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

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVCongenital Peribronchial Myofibroblastic Tumor

Disease, Respiratory Tract
- Disorder of lung
  - Neoplasm of lung
    - Lung Soft Tissue Neoplasm
      - Congenital 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

See all (1)

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: 37081446 Free 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

See all (3)

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: 37081446 Free 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: 26496330 Free 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

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

See all (8)

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: 37081446 Free 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: 26496330 Free 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

See all (5)