Giant neurofibromatosis of the chest wall : Two patient reports

Two patients with severely deforming giant neurofibromatosis of the chest wall secondary to von Recklinghausen's disease are presented. Pain, respiratory compromise, recurrent ulcerations, cosmetic considerations, and the malignant potential of these lesions indicated wide excision and reconstruction. It is impossible to completely eradicate all neurofibromas, which may affect virtually every nerve within the chest wall including the mediastinum. However, excision of the primary mass may reduce the possibility of malignant degeneration into neurofibrosarcoma or malignant schwannoma. This type of major, full-thickness chest wall resection is now possible using musculocutaneous flaps to achieve satisfactory closure with a single-stage procedure with minimal morbidity and a short hospital stay. At the 10-year follow-up, neither patient exhibited evidence of recurrence.