You stop at the first rule that applies to the case you are processing.

Code the histology when only one histologic type is identified. According to the WHO Classification, metaplastic carcinoma is a general term for a group of neoplasms characterized by a mixture of adenocarcinoma with dominant areas of spindle cell, squamous, and/or mesenchymal differentiation.

2011
20110105 Multiple primaries/Histology--Heme & Lymphoid Neoplasms: How many primaries should be reported for a bone marrow biopsy diagnosis of "lymphoproliferative disorder, small cell lymphocytic lymphoma/small cell lymphocytic leukemia consistent with marginal zone lymphoma"? According to our hematopoietic/lymphoid neoplasm physician expert, abstract one primary with the histology code 9699/3 [marginal zone lymphoma]. The pathologist is using the expression "small lymphocytic lymphoma" in a descriptive manner (marginal zone lymphoma is comprised of small lymphocytes) rather than in a "diagnostic" manner. 2011
20110150 Ambiguous Terminology--Heme & Lymphoid Neoplasms: As ambiguous terminology is not used to code histology for Heme & Lymphoid primaries, how is the histology coded when a patient has a clinical diagnosis of "consistent with a myelodysplastic syndrome"? See Discussion.

The physician states the "patient's clinical picture certainly is most consistent with MDS." Several FISH probes were performed on peripheral blood, specifically looking for the 5q minus syndrome as well as other molecular rearrangements to suggest or confirm MDS. These studies came back as normal. The initial bone marrow also came back negative. The physician then states, "The suspicion was that this represented a myelodysplastic syndrome despite the normal cytogenetics. Additional studies performed on the date of the clinic visit included the FISH for the 5q minus syndrome as well as CD59 to exclude PNH. Both of these were negative. Therefore, at this juncture, the patient has a macrocytic anemia not yet requiring transfusion support with a normal white count and an elevated platelet count and a hypercellular bone marrow. This is certainly consistent with a myelodysplastic syndrome."

Per coding guidelines, ambiguous terminology is not used to code histology, only for reportability. What is the histology code for this case?

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

Code the histology as Myelodysplastic syndrome, unclassifiable [9989/3].

Ambiguous terminology is used to accession cases (determine reportability). While ambiguous terminology is generally not used to code a specific histology, it can be used to code histology if it is the .

The statement that you do not use ambiguous terms to code histology is intended for those NOS histologies with an ambiguous term being used to describe the subtype. For example, if the physician states this is a myelodysplastic syndrome, NOS, refractory thrombocytopenia. The correct histology would be MDS, NOS [9989/3] and not refractory thrombocytopenia [9992/3].

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
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
20110068 MP/H Rules/Multiple primaries--Bladder: Which multiple primary rule is used to determine the number of primaries to accession when a patient has a papillary transitional cell carcinoma of the bladder diagnosed in 2009 followed by a high grade invasive urothelial carcinoma with neuroendocrine features per immunohistochemistry diagnosed in 2010? See Discussion.

A patient has papillary transitional cell carcinoma of the bladder in March of 2009. In June of 2010 the patient has another TURBT that demonstrates a flat in situ and invasive high grade urothelial carcinoma. The path addendum indicates, "Genzyme IHC show results consistent with high grade invasive urothelial carcinoma with neuroendocrine features." Two months later a liver biopsy shows poorly differentiated malignant tumor. The path addendum indicates, "Genzyme IHC results show metastatic poorly differentiated carcinoma with neuroendocrine features, favor bladder primary."

Is the latter a second bladder primary with histology code 8246/3 [neuroendocrine carcinoma]?

NOTE: Neuroendocrine is not listed as an urothelial tumor in Table 1 of MP/H Rules.

Use the Multiple Primary and Histology Coding Rules Manual for cases diagnosed 2007 or later to determine the number of primaries. This is a single primary. The 2010 diagnosis is urothelial carcinoma. The presence of "neuroendocrine features" does not change the histologic category.

The steps used to arrive at this decision are:

Open the Multiple Primary and Histology Coding Rules manual. Once in the manual, locate the Urinary MP rules under one of the three formats (i.e., flowchart, matrix or text). The rules are intended to be reviewed in consecutive order within the module. You stop at the first rule that applies to the case you are processing.

Start at the MULTIPLE TUMORS module start at rule M3.

. Bladder tumors with any combination of transitional cell carcinoma and papillary transitional carcinoma are a single primary.

2011
20110092

MP/H Rules/Multiple primaries--Breast: How many primaries are accessioned when a pathology specimen reveals one tumor with invasive mucinous carcinoma [8480/3] and a second tumor with in situ ductal carcinoma, solid and cribriform types [8523/2]?

For cases diagnosed 2007 or later, accession two primaries, invasive mucinous carcinoma [8480/3] and in situ ductal carcinoma, solid and cribriform types [8523/2].

The steps used to arrive at this decision are:

Go to the Breast MP rules found in the Multiple Primary and Histology Coding Rules Manual after determining the histology of each tumor (8480/3 and 8523/2).

Start at the MULTIPLE TUMORS module, rule M4. These tumors have ICD-O-3 histology codes that are different at the second (xxx) and third (xxx) number and are, therefore, multiple primaries.

 2011
20110044

MP/H Rules/Histology--Corpus uteri: What are the histologies for the primaries to be reported when the endometrium contains two separate tumors composed of adenocarcinoma with multiple differentiations as well as a separate small focus of clear cell carcinoma? See Discussion.

 

The resected specimen showed, "Adenocarcinoma of endometrium with the following features: Histologic type: Endometrioid with squamous and focal clear cell differentiation. A second focus of endometrial adenocarcinoma is present in the fundus with admixed complex atypical hyperplasia in a polypoid, non-invasive mass. The second tumor is endometrioid with secretory differentiation. COMMENT: The tissue in between the two tumors is sampled, and contains foci of endometrial adenocarcinoma that is superficially present within the endometrium, as well as a small focus of clear cell carcinoma measuring 0.2 cm."

Per MP/H rules M17, this is counted as multiple primaries because the histology codes differ at the third digit: 8323/3, 8382/3, 8310/3. The Multiple Primary rules make no reference to the histology tables. There is also no rule to ignore the in situ tumor. In addition, the histology table in the 2007 MP/H Rules Manual for Other Sites does not include "secretory differentiation" as a type of GYN malignancy.

After consultation with our expert pathologist, the decision is report this case as a single primary. There was some confusion about how to apply the current MP/H rules to this pathology report given 1) the definition of M16 and M17 and 2) the likelihood for a single endometrial primary to present with several differentiations. According to our expert pathologist, "I would regard this case as a single endometrial primary with extensive endometrial involvement and several types of differentiation, all of which are seen in endometrial carcinomas."

Next, the Multiple Primary and Histology Coding Rules Manual is the correct source for coding histology for cases diagnosed 2007 or later.

The following steps are used to determine the histology code.

Open the Multiple Primary and Histology Coding Rules manual. For an endometrial primary, use the Other Sites Histo rules to determine the histology code because endometrium does not have site specific rules.

Start with the MULTIPLE TUMORS ABSTRACTED AS A SINGLE PRIMARY module, Rule H18. The rules are intended to be reviewed in consecutive order within the module from Rule H18 to Rule H31. You stop at the first rule that applies to the case you are processing.

Code the appropriate combination/mixed code (Table 2) when there are multiple specific histologies. GYN malignancies with multiple types of adenocarcinoma have histology coded to 8323/3 [mixed cell adenocarcinoma] per rule H30.

 2011