Abstract

 

Neurofibromatosis is a an autosomal dominantly inherited disease manifested in the skin, nervous system and the eyes. A neurofibromatozis patient with a huge mediastinal neurofibroma is presented since a severe bleeding occurred during the resection of the tumor. Case: A 27-year-old man applied with a chest pain. On physical examination pectus carinatum and scoliosis were present, and he had widespread freckles, cafe au lait spots and nodules in the skin. Bilateral Lish nodules were determined. Thoracic CT revealed a 15x20 cm posterior mediastinal mass compressing adjacent vertebra. Biopsy of one of the skin nodule was reported as neurofibroma. The patient was diagnosed as neurofibromayosis type 1 by means of these findings. A thoracotomy was performed. The mediastinal mass was needed to be resected quickly because of a very serious bleeding from the tumor. In spite of a good bleeding control, a serious drainage occurred, and a rethoracotomy was needed. There was a serious oozing from the bed of the resected tumor. The bleeding was controlled with hemostatic agents and compression. We used 24 units of blood. Histopathological examination revealed atypical neurofibroma. A 15x20 cm chest wall hematoma arised over the old thoracotomy incision after four months, and resolved spontaneously. Huge mediastinal neurofibroma may develope in patients with neurofibromatosis. Resection is recommended in approprite patients. Massive hemorrhage may occur during resection of the neurofibromas due to vascular abnormalities from the tumor and the other relevant vessels in patients with neurofibromatosis.

Key Words: Neurofibromatosis; Massive Hemorrhage; Spontaneous; Mediastinum.