Primary ovarian peripheral primitive neuroectodermal tumor presented with breast metastasis; Case report

Area of Science:

• Oncology
• Pathology
• Medical Imaging

Background:

• Ewing sarcoma family tumors (ESFT) are rare and challenging to diagnose, particularly extraskeletal forms.
• Metastatic ESFTs are associated with poor prognoses despite therapeutic advancements.

Observation:

• A 21-year-old woman presented with a breast mass and abdominal distention, initially suspected as primary breast cancer.
• Biopsy revealed metastatic extraskeletal Ewing sarcoma in the breast, with the primary tumor identified as originating in the ovary.
• Imaging confirmed widespread metastasis, including to the dura, spine, and bone, with optic nerve involvement.

Findings:

• Histopathology and immunohistochemistry confirmed Ewing sarcoma, originating from the ovary and metastasizing to the breast.
• Initial chemotherapy (VDC/IE) achieved tumor cytoreduction, but disease progression occurred within two years.
• Subsequent treatment with radiotherapy and high-dose ifosfamide chemotherapy was ineffective, leading to patient demise.

Implications:

• This case highlights the diagnostic difficulties and aggressive nature of metastatic extraskeletal Ewing sarcoma.
• Effective treatment protocols for advanced ESFT remain elusive, necessitating further research.
• Improved management strategies are crucial for enhancing outcomes in patients with metastatic ESFT.