title: Respiratory insufficiency in a newborn with mesenchymal hamartoma of the chest wall occupying the thoracic cavity.
abstract: The authors describe a newborn patient with mesenchymal hamartoma of the chest wall associated with pulmonary hypoplasia. A massive thoracic tumor was diagnosed by prenatal ultrasonography and magnetic resonance imaging at the 28th week of gestation. She was delivered through cesarean delivery at the 36th gestational week. Respiratory distress because of pulmonary hypoplasia necessitated neonatal intensive care. The tumor extensively involved the left hemithorax including all 12 ribs and the first 10 thoracic vertebrae, resulting in marked deformity of the thorax. At 5 days of age, she underwent the incisional biopsy through a left thoracotomy. Histopathology of biopsy specimens showed multiple components of mesenchymal origin including premature cartilage, bone, and cystic lesions resembling aneurysmal bone cyst. The tumor then showed a rapid overgrowth, but subsequently exhibited a self-limited growth for months, in which her respiratory condition gradually improved to spontaneous breathing without oxygenation support. The present case advocates perinatal preparations for associated pulmonary hypoplasia and conservative management for the neoplasm in fetuses prenatally diagnosed as having this unique pathological entity.
authors.affiliation: Division of Surgery, Children's Research Hospital, Kyoto Prefectural University of Medicine, Kyoto 602-0841, Japan. shimo@koto.kpu-m.ac.jp <shimo@koto.kpu-m.ac.jp>
authors.forename: Takashi
authors.initials: T
authors.lastname: Shimotake
authors.forename: Shigehisa
authors.initials: S
authors.lastname: Fumino
authors.forename: Shigeyoshi
authors.initials: S
authors.lastname: Aoi
authors.forename: Tomoki
authors.initials: T
authors.lastname: Tsuda
authors.forename: Naomi
authors.initials: N
authors.lastname: Iwai
mesh_terms: D001706:Biopsy; D005260:Female; D006222:Hamartoma / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology; D006801:Humans; D007231:Infant, Newborn; D008648:Mesoderm; D012075:Remission, Spontaneous; D012127:Respiratory Distress Syndrome, Newborn / Q000209:etiology*; D013899:Thoracic Neoplasms / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology; D016216:Ultrasonography, Prenatal