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

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Abstract

Introduction

Melanotic neuroectodermal tumor of infancy (MNT1) is a rare congenital pigmented neoplasm of neural crest origin, locally aggressive, and rapidly growing that develops during the first year of life. It most commonly arises from the maxilla, the cranial vault, and the mandible. Early diagnosis and radical surgery are critical for a long-term outcome.

Methods

A literature search through PUBMED revealed 43 cases of MNT1 arising in the skull. We reviewed the available literature and studied the presenting symptoms, diagnostic procedures, treatment, rates of recurrences, malignancy, and data of follow-up. We report two further cases of infants aged 4 and 10 months, respectively, with MNT1 arising from the cranial vault who underwent radical excision of the lesion.

Conclusion

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.

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Authors and Affiliations

  1. Department of Pediatric Surgery, Aristotle University, G.H. Papageorgiou, Ring Road N. Efkarpia, 56403, Thessaloniki, Greece

    Vassilis Lambropoulos, Andreas Neofytou, Dimitris Sfougaris, Vassilis Mouravas & Anastasios Petropoulos

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  1. Vassilis Lambropoulos
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  2. Andreas Neofytou
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  3. Dimitris Sfougaris
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  4. Vassilis Mouravas
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  5. Anastasios Petropoulos
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Correspondence to Vassilis Lambropoulos.

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Comment

“Lumps and bumps” on the skull of neonates and infants encompass a wide differential diagnosis. This well-written paper is a compilation of the information obtained from previously published articles on melanotic neuroectodermal tumors of the skull in infants. At the occasion of this review, the authors reports on two new cases. An important effort has been made to review all the individual case reports. The illustrations are of good quality. Owing to the restricted total number of cases, the chance for every single pediatric surgeon or neurosurgeon to come across such a diagnosis is rare. Nevertheless, he or she could refer with confidence to this well-documented contribution of Lambropoulos et al.

Benedict Rilliet

Geneva, Switzerland

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Lambropoulos, V., Neofytou, A., Sfougaris, D. et al. Melanotic neuroectodermal tumor of infancy (MNT1) arising in the skull. Short review of two cases. Acta Neurochir 152, 869–875 (2010). https://doi.org/10.1007/s00701-009-0472-5

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