Intra Peritoneal Malignant Triton Tumor: A Case Report and Literature Review

Abstract

BACKGROUND: Malignant triton tumor (MTT) is a variant of the typical malignant peripheral nerve sheath tumors with rhabdomyoblastic differentiation what mostly occur in the context of neurofibromatosis type 1. CASE PRESENTATION: Here, we report on a case of a 15-year-old male with a clinical history of neurofibromatosis type 1 who presented to the pediatric emergency department with an intraperitoneal tumor of about 20 cm of diameter. The biopsy of this tumor confirmed histology of rhabdomyosarcoma. The patient received six cycles of chemotherapy. After that, resection of the tumor was performed. Histological findings revealed a malignant peripheral nerve sheath tumors (MPNST) of Triton type. Then he received 28 sessions of radiation therapy. The child died 11 months after radiation therapy. CONCLUSION: Malignant triton tumor is a very aggressive tumor. A multidisciplinary approach is required for diagnosis and treatment of this rare pediatric tumor.