Restaging role of 18F-FDG-PET/CT in a patient with a Malignant Peripheral Nerve Sheath Tumor (MPNST) caused by neurofibromatosis – case report

Abstract

Malignant Peripheral Nerve Sheath Tumors (MPNST) are a malignant disease that originates in the cells that cover and protect the peripheral nerves. They are a rare disease, accounting for 6% of soft tissue sarcomas. We present a patient with a malignant peripheral nerve sheath tumor on the left brachium with neurofibromatosis. The patient underwent whole body PET/CT for restaging after total extirpation of a tumor formation originated from n. musculocutaneous in 09.2017, an excision of a recurrent tumor formation in the proximal half of the brachium invading into m. biceps brachii in 12.2017, a performed MRI in 02.2018 - with data for multiple MPSNT in the area between the two previous operations - and subsequent definitive radiotherapy (boost) in the area of the tumor formation and 6 cycles of chemotherapy with Epirubicin. After restaging PET/CT scan of the whole body was performed, progression of the disease was defined - increased in size and activity subpleural lesion in the lower lobe of the right lung.