Congenital Peribronchial Myofibroblastic Tumor: A Case Report and Review of the Literature

Abstract

Background: Congenital peribronchial myofibroblastic tumor is a rare, solid mesenchymal tumor of the neonate, usually associated with non-immune hydrops fetalis. Case Report: We present a case of congenital peribronchial myofibroblastic tumor, in whom a right lung mass was detected in intrauterine life. 12 days after delivery by caesarean section, right lobectomy was performed. The tumor was limited to the lung, and was composed of spindle cells, proliferating around a bronchial unit. Central necrosis and 4-5 mitoses per 10 high power fields were present. The patient is well 26 months after surgery. Conclusion: We report this rare tumor with clinical, radiological and pathologic findings and a review of the literature. Turkish Başlık: Konjenital Peribronşiyal Miyofibroblastik Tümör: Olgu Sunumu ve Literatürün Gözden Geçirilmesi Anahtar Kelimeler: akciğer tümörü, konjenital, miyofibroblastik tümör Arkaplan: Konjenital peribronşiyal miyofibroblastik tümör neonatal dönemin nadir, solid mezenkimal tümörü olup, sıklıkla non-immün hidrops ile birliktedir. Olgu Sunumu: Sunulan olguda, intrauterin dönemde sağ akciğerinde kitle saptanmış ve doğumdan 12 gün sonra sağ lobektomi uygulanmıştır. Tümör akciğere sınırlı olup, bronşiyolleri çevreleyerek çoğalan iğsi hücrelerden oluşmaktaydı. Santral nekroz ve on büyük büyütme alanında dört-beş mitoz saptandı. Hasta cerrhi sonrası 20. ayında olup kontrollerinde sağlıklı seyretmektedir. Sonuç: Biz, nadir görülen bu tümörü klinik, radyolojik ve patolojik bulguları ve literatürü gözden geçirerek sunmayı amaçladık.

Keywords

• Congenital, lung tumor, myofibroblastic tumor

Congenital Peribronchial Myofibroblastic Tumor: A Case Report and Review of the Literature

Öz

References

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