Journal of Neuro-Oncology

, Volume 94, Issue 3, pp 383–388 | Cite as

Expression and significance of EGFR in malignant peripheral nerve sheath tumor

  • Daniel Keizman
  • Josephine Issakov
  • Isaac Meller
  • Natalie Meimon
  • Maya Ish-Shalom
  • Osnat Sher
  • Ofer MerimskyEmail author
Clinical Study - Patient Study


Malignant peripheral nerve sheath tumor (MPNST) is an aggressive sarcoma. Epidermal growth factor receptor (EGFR) may play a putative role in its pathogenesis, and be targeted for therapeutic purposes. The study was aimed at investigating the expression and prognostic influence of EGFR in MPNST. Primary and metastatic MPNSTs were immunostained with antibodies to EGFR. The total EGFR expression (membranous and cytoplasmic) was analyzed by morphometry, grade of positivity and the intensity (score 0–3). An EGFR composite score (range 0–300) was calculated by multiplying the intensity by the grade. A composite score >10 was considered as EGFR overexpression. Score was correlated with clinical behavior. Forty-three percentage of 46 patients with MPNST overexpressed EGFR in the primary tumor, and had a higher prevalence of advanced-stage tumors (≥IIc, 46% vs. 80%, P = 0.011). Patients without overexpression had a higher prevalence of tumors with a low mitotic rate (31% vs. 0%, P = 0.049). Neurofibromatosis was more prevalent in patients with EGFR overexpression (75% vs. 42%, P = 0.007). Five year disease free survival (mean 30.1 vs. 17.4 months, P = 0.048), time to progression (mean 9.2 vs. 5.2 months, P = 0.005) and 5 year survival (52% vs. 25%, P = 0.041, mean 54 vs. 43 months) were significantly higher among patients without overexpression. EGFR appeared to play a role in MPNST progression. EGFR overexpression was correlated with worse prognostic variables and course. Clinical trials of targeting EGFR in MPNST are warranted.


Malignant peripheral nerve sheath tumor (MPNST) Epidermal growth factor receptor (EGFR) Prognosis Survival 


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Copyright information

© Springer Science+Business Media, LLC. 2009

Authors and Affiliations

  • Daniel Keizman
    • 1
  • Josephine Issakov
    • 2
  • Isaac Meller
    • 3
  • Natalie Meimon
    • 1
  • Maya Ish-Shalom
    • 4
  • Osnat Sher
    • 2
  • Ofer Merimsky
    • 1
    Email author
  1. 1.Unit of Bone and Soft Tissue Oncology, Division of OncologyTel Aviv Sourasky Medical Center (TASMC), Affiliated with Sackler School of Medicine, Tel-Aviv UniversityTel AvivIsrael
  2. 2.Unit of Bone and Soft Tissue PathologyTel Aviv Sourasky Medical Center (TASMC), Affiliated with Sackler School of Medicine, Tel-Aviv UniversityTel AvivIsrael
  3. 3.The National Unit of Orthopedic OncologyTel Aviv Sourasky Medical Center (TASMC), Affiliated with Sackler School of Medicine, Tel-Aviv UniversityTel AvivIsrael
  4. 4.Department of EndocrinologyTel Aviv Sourasky Medical Center (TASMC), Affiliated with Sackler School of Medicine, Tel-Aviv UniversityTel AvivIsrael

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