MP/H Rules/Histology--Brain and CNS: What is the histology code for a tumor originating in the cerebellum and extending into the fourth venrticle described as a glioblastoma with primitive neuroectodermal tumor component (WHO Grade IV)?
The WHO Classification of CNS tumours lists glioblastoma with primitive neuroectodermal tumor component as a subtype of glioblastoma and assigns 9440/3. Also referred to as glioblastoma with a primitive neuronal component.
Multiple Primaries (Pre-2007)--Breast: When a breast cancer is treated with less than a total mastectomy and more than 2 months later a tumor of the same histology is diagnosed in the same breast with no statement of "recurrence," is this a new primary?
For tumors diagnosed prior to 2007:
Count as 2 primaries when a subsequent malignant breast tumor is diagnosed more than 2 months later unless stated to be a recurrence. For cases diagnosed after 1/1/94, an in situ followed by an invasive breast cancer is counted as two primaries even if stated to be a recurrence.
For tumors diagnosed 2007 or later, refer to the MP/H rules. If there are still questions about how this type of tumor should be coded, submit a new question to SINQ and include the difficulties you are encountering in applying the MP/H rules.
MP/H Rules/Histology--Colon: What histology would be coded when the right colon demonstrates a combined adenocarcinoma and high grade small cell neuroendocrine carcinoma [forming the dominant component] arising in a villotubular adenoma and the liver biopsy demonstrates metastatic high grade small cell neuroendocrine carcinoma?
For cases diagnosed 2007 or later, start with rule H1 in the Single Tumor module. Stop at rule H4. Assign code 8263 [adenocarcinoma in tubulovillous adenoma].
Stop at the first rule that applies. Code histology based on a specimen from the primary site whenever available.
Reportability/Histology--Heme & Lymphoid Neoplasms: Is Monoclonal B-lymphocytosis of uncertain significance (MLUS) reportable? If so, what is the correct histology code?
Per Appendix F, monoclonal B-lymphocytosis of uncertain significance (MLUS) is not reportable.
Some papers point out that a lymphocyte count less than five thousand is equivalent to monoclonal B-lymphocytosis of uncertain significance (MLUS) or monoclonal B-cell lymphocytosis (MBL). A lymphocyte count of five to thirty thousand could be smoldering chronic lymphocytic leukemia (CLL). The diagnosis of MLUS is a benign process that does not meet the criteria for CLL.
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.
Histology (Pre-2007)--Sarcoma: How is "acral myxoinflammatory fibroblastic sarcoma" coded?
For tumors diagnosed prior to 2007:
The ICD-O-3 histology code is 8811/3 [Fibromyxosarcoma] according to the WHO Classification of Tumours of Soft Tissue and Bone. WHO defines myxoinflammatory fibroblastic sarcoma (MIFS) as "a unique low grade sarcoma with myxoid stroma, inflammatory infiltrate and virocyte-like cells that predominantly involves the hands and feet."
For tumors diagnosed 2007 or later, refer to the MP/H rules. If there are still questions about how this type of tumor should be coded, submit a new question to SINQ and include the difficulties you are encountering in applying the MP/H rules.
Multiple Primaries (Pre-2007)--Testis: How many primaries should be reported when seminoma is diagnosed simultaneously in both testicles and both tumors are encapsulated?
For tumors diagnosed prior to 2007:
Report this cases as two primaries, unless there is information in the record confirming one primary.
For tumors diagnosed 2007 or later, refer to the MP/H rules. If there are still questions about how this type of tumor should be coded, submit a new question to SINQ and include the difficulties you are encountering in applying the MP/H rules.
Primary site--Heme & Lymphoid Neoplasms: Is a peripheral blood equivalent to bone marrow biopsy for the purposes of Rule PH26 and code the primary site to C421 [Bone marrow] for a marginal zone lymphoma found in peripheral blood when there was no additional workup (e.g., scans, etc.) for this case?
Code the primary site to C421 [bone marrow]. Our hematopoietic specialty physicians state that involvement of peripheral blood is equivalent to bone marrow involvement because the marrow produces blood. In the absence of any other involvement, per Module 7 (Coding primary sites for lymphomas) Rule PH26, it states to code the primary site to bone marrow when the only involvement is bone marrow.
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.
EOD-Extension/SEER Summary Stage 2000--Kidney/Eye: What codes are used to represent these fields for simultaneous bilateral Wilms tumor or simultaneous bilateral retinoblastoma?
For cases diagnosed 1998-2003:
Code the EOD-Extension field to 85 [Metastasis] and the SEER Summary Stage 2000 field to 7 [Distant] for both types of tumor. Each kidney and each eye are staged separately in the AJCC, 6th ed., but for SEER we would abstract these diagnoses as one case and code the EOD and stage fields to distant to reflect the involvement of both eyes or both kidneys.
Reportability/Behavior Code--Soft Tissue: Is a final diagnosis of a forearm mass diagnosed as "Angiomatoid malignant fibrous histiocytoma [see note]" reportable? The NOTE reads "Angiomatoid malignant fibrous histiocytoma is a low grade borderline lesion with a tendency for local recurrence, but a very low potential for distant metastases." Is behavior /1 or /3?
Angiomatoid malignant fibrous histiocytoma is reportable with a behavior code of /3 according to ICD-O-3. The Final Diagnosis takes precedence over the "note."
Reportability/MP/H Rules/Histology: Is malignant perivascular epithelioid cell tumor (PEComa) reportable, and if so, what is the histology code?
Malignant perivascular epithelioid cell tumor (PEComa) is reportable because it is malignant. Assign 8005/3 to malignant PEComa.
We consulted an ICD-O-3 expert who explained that some PEComas such as angiomyolipoma and lymphangiomyomatosis have specific ICD-O codes and their malignant counterparts may be coded to 8860/3 and 9174/3 respectively. There are no separate ICD-O codes for other specific PEComas, e.g., clear cell “sugar” tumor of lung, clear cell myomelanocytic tumor of the falciform ligament and some “unusual” clear cell tumors occurring in other organs—or for PEComa, NOS. These PEComas may therefore be coded to 8005 as clear cell tumors NOS; in other words as clear cell tumors that are not clear cell variants of carcinomas, sarcomas, or other specific tumor type.
Please note, PEComa is non-specific as to behavior. Unless the pathologist states that it is malignant, (as was the case for this question), the default code is 8005/1 (non-reportable).
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