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Report Produced: 02/23/2026 10:21 AM

Report Question ID Question Discussion Answer Year
20120064

Reportability--Heme & Lymphoid Neoplasms: If hemophagocytic lymphohistiocytosis treated with several rounds of chemotherapy is reportable, what is the primary site?

Patient was diagnosed with hemophagocytic lymphohistiocytosis on blood and bone marrow biopsy. This was also referred to in the chart as hemophagocytosis and hemophagocytic syndrome. Hemophagocytic syndrome is listed in the Heme DB as 9724/3. The patient had several rounds of fairly aggressive chemotherapy. Would the correct primary site for histology 9724/3 be C421 [bone marrow], or C779 [lymph nodes, NOS]? See SINQ 20100113.

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

Hemophagocytic syndrome, also known as hemophagocytic lymphohistiocytosis (HLH), is not reportable. Per Appendix F, HLH is caused by an over stimulated immune system (infection, etc.). It is a clinical syndrome associated with a variety of underlying conditions. To be reportable, a child's diagnosis must state "fulminant hemophagocytic syndrome" to be reportable (9724/3). This is not the situation in this case.

"Hemophagocytic lymphohistiocytosis" is also listed in Appendix F: Non-Reportable List for Hematopoietic Diseases.

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.

 2012
20120095 MP/H Rules/Multiple primaries--Breast: How many primaries are accessioned if a patient is diagnosed with inflammatory carcinoma of the left breast, (ductal with apocrine features type on biopsy), and an incidental lobular carcinoma in the right breast? See Discussion. A 1.2 cm lobular carcinoma was incidentally discovered during the work-up of the patient's left breast that was inflammatory carcinoma. The lobular carcinoma on the right was localized without any skin involvement. Rule M6 indicates inflammatory breast carcinoma in either breast is a single primary. Does rule M6 apply when the patient has inflammatory carcinoma in one breast and a separate lobular carcinoma in the other?

For cases diagnosed 2007 or later, accession two primaries, ductal with apocrine features in the left breast and lobular carcinoma in the right breast.

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 Breast MP rules because site specific rules exist for this primary.

Start at the MULTIPLE TUMORS module, rule M4. The rules are intended to be reviewed in consecutive order within a module. The patient has tumors in both the right and left breasts.

Rule M6 does not apply because inflammatory carcinoma involves only the left breast and the patient has a different histology in the right breast and there is no mention of inflammatory carcinoma in that breast. In this situation continue to the next applicable rule.

2012
20120059 Primary site/Reportability--Breast: Is a "right nipple skin" biopsy that demonstrates squamous cell carcinoma reportable using a primary site of C500? See Discussion. In the 2011 SEER Manual Reportability Examples, example 3, it states a "biopsy-proven squamous cell carcinoma of the nipple" is reportable when the subsequent resection shows "no evidence of residual malignancy in the nipple epidermis." However, this example does not specify the biopsy is from the nipple skin and the ICD-O-3 does not list nipple skin as a synonym for code C500.

Because the site is specifically stated to "skin" of nipple [C44.5], this case is not reportable.

If possible, you may wish to confirm the type of biopsy performed. If the biopsy was done by FNA or needle biopsy, the biopsy tissue should contain a full-thickness of skin and subcutaneous breast (nipple) tissue. If that is the case, this tumor would likely be a reportable squamous cell carcinoma of nipple [C50.0]. If, however, this was a punch biopsy it is more likely a non-reportable squamous cell carcinoma of the skin [C44.5].

2012
20120012 Histology--Heme & Lymphoid Neoplasms: How is histology coded if the pathology report shows diffuse large B-cell lymphoma arising in a small cell lymphoma - Richter's transformation, also compatible with chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma (SLL)?

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

Code the histology to 9680/3 [diffuse large B-cell lymphoma (DLBCL)].

For CLL (and CLL/SLL), Richter's transformation represents when CLL changes into DLBCL. In this case, there was a biopsy that demonstrated a diagnosis of the chronic disease (CLL/SLL) transforming (Richter's transformation) into an acute disease DLBCL.

Per Rule M8, one is instructed to abstract the acute neoplasm as a single primary when both a chronic (CLL/SLL) and an acute neoplasm (diffuse large B-cell lymphoma (DLBCL)) are diagnosed simultaneously there is documentation of only one positive bone marrow biopsy, lymph node biopsy or tissue biopsy.

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.

2012
20120039

Primary site--Heme & Lymphoid Neoplasms: What primary site and heme rule applies when a PET scan shows bilateral renal masses, hypermetabolic liver lesions and retroperitoneal lymphadenopathy, a right kidney biopsy was positive for diffuse large B-cell lymphoma, and the bone marrow biopsy was negative? See Discussion.

Patient has a history of chronic lymphocytic leukemia (CLL). February 2011 abdomen/pelvis x-ray showed development of bilateral renal masses. April 2011 PET scan showed intense areas of hypermetabolic activity corresponding to known bilateral renal masses, new hypermetabolic liver lesions, as well as left upper retroperitoneal lymphadenopathy. All findings are worrisome for malignancy. March 2011 right kidney mass biopsy was positive for diffuse large B-cell lymphoma (DLBCL). Bone marrow biopsy was negative for lymphoma.

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

Per Rule PH25, code the primary site of the diffuse large B-cell lymphoma to C649 (kidneys) and laterality to 4 (bilateral). Per PH25, code the primary site to the organ when a lymphoma is present in an and that . This patient had involvement of an organ (bilateral kidneys) as well as regional lymph nodes for that organ. The retroperitoneal lymph nodes are regional for the kidney.

The diffuse large B-cell lymphoma is an acute transformation of the chronic lymphocytic leukemia. Because the DLBCL occurred more than 21 days after the CLL, it is a new primary per Rule M10.

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.

 2012
20130151 Primary site--Heme & Lymphoid Neoplasms: What is the primary site when a splenectomy shows "T large granular lymphocytic leukemia" and the peripheral blood flow cytometry is negative? See Discussion. The physician note states there is no evidence of leukemia on peripheral blood. The disease is localized to the spleen. Is the primary site coded to the bone marrow [C421] or can it be coded to the spleen [C422]?

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

Code the primary site to C421 [bone marrow]. Leukemias are coded to the bone marrow per the Heme DB.

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.

2013
20130120 Primary site--Heme & Lymphoid Neoplasms: What is the primary site for a Langerhans cell Sarcoma of the lower extremity?

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

If the bone is involved, code the primary site to bone. Langerhans more commonly starts in the bone and extends to the soft tissue.

If bone is not involved, code primary site to C492, Connective, subcutaneous and other soft tissues of lower limb and hip.

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.

 2013
20130090 MP/H Rules/Primary site/Histology--Colon/Rectum: How are the primary site and histology to be coded for a diagnosis of familial polyposis with malignant tumors in the sigmoid and rectum? See Discussion.

Preoperative diagnosis was familial polyposis with rectal and rectosigmoid cancer.

The pathology report from the colon resection showed:

  1. 5 mm tumor in the sigmoid. Histologic type: adenocarcinoma.

  • 1.9 cm tumor in the distal rectum. Histologic type: adenocarcinoma with signet ring cell differentiation.

  • 4 cm tumor in the proximal rectum. Histologic type: adenocarcinoma.

    • Gross description: The mucosa of the colon is tan pink with polyposis throughout; more than 1000 tan sessile polyps.

    Should this be a single primary per MP/H Rule M3, histology coded to 8220/3 [familial polyposis] per MP/H Rule H17, and primary site coded to C199?

    This case should be accessioned as a single primary. Code the primary site to the colon and rectum [C199] and the histology to adenocarcinoma in familial polyposis coli [8220/3] per MP/H Rule H17.

    For cases of familial polyposis, when the rectosigmoid or rectum are involved, assign code C199 [colon and rectum]. When the rectosigmoid or rectum are not involved, assign code C189 [colon, NOS].

    		2013
    		20130051 Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned when biopsies of the left and right tonsils show diffuse large B-cell lymphoma and there is no other evidence of involvement? See Discussion. Scans are negative for lymphadenopathy and the bone marrow biopsy was benign. Radiation Oncology staged this as localized bilateral tonsil primary lymphoma.

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

    This case should be accessioned as a single primary, diffuse large B-cell lymphoma [9680/3] of bilateral tonsils. Per Rule M2, a single histology is a single primary. Note 1 for Rule M2 states bilateral involvement of lymph nodes and/or organs is still a single primary.

    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.

    		2013
    		20130100

    Multiple primaries/Primary site--Heme & Lymphoid Neoplasms: How many primaries are there and how should I code the primary site(s)? See discussion.

    Patient had a hemicolectomy and a salpingo-oophorectomy and was found to have diffuse large B cell lymphoma in the colon (10 cm cecal mass), 3/16 regional lymph nodes involved with lymphoma. Fallopian tube showed involvement with diffuse large B Cell lymphoma.

    Multiple primaries - Colon and fallopian tube?

    One primary - Colon? Stage IV, or lymphoma from an unknown primary? Note: There were no other lymph nodes involved.

    Use Rule M2. Abstract as a single primary when there is a single histology.

    When you have questions about how to code the primary site, start with the abstractor notes. If the answer isn't found there go to Module 7 (a specific module to help code primary site for lymphomas).

    The abstractor notes for DLBCL in this case do not provide information you can use for this case. Go to Module 7 in the PH rules.

    Use Rule PH25 Code the primary site to the organ when lymphoma is present in an organ and that organ’s regional lymph nodes. Code the primary site to colon (organ and regional lymph nodes involved). The fallopian tube is secondary involvement. As is common with lymphomas, there can be more than one organ involved. You can differentiate the primary site from the secondary site(s) because of the large colon mass with regional lymph node involvement.

    		2013