BACKGROUND: Melanotic neuroectodermal tumor is a mostly benign tumor, rare in childhood, essentially located in the head and neck region. CASE REPORT: A two-month-old girl was seen for a rapidly increasing odontogenic tumor which appeared cystic at the CT scan. After enucleation, this premaxillar tumor recurred one month later with an osteogenic aspect at the CT scan. Urine catecholamine excretion was normal; Methyl Iodo Benzyl Guanidin scintigraphy failed to show any fixation and electron microscopy examination of the biopsy showed several varieties of melanocytes. A partial maxillectomy was performed by oral approach. The patient is well 4 1/2 years later. CONCLUSION: This observation confirms the recurrence potential of this tumor and the cosmetic interest of the oral surgical approach.
BACKGROUND:Melanotic neuroectodermal tumor is a mostly benign tumor, rare in childhood, essentially located in the head and neck region. CASE REPORT: A two-month-old girl was seen for a rapidly increasing odontogenic tumor which appeared cystic at the CT scan. After enucleation, this premaxillar tumor recurred one month later with an osteogenic aspect at the CT scan. Urine catecholamine excretion was normal; Methyl Iodo Benzyl Guanidin scintigraphy failed to show any fixation and electron microscopy examination of the biopsy showed several varieties of melanocytes. A partial maxillectomy was performed by oral approach. The patient is well 4 1/2 years later. CONCLUSION: This observation confirms the recurrence potential of this tumor and the cosmetic interest of the oral surgical approach.