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| Report | Question ID | Question | Discussion | Answer | Cancer Site Category | Data Item Category | Year |
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20170044 | Histology--Sarcoma: What is the histology code for epithelioid angiosarcoma? |
Assign 9120/3 for epithelioid angiosarcoma. |
Sarcoma | Histology | 2017 | |
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20150052 | Primary Site--Sarcoma: What is the best primary site code for an undifferentiated sarcoma of the pulmonary artery? See discussion. |
Consolidation of the case: The operating hospital stated: SOFT TISSUE: Resection: Procedure: Radical resection Other: Pneumonectomy Tumor Site: Right pulmonary artery - They used code C383 (mediastinum NOS). The consulting hospital stated: Lung, right, pneumonectomy: High grade sarcoma consistent with intimal sarcoma; tumor involves pulmonary artery. They used code C449 (other soft tissue NOS). Would C493 (soft tissue thorax) be correct? |
Code the primary site to pulmonary artery, C493. According to the WHO classification of tumors, intimal sarcomas are malignant mesenchymal tumors arising in large blood vessels. They show mostly intraluminal growth with obstruction of the vessel. They may occur in the pulmonary vessels or, less often, in the aorta. |
Sarcoma | Primary site | 2015 |
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20190063 | Solid Tumor Rules (2018)/Histology--Sarcoma: How is histology coded for a CIC gene rearrangement sarcoma? See Discussion. |
According to the literature, CIC gene rearrangement sarcomas in young patients are soft tissue sarcomas with an aggressive clinical course and may have previously been grouped under the Ewing-like family of tumors or as undifferentiated round cell sarcomas. There is currently no guideline in the solid tumor rules for coding a CIC gene rearrangement sarcoma. However, coding the histology to 8800 (sarcoma, NOS) seems unlikely to capture the more aggressive nature of these tumors. Can a more specific histology be coded? |
Code as undifferentiated round cell sarcoma (8803/3). The CIC rearrangement exists as a distinct molecular and clinical subset of small round cell tumors, and though similar, is felt to be a distinct entity from Ewing sarcoma. According to WHO Classification of Soft Tissues and Bone, 4th Edition, CID-DUX4 is a recurrent gene fusion associated with pediatric round cell undifferentiated soft tissue sarcoma (USTS). Although the genes involved in the fusion are different from those in Ewing sarcoma, the CIC-DUX4 protein has been shown to upregulate genes of the ETS family of genes thus providing a molecular link between Ewing sarcoma and round cell USTS. In contrast, there are strong arguments to suggest that Ewing-like sarcomas represent a separate and distinct entity. |
Sarcoma | Histology | 2019 |
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20051040 | Primary Site--Sarcoma: What is the correct topography code for a partial lung lobectomy with pathology diagnosis of "pulmonary sarcoma with smooth muscle differentiation"? See Discussion. | Operative report: palpable 2x2cm mass in the mediastinal surface of the rt middle lobe and the contiguous upper lobe together.
Path comment after partial lung lobectomy: In all likelihood this is a malignant process occurring in smooth muscle changes surrounding vessels within the lung versus an undifferentiated epithelial tumor.
ADDENDUM DX: low grade pulmonary sarcoma with smooth muscle differentiation.
Consultant's report concurs with that of the original pathologist's report of malignant neoplasm compatible with smooth muscle origin. |
This case is unique. Assign topography code C493 [Connective, subcutaneous and other soft tissue of thorax]. Based on the information provided, this sarcoma has smooth muscle differentiation and originated in the muscle. Code the primary site to muscle. | Sarcoma | Primary site | 2005 |
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20031051 | Histology (Pre-2007)/Sarcoma: What code is used to represent the histology "Ewing's Sarcoma/Primitive Neuroectodermal Tumor (PNET)"? See Description. | A comment on one path report states "some authors consider both Ewing's & PNET to be the same biologic entity given that they share the same translocation between chromosomes 11 & 22." The pathologists at our children's hospital agree with this statement and contend that the two should have the same histologic code. | For tumors diagnosed prior to 2007:
Code histology as 9260/3, Ewing sarcoma. Ewing sarcoma is a specific histology on the continuum of primitive neuroectodermal tumors. Code Ewing sarcoma as it is more specific than PNET, NOS.
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. |
Sarcoma | Histology | 2003 |
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20160044 | MP/H Rules/Histology--Sarcoma: What is the appropriate histology code for a final diagnosis of undifferentiated pleomorphic sarcoma and/or pleomorphic sarcoma, undifferentiated? See Discussion. |
Does the Other Sites MP/H Rule H17 apply in this case, which results in coding the higher histology 8805/3 (undifferentiated sarcoma)? Or does the "undifferentiated" statement only refer to grade, which results in coding histology to 8802/3 (pleomorphic sarcoma)? |
Assign 8802/34 to pleomorphic cell sarcoma/undifferentiated pleomorphic sarcoma. Pleomorphic is more important than undifferentiated when choosing the histology code in this case. Undifferentiated can be captured in the grade code. |
Sarcoma | Histology | 2016 |
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20081120 | MP/H Rules--Sarcoma: How many primaries should be abstracted for chondrosarcoma of right toe in 2002, of right lower leg in 2006 and right tibia in 2007? See Discussion. | A patient had a myxoid chondrosarcoma of the right toe in 2002. This was amputated and staged as T2 - high grade. Patient had a recurrence in the lower right leg in 2006. At this time he had a below knee amputation. The tumor in 2006 was stated to be similar histologically to the 2002 tumor with pathologic comparison done. Then in 2007 the patient presents with pain in right knee and stump. CT says compatible with recurrent disease, but no copies of path sent. Patient then had an above knee amputation, with diagnosis of clinically recurrent chondrosarcoma of tibia. How many primaries should be abstracted? Is 2007 diagnosis a new primary? | For cases diagnosed 2007 or later: Abstract two primaries in this case, 2002 and 2007. The first primary was diagnosed in 2002. The 2006 diagnosis would not be a new primary according to the rules in effect at that time (2004 SEER manual, page 11, rule 5, exception 1). Use the current MP/H rules to compare the 2007 diagnosis to the 2002 diagnosis. Start with rule M3 and stop at rule M10. The 2007 diagnosis is a separate primary. |
Sarcoma | N/A | 2008 |
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20140050 | MP/H Rules/Histology--Sarcoma: What would be the morphology code for a low grade myofibroblastic sarcoma of the left distal forearm? I tried several different combinations but the closest I could come up with is myosarcoma. |
Assign code 8825/3. Apply the ICD-O-3 Matrix Concept, Rule F, page 29 of the hardcover ICD-O-3. The WHO Classification of Soft tissue and Bone, page 85, lists low grade myofibroblastic sarcoma, also called myofibrosarcoma, 8825/3. |
Sarcoma | Histology | 2014 | |
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20051056 | 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. |
Sarcoma | Histology | 2005 | |
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20140048 | MP/H Rules/Histology--Sarcoma: Is 8811/3 the correct code for myxofibrosarcoma (myxoid malignant fibrous histiocytoma) high-grade (grade 3/3)? |
8811/3 is the correct code for myxofibrosarcoma. See Rule J on page 33 in ICD-O-3. Fibromyxosarcoma is equivalent to myxofibrosarcoma. |
Sarcoma | Histology | 2014 |
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