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Report Produced: 06/15/2025 08:05 AM

Report Question ID Question Discussion Answer Year
20130128 Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned if a patient has a history of chronic myelomonocytic leukemia and a 12/08/2011 subsequent biopsy of the left leg that confirms leukemia cutis? See Discussion. Patient with a history of chronic myelomonocytic leukemia has been undergoing treatment with Dacogen for three years. On 12/8/11 the patient had a biopsy of the left leg that confirmed a diagnosis of leukemia cutis. How is the leukemia cutis coded?

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

Accession a single primary, chronic myelomonocytic leukemia [9945/3], per Rule M2. Accession a single primary when there is a single histology.

This is not a new primary. Leukemia cutis is the infiltration of neoplastic leukocytes into the skin from the existing leukemia. This is an advanced phase of the leukemia and has a poor prognosis.

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
20130002

Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned, and what is the year of diagnosis, when the patient was initially diagnosed with poorly differentiated, diffuse lymphocytic lymphoma, small cleaved cell [9591/3] in 1991, followed by multiple recurrences and transformations? See Discussion.

5/1991 Left groin biopsy: Poorly differentiated, diffuse lymphocytic lymphoma, small cleaved cell [9591/3].

Subsequently, the patient had multiple recurrences.

7/1/08 Left axillary biopsy: Disease transformed to malignant lymphoma, large B-cell and a small focus of follicular lymphoma.

Patient was followed until there was no evidence of disease.

4/22/10 Left axillary biopsy: Recurrence of follicular lymphoma, grade 1. No large cell component was found. The bone marrow biopsy was negative for lymphoma. The patient was on observation.

11/02/10 MD note indicates the disease progressed to follicular lymphoma, grade 3. No large cell component was identified. The patient clinically has no evidence of disease on maintenance Rituxan.

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, non-Hodgkin lymphoma (previously called poorly differentiated, diffuse lymphocytic lymphoma, small cleaved cell) [9591/3] diagnosed in 1991.

Determining the number of primaries is based on the rules in effect at the time of each diagnosis. The original lymphoma was diagnosed in 1991 and the first transformation to follicular lymphoma in 2008. The pre-2010 rules for coding histology and determining multiple primaries must be applied first because the rules changed for diagnoses occurring 2010 or later. Per the Single Versus Subsequent Primaries Table, poorly differentiated, diffuse lymphocytic lymphoma, small cleaved cell [9591/3] is the same primary as follicular lymphoma [9690].

The Heme DB and Manual are used to confirm that the 2010 recurrences of follicular lymphoma, grade 1 [9695/3], and follicular lymphoma, grade 3 [9698/3], are the same primary according to the Heme Calculator check required per Rule M15. Per the Heme DB page, the diagnoses follicular lymphoma, grade 3 [9698/3] and follicular lymphoma, grade 1 [9695/3] are comparable to follicular lymphoma [9690] as stated in the 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.

 2013
20130092

Reportability--Head & Neck: What are the correct site and histology codes if a glomus tympanicum tumor of the middle ear is reportable?

Glomus tympanicum tumors of the middle ear are not reportable. The 2005 WHO Classification of Head and Neck Tumors classified these tumors as a borderline [/1] behavior and recorded them in the ICD-O-3 with histology code 8690 [glomus jugulare tumor, NOS].

According to WHO, "the distinction between jugular and tympanic paragangliomas can easily be made in the patient by modern imaging methods ... the jugular neoplasm is identified as arising from the jugular bulb region ... while the tympanic neoplasm is confined to the middle ear." Benign and borderline neoplasms of the middle ear [C301] are not reportable. The middle ear is not a reportable CNS site for benign and borderline tumors.

 2013
20130013

Reportability--Heme & Lymphoid Neoplasms: Is Mast Cell Activation Syndrome (MCAS) reportable?

MCAS has been given an ICD-9 code of 202.60 by our medical record coders. In the Progress Notes, the physicians state this is not the same as systemic mastocytosis. There is no listing for MCAS in the Hematopoietic and Lymphoid Database.

Mast Cell Activation Syndrome (MCAS) is not a reportable neoplasm unless it is specifically stated to be a result of a mast cell proliferative disorder that is reportable.

Per our expert pathologist, MCAS is a relatively new term used for conditions in which patients experience the symptoms of mast cell mediators in the absence of an increase/proliferation of mast cells. The diagnosis of this group of disorders is based in part on a complex of symptoms and on the demonstration of no increase in mast cells. Some of these diseases are difficult to separate from mastocytosis (which is reportable). Currently, this group of disorders is not part of the systemic mastocytosis/mast cell leukemia/mast cell sarcoma spectrum.

 2013
20130152 Primary site/Histology--Brain and CNS: How is the primary site and histology coded for a 2013 diagnosis of squamous cell carcinoma arising in a dermoid cyst of the third ventricle? See Discussion. Patient has a dermoid cyst of the third ventricle of the brain diagnosed in 1998. In 2013 the cyst was removed and was diagnosed as squamous cell carcinoma. An internet search revealed a journal article in the Journal or Neuro-Oncology that states, "Although rare, malignant transformation of intracranial epithelial cysts has a poor prognosis." The combination of site C715 [third ventricle, NOS] and histology 8070/3 [squamous cell carcinoma] fails SEER Edit IF 38_3: Primary site and Morphology Impossible. According to the literature, intracranial squamous cell carcinoma is very rare with most cases arising from a preexisting benign epidermoid cyst. The combination of C71_ and 8070/3 should be allowed. We will submit a request to have this edit revised. 2013
20130202 Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are reported when a solitary plasmacytoma diagnosed in 2010 (T spine) is followed by another solitary plasmacytoma (L spine, different primary site) in 2013? See Discussion.

In the Heme Manual it indicates one is to abstract a second primary when a solitary plasmacytoma (chronic) is followed by a plasma cell myeloma (acute) greater than 21 days after the chronic diagnosis.

The Heme Manual does not indicate what to do when a solitary plasmacytoma diagnosed in 2010 (T spine) is followed by another solitary plasmacytoma (L spine, different primary site) in 2013. The physician specifically stated the patient does not have multiple myeloma. Is this case one or two primaries?

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

Per Rule M2, this is a single primary. According to Rule M2, the single histology is always the 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
20130199

MP/H Rules/Multiple primaries--Breast: Does breast Rule M10, 'Tumors that are lobular (8520) and intraductal or duct are a single primary" apply if you have two tumors in the same breast, one ductal and the other tubulolobular (8524) or are they separate primaries per Rule M12?

Apply Rule M10 to this case. Tubulolobular is now classified as a variant of lobular. Code to lobular, NOS (8520) because Tubulolobular does not have a specific ICD-O-3 code.

 2013
20130116 Histology/Primary site--Heme & Lymphoid Neoplasms: How are the histology and primary site coded if a pleurocentesis is compatible with plasmablastic plasmacytoma/lymphoma when no further information is available?

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

Code the histology to 9735/3 [plasmablastic lymphoma] and the primary site to C809 [unknown] per Rule PH27.

Code the histology specified when the only histology for the case is preceded by ambiguous terminology. For this case, code the histology to plasmablastic lymphoma because it is the only histology mentioned in the diagnosis.

Per the Heme DB Abstractor Notes section for plasmablastic lymphoma, most patients present with Stage III-IV disease. The positive pleural fluid is likely due to advanced disease. In the absence of any other information for this case, Rule PH27 applies, "Code primary site to unknown primary site 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 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
20130104 Primary site--Heme & Lymphoid Neoplasms: How is the primary site coded for a diagnosis of intrasinusoidal diffuse large B-cell lymphoma involving lymph nodes, the liver and the bone marrow? See Discussion.

Intrasinusoidal DLBCL was diagnosed by liver biopsy. The bone marrow was involved based on abnormal cytogenetic findings. Per a physician's note, a PTA CT Abd/Pelvis showed hepatosplenomegaly and mild periportal/peripancreatic lymphadenopathy. A GI physician stated the lymphoma involves the veins of the liver.

Should the primary site be coded to the liver [C220] and the histology to 9680/3 [DLBCL]?

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

Code the primary site to the intra-abdominal lymph nodes [C772] per Rule PH20.

Code the primary site to the specific lymph node region when multiple lymph node chains within the same region as defined by the ICD-O-3 are involved. Periportal and peripancreatic nodes are both intra-abdominal region nodes.

Based on the information provided, there is involvement of lymph nodes, the liver, spleen and bone marrow, but no other documentation of the primary site. Given that a primary lymphoma of the liver is very rare; it is unlikely that this lymphoma arose from the liver. Involvement of the liver and spleen is very common for patients with lymphoma. The involvement of the liver, spleen and bone marrow is coded in the CS fields as Stage IV 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
20130208

Histology--Heme & Lymphoid Neoplasms: How is histology coded when a bone marrow shows slightly hypercellular marrow with acute myeloid leukemia, non-M3 type and the flow cytometry is also consistent with acute myeloid leukemia, non-M3 type?

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

Without further information as to the type of acute myeloid leukemia, code the histology to 9861/3 [acute myeloid leukemia, NOS]. If further information on the specific acute myeloid leukemia becomes available, update the histology code.

Document that the pathology report states the acute myeloid leukemia is a "non-M3 type" in a text field. This documentation will help explain the choice of 9861/3 for this case. M3 refers to one of the eight FAB subtypes described by a group of French, American, and British leukemia experts in the 1970's who divided acute myeloid leukemias into subtypes, M0 through M7. They classified the disease based on the type of cell from which the leukemia developed and how mature the cells were. This was based largely on how the leukemia cells looked under the microscope after routine staining.

In this case, all we know is that the histology does not pathologically represent the M3 (acute promyelocytic leukemia (APL)) form of acute myeloid leukemia. We do not know which type of acute myeloid leukemia it does represent.

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