p53 Expression in neurofibroma and malignant peripheral nerve sheath tumor: An immunohistochemical study of sporadic and NF1-associated tumors

Kevin C. Halling, Bernd W. Scheithauer, Amy C. Halling, Antonio G. Nascimento, Steven C. Ziesmer, Patrick C. Roche, Peter C. Wollan

Research output: Contribution to journalArticle

75 Scopus citations

Abstract

Malignant peripheral nerve sheath tumors (MPNST) are highly malignant sarcomas arising either de novo or in transition from neurofibroma. Although relatively little is known of the molecular genetic alterations that underlie their formation, recent DNA sequencing studies have demonstrated the presence of p53 mutations in some MPNST. This tumor-suppressor gene has been implicated in the progression of a variety of human malignancies, including sarcomas. Employing the anti-p53 monoclonal antibody Do-7, this retrospective immunohistochemical study of p53 gene overexpression in MPNST found reactivity to be present in 68% and to be significant in degree in 57%. In contrast, although some degree of p53 overexpression was present in 48% of neurofibromas, none stained strongly and only 1 of the 27 (4%), a cellular example, showed significant staining. No difference in the frequency or degree of p53 staining was noted between MPNSTs from patients with or without neurofibromatosis 1. The observed overexpression of the gene product, possibly the reflection of a p53 gene mutation, suggests a ride for p53 in the progression of neurofibroma to MPNST. Although the prognostic of p53 overexpression in MPNST remains to be confirmed, in the present series immunopositive tumors were associated with a shorter median patient survival (18 months) than were tumors showing no reactivity (82 months) (P = .02).

Original languageEnglish (US)
Pages (from-to)282-288
Number of pages7
JournalAmerican journal of clinical pathology
Volume106
Issue number3
DOIs
StatePublished - Sep 1996

Keywords

  • Malignant peripheral nerve sheath tumor
  • Neurofibroma
  • Prognosis
  • Tumor-suppressor gene
  • p53

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

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    Halling, K. C., Scheithauer, B. W., Halling, A. C., Nascimento, A. G., Ziesmer, S. C., Roche, P. C., & Wollan, P. C. (1996). p53 Expression in neurofibroma and malignant peripheral nerve sheath tumor: An immunohistochemical study of sporadic and NF1-associated tumors. American journal of clinical pathology, 106(3), 282-288. https://doi.org/10.1093/ajcp/106.3.282