Pleuropulmonary Blastoma in a Child Presenting with
Spontaneous Pneumothorax
JAMAREE TEERATAKULPISARN, MD*, SURAPON WIANGNON, MD*,
JIRAPORN SRINAKARIN, MD**, CHUSAK KUPTARNOND, MD***,
DARUNEE JINT AKANON, DDS, MSc****
Affiliation : * Departments of Pediatrics,
** Departments of Radiology,
***Departments of Surgery,
****Departments of Pathology, Faculty of Medicine, Khon Kaen University, Khon Kaen 40002, Thailand.
AbstractThe authors described a 27-month-old boy with the diagnosis of pleuropulmonary blastoma
who presented with spontaneous pneumothorax. The child was admitted to our hospital with the chief
complaint of respiratory distress for 8 months. Initial chest X-ray revealed tension pneumothorax on
the right side. After chest tube insertion to the right side, a repeated chest X-ray showed minimal pleural
effusion and a mass-like lesion at the right lower lung field. Computed tomography (CT) of the chest
showed a cavity with intramural mass confined in the right lower lung accompanied with hydro
pneumothorax. The surgery revealed a cystic and solid mass occupying the right pleural space medially
displacing the right lower lung. Total removal of the mass was performed, the histopathologic findings
revealed a mixed cystic and solid type of pleuropulmonary blastoma which was composed of primitive
blastema with multidirectional differentiation. Combination chemotherapy consisting of vincristine,
cyclophosphamide, doxorubicin and dactinomycin was administered two weeks after surgery. The child
has been well for almost 6 months since the surgery, without any signs of metastasis or recurrence.
Keywords : Pleuropulmonary Blastoma, Pneumothorax
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