Pediatric Radiology

, Volume 47, Issue 13, pp 1766–1775 | Cite as

Neonatal congenital lung tumors — the importance of mid-second-trimester ultrasound as a diagnostic clue

  • Stephan L. Waelti
  • Laurent GarelEmail author
  • Dorothée Dal Soglio
  • Françoise Rypens
  • Michael Messerli
  • Josée Dubois
Original Article



The differential diagnosis for primary lung masses in neonates includes a variety of developmental abnormalities; it also consists of the much rarer congenital primary lung tumors: cystic pleuropulmonary blastoma (cystic PPB), fetal lung interstitial tumor (FLIT), congenital peribronchial myofibroblastic tumor (CPMT), and congenital fibrosarcoma. Radiologic differentiation between malformations and tumors is often very challenging.


The objective was to establish distinctive features between developmental pulmonary abnormalities and primary lung tumors.

Materials and methods

We conducted a retrospective study of 135 congenital lung lesions at a university mother and child center over a period of 10 years (2005–2015). During this time, we noted four tumors (two cystic PPBs and two FLITs) and 131 malformations. We recorded the following parameters: timing of conspicuity in utero (mid-second trimester, third trimester, or not seen prenatally), presence of symptoms at birth, prenatal and perinatal radiologic findings, and either histological diagnoses by pathology or follow-up imaging in non-operated cases.


All lesions except the four tumors were detected during mid-second-trimester ultrasound. In none of the tumors was any pulmonary abnormality found on the mid-second-trimester sonogram, contrary to the developmental pulmonary abnormalities.


The timing of conspicuity in utero appears to be a key feature for the differentiation between malformations and tumors. Lesions that were not visible at the mid-second-trimester ultrasound should be considered as tumor. A cystic lung lesion in the context of a normal mid-second-trimester ultrasound is highly suggestive of a cystic PPB. Differentiating the types of solid congenital lung tumors based upon imaging features is not yet feasible.


Computed tomography Fetus Lungs Malformations Neonates Radiography Tumors Ultrasound 



The results of this study were presented at the International Pediatric Radiology Conjoint Meeting and Exhibition 2016 in Chicago, IL.

Compliance with ethical standards

Conflicts of interest



  1. 1.
    Lazar DA, Cass DL, Dishop MK et al (2011) Fetal lung interstitial tumor: a cause of late gestation fetal hydrops. J Pediatr Surg 46:1263–1266CrossRefPubMedGoogle Scholar
  2. 2.
    Stocker JT (2009) Cystic lung disease in infants and children. Fetal Pediatr Pathol 28:155–184CrossRefPubMedGoogle Scholar
  3. 3.
    Langston C (2003) New concepts in the pathology of congenital lung malformations. Semin Pediatr Surg 12:17–37CrossRefPubMedGoogle Scholar
  4. 4.
    Calvo-Garcia MA, Lim FY, Stanek J et al (2014) Congenital peribronchial myofibroblastic tumor: prenatal imaging clues to differentiate from other fetal chest lesions. Pediatr Radiol 44:479–483CrossRefPubMedGoogle Scholar
  5. 5.
    Dishop MK, Kuruvilla S (2008) Primary and metastatic lung tumors in the pediatric population: a review and 25-year experience at a large children's hospital. Arch Pathol Lab Med 132:1079–1103PubMedGoogle Scholar
  6. 6.
    Dishop MK, McKay EM, Kreiger PA et al (2010) 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 34:1762–1772CrossRefPubMedGoogle Scholar
  7. 7.
    Huppmann AR, Coffin CM, Hoot AC et al (2011) Congenital peribronchial myofibroblastic tumor: comparison of fetal and postnatal morphology. Pediatr Dev Pathol 14:124–129CrossRefPubMedGoogle Scholar
  8. 8.
    Stanton M, Njere I, Ade-Ajayi N et al (2009) Systematic review and meta-analysis of the postnatal management of congenital cystic lung lesions. J Pediatr Surg 44:1027–1033CrossRefPubMedGoogle Scholar
  9. 9.
    Priest JR, McDermott MB, Bhatia S et al (1997) Pleuropulmonary blastoma: a clinicopathologic study of 50 cases. Cancer 80:147–161CrossRefPubMedGoogle Scholar
  10. 10.
    Priest JR, Magnuson J, Williams GM et al (2007) Cerebral metastasis and other central nervous system complications of pleuropulmonary blastoma. Pediatr Blood Cancer 49:266–273CrossRefPubMedGoogle Scholar
  11. 11.
    Hill DA, Jarzembowski JA, Priest JR et al (2008) Type I pleuropulmonary blastoma: pathology and biology study of 51 cases from the international pleuropulmonary blastoma registry. Am J Surg Pathol 32:282–295CrossRefPubMedGoogle Scholar
  12. 12.
    Messinger YH, Stewart DR, Priest JR et al (2015) Pleuropulmonary blastoma: a report on 350 central pathology-confirmed pleuropulmonary blastoma cases by the international Pleuropulmonary Blastoma registry. Cancer 121:276–285CrossRefPubMedGoogle Scholar
  13. 13.
    Priest JR, Williams GM, Hill DA et al (2009) Pulmonary cysts in early childhood and the risk of malignancy. Pediatr Pulmonol 44:14–30CrossRefPubMedGoogle Scholar
  14. 14.
    Brock KE, Wall J, Esquivel M et al (2015) Congenital peribronchial myofibroblastic tumor: case report of an asymptomatic infant with a rapidly enlarging pulmonary mass and review of the literature. Ann Clin Lab Sci 45:83–89PubMedGoogle Scholar
  15. 15.
    Priest JR, Hill DA, Williams GM et al (2006) Type I pleuropulmonary blastoma: a report from the international Pleuropulmonary Blastoma registry. J Clin Oncol 24:4492–4498CrossRefPubMedGoogle Scholar
  16. 16.
    Hill DA (2005) USCAP specialty conference: case 1-type I pleuropulmonary blastoma. Pediatr Dev Pathol 8:77–84CrossRefPubMedGoogle Scholar
  17. 17.
    Naffaa LN, Donnelly LF (2005) Imaging findings in pleuropulmonary blastoma. Pediatr Radiol 35:387–391CrossRefPubMedGoogle Scholar
  18. 18.
    Boman F, Hill DA, Williams GM et al (2006) Familial association of pleuropulmonary blastoma with cystic nephroma and other renal tumors: a report from the international Pleuropulmonary Blastoma registry. J Pediatr 149:850–854CrossRefPubMedGoogle Scholar
  19. 19.
    Miniati DN, Chintagumpala M, Langston C et al (2006) Prenatal presentation and outcome of children with pleuropulmonary blastoma. J Pediatr Surg 41:66–71CrossRefPubMedGoogle Scholar
  20. 20.
    Reiss A, Goldberg Y, Monichor M, Drugan A (2005) Congenital pulmonary myofibroblastic tumor — pathology and prenatal sonographic appearance. Prenat Diagn 25:1064–1066CrossRefPubMedGoogle Scholar
  21. 21.
    McGinnis M, Jacobs G, El-Naggar A, Redline RW (1993) Congenital peribronchial myofibroblastic tumor (so-called 'congenital leiomyosarcoma'). A distinct neonatal lung lesion associated with nonimmune hydrops fetalis. Mod Pathol 6:487–492PubMedGoogle Scholar
  22. 22.
    Haller JO, Kauffman SL, Kassner EG (1977) Congenital mesenchymal tumour of the lung. Br J Radiol 50:217–219CrossRefPubMedGoogle Scholar
  23. 23.
    Jimenez JF, Uthman EO, Townsend JW et al (1986) Primary bronchopulmonary leiomyosarcoma in childhood. Arch Pathol Lab Med 110:348–351PubMedGoogle Scholar
  24. 24.
    Horikoshi T, Kikuchi A, Matsumoto Y et al (2005) Fetal hydrops associated with congenital pulmonary myofibroblastic tumor. J Obstet Gynaecol Res 31:552–555CrossRefPubMedGoogle Scholar
  25. 25.
    de Noronha L, Cecilio WA, da Silva TF et al (2010) Congenital peribronchial myofibroblastic tumor: a case report. Pediatr Dev Pathol 13:243–246CrossRefPubMedGoogle Scholar
  26. 26.
    Kim Y, Park HY, Cho J et al (2013) Congenital peribronchial myofibroblastic tumor: a case study and literature review. Korean J Pathol 47:172–176CrossRefPubMedPubMedCentralGoogle Scholar
  27. 27.
    Loh ML, Ahn P, Perez-Atayde AR et al (2002) Treatment of infantile fibrosarcoma with chemotherapy and surgery: results from the Dana-Farber Cancer Institute and Children's hospital, Boston. J Pediatr Hematol Oncol 24:722–726CrossRefPubMedGoogle Scholar
  28. 28.
    Steelman C, Katzenstein H, Parham D et al (2011) Unusual presentation of congenital infantile fibrosarcoma in seven infants with molecular-genetic analysis. Fetal Pediatr Pathol 30:329–337CrossRefPubMedGoogle Scholar
  29. 29.
    Tarik E, Lamiae R, Abdelouahed A (2013) Unusual case of congenital/infantile fibrosarcoma in a new born. Afr J Paediatr Surg 10:185–187CrossRefPubMedGoogle Scholar
  30. 30.
    Minard-Colin V, Orbach D, Martelli H et al (2009) Soft tissue tumors in neonates. Arch Pediatr 16:1039–1048CrossRefPubMedGoogle Scholar
  31. 31.
    Sahin D, Koç N, Etker S et al (2010) Congenital pulmonary fibrosarcoma in a newborn with hypoglycemia and respiratory distress: case report. Turk J Pediatr 52:325–329PubMedGoogle Scholar
  32. 32.
    Picard E, Udassin R, Ramu N et al (1999) Pulmonary fibrosarcoma in childhood: fiber-optic bronchoscopic diagnosis and review of the literature. Pediatr Pulmonol 27:347–350CrossRefPubMedGoogle Scholar
  33. 33.
    Scheier M, Ramoni A, Alge A et al (2008) Congenital fibrosarcoma as cause for fetal anemia: prenatal diagnosis and in utero treatment. Fetal Diagn Ther 24:434–436CrossRefPubMedGoogle Scholar
  34. 34.
    Ainsworth KE, Chavhan GB, Gupta AA et al (2014) Congenital infantile fibrosarcoma: review of imaging features. Pediatr Radiol 44:1124–1129CrossRefPubMedGoogle Scholar
  35. 35.
    Lee MJ, Cairns RA, Munk PL, Poon PY (1996) Congenital-infantile fibrosarcoma: magnetic resonance imaging findings. Can Assoc Radiol J 47:121–125PubMedGoogle Scholar
  36. 36.
    Pettinato G, Manivel JC, Saldana MJ et al (1989) Primary bronchopulmonary fibrosarcoma of childhood and adolescence: reassessment of a low-grade malignancy. Clinicopathologic study of five cases and review of the literature. Hum Pathol 20:463–471CrossRefPubMedGoogle Scholar
  37. 37.
    Kuhnen C, Harms D, Niessen KH et al (2001) Congenital pulmonary fibrosarcoma. Differential diagnosis of infantile pulmonary spindle cell tumors. Pathologe 22:151–156CrossRefPubMedGoogle Scholar
  38. 38.
    Feinberg A, Hall NJ, Williams GM et al (2016) Can congenital pulmonary airway malformation be distinguished from type I pleuropulmonary blastoma based on clinical and radiological features? J Pediatr Surg 51:33–37CrossRefPubMedGoogle Scholar
  39. 39.
    Kunisaki SM, Barnewolt CE, Estroff JA et al (2007) Large fetal congenital cystic adenomatoid malformations: growth trends and patient survival. J Pediatr Surg 42:404–410CrossRefPubMedGoogle Scholar
  40. 40.
    Adzick NS (2003) Management of fetal lung lesions. Clin Perinatol 30:481–492CrossRefPubMedGoogle Scholar
  41. 41.
    Crombleholme TM, Coleman B, Hedrick H et al (2002) Cystic adenomatoid malformation volume ratio predicts outcome in prenatally diagnosed cystic adenomatoid malformation of the lung. J Pediatr Surg 37:331–338CrossRefPubMedGoogle Scholar
  42. 42.
    Ng C, Stanwell J, Burge DM, Stanton MP (2014) Conservative management of antenatally diagnosed cystic lung malformations. Arch Dis Child 99:432–437CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany 2017

Authors and Affiliations

  • Stephan L. Waelti
    • 1
  • Laurent Garel
    • 1
    Email author
  • Dorothée Dal Soglio
    • 2
  • Françoise Rypens
    • 1
  • Michael Messerli
    • 3
  • Josée Dubois
    • 1
  1. 1.Department of Medical Imaging, Sainte-Justine HospitalUniversity of MontrealQuebecCanada
  2. 2.Department of Pathology, Sainte-Justine HospitalUniversity of MontrealQuebecCanada
  3. 3.Department of Nuclear MedicineUniversity Hospital Zurich, University of ZurichZurichSwitzerland

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