Abstract
Current guidelines are vague for the management of soft tissue sarcomas, specifically malignant peripheral nerve sheath tumors (MPNST), regarding staging the disease with the use of routine abdominal imaging. The most recent guidelines from the National Comprehensive Cancer Network (NCCN) recommends to "consider" abdominal/pelvic CT imaging for certain sub groups of sarcomas (e.g., myxoid/round cell liposarcoma, epithelial sarcoma, angiosarcoma, leiomyosarcoma), but provide no guidance on other sarcoma subtypes regardless of tumor size. We report a case of a very large large MPNST in a 40 year-old-female with neurofibromatosis type 1 who was incidentally found to have adrenal metastasis.