Melanotic neuroectodermal tumor of infancy (MNT1) arising in the skull. Short review of two cases

Abstract

Melanotic neuroectodermal tumor of infancy should be included in the differential diagnosis of skull lesions in infants. Radical surgery must be considered as the treatment of choice and close follow-up for at least 2 years is necessary.

Calvarial malignant melanotic neuroectodermal tumour of infancy presenting with widespread intracranial metastasis

“…The tumour is most often reported in the head and neck region with the majority of cases located in the maxilla (69%), the cranial vault (11%), the mandible (6%) and less commonly the brain, mediastinum, thigh, epididymis, foot and brain (Butt et al, 2009;Lambropoulos et al, 2010). Anecdotal reportage from the epididymis, mediastinum, skin and the female reproductive organs are available (Lacy and Kuhar, 2010;Lambropoulos et al, 2010).…”

“…Anecdotal reportage from the epididymis, mediastinum, skin and the female reproductive organs are available (Lacy and Kuhar, 2010;Lambropoulos et al, 2010). Calvarial lesions arise at sutures, most commonly at the anterior fontanelle followed by the temporoparietal and fronto-temporosphenoid regions (George et al, 1995).…”

Melanotic Progonoma: An Unusual Pathology for an Infantile Midline Scalp Mass

“…Rapid growth and MR characteristics can be clues to predict an unusual pathology other than scalp dermoid tumor in similar cases. High recurrence rate and malignant transformation potential have been reported [4], so radical surgery and follow-up at close intervals are warranted to improve long-term outcome [5]. Considering the signal variability of dermoid tumors of the scalp, we did not expect the current pathology; however, we radically excised the lesion.…”

“…Considering the signal variability of dermoid tumors of the scalp, we did not expect the current pathology; however, we radically excised the lesion. Early diagnosis of the lesion on the basis of its distinctive clinicopathological and imaging features is mandatory as more and more cases are reported [4,5]. …”

Giant melanotic neuroectodermal tumor of infancy (melanotic progonoma) of the head and neck: report of a malignant case

“…Although MNTI is mostly benign, a malignancy rate of 6.6% has been reported. 15,20 Postoperative recurrence rates for MNTI range from 10% to 60%. 11 Late recurrence following resection is rare; however, 39.3% of all cases occur within 4 weeks of surgery and up to 71.4% of cases recur within 4 months.…”