[Elephantiasis of the thoracic wall within the scope of von Recklinghausen neurofibromatosis, case report].
Neurofibromas are benign tumors arising from neuroectodermal tissues. They may occur as solitary lesions, or multiple, in which case they are referred to as neurofibromatosis, or von Recklinghausen's disease. We report about a 17-year-old patient with neurofibromatous elephantiasis located in the lateral aspect of the left hemithorax. CT showed massive chest wall infiltration and retroperitoneal tumor growth, but no abnormalities of the cranium. Our surgical treatment consisted of a two-stage, palliative tumor debulking and defect coverage with a splitthickness skin graft. The operative and postoperative course was uneventful.
Wechselberger
Gottfried
G
Universitätsklinik für Plastische und Wiederherstellungschirurgie, Ludwig Boltzmann Institut für Qualitätssicherung in der Plastischen Chirurgie, Innsbruck. Gottfried.Wechselberger@uibk.ac.at
Bauer
Thomas
T
Schoeller
Thomas
T
Ohlbauer
Markus
M
Piza-Katzer
Hildegunde
H
D000293:Adolescent; D005808:Genes, Recessive; D006801:Humans; D008297:Male; D009361:Neoplasm Invasiveness; D018318:Neurofibroma, Plexiform / Q000175:diagnosis* / Q000235:genetics / Q000601:surgery; D009456:Neurofibromatosis 1 / Q000175:diagnosis* / Q000235:genetics / Q000601:surgery; D010166:Palliative Care; D012086:Reoperation; D012186:Retroperitoneal Neoplasms / Q000175:diagnosis* / Q000235:genetics / Q000601:surgery; D013526:Surgical Mesh; D013899:Thoracic Neoplasms / Q000175:diagnosis* / Q000235:genetics / Q000601:surgery; D014057:Tomography, X-Ray Computed