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The histologies stated for this case are Ewing sarcoma (9260) and PNET, primitive neuroectodermal tumor,(9364)*. Use the Other Site Rules, starting with H8. Stop at H17 and assign the higher histology code -- 9364/3 [Peripheral neuroectodermal tumor].

*The term "PNET" is used for two different tumors. One is primitive neuroectodermal tumor (9473) and pertains to brain tumors per ICD-O-3 review. The other is peripheral primitive neuroectodermal tumor (pNET or PPNET 9364) and pertains to bone or soft tissue tumors. This case is stated to be soft tissue and Ewing sarcoma, so it is 9364 rather than 9473.

Code microwave tumor ablation as surgery. For lung, assign code 15.

This question was discussed by the technical advisory group – a small group of representatives from each standard setter which meets periodically. The group agreed on this consensus answer.

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

Accession two primaries per Rule M10, the first is a chronic neoplasm RAEB2 [9983/3] and the second is an acute neoplasm AML, NOS [9861/3]. Rule M10 states abstract as multiple primaries when a neoplasm is originally diagnosed in a chronic phase (MDS RAEB2) and an acute disease (AML) is diagnosed more than 21 days later. This is the rule that fits your case.

There are several important pieces of information. There were two bone marrows biopsies; one confirmed the chronic disease and a second confirmed the acute disease. The dates of the bone marrows are more than 3 months apart. Because you have a chronic and an acute disease, Rules M8-M13 in the coding manual apply.

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.

Per Rule M10, this case should be accessioned as two primaries. According to Rule M10, one is to abstract as 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 histology for the 1996 chronic neoplasm is coded to 9670/3 [small lymphocytic lymphoma]. The histology for the 2012 acute neoplasm is 9680/3 [diffuse large B-cell 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.

For cases diagnosed 1/1/2003 and after: A bone marrow transplant can be first course of therapy for cases in which there has been no progression of disease between the initial therapy (e.g., surgery, radiation, chemotherapy) and the bone marrow transplant. Code Hematologic Transplant and Endocrine Procedures field to 10-12 or 40 (depending on the type of bone marrow transplant performed).

Do not use leukemia treatment time guidelines when coding breast cancer treatment.

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

Per Rule PH30, one is to use the to determine the primary site and histology when rules PH1-PH29 do apply. Code the primary site to C421 [bone marrow] because that is the only site listed under the Primary Site section of the Heme DB.

Under the Abstractor Notes section in the Heme DB, it indicates that the bone marrow is always involved, and the white and red pulp of the spleen may be involved with systemic mastocytosis. This is how this patient presented; therefore, the bone marrow is the primary site. The spleen is secondarily involved because the spleen cleanses the blood and the neoplastic cells have infiltrated the red and white pulp of the spleen. The same is true for the lymph nodes. Although the lymph nodes are rarely involved, they may be involved when the patient has systemic mastocytosis.

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.

Rathke cleft cyst is not reportable. Cysts are not neoplastic. However, Rathke pouch tumor (C751, 9350/1) is a reportable neoplasm for cases diagnosed 2004 and later. The Rathke pouch is coded to the pituitary gland. Benign and borderline pituitary tumors have been reportable since 2004.

Primitive myxoid mesenchymal tumor of infancy (PMMTI) is reportable. PMMTI is listed in the new WHO 5th edition Classification of Soft Tissue and Bone Tumors under round cell sarcomas. This is a variant of BCOR sarcomas. There is a new ICD-O histology code assigned for cases diagnosed in 2022 or later (9368/3). Code this 2019 case to round cell sarcoma, undifferentiated 8803/3. Use text fields to explain the details.

Report intracholecystic papillary neoplasm (ICPN) with high-grade dysplasia (8503/2) of the gallbladder.

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

Myelodysplastic disorder is a synonym for myelodysplastic syndrome (MDS). If no further workup is done or no additional information can be found, code the histology of myelodysplastic disorder to 9989/3 [MDS] for cases diagnosed 1/1/2010 and later.

Refer to the Abstractor Notes section in the Heme DB, Abstractor Notes for MDS. Myelodysplastic (disorder) syndrome is a NOS term. Usually when this diagnosis is made, the physician will conduct further tests to determine a more specific disease in the Myeloproliferative Neoplasms group. Other specific histologies include: refractory anemia with unilineage dysplasia, refractory anemia with ring sideroblasts, refractory cytopenia with multilineage dysplasia, refractory anemia with excess blasts, myelodysplastic syndrome with del(5q), childhood myelodysplastic syndrome. If a more specific disease is diagnosed, code to that specific neoplasm.

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.