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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 2010 Dec;34(12):1762-72

Date

11/26/2010

Pubmed ID

21107081

DOI

10.1097/PAS.0b013e3181faf212

Scopus ID

2-s2.0-78650396655   36 Citations

Abstract

The differential diagnosis of congenital lung lesions includes a variety of pulmonary malformations, and uncommon or rare neoplasms such as the pleuropulmonary blastoma (PPB) and congenital peribronchial myofibroblastic tumor (CPMT). Although most of the congenital lesions have a predominantly cystic appearance, the exceptions of a more solid process are the type 3 congenital cystic adenomatoid or pulmonary airway malformation (CCAM-CPAM) and the CPMT. The clinical and pathologic features of a unique solid or mixed solid/cystic lung mass composed of immature interstitial mesenchyme in association with irregular airspace-like structures mimicking abnormal incompletely developed lung are presented in this report of 10 infants (7 males, 3 females) whose tumor-like lesions were detected in the prenatal period to 3 months of age (median, 1-day old). A lobectomy was done in all 10 infants and 1 infant received adjuvant chemotherapy. One of the surgical resections occurred as an ex utero, antenatal procedure because of fetal ascites. There have been no reported recurrences in those patients with greater than 12 months of follow-up ranging from 15 to 182 months (9 cases). Because of the morphologic resemblance of this mass-like lesion to fetal lung at 20 to 24 weeks gestation (as though any further pulmonary development was arrested in these localized lesions), we are proposing the designation of fetal lung interstitial tumor (FLIT) whose pathogenetic relationship, if any, to type 1 (cystic) pleuropulmonary blastoma remains uncertain to date.

Author List

Dishop MK, McKay EM, Kreiger PA, Priest JR, Williams GM, Langston C, Jarzembowski J, Suchi M, Dehner LP, Hill DA

Authors

Jason A. Jarzembowski MD, PhD Vice Chair, Chief, Professor in the Pathology department at Medical College of Wisconsin
Mariko Suchi MD, PhD Associate Professor in the Pathology department at Medical College of Wisconsin




MESH terms used to index this publication - Major topics in bold

Adenocarcinoma
Combined Modality Therapy
Cystic Adenomatoid Malformation of Lung, Congenital
Diagnosis, Differential
Female
Humans
Infant
Infant, Newborn
Lung Neoplasms
Male
Prenatal Diagnosis
Pulmonary Blastoma
jenkins-FCD Prod-482 91ad8a360b6da540234915ea01ff80e38bfdb40a