Recurrent melanotic neuroectodermal tumor of infancy

Abstract

(Editorial Comment: I view that adjuvant therapy may be necessary to control residual or recurrent disease in this unusual tumor.) Melanotic neuroectodermal tumor of infancy (MNTI) is an uncommon neoplasm found primarily in children less than one year of age. First described in the early 1900s by Krompecher as a congenital melanocarcinoma, 1 the nomenclature of this neoplasm has undergone multiple changes that have been based on histologic and biochemical studies. Since Borello and Gorlin’s discovery of the associated high urinary excretion of vanillylmandelic acid (VMA) in the late 1960s, 2 MNTI has been thought to be a derivative of neural crest cells. More recently, Hoshino et al have demonstrated high levels of urine catecholamines in a case of MNTI, 3 which further supports the neural origin of this rare neoplasm. In a review of the world literature, approximately 227 cases of MNTI have been reported, most of which (93%) involve the head and neck region. The maxilla (69%) is the most commonly affected site, followed by the skull (11%), the mandible (6%), and less commonly, brain, mediastinum, thigh, epididymis, foot, and shoulder. 4 This tumor is reported to be malignant in only 4% of the cases 5 but is considered to be locally aggressive.