NÖROFİBROMATOZİS İLE AKCİĞER KANSERİ BİRLİKTELİĞİ: OLGU SUNUMU

Öz

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.

Anahtar Kelimeler

• Von Reclinghausen nöröfibromatozis, akciğer kanseri

NEUROFIBROMATOSIS TOGETHER WITH LUNG CANCER: CASE REPORTS

Öz

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.

Anahtar Kelimeler

• Von Reclinghausen neurofibromatosis, lung cancer

Kaynakça

1. 1. Dunn HG. Neurology. In: Kelly WN, ed. Text book of ›nternational medicine. Philadelphia. Lippincott-Raven;1997: 2398-9.
2. 2. Riccardi VM. Von Reclinghausen neurofibromatosis. N Engl J Med 1981; 305: 1617-27.
3. 3. Zamora AC, Collard HR, Wolters PJ, Webb WR, King TE. Neurofibromatosis- associated lung disase: a case series and literture review. Eur Respir J 2007; 29: 201-4.
4. 4. Rossi SE, Erasmus JJ, McAdams HP, Donelly EF. Thoracic manifestations of neurofibramaotosis-1. AJR 1999; 173: 1631-8.
5. 5. Shimizu E, Shinohara T, Mor N, Yokota J, Tani K, Izumi K, et al. Loss of heterozygosity on chromosome arm 17p in small cell lung carsinoma, but in neurofibrpomas ina patient with von Recklinghousen neorofibromatosiss. Cancer 1993; 71: 725-8.
6. 6. Okada E, Shozawa T. Von Reclinghousen’ disease assosiated with malignant pheochromocytoma. Acta Pathol Jpn 1984; 65: 623-6.
7. 7. Ito K, Yanagihara K, Okubo K, Kuwabara M. A case of lung cancer in a patient with von Recklinghausen’s diseases. Nihon Kyobu Shikkan Gakkai Zasshi 1992; 30: 317-21.
8. 8. Dikensoy Ö, Tunçözgür B, Erba¤l›c› Z, Filiz A. Primary adenocarsinoma of the lung in a case of von REclinghausen neurofibromatosis. Turkish Journal of Cancer 2002; 32(1): 32-6.

9. 9. Gupta KB, Kumar V, Tandon S, Gill M. Primary carsinoma of the lung in von Recklinghausen neurofibromatosis. Lung ‹ndia 2009; 26(4): 130-2.
10. 10. Gök M, Börüban C, Toy H, Kanat F, Kurt E, Uzun K. Akci¤er kanseri ile nörofibromatozis birlikteli¤i. T›p Araflt›rma Dergisi 2006; 4 (1): 48-51.
11. 11. Parkin GM, Pisani P, Ferlay J. Global cancer statics. CA Cancer Clin 1999; 49: 33-64.
12. 12. Shimizu Y, Tsuciya S, Watabe S. Von Reclinghousen’s disease with lung cancer derivated from the wall of emphysematous bullae. Intern Med 1994; 33:167-71.
13. 13. Halilçolar H, Tatar D, Ertu¤rul G, Çakan M, Gayaf M, Kömürcüo¤lu B. Epidemiyoloji. Akkoçlu A, Öztürk C ed. Akci¤er kanseri multidisipliner yaklafl›m. (Toraks Kitaplar›- Say› 1). Ankara: Bilimsel T›p Kitapevi; 1999: 17-22.