The Effect of Gross Total Resection on Patients with Pleuropulmonary Blastoma
Pleuropulmonary blastoma (PPB) is the most common primary lung cancer in children. While rare, these tumors are highly aggressive. Tumor recurrence and overall survival are dependent on histologic grade and extent of surgical resection. We sought to examine our institutional experience with PPB to determine the effect of gross total resection (GTR) on recurrence and patient outcomes. (Source: Journal of Surgical Research)
Source: Journal of Surgical Research - April 26, 2020 Category: Surgery Authors: Abigail K. Zamora, Michael J. Zobel, Shadassa Ourshalimian, Shengmei Zhou, Nick M. Shillingford, Eugene S. Kim Tags: Pediatric/Congenital/Developmental Source Type: research
Identification of Homozygous Somatic DICER1 Mutation in Pleuropulmonary Blastoma
We present a 3-year-old boy with type II PPB. Multimodal treatment consisting of surgery and neoadjuvant chemotherapy was effective. DICER1 mutations were examined by Sanger sequencing, microarray comparative genomic hybridization, and microsatellite markers. The results revealed that a somatic biallelic DICER1 mutation with uniparental disomy was present in the tumor tissue. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - April 25, 2020 Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research
Pleuropulmonary Blastoma misinterpreted as spontaneous pneumothorax in an infant
Publication date: Available online 17 April 2020Source: The Annals of Thoracic SurgeryAuthor(s): Till Ploenes, Hans-Ulrich Schildhaus, Dirk Theegarten, Florian Stehling, Uta Dirksen, Dirk Stefani, Clemens Aigner (Source: The Annals of Thoracic Surgery)
Source: The Annals of Thoracic Surgery - April 17, 2020 Category: Cardiovascular & Thoracic Surgery Source Type: research
Pleuropulmonary Blastoma misinterpreted as spontaneous pneumothorax in an infant.
PMID: 32305285 [PubMed - as supplied by publisher] (Source: The Annals of Thoracic Surgery)
Source: The Annals of Thoracic Surgery - April 15, 2020 Category: Cardiovascular & Thoracic Surgery Authors: Ploenes T, Schildhaus HU, Theegarten D, Stehling F, Dirksen U, Stefani D, Aigner C Tags: Ann Thorac Surg Source Type: research
Pleuropulmonary blastoma type I and congenital pulmonary airway malformation type 4: distinct entities or sides of the same coin?
(Source: Virchows Archiv)
Source: Virchows Archiv - April 15, 2020 Category: Pathology Source Type: research
Pleuropulmonary blastoma: a report from the TREP (Tumori Rari in Et à Pediatrica) Project.
CONCLUSIONS: The TREP Registry showed an excellent capacity for registering cases of PPB. Patients received homogeneous treatment after the TREP Project started. Long-term outcomes were excellent for type I and unsatisfactory for type II and III. Tumor invasiveness and IRS stage were of prognostic value.
PMID: 32270754 [PubMed - in process] (Source: Tumori)
Source: Tumori - April 11, 2020 Category: Cancer & Oncology Tags: Tumori Source Type: research
Type II pleuropulmonary blastoma in a 2-year-old girl: a case report.
We present a case of PPB in a 2-year-old girl who was brought to the pediatric clinic for fever, cough and respiratory distress. Imaging studies showed a heterogeneous solid-cystic mass (12∕9∕11 cm) in the upper right pulmonary lobe. Through right thoracotomy, a specimen was obtained, the histopathological and immunohistochemical features of the specimen being suggestive for type II PPB. Aggressive chemotherapy and right pneumonectomy resulted in control of disease, the patient being currently in complete remission four years after the diagnosis.
PMID: 32239109 [PubMed - in process] (Source: Romanian Journal of...
Source: Romanian Journal of Morphology and Embryology - April 5, 2020 Category: General Medicine Tags: Rom J Morphol Embryol Source Type: research
Prominent entrapment of respiratory epithelium in primary and metastatic intrapulmonary non-epithelial neoplasms: a frequent morphological pattern closely mimicking adenofibroma and other biphasic pulmonary lesions
This study highlights frequent respiratory epithelium entrapment in diverse non-epithelial lung tumors, both primary and metastatic. Recognition of this finding and use of adjunct IHC combined with clinical history should help to avoid misinterpretation as primary pulmonary biphasic neoplasm or as harmless adenofibroma. The vast majority of morphologically defined lung adenofibromas represent adenofibroma-like variants of histogenetically diverse entities so that a diagnosis of adenofibroma should be rendered only very rest rictively and then as a diagnosis by exclusion. (Source: Virchows Archiv)
Source: Virchows Archiv - March 18, 2020 Category: Pathology Source Type: research
Pleuropulmonary blastoma type I might arise in congenital pulmonary airway malformation type 4 by acquiring a Dicer 1 mutation
AbstractCongenital pulmonary airway malformation (CPAM) occurs most commonly in infants. It is divided into 5 types. The most common types 1 and 2 are cystic, type 0 presents as bronchial buds without alveolar tissue, most likely corresponding to alveolar dysgenesis, while type 3 is composed of branching bronchioles and appears as a solid lesion. A defect in the epithelial-mesenchymal crosstalk might be the underlying mechanism for all. Type 4 is a peripheral cystic lesion with a thin cyst wall covered by pneumocytes. CPAM 4 has been mixed up with pleuropulmonary blastoma (PPB) type I and some authors question its existenc...
Source: Virchows Archiv - March 18, 2020 Category: Pathology Source Type: research
Gynecologic and reproductive health in patients with pathogenic germline variants in DICER1.
CONCLUSION: In these DICER1-carrier females, DICER1-related gynecological tumors occurred during childhood or adolescence in some after which women generally experienced healthy reproductive lives. Individual education and screening for these tumors is warranted. The high rate of DICER1-related multinodular goiter resulting in pre- and post-pregnancy thyroidectomy underscores the importance of thyroid monitoring during pregnancy to ensure maternal and fetal wellbeing.
PMID: 31952842 [PubMed - as supplied by publisher] (Source: Gynecologic Oncology)
Source: Gynecologic Oncology - January 14, 2020 Category: Cancer & Oncology Authors: Merideth MA, Harney LA, Vyas N, Bachi A, Carr AG, Hill DA, Dehner LP, Schultz KAP, Stewart DR, Stratton P Tags: Gynecol Oncol Source Type: research
Inequalities in diagnosis and registration of pediatric very rare tumors: a European study on pleuropulmonary blastoma
Conclusion: The number of cases observed is consistent with expectations, but disparities exist across Europe. Difficulties in diagnosing PPB emerged in most Eastern countries. The incidence rate of PPB may be underestimated. The creation of VRT-dedicated groups and a European Registry for VRTs could help to reduce inequalities.What is Known:• Very rare pediatric tumors are often not recognized, despite representing almost 11% of childhood cancers .• Pleuropulmonary blastoma is a rare pediatric tumor with a poor prognosis.What is New:• The ability to diagnose and register pleuropulmonary blastoma varies in Europe.Reg...
Source: European Journal of Pediatrics - January 3, 2020 Category: Pediatrics Source Type: research
Pleuropulmonary Blastoma - A retrospective single institute experience of a rare malignancy
Pleuropulmonary blastoma (PPB) is a rare and aggressive neoplasm of childhood arising from the pleuropulmonary mesenchyme. We evaluate the clinic-patho-radiological profile, treatment response and over-all outcomes for patients with PPB treated at the Tata Memorial Hospital (TMH). (Source: International Journal of Radiation Oncology * Biology * Physics)
Source: International Journal of Radiation Oncology * Biology * Physics - October 23, 2019 Category: Radiology Authors: N. Khanna, J. Bhatia, M. Prasad, G. Chinnaswamy, T. Vora, M. Ramadwar, B. Rekhi, S. Qureshi, S. Kembhavi, S. Shah, S. Laskar Source Type: research
Imaging of DICER1 syndrome
AbstractDICER1 syndrome is a highly pleiotropic tumor predisposition syndrome that has been increasingly recognized in the last 10 years. Diseases in the syndrome result from mutations in both copies of the geneDICER1, a highly conserved gene that is critically implicated in micro-ribonucleic acid (miRNA) biogenesis and hence modulation of messenger RNAs. In general, susceptible individuals carry an inherited germline mutation that disables one copy ofDICER1; within tumors, a very characteristic second mutation alters function of the other gene copy. About 20 hamartomatous, hyperplastic or neoplastic conditions comprise DI...
Source: Pediatric Radiology - September 30, 2019 Category: Radiology Source Type: research
Type I pleuropulmonary blastoma presenting as congenital pulmonary airway malformation: A report of two cases.
Abstract
Pleuropulmonary blastoma (PPB) is a rare aggressive intrathoracic tumor which is believed to originate from embryonic uncommitted lung mesenchymal cells, which are important for developing the lung. Type I PPB is cystic, type II is cystic and solid, while type III is predominantly solid. Diagnosing type 1 PPB is a challenge for both radiologists as well as pathologists. Owing to its purely cystic nature, type I PPB it is often mistaken for unrelated entities such as congenital pulmonary airway malformation and congenital lobar emphysema which delays surgical intervention. Here, we report two such ...
Source: Indian Journal of Pathology and Microbiology - September 30, 2019 Category: Pathology Authors: Kumari K, Longchar M, Gunathilaka G, Narange P, Aggarwal S, Arava S Tags: Indian J Pathol Microbiol Source Type: research
Invasive mucinous adenocarcinoma of the lung arising in a type 1 congenital pulmonary airway malformation in a 68-year-old patient: a case report.
Conclusions: Surgical resection should be considered in adults with asymptomatic cysts to prevent malignant transformation. For further analysis, histopathological examination of specimen is essential for a proper diagnosis and eventually further postoperative treatment.
PMID: 31262240 [PubMed - as supplied by publisher] (Source: Acta Chirurgica Belgica)
Source: Acta Chirurgica Belgica - July 4, 2019 Category: Surgery Tags: Acta Chir Belg Source Type: research
