Malignant peripheral nerve sheath tumours of the pericardium in a patient with Neurofibromatosis type 1: The diagnostic value of 18F-FDG PET-CT and I-123 MIBG SPECT-CT.

ABSTRACT

A 25 year old female with known neurofibromatosis type 1 with a large anterior mediastinal mass was investigated. F18-FDG PET-CT revealed a radiotracer avid anterior mediastinal mass with SUVmax of 4.3 and demonstrating a hypoactive center. The Iodine-123 MIBG SPECT-CT study performed subsequently did not demonstrate any uptake, thereby excluding for the most part the diagnoses of paraganglioma or neuroblastoma. At final pathology, a malignant peripheral nerve sheath tumour (MPNST) of the pericardium with areas of chondrosarcomatous and angiosarcomatous differentiation was diagnosed.