Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society最新文献

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Extrahepatic Nonreticuloendothelial Siderosis Is Not Specific to Gestational Alloimmune Liver Disease. 肝外非网状内皮性铁沉着不是妊娠期同种免疫肝病所特有的。
IF 1.9
Hao Wu, William Ferguson, Eumenia Castro, Debra Kearney, Milton Finegold, Kalyani Patel
{"title":"Extrahepatic Nonreticuloendothelial Siderosis Is Not Specific to Gestational Alloimmune Liver Disease.","authors":"Hao Wu,&nbsp;William Ferguson,&nbsp;Eumenia Castro,&nbsp;Debra Kearney,&nbsp;Milton Finegold,&nbsp;Kalyani Patel","doi":"10.1177/1093526619826429","DOIUrl":"https://doi.org/10.1177/1093526619826429","url":null,"abstract":"<p><p>Autopsy reports of 78 stillbirths and early infant deaths (up to age 8 weeks) were reviewed to investigate the prevalence of extrahepatic nonreticuloendothelial siderosis (EHNRS) in the context of neonatal liver failure. Of these, 10 liveborns (12.8%), M:F 3:2, with mean gestational age 37.6 weeks (range: 35-39) and mean age at the time of demise 19.1 days (range: 7-42), showed significant liver injury: infection (n = 7, viral > fungal), congenital malformations (n = 2), and ischemia (n = 1). None had maternal history of gestational alloimmune liver disease (GALD) or previous fetal/neonatal death due to liver failure. Seven of 10 cases (70%) showed EHNRS: pancreas (n = 6), kidneys (n = 4), thyroid and adrenal glands (n = 3), and bronchial glands and heart (n = 2). Iron deposition was most frequent in the pancreas (60%), most diffuse in the kidneys, and seen in at least 2 organs, with pancreas and kidney being the most frequent combination. Hepatic C5b-9 expression was variable (1+ to 4+) except 1 case (100% necrosis). The duration of illness and the mean age at the time of demise tended to be higher in those with EHNRS. In summary, hepatic and EHNRS, with or without C5b-9 expression, are not specific for GALD. Other causes of liver failure should be investigated as clinically and pathologically appropriate.</p>","PeriodicalId":520743,"journal":{"name":"Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society","volume":" ","pages":"356-364"},"PeriodicalIF":1.9,"publicationDate":"2019-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1177/1093526619826429","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"36921777","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 7
Perspectives in Pediatric Pathology, Chapter 10. Ectopic and Heterotopic Tissues in the Testis. 儿科病理学展望,第10章。睾丸中的异位和异位组织。
IF 1.9
Manuel Nistal, Ricardo Paniagua, Pilar González-Peramato, Miguel Reyes-Múgica
{"title":"Perspectives in Pediatric Pathology, Chapter 10. Ectopic and Heterotopic Tissues in the Testis.","authors":"Manuel Nistal,&nbsp;Ricardo Paniagua,&nbsp;Pilar González-Peramato,&nbsp;Miguel Reyes-Múgica","doi":"10.2350/14-04-1469-PB.1","DOIUrl":"https://doi.org/10.2350/14-04-1469-PB.1","url":null,"abstract":"10. Ectopic and Heterotopic Tissues in the Testis MANUEL NISTAL, RICARDO PANIAGUA, PILAR GONZÁLEZ-PERAMATO, AND MIGUEL REYES-MÚGICA* Pathology, Hospital La Paz, Universidad Autónoma de Madrid, Calle Arzobispo Morcillo #2, Madrid 28029, Spain Department of Cell Biology, Universidad de Alcalá, Madrid, Spain Department of Pathology, Children’s Hospital of Pittsburgh of UPMC, One Children’s Hospital Drive, 4401 Penn Avenue, Pittsburgh, PA 15224, USA","PeriodicalId":520743,"journal":{"name":"Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society","volume":" ","pages":"446-57"},"PeriodicalIF":1.9,"publicationDate":"2015-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2350/14-04-1469-PB.1","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"32569462","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 1
Perspectives in Pediatric Pathology, Chapter 9. Alterations in the Number and Location of the Testis. 儿科病理学透视,第9章。睾丸数量和位置的改变。
IF 1.9
Manuel Nistal, Ricardo Paniagua, Pilar González-Peramato, Miguel Reyes-Múgica
{"title":"Perspectives in Pediatric Pathology, Chapter 9. Alterations in the Number and Location of the Testis.","authors":"Manuel Nistal,&nbsp;Ricardo Paniagua,&nbsp;Pilar González-Peramato,&nbsp;Miguel Reyes-Múgica","doi":"10.2350/14-04-1468-PB.1","DOIUrl":"https://doi.org/10.2350/14-04-1468-PB.1","url":null,"abstract":"Perspectives in Pediatric Pathology, Chapter 9. Alterations in the Number and Location of the Testis MANUEL NISTAL, RICARDO PANIAGUA, PILAR GONZÁLEZ-PERAMATO, AND MIGUEL REYES-MÚGICA Department of Pathology, Hospital La Paz, Universidad Autónoma de Madrid, Calle Arzobispo Morcillo No. 2, Madrid 28029, Spain Department of Cell Biology, Universidad de Alcala, Madrid, Spain Department of Pathology, Children’s Hospital of Pittsburgh, University of Pittsburgh Medical Center, One Children’s Hospital Drive, 4401 Penn Avenue, Pittsburgh, PA 15224, USA","PeriodicalId":520743,"journal":{"name":"Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society","volume":" ","pages":"433-45"},"PeriodicalIF":1.9,"publicationDate":"2015-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2350/14-04-1468-PB.1","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"32570013","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 1
Pediatric Pathology In The Year 2050. 2050年的儿科病理学。
IF 1.9
Don B Singer
{"title":"Pediatric Pathology In The Year 2050.","authors":"Don B Singer","doi":"10.2350/15-06-1664-OA.1","DOIUrl":"https://doi.org/10.2350/15-06-1664-OA.1","url":null,"abstract":"<p><p>The study of pathology in fetuses, infants, and children had its beginnings in the mid-19th century. Now, 165 years later, hundreds of pediatric pathologists are in up-to-date practices throughout the world. They, and all medical practitioners, are just beginning to delve into the nanotechnical wave. Nanotechnology refers to the structure and activity of minute particles, molecules, compounds, and atoms. By 2050, as nanotechnical studies develop further, new diseases and variations of old diseases will be discovered. Aggregation of medical data from billions of people, a process known as crowd sourcing, will be digitally interconnected to the new findings with computers. Pediatric pathologists will contribute to this expanding science with new laboratory instruments, including ultramodern microscopes known as Omniscopes. Robots will be programmed to perform autopsies and process surgical specimens. Analyzers in chemistry, microbiology, hematology, and genetics will, in 2050, produce dozens or even hundreds of results within minutes. These advances will lead to better treatments and overall better health for everyone. </p>","PeriodicalId":520743,"journal":{"name":"Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society","volume":" ","pages":"512-8"},"PeriodicalIF":1.9,"publicationDate":"2015-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2350/15-06-1664-OA.1","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"34041439","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Nephroblastomas show low expression of microR-204 and high expression of its target, the oncogenic transcription factor MEIS1. 肾母细胞瘤低表达微or -204,高表达其靶基因致癌转录因子MEIS1。
IF 1.9
Karin Koller, Martin Pichler, Karin Koch, Martina Zandl, Verena Stiegelbauer, Ivo Leuschner, Gerald Hoefler, Barbara Guertl
{"title":"Nephroblastomas show low expression of microR-204 and high expression of its target, the oncogenic transcription factor MEIS1.","authors":"Karin Koller,&nbsp;Martin Pichler,&nbsp;Karin Koch,&nbsp;Martina Zandl,&nbsp;Verena Stiegelbauer,&nbsp;Ivo Leuschner,&nbsp;Gerald Hoefler,&nbsp;Barbara Guertl","doi":"10.2350/13-01-1288-OA.1","DOIUrl":"https://doi.org/10.2350/13-01-1288-OA.1","url":null,"abstract":"<p><p>By comparing several studies we identified a possible deregulation of the transcription factors PBX2 (pre-B-cell leukemia homeobox 2) and one of its binding partners, MEIS1 (Meis homeobox 1) in nephroblastomas. The regulation of MEIS1 is complex, and its expression is known to be influenced by changes of promoter methylation and binding of microRNA-204 (miR-204). Therefore, in our study, we assessed the expression of MEIS1 and PBX2 and the factors regulating expression of MEIS1 in nephroblastomas. MEIS1 and PBX2 messenger RNA (mRNA) and protein levels were investigated by quantitative real-time-polymerase chain reaction (qRT-PCR) and immunohistochemistry. Promoter methylation of MEIS1 was evaluated using a methylation-specific PCR assay. Expression levels of miR-204 were examined by qRT-PCR. Eighteen of 21 nephroblastomas showed a high level of MEIS1 mRNA, and 22 of 26 samples had a specific nuclear protein expression. MicroRNA-204 had a statistically significantly lower expression in all nephroblastomas investigated compared with renal parenchyma, but no change of MEIS1 promoter methylation status was noted. Eleven of 23 nephroblastomas had a high expression of PBX2 mRNA, and 15 of 23 samples had a specific nuclear protein expression was noted. In our study, we demonstrated an expression of MEIS1 and its binding partner PBX2 in most nephroblastomas. The statistically significantly lower expression of miR-204 in all nephroblastomas investigated might point to an involvement of miR-204 in the regulation of MEIS1 in nephroblastomas.</p>","PeriodicalId":520743,"journal":{"name":"Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society","volume":" ","pages":"169-75"},"PeriodicalIF":1.9,"publicationDate":"2014-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2350/13-01-1288-OA.1","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40300595","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 22
IMAGe association: report of two cases in siblings with adrenal hypoplasia and review of the literature. 影像关联:兄弟姐妹肾上腺发育不全2例报告及文献复习。
IF 1.9
Katherine Phillips, May R Arroyo, Lizette Vila Duckworth
{"title":"IMAGe association: report of two cases in siblings with adrenal hypoplasia and review of the literature.","authors":"Katherine Phillips,&nbsp;May R Arroyo,&nbsp;Lizette Vila Duckworth","doi":"10.2350/14-01-1421-OA.1","DOIUrl":"https://doi.org/10.2350/14-01-1421-OA.1","url":null,"abstract":"<p><p>We report the postmortem findings of two siblings with gross and microscopic features consistent with IMAGe association (Intrauterine growth retardation, Metaphyseal dysplasia, Adrenal hypoplasia congenita, and Genital anomalies) with an emphasis on the histopathology of the adrenal gland in this rare syndrome. The first sibling was an 8-week old male diagnosed postnatally with primary adrenal insufficiency. There was no deletion of the DAX1 gene by FISH. Examination at autopsy revealed dysmorphic features including frontal bossing, epicanthal folds, flat philtrum, cryptorchidism, penile chordee, overriding fourth toe, and height and weight below 3rd percentile. Grossly, the adrenal glands were not identified; however, microscopic examination of the suprarenal soft tissue revealed a 3 mm focus of disorganized fetal adrenal cortex with distended \"cytomegalic\" cells with abundant pink eosinophilic cytoplasm, vesicular nuclei, and cytoplasmic vacuolization. A minute focus of permanent adult cortex was also seen, but no adrenal medulla was identified. An autopsy of the sibling, who died 12 years previously at day 9 of life, revealed dysmorphic facial features with cryptorchidism and a large phallus. The adrenal glands were grossly hypoplastic (11 mm). Histologically, the adrenal glands showed disorganized fetal cortex with cytomegalic cells, a larger amount of permanent adult cortex, and bizarre nuclei with numerous pseudoinclusions. While there is currently limited information regarding the histopathologic adrenal findings in IMAGe association, our small case series suggests overlapping features between X-linked recessive congenital adrenal hypoplasia (cytomegalic cells with lack of permanent adult cortex) and autosomal recessive congenital adrenal hypoplasia (diminished permanent adult cortex without cytomegalic cells).</p>","PeriodicalId":520743,"journal":{"name":"Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society","volume":" ","pages":"204-8"},"PeriodicalIF":1.9,"publicationDate":"2014-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2350/14-01-1421-OA.1","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40299870","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 1
Postmortem ultrasonography of the macerated fetus complements autopsy following in utero fetal demise. 浸泡胎儿的死后超声检查补充了子宫内胎儿死亡后的尸检。
IF 1.9
Mary Ashley Cain, Claude B Guidi, Thora Steffensen, Valerie E Whiteman, Enid Gilbert-Barness, Dennis R Johnson
{"title":"Postmortem ultrasonography of the macerated fetus complements autopsy following in utero fetal demise.","authors":"Mary Ashley Cain,&nbsp;Claude B Guidi,&nbsp;Thora Steffensen,&nbsp;Valerie E Whiteman,&nbsp;Enid Gilbert-Barness,&nbsp;Dennis R Johnson","doi":"10.2350/14-02-1439-CR.1","DOIUrl":"https://doi.org/10.2350/14-02-1439-CR.1","url":null,"abstract":"<p><p>Postmortem evaluation following an in utero fetal demise is essential for determining cause of death and counseling regarding future pregnancies. Severe maceration and fetal size along with patient desires may limit the physician's ability to perform a complete autopsy. In the cases presented, we demonstrate the utility of postmortem ultrasonography as an adjunct to traditional autopsy following fetal demise.</p>","PeriodicalId":520743,"journal":{"name":"Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society","volume":" ","pages":"217-20"},"PeriodicalIF":1.9,"publicationDate":"2014-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2350/14-02-1439-CR.1","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40298267","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 7
Histologic differences in placentas of preeclamptic/eclamptic gestations by birthweight, placental weight, and time of onset. 出生体重、胎盘重量和发病时间对子痫前期/子痫妊娠胎盘组织学差异的影响。
IF 1.9
Matthew W Stark, Latoya Clark, Randall D Craver
{"title":"Histologic differences in placentas of preeclamptic/eclamptic gestations by birthweight, placental weight, and time of onset.","authors":"Matthew W Stark,&nbsp;Latoya Clark,&nbsp;Randall D Craver","doi":"10.2350/13-09-1378-OA.1","DOIUrl":"https://doi.org/10.2350/13-09-1378-OA.1","url":null,"abstract":"<p><p>With preeclampsia/eclampsia (PE/E), infants more often are either large or small for gestational age. We explored whether the differences in infant birthweight (BW), placental weights (PW), or time of onset are associated with histologic features of maternal vascular underperfusion. A retrospective chart identified 243 PE/E gestations between 2007 and 2010. Gestational age only was known at slide review. Investigated features included increased syncytial knots, villous agglutination, increased intervillous fibrin, distal villous hypoplasia, acute atherosis, mural hypertrophy of membrane arterioles, muscularized basal plate arteries, increased placental site giant cells, increased immature intermediate trophoblasts, infarcts, and villitis. The results were correlated with BW, PW, and onset time PE/E. One hundred thirty-eight PE/E gestations were identified with adequate slides and history. Increased BW placentas had decreased syncytial knots and increased mural hypertrophy of membrane arterioles. Decreased BW had increased placenta site giant cells. Increased PW had decreased distal villous hypoplasia. Decreased PW had increased syncytial knots, increased intervillous fibrin, and increased acute atherosis. Early-onset disease had increased syncytial knots, distal villous hypoplasia, villous agglutination, and infarcts. This suggests PE/E is not a single process resulting in a uniform distribution of lesions but, rather, is composed of several different processes manifesting a single clinical presentation.</p>","PeriodicalId":520743,"journal":{"name":"Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society","volume":" ","pages":"181-9"},"PeriodicalIF":1.9,"publicationDate":"2014-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2350/13-09-1378-OA.1","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40307242","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 28
Evaluation of serial urine viral cultures for the diagnosis of cytomegalovirus infection in neonates and infants. 尿系列病毒培养对新生儿和婴儿巨细胞病毒感染诊断的评价。
IF 1.9
Karen M Chisholm, Natali Aziz, Michal McDowell, Frances P Guo, Nivedita Srinivas, William E Benitz, Mary E Norton, Kathleen Gutierrez, Ann K Folkins, Benjamin A Pinsky
{"title":"Evaluation of serial urine viral cultures for the diagnosis of cytomegalovirus infection in neonates and infants.","authors":"Karen M Chisholm,&nbsp;Natali Aziz,&nbsp;Michal McDowell,&nbsp;Frances P Guo,&nbsp;Nivedita Srinivas,&nbsp;William E Benitz,&nbsp;Mary E Norton,&nbsp;Kathleen Gutierrez,&nbsp;Ann K Folkins,&nbsp;Benjamin A Pinsky","doi":"10.2350/14-01-1432-OA.1","DOIUrl":"https://doi.org/10.2350/14-01-1432-OA.1","url":null,"abstract":"<p><p>Cytomegalovirus (CMV) is the most common cause of congenital infection worldwide. Urine viral culture is the standard for CMV diagnosis in neonates and infants. The objectives of this study were to compare the performance of serial paired rapid shell vial cultures (SVC) and routine viral cultures (RVC), and to determine the optimal number of cultures needed to detect positive cases. From 2001 to 2011, all paired CMV SVC and RVC performed on neonates and infants less than 100 days of age were recorded. Testing episodes were defined as sets of cultures performed within 7 days of one another. A total of 1264 neonates and infants underwent 1478 testing episodes; 68 (5.4%) had at least one episode with a positive CMV culture. In episodes where CMV was detected before day 21 of life, the first specimen was positive in 100% (16/16) of cases. When testing occurred after 21 days of life, the first specimen was positive in 82.7% (43/52) of cases, requiring three cultures to reach 100% detection. The SVC was more prone to assay failure than RVC. Overall, when RVC was compared to SVC, there was 86.0% positive agreement and 99.9% negative agreement. In conclusion, three serial urine samples are necessary for detection of CMV in specimens collected between day of life 22 and 99, while one sample may be sufficient on or before day of life 21. Though SVC was more sensitive than RVC, the risk of SVC failure supports the use of multimodality testing to optimize detection.</p>","PeriodicalId":520743,"journal":{"name":"Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society","volume":" ","pages":"176-80"},"PeriodicalIF":1.9,"publicationDate":"2014-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2350/14-01-1432-OA.1","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40300337","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 2
Plexiform fibromyxoma: report of two pediatric cases and review of the literature. 丛状纤维黏液瘤:小儿2例报告及文献复习。
IF 1.9
Lizette Vila Duckworth, Raul S Gonzalez, Matthew Martelli, Chen Liu, Cheryl M Coffin, John D Reith
{"title":"Plexiform fibromyxoma: report of two pediatric cases and review of the literature.","authors":"Lizette Vila Duckworth,&nbsp;Raul S Gonzalez,&nbsp;Matthew Martelli,&nbsp;Chen Liu,&nbsp;Cheryl M Coffin,&nbsp;John D Reith","doi":"10.2350/13-09-1373-OA.1","DOIUrl":"https://doi.org/10.2350/13-09-1373-OA.1","url":null,"abstract":"<p><p>Plexiform fibromyxoma is a distinctive mesenchymal neoplasm usually arising in the gastric antrum. We report 2 cases of this entity in pediatric patients, including the first case arising in the esophagus. The patients were a 16-year-old female who presented with chest pain and was found on computed tomographic scan to have a midesophageal mass at the level of the carina, and an 11-year-old female with a gastric mass. Both patients underwent surgical resection of their tumors, which histologically exhibited a plexiform growth pattern with multiple nodules in the muscularis propria and infiltrative borders. The nodules were composed of a rich myxoid stroma with bland uniform spindle cells, no mitoses or necrosis, and delicate blood vessels in the background. Immunohistochemical studies demonstrated that the tumor cells were immunoreactive with smooth muscle actin and not reactive with S-100, CD34, desmin, and c-kit (CD117). We report the first case of plexiform fibromyxoma originating in the esophagus, emphasize its occurrence in pediatric patients, and review the related literature.</p>","PeriodicalId":520743,"journal":{"name":"Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society","volume":" ","pages":"21-7"},"PeriodicalIF":1.9,"publicationDate":"2014-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2350/13-09-1373-OA.1","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40266367","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 40
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