Pleuropulmonary blastoma is a rare malignancy in young adult and childhood

Abstract

Case 1 A 3.5-year-old girl presented with 3 week history of shortness of breath, non-productive cough, fever and right-sided non-pleuritic chest pain. She was taken two courses of oral antibiotics but without any improvement. She was previously fit and well without history of exposure to second hand smoking. Initial investigations showed normal complete blood counts, liver and kidney function tests. Serum electrolytes and urine analysis were also normal. Chest radiography revealed complete opacification of the right hemithorax, contralateral mediastinal shifting consistent with right sided large pleural effusion (Fig. 1). Contrast enhanced chest computed tomography scan (CT scan) confirmed large right pleural effusion with associated mediastinal shift and complete right lung collapse. No abnormality was reported in the left hemithorax or upper abdomen. Right thoracotomy and decortication revealed large un-resectable masses with adherent deposits on the diaphragm and enlarged pericardial lymph nodes. The right middle lobe was obliterated. Multiple biopsies were taken from tumor and lymph nodes. Pleural fluid analysis demonstrated an exudative pattern. Bone marrow aspiration and biopsy were normal.Due to her critical condition with suspicion of primitive neuroec-

A rare case of type III pleuropulmonary blastoma infiltrating the left heart in an 11-year-old girl

“…Patients with type I PPB can be treated by surgical resection with negative margins and no tumor spill with possible additional chemotherapy ( 11 ). Complete resection might be achieved since type I PBB has a definable margin.…”

“…However, Paolo et al said that local radiotherapy after initial radical surgery is an effective adjunct therapy ( 3 ). Although the European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT) recommends giving neoadjuvant chemotherapy before surgery ( 14 ), the urgency to relieve the circulatory obstruction and the possibility of worsening the malnutrition condition are the reason not to give neoadjuvant therapy like what was done in some cases ( 8 , 11 , 15 ) and immediate debulking surgery can be considered based on EXPeRT. ( 14 ) Recommendations from EXPeRT are expert opinions and standard-evidence-based therapy for rare tumors like PPB has not been established yet.…”