We present a prenatal case of congenital peribronchial myofibroblastic tumor referred as a congenital pulmonary airway malformation (CPAM) with hydrops and polyhydramnios at 30 weeks’ gestational age. US and fetal MRI findings did not fit with the referral diagnosis, raising the possibility of intrinsic lung tumor. Fetal hydrops worsened and the baby was successfully delivered by ex utero intrapartum treatment (EXIT) to resection at 31 weeks’ gestational age. To the best of our knowledge, this is a unique case of congenital peribronchial myofibroblastic tumor that underwent comprehensive prenatal evaluation and EXIT procedure with good outcome.
This is a preview of subscription content, log in to check access.
Conflicts of interest
McGinnis M, Jacobs G, el-Naggar A et al (1993) Congenital peribronchial myofibroblastic tumor (so-called ‘congenital leiomyosarcoma’). A distinct neonatal lung lesion associated with nonimmune hydrops fetalis. Mod Pathol 6:487–492PubMedGoogle Scholar
Reiss A, Goldberg Y, Monichor M et al (2005) Congenital pulmonary myofibroblastic tumor—pathology and prenatal sonographic appearance. Prenat Diagn 25:1064–1066PubMedCrossRefGoogle Scholar
Horikoshi T, Kikuchi A, Matsumoto Y et al (2005) Fetal hydrops associated with congenital pulmonary myofibroblastic tumor. J Obstet Gynaecol Res 31:552–555PubMedCrossRefGoogle Scholar
Rypens F, Metens T, Rocourt N et al (2001) Fetal lung volume: estimation at MR imaging—initial results. Radiology 219:236–241PubMedCrossRefGoogle Scholar
Beydon N, Larroquet M, Coulomb A et al (2013) Comparison between US and MRI in the prenatal assessment of lung malformations. Pediatr Radiol 43:685–696PubMedCrossRefGoogle Scholar
Dishop MK, Kuruvilla S (2008) Primary and metastatic lung tumors in the pediatric population: a review and 25-year experience at a large children’s hospital. Arch Pathol Lab Med 132:1079–1103PubMedGoogle Scholar
Dishop MK, McKay EM, Kreiger PA et al (2010) Fetal lung interstitial tumor (FLIT): a proposed newly recognized lung tumor of infancy to be differentiated from cystic pleuropulmonary blastoma and other developmental pulmonary lesions. Am J Surg Pathol 34:1762–1772PubMedCrossRefGoogle Scholar
Huppmann AR, Coffin CM, Hoot AC et al (2011) Congenital peribronchial myofibroblastic tumor: comparison of fetal and postnatal morphology. Pediatr Dev Pathol 14:124–129PubMedCrossRefGoogle Scholar