PMID- 16373056
OWN - NLM
STAT- MEDLINE
DCOM- 20060524
LR  - 20191109
IS  - 1699-048X (Print)
IS  - 1699-048X (Linking)
VI  - 7
IP  - 10
DP  - 2005 Nov
TI  - Lumbar region intra-spinal primitive neuroectodermal tumour (PNET) combined with 
      neurofibromatosis type 1.
PG  - 464-7
AB  - Primitive neuroectodermal tumours (PNET) are aggressive neoplasias that are 
      diagnosed, usually, in infancy. Their appearance in adulthood is rare and, 
      exceptionally, in association with neurofibromatosis type I (NF-1). We present a 
      case of a 37 year-old man with NF-1 combined with PNET in the intra-arachidial 
      lumbar region. Diagnosis was by Nuclear Magnetic Resonance (NMR) and biopsy of 
      soft tissue mass which showed a PNET with undifferentiated round cells and 
      immunohistochemically positive for CD99, neurone-specific enolase, synaptophys in 
      and LEU-7. Surgery was performed with spine decompression and resection of 80% of 
      the tumour, with symptoms improvement. Radiotherapy was administered on the 
      lumbosacral column, but only up to 30 Gy because of severe actinic enteritis and 
      pan-cytopenia grade III. Six months later, the patient was hospitalized with 
      deterioration in his overall clinical status with multi-organ involvement. The 
      patient died and an autopsy was performed. The initial treatment of the PNET is 
      surgery and, if possible, the radical extirpation of the tumour. Administration 
      of radiotherapy and chemotherapy appears to increase survival. We comment on the 
      clinical, histological, cytological and immunohistochemical aspects together with 
      a review of the literature. To the best of our knowledge this is the first 
      documentation of such a case.
FAU - Bohn Sarmiento, Uriel
AU  - Bohn Sarmiento U
AD  - Servicio de Oncologia Medica, Hospital General Universitario de Gran Canaria Dr. 
      Negrin. Las Palmas de Gran Canaria, Espana. ubosar@telefonica.net
FAU - Aguiar Bujanda, David
AU  - Aguiar Bujanda D
FAU - Camacho Galan, Rafael
AU  - Camacho Galan R
FAU - Rivero Vera, Jose C
AU  - Rivero Vera JC
FAU - Aguiar Morales, Jose
AU  - Aguiar Morales J
LA  - eng
PT  - Case Reports
PT  - Journal Article
PT  - Review
PL  - Italy
TA  - Clin Transl Oncol
JT  - Clinical & translational oncology : official publication of the Federation of 
      Spanish Oncology Societies and of the National Cancer Institute of Mexico
JID - 101247119
SB  - IM
MH  - Adult
MH  - Humans
MH  - Lumbar Vertebrae
MH  - Male
MH  - Neoplasms, Multiple Primary/*diagnosis
MH  - Neuroectodermal Tumors, Primitive/*diagnosis
MH  - Neurofibromatosis 1/*diagnosis
MH  - Spinal Cord Neoplasms/*diagnosis
RF  - 13
EDAT- 2005/12/24 09:00
MHDA- 2006/05/25 09:00
CRDT- 2005/12/24 09:00
PHST- 2005/12/24 09:00 [pubmed]
PHST- 2006/05/25 09:00 [medline]
PHST- 2005/12/24 09:00 [entrez]
AID - 13080776 [pii]
AID - 10.1007/BF02716598 [doi]
PST - ppublish
SO  - Clin Transl Oncol. 2005 Nov;7(10):464-7. doi: 10.1007/BF02716598.