Pulmonary lipoblastoma
Lipoblastoma of the lung is a fat derived thoracic tumor.
Epidemiology
They occur during infancy and early childhood. More than 90% of cases are diagnosed in children less than 3 years of age, with nearly 75% occurring before the age of 12 months
Pathology
Lipoblastomas are rare soft-tissue mesenchymal tumors principally derived of embryonic white fat . They typically occur in the extremities (70% of cases), with the remainder of cases in mediastinal, retroperitoneal, or paravertebral areas. Occurrence in the lung is rare. They can also involve the chest wall or pleura
They may be divided into two categories:
- lipoblastoma: more common superficial well-defined mass
- lipoblastomatosis: deep, unencapsulated infiltrative lesion
Radiographic features
Plain radiograph
Nonspecific findings - may be seen as a soft-tissue-opacity mass on plain radiographs.
CT
Seen as a fat containing lesion with predominant intratumoral stranding.
MRI
Maybe useful in selected cases. A notable characteristic on MR imaging includes intratumoral streaks and whorls that correspond to the tumor’s fibrovascular network. Cystic changes can be seen. However, lipoblastomas lack the fluid or calcific components.
Differential diagnosis
- myxoid liposarcomas may have a similar CT appearance but are relatively uncommon in children