Levent DALAR, Filiz KOŞAR, Nur ÜRER, Sedat ALTIN, Sinem Nedime SÖKÜCÜ, Emel ERYÜKSEL, Ahmet Levent KARASULU

Central airway obstruction due to malignant fibrous histiocytoma metastasis in a case with Mounier-Kuhn syndrome

Malign fibröz histiyositoma erişkinlerde görülen yumuşak doku sarkomları arasında sık izlenen tiplerden biridir. Akciğeren sık rastlanan metastaz alanıdır. Mounier-Kuhn sendromu ise trakea ve bronşların ileri derecede genişlemesiyle karakte-rizedir. Ana hava yolu obstrüksiyonu, endoluminal, ekstraluminal ya da her iki komponentin birarada olduğu akciğer vemediasten kitlelerinde karşımıza çıkabilir. Bu yazıda, nadir görülen bir klinik durumun dev mediastinal malign fibroz his-tiyositoma metastazına sekonder olarak oluşması ve diğer yandan kitlenin yol açtığı ana hava yolu obstrüksiyonunun çö-zülmesinde yaşanan zorlukların klinik önemini vurgulamak istedik. Mounier-Kuhn sendromu ve ana hava yolu obstrüksi-yonu birlikteliği ve bu durumun yönetilmesinde yaşanan güçlükler İngilizce literatürde daha önce sunulmamıştır.

Mounier-Kuhn sendromlu olguda malign fibröz histiyositom metastazına bağlı ana hava yolu obstrüksiyonu

Malign fibrous histiocytoma is one of the most observed soft tissue sarcomas seen in the adults. The most common metas-tasis region is the lung and metastasis. Mounier-Kuhn syndrome is characterized by the highly dilatation of the trachea andbronchi. We may encounter with the major airway obstruction in the endoluminal or extraluminal lung and mediastinalmasses or those with both components together. In this article, we would like to highlight the occurrence of a rare seen cli-nical situation secondary to the giant mediastinal malign fibrous histiocytoma metastasis and the clinical difficulties expe-rienced in resolving of the main airway obstruction caused by the mass. Since the lack of the similar studies conductedpreviously, we found the case worth presenting.

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