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Per Appendix F, do not report this case based on the information provided.

The oncologist likely used the pathology report and clinical factors to determine the diagnosis of hemophagocytic lymphohistiocytosis, which is not reportable. Hemophagocytic lymphohistiocytosis is caused by an over stimulated immune system (infection, etc.). This clinical syndrome is associated with a variety of underlying conditions. To be reportable, it must state "fulminant hemophagocytic syndrome" (in a child) to be reportable (9724/3).

The pathology report for this case is not definitive. It states that the process "may" represent the EBV-associated lymphoproliferative disorder in children.

Follow back on this case to confirm reportability if possible.

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

Code the primary site to multiple lymph node regions, NOS [C778] per Rule PH21 when multiple lymph node regions, as defined by the ICD-O-3, are involved and it is not possible to identify the lymph node region where the lymphoma originated

In the Abstractor Notes section in the Heme DB for PTLD it states PTLD commonly involves lymph nodes, GI tract, lungs and the liver. This patient has extensive lymph node involvement. Rule PH26 states to code the primary site to the bone marrow when ONLY the bone marrow is involved; however, that does not apply in this case.

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.

Rule PH21 applies to this situation which states to 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. Axillary nodes are coded to C773 and inguinal nodes are coded to C774. There are two lymph node regions involved. Code the primary site to C778 [multiple lymph nodes].

If this patient had only bilateral axillary OR only bilateral inguinal nodes are involved, then PH20 would have applied and you would code to the specific lymph node region mentioned.

Code the primary site to C068 [overlapping lesion of the mouth] per Rule PH24. Code the primary site to the organ when lymphoma is present only in an organ.

This lesion overlaps the left soft tissue of the maxilla (the maxillary gingiva) [C030] and the left vestibule of the mouth [C061]. There is no documentation indicating in which specific site the lesion arose. The maxilla is the upper jawbone. The soft tissue that overlies the maxilla is a part of the oral cavity. It is reasonable to interpret the documentation such that the tumor in the maxilla is an extension of the overlapping oral mucosa tumor.

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.

Hyalinizing trabecular neoplasm is not reportable.

Hyalinizing trabecular neoplasm, or hyalinizing trabecular tumor, is a synonym for hyalinizing trabecular adenoma [8336/0] in the ICD-O-3. The 2004 WHO classification states that "fine needle aspiration biopsy is often interpreted as papillary carcinoma because of the nuclear features in the tumor."

For cases diagnosed 2007 or later, accession a single primary, adenocarcinoma [8140/3] of the left upper lobe lung [C341]. The steps used to arrive at this decision are:

Open the Multiple Primary and Histology Coding Rules Manual. Choose one of the three formats (i.e., flowchart, matrix or text). Go to the Lung MP rules because site specific rules exist for this primary.

Start at the MULTIPLE TUMORS module, rule M3. The rules are intended to be reviewed in consecutive order within a module. The patient has two tumors in the same lung with the same histology.

Do not use the AJCC Manual to make multiple primary decisions. Use the MP/H Rules to determine the number of primaries to accession.

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

Castleman disease, NOS, is not reportable for cases diagnosed 2010 and later. However, when Castleman disease is diagnosed in connection with large B-cell lymphoma [9738/3], it is reportable.

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.

Assign code 5 [T-cell] for anaplastic large cell lymphoma [9714/3] unless the pathologist specifies that the histology is a B-cell disease process. See Grade rule G2, Note 2.

In the Heme DB, there is a default value in the Grade field for histologies that do not have a grade specified. However, this particular histology does not default to code 9. There was an error in the Grade section of the 2010 and 2012 versions of Heme DB that has now been corrected in the latest release.