Melanotic neuroectodermal tumor of infancy: 2 decades of clinical experience with 18 patients

Abstract

Purpose: The purpose of this article is to report our experience in the management of 18 patients with melanotic neuroectodermal tumor of infancy involving the maxillary alveolus.

Patients and methods: All patients presented with hard nontender swelling involving the upper alveolus with facial deformity. Analysis included hematocrit, coagulation profile, serum creatinine, and screening for vanillyl mandelic acid and catecholamines. Imaging studies included x-ray of the maxilla and chest, ultrasound of the abdomen, computed tomography scan (1990 to 1999), and magnetic resonance imaging (after 1999). All surgeries were performed using endotracheal anesthesia, and complete gross excision of the tumor was achieved with coverage of the defect with mucoperiosteal flaps. All specimens were subjected to histopathology and immunohistochemistry.

Results: The expansion of the alveolus produced by the tumor improved in 4 to 6 months. Subsequent dentition was affected by the removal of involved tooth buds during the operation. All the patients are in regular follow-up (maximum 206 months) and there has been no local recurrence or distant metastasis. Mean follow-up time was 130.8 months (95% confidence interval, 168.8-210.6). Overall survival at 17 years was 85.6%. Median survival could not be established due to statistically insignificant sample size, while mean survival time was 189.7 months (95% confidence interval, 103.7-157.8).

Conclusions: In the absence of metastatic disease, melanotic neuroectodermal tumors of infancy can be successfully managed by local excision.