Diagnosis and treatment of pleuropulmonary blastoma—single center experience

Abstract

Pleuropulmonary blastoma (PPB) is a rare and potentially aggressive intrathoracic disembryonic neoplasm typically occurring in children less than 6 years of age. We assessed the relative incidence, clinical characteristics, treatment outcome, and the prognostic factors for long-term survival in patients with PPB treated at our institution over a 25-year period, and compared these data with reports in the literature. From 1985 to 2010, 11 children (4 males and 7 females), with a median age of 5.4 years (range, 1-12 years) were treated at our hospital. Here we described the main characteristics of these patients, the diagnostic methods, and treatment modalities used. During a median follow-up period of 80, 9 months, the overall survival (OS) and disease-free survival (DFS) rates were 54, 6% and 45, 5%, respectively. Two patients survived for more than 20 years. The main prognostic factors for long-term survival were the diseases type I and II and treatment with radical surgery. Our results show that in order to improve the prognosis of patients with PPB a timely in our opinion and accurate diagnosis needs to be established and treatment should be offered according to the disease type and extend of dissemination.

A pair of DICER1‐positive monozygotic twins: One with pleuropulmonary blastoma, another with acute transient hepatitis

“…Pleuropulmonary blastoma (PPB) is a rare but potentially aggressive tumor that arises from mesenchymal cells of the lung. It accounts for 0.5% of all pediatric malignancies . Between 25 and 50 children are diagnosed with PPB annually in the United States; it shows no predilection for gender .…”

“…Between 25 and 50 children are diagnosed with PPB annually in the United States; it shows no predilection for gender . More than 90% of PPB cases arise in children less than 6 years of age . There are three pathologic types of PPB: type Ⅰ or purely cystic, type Ⅱ or cystic/solid, and type Ⅲ or purely solid.…”

Deformación de la pared torácica como presentación de un blastoma pleuropulmonar, caso clínico

“…La cirugía electiva es segura, previene el desarrollo de síntomas y reduce las complicaciones [13][14] . El BPP usualmente produce síntomas respiratorios, pero puede ser un diagnóstico incidental 15 . Si bien entre el diagnóstico y el tratamiento definitivo el paciente desarrolló dificultad respiratoria manifestada como retracción subcostal, de acuerdo a nuestra búsqueda, este es el primer reporte de un BPP que se presentó inicialmente como deformidad torácica.…”

“…La resección quirúr-gica completa es esencial: otorga la mejor probabilidad de sobrevida a largo plazo. La radioterapia tiene rol en las recidivas o enfermedad residual [15][16] . Las recomendaciones actuales para el tipo I incluyen quimioterapia adyuvante, pero todavía no es claro si previene la progresión 15 .…”

“…La radioterapia tiene rol en las recidivas o enfermedad residual [15][16] . Las recomendaciones actuales para el tipo I incluyen quimioterapia adyuvante, pero todavía no es claro si previene la progresión 15 . El seguimiento con TC de tórax es requerido hasta los 4-5 años de edad.…”