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Journal of Indian Society of Pedodontics and Preventive Dentistry Official publication of Indian Society of Pedodontics and Preventive Dentistry
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Year : 2014  |  Volume : 32  |  Issue : 1  |  Page : 71-73

Oral melanotic neuroectodermal tumor of infancy

1 Department of Pedodontics and Preventive Dentistry, Kothiwal Dental College and Research Centre, Moradabad, Uttar Pradesh, India
2 Department of Oral and Maxillofacial Surgery, Kothiwal Dental College and Research Centre, Moradabad, Uttar Pradesh, India

Date of Web Publication15-Feb-2014

Correspondence Address:
Seema Chaudhary
Department of Pedodontics and Preventive Dentistry, Kothiwal Dental College and Research Centre, Moradabad - 244 001, Uttar Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0970-4388.127064

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Melanotic neuroectodermal tumor of infancy (MNTI) is an extremely rare, benign neoplasm of neural crest origin composed of relatively primitive pigment-producing cells. It usually affects new born or infants of <1 year of age, generally involving the face or cranium. This case report describes a 4-month-old child with MNTI involving the anterior region of maxilla. A partial maxillectomy was performed on the left side of maxilla and the patient is now under follow-up.

Keywords: Anterior maxilla, infant, melanotic -neuroectodermal tumour of infancy

How to cite this article:
Chaudhary S, Manuja N, Ravishankar CT, Sinha A, Vijayran M, Singh M. Oral melanotic neuroectodermal tumor of infancy. J Indian Soc Pedod Prev Dent 2014;32:71-3

How to cite this URL:
Chaudhary S, Manuja N, Ravishankar CT, Sinha A, Vijayran M, Singh M. Oral melanotic neuroectodermal tumor of infancy. J Indian Soc Pedod Prev Dent [serial online] 2014 [cited 2023 Feb 4];32:71-3. Available from: http://www.jisppd.com/text.asp?2014/32/1/71/127064

   Introduction Top

Melanotic neuroectodermal tumor of infancy (MNTI) is a relatively uncommon osteolytic-pigmented neoplasm that primarily affects the jaws of new born infants. It is a locally aggressive, benign lesion of neural crest origin that occurs exclusively in infants younger than year of age. [1] What make this tumor unique and interesting is its characteristic predilection for anterior maxilla (premaxilla) and the presence of pigment melanin which gives the tumor distinct clinicopathological, immunohistochemical, ultrastructural and imaging features.

It is a non-ulcerative, painless, rapid expansile tumefaction, pigmented tumor, although the pigmentation cannot always be observed through the covering tissues. [2] The majority of these tumors are slow growing, benign tumors with rate of recurrence has been estimated at 15% and the chance of malignant differentiation at 6.6%. [2] It usually arises in the head and neck region and predominantly affects the maxilla, although other sites, such as brain, epididymis, mediastinum, femur and ovary have also been reported. [3] The most common site of occurrence is the anterior maxillary alveolar ridge (70% cases) followed by the skull (10.8%), brain (5.8%) and mandible (4.3%). [4]

Clinically, MNTI of the jaws appears as an expansile tumor mass, frequently involving tooth displacement. [5] With plain radiographs, MNTI appear as intrabony expansive areas of radiolucency, usually with poorly demarcated margins, probably as a result of rapid tumor growth and a tendency to be locally invasive. [3] The present report describes a case of MNTI in a 4-month-old child requiring a partial-maxillectomy, along with an overview of the current pathological knowledge and therapeutic recommendations.

   Case Report Top

The present case report is about a 4-month-old male child who presented with a 15 day history of a smooth, firm and rapidly growing mass involving the maxillary alveolar ridge. Initially, it was a pea-sized swelling which was growing rapidly. There was no history of airway or feeding difficulties. There was a history of pregnancy and delivery was normal with no history of medication during the pregnancy period. Growth and development of the infant were adequate for his age. On physical examination, there was a dark mass involving the premaxilla that was non-tender and non-pulsatile.

On intraoral examination, a swelling measuring about 2 cm × 2 cm in diameter was seen in the pre-maxillary region involving the left maxillary buttress and extending to the superior nasal floor [Figure 1]. Expansion of the anterior maxillary alveolar ridge with obliteration of labial vestibule was seen. On inspection, swelling was smooth and the overlying mucosa was intact and stretched. The lesion was bony hard, fixed, non-fluctuant and non-tender on palpation with no palpable lymph nodes. An intraoral periapical radiograph revealed a multilocular osteolytic lesion. The results of routine laboratory investigations were within normal limits. The urine levels of vanillylmandelic acid (VMA) were elevated (6-9.5 mg). A provisional diagnosis of vascular malformation or hemangioma was made earlier.
Figure 1: Image showing swelling in the pre-maxillary region

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Treatment was planned. Surgical excision of the lesion was carried out under general anesthesia. The lesion was removed along with the labial mucosa and premaxilla [Figure 2]. However, the child was provided with a splint just after the surgery.
Figure 2: Image showing removed lesion with deciduous central incisor

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Differential diagnosis

Most of the jaw swellings that occur in the infants are usually benign odontogenic cysts or tumors. However, some lesions although benign tend to have an alarming growth rate. The clinical differential diagnosis of a rapidly growing swelling of the anterior maxilla in an infant includes congenital epulis, teratoma, neuroblastoma, Ewing's sarcoma, rhabdomyosarcoma, melanoma, congenital eruption cyst, Burkits lymphoma, Langerhans cell histiocytosis, hemangioma.

   Histopathological Findings Top

H and E sections of this tumor revealed the presence of characteristic biphasic cell population intermixed with fibrocellular fibrous connective tissue stroma with prominent melanin pigmentation [Figure 3]. One group of biphasic cells are made up of irregular nests separated by dense fibrous stroma with large epithelium like cells filled with abundant melanin pigment. Second cell type consist of clusters of poorly cohesive nests of small non melanin containing ovoid cells with minimum cytoplasm and hyperchromatic nuclei resembling neuroblast or lymphoblast like cells, which are present in alveolar pattern or as isolated nests in the stroma.
Figure 3: Histopathological picture

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   Discussion Top

MNTI was first described by Krompecher in 1918; he called it congenital melanocarcinoma. Since then, there has been uncertainty about its cellular origin. He described MNTI as a pigmented tumor of the maxilla associated with a developing tooth and elements of dental lamina in a 2-month-old infant. [6] The origin presumed earlier was from odontogenic or retinal rests, and this led to a plethora of names such as such as retinal anlage tumor, pigmented ameloblastoma, melanotic adamantinoma, retinal choristoma, melanotic epithelial odontoma, melanotic progonoma, pigmented teratoma, atypical melanoblastoma, pigmented epulis and retinoblastic teratoma.

The tumor usually occurs in the head and neck region because of its origin from the neuroectoderm. Anterior maxilla (premaxilla) is the most common site of origin, occurring in almost 71% of cases. Rarely, it can arise in the skull, mandible, brain, meninges, transverse sinus, retina, mediastinum, bones, soft-tissue, uterus and epididymis.

The classic clinical presentation is a sessile, firm swelling involving the upper anterior alveolar ridge and anterior hard palate. The presence of melanin gives it a bluish hue, often mimicking a vascular malformation. It may result in facial asymmetry, dentition displacement and difficulty in feeding. On gross examination, the tumor appears dark blue because of its pigment content. There is a suggestion of pseudo-encapsulation due to reactive bone formation. [5]

However, based on immunohistochemical, ultrastructural, and electron microscope studies and on occasional high urinary excretion of VMA, it is now agreed that the tumor originates from the neural crest. Therefore, the tumor is most commonly referred to as a melanotic (melanocytic) neuroectodermal tumor of infancy, a term first coined by Borello and Gorlin in 1966. [7] A high level of urinary VMA is useful for diagnosing tumors of neural crest origin.

Local excision is the treatment of choice and is usually curative. Some clinicians prefer simple curettage, although others advocate that a 5 mm margin of normal tissue should be obtained. Excision involves largely blunt dissection of the egg-shell thin fibro-osseous capsule from the thinned and expanded hard palate. The average recurrence rate after surgery is about 20%. [5]

In cases where absolute surgical eradication is not possible, such as in midline lesions or those approximating the cranial base, chemotherapy and radiotherapy are potential alternatives. However, this is controversial. Permanent reconstruction of the maxillary alveolus and the missing dentition may have to be delayed until growth is completed, often in the teenage years. In the interim, removable partial dentures may be necessary.

   References Top

1.Retna Kumari N, Sreedharan S, Balachandran D. Melanotic neuroectodermal tumour of infancy: A case report. J Indian Soc Pedod Prev Dent 2007;25:148-51.  Back to cited text no. 1
2.Madrid C, Aziza J, Hlali A, Bouferrache K, Abarca M. Melanotic neuroectodermal tumour of infancy: A case report and review of the aetiopathogenic hypotheses. Med Oral Patol Oral Cir Bucal 2010;15:e739-42.  Back to cited text no. 2
3.Gaiger de Oliveira M, Thompson LD, Chaves AC, Rados PV, da Silva Lauxen I, Filho MS. Management of melanotic neuroectodermal tumor of infancy. Ann Diagn Pathol 2004;8:207-12.  Back to cited text no. 3
4.Neven J, Hulsbergen-van der Kaa C, Groot-Loonen J, de Wilde PC, Merkx MA. Recurrent melanotic neuroectodermal tumor of infancy: A proposal for treatment protocol with surgery and adjuvant chemotherapy. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2008;106:493-6.  Back to cited text no. 4
5.Kruse-Lösler B, Gaertner C, Bürger H, Seper L, Joos U, Kleinheinz J. Melanotic neuroectodermal tumor of infancy: Systematic review of the literature and presentation of a case. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2006;102:204-16.  Back to cited text no. 5
6.Lee CH, Hong SP, Lim CY, Chi JG. Melanotic Neuroectodermal tumor of Infancy. Journal of Korean Medical Science.1986;1:63-7.  Back to cited text no. 6
7.Borello ED, Gorlin RJ. Melanotic neuroectodermal tumor of infancy - A neoplasm of neural crese origin. Report of a case associated with high urinary excretion of vanilmandelic acid. Cancer 1966;19:196-206.  Back to cited text no. 7


  [Figure 1], [Figure 2], [Figure 3]

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