Neurofibromatosis type 1: brain stem tumours

被引:47
作者
Bilaniuk, LT
Molloy, PT
Zimmerman, RA
Phillips, PC
Vaughan, SN
Liu, GT
Sutton, LN
Needle, M
机构
[1] CHILDRENS HOSP, DIV NEUROL, PHILADELPHIA, PA 19104 USA
[2] CHILDRENS HOSP, DEPT NEUROOPHTHALMOL, PHILADELPHIA, PA 19104 USA
[3] CHILDRENS HOSP, DIV NEUROSURG, PHILADELPHIA, PA 19104 USA
[4] CHILDRENS HOSP, DIV ONCOL, PHILADELPHIA, PA 19104 USA
关键词
D O I
10.1007/s002340050484
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
We describe the clinical and imaging findings of brain stem tumours in patients with neurofibromatosis type 1 (NF1). The NF1 patients imaged between January 1984 and January 1996 were reviewed and 25 patients were identified with a brain stem tumour. Clinical, radiographical and pathological results were obtained by review of records and images. Brain stem tumour identification occurred much later than the clinical diagnosis of NF1. Medullary enlargement was most frequent (68 %), followed by pontine (52 %) and midbrain enlargement (44 %). Patients were further subdivided into those with diffuse (12 patients) and those with focal (13 patients) tumours. Treatment for hydrocephalus was required in 67 % of the first group and only 15 % of the second group. Surgery was performed in four patients and revealed fibrillary astrocytomas, one of which progressed to an anaplastic astrocytoma. In 40 % of patients both brain stem and optic pathway tumours were present. The biological behaviour of brain stem tumours in NF1 is unknown. Diffuse tumours in the patients with NF1 appear to have a much more favourable prognosis than patients with similar tumours without neurofibromatosis type 1.
引用
收藏
页码:642 / 653
页数:12
相关论文
共 32 条
[1]   Growth rate characteristics of acoustic neuromas associated with neurofibromatosis type 2 [J].
Abaza, MM ;
Makariou, E ;
Armstrong, M ;
Lalwani, AK .
LARYNGOSCOPE, 1996, 106 (06) :694-699
[2]   NEUROFIBROMATOSIS TYPE-1 AND TYPE-2 - CRANIAL MR FINDINGS [J].
AOKI, S ;
BARKOVICH, AJ ;
NISHIMURA, K ;
KJOS, BO ;
MACHIDA, T ;
COGEN, P ;
EDWARDS, M ;
NORMAN, D .
RADIOLOGY, 1989, 172 (02) :527-534
[3]   BRAIN-STEM GLIOMAS - A CLASSIFICATION-SYSTEM BASED ON MAGNETIC-RESONANCE-IMAGING [J].
BARKOVICH, AJ ;
KRISCHER, J ;
KUN, LE ;
PACKER, R ;
ZIMMERMAN, RA ;
FREEMAN, CR ;
WARA, WM ;
ALBRIGHT, L ;
ALLEN, JC ;
HOFFMAN, HJ .
PEDIATRIC NEUROSURGERY, 1991, 16 (02) :73-83
[4]   COMPUTED-TOMOGRAPHY OF BRAIN-STEM GLIOMAS IN CHILDREN [J].
BILANIUK, LT ;
ZIMMERMAN, RA ;
LITTMAN, P ;
GALLO, E ;
RORKE, LB ;
BRUCE, DA ;
SCHUT, L .
RADIOLOGY, 1980, 134 (01) :89-95
[5]  
BLATT J, 1986, CANCER, V57, P1225, DOI 10.1002/1097-0142(19860315)57:6<1225::AID-CNCR2820570627>3.0.CO
[6]  
2-P
[7]  
BOGNANNO JR, 1988, AM J ROENTGENOL, V151, P381
[8]   MRI (NMR) IN THE DIAGNOSIS OF BRAIN-STEM TUMORS [J].
BRADAC, GB ;
SCHORNER, W ;
BENDER, A ;
FELIX, R .
NEURORADIOLOGY, 1985, 27 (03) :208-213
[9]   MR OF CENTRAL-NERVOUS-SYSTEM NEOPLASIA OF THE PHACOMATOSES [J].
BRAFFMAN, BH ;
BILANIUK, LT ;
ZIMMERMAN, RA .
SEMINARS IN ROENTGENOLOGY, 1990, 25 (02) :198-217
[10]  
Cohen B H, 1989, Oncology (Williston Park), V3, P23