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Young age at presentation and a personal and/or family history of cancer suggest an underlying germline mutation. It is important to identify these patients for genetic counselling and surveillance for other disease manifestations.
American Joint Committee on Cancer (AJCC), Chicago, Illinois. The original source for this material is the AJCC Cancer Staging Handbook, Seventh Edition (2010) published by Springer Science and Business Media LLC,
Soft Tissue Sarcoma (STS) – Non-metastatic, Non-GIST, Non-Fibromatosis, non-angiosarcoma
Ewing sarcoma (ES) comprises a group of small round cell sarcomas with characteristic molecular findings consisting of non-random chromosomal translocations between the EWSR1 gene on chromosome 22 (or its FUS homolog) and one of several genes belonging to the ETS family of transcription factors (most commonly FLI1).
The diagnosis of ES encompasses lesions previously classified as Askin’s tumours, peripheral neuroectodermal tumours (PNET) and other names that are no longer part of the WHO classification.
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