
Primitive Neuroectodermal Tumour: A Rare Association with Neurofibromatosis Type 1
Author(s) -
Hatice Gümüş,
Metehan Gümüş,
Uğur Fırat,
Hakan Önder,
Fatih İnci
Publication year - 2015
Publication title -
the annals of clinical and analytical medicine
Language(s) - English
Resource type - Journals
ISSN - 2667-663X
DOI - 10.4328/jcam.867
Subject(s) - medicine , neurofibromatosis , association (psychology) , neurofibromatosis type i , pathology , dermatology , philosophy , epistemology
Neurofibromatosis type 1 (NF–1), is a common hereditary disease which often take the form of benign tumours. Spinal primitive neuroectodermal tumour (PNET) is very rare. Herein we report US, CT and MRI findings of a female patient with PNET involving the spinal epidural and paravertebral spaces with neurofibromatosis type 1. A 37-year-old woman has NF–1 presented with lower back pain and progressive weakness of the right lower extremities. US revealed a heterogenous well defined solid mass. CT and MRI revealed heterogenously enhanced mass originating from the right neural foramen at the level of L5–S1. Adjacent to the lesion, there was a large hernia sac, including bowel loops and mesentery, in the posterolateral abdominal wall. The definitive diagnosis was made by transabdominal percutaneous tru-cut biopsy. Although it is rare, the possibility of PNET should be kept in mind in assessing the spinal epidural lesions