Maxillar localization of a congenital peripheral primitive neuroectodermal tumor: A case report

Clinical photograph showing the congenital mass localized in the mouth and the left nasal cavity. The tumor appeared hemorrhagic and necrotic.

Computed Tomography (CT) scan with axial imaging showing a tumoral mass dependant of the maxillary bone invading the left nasal cavity with an important periostic reaction (white arrow).

Computed Tomography (CT) scan with axial and coronal imaging showing the recurrence of the tumoral process invading the ethmoïdal bone and the orbit roof (white arrow).

(A) Photomicrographs showing that the tumor is composed of densely packed cells with hyperchromatic nuclei (black arrow) (hematoxylin and eosin at 200× magnification); (B) mitosis are shown. An aspect of rosette is visible (black arrow)) (hematoxylin and eosin at 400× magnification).

Immunohistochemical stainings shown by black arrows for (A) synaptophysin; (B) HMB45; and (C) AE1-AE3. Peripheral tumoral cells are positive for HMB45 and AE1-AE3 stainings (B and C; magnification ×400). Central tumoral cells are positive for synaptophysin (A; magnification ×400).