PMID- 21516517
OWN - NLM
STAT- MEDLINE
DCOM- 20120507
LR  - 20161125
IS  - 0942-0940 (Electronic)
IS  - 0001-6268 (Linking)
VI  - 153
IP  - 7
DP  - 2011 Jul
TI  - Primary Ewing's sarcoma of cranial bones: analysis of ten patients.
PG  - 1477-85
LID - 10.1007/s00701-011-1028-z [doi]
AB  - OBJECTIVE: Ewing's sarcomas are the second most common bone tumors in children 
      and primary involvement of the cranium is uncommon. We analyzed retrospectively 
      the data of ten patients with this rare subset of disease, who had been treated 
      at our institute since 2005. Our aim was to assess the outcomes, recurrence rates 
      and the selection of appropriate treatment methods. METHODS: The patients were 
      reviewed with respect to their clinical presentations, treatment, and outcomes. 
      Computed tomographic scanning of the brain was performed for all patients. 
      Skeletal surveys with routine radiographs and technetium-99 bone scans to detect 
      extracranial Ewing's sarcomas were performed for all patients. For all ten 
      patients, radical tumor excision was achieved surgically. Chromosomal 
      translocation studies were carried out on paraffin blocks for nine patients, 
      using fluorescence in situ hybridization (FISH) and polymerase chain reaction 
      (PCR). All patients were then subjected to adjuvant multidrug chemotherapy and 
      radiotherapy. The follow-up periods ranged from 2 months to 5 years (mean, 17.6 
      months). RESULTS: The predominant presenting features were headaches, increased 
      intracranial pressure, scalp swelling and trigeminal nerve involvement. The 
      erosion of dura and intradural extension was noted in eight patients in our 
      series. All nine patients, in whom FSIH and reverse transcriptase PCR (RT-PCR) 
      was done, tested positive for EWS-FLI1(t22:12) translocation. All patients 
      underwent radical excision within safe limits, followed by chemoradiation. Three 
      patients had local recurrences, which were detected within 12 months after 
      surgery. All three of them died within weeks of presentation with recurrence. One 
      patient experienced a recurrence after 30 months. This recurrent tumor was 
      completely excised, and additional chemotherapy was administered. There was a 
      local recurrence again after 18 months that was treated with surgery and 
      chemoradiation, and the patient is still surviving 5 years after the primary 
      surgery. One patient had metastasis at presentation and died within 2 months of 
      surgery. The remaining five seem to have good outcomes, though the follow-ups 
      were not very long. CONCLUSION: The treatment of primary Ewing's sarcoma of the 
      cranium still remains to be radical surgery, aggressive multidrug chemotherapy, 
      and radiotherapy. Neoadjuvant chemotherapy may not work in patients with large 
      intracranial extension due to raised pressure making decompression imperative. 
      The outcome is usually good if there is no early recurrence. Early recurrence, 
      presence of metastasis and extremes of age probably bears a poor outcome. 
      However, a larger series is required to confirm these findings.
FAU - Salunke, Pravin Shashikant
AU  - Salunke PS
AD  - Department of Neurosurgery, PGIMER, Chandigarh, India 160012. 
      drpravin_salunke@yahoo.co.uk
FAU - Gupta, Kirti
AU  - Gupta K
FAU - Malik, Vinod
AU  - Malik V
FAU - Kumar, Narendra
AU  - Kumar N
FAU - Henke, Lauren E
AU  - Henke LE
FAU - Cai, Chunyu
AU  - Cai C
FAU - Chen, Wei-Shen
AU  - Chen WS
FAU - Pfeifer, John D
AU  - Pfeifer JD
LA  - eng
PT  - Case Reports
PT  - Journal Article
DEP - 20110426
PL  - Austria
TA  - Acta Neurochir (Wien)
JT  - Acta neurochirurgica
JID - 0151000
SB  - IM
CIN - Acta Neurochir (Wien). 2012 Mar;154(3):565-6; author reply 567-8. PMID: 22234792
MH  - Adolescent
MH  - Adult
MH  - Child
MH  - Child, Preschool
MH  - Female
MH  - Humans
MH  - In Situ Hybridization, Fluorescence
MH  - Male
MH  - Middle Aged
MH  - Neoplasm Recurrence, Local/diagnostic imaging/genetics/surgery
MH  - Neurosurgical Procedures/methods
MH  - Polymerase Chain Reaction
MH  - Radiography
MH  - Retrospective Studies
MH  - Sarcoma, Ewing/*diagnosis/genetics/*surgery
MH  - *Skull/diagnostic imaging/pathology/surgery
MH  - Skull Neoplasms/*diagnosis/genetics/*surgery
MH  - Young Adult
EDAT- 2011/04/26 06:00
MHDA- 2012/05/09 06:00
CRDT- 2011/04/26 06:00
PHST- 2010/11/14 00:00 [received]
PHST- 2011/04/08 00:00 [accepted]
PHST- 2011/04/26 06:00 [entrez]
PHST- 2011/04/26 06:00 [pubmed]
PHST- 2012/05/09 06:00 [medline]
AID - 10.1007/s00701-011-1028-z [doi]
PST - ppublish
SO  - Acta Neurochir (Wien). 2011 Jul;153(7):1477-85. doi: 10.1007/s00701-011-1028-z. 
      Epub 2011 Apr 26.