Primitive neuroectodermal tumor in a child with Currarino syndrome
Background. Curarino syndrome is a rare and complex anomaly with the triad of anorectal malformation, presacral mass and sacral bone deformation. The most common cause of the presacral mass is meningioma, but teratoma is the diagnosis in about one-third of the cases. Malignant transformation...
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| Format: | Article |
| Language: | English |
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Hacettepe University Institute of Child Health
2022-04-01
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| Series: | The Turkish Journal of Pediatrics |
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| Online Access: | https://turkjpediatr.org/article/view/163 |
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| author | Memnune Nur Çebi Gizem Yılmaz Gökçe Çelikdemir Rahşan Özcan Süheyla Ocak Tülin Tiraje Celkan Nil Çomunoğlu |
| author_facet | Memnune Nur Çebi Gizem Yılmaz Gökçe Çelikdemir Rahşan Özcan Süheyla Ocak Tülin Tiraje Celkan Nil Çomunoğlu |
| author_sort | Memnune Nur Çebi |
| collection | DOAJ |
| description |
Background. Curarino syndrome is a rare and complex anomaly with the triad of anorectal malformation, presacral mass and sacral bone deformation. The most common cause of the presacral mass is meningioma, but teratoma is the diagnosis in about one-third of the cases. Malignant transformation of teratoma in the form of carcinoma, rhabdomyosarcoma and leukemia have previously been reported on rare occasions.
Case. A 19 month-old-girl was referred with a presacral mass of 29mm x 23mm x 24mm. She was diagnosed as Currarino syndrome. The presacral mass was surgically resected and pathological examination revealed a foci of primitive neurectodermal tumor.
Conclusions. This is the first case of Currarino syndrome with a primitive neuroectodermal tumor (PNET) foci in the presacral mass. Considering the risk of malignant transformation, the accurate pathological examination is important for complete systemic evaluation and treatment plan in these children.
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| format | Article |
| id | doaj-art-c79d31900b124ccda409672a95f709cc |
| institution | OA Journals |
| issn | 0041-4301 2791-6421 |
| language | English |
| publishDate | 2022-04-01 |
| publisher | Hacettepe University Institute of Child Health |
| record_format | Article |
| series | The Turkish Journal of Pediatrics |
| spelling | doaj-art-c79d31900b124ccda409672a95f709cc2025-08-20T02:01:54ZengHacettepe University Institute of Child HealthThe Turkish Journal of Pediatrics0041-43012791-64212022-04-0164210.24953/turkjped.2021.264Primitive neuroectodermal tumor in a child with Currarino syndromeMemnune Nur Çebi0Gizem Yılmaz1Gökçe Çelikdemir2Rahşan Özcan3Süheyla Ocak4Tülin Tiraje Celkan5Nil Çomunoğlu6Departments of Pediatric Hematology and Oncology, İstanbul University Cerrahpaşa Faculty of Medicine, İstanbul, Turkey.Departments of Pediatric Hematology and Oncology, İstanbul University Cerrahpaşa Faculty of Medicine, İstanbul, Turkey.Departments of Pediatric Surgery, İstanbul University Cerrahpaşa Faculty of Medicine, İstanbul, Turkey.Departments of Pediatric Surgery, İstanbul University Cerrahpaşa Faculty of Medicine, İstanbul, Turkey.Departments of Pediatric Hematology and Oncology, İstanbul University Cerrahpaşa Faculty of Medicine, İstanbul, Turkey.Departments of Pediatric Hematology and Oncology, İstanbul University Cerrahpaşa Faculty of Medicine, İstanbul, Turkey.Departments of Pathology, İstanbul University Cerrahpaşa Faculty of Medicine, İstanbul, Turkey. Background. Curarino syndrome is a rare and complex anomaly with the triad of anorectal malformation, presacral mass and sacral bone deformation. The most common cause of the presacral mass is meningioma, but teratoma is the diagnosis in about one-third of the cases. Malignant transformation of teratoma in the form of carcinoma, rhabdomyosarcoma and leukemia have previously been reported on rare occasions. Case. A 19 month-old-girl was referred with a presacral mass of 29mm x 23mm x 24mm. She was diagnosed as Currarino syndrome. The presacral mass was surgically resected and pathological examination revealed a foci of primitive neurectodermal tumor. Conclusions. This is the first case of Currarino syndrome with a primitive neuroectodermal tumor (PNET) foci in the presacral mass. Considering the risk of malignant transformation, the accurate pathological examination is important for complete systemic evaluation and treatment plan in these children. https://turkjpediatr.org/article/view/163PNETcurrarino syndromemalignant transformationpresacral teratoma |
| spellingShingle | Memnune Nur Çebi Gizem Yılmaz Gökçe Çelikdemir Rahşan Özcan Süheyla Ocak Tülin Tiraje Celkan Nil Çomunoğlu Primitive neuroectodermal tumor in a child with Currarino syndrome The Turkish Journal of Pediatrics PNET currarino syndrome malignant transformation presacral teratoma |
| title | Primitive neuroectodermal tumor in a child with Currarino syndrome |
| title_full | Primitive neuroectodermal tumor in a child with Currarino syndrome |
| title_fullStr | Primitive neuroectodermal tumor in a child with Currarino syndrome |
| title_full_unstemmed | Primitive neuroectodermal tumor in a child with Currarino syndrome |
| title_short | Primitive neuroectodermal tumor in a child with Currarino syndrome |
| title_sort | primitive neuroectodermal tumor in a child with currarino syndrome |
| topic | PNET currarino syndrome malignant transformation presacral teratoma |
| url | https://turkjpediatr.org/article/view/163 |
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