NÖROFİBROMATOZİS İLE AKCİĞER KANSERİ BİRLİKTELİĞİ: OLGU SUNUMU

Year 2012, Volume: 26 Issue: 2, 123 - 128, 01.10.2012

Berna Kömürcüoğlu Burak Eşkut Kadri A. Çırak Serpil Tekgül Semra Bilaçeroğlu

Abstract

Von Reclinghausen nöröfibromatozis (NF-1), otozomal dominant geçişli nöröcutanöz sistemi tutan bir hastalıktır. Ana klinik bulguları; multipl nöröfibromlar ve ciltte cafe- au- lait lekeleridir. NF-1 kansere yatkınlık yaratan sendromlar içinde en sık rastlanıdır ve özellikle de nöral crestten köken alan tümörlere yatkınlık yaratır. Malign swannoma, neurofibrosarkom, intrakranial gliom ve feokromasitoma NF-1'li hastalarda en sık görülen nörölojik sistem kanserleridir. NF-1'de olguların yaklaşık %10-20'sinde akciğer tutulumu izlenir. En sık rastlanan akciğer tutulumu özellikle üst zonlarda ince duvarlı büllöz lezyonlar ve fibrozisdir. Ancak NF-1' de primer akciğer kanseri nadirdir ve literatürde olgu sunumları şeklinde bildirilmiştir (%2-5) . 43 yaşında bir erkek olguda NF-1 ve primer akciğer karsinomu birlikteliği sunuldu ve NF-1'de akciğer kanseri gelişimine neden olabilecek risk faktörleri tartışıldı.

Keywords

Von Reclinghausen nöröfibromatozis, akciğer kanseri

NEUROFIBROMATOSIS TOGETHER WITH LUNG CANCER: CASE REPORTS

Abstract

Von Reclinghausen neurofibramatosis (NF-1) is an autosomal dominant disease which targeted neurocutanous system. Cardinal features are multiple neurofibromas and cafe-au-lait spots. NF-1 is the most common inherited syndrome predisposing to neoplasia, spesialy neural crest derivated tumors. Malignant schannoma, neurofibrosarcoma, intracranial glioma and pheochromacytoma were most frequently seen neurolojik malignancies in NF-1 patients. Pulmonary involvement was seen 10-20% of the NF-1 cases. The most common pulmonary manifestations in NF-1 were thin walled bullae formation and fibrosis predominantly in upper zones. However occurance of primary lung carsinoma in assosiation with NF-1 is very rare. 43 years old male patient with NF-1 and primary lung epidermoid carsinoma is presented. This case is presented to discuss the risk factors of the patient with NF-1 to developt lung carsinoma.

Keywords

Von Reclinghausen neurofibromatosis, lung cancer

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