منابع مشابه
Nasal chondromesenchymal hamartoma in an adolescent with pleuropulmonary blastoma.
Nasal chondromesenchymal hamartoma (NCMH) is an extremely rare benign lesion arising in the sino-nasal tract. They usually affect children below one year of age. The pathogenesis of these lesions is poorly understood however, they have been associated with pleuropulmonary blastoma (PPB). Although benign, malignant transformation has been reported in the literature. On literature review, we foun...
متن کاملPleuropulmonary Blastoma in Children: A Case Report
Pleuropulmonary Blastoma [PPB] is a very rare, highly aggressive and malignant tumor that originates from either the lungs or pleura. It occurs mainly in children aged less than five or six years. It has poor prognosis with three different subtypes: cystic [type I], combined cystic and solid [type II] and solid [type III]. PPB is treated with aggressive multimodal therapies including surgery an...
متن کاملBiallelic DICER1 mutations in sporadic pleuropulmonary blastoma.
Pleuropulmonary blastoma (PPB) is a rare pediatric malignancy whose pathogens are poorly understood. Recent reports suggest that germline mutations in the microRNA-processing enzyme DICER1 may contribute to PPB development. To investigate the genetic basis of this cancer, we performed whole-exome sequencing or targeted deep sequencing of multiple cases of PPB. We found biallelic DICER1 mutation...
متن کاملPleuropulmonary blastoma in an adult patient: Report of a case
Pleuropulmonary blastoma (PPB) typically presents in young children, most younger than 5 years, as dysontogenetic neoplasm. It has poor prognosis with three different subtypes: cystic (type I), combined cystic and solid (type II) and solid (type III). Our patient was admitted with dyspnea and was diagnosed at 25 years of age. He had upper right lobectomy and diagnosed as Type II PPB followed by...
متن کاملPleuropulmonary Blastoma in an Infant Presenting with Tension Pneumothorax
Pleuropulmonary blastoma is one of the rarest tumours occurring in childhood. The pathogenesis and therapeutic management of this condition remain controversial. We report the case of an 8-month-old girl with cystic pleuropulmonary blastoma (type 1), presenting with respiratory distress and a tension pneumothorax. The clinical and radiological aspects of the pleuropulmonary blastoma are also di...
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ژورنال
عنوان ژورنال: Journal of Pediatric Surgery Case Reports
سال: 2020
ISSN: 2213-5766
DOI: 10.1016/j.epsc.2020.101482