Related Experiment Video
Updated: May 29, 2026
08:57
Identifying, Diagnosing, and Grading Malignant Peripheral Nerve Sheath Tumors in Genetically Engineered Mouse Models
Published on: May 17, 2024
Malignant peripheral nerve sheath tumour presenting as a pneumothorax
A Abbas1, H Jones, G T Kingston
1Radiology department, Southampton University Hospitals NHS Trust, Southampton, Hampshire, UK. drausami_abbas@msn.com
The British Journal of Radiology
|September 22, 2011
Summary
Malignant peripheral nerve sheath tumors (MPNSTs) are rare, aggressive sarcomas. This report details a unique case of MPNST presenting as a primary pneumothorax without prior neurofibromatosis history.
Area of Science:
- Oncology
- Pathology
- Thoracic Surgery
Background:
- Malignant peripheral nerve sheath tumors (MPNSTs) are rare, aggressive soft-tissue sarcomas.
- They commonly arise in patients with neurofibromatosis Type 1 (NF1).
- Typical presentations involve soft-tissue masses with potential nerve involvement.
Observation:
- A 34-year-old male presented with a right-sided pneumothorax.
- Imaging revealed an apical pleural mass.
- The patient had no history of neurofibromatosis or widespread metastatic disease.
Findings:
- Histopathology confirmed the apical pleural mass as a malignant peripheral nerve sheath tumor.
- This represents the first reported case of MPNST primarily presenting as a pneumothorax without known NF1.
- The patient was systemically well prior to this presentation.
Implications:
- MPNST should be considered in the differential diagnosis of unexplained pneumothorax, especially with apical pleural lesions.
- This case highlights the diverse and rare presentations of MPNST.
- Further research may elucidate the specific mechanisms leading to such presentations.
Related Concept Videos
01:27Pneumothorax-II
Pneumothorax is a medical condition defined by the buildup of air in the pleural space between the lungs and the chest wall. This accumulation of air can lead to partial or complete lung collapse, resulting in a range of clinical manifestations. Understanding the clinical presentation and effective management strategies is crucial for healthcare professionals in providing timely and appropriate care to individuals with pneumothorax.
Clinical Manifestations:
Clinical Manifestations:
01:26Pneumothorax-I
A pneumothorax is a condition where air builds up in the space between the lung and the chest wall, causing the lung to collapse. This condition arises when air enters the space between the parietal and visceral pleura, disrupting the negative pressure essential for lung inflation. This can lead to a partial or complete collapse of the lung.
Pneumothorax can be even further classified as spontaneous, traumatic, and tension pneumothorax.
Pneumothorax can be even further classified as spontaneous, traumatic, and tension pneumothorax.
01:08Pneumothorax II: Pathophysiology
Pneumothorax means the presence of air in the pleural space — the thin potential gap between the visceral and parietal pleura. This condition disrupts the normal pressure balance that keeps the lungs inflated, leading to partial or complete collapse of the affected lung.Normal physiologyUnder normal conditions, the pleural space maintains a slightly negative intrapleural pressure, which keeps the lungs expanded against the chest wall. This negative pressure creates a delicate balance between...
01:30Pleural Disorders: Types and Brief Description
The pleura is a vital part of the respiratory system. It's a double-layered membrane surrounding the lungs and lining the chest cavity. The two layers of the pleura are:
01:13Pleura of the Lungs
The lungs are nestled in a cavity, shielded by the pleura. The pleura, a form of serous membrane, wraps around each lung. This membrane arrangement consists of two layers: the visceral and parietal pleurae. The visceral pleura lines the surface of the lungIn contrast, the parietal pleura is the outer layer and contacts to the thoracic wall, the mediastinum, and the diaphragm. The hilum is the point of connection between the visceral and parietal layers. The space between the parietal and...
01:30Pneumonia I: Introduction
Pneumonia is an acute respiratory infection that targets the lungs, specifically the alveoli. These tiny air sacs, essential for oxygen exchange, become engorged with pus and fluid, severely hindering breathing, decreasing oxygen absorption, and causing significant pain and discomfort during respiration.
Risk Factors
Various factors influence the likelihood of developing pneumonia. Age plays a crucial role, with infants, children under two, and individuals over 65 at increased risk due to their...
Risk Factors
Various factors influence the likelihood of developing pneumonia. Age plays a crucial role, with infants, children under two, and individuals over 65 at increased risk due to their...