Von Recklinghausen's disease with a malignant meningeal, cerebral and optic nerve tumour and bilateral vagal schwannomas: Possible mesenchymal histogenesis on light and electron microscopy

Abstract

The clinical, histopathologic and fine structural features of multiple unusual tumours detected in a 20-year-old patient with von Recklinghausen's disease, who died within a year of onset of symptoms of a rapidly expanding intracranial tumour, are described. The tumour was found to involve the falx cerebri, the basal leptomeninges and dura mater, both olfactory and optic nerves, both frontal lobes, the right temporal lobe and middle cerebral peduncle, both middle cerebellar peduncles, and with a metastasis in a cervical node. On light and electron microscopy this tumour appeared to be a fibroblastic meningeal sarcoma with giant cells, mitotic figures, a rich reticulin matrix throughout, and tumour cells full of rough ER but without any glial filaments. Also very unusual was the involvement of both vagus nerves in their cervical and intrathoracic portions, by a schwannomatous benign tumour and with a non-chromaffin paraganglioma at its termination in the oesophagus. One of the few cutaneous “neurofibromas” was also schwannian, containing tumour cells with a basement membrane on electron microscopy. Most, if not all, of these tumours appeared mesenchymal in origin, more aggressive in behaviour and carrying a graver prognosis in von Recklinghausen's disease.