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Report Produced: 09/15/2025 12:21 PM

Report Question ID Question Discussion Answer Year
20130161 Primary Site--Heme & Lymphoid Neoplasms: Is the primary site coded to C779 or C421 for a bone marrow that is positive for B-cell acute lymphoblastic leukemia, the peripheral blood demonstrates leukemic involvement and the PET scan shows involvement of abdominal lymph nodes, spleen and throughout the bones? See Discussion.

1/11/13 Bone marrow bx: B-cell acute lymphoblastic leukemia. Flow cytometry of peripheral blood shows leukemia involvement.

PET scan shows involvement of abdominal lymph nodes, spleen and throughout the bones. The patient has an elevated WBC, anemia and thrombocytopenia.

The answer to SINQ 20120047 (which is no longer visible in the system) said to code B lymphoblastic leukemia/lymphoma to bone marrow for primary site if there is bone marrow involvement. The Heme/Lymph Manual Rule PH7 says to code bone marrow as the primary site if bone marrow is the only site involved.

Following the manual, the primary site would be C779. However, according to the answer to SINQ 20120047, the primary site would be C421. Which is correct?

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

Per the Heme DB, the histology B-cell acute lymphoblastic leukemia is synonymous with B lymphoblastic leukemia/lymphoma, NOS. Per Rule PH8, for a neoplasm that can manifest as either leukemia lymphoma or leukemia lymphoma, one is to code the primary site to the site of origin when lymph node(s) or lymph node region(s), tissue(s) or organs are involved. The Note 4 instruction states it is necessary to go to Module 7 (Rules PH18-PH27) to code the more specific primary site. In this case, use Rule PH22 to code primary site to C779 [lymph nodes, NOS] for the case you describe.

In this case, there is involvement of abdominal lymph nodes, spleen, bone marrow and bone. There is no indication of the primary site. Per the Heme DB, the most frequent sites of involvement for the lymphoma are bone and lymph nodes. This is a Stage IV lymphoma.

The now inactivated SINQ 20120047, stated that based on the sites of involvement, this histology could be coded as either leukemia or lymphoma. If the only involvement is the bone marrow, the site is coded to C421 [bone marrow]. The involvement of peripheral blood does not change the primary site because such involvement is part of the leukemic process.

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
20130200 Primary Site--Heme & Lymphoid Neoplasms: What is the primary site for a diffuse large B-cell lymphoma involving the testicles, stomach, rectum and bone marrow, when no lymph nodes are involved?

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

Per PH27, code the primary site to C809 [unknown]. Rule PH27 states one is to code the primary site to unknown [C809] when there is no evidence of lymphoma in lymph nodes AND the physician documents in the medical record that he/she suspects that the lymphoma originates in an organ(s) OR there is multiple organ involvement without any nodal involvement.

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
20000487 Primary Site--Kaposi Sarcoma: Would the following Kaposi primaries be examples of cases not coded to skin for primary site? See discussion.

1. KS developed initially as a lesion in the oral cavity and followed by the appearance of skin lesions.

2. KS found in a resected parotid gland with metastasis to the parotid gland lymph node. No skin lesions identified.

3. KS discovered in a biopsied 3 cm axillary lymph node. Clinically, the patient had hepatosplenomegaly, ascites, and extensive mesenteric lymph nodes. (No mention of skin.)

Code the Primary Site field as follows:

1. C44.9 [Skin, NOS] as the default value when lesions develop simultaneously in skin and non-skin areas.

2. C07.9 [Parotid gland]

3. C44.9 [Skin, NOS] as the default value when there is no mention of lesions in the skin or other primary site.

Edward Klatt states in Practical AIDS Pathology, "...Visceral Kaposi (involving one or more internal organ sites) is also present in three-fourths of cases, but may not be diagnosed prior to autopsy. Visceral involvement frequently includes the lung, lymph nodes and gastro-intestinal tract."

 2000
20041015 Primary Site--Lymphoma: How should this field be coded when a diffuse large B-cell lymphoma is found in the femur and in the soft tissue of the anterior chest wall but all CT scans are negative for lymphadenopathy?

For cases diagnosed prior to 1/1/2010:Code the Primary Site field to C809 [Unknown primary site]. The primary site of diffuse large B cell lymphoma can be either nodal or extranodal. The case described above is likely extranodal because there is no evidence of lymph node involvement. Because the extranodal site of origin is unknown, code the Primary Site to C809.

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.

 2004
20000848 Primary Site--Lymphoma: How should you code the primary site for a lymphoma that presents in a "mediastinal mass"? Code the Primary Site field to C77.1 [mediastinal lymph nodes]. 2000
20000849 Primary Site--Lymphoma: How should you code the primary site for a lymphoma that presents with involvement of an extranodal site and regional lymph nodes? See discussion.

1. Lymphoma involves the spleen and the splenic lymph nodes.

2. MALT Lymphoma involves the stomach and the gastric and iliac lymph nodes.

1. Code the Primary Site field to C42.2 [spleen].

2. Code the Primary Site field to C16._ [stomach].

When lymphoma presents in an extranodal site and in the regional lymph nodes for that extranodal site, code the Primary Site field to the extranodal site. The typical disease process is that lymphoma can spread from an extranodal organ to its regional lymph nodes. It cannot metastasize from the regional lymph node to the extranodal organ. The exception to this would be if the lymph nodes presented as one large mass that extended into the regional organ.

 2000
20021146 Primary Site--Lymphoma: Is the primary site likely to be extranodal for a lymphoma that presents in an extranodal site and lymph nodes which are regional for that site? Is the primary site also likely to be extranodal if an extranodal site and lymph nodes are excised? See discussion. Example: Work-up included a negative CXR. A CT showed multiple dilated loops of small bowel consistent with obstruction and nodular prominence at the base of bladder. Laparotomy with resection of small bowel and multiple biopsies of enlarged mesentric lymph nodes performed. Final path diagnosis: Lymphoma in a "mesenteric mass" and in "small bowel." There was no mention of lymph nodes in the final diagnosis and the detailed micro described the mesenteric mass as just adipose tissue replaced by lymphoma. However, the gross for that specimen states 4 lymph nodes were found in the fat. The small bowel micro described an ulcerated lesion of the small bowel extending into muscularis.

For cases diagnosed prior to 1/1/2010:Code the Primary Site field to C17.9 [small bowel] for the example. When an extranodal organ and that organ's regional nodes are involved, the extranodal site is most likely the primary, unless there is extension from the regional nodes to the organ. If the primary site cannot be determined for a lymphoma diagnosed in both a nodal and extranodal site, code to C77.9 [lymph nodes NOS].

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.

 2002
20031062 Primary Site--Melanoma: How would this field be coded for a pleural effusion consistent with metastatic melanoma and "no skin lesions?" Code primary site as C44.9 [Skin, NOS]. ICD-O-3 does not list a suggested site code for 8720/3 because melanoma can arise in other parts of the body. However, C44.9 [Skin, NOS] is the default when the primary cannot be found. 2003
20021031 Primary Site--Meninges: Should the primary site for a meningioma of the right frontal lobe be coded to C71.1 or C70.0? See discussion. In the opinion of some neurologists it is more important to capture the lobe in which the meningioma is located rather than code the primary site to meninges. Should a meningioma always be coded to meninges for primary site? Code the Primary Site field to C70.0 [cerebral meninges], the suggested site code for most meningiomas. Meningiomas arise from the meninges, not the brain (although they can invade brain). ICD-O-3 does not differentiate the specific location of the brain that the meninges cover. The information of interest to neurologists would have to be captured in an optional or user-defined field. 2002
20021090 Primary Site--Ovary/Peritoneum: How should the Primary Site field be coded when no resection is done and it is uncertain whether the primary site is in the ovary or the peritoneum? See discussion. CT: ascites, omental cake and peritoneal studding. H&P impression: probable ovarian or peritoneal primary. Repeat CT: no enlarged adnexal mass seen to suggest ca of ovary, but possibility couldn't be ruled out. Omental bx: Metastatic ca. Comment: "IHC stains have been performed and are not typical of ovarian ca, although do not exclude an ovarian primary." After the bx, there were two clinical diagnoses written a month apart with no evidence of further work-up between those dates. The first diagnosis was "ovarian ca". The second was "Peritoneal carcinomatosis 2 month ago; Primary is unknown, possibly ovarian."

Use the best information available to identify the primary site. In this case, it is the physician's clinical assessment. Code the Primary Site to C56.9 [Ovary] for this example because the ovary is indicated to be the primary site according to the physicians involved.

When there is no surgical procedure involving the removal of the ovaries, code the Primary Site based on the clinical assessment of the disease location. If the disease is only noted to be in the peritoneum, code site to peritoneum, NOS. If the disease is seen clinically in both the ovary and the peritoneum, code site to ovary.

 2002