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546 A Giant Orbital Plexiform Neurofibroma with Massive Intracranial Extention in a Newborn
  1. N Yazici1,
  2. H Gulcan2,
  3. O Alkan3,
  4. R Yaycioglu4,
  5. N Bal5,
  6. F Sarialioglu1
  1. 1Department of Pediatric Oncology
  2. 2Department of Neonatology
  3. 3Department of Radiology
  4. 4Department of Eye Disorders
  5. 5Department of Pathology, Baskent University, Adana, Turkey

Abstract

Orbital masses in newborns are rare. Herein a newborn with a giant orbital tumor is presented. A 10-day female baby was admitted to hospital with proptosis. She was the first child of a 29 year-old mother, was born from an uneventful pregnancy. Her birth weight was 3000 gr. No consanguinity between the parents and history of neurofibromatosis in family were present. In physical examination, the baby had bilateral prominent proptosis and rest of the physical examination was unremarkable. The initial diagnosis was metastatic neuroblastoma. MRI of the brain showed a huge mass involving bilateral cavernous sinus, perimedullary cistern, orbita and orbital apex. Neuroblastoma markers including urine VMA, NSE, bone-marrow aspiration examination, abdominal ultrasonography and a two-diamentional chest x-ray were normal. Although the initial radiological diagnosis was plexiform neurofibroma, an open biopsy was performed to rule out other possibilities specially orbital malignant tumors. Histopathological diagnosis was a typical plexiform neurofibroma. The child has been treated with palliative measures and for seizures. Although it was a benign histology, the clinical picture was drastic. Like an infant with a malignant tumor chemotherapy was planned. But the family refused chemotherapy and any other form of antineoplastic therapy. In conclusion, the plexiform neurofibroma must be taken in consideration in newborn infant with orbital tumors.

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