Melanotic neuroectodermal tumor of infancy a review of seven cases

Abstract

The melanotic neuroectodermal tumor of infancy (MNTI) is a rare, usually benign, pigmented neuroectodermal tumor which most often involves the maxilla. The authors reviewed seven cases of MNTI, with patient ages of our patients ranged from nine weeks to 18 months; six of the seven were less than six months old at initial diagnosis. Four patients were males, and all were white. One tumor each was located in the femur, the temporal bone, and the epididymis; the remaining lesions occurred in the maxilla. Three of the four maxillary tumors recurred locally; the epididymal and femoral tumors metastasized. Two of these cases had unique clinical or pathologic features. The case of the femoral tumor is remarkable in that it is the first reported one of MNTI presenting in a long bone. This tumor was aggressively malignant; within two months after its discovery, a large mass of similar tumor was formed in the pelvis, and the tumor resulted in the patient's death. To the authors' knowledge, the case of the temporal bone tumor is the first one of MNTI in which neuronal differentiation of the neuroblastic cells is convincingly demonstrated. This finding provides additional evidence in support of the neuroectodermal theory of origin of these neoplasms.

Melanotic neuroectodermal tumour of infancy: A case report and review of the aetiopathogenic hypotheses

“…Its free form found in great quantity is regarded as a marker of the neuroectodermal tumours. The disappearance of high rates of VMA after MNTI surgical removal confirms the neurocristopathic hypothesis (6). Nevertheless, a significant number of cases of MNTI associated with normal levels of VMA appear in the literature.…”

“…This lesion is regarded as benign, although it can show locally aggressive behaviour, with gradual invasion of the surrounding bone and the sinuses. Recurrences have been described after surgical excision, and in exceptional cases, metastasis have been discovered (6). The rate of recurrence has been estimated at 15% and the rate of malignant transformation at 6.6% (4).…”

Extensive Melanotic Neuroectodermal Tumor of Infancy

“…Pigmentation may or may not be seen in the overlying soft tissue. This condition is generally found in patients under the age of 1 year, usually without predilection for sex [1,8,10,11], although in some review studies of cases described in the literature, we observed a slight predilection for the male sex [1,8,[12][13][14]. In spite of being locally invasive, these tumors are generally described as being benign.…”

Melanotic neuroectodermal tumour of infancy

“…The local recurrence rate following excision ranges from 10% to 15%, although it has been reported as high as 45% (11,12,25,41,42). Shaia et al (38) examined the risk of recurrence over the past 20 years and found that it did not differ from historical data since the tumour was first diagnosed 90 years previously.…”