Retroperitoneal "Triton" tumor

Malignant Triton tumor is a malignant peripheral nerve sheat tumor with rhabdomioblastic differentiation. These tumors are frequently associated with Neurofibromatosis type 1, sporadic cases being exteremly rare. Retroperitoneal localization have the most unfavorable prognois due to delayed diagnosis but also due to relation to adjacant organs. Preoperative diagnosis is inaccurate, but core needle biopsy gives more promising results. Aggressive surgical management remains the most effective modality since adjuvant forms of treatment like irradiation or chemotherapy do not have reproducible results. We present a 60-year-old female patient in whom a retroperitoneal presacral mass was postoperatively diagnosed as Triton tumor. At time of diagnosis, no visible metastases were present. The posterior pelvic exenteresis was performed. Intended chemotherapy was never started since multiple pulmonary, hepatic and splenic metastases were diagnosed only a month after surgery, with rapid lethal outcome. This case demonstrates the bad prognosis of malignant retroperitoneal tumors. Diagnostic tools such as refined biopsy techniques or cytogenetic analysis might help in differentiating patients who will benefit from radical surgery.