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Congenital Peribronchial Myofibroblastic Tumor

MedGen UID:
690109
•Concept ID:
C1266124
•
Neoplastic Process
Synonyms: Congenital Bronchopulmonary Leiomyosarcoma; Congenital Fibrosarcoma; Congenital Mesenchymal Malformation of Lung; Congenital peribronchial myofibroblastic tumor; Congenital Pulmonary Myofibroblastic Tumor; Myofibroblastic tumor, peribronchial; Neonatal Pulmonary Hamartoma
SNOMED CT: Congenital peribronchial myofibroblastic tumor (128740007); Myofibroblastic tumor, peribronchial (128740007)

Definition

A rare lung neoplasm that occurs in the intrauterine and perinatal period. It is characterized by the proliferation of spindle cells in an interstitial, peribronchial pattern. Surgical resection of the involved lung parenchyma is the treatment of choice. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCongenital Peribronchial Myofibroblastic 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

Congenital peribronchial myofibroblastic tumor (CPMT): a case report with long term follow-up and next-generation sequencing (NGS).
Zhou P, Li S, Wang W, Tang Y, Jiang L
BMC Pediatr 2023 Apr 20;23(1):184. doi: 10.1186/s12887-023-04001-5. PMID: 37081446Free PMC Article
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
Primary and metastatic lung tumors in the pediatric population: a review and 25-year experience at a large children's hospital.
Dishop MK, Kuruvilla S
Arch Pathol Lab Med 2008 Jul;132(7):1079-103. doi: 10.5858/2008-132-1079-PAMLTI. PMID: 18605764

Diagnosis

Congenital peribronchial myofibroblastic tumor (CPMT): a case report with long term follow-up and next-generation sequencing (NGS).
Zhou P, Li S, Wang W, Tang Y, Jiang L
BMC Pediatr 2023 Apr 20;23(1):184. doi: 10.1186/s12887-023-04001-5. PMID: 37081446Free PMC Article
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
A Congenital Peribronchial Myofibroblastic Tumor Detected in a Premature Infant at 28 Weeks but That Resolved in the Late Stage of Pregnancy: A Case Report.
Xia B, Yu G, Hong C, Zhang L, Tang J, Liu C
Medicine (Baltimore) 2015 Oct;94(42):e1842. doi: 10.1097/MD.0000000000001842. PMID: 26496330Free PMC Article
Congenital peribronchial myofibroblastic tumor: case report of an asymptomatic infant with a rapidly enlarging pulmonary mass and review of the literature.
Brock KE, Wall J, Esquivel M, Newman B, Marina N, Albanese C, Hazard FK
Ann Clin Lab Sci 2015 Winter;45(1):83-9. PMID: 25696016
Congenital peribronchial myofibroblastic tumor: prenatal imaging clues to differentiate from other fetal chest lesions.
Calvo-Garcia MA, Lim FY, Stanek J, Bitters C, Kline-Fath BM
Pediatr Radiol 2014 Apr;44(4):479-83. Epub 2013 Oct 31 doi: 10.1007/s00247-013-2817-8. PMID: 24169903

Prognosis

Congenital peribronchial myofibroblastic tumor (CPMT): a case report with long term follow-up and next-generation sequencing (NGS).
Zhou P, Li S, Wang W, Tang Y, Jiang L
BMC Pediatr 2023 Apr 20;23(1):184. doi: 10.1186/s12887-023-04001-5. PMID: 37081446Free PMC Article
A Congenital Peribronchial Myofibroblastic Tumor Detected in a Premature Infant at 28 Weeks but That Resolved in the Late Stage of Pregnancy: A Case Report.
Xia B, Yu G, Hong C, Zhang L, Tang J, Liu C
Medicine (Baltimore) 2015 Oct;94(42):e1842. doi: 10.1097/MD.0000000000001842. PMID: 26496330Free PMC Article
Congenital peribronchial myofibroblastic tumor: case report of an asymptomatic infant with a rapidly enlarging pulmonary mass and review of the literature.
Brock KE, Wall J, Esquivel M, Newman B, Marina N, Albanese C, Hazard FK
Ann Clin Lab Sci 2015 Winter;45(1):83-9. PMID: 25696016
Congenital peribronchial myofibroblastic tumor: comparison of fetal and postnatal morphology.
Huppmann AR, Coffin CM, Hoot AC, Kahwash S, Pawel BR
Pediatr Dev Pathol 2011 Mar-Apr;14(2):124-9. Epub 2010 Apr 5 doi: 10.2350/09-12-0767-CR.1. PMID: 20367454
Primary and metastatic lung tumors in the pediatric population: a review and 25-year experience at a large children's hospital.
Dishop MK, Kuruvilla S
Arch Pathol Lab Med 2008 Jul;132(7):1079-103. doi: 10.5858/2008-132-1079-PAMLTI. PMID: 18605764

Clinical prediction guides

Congenital peribronchial myofibroblastic tumor: case report of an asymptomatic infant with a rapidly enlarging pulmonary mass and review of the literature.
Brock KE, Wall J, Esquivel M, Newman B, Marina N, Albanese C, Hazard FK
Ann Clin Lab Sci 2015 Winter;45(1):83-9. PMID: 25696016
Congenital peribronchial myofibroblastic tumor: a case report.
de Noronha L, Cecílio WA, da Silva TF, Maggio EM, Serapião MJ
Pediatr Dev Pathol 2010 May-Jun;13(3):243-6. doi: 10.2350/07-05-0282.1. PMID: 20064015

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