Mediastinum

 

Neurogenic tumours of the mediastinum

Neurogenic tumours account for 20-30% of all mediastinal tumours rising to 50-60% in children. Von Recklinhausen’s neurofibromatosis is associated with an increased incidence of neurogenic tumours of all histological types.

Most tumours are benign neurilemmomas (also known as schwannomas) or ganglioneuromas (see below) and are completely cured by excision. Though benign, resection is indicated as the benign tumours are indistinguishable from malignant tumours on imaging, and fine needle aspiration cytology is unreliable. Even when benign permeation of tumour along tissue planes can lead to compression of vital structures and can preclude resection.

Treatment

  1. Benign – resection

  2. Complete resection also leads to high cure rates in the intermediate malignancy ganglioneuroblastoma and even frankly malignant neuroblastoma and paraganglionoma.

  3. Malignant schwannoma can rarely be excised completely and leads to death within a year of diagnosis: incompletely excised paraganglionoma usually proves fatal regardless of adjuvant therapy though survival is substantially longer.

  4. Neuroblastoma is more sensitive to chemotherapy and when used in combination with radiotherapy even incompletely excised tumours can achieve reasonable long-term survival.

  5. Radiotherapy can also reduce local recurrence of incompletely excised neurilemmoma, neurofibroma and ganglioneuroblastoma

 

Classification of mediastinal neurogenic tumours