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Glandular peripheral nerve sheath tumors

J M Woodruff1, W N Christensen

  • 1Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, NY 10021.

Cancer
|December 15, 1993
PubMed
Summary

Glandular peripheral nerve tumors (PNT) are rare and often malignant. Most patients with these tumors, particularly those with neurofibromatosis-1 (NF-1), have a poor prognosis and high mortality rate.

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Area of Science:

  • Oncology
  • Pathology
  • Neurosurgery

Background:

  • Glandular peripheral nerve tumors (PNT) are uncommon and their classification is debated.
  • Understanding the characteristics of glandular PNT is crucial for determining prognosis.

Purpose of the Study:

  • To investigate the clinicopathological features and outcomes of patients with glandular PNT.
  • To clarify the nature of glandular PNT and their association with other conditions like neurofibromatosis-1 (NF-1).

Main Methods:

  • A review of 11 patients with glandular PNT from the authors' laboratory.
  • Analysis of 27 previously reported cases of glandular PNT from the literature.
  • Histopathological examination and review of patient follow-up data.

Main Results:

  • Glandular PNT occurred in patients aged 8–68 years, with a mean age of 28.
  • Neurofibromatosis-1 (NF-1) was present in 74% of patients.
  • 92% of tumors were histologically malignant, with a high mortality rate (71% of patients died with tumor).

Conclusions:

  • Glandular PNT are predominantly malignant and associated with a poor prognosis.
  • The high incidence of NF-1 in these tumors warrants further investigation.
  • Accurate classification and understanding of glandular PNT are essential for patient management.

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