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.
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.
Department of Pathology and Laboratory Medicine, The Children's Hospital, Aurora, CO, USA.
Megan K
MK
Dishop
Eileen M
EM
McKay
Portia A
PA
Kreiger
John R
JR
Priest
Gretchen M
GM
Williams
Claire
C
Langston
Jason
J
Jarzembowski
Mariko
M
Suchi
Louis P
LP
Dehner
D Ashley
DA
Hill
D000230:Adenocarcinoma / Q000151:congenital / Q000175:diagnosis* / Q000628:therapy; D003131:Combined Modality Therapy; D015615:Cystic Adenomatoid Malformation of Lung, Congenital / Q000175:diagnosis; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008175:Lung Neoplasms / Q000151:congenital / Q000175:diagnosis* / Q000628:therapy; D008297:Male; D011296:Prenatal Diagnosis; D018202:Pulmonary Blastoma / Q000151:congenital / Q000175:diagnosis*