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SEER Inquiry System - Report
Produced: 06/27/2025 8:47 PM
Question 20120072
Inquiry Details
References:
Heme & Lymph Manual & DB
Question:
Primary site--Heme & Lymphoid Neoplasms: How is the primary site coded for a diagnosis of multifocal Langerhans cell histiocytosis with involvement of the bone, liver, spleen and retroperitoneum?
Discussion:
Answer:
For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.
Per Rule PH30, use the Heme DB to determine the primary site and histology when rules PH1-PH29 do not apply. Code the primary site to C419 [bone, NOS], assuming there are multiple bones involved in this case. If only one bone is involved, code the primary site to the specified bone.
In the Abstractor Notes section in the Heme DB, it indicates the primary site may differ for LCH in the solitary disease and multisystem disease. This patient has multisystem disease with involvement of the bone, liver, spleen and retroperitoneum. The most common sites for multisystem involvement include three of the four above sites (bone, liver, and spleen).
Determine the primary site based on the knowledge of the usual sites of involvement for this disease, the actual sites of involvement for the case presented, and identifying which sites of involvement are likely metastatic and which are the potential primary sites. There are two potential primary sites of involvement: the bone and the retroperitoneum. Bone is a common site of involvement for LCH while the retroperitoneum is not. Code the primary site to C419 [bone, NOS] because multiple bones are involved for this patient and bone is the most common site for LCH based on the documentation in the Abstractor Notes.
The spleen and liver are typically not primary sites for this disease process. They become involved when there is multisystem involvement because they filter the blood. They are typically sites of metastatic involvement. This information will be added to the ABSTRACTOR NOTE section.
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.
History:
This SINQ question has been updated to the Hematopoietic & Lymphoid Neoplasm Manual & Database published January 2014.
The original answer below was written based on the rules in 20102
For cases diagnosed 2012 and later, access the Hematopoietic Database at
http://seer.cancer.gov/tools/heme/.
Click on Hematopoietic Project. Click on Hematopoietic and Lymphoid Database. The 2012 Hematopoietic Coding Manual (PDF) button will appear to indicate the correct version of the program is available now for query.
Code the primary site as bone, NOS [C41.9], assuming there are multiple bones involved in this case. If only one bone is involved, code the primary site to that bone. The steps used to arrive at this decision are:
Enter in the Heme DB to find the histology. Click on the SEARCH button. Ensure that the term "Langerhans cell histiocytosis" [9751/3] is highlighted on the screen.
Check the PRIMARY SITE(S) section. No specific primary site is stated. There is a reference to the Abstractor Notes and Module 7. (Indicating Module 7 appears to be a typo. Module 10 is the appropriate module.)
Scroll down to the ABSTRACTOR NOTE section. This indicates the primary site may differ for LCH in the solitary disease and multisystem disease. This patient has multisystem disease with involvement of the bone, liver, spleen and retroperitoneum. The most common sites for multisystem involvement include three of the four above sites (bone, liver, and spleen).
Click on the 2012 HEMATOPOIETIC CODING MANUAL (PDF) button. Once in the manual, go to the Primary Site and Histology Coding Rules. Scroll down to Module 10. Start at Rule PH42. The rules are intended to be reviewed in consecutive order within the module. Stop at the first rule that applies to the case you are processing. The note refers one to the ABSTRACTOR NOTE in the Heme DB.
Determine the primary site based on the knowledge of the usual sites of involvement for this disease, the actual sites of involvement for the case presented, and identifying which sites of involvement are likely metastatic and which are the potential primary sites. There are two potential primary sites of involvement to consider: the bone and the retroperitoneum. Bone is a common site of involvement for LCH while the retroperitoneum is not. Code the primary site as C41.9 [bone, NOS] because multiple bones are involved for this patient and bone is the most common site for LCH based on the documentation in the ABSTRACTOR NOTE.
The spleen and liver are typically not considered primary sites for this disease process. They become involved when there is multisystem involvement because they filter the blood. They are typically sites of metastatic involvement. This information will be added to the ABSTRACTOR NOTE section.
