Melanotic neuroectodermal tumour of infancy

Abstract

Melanotic neuroectodermal tumour of infancy is an extremely rare neoplasm arising in newborns and young children, typically involving the face or cranium. A case arising from the maxilla, requiring extensive resection with a near-total maxillectomy, is presented. A thorough review of the literature on this unusual tumour is provided, with emphasis on prognostic factors and appropriate treatment.Key Words: Maxillectomy; Melanotic; MNTI; Pediatric; Pigmented Tumeur mélanotique neuroectodermique de l'enfance La tumeur mélanotique neuroectodermique de l'enfance, qui touche surtout la face et le crâne, est extrêmement rare chez les nouveau-nés et les jeunes enfants. Il sera question, dans l'article, d'un cas de tumeur du maxillaire, qui a nécessité une résection étendue, voire une maxillectomie quasi totale. Un examen exhaustif de la documentation sur ce type peu fréquent de tumeur, plus particulièrement sur les facteurs pronostiques et le traitement approprié, complétera l'étude du sujet.

Congenital Granular Cell Epulis: Classic Presentation and Its Differential Diagnosis

“…Clinically, major differential diagnoses of CGCE include rhabdomyoma, infantile myofibroma, melanotic neuroectodermal tumor of infancy, peripheral odontogenic fibroma, and neurofibroma, among others [2,20,21]. Many of these potential diagnoses also present in pre-natal stages but can be readily differentiated from CGCE due to their variation in anatomical location and histologic appearance.…”

Melanotic neuroectodermal tumour of infancy of the jaws: an analysis of diagnostic features and treatment

“…Judd et al did not remove the whole lesion in two enucleations in order to avoid mutilation of other structures, and no recurrence was ob- served after 30 months 17 . On the other hand, other authors have suggested tumour resection with wide surgical excision of at least 5 mm 21,22 , stating that recurrence was due to incomplete removal of the lesion 22 . In addition to incomplete excision, recurrence could also be a consequence of dissemination during surgery or multicentric tumours, factors that have been suggested in some case reports 23,24 .…”

Melanotic neuroectodermal tumour‐ difficult airway management in an infant

“…In 70% of cases it invades the overlying maxilla with the incisal area the most frequently involved site. Mandibular, cranial, cerebral and genital involvement have also been described [1,2]. In our patient, the tumour originated from the right maxillary alveolar process and had progressed from pea-sized to a large mass causing difficulty in feeding.…”

“…It can invade locally with bony destruction, tooth displacement and feeding difficulties. It is generally described as benign despite being capable of aggressive local invasion [2]. In 70% of cases it invades the overlying maxilla with the incisal area the most frequently involved site.…”

“…It arises predominantly from the head and neck, particularly the maxilla [1]. There are reports of neuroectodermal tumours arising from the maxilla in which a maxillectomy was performed [2]. These patients pose unique challenges to the anaesthetist in terms of airway management.…”