title: Congenital peribronchial myofibroblastic tumor: comparison of fetal and postnatal morphology.
abstract: Congenital peribronchial myofibroblastic tumor (CPMT) is a solid pulmonary tumor found in fetuses and neonates with pathology characterized by a proliferation of bland spindled cells with or without irregular cartilaginous islands. It has previously been reported in the literature as a fibrosarcoma, leiomyosarcoma, and hamartoma, among other names. Although complications such as fetal hydrops can occur, the prognosis is generally good if the infant can survive long enough for mass resection. We present a case of a CPMT resected by antenatal fetal surgery at 23 weeks in gestation, with additional tumor resected following birth when the infant was 6 weeks of age. The pathology of this lesion showed a marked increase in the cartilaginous component after birth as well as a decrease in cellularity and mitotic activity. This case presents a unique opportunity to examine the progression of a congenital tumor excised by prenatal and postnatal resections.
authors.affiliation: Department of Pathology and Laboratory Medicine, The Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA.
authors.forename: Alison R
authors.initials: AR
authors.lastname: Huppmann
authors.forename: Cheryl M
authors.initials: CM
authors.lastname: Coffin
authors.forename: Andrew C
authors.initials: AC
authors.lastname: Hoot
authors.forename: Samir
authors.initials: S
authors.lastname: Kahwash
authors.forename: Bruce R
authors.initials: BR
authors.lastname: Pawel
mesh_terms: D005260:Female; D005315:Fetal Diseases / Q000473:pathology* / Q000601:surgery; D046128:Fetal Therapies; D005333:Fetus; D005354:Fibrosarcoma / Q000473:pathology* / Q000601:surgery; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008175:Lung Neoplasms / Q000473:pathology* / Q000601:surgery; D011247:Pregnancy; D011296:Prenatal Diagnosis