Radiography

Radiographic findings of primary PPB are as follows:

  • benign-appearing cystic lung disease with or without pneumothorax
  • partial opacification of a portion of the lung suggesting pneumonia
  • complete opacification of the hemithorax
  • Mediastinal shift and tension pneumothorax
  • Pleural effusion

CT scans may show a mass lesion or suggest lobar or larger consolidation. CTs may also show multiloculated cystic or cystic/solid changes. PPB often appears to involve the lung parenchyma and pleura, but the chest wall peripherally or the mediastinum centrally may be involved as well. Because of the constitutional/syndromic peculiarities of PPB, bilateral manifestations must be assiduously sought in radiographic studies. The rare possibility of vascular invasion and superior vena cava syndrome must also be considered; pulmonary vein invasion with extension into the left atrium has been reported.

The radiography of metastatic PPB depends on the organ system(s) affected, discussed above.

Type I PPB

 Radiology unilocular cyst

Type I PPB: 1. Unilocular cyst in a 9 month-old child; resected; dx: Type I PPB

radiology unilocular cyst later

Type I PPB: 2. Same child as above 16 months later; no treatment had been given; additional cysts enlarging slightly; resected; dx: benign lung cysts.

Type II PPB

 radiology type2 39months

Type II PPB: This Type II PPB developed 39 months after the child first had a pneumothorax. Prior multifocal and bilateral cysts were judged to be benign. The child is well ~48 months after Type II diagnosis.

radiology mostly cystic

Type II PPB in a mostly cystic lesion (photo courtesy of Maureen O’Sullivan, MD)

radiology multilocular cyst

 Type II PPB in a multilocular cyst (photo courtesy of Maureen O’Sullivan, MD)

Type III PPB

radiology type3 ct scan

Type III PPB: Initial chest CT scan and CXR in Type III PPB. (This child developed brain metastasis 13 months later, while the chest disease was under control. The child is well 16 months after brain metastasis.)

Example of Cerebral Metastases in PPB radiology CNS Metastasis

CNS Metastasis CNS Metastasis in PPB: Reg #033. type III PPB, 6months after diagnosis.

Example of Cerebral Metastases in PPB (following earlier embolic stroke) radiology Cerebral-Metastasis-Reg-118

Enhanced CT Scan of Cerebral Metastasis
This cerebral metastasis became symptomatic and was diagnosed approximately one year after the initial post-operative embolic infarct. See earlier photo. Registry Case #118. (See Tan Kendrick A. Cerebral metastasis proven 1 year after an embolic cerebral infarct from pleuropulmonary blastoma [Letter to the Editor]. Pediatr Radiol 2004;34(3):283.) Courtesy of Dr Anne Tan Kendrick.