It's Rare So Be Aware: Pleuropulmonary Blastoma Mimicking Congenital Pulmonary Airway Malformation

Abstract

Pleuropulmonary blastoma (PPB) is a rare aggressive malignant tumor of infancy and early childhood. The tumor arises in the lung and pleura and is regarded as a pulmonary dysontogenetic or embryonic neoplasm. Four types are defined in literature. Type I PPB is a rare, cystic lung neoplasm in infants characterized by subtle malignant changes and a good prognosis. Recurrences after type I PPB are usually advanced with a poor prognosis.We report this case to increase awareness about this entity so that the pediatricians, pediatric surgeons, radiologist, and pathologist recognize it early.

Type II pleuropulmonary blastoma in a 3-years-old female with dyspnea: a case report and review of literature

“…The progression over time from type I to type III tumor is well documented. Natural biological progression explains the correlation of the pathological type with age at the diagnosis and patient outcome [12] .…”

Solitary round pulmonary lesions in the pediatric population: a pictorial review

“…There are five types of CPAM according to the location of the developmental site of malformation: tracheobronchial (type 0) consists of small cysts; bronchial/bronchiolar (type 1) is the most common and consists of cysts measuring 3–10 cm; bronchiolar (type 2) is composed of smaller cysts (0.5–2 cm) and solid tissue; distal acinar (type 3) is composed of cysts that are so small the mass appears to be solid; and finally the bronchiolar/alveolar (type 4) contains cysts as large as 10 cm (4,5). They are usually detected in neonates presenting with respiratory distress and rarely in older children presenting with recurrent infection (3).…”

“…PPB is a very rare, highly aggressive, and malignant tumor, originating from either the lungs or pleura and occurring mainly in children aged < 6 years. They are classified into three types: type I PPB is completely cystic, henceforth is often mistaken with other non-neoplastic cystic lesions such as CPAMs; type II PPB is a mixed lesion with both cystic and solid components; and type ΙΙΙ occurs as an entirely solid mass (5,11,12). The common presenting symptoms are dry cough, fever, dyspnea, tachypnea, chest pain, fatigue, pneumothorax, and respiratory infections that do not resolve with antibiotic therapy.…”

“…They are difficult to diagnose preoperatively since the tumor has no characteristic imaging findings. May appear as a cystic lung lesion or cause a partial or complete opacification of the hemithorax with mediastinal deviation to the contralateral side (5,11).…”

“…Surgical resection is recommended when such malignancy is suspected. Adjuvant chemotherapy also has an important role (5). Suspicion for PPB must be high in any child presenting with a cystic lung lesion and specifically in one with poor weight gain.…”