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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.

8802/3 applies to soft tissue tumors and 8830/3 applies to tumors arising in bone. The 2018 ICD-O update lists undifferentiated pleomorphic sarcoma as code 8802/3 and 8830/3 applies to undifferentiated high grade pleomorphic sarcoma of bone and is specific to C40 _. This is still valid in ICD-O-3.2. The 2018 update also noted undifferentiated pleomorphic sarcoma, NOS was a new term for 8830 based on WHO documentation available at that time. However that is incorrect and ICD-O-3.2 provides the correct codes.

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.

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.

Code the EOD-Extension field to 13 [Visceral (e.g., pulmonary, gastrointestinal tract, spleen, other)], because of the positive bone marrow. Code the EOD-Lymph Nodes field to 3 [Both clinically enlarged palpable lymph nodes (adenopathy) and pathologically positive lymph nodes], for the pathologically positive node.

Note: Potential revision of the extension scheme will be referred to SEER Medical Advisory Group (SMAG).

Apply 2007 MP/H rule H6 and assign the numerically higher ICD-O-3 code that reflects PNET (9364/3).

According to the WHO Tumors of Soft Tissue and Bone, though Ewing sarcoma ICD-O-3 code is 9260/3, Ewing sarcoma with a higher degree of neuroectodermal differentiation present is classically termed peripheral neuroectodermal tumors (PNET).  WHO does not offer guidance how to classify tumors stated to be Ewing sarcoma PNET.

Histology code 9364/3 is assigned for a Ewing/PNET that arises outside of the brain/CNS. Peripheral neuroectodermal tumor (PNET) and peripheral primitive neuroectodermal tumor (PPNET) are Ewing family tumors.

Histology code 9473/3 (PNET, primitive neuroectodermal tumor, central primitive neuroectodermal tumor, or supratentorial PNET) is only used for tumors arising inside the brain/CNS.

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.

This case is reportable. The diagnosis states "suspicious for adenocarcinoma." "Suspicious for" is a reportable ambiguous term.

The additional stains supported this "suspicious" diagnosis. A more definitive diagnosis could not be made based on this specimen.

Assuming the smal cell is a seperate tumor, accession two primaries, adenocarcinoma (8140/3) of the prostate and SmCC (8041/3) of the prostate using Rule M19 of the current Other Sites Solid Tumor Rules.  As these two tumors are less than a year apart, Rule M4 does not apply; however, Rule 19 does apply as these are two distinct histology types. It takes time for an acinar tumor to transform into the small cell and it is usually triggered by hormone and/or radiation treatment.