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pleuropulmonary blastoma

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https://www.readbyqxmd.com/read/29081372/multiphoton-microscopy-a-novel-diagnostic-method-for-solid-tumors-in-a-prospective-pediatric-oncologic-cohort-an-experimental-study
#1
Oliver J Muensterer, Sibylle Waldron, Yoon Jung Boo, Claudius Ries, Lisa Sehls, Frank Simon, Larissa Seidmann, Jérôme Birkenstock, Jan Gödeke
BACKGROUND: The prognosis of solid pediatric tumors strongly correlates with accurate staging and complete local control. Currently, surgeons rely on macroscopic cues and intraoperative cryosection to determine resection borders. Multiphoton Microscopy (MPM) is a real time technique that allows imaging of tissue without time-consuming tissue processing. PURPOSE: This pilot study evaluates the diagnostic potential of MPM in pediatric solid tumors compared to routine histopathology...
October 30, 2017: International Journal of Surgery
https://www.readbyqxmd.com/read/29046943/congenital-pulmonary-airway-malformations-state-of-the-art-review-for-pediatrician-s-use
#2
REVIEW
Claire Leblanc, Marguerite Baron, Emilie Desselas, Minh Hanh Phan, Alexis Rybak, Guillaume Thouvenin, Clara Lauby, Sabine Irtan
Congenital pulmonary airway malformations or CPAM are rare developmental lung malformations, leading to cystic and/or adenomatous pulmonary areas. Nowadays, CPAM are diagnosed prenatally, improving the prenatal and immediate postnatal care and ultimately the knowledge on CPAM pathophysiology. CPAM natural evolution can lead to infections or malignancies, whose exact prevalence is still difficult to assess. The aim of this "state-of-the-art" review is to cover the recently published literature on CPAM management whether the pulmonary lesion was detected during pregnancy or after birth, the current indications of surgery or surveillance and finally its potential evolution to pleuro-pulmonary blastoma...
December 2017: European Journal of Pediatrics
https://www.readbyqxmd.com/read/29037807/dicer1-related-sertoli-leydig-cell-tumor-and-gynandroblastoma-clinical-and-genetic-findings-from-the-international-ovarian-and-testicular-stromal-tumor-registry
#3
Kris Ann P Schultz, Anne K Harris, Michael Finch, Louis P Dehner, Jubilee B Brown, David M Gershenson, Robert H Young, Amanda Field, Weiying Yu, Joyce Turner, Nicholas G Cost, Dominik T Schneider, Douglas R Stewart, A Lindsay Frazier, Yoav Messinger, D Ashley Hill
BACKGROUND: Ovarian sex cord-stromal tumors (OSCST) include juvenile granulosa cell tumors (JGCT), Sertoli-Leydig cell tumor (SLCT) and gynandroblastoma (GAB) among others. These ovarian sex cord-stromal tumors as well as other tumors including pleuropulmonary blastoma (PPB) may be associated with DICER1 mutations. We sought to describe the clinical and genetic findings from the first 107 individuals enrolled in the International Ovarian and Testicular Stromal Tumor Registry. METHODS: Medical and family history were obtained for individuals consecutively enrolled in the International Ovarian and Testicular Stromal Tumor Registry...
October 13, 2017: Gynecologic Oncology
https://www.readbyqxmd.com/read/28991133/pleuropulmonary-blastoma-a-single-center-case-series-of-6-patients
#4
Janie M Pierce, Priscilla LaCroix, Kenneth Heym, William P Bowman, Linda Margraf, Jose Iglesias, Anish Ray
Pleuropulmonary blastoma (PPB) is a rare malignancy of childhood which when left untreated often shows pathologic progression resulting in a more aggressive neoplasm with an increasingly poor prognosis. Because of this it is important to diagnose and initiate treatment early. However, early stage PPB can appear as a cystic lung lesion on imaging and can be easily misdiagnosed given the rarity of the malignancy. Moreover, current therapeutic guidelines for these lesions are not well established, making treatment decisions and management difficult for clinicians...
November 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28960912/dicer1-syndrome-approach-to-testing-and-management-at-a-large-pediatric-tertiary-care-center
#5
Kalene van Engelen, Anita Villani, Jonathan D Wasserman, Laura Aronoff, Mary-Louise C Greer, Marta Tijerin Bueno, Bailey Gallinger, Raymond H Kim, Ronald Grant, M Stephen Meyn, David Malkin, Harriet Druker
BACKGROUND: To expand the current knowledge of DICER1 syndrome and to propose criteria for genetic testing based on experience at a pediatric tertiary care center. PROCEDURE: This study involved a retrospective chart review of the 78 patients (47 probands and 31 family members) seen in the Cancer Genetics Program at The Hospital for Sick Children (SickKids) who were offered genetic testing for DICER1. RESULTS: Of 47 probands offered genetic testing for DICER1, 46 pursued testing: 11 (23...
January 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28898554/pulmonary-blastoma-in-a-child-report-of-a-case-with-cytological-findings
#6
Mou Das, Priyanka Maity Chaudhuri, Anis Bandyopadhyay, Uttara Chatterjee
Pulmonary blastoma is an uncommon tumour. It constitutes <0.1% of all resected lung cancers. It is even more rare in children with only a few case reports describing this entity in them. Pulmonary blastoma should not be confused with pleuropulmonary blastoma which is a paediatric lung tumour with different morphology and better outcome. Here we take the opportunity of describing pulmonary blastoma in a 3 years old child along with its cytological findings.
September 12, 2017: Diagnostic Cytopathology
https://www.readbyqxmd.com/read/28884206/neonatal-congenital-lung-tumors-the-importance-of-mid-second-trimester-ultrasound-as-a-diagnostic-clue
#7
Stephan L Waelti, Laurent Garel, Dorothée Dal Soglio, Françoise Rypens, Michael Messerli, Josée Dubois
BACKGROUND: The differential diagnosis for primary lung masses in neonates includes a variety of developmental abnormalities; it also consists of the much rarer congenital primary lung tumors: cystic pleuropulmonary blastoma (cystic PPB), fetal lung interstitial tumor (FLIT), congenital peribronchial myofibroblastic tumor (CPMT), and congenital fibrosarcoma. Radiologic differentiation between malformations and tumors is often very challenging. OBJECTIVE: The objective was to establish distinctive features between developmental pulmonary abnormalities and primary lung tumors...
December 2017: Pediatric Radiology
https://www.readbyqxmd.com/read/28871588/primary-pleuropulmonary-synovial-sarcoma-with-brain-metastases-in-a-paediatric-patient-an-unusual-presentation
#8
Pushpak Chandrakant Chirmade, Sonia Parikh, Asha Anand, Harsha Panchal, Apurva Patel, Sandip Shah
Primary lung neoplasms are rare in children. The most common primary lung malignancies in children are pleuropulmonary blastoma and carcinoid tumour. Synovial sarcoma (SS) accounts for approximately 1% of all childhood malignancies. In absolute terms, the SS of the lungs and pleura are extremely rare and pose a diagnostic difficulty. Soft tissue sarcomas usually have a high potential for metastases, however, metastasis to the brain is rare, even in widely disseminated disease, and it has been described only in 3 case reports previously...
2017: Advances in Respiratory Medicine
https://www.readbyqxmd.com/read/28748527/the-prevalence-of-dicer1-pathogenic-variation-in-population-databases
#9
Jung Kim, Amanda Field, Kris Ann P Schultz, D Ashley Hill, Douglas R Stewart
The DICER1 syndrome is associated with a variety of rare benign and malignant tumors, including pleuropulmonary blastoma (PPB), cystic nephroma (CN) and Sertoli-Leydig cell tumor (SLCT). The prevalence and penetrance of pathogenic DICER1 variation in the general population is unknown. We examined three publicly-available germline whole exome sequence datasets: Exome Aggregation Consortium (ExAC), 1,000 Genomes (1,000 G) and the Exome Sequencing Project (ESP). To avoid over-estimation of pathogenic DICER1 variation from cancer-associated exomes, we excluded The Cancer Genome Atlas (TCGA) variants from ExAC...
November 15, 2017: International Journal of Cancer. Journal International du Cancer
https://www.readbyqxmd.com/read/28728220/-diagnostic-utility-of-tyrosine-hydroxylase-in-peripheral-neuroblastic-tumors
#10
N Zhang, N Liu, L J He
Objective: To investigation the diagnostic utility of tyrosine hydroxylase (TH) immunohistochemically as a marker of peripheral neuroblastic tumors(pNT). Methods: The study included 1 024 cases, 643 primary and metastatic pNT cases, 381 non-pNT cases, including small round cell tumors such as primitive neuroectodermal tumor (PNET), rhabdomyosarcoma, lymphoma, nephroblastoma, as well as other more common tumors (medulloblastoma, hepatoblastoma, pleuropulmonary blastoma, renal clear cell sarcoma, Langerhans cell histiocytosis, lipoblatoma etc)...
July 8, 2017: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
https://www.readbyqxmd.com/read/28688118/a-pair-of-dicer1-positive-monozygotic-twins-one-with-pleuropulmonary-blastoma-another-with-acute-transient-hepatitis
#11
Sihui Li, Siyu Cai, Xisi Wang, Dawei Zhang, Libing Fu, Qi Zeng, Xiaoxia Peng, Xiaoli Ma
Pleuropulmonary blastoma (PPB) is the most common primary malignant neoplasm of the lung in children that is associated with a germline mutation in DICER1. In this report, we share an interesting case of a pair of monozygotic twins: one of them developed PPB when she was 4-year old, while the other developed acute transient hepatitis when she was 5-year old. Next-Gen sequencing for DICER1 mutations of their family revealed that both twins and their mother had c.C3675A mutation. The mother also had a history of multinodular goiter...
December 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28624956/dicer1-mutations-in-twelve-chinese-patients-with-pleuropulmonary-blastoma
#12
Siyu Cai, Xisi Wang, Wen Zhao, Libing Fu, Xiaoli Ma, Xiaoxia Peng
Our aim is to examine the impact of DICER1 mutations on the pathogenesis of pleuropulmonary blastoma (PPB) by evaluating the mutation frequency and investigating the family history of Chinese patients with PPB. The family histories of 12 children with PPB recruited consecutively were surveyed. Blood samples from patients and their first-degree relatives were tested for DICER1 mutations. Whole-genome sequencing of blood samples and formalin-fixed and paraffin-embedded (FFPE) tumor tissue was performed in one family with twins...
June 14, 2017: Science China. Life Sciences
https://www.readbyqxmd.com/read/28620008/pten-dicer1-fh-and-their-associated-tumor-susceptibility-syndromes-clinical-features-genetics-and-surveillance-recommendations-in-childhood
#13
REVIEW
Kris Ann P Schultz, Surya P Rednam, Junne Kamihara, Leslie Doros, Maria Isabel Achatz, Jonathan D Wasserman, Lisa R Diller, Laurence Brugières, Harriet Druker, Katherine A Schneider, Rose B McGee, William D Foulkes
PTEN hamartoma tumor syndrome (PHTS), DICER1 syndrome, and hereditary leiomyomatosis and renal cell cancer (HLRCC) syndrome are pleiotropic tumor predisposition syndromes that include benign and malignant neoplasms affecting adults and children. PHTS includes several disorders with shared and distinct clinical features. These are associated with elevated lifetime risk of breast, thyroid, endometrial, colorectal, and renal cancers as well as melanoma. Thyroid cancer represents the predominant cancer risk under age 20 years...
June 15, 2017: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
https://www.readbyqxmd.com/read/28474256/pediatric-imaging-in-dicer1-syndrome
#14
Marta Tijerin Bueno, Claudia Martínez-Ríos, Alejandro De la Puente Gregorio, Rayan A Ahyad, Anita Villani, Harriet Druker, Kalene van Engelen, Bailey Gallinger, Laura Aronoff, Ronald Grant, David Malkin, Mary-Louise C Greer
BACKGROUND: DICER1 syndrome, arising from a mutation in the DICER1 gene mapped to chromosome 14q32, is associated with an increased risk of a range of benign and malignant neoplasms. OBJECTIVE: To determine the spectrum of abnormalities and imaging characteristics in patients with DICER1 syndrome at a tertiary pediatric hospital. MATERIALS AND METHODS: This retrospective analysis evaluated imaging in patients ≤18 years with DICER1 germline variants between January 2004 and July 2016...
May 4, 2017: Pediatric Radiology
https://www.readbyqxmd.com/read/28469342/a-case-report-of-pleuropulmonary-blastoma-presenting-as-tension-pneumothorax
#15
Archana Addanki, Krishna Chaitanya, Sandip Bartakke, Srividya Sethuratnam
Pleuropulmonary blastoma (PPB) is a very rare, highly aggressive, and malignant tumor that originates from either lungs or pleura. It occurs mainly in children aged <5 or 6 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 and chemotherapy. We present a case of PPB in a 3-year-old girl who presented with tension pneumothorax.
January 2017: Indian Journal of Medical and Paediatric Oncology
https://www.readbyqxmd.com/read/28441828/-clinical-analysis-of-seven-cases-of-pulmonary-malignant-tumors-in-children
#16
D Xu, Z M Chen, W Z Gu, Y S Wang, M X Huang, L F Tang, Y Y Zhang, Y Jiang
Objective: To understand the clinical manifestation, imaging characteristics and outcomes of pulmonary malignant tumors in children. Method: We retrospectively collected information about seven cases of pulmonary malignant tumors in children in our hospital from Jan 2010 to Dec 2016. The information included clinical manifestation, imaging characteristics, pathologic results, and treatment. Result: (1) All the seven patients firstly visited pediatric internal medicine departments. Symptoms included cough (n=6), dyspnea (n=4), fever (n=2), anorexia (n=2), chest tightness (n=1), chest pain (n=1), lameness (n=1), abdominal distension and constipation (n=1)...
April 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28405541/it-s-rare-so-be-aware-pleuropulmonary-blastoma-mimicking-congenital-pulmonary-airway-malformation
#17
Fayza Haider, Khulood Al Saad, Fatima Al-Hashimi, Hakima Al-Hashimi
Pleuropulmonary blastoma (PPB) is a rare aggressive malignant tumor of infancy and early childhood. The tumor arises in the lung and pleura and is regarded as a pulmonary dysontogenetic or embryonic neoplasm. Four types are defined in literature. Type I PPB is a rare, cystic lung neoplasm in infants characterized by subtle malignant changes and a good prognosis. Recurrences after type I PPB are usually advanced with a poor prognosis. We report this case to increase awareness about this entity so that the pediatricians, pediatric surgeons, radiologist, and pathologist recognize it early...
January 2017: Thoracic and Cardiovascular Surgeon Reports
https://www.readbyqxmd.com/read/28382840/strong-expression-and-amplification-of-igf1r-in-pleuropulmonary-blastomas
#18
Christian Vokuhl, Laura de Leon-Escapini, Ivo Leuschner
Pleuropulmonary blastoma (PPB) is a rare malignant intrathoracic tumor primarily affecting children under 5 years of age. PPBs are histologically divided into 3 subtypes: Type I PPBs are composed of multiple cysts, and type III is a solid lesion with a variable morphologic appearance. Type II has a mixed morphology consisting of cystic and solid areas. The genetics of PPB are poorly understood. We analyzed 16 cases of the Kiel Paediatric Tumor Registry with the diagnosis of PPB by comparative genomic hybridization and confirmed some genetic changes by fluorescence in situ hybridization...
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28222777/identification-of-somatic-and-germ-line-dicer1-mutations-in-pleuropulmonary-blastoma-cystic-nephroma-and-rhabdomyosarcoma-tumors-within-a-dicer1-syndrome-pedigree
#19
Lorena Fernández-Martínez, José Antonio Villegas, Íñigo Santamaría, Ana S Pitiot, Marta G Alvarado, Soledad Fernández, Héctor Torres, Ángeles Paredes, Pilar Blay, Milagros Balbín
BACKGROUND: DICER1 syndrome is a pediatric cancer predisposition condition causing a variety of tumor types in children and young adults. In this report we studied a family with two relatives presenting a variety of neoplastic conditions at childhood. METHODS: Germ-line mutation screening of the complete coding region of the DICER1 gene in genomic DNA from the proband was performed. The presence of somatic DICER1 mutation and further alterations in driver genes was investigated in genomic DNA obtained from available tumor samples...
February 21, 2017: BMC Cancer
https://www.readbyqxmd.com/read/28103635/the-impact-of-local-control-in-the-treatment-of-type-ii-iii-pleuropulmonary-blastoma-experience-of-the-cooperative-weichteilsarkom-studiengruppe-cws
#20
Monika Sparber-Sauer, Guido Seitz, Sylvia Kirsch, Christian Vokuhl, Ivo Leuschner, Tobias M Dantonello, Monika Scheer, Thekla von Kalle, Gustaf Ljungman, Stefan S Bielack, Thomas Klingebiel, Joerg Fuchs, Ewa Koscielniak
BACKGROUND AND OBJECTIVES: This study aims at examining the potential survival benefits of primary versus secondary surgery in the management of children diagnosed with pleuropulmonary blastoma (PPB) type II/III. PATIENTS AND METHODS: Disease characteristics, treatment, and survival of 29 children with localized PPB type II/III, treated in six prospective Cooperative Weichteilsarkom Studiengruppe (CWS) trials, were reviewed retrospectively. RESULTS: Five year event free survival (EFS) and overall survival (OS) of children treated according to CWS protocols was 72%...
February 2017: Journal of Surgical Oncology
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