Diagnosis and Treatment of Pleuropulmonary Blastoma in Children: A Single-Center Report of 41 Cases
This study was performed to investigate the age at onset, clinical manifestations, pathological types and features, treatment, and prognosis of pleuropulmonary blastoma (PPB) in children in an attempt to reduce the misdiagnosis rate and achieve early detection and timely intervention. (Source: Journal of Pediatric Surgery)
Source: Journal of Pediatric Surgery - July 1, 2019 Category: Surgery Authors: Na Zhang, Qi Zeng, Xiaoli Ma, Chenghao Chen, Jie Yu, Xu Zhang, Dong Yan, Changqi Xu, Dingyi Liu, Qian Zhang Source Type: research
Extraluminal Use of a Pediatric Bronchial Blocker With Confirmation by Point-of-Care Ultrasound: A Case Report
Lung isolation for pediatric thoracic surgery is especially challenging in a patient with chronic lung infection and need to protect the nonoperative lobes from the spread of infection during anesthesia and surgery. Typically, for pediatric thoracic surgery, a mainstem intubation or placement of an intraluminal bronchial blocker is sufficient for lung isolation. The patient whose case is reported here suffered from a pleuropulmonary blastoma compressing the left lower lobe bronchus and resultant chronic infection involving the left lower lobe. In this unusual situation, to isolate the chronic lung infection and to provide ...
Source: A&A Case Reports - July 1, 2019 Category: Anesthesiology Tags: Case Reports Source Type: research
Pleuropulmonary blastoma manifesting as spontaneous pneumothorax: an unusual presentation
Abstract Coronary arteries originating from the contralateral (noncoronary) sinus and having an interarterial course, in which they run from the ascending aorta to the pulmonary trunk, is a potentially fatal anomaly. Computed tomography (CT) angiography facilitates the recognition and therapeutic planning of such anomalies because of its ability to acquire high-resolution images of the entire course of the coronary artery, as well as of the accompanying atherosclerotic involvement. The right coronary artery originating from the left coronary sinus is the most prevalent anomaly of this type and usually implies a better prog...
Source: Radiologia Brasileira - June 13, 2019 Category: Radiology Source Type: research
Intraocular medulloepithelioma & #8211; A review of clinical features, DICER 1 mutation, and management
Sameeksha H Tadepalli, Carol L Shields, Jerry A Shields, Santosh G HonavarIndian Journal of Ophthalmology 2019 67(6):755-762
Intraocular medulloepithelioma is a nonhereditary neoplasm of childhood arising from primitive medullary epithelium. It most often involves the ciliary body. Most patients present between 2 and 10 years of age with loss of vision, pain, leucocoria, or conjunctival congestion. The mass appears as a grey-white ciliary body lesion with intratumoral cysts. Presence of a neoplastic cyclitic membrane with extension to retrolental region is characteristic. Secondary manifestations like cataract and neovasc...
Source: Indian Journal of Ophthalmology - May 23, 2019 Category: Opthalmology Authors: Sameeksha H Tadepalli Carol L Shields Jerry A Shields Santosh G Honavar Source Type: research
Type I regressed pleuropulmonary blastoma in a 10-year-old boy
AbstractPleuropulmonary blastoma (PPB) is a rare, malignant tumor of the lung and is the most common primary pulmonary malignancy in children. Here, we report a case of a boy who was diagnosed with type I regressed PPB after being mislabeled with congenital pulmonary malformation. A 10-year-old boy presented to our hospital with a history of worsening dyspnea. Since birth, his clinical status and radiographic images were concerning for congenital lobar emphysema that was managed conservatively. A chest computed tomography (CT) scan confirmed the persistence of a large cystic lesion and a diagnostic and therapeutic cystecto...
Source: Indian Journal of Thoracic and Cardiovascular Surgery - May 7, 2019 Category: Cardiovascular & Thoracic Surgery Source Type: research
An update on the central nervous system manifestations of DICER1 syndrome
AbstractDICER1 syndrome is a rare tumor predisposition syndrome with manifestations that predominantly affect children and young adults. The syndrome is typically caused by heterozygous germline loss-of-functionDICER1 alterations accompanied on the other allele by somatic missense mutations occurring at one of a few mutation hotspots within the sequence encoding the RNase IIIb domain.DICER1 encodes a member of the microRNA biogenesis machinery. The syndrome spectrum is highly pleiotropic and features a unique constellation of benign and malignant neoplastic and dysplastic lesions. Pleuropulmonary blastoma (PPB), the most...
Source: Acta Neuropathologica - April 4, 2019 Category: Neurology Source Type: research
Extrapleural pneumonectomy for advanced pleuropulmonary blastoma
ConclusionExtrapleural pneumonectomy is feasible in some circumstances to achieve oncologic control in cases of advanced PPB. This should be kept as part of the surgeons' armamentarium. (Source: Journal of Pediatric Surgery Case Reports)
Source: Journal of Pediatric Surgery Case Reports - February 13, 2019 Category: Surgery Source Type: research
The prevalence of germline DICER1 pathogenic variation in cancer populations
ConclusionThis is the largest comprehensive analysis ofDICER1 pathogenic variation in adult and pediatric cancer populations using publicly available data. The observation of germlineDICER1 variation with uterine corpus endometrial carcinoma merits additional investigation. (Source: Molecular Genetics & Genomic Medicine)
Source: Molecular Genetics & Genomic Medicine - January 22, 2019 Category: Genetics & Stem Cells Authors: Jung Kim,
Kris Ann P. Schultz,
Dana Ashley Hill,
Douglas R. Stewart Tags: ORIGINAL ARTICLE Source Type: research
Placental Mesenchymal Dysplasia: an underdiagnosed placental pathology with various clinical outcomes
CONCLUSIONSPMD was substantially under-diagnosed before delivery in this cohort. Sonographers, fetal medicine specialists, obstetricians and pathologists should all suspect PMD in cases of an enlarged placenta and should look for fetal abnormalities. Diagnostic genetic testing should be discussed to exclude partial molar pregnancy. Close pregnancy follow-up is indicated due to the high risk of associated fetal or maternal adverse outcomes. (Source: European Journal of Obstetrics and Gynecology and Reproductive Biology)
Source: European Journal of Obstetrics and Gynecology and Reproductive Biology - January 18, 2019 Category: OBGYN Source Type: research
Congenital Cystic Lung Lesions: Redefining the Natural Distribution of Subtypes and Assessing the Risk of Malignancy
Asymptomatic cystic lung lesions—congenital pulmonary airway malformations (CPAMs), sequestrations, and bronchogenic cysts—are commonly diagnosed prenatally. Indications to resect are to eliminate risk of malignancy or infection. CPAMs consist of a spectrum of malformations, with type 1 historically considered the most common. Mucinous cell clusters, seen almost exclusively in type 1, are premalignant lesions at risk for progression to mucinous adenocarcinoma. We reviewed and classified 2.5 years of consecutive, prenatally diagnosed lesions as extralobar sequestration, intralobar sequestration, type 1 CPAM, type 2 CPAM...
Source: The American Journal of Surgical Pathology - December 13, 2018 Category: Pathology Tags: Original Articles Source Type: research
Pleuropulmonary bastoma in children: an unusual presentation mimicking pneumonia
We report the case of a 3 year-old boy who presented cough and intermittent fever for one month. After multiple antibiotics courses, he presented a mild hemoptysis and dyspnea with anemia and biological inflammatory syndrome but no change in general condition. Chest X-Ray showed a left parenchymal opacity extending to the entire left lung with mediastinal deviation to the right. Ultrasonography didn’t show pleural effusion. We investigated the nature of the lesion.Methods: CT scan showed a voluminous heterogeneous intrathoracic mass with complete left lower lobe retraction, along with two right pulmonary nodules. A t...
Source: European Respiratory Journal - November 19, 2018 Category: Respiratory Medicine Authors: Berdah, L., Boudjemaa, S., Boutroux, H., Piloquet, J.-E., Coulomb, A., Ducou Le Pointe, H., Clement, A. Tags: Paediatric bronchology Source Type: research
Pleuropulmonary Blastoma Developing in a Case of Misinterpreted Congenital Pulmonary Airway Malformation: a Case Report.
CONCLUSION: We describe the cytological and histological findings of a case of PPBII/III evolving from a PPB I originally thought to be a CPAM type IV. This supports the theory that PPB I may progress to a more aggressive type II with time, and highlights the importance of the adequately treating the PPB I to prevent this transformation.
PMID: 30358469 [PubMed - as supplied by publisher] (Source: Fetal and Pediatric Pathology)
Source: Fetal and Pediatric Pathology - October 27, 2018 Category: Pathology Tags: Fetal Pediatr Pathol Source Type: research
Wilms tumor, pleuropulmonary blastoma, and DICER1: case report and literature review
Pleuroblastoma (PPB) is a rare pediatric tumor which, in 30% of cases, is associated with cystic nephroma. It has been recently linked to the DICER1 mutation as part of a predisposition syndrome for various tu... (Source: World Journal of Surgical Oncology)
Source: World Journal of Surgical Oncology - August 10, 2018 Category: Cancer & Oncology Authors: Olivier Abbo, Kalitha Pinnagoda, Laurent Brouchet, Bertrand Leobon, Fr édérique Savagner, Isabelle Oliver, Philippe Galinier, Marie-Pierre Castex and Marlène Pasquet Tags: Case report Source Type: research
Revisiting pleuropulmonary blastoma and atypical choroid plexus papilloma in a young child: DICER1 syndrome or not?
Pediatric Blood&Cancer, EarlyView. (Source: Pediatric Blood and Cancer)
Source: Pediatric Blood and Cancer - June 26, 2018 Category: Cancer & Oncology Authors: Anne ‐SophieChong
,
SomayyehFahiminiya
,
DouglasStrother
,
JohnPriest
,
SteffenAlbrecht
,
BarbaraRivera
,
William D.Foulkes Source Type: research
Revisiting pleuropulmonary blastoma and atypical choroid plexus in a young child: DICER1 syndrome or not?
Pediatric Blood&Cancer, EarlyView. (Source: Pediatric Blood and Cancer)
Source: Pediatric Blood and Cancer - June 26, 2018 Category: Cancer & Oncology Authors: Anne ‐SophieChong
,
SomayyehFahiminiya
,
DouglasStrother
,
JohnPriest
,
SteffenAlbrecht
,
BarbaraRivera
,
William D.Foulkes Source Type: research
