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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.

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

Idiopathic thrombocytic purpura (ITP) is not a synonym for refractory thrombocytopenia (RT). ITP is not a reportable disease. See Appendix F.

Under the Alternate Names section in the Heme DB, the only synonym for refractory thrombocytopenia is "RT." ITP is not listed as a synonym for refractory thrombocytopenia.

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.

Assuming the same primary site for the 2008 lesion, according to the current MP/H rules the high grade fibrosarcoma [8810/3] is a new primary per Head & Neck MPH rule 11 because it is a different histology.

The revised MP/H rules will include tables to define tumors that de-differentiate (transform) and recur with what is seemingly a different histology. Although the rules will be changed in the future, we must use the rules in place at this time for this case.

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

Use Rule PH21 to code the primary site to C778 [lymph nodes of multiple regions]. The spleen is not listed under the Primary Site(s) section in the Heme DB for diffuse large B-cell lymphoma. Per Rule PH21 code the primary site to multiple lymph node regions, NOS (C778) when multiple lymph node regions, as defined by ICD-O-3, are involved and it is not possible to identify the lymph node region where the lymphoma originated. The spleen is a primary site for only a few lymphomas (noted in the Heme DB). Because the spleen filters blood, it is often reactive (splenomegaly) or frankly involved with the lymphoma. That reaction or involvement, however, does not affect the primary site coding. Only the involved nodes are used in coding 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.

The histology codes for lung tumors are based on the World Health Organization Classification of Lung Tumors. Chart 1 in the MP/H Lung Equivalent Terms, Definitions, Charts, Tables and Illustrations (2007 MP/H Rules Manual) illustrates the WHO Classification of Lung Tumors.

Using Chart 1, note that papillary adenocarcinoma [8260] is located under the Adenocarcinoma (NOS) branch. The histology in question was stated to be "micropapillary adenocarcinoma" and not "papillary carcinoma." Papillary carcinoma, NOS [8050] is not actually located on the chart. However, papillary squamous cell carcinoma is listed under the Squamous Cell Carcinoma, NOS branch, histology code 8052.

Next, look up papillary carcinoma [8050] in the Morphology - Numerical listing section of the ICD-O-3. Papillary carcinoma, NOS is a Squamous Cell Neoplasm. (Refer also to SINQ 20091040.)

The key word used to determine the appropriate histology in this case is "adenocarcinoma." This is a papillary adenocarcinoma and not a papillary squamous neoplasm.

For cases diagnosed 2007 or later: The 2011 diagnosis of adenocarcinoma, NOS in the left lower lobe lung is a separate primary.

The steps used to arrive at this decision are:

Open the Multiple Primary and Histology Coding Rules manual. For a lung primary, use the Lung Multiple Primary rules to determine the number of primaries.

The 2010 right lung bi-lobectomy showed two separate tumors that were determined to be two primaries: clear cell adenocarcinoma [8310/3] and adenocarcinoma, NOS [8140/3]. The histology of the new left lung mass is adenocarcinoma, NOS [8140/3].

Start at Rule M3 using the MULTIPLE TUMORS module because this patient has more than one tumor. The rules are intended to be reviewed in consecutive order within the module (i.e., from Rule M3 to Rule M12 in this case). Stop at the first rule that applies to the case you are processing. This patient has two tumors in each lung with ICD-O-3 histology codes that are different at the second (xxxx) digit. Abstract the LLL adenocarcinoma as a new primary [C343, 8140/3].

The patient has two tumors in each lung. The right lung showed adenocarcinoma and clear cell adenocarcinoma. The two tumors in the left lung were both adenocarcinomas. Clear cell adenocarcinoma [8310] on the right is different at the second digit from adenocarcinoma [8140] on the left. Rule M12 cannot be applied to this case, because Rule M7 is the first rule that applies to this case when processing the rules in consecutive order.

For cases diagnosed 2007-2014:

Code the Primary Site field to C210 [Anus, NOS]. This is an unusual and rare presentation. According to our expert pathologist, "There is no ideal site code [for] this case. I would code to C210. In this location it can at least be located by anyone who wants to get a look at such lesions. Because of the unusual location of this tumor, I would like to be able to code it to perineum, but it will be totally lost in those site codes as they represent extensive areas beyond perianal (skin of trunk, soft tissue of pelvis, and pelvis, respectively)... I would not code to rectum [because it would be] lost among too many primary rectal carcinomas."

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.