Primary Pleuropulmonary Neoplasms in Childhood: Fourteen Cases from a Single Center

Background: We aimed to review clinical characteristics, treatment results and outcome of pediatric patientswith primary pleuropulmonary neoplasms.
Methods: Medical records of 14 cases diagnosed between 1972-2009were reviewed retrospectively.
Results: The male/female ratio was 5/9 and the mean age at diagnosis was 9.1years (2-16). All but one were symptomatic, presenting with fever, coughing, dyspnea, or weight loss. One patientpresented with hemoptysis, and another with digital clubbing. One mesothelioma was diagnosed incidentally.Some 8/14 patients were initially diagnosed as having pneumonia (median delay in diagnosis of 2.5 months).Diagnoses included pleuropulmonary blastoma (PPB, n=5), inflammatory pseudotumor (n=3), mesothelioma(n=2), mucoepidermoid carcinoma (MEC, n=2), and carcinoid tumor (n=2). Patients with PPB underwent surgeryand received chemotherapy ± radiotherapy. Two carcinoid tumor cases underwent surgery, one further receivedchemotherapy. Patients with mesothelioma were treated with chemotherapy. Inflammatory pseudotumors wereall resected. Two cases with MEC received chemotherapy, one after surgery. 2/5 PPB patients survived withoutrecurrence, 3 died; all carcinoid tumors and inflammatory pseudotumors were alive; 1/2 MEC patients wasalive after 252 months, the other one was lost without disease; 1/2 mesothelioma patients was alive withoutdisease, the other was died. For all cases, median follow-up was 30.5 months (0.6-252).
Conclusions: Primarypleuropulmonary tumors are rare but clinical presentation can be varied and delay in diagnosis is common.Children with persistent coughs, recurrent pneumonia or hemoptysis should be considered as indicators forearly diagnosis, very important because the prognosis of these tumors varies with histology and stage.