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Reportability--Heme & Lymphoid Neoplasms: Is EBV-positive hemophagocytic lymphohistiocytosis (HLH) reportable when diagnosed in a 5 year old child and resulted in death in less than two months?
Hemophagocytic lymphohistiocytosis (HLH) is not a reportable disease because it is not listed in the Heme DB.
Per our expert pathologist consultant, "HLH is a lymphocyte driven hemophagocytic syndrome which may be either genetically based or caused by over-activated lymphoid cells, often in response to a viral infection. It is an abnormal immune response and is not considered a malignant disease, and is, therefore, not reportable. It is not synonymous with EBV-positive T-cell lymphoproliferative disease of childhood (9724/3)."
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.
Reportability--Brain and CNS: Does a neurofibroma actually arise in peripheral nerve roots like a schwannoma even if it is referred to as a "C6-T1 intradural spinal cord tumor" and is therefore not reportable?
Schwannomas and neurofibromas of the peripheral nerves are not reportable. Schwannomas of the nerve root or spinal dura are reportable.
No. This is not a reportable hematologic condition. When you do not find a hematologic or lymphoid condition listed in the Heme DB, it is not reportable. Hemophagocytic lymphohistiocytosis is an uncommon hematologic disorder. The patient usually presents with fever, splenomegaly, and jaundice. Laboratory findings are lymphocytosis and histiocytosis. Pathology findings are hemophagocytosis.
Appendix F lists this term as non-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.
2004 SEER Manual Errata/CS Lymph Nodes--Head & Neck: On page C-353, in the supraglottic larynx schema, there is no mention of Level IV nodes in the CS Lymph Node codes.
This answer was provided in the context of CSv1 coding guidelines. The response may not be used after your registry database has been converted to CSv2.The CS Steering Committee is aware of this issue and is working to resolve it.
Primary site--Heme & Lymphoid Neoplasms: How is the primary site to be coded for a 2010 diagnosis of follicular lymphoma involving the spleen and lymph nodes above and below the diaphragm?
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 follicular 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.
Histology (Pre-2007)--Lung: Should "moderately differentiated adenocarcinoma of scar type, intermixed with bronchiolo-alveolar carcinoma" be coded to 8250 [bronchiolo-alveolar adenocarcinoma, NOS] or 8255 [adenocarcinoma of mixed subtypes]?
For tumors diagnosed prior to 2007:
Code Histology to 8255 [Adenocarcinoma with mixed subtypes]. This is a single tumor containing both a scar carcinoma and a bronchiolo-alveolar carcinoma--use 8255. The synonym for 8255 is adenocarcinoma combined with other types of carcinoma (not just subtypes).
For tumors diagnosed 2007 or later, refer to the MP/H rules. If there are still questions about how this type of tumor should be coded, submit a new question to SINQ and include the difficulties you are encountering in applying the MP/H rules.
Behavior--Breast: Should the behavior change to /3, invasive, to get a case to clear edits? The histology of this breast case is ductal carcinoma in situ (DCIS), 8500/2. Lymph nodes are positive for micro-mets (0.2 mm-2 mm). SEER Summary Stage: 3, regional lymph nodes positive. This creates an edit for SEER Summary Stage due to the behavior code of /2, in situ.
Code the behavior to /3, not just to pass edits, but because this is an invasive case based on the positive lymph nodes.
For most cases, behavior is based on the primary tumor, but in situations like this where an invasive component cannot be found and there are positive lymph nodes, the /3 behavior is assigned based on the positive lymph nodes.
Behavior--Prostate: What is the correct behavior of intraductal carcinoma from a prostate biopsy with a Gleason score 4+4=8. While highly aggressive, but not suggestive of invasion, coding behavior as /2 seems inappropriate.
WHO classifies intraductal carcinoma of the prostate 8500/2. According to WHO, "the hallmark of intraductal carcinoma of the prostate is a proliferation of prostate carcinoma cells that is within and may significantly expand the native prostatic ducts and acini, with the basal cell layer at least partially preserved." Further, differentiation between intraductal carcinoma and infiltrating high-grade carcinoma of the prostate may require basal cell stains. Under Prognosis, WHO states: " intraductal carcinoma of the prostate on prostate biopsies is often associated with high-grade cancer (with a mean Gleason score of 8) ."
So while it may seem counter-intuitive, assign behavior code /2 when the diagnosis is intraductal carcinoma of the prostate.
MP/H Rules/Multiple Primaries--Bladder/Renal Pelvis: Is a non-invasive papillary transitional cell carcinoma of the bladder diagnosed one year after the occurrence of an invasive papillary transitional cell carcinoma of the renal pelvis reported as one or two primaries?
For cases diagnosed 2007 or later:
This is a single primary with renal pelvis as primary site.
Use the 2007 MP/H rules to determine if the 2007 diagnosis is a new primary. Use the Urinary rules, multiple tumors module. Start with rule M3. Follow the rules down to Rule M8 and stop. This is an example of implantation effect.