PMID- 2987425
OWN - NLM
STAT- MEDLINE
DCOM- 19850628
LR  - 20190908
IS  - 0167-594X (Print)
IS  - 0167-594X (Linking)
VI  - 3
IP  - 1
DP  - 1985
TI  - Primitive neuroectodermal tumours of the cerebrum. Pathology and treatment.
PG  - 23-33
AB  - Eighteen cases of cerebral tumour composed partly or totally of primitive 
      embryonal cells are reported. These lesions comprise 2.8% of all primary cerebral 
      hemisphere tumours in the histopathology files of The Royal Marsden Hospital 
      between 1971 and 1980 inclusive. Most exhibited some degree of differentiation 
      towards neuronal or glial elements and, as more than one type of differentiation 
      was often present in the same lesion, we agree with others that the term 
      primitive neuroectodermal tumour (PNET) is more appropriate to describe these 
      lesions than terms based on histogenesis. The extent of the primitive component 
      varied, but usually accounted for more than 80% of the tumour. Although the 
      tumours bear some similarities to posterior fossa medulloblastomas, they exhibit 
      important differences in histology, immunohistology, natural history and response 
      to treatment. Nearly all PNETs examined expressed some glial fibrillary acidic 
      (GFAP) both in primitive areas and zones of astrocytic differentiation. GFAP 
      staining may thus be of value in distinguishing PNETs from undifferentiated 
      non-neurogenic tumours. Of 14 patients referred for radiotherapy, the survival 
      rate at 3 years was 29% (4/14) and 5 years 25% (3/12). Patients with tumours in 
      which at least 90% of the tissue was undifferentiated exhibited an extremely poor 
      prognosis with none of 9 patients still alive at 3 years in contrast to 3 of 5 
      patients (60%) with tumours showing less than 90% undifferentiation. Radical 
      tumour removal, where feasible, followed by irradiation of the whole 
      cerebrospinal axis is recommended. Adjuvant chemotherapy with such agents as CCNU 
      and Vincristine may be of value: the 3 long term survivors in the present series 
      (7-11 years), including one who presented disseminated intracranial disease, 
      received such adjuvant treatment.
FAU - Gaffney, C C
AU  - Gaffney CC
FAU - Sloane, J P
AU  - Sloane JP
FAU - Bradley, N J
AU  - Bradley NJ
FAU - Bloom, H J
AU  - Bloom HJ
LA  - eng
PT  - Journal Article
PL  - United States
TA  - J Neurooncol
JT  - Journal of neuro-oncology
JID - 8309335
RN  - 0 (Glial Fibrillary Acidic Protein)
RN  - 0 (Reticulin)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Astrocytes/ultrastructure
MH  - Brain/pathology
MH  - Brain Neoplasms/*pathology/therapy
MH  - Cerebellar Neoplasms/pathology
MH  - Child
MH  - Child, Preschool
MH  - Endothelium/pathology
MH  - Ependyma/pathology
MH  - Female
MH  - Glial Fibrillary Acidic Protein/metabolism
MH  - Humans
MH  - Immunoenzyme Techniques
MH  - Infant
MH  - Male
MH  - Medulloblastoma/pathology
MH  - Necrosis
MH  - Neoplasms, Germ Cell and Embryonal/*pathology/therapy
MH  - Neurons/ultrastructure
MH  - Oligodendroglia/ultrastructure
MH  - Reticulin/metabolism
EDAT- 1985/01/01 00:00
MHDA- 1985/01/01 00:01
CRDT- 1985/01/01 00:00
PHST- 1985/01/01 00:00 [pubmed]
PHST- 1985/01/01 00:01 [medline]
PHST- 1985/01/01 00:00 [entrez]
AID - 10.1007/BF00165168 [doi]
PST - ppublish
SO  - J Neurooncol. 1985;3(1):23-33. doi: 10.1007/BF00165168.