A Case of Cutaneous Neurofibroma Intimately Contacted with Intrathoracic and Chest Wall Plexiform Neurofibroma in Von Recklinghausen's Disease

ABSTRACT

Congenital neurofibromatosis type 1, or Von Recklinghausen's disease is an autosomal dominant disorder characterized by neurofibroma, pigmented skin lesions (Cafe-au-lait macules), iris hamartomas and meningeal tumors, but rarely, by autonomic ganglia tumors, such as pheochromocytomas. We have experienced an intrathoracic and chest wall plexiform neurofibroma intimately contacted with collagenoma-like, dome-shaped skin lesions of type 1 neurofibromatosis, which are relatively rare and interesting, but can be regarded as typical findings in neurofibromatosis. Although intrathoracic neurogenic tumors are not uncommon, cases like ours are interesting, as the feature of collagenoma-like skin neurofibroma was very closely apposited with chest wall neurofibroma. Our case had no aypical features of malignancy and the patient was clinically followed up without recurrence.