MEDİASTİNAL KİTLE NEDENİYLE SOLUNUM YETMEZLİĞİ GELİŞEN NÖROFİBROMATOZİS TİP 1 OLGUSU

Öz

Nörofibromatozis, nörokuta nöz sendromlar içinde en sık görüleni olup 1/2000- 1/3000 doğumda bir sıklıkta ortaya çıkar. Doğuştan mental retarde olan 25 yaşında kadın hasta, akciğer radyogram ında mediastinal kitle lezyonu ve arteriyel kan gazında hipoksi saptanması üzerine hastaneye yatırıldı. İleri derecede dispne yakınması nedeniyle palyatif toraks radyoterapisi uygulandı. Hastanın toraks duvarındaki multipl nodülopapüller cilt lezyonlarından ve mediastinal kitle lezyonundan biyopsi yapıldı. Her iki biyopsi sonucu "nörofibrom"la uyumlu bulundu. Olgumuzda klinik ve histolojik olarak benign bir hastalık olan nörofibromatozis tip 1'e (Von Recklinghausen hastalığı) bağlı mediastinal kitle bulundu. Benign yapıda olmasına karşın mediastinal kitlenin yaptığı basıya bağlı solunum yetmezliği gelişen hasta, nörofibromatozis konusundaki literatür bilgileri eşliğinde sunuldu.

Anahtar Kelimeler

• Mediastinal kitle, nörofibromatozis tip 1, Von Recklinghausen hastalığı

NEUROFIBROMATOSIS TYPE 1 CASE WITH RESPIRATORY INSUFFICIENCY DUE TO MEDIASTINAL MASS

Abstract

Neurofibromatosis is the most common one among neurocutaneous syndromes; its incidence is 1/2000-1/3000. A congenitally mental retarded 25-years-old female patient was hospitalized due to a mediastinal mass lesion in chest radiogram and hypoxia in her arterial blood gasses. Paliative radiotherapy was performed due to serious dyspnea. Biopsies taken from the multiple nodulopapullar skin lesions on her thoracic wall and from her mediastinal mass lesion were compatible with "neurofibroma". She was diagnosed to have the benign disease neurofibromatosis type 1 (Von Recklinghausen disease) and this was confirmed clinically and histologically. The patient with respiratory insufficiency due to pressure of mediastinal mass is presented with information from literature.

Keywords

• Mediastinal mass, neurofibromatosis type 1, Von Recklinghausen disease

Kaynakça

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