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

This case should be accessioned as one primary. Per Rule PH26, code the primary site to bone marrow (C421) when lymphoma is present only in the bone marrow. (We assumed all available physical exams, scans, and other work-up were negative for lymph node, tissue, or organ involvement.) Histology is coded to 9680/3 [Diffuse large B-cell lymphoma (DLBCL)]. Under the Alternate Names section of the Heme DB, a synonym for DLBCL is B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt 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 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.

Per Rule M3, abstract a single primary when a sarcoma (myeloid sarcoma) is diagnosed either simultaneously or after a leukemia of the same lineage (acute myeloid leukemia). Per the notes for Rule M3, the sarcoma is a solid manifestation of the associate leukemia.

Per PH10, code the histology to 9861/3 [acute myeloid leukemia] and the primary site to C421 [bone marrow]. PH10 states one is to code the primary site bone marrow (C421) and code the histology acute myeloid leukemia, NOS (9861/3) or any of the specific AML histologies (9840/3, 9865/3-9867/3, 9869/3-9874/3, 9891/3, 9895/3-9898/3, 9910/3, 9911/3 and 9931/3) when the diagnosis is myeloid sarcoma (9930/3) AND there is a simultaneous or previous diagnosis of acute myeloid leukemia.

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 prior to 1/1/2010:

There is considerable difference between therapy-related myelodysplastic syndrome (MDS) and drug-induced sideroblastic anemia (SA).

Therapy-related MDS is the result of irreversible damage to the bone marrow caused by certain kinds of myelotoxic drugs used to treat cancer. Examples are Cytoxan and Etoposide. There is usually a 10+ year delay between the first primary and its treatment and the therapy-related MDS. Therapy-related MDS is not reversible and is reportable as a malignancy. Because the drugs were almost always given to treat a malignancy, therapy-related MDS is almost always a second primary.

Drug-induced SA is not reportable as a malignancy. Drug-induced SA is the result of short term effects of certain drugs on the bone marrow. Drug-induced SA is reversible, as the marrow recovers once the drugs are out of the system.

For cases diagnosed 2010 forward, 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.

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

Currently, lymphoma in situ is not reportable. It is true that this is a recently identified pathologic entity. Our experts say that there is still some controversy to be ironed out regarding the criteria for identifying an in situ lymphoma. Their recommendation was to wait until clear guidelines had been established for the pathologists before we start collection of in situ lymphomas. We anticipate collecting these entities in the future.

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.

Code the histology using the pathology report from the most representative specimen, even if that histology is less specific.

For the case example above, code 8140 [adenocarcinoma, NOS].

The rationale is that a diagnosis from a smaller specimen will be less accurate and less representative of the true histology compared to a larger tumor specimen.

For cases diagnosed prior to 1/1/2010:The general instructions for assigning histology codes are to code as precisely as possible. Acute myeloid leukemia with maturation is the definition of the FAB M2 category. A pathologist does not need to provide every word in the term associated with an ICD-O code; pathologists don't always talk that way. AML M2 is a very specific diagnosis and should be coded to 9874/3.

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.

Assign code 8262/3 [Villous adenocarcinoma].

According to the WHO Classification of Tumours, Breast and Female Genital Organs (2003), villoglandular is one of four variants of endometroid adenocarcinoma. The corresponding ICD-O-3 code according to WHO is 8262/3.

For uterine primaries, code the abbreviation "ca" to 8010/3 [carcinoma, NOS].

When coding death certificate only (DCO) cases, if the site is coded to an unknown primary and no specific histology information is available other than the abbreviation "ca," interpret ca as cancer (8000/3) per NAACCR Procedure Guidelines for Registries, Series V; Resolving Death Clearance Issues, page V-15.

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.