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Pleuropulmonary Blastoma
Published in Dongyou Liu, Handbook of Tumor Syndromes, 2020
Raúl Barrera-Rodríguez, Carlos Pérez-Malagón
Pleuropulmonary blastoma (PPB) is an extremely rare and aggressive mesenchymal neoplasm of the lung which affects children from the newborn period to 12 years of age (mean age of 2–5 years). Most cases are diagnosed before 4 years of age with no predilection for gender [1–3]. It is also known as pulmonary blastoma of childhood or pneumoblastoma.
Bronchogenic cyst
Published in Alisa McQueen, S. Margaret Paik, Pediatric Emergency Medicine: Illustrated Clinical Cases, 2018
Congenital pulmonary malformation and resection. Congenital pulmonary malformations (CPMs) of the lung are present in 1:11,000 to 1:35,000 live births. In the neonate, they have a wide range of presentations from respiratory failure to a well-appearing infant. In children, they usually present with cough but can also present with dyspnea and fever. Hemoptysis is a rarer presentation. About 13% of children will present with a pneumonia or recurrent respiratory infections and 10% present with a pneumothorax. In children, congenital cystic adenomatoid malformations are the most common type of CPMs to convert into cancer with bronchogenic cysts (pictured earlier) as the second most common. In symptomatic patients, resection through lobectomy (versus segmentectomy or removal of cyst only) is the most common treatment. Controversy exists over the treatment of incidental or asymptomatic CPMs. Some surgeons recommend prophylactic surgery to prevent future infections, enhance compensatory lung growth, and to prevent development of malignancy. Most studies cite the lifetime malignant transformation rate of CPMs to be less than 5%. Pleuropulmonary blastoma is the most common cancer associated with CPMs in children and can develop from a cyst at any time. There is no consensus about surveillance of these cysts for malignancy as chest x-rays are not sensitive enough and repeated CT scans increase the risk for iatrogenic cancer. Other cancers that CPMs can evolve into include rhabdomyosarcoma, adenocarcinoma, squamous cell carcinoma, and mesenchymoma.
Congenital malformations of the lung
Published in Prem Puri, Newborn Surgery, 2017
Shannon M. Koehler, Keith T. Oldham
Type 4 CPAMs are also macrocystic and are made up of large cysts of size up to 10 cm.28,29 This subtype represents 10–15% of all CPAMs, and also like Type 1 CPAMs, these patients have a generally good prognosis.28 Unlike Type 1, which are bronchial, these lesions are peripherally oriented, and as a result, patients may present with respiratory distress in the neonatal period or with a pneumothorax in older children.28,29 As with the other forms of CPAMs, the treatment of choice is surgical resection. It is important to examine these lesions closely intraoperatively as they can be confused with pleuropulmonary blastoma, which would have a solid component.28
Pleuropulmonary blastoma: Difficulty in diagnosis and treatment of a case in Vietnam
Published in Pediatric Hematology and Oncology, 2021
Bui Ngoc Lan, Le Thi Kim Ngoc, Hoang Ngoc Thach, Phan Canh Duy
Pleuropulmonary blastoma is a malignant primary intrathoracic tumor rarely seen in children, accounting for under 1% of primary tumors in the lungs in children less than 6 years old.3 Every year, our department has over 300 new cases, but for the last 10 years, we have only registered 5 patients with PPB: 1 patient (Patient 1) is alive, 2 patients (B and C) died due to toxicity, 1 patient (D) died due to progressive disease, and 1 patient (E) refused treatment. We sent the samples of the first case we encountered (patient B) to Washington University in St Louis, Missouri, USA. At that time (June 2011), Dr Louis P. Dehner reviewed pathology and Dr Yoav Messinger, the director of International PPB Registry, consulted to help us treat patient B with PPB type III. Patient B ultimately died due to toxicity. Our institutional experience with PPB includes 4 deaths prior to the successful case reported here.
Pleuropulmonary Blastoma Developing in a Case of Misinterpreted Congenital Pulmonary Airway Malformation: a Case Report
Published in Fetal and Pediatric Pathology, 2018
Moupali Ghosh, Nelofar Islam, Arindam Ghosh, Priyanka Maity Chaudhuri, Koushik Saha, Uttara Chatterjee
Pleuropulmonary blastoma (PPB) is a lung tumor seen mainly in children. It is a dysontogenic tumor of thoracopulmonary mesenchyme [1]. It can sometimes be associated with congenital cystic diseases of lungs and has been reported to develop in a background of congenital pulmonary airway malformation (CPAM) [2–5]. We describe a one and a half year old boy with a large, recurrent right-sided lung mass. Fine needle aspiration cytology (FNAC) from the mass suggested the possibility of PPB which was confirmed on Trucut biopsy. The child had been earlier diagnosed as CPAM for which he had undergone lobectomy at the age of 6 months.