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Ewing Sarcoma in a Patient With Cowden Syndrome.

  • Author(s): Chandhanayingyong, Mod C
  • Bernthal, Nicholas M
  • Ungarreevittaya, Piti
  • Nelson, Scott D
  • Chawla, Sant P
  • Singh, Arun S
  • et al.

A 47-year-old woman, initially diagnosed in 1996 with Cowden syndrome (CS), PTEN-mutant bilateral breast cancer, a thyroid nodule, and uterine fibroids, presented to UCLA in 2013 with Ewing sarcoma of the pelvic bone. Her treatment course included mastectomies, hysterectomy/oophorectomy, and total thyroid resection, and chemotherapy, radiation, and hemipelvectomy for Ewing sarcoma. This case report illustrates the unusual presentation of Ewing sarcoma in a patient with PTEN-mutant CS, the probable underlying molecular pathogenesis, long-term management, and therapeutic considerations.

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