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Fetal lung interstitial tumor

MedGen UID:
1830091
•Concept ID:
C5679952
•
Neoplastic Process
Synonyms: Fetal Lung Interstitial Tumor; fetal lung interstitial tumor; FLIT; flit; Immature interstitial mesenchymal tumor; immature interstitial mesenchymal tumor; immature interstitial mesenchymal tumour
 
Monarch Initiative: MONDO:0017289
Orphanet: ORPHA284362

Definition

A congenital benign tumor of lung interstitial mesenchymal cells that presents with respiratory symptoms in neonates. Images reveal a well-circumscribed solid or partially cystic lung mass. Microscopical examination reveals widened alveolar septa that contain bland, uniform mesenchymal cells. Some cases show ALK rearrangement and A2M-ALK gene fusion. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVFetal lung interstitial tumor

Professional guidelines

PubMed

Neonatal congenital lung tumors - the importance of mid-second-trimester ultrasound as a diagnostic clue.
Waelti SL, Garel L, Soglio DD, Rypens F, Messerli M, Dubois J
Pediatr Radiol 2017 Dec;47(13):1766-1775. Epub 2017 Sep 7 doi: 10.1007/s00247-017-3953-3. PMID: 28884206

Recent clinical studies

Etiology

Primary Lung Tumors in Children: Radiologic-Pathologic Correlation From the Radiologic Pathology Archives.
Lichtenberger JP 3rd, Biko DM, Carter BW, Pavio MA, Huppmann AR, Chung EM
Radiographics 2018 Nov-Dec;38(7):2151-2172. doi: 10.1148/rg.2018180192. PMID: 30422774
Neonatal congenital lung tumors - the importance of mid-second-trimester ultrasound as a diagnostic clue.
Waelti SL, Garel L, Soglio DD, Rypens F, Messerli M, Dubois J
Pediatr Radiol 2017 Dec;47(13):1766-1775. Epub 2017 Sep 7 doi: 10.1007/s00247-017-3953-3. PMID: 28884206
Fetal lung interstitial tumor: a cause of late gestation fetal hydrops.
Lazar DA, Cass DL, Dishop MK, Adam K, Olutoye OA, Ayres NA, Cassady CI, Olutoye OO
J Pediatr Surg 2011 Jun;46(6):1263-6. doi: 10.1016/j.jpedsurg.2011.02.056. PMID: 21683234

Diagnosis

Spontaneous Partial Regression of Fetal Lung Interstitial Tumor With A2M::ALK Rearrangement in a Neonate.
Tan-Garcia A, Lee YT, Kuick CH, Soh SY, Chang KT, Merchant K
Pediatr Dev Pathol 2024 Mar-Apr;27(2):187-192. Epub 2023 Oct 11 doi: 10.1177/10935266231189929. PMID: 37818649
Fetal lung interstitial tumor: Prenatal presentation of a rare fetal malignancy.
Phillips J, Blask A, DiPoto Brahmbhatt A, Lawrence A, Timofeev J, Badillo A, Andescavage N
J Neonatal Perinatal Med 2019;12(4):473-477. doi: 10.3233/NPM-180059. PMID: 31256075
Primary Lung Tumors in Children: Radiologic-Pathologic Correlation From the Radiologic Pathology Archives.
Lichtenberger JP 3rd, Biko DM, Carter BW, Pavio MA, Huppmann AR, Chung EM
Radiographics 2018 Nov-Dec;38(7):2151-2172. doi: 10.1148/rg.2018180192. PMID: 30422774
Fetal lung interstitial tumor: a cause of late gestation fetal hydrops.
Lazar DA, Cass DL, Dishop MK, Adam K, Olutoye OA, Ayres NA, Cassady CI, Olutoye OO
J Pediatr Surg 2011 Jun;46(6):1263-6. doi: 10.1016/j.jpedsurg.2011.02.056. PMID: 21683234
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.
Dishop MK, McKay EM, Kreiger PA, Priest JR, Williams GM, Langston C, Jarzembowski J, Suchi M, Dehner LP, Hill DA
Am J Surg Pathol 2010 Dec;34(12):1762-72. doi: 10.1097/PAS.0b013e3181faf212. PMID: 21107081

Therapy

Fetal lung interstitial tumor: Prenatal presentation of a rare fetal malignancy.
Phillips J, Blask A, DiPoto Brahmbhatt A, Lawrence A, Timofeev J, Badillo A, Andescavage N
J Neonatal Perinatal Med 2019;12(4):473-477. doi: 10.3233/NPM-180059. PMID: 31256075

Prognosis

Inflammatory myofibroblastic tumors of the lung carrying a chimeric A2M-ALK gene: report of 2 infantile cases and review of the differential diagnosis of infantile pulmonary lesions.
Tanaka M, Kohashi K, Kushitani K, Yoshida M, Kurihara S, Kawashima M, Ueda Y, Souzaki R, Kinoshita Y, Oda Y, Takeshima Y, Hiyama E, Taguchi T, Tanaka Y
Hum Pathol 2017 Aug;66:177-182. Epub 2017 Jul 11 doi: 10.1016/j.humpath.2017.06.013. PMID: 28705706
Fetal lung interstitial tumor: a cause of late gestation fetal hydrops.
Lazar DA, Cass DL, Dishop MK, Adam K, Olutoye OA, Ayres NA, Cassady CI, Olutoye OO
J Pediatr Surg 2011 Jun;46(6):1263-6. doi: 10.1016/j.jpedsurg.2011.02.056. PMID: 21683234

Clinical prediction guides

Inflammatory myofibroblastic tumors of the lung carrying a chimeric A2M-ALK gene: report of 2 infantile cases and review of the differential diagnosis of infantile pulmonary lesions.
Tanaka M, Kohashi K, Kushitani K, Yoshida M, Kurihara S, Kawashima M, Ueda Y, Souzaki R, Kinoshita Y, Oda Y, Takeshima Y, Hiyama E, Taguchi T, Tanaka Y
Hum Pathol 2017 Aug;66:177-182. Epub 2017 Jul 11 doi: 10.1016/j.humpath.2017.06.013. PMID: 28705706

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