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

Kaynakça

1. Jones CJ. Unusual hamartoma of the lung in a newborn infant. Arch Pathol 1949;48:150-4.
2. McGinnis M, Jacobs G, el-Naggar A, Redline RW. Congenital peribronchial myofibroblastic tumor (so-called “congenital leiomyosarcoma”). A distinct neonatal lung lesion associated with nonimmune hydrops fetalis. Mod Pathol 1993;6:487-92. Robb D. A case of neonatal fibrosarcoma of lung. Br J Surg 1958;46:173-4. [CrossRef]
3. Guccion JG, Rosen SH. Bronchopulmonary leiomyosarcoma and fibrosarcoma. A study of 32 cases and review of the literature. Cancer 1972;30:836-47. [CrossRef]
4. Jimenez JF, Uthman EO, Townsend JW, Gloster ES, Seibert JJ. Primary bronchopulmonary leiomyosarcoma in childhood. Arch Pathol Lab Med 1986;110:348-51.
5. Pettinato G, Manivel JC, Saldana MJ, Peyser J, Dehner LP. Primary bronchopulmonary fibrosarcoma of childhood and adolescence: reassessment of a low-grade malignancy. Clinicopathologic study of five cases and review of the literature. Hum Pathol 1989;20:463-71. [CrossRef]
6. Haller JO, Kauffman SL, Kassner EG. Congenital mesenchymal tumour of the lung. Br J Radiol 1977;50:217-9. [CrossRef] Warren JS, Seo IS, Mirkin LD. Massive congenital mesenchymal malformation of the lung: a case report with ultrastructural study. Pediatr Pathol 1985;3:321-8. [CrossRef]
7. Khong TY, Keeling JW. Massive congenital mesenchymal malformation of the lung: another cause of non-immune hydrops. Histopathology 1990;16:609-11. [CrossRef]
8. Alobeid B, Beneck D, Sreekantaiah C, Abbi RK, Slim MS. Congenital pulmonary myofibroblastic tumor: a case report with cytogenetic analysis and review of the literature. Am J Surg Pathol 1997;21:610-4. [CrossRef]

9. Reiss A, Goldberg Y, Monichor M, Drugan A. Congenital pulmonary myofibroblastic tumor--pathology and prenatal sonographic appearance. Prenat Diagn 2005;25:1064-6. [CrossRef]
10. Horikoshi T, Kikuchi A, Matsumoto Y, Tatematsu M, Takae K, Ogiso Y, et al. Fetal hydrops associated with congenital pulmonary myofibroblastic tumor. J Obstet Gynaecol Res 2005;31:552-5. [CrossRef]
11. Huppmann AR, Coffin CM, Hoot AC, Kahwash S, Pawel BR. Congenital peribronchial myofibroblastic tumor: comparison of fetal and postnatal morphology. Pediatr Dev Pathol 2011;14:124-9. [CrossRef] de Noronha L, Cecílio WA, da Silva TF, Maggio EM, Serapião MJ. Congenital peribronchial myofibroblastic tumor: a case report. Pediatr Dev Pathol 2010;13:243-6. [CrossRef]