Intrathoracic peripheral nerve sheath tumors - A clinicopathological study of 75 cases

Jennifer M. Boland, Thomas V. Colby, Andrew L. Folpe

Research output: Contribution to journalArticle

14 Citations (Scopus)

Abstract

Although peripheral nerve sheath tumors (PNSTs) are common in the posterior mediastinum, they are rare in other mediastinal compartments and in the pleuropulmonary parenchyma. We sought to characterize the clinicopathological features of PNSTs occurring in the lung, pleura, and mediastinum. Diagnoses were confirmed by slide review. Study cases include 21 benign pleuropulmonary PNSTs, 49 benign mediastinal PNSTs, and 5 malignant PNSTs. Benign pleuropulmonary tumors comprised 13 schwannomas, 6 neurofibromas, 1 perineurioma, and 1 ganglioneuroma. Six lesions were endobronchial (3 neurofibromas, 1 schwannoma, 1 perineurioma, 1 ganglioneuroma), whereas the remaining schwannomas and neurofibromas formed parenchymal masses (usually pleural based). Benign mediastinal PNSTs (46 posterior, 2 middle, and 1 anterior) were all schwannomas and showed a female predominance. None of the patients with benign PNSTs experienced recurrence. Of the 5 malignant PNSTs, 4 were pleuropulmonary (3 pleural based) and 1 occurred in the anterior mediastinum. Two of the 5 patients had a history of neurofibromatosis type 1 (aged 27 and 45 years). At last follow-up, 3 of the 5 patients had died of disease, 1 was alive with disease, and 1 was alive with no evidence of disease (41 months). Although rare, a wide histologic range of PNSTs occur in the lung. Although neurofibroma, perineurioma, and ganglioneuroma were observed as endobronchial lesions, most pulmonary schwannomas were pleural-based masses. Mediastinal PNSTs are dominated by posterior mediastinal schwannomas, although schwannomas rarely occur in the other mediastinal compartments as well. Malignant PNSTs are very rare in the thorax, where they show aggressive behavior.

Original languageEnglish (US)
Pages (from-to)419-425
Number of pages7
JournalHuman Pathology
Volume46
Issue number3
DOIs
StatePublished - Mar 1 2015

Fingerprint

Nerve Sheath Neoplasms
Neurilemmoma
Neurofibroma
Ganglioneuroma
Mediastinum
Lung
Neurofibromatosis 1
Pleura
Thorax

Keywords

  • Lung
  • Mediastinum
  • Nerve sheath
  • Neurofibroma
  • Pleura
  • Schwannoma

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Medicine(all)

Cite this

Intrathoracic peripheral nerve sheath tumors - A clinicopathological study of 75 cases. / Boland, Jennifer M.; Colby, Thomas V.; Folpe, Andrew L.

In: Human Pathology, Vol. 46, No. 3, 01.03.2015, p. 419-425.

Research output: Contribution to journalArticle

Boland, Jennifer M. ; Colby, Thomas V. ; Folpe, Andrew L. / Intrathoracic peripheral nerve sheath tumors - A clinicopathological study of 75 cases. In: Human Pathology. 2015 ; Vol. 46, No. 3. pp. 419-425.
@article{a187f1d1b5ee4e23a4720e2753e6408c,
title = "Intrathoracic peripheral nerve sheath tumors - A clinicopathological study of 75 cases",
abstract = "Although peripheral nerve sheath tumors (PNSTs) are common in the posterior mediastinum, they are rare in other mediastinal compartments and in the pleuropulmonary parenchyma. We sought to characterize the clinicopathological features of PNSTs occurring in the lung, pleura, and mediastinum. Diagnoses were confirmed by slide review. Study cases include 21 benign pleuropulmonary PNSTs, 49 benign mediastinal PNSTs, and 5 malignant PNSTs. Benign pleuropulmonary tumors comprised 13 schwannomas, 6 neurofibromas, 1 perineurioma, and 1 ganglioneuroma. Six lesions were endobronchial (3 neurofibromas, 1 schwannoma, 1 perineurioma, 1 ganglioneuroma), whereas the remaining schwannomas and neurofibromas formed parenchymal masses (usually pleural based). Benign mediastinal PNSTs (46 posterior, 2 middle, and 1 anterior) were all schwannomas and showed a female predominance. None of the patients with benign PNSTs experienced recurrence. Of the 5 malignant PNSTs, 4 were pleuropulmonary (3 pleural based) and 1 occurred in the anterior mediastinum. Two of the 5 patients had a history of neurofibromatosis type 1 (aged 27 and 45 years). At last follow-up, 3 of the 5 patients had died of disease, 1 was alive with disease, and 1 was alive with no evidence of disease (41 months). Although rare, a wide histologic range of PNSTs occur in the lung. Although neurofibroma, perineurioma, and ganglioneuroma were observed as endobronchial lesions, most pulmonary schwannomas were pleural-based masses. Mediastinal PNSTs are dominated by posterior mediastinal schwannomas, although schwannomas rarely occur in the other mediastinal compartments as well. Malignant PNSTs are very rare in the thorax, where they show aggressive behavior.",
keywords = "Lung, Mediastinum, Nerve sheath, Neurofibroma, Pleura, Schwannoma",
author = "Boland, {Jennifer M.} and Colby, {Thomas V.} and Folpe, {Andrew L.}",
year = "2015",
month = "3",
day = "1",
doi = "10.1016/j.humpath.2014.11.017",
language = "English (US)",
volume = "46",
pages = "419--425",
journal = "Human Pathology",
issn = "0046-8177",
publisher = "W.B. Saunders Ltd",
number = "3",

}

TY - JOUR

T1 - Intrathoracic peripheral nerve sheath tumors - A clinicopathological study of 75 cases

AU - Boland, Jennifer M.

AU - Colby, Thomas V.

AU - Folpe, Andrew L.

PY - 2015/3/1

Y1 - 2015/3/1

N2 - Although peripheral nerve sheath tumors (PNSTs) are common in the posterior mediastinum, they are rare in other mediastinal compartments and in the pleuropulmonary parenchyma. We sought to characterize the clinicopathological features of PNSTs occurring in the lung, pleura, and mediastinum. Diagnoses were confirmed by slide review. Study cases include 21 benign pleuropulmonary PNSTs, 49 benign mediastinal PNSTs, and 5 malignant PNSTs. Benign pleuropulmonary tumors comprised 13 schwannomas, 6 neurofibromas, 1 perineurioma, and 1 ganglioneuroma. Six lesions were endobronchial (3 neurofibromas, 1 schwannoma, 1 perineurioma, 1 ganglioneuroma), whereas the remaining schwannomas and neurofibromas formed parenchymal masses (usually pleural based). Benign mediastinal PNSTs (46 posterior, 2 middle, and 1 anterior) were all schwannomas and showed a female predominance. None of the patients with benign PNSTs experienced recurrence. Of the 5 malignant PNSTs, 4 were pleuropulmonary (3 pleural based) and 1 occurred in the anterior mediastinum. Two of the 5 patients had a history of neurofibromatosis type 1 (aged 27 and 45 years). At last follow-up, 3 of the 5 patients had died of disease, 1 was alive with disease, and 1 was alive with no evidence of disease (41 months). Although rare, a wide histologic range of PNSTs occur in the lung. Although neurofibroma, perineurioma, and ganglioneuroma were observed as endobronchial lesions, most pulmonary schwannomas were pleural-based masses. Mediastinal PNSTs are dominated by posterior mediastinal schwannomas, although schwannomas rarely occur in the other mediastinal compartments as well. Malignant PNSTs are very rare in the thorax, where they show aggressive behavior.

AB - Although peripheral nerve sheath tumors (PNSTs) are common in the posterior mediastinum, they are rare in other mediastinal compartments and in the pleuropulmonary parenchyma. We sought to characterize the clinicopathological features of PNSTs occurring in the lung, pleura, and mediastinum. Diagnoses were confirmed by slide review. Study cases include 21 benign pleuropulmonary PNSTs, 49 benign mediastinal PNSTs, and 5 malignant PNSTs. Benign pleuropulmonary tumors comprised 13 schwannomas, 6 neurofibromas, 1 perineurioma, and 1 ganglioneuroma. Six lesions were endobronchial (3 neurofibromas, 1 schwannoma, 1 perineurioma, 1 ganglioneuroma), whereas the remaining schwannomas and neurofibromas formed parenchymal masses (usually pleural based). Benign mediastinal PNSTs (46 posterior, 2 middle, and 1 anterior) were all schwannomas and showed a female predominance. None of the patients with benign PNSTs experienced recurrence. Of the 5 malignant PNSTs, 4 were pleuropulmonary (3 pleural based) and 1 occurred in the anterior mediastinum. Two of the 5 patients had a history of neurofibromatosis type 1 (aged 27 and 45 years). At last follow-up, 3 of the 5 patients had died of disease, 1 was alive with disease, and 1 was alive with no evidence of disease (41 months). Although rare, a wide histologic range of PNSTs occur in the lung. Although neurofibroma, perineurioma, and ganglioneuroma were observed as endobronchial lesions, most pulmonary schwannomas were pleural-based masses. Mediastinal PNSTs are dominated by posterior mediastinal schwannomas, although schwannomas rarely occur in the other mediastinal compartments as well. Malignant PNSTs are very rare in the thorax, where they show aggressive behavior.

KW - Lung

KW - Mediastinum

KW - Nerve sheath

KW - Neurofibroma

KW - Pleura

KW - Schwannoma

UR - http://www.scopus.com/inward/record.url?scp=84924537861&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84924537861&partnerID=8YFLogxK

U2 - 10.1016/j.humpath.2014.11.017

DO - 10.1016/j.humpath.2014.11.017

M3 - Article

VL - 46

SP - 419

EP - 425

JO - Human Pathology

JF - Human Pathology

SN - 0046-8177

IS - 3

ER -