Recurrent melanotic neuroectodermal tumor in the orbit successfully treated with resection followed by pediculated periosteal flaps.

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare tumor arising mainly in the orofacial bones. Among more than 200 cases described in articles, only seven cases involved orbital regions. We present a case of newborn with recurrent MNTI in the lateral wall of right orbit. The patient underwent complete surgical resections at onset and relapse. The bone defects following the second surgery were successfully reconstituted with pediculated periosteal flaps from parietofrontal bones. In this article, we describe clinical course of recurrent MNTI in the orbit and discuss management of the tumor involving the orbit. (c) 2008 Wiley-Liss, Inc.