Advanced Primitive Neuroectodermal Tumor (PNET)/Extraskeletal Ewing Sarcoma of the Vagina with Bone Metastasis and Multi-Organ Failure: A Case Report

Ewing's sarcoma and Primitive Neuroectodermal Tumors (PNETs) are rare round cell sarcomas, with vaginal PNETs being particularly uncommon. Only ۱۴ cases have been reported from ۱۹۶۰ to ۲۰۲۰. This report presents a ۲۶-year-old female with a vaginal PNET, exhibiting progressive symptoms and advanced disease at diagnosis. The patient experienced back pain, vaginal discharge, and spotting for seven months. Examination revealed a bleeding vaginal mass diagnosed as PNET. Imaging showed a large tumor with pelvic bone marrow involvement, lymphadenopathy, hydronephrosis, and metastatic bone disease. Laboratory tests indicated anemia, leukocytosis, and hypercoagulability. Despite treatment, the patient’s condition rapidly worsened, leading to respiratory distress, multi-organ failure, and death. This case highlights the aggressive nature and poor prognosis of vaginal PNETs and emphasizes the need for early diagnosis and awareness to improve management outcomes.Case Report: The patient initially experienced low back pain, vaginal discharge, and spotting for seven months before seeking medical attention. Examination revealed a friable, easily bleeding vaginal mass, which was diagnosed as PNET through biopsy and immunohistochemistry. Imaging studies confirmed a large malignant mass involving the vagina, with associated pelvic bone marrow replacement, para-iliac lymphadenopathy, Grade II left hydronephrosis, and metastatic bone disease affecting the proximal right humerus and femur. Laboratory findings showed severe anemia, leukocytosis, and elevated inflammatory markers, indicating a hypercoagulable state. Despite efforts to manage the disease, the patient’s condition rapidly deteriorated, leading to severe respiratory distress, multi-organ failure, and death. This case illustrates the aggressive nature of vaginal PNETs, characterized by widespread metastases and systemic complications. The advanced stage at diagnosis and the presence of poor prognostic factors highlights the challenges in managing such rare malignancies. Early diagnosis and comprehensive treatment strategies are essential to improve patient outcomes, though prognosis remains poor in advanced stages. Conclusion: This case contributes to the limited literature on vaginal PNETs and underscores the importance of raising clinical awareness for timely recognition and management of these rare tumors.

کلیدواژه ها:

Extraosseous Ewing&rsquo ، s sarcomas ، PNETs ، Vagina ، Case report