Peripheral malignant neuroectodermal tumors are rare and agressive sma
ll-cell tumors seen predominantly in children and young adults. Medias
tinal location is very uncommon. A mediastinal primitive neuroectoderm
al tumor was diagnosed in a 27-year-old man. Despite treatment combini
ng surgery, radiotherapy and chemotherapy, disease recurred locally al
ong with lungs, spinal and epidural metastases, leading to death. CT a
nd MRI examinations show heterogenous masses with necrosis, hemorrhage
and intense enhancement after Gadolinium injection. The CT and MRI fi
ndings are not specific and the diagnosis is based on pathologic, immu
nohistochemical and electron microscopic features. Although uncommon,
peripheral neuroectodermal tumor should be considered in the different
ial diagnosis of posterior mediastinal masses in children and young ad
ults.