The Turkish Journal of Pediatrics 2020 , Vol 62 , Num 5
High-grade neuroepithelial tumor with medulloepithelioma-like areas out of the central nervous system in an infant with hemihypertrophy: a unique association
İbrahim Karnak 1 ,Gökhan Gedikoğlu 2 ,Berna Oğuz 3 ,Figen Söylemezoğlu 2 ,Armita Bahrami 4 ,Jason Chiang 4 ,Tezer Kutluk 5
1 Departments of Pediatric Surgery, Hacettepe University Faculty of Medicine, Ankara, Turkey
2 Departments of Pathology, Hacettepe University Faculty of Medicine, Ankara, Turkey
3 Departments of Radiology, Hacettepe University Faculty of Medicine, Ankara, Turkey
4 Department of Pathology, Division of Clinical Pathology, St. Jude Children’s Research Hospital, Memphis, USA
5 Departments of Pediatric Oncology, Hacettepe University Faculty of Medicine, Ankara, Turkey
DOI : 10.24953/turkjped.2020.05.017 Background. High-grade neuroepithelial tumor with areas resembling medulloepithelioma was diagnosed in an infant with coccygeal and inguinal masses. Hemihypertrophy is associated with Wilms tumor, hepatoblastoma and pancreatic tumors in children.

Case. The authors report on the first case of peripheral HNET associated with hemihypertrophy in an infant, with special discussion on histopathological differential diagnosis and management of this rare and highly malignant tumor.

Conclusions. HNET should be included into the list of hemihypertrophy associated tumors. Complete surgical excision with free margins is essential for the successful treatment of such cases and should be tried in suitable cases at the time of diagnosis. Continued treatment should be decided individually on a case to case basis. Keywords : high-grade neuroepithelial tumor, medulloepithelioma, inguinal, coccygeal, tumor, hemihypertrophy, infant

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