The misdiagnosis of pleuropulmonary blastoma in children

Abstract

Objective To analyze the reasons of misdiagnosis of pleuropulmonary blastoma in children. Methods Thirty two cases of pleuropulmonary blastoma from January 1979 to January 2007 were reviewed. They were divided into 3 types according to Dehuer classification with pathological and immunohistochemical evidences. The reasons of misdiagnosis of this disease were analyzed by clinical manifestations, X-ray and CT examination. And the survival rate was also recorded. Results The mean age of diagnosis gained was 4.0 ± 2.7 years (3 months to 10 years). The initial symptom was cough in all children. Twenty eight children had fever. Lately dyspnea appeared in 18 children. Chest radiography showed lung cyst formation in 6 children, pyopneumothorax or pleurisy in 5, pneumonia in 18, and mediastinal mass in 2. Of all these children, except for 2 with autopsy and 3 with biopsy, operations were performed on the other 27 children, and followed by postoperative chemotherapy. Mean follow-up time was 36. 5 months (1 to 144 months). Thirteen children survived, 18 children died and 1 child lost follow-up. Two-year survival rate and 5-year survival rate was 35.5% and 29.0%, respectively. Mean duration from diagnosis to death ranged from 1 day to 26 months. Conclusions Pleuropulmonary blastoma in children is a rare malignant tumor, whose clinical manifestation is quit different from such a tumor in adults, and is very easy to get misdiagnosis. Recognition of this disease, early diagnosis, complete resection, and comprehensive therapy are the key measures to get satisfactory prognosis of pleuropulmonary blastoma in children. Key words: Lung neoplasms; Pleural neoplasms