Melanotic neuroectodermal tumour of infancy involving the orbit and maxilla: surgical management and follow-up strategy

Franchi, G.; Sleilati, Fadi; Soupre, V.; Diner, P.A.; Vazquez, Marie‐Paule; Boudjemaa, Sabah; Josset, P

doi:10.1054/bjps.2002.3910

Cited by 22 publications

(11 citation statements)

References 11 publications

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“…Surgical excision remains the mainstay of treatment for patients with MNTI, as the only modality with proven efficacy (2,15). Given the benign nature of most tumours, there has been some controversy over the optimal surgical management and necessity of adequate margins.…”

Section: Discussion and Literature Reviewmentioning

confidence: 99%

“…Given the benign nature of most tumours, there has been some controversy over the optimal surgical management and necessity of adequate margins. Views range from conservative enucleation, to curettage of adjacent tissue, to recommended margins of up to 5 mm (2,18,20,36,37). Efforts should be directed toward the preservation of vital structures and organs (38).…”

Section: Discussion and Literature Reviewmentioning

confidence: 99%

“…M elanotic neuroectodermal tumour of infancy (MNTI) is a rare, pigmented neoplasm generally arising in infants during the first year of life (1)(2)(3). This benign tumour originates from the soft tissue overlying the maxilla in more than 70% of cases, although mandibular, cranial, cerebral and genital involvement have also been described (4,5).…”

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confidence: 99%

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Melanotic neuroectodermal tumour of infancy

Hamilton¹,

MacRae²,

Agrawal³

2008

Plastic Surgery

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Melanotic neuroectodermal tumour of infancy is an extremely rare neoplasm arising in newborns and young children, typically involving the face or cranium. A case arising from the maxilla, requiring extensive resection with a near-total maxillectomy, is presented. A thorough review of the literature on this unusual tumour is provided, with emphasis on prognostic factors and appropriate treatment.Key Words: Maxillectomy; Melanotic; MNTI; Pediatric; Pigmented Tumeur mélanotique neuroectodermique de l'enfance La tumeur mélanotique neuroectodermique de l'enfance, qui touche surtout la face et le crâne, est extrêmement rare chez les nouveau-nés et les jeunes enfants. Il sera question, dans l'article, d'un cas de tumeur du maxillaire, qui a nécessité une résection étendue, voire une maxillectomie quasi totale. Un examen exhaustif de la documentation sur ce type peu fréquent de tumeur, plus particulièrement sur les facteurs pronostiques et le traitement approprié, complétera l'étude du sujet.

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Section: Discussion and Literature Reviewmentioning

confidence: 99%

Section: Discussion and Literature Reviewmentioning

confidence: 99%

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Melanotic neuroectodermal tumour of infancy

Hamilton¹,

MacRae²,

Agrawal³

2008

Plastic Surgery

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“…While typically considered a benign lesion, a malignant potential has been demonstrated at 6.5% and treatment is primarily surgical, though recent advances in tumor genetics have identified mutations conferring chemotherapy sensitivity or resistance [ 3 , 4 ]. One of the most adverse features of, and difficulty in treating, these lesions is its high rate of recurrence demonstrated at 18.5% in a recent series, but noted as high as 45% in other reports [ 5 ]. While these lesions most commonly occur in the head and neck region, review of the otolaryngology literature shows a paucity of information regarding these uncommon tumors.…”

Section: Introductionmentioning

confidence: 99%

Postauricular Melanocytic Neuroectodermal Tumor of Infancy: A Rare Site of a Rare Tumor—MNTI as a Postauricular Mass with Literature Review

Evans

Woolley

2018

Case Reports in Otolaryngology

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Melanocytic neuroectodermal tumor of infancy (MNTI) is a rare osteolytic neoplasm of neural crest cell origin. There are less than 500 documented cases, most frequently affecting the maxilla of infants less than 1 year old. We present a unique case of a two-month-old male with a progressive postauricular mass since birth, confirmed to be a MNTI. The lesion required three resections over the course of five months, with rapid recurrence ultimately requiring a craniectomy, highlighting the difficulty in treating these tumors. Histological and radiographic features were reviewed; an updated literature review for identifying and treating these lesions is presented.

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“…Many cases of incompletely resected tumors do not demonstrate regrowth, and residual neoplastic tissue may involute spontaneously. 10 In cases of local tumor recurrence, adjuvant chemotherapy has been reported as a successful treatment modality. 9 The patient in this case presented with a disfiguring and locally invasive maxillary MNTI for which he underwent a right partial maxillectomy and inferior turbinectomy via a facial degloving approach.…”

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confidence: 99%

Mass Originating From the Maxillary Alveolar Crest in an Infant

Marston

Black

Pambuccian

et al. 2014

JAMA Otolaryngol Head Neck Surg

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An infant presented with a large mass protruding from the right maxillary alveolar ridge. The mass was first noted at 2 months of age and was initially thought to be an odontogenic cyst. The mass expanded rapidly over the next several months and was associated with intermittent increased work of breathing, feeding difficulty, and a 4.5-kg weight loss. Intraoral examination revealed a large, firm, gray mass originating from the right maxillary alveolar ridge (Figure, A). Contrast computed tomographic imaging showed an expansile multilobulated heterogeneously enhancing mass measuring 4.1 × 4.8 × 4.4 cm originating from the right maxillary alveolar ridge with displacement of the floor of the maxillary sinus. Within the mass there was a central "sunburst" representing new bone formation. The patient was initially taken to the operating room for open biopsy. The specimen consisted of small blue cells with a neuroblastic appearance and cuboidal cells with variable melanin pigmentation forming glandlike epithelial structures within a background of dense fibrous stroma (Figure, B and C). Immunohistochemical analysis revealed that both the epithelial and neural components were positive for neuron-specific enolase. The epithelial cells stained positive for cytokeratin AE1/AE3 (Figure, D), tyrosinase, and S-100, while the neuroblastic cells stained positive for synaptophysin with 20% Ki-67 positivity. The patient's urinary vanillylmandelic acid levels were within reference range.

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Melanotic neuroectodermal tumour of infancy involving the orbit and maxilla: surgical management and follow-up strategy

Cited by 22 publications

References 11 publications

Melanotic neuroectodermal tumour of infancy

Melanotic neuroectodermal tumour of infancy

Postauricular Melanocytic Neuroectodermal Tumor of Infancy: A Rare Site of a Rare Tumor—MNTI as a Postauricular Mass with Literature Review

Mass Originating From the Maxillary Alveolar Crest in an Infant

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