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Report Produced: 08/04/2024 02:23 AM

Report Question ID Question Discussion Answer Year
20130206 Primary site--Heme & Lymphoid Neoplasms: What rule applies to code a primary site for a peripheral blood diagnosis of marginal zone lymphoma that has a positive flow cytometry/FISH analysis when no biopsies are performed, scans show no evidence of disease, exam indicates no lymph nodes are palpable and the physician's clinical diagnosis "marginal zone lymphoma, unspecified site, stage 1"? See Discussion.

PE: No palpable lymph nodes.

PET scan: No spleen or lymph node uptake; no uptake anywhere in the body.

Peripheral blood and flow cytometry/FISH analysis diagnosis: Marginal zone lymphoma.

No bone marrow or biopsy of any lymph nodes done. Doctor states "marginal zone lymphoma, unspecified site, stage 1."

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

Per Rule PH27, code the primary site to C809 [unknown primary]. According to Rule PH27 one is to code the 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.

If further workup is done and a primary site is determined, update the primary site for this case.

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
20130085

Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned when a patient was treated in 1999 with Vidaza for myelodysplastic syndrome and had a recent biopsy that demonstrated a transformation to acute myeloid leukemia?

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, acute myeloid leukemia [9861/3].

MDS diagnosed prior to 1/1/2001 is not a reportable disease process. However, because MDS is currently a reportable disease process, it must be considered when trying to determine whether the AML represents a separate primary.

  • If the Heme DB indicates MDS and AML represent different (separate) disease processes, only one primary is reported (i.e., AML) because the 1999 diagnosed MDS is not reportable.
  • If the Heme DB indicates MDS and AML represent the same disease process, then no primaries are reported because MDS was not reportable in 1999.

Rule M2 does not apply to this case because more than one histology is mentioned in the scenario. According to the Heme DB, MDS can transform to AML. Rules M8-M13 apply to cases involving transformation. In this case, Rule M10 applies because the patient was diagnosed with a chronic neoplasm (myelodysplastic syndrome) followed greater than 21 days later by an acute neoplasm (AML).

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
20130113

Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned if a patient diagnosed and treated for multiple myeloma is subsequently diagnosed with multiple large plasmacytomas involving the scalp and thorax? See Discussion

The patient was diagnosed with multiple myeloma, underwent treatment and subsequently was in remission. The patient later presented with lesions on the scalp and thorax lesions. The final diagnosis on the pathology report for the scalp lesion was multiple myeloma with plasmablastic transformation (high grade). The physician states this is an aggressive, recurrent multiple myeloma with multiple large plasmacytomas involving the scalp and thorax.

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

Accession a single primary, multiple myeloma [9732/3] per Rule M2.

The multiple myeloma is in an advanced stage when plasma cells are being deposited on the scalp and thorax. Clinically, those plasma cells are rightly called plasmacytomas by the physician. However, the patient has a late-stage multiple myeloma causing the plasma cells/plasmacytomas. Note that under the myeloma Recurrence and Metastases section of the Heme DB it indicates that extramedullary involvement (e.g., the scalp and thorax involvement) usually indicates advanced disease. Therefore, this scenario represents a case of a single histology that is accessioned as a single primary per Rule M2.

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
20130148 Reportability--Brain and CNS: Are "spinal" schwannomas reportable if stated to be extradural, vertebral nerve sheath, or of specific vertebrae? See Discussion.

Are any of the following cases reportable?

Example 1: Clinical Diagnosis: Extradural spinal cord tumor compatible with schwannoma. What assumptions should be made about reportability if the tumor is described as being extradural? The extradural spinal cord includes epidural fat surrounding the thecal sac and exiting nerve roots. Does this mean there are not nerve roots in the extradural spinal cord?

Example 2: Final Pathologic Diagnosis: Designated "C3-4 nerve sheath tumor" excision: Morphologic and immunohistochemical findings consistent with cellular schwannoma. When stated to be a "nerve sheath tumor" does that mean peripheral nerve (C47_) involvement or nerve root (C72_) involvement?

Example 3: Final Pathologic Diagnosis: T-8 vertebral tumor resection: Schwannoma with degenerative changes (calcification, cyst formation) - ganglion and nerve are identified. There is no mention clinically or pathologically whether this tumor is "intradural" or "of the nerve root." In the absence of information about whether the location of the tumor is intradural or involving the nerve root, is it assumed that it does involve this part of the spinal cord when a specific vertebrae is removed?

Extradural schwannomas are not reportable. Neither vertebral nerve sheath nor a location of/on a specific vertebrae confirm the origin as being either extradural or intradural. Do not report a schwannoma if it cannot be determined to be "intradural" or "of the nerve root." 2013
20130172 Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned and what is the histology for each if a bone marrow diagnosis reveals co-existent systemic mastocytosis and a lymphoplasmacytic neoplasm? See Discussion.

11/7/12 Peripheral blood flow cytometry: small population of clonal CD5- CD10- B-cells consistent with a B-cell lymphoproliferative process.

1/16/13 Bone marrow final diagnosis: co-existent systemic mastocytosis and lymphoplasmacytic neoplasm.

B-cell component of lymphoplasmacytic neoplasm constitutes 20% of bone marrow cellularity and the plasma cell component approximately 20%. The differential diagnosis includes marginal zone lymphoma with plasmacytic differentiation and lymphoplasmacytic lymphoma.

Flow cytometry: kappa monotypic B-cells and plasma cells.

Comment: Co-existence of systemic mastocytosis and mature B-cell lymphoma meets the criteria for Systemic mastocytosis with Associated Clonal Hematological Non-Mast Cell Lineage Disease (SM-AHNMD).

From our physician's progress note: KIT-D816V-positive, CD117+/CD25+ /SM-AHNMD(40% of the nucleated cells as spindled mast cells) but also seemingly two distinct lymphoid neoplasms, a CD5-negative/CD10-negative B-cell lymphoproliferative neoplasm consistent with occupying another 20% of the nucleated marrow space, together with an IgG-kappa-restricted (non-reportable diagnosis) occupying another 20% of the nucleated marrow space (and an accompanying 2.0 g/dl M-spike without hypercalcemia or anemia).

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

Under the Alternate Names section of the Heme DB, systemic mastocytosis with Associated Clonal Hematological Non-Mast Cell Lineage Disease (SM-AHNMD) is a synonym for systemic mastocytosis. Per Rule M2, this is one primary. Abstract a single primary when there is a single histology. Code the histology to 9741/3 [systemic mastocytosis].

Per the pathology report, the two diagnoses of systemic mastocytosis and mantle cell lymphoma meet the criteria for SM-AHNMD. The B-cell lymphoma is a symptom/marker of the AHNMD. In systemic mastocytosis with AHNMD, a myeloid or lymphatic malignancy is diagnosed with the SM. The prognosis is usually dominated by the non-mast cell malignancy.

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
20130024

MP/H Rules/Histology--Bladder: How many primaries are accessioned and what rule applies when the patient has a mixed tumor with a urothelial carcinoma, NOS and a more specific histologic type followed by a diagnosis of urothelial carcinoma? See Discussion.

The MP/H Rules do not specifically cover how to process urothelial carcinomas with a more specific type of carcinoma.

Patient 1: Diagnosed in April 2010 with invasive urothelial carcinoma with signet ring features of the bladder. Site and histology are coded as C679 [bladder] and 8490/3 [signet ring cell carcinoma]. In January 2012 a subsequent diagnosis of invasive urothelial carcinoma of the bladder is made [C679, 8120/3].

Patient 2: Diagnosed in November 2009 with invasive papillary urothelial carcinoma with micropapillary and mucinous features of the bladder. Site and histology are coded C679 [bladder] and 8480/3 [mucinous carcinoma]. In April 2012 a subsequent diagnosis of high grade papillary and flat urothelial carcinoma without evidence of invasion is made [C679, 8130/2].

Does rule M9 apply and these are new primaries?

For cases diagnosed 2007 and later, accession two primaries for each patient, signet ring cell carcinoma of the bladder and invasive urothelial carcinoma of the bladder for patient 1 and mucinous carcinoma of the bladder and non-invasive papillary urothelial carcinoma of the bladder for patient 2.

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

Start at the MULTIPLE TUMORS module, rule M3. The rules are intended to be reviewed in consecutive order within a module. For both patients, rule M9 applies because the tumors have histology codes that are different at the second (xxx) number.

This guideline will be reviewed for the next version of the MP/H Rules.

 2013
20130033

Histology--Heme & Lymphoid Neoplasms: How is the histology coded for a low grade B-cell lymphoma with plasmacytic differentiation?

This answer has been corrected. Previous answer is shown below under "History."

Assign 9591/3 for this case.

See also SINQ 20190070.

 2013
20130177 MP/H Rules/Histology--Bladder: What rule and histology code apply when a TURB final diagnosis is small cell neuroendocrine carcinoma and high grade urothelial carcinoma? See Discussion.

The patient has a 6 cm tumor arising in posterior-lateral bladder extending to prostate, obliterates seminal vesicle, and invades pelvic wall.

TURB final diagnosis: Small cell neuroendocrine carcinoma. High grade urothelial carcinoma involves 10% of tumor.

Following the current MP/H single tumor rules, it appears Rule H8 applies. Per Rule H8, code the numerically higher code of 8120. By following this rule, it does not seem the histology code fairly represents this tumor.

There is currently no rule in the urinary site MP/H Rules for this combination of histologies. The best option is to code the histology to 8045/3 [mixed small cell carcinoma], a combination of small cell with other types of carcinoma. The presence of small cell carcinoma drives the treatment decisions for this case.

This issue will be addressed in the next revision of the MP/H Rules.

2013
20130084 Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned when a patient is diagnosed on a 3/16/12 lymph node biopsy with diffuse large B-cell lymphoma which was followed on 4/18/12 with bone marrow biopsy diagnosis of follicular lymphoma? See Discussion. The patient has extensive right-sided cervical, supraclavicular, hilar, mediastinal and gastrohepatic adenopathy. A cervical node biopsy on 3/16/2012 showed DLBCL. On 04/18/2012 a bone marrow biopsy showed follicular lymphoma. The patient was started on CHOP/Rituxan after the bone marrow biopsy.

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

This case is accessioned as a single primary, diffuse large B-cell lymphoma [9680/3] per Rule M12. Abstract the acute neoplasm (DLBCL) when a patient is originally diagnosed with an acute neoplasm and the neoplasm reverts to the chronic neoplasm (follicular lymphoma) AND the patient has not been treated for the acute neoplasm.

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
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