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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 : 2020  |  Volume : 38  |  Issue : 3  |  Page : 319-322

Oral melanotic neuroectodermal tumor of infancy: Management of a case affecting the maxilla

1 Department of Pedodontics and Preventive Dentistry, Government College of Dentistry, Indore, Madhya Pradesh, India
2 Department of Oral Pathology, Government College of Dentistry, Indore, Madhya Pradesh, India
3 Department of Oral and Maxillofacial Surgery, Government College of Dentistry, Indore, Madhya Pradesh, India
4 Department of Oral Diagnosis, Government College of Dentistry, Indore, Madhya Pradesh, India

Date of Submission13-Jun-2018
Date of Decision08-Apr-2019
Date of Acceptance07-May-2019
Date of Web Publication29-Sep-2020

Correspondence Address:
Dr. Anuradha Agrawal
Department of Pedodontics and Preventive Dentistry, Government College of Dentistry, Indore - 452 001, Madhya Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/JISPPD.JISPPD_169_18

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Melanotic neuroectodermal tumor is a rare benign congenital neoplasm involving the head-and-neck region found in infants <1 year of age. The lesion most commonly affects the maxilla. We report the case of a 4-month-old male child with a tumor involving the anterior region of the maxilla along with a brief review of literature, differential diagnosis, and treatment.

Keywords: Anterior maxilla, infancy, melanotic neuroectodermal tumor

How to cite this article:
Agrawal A, Joshi J, Agrawal D, Kumar P, Modi B. Oral melanotic neuroectodermal tumor of infancy: Management of a case affecting the maxilla. J Indian Soc Pedod Prev Dent 2020;38:319-22

How to cite this URL:
Agrawal A, Joshi J, Agrawal D, Kumar P, Modi B. Oral melanotic neuroectodermal tumor of infancy: Management of a case affecting the maxilla. J Indian Soc Pedod Prev Dent [serial online] 2020 [cited 2022 Aug 17];38:319-22. Available from: http://www.jisppd.com/text.asp?2020/38/3/319/296626

   Introduction Top

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare benign tumor of early infancy with rapid expansile growth, high recurrence rate, and neural crest origin.[1] The tumor has been referred to in the past by many terms such as “pigmented epulis,” “retinal anlage tumor,” “congenital melanocarcinoma,” “melanotic epithelial odontoma,” “melanotic ameloblastoma,” and “melanotic progonoma” to name a few.[2]

MNTI is a pigmented tumor, although pigmentation may not be evident clinically, located in the head-and-neck region of young children <1 year of age. It has the potential for malignant transformation of 6.6%. Majority of the lesions arise in the maxilla (68%–80%) followed by the skull (10.8%), the mandible (5.8%), and the brain (4.3%).[3]

We report the clinical and histopathological features of MNTI in a 4-month-old child treated surgically, with a review of literature, differential diagnosis, and treatment.

   Case Report Top

A 4-month-old male child was referred to the Department of Pedodontics, Government College of Dentistry, Indore, with complaints of rapidly enlarging swelling of the oral cavity for 1 month. There was difficulty in feeding due to swelling. There was no history of medication during pregnancy and no relevant past medical history. On clinical examination, there was a 4.5 cm × 3.5 cm × 3.5 cm-size, firm-to-hard swelling arising from the right side of the upper jaw. The swelling was bluish in color and nontender with nonulcerated intact mucosa [Figure 1].
Figure 1: Clinical intraoral photograph showing exposed tumor in the maxillary region

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Computed tomography (CT) scan of the head showed an exophytic expansile mass arising from the right side of the hard palate surrounded by a thin rim of sclerotic bone. The lesion was associated with remodeling of the adjacent bone but had no evidence of erosion [Figure 2].
Figure 2: Computed tomography scan showing radiolucent expansile tumor in the maxilla

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An incisional biopsy was inconclusive; thus, surgical excision with curettage was done in general anesthesia [Figure 3]. A peroral incision with 1 cm margin all around the tumor was taken, and the tumor was excised in toto by electrocautery and blunt dissection. Margins of the wound were closed by 3.0 vicryl suture. The postoperative course was uneventful. Histopathology report showed characteristic biphasic nature with small, round cell at the center and peripheral epithelioid cells containing melanin pigment arranged in an alveolar pattern in a fibrovascular background [Figure 4].
Figure 3: Postoperative photograph after wide excision and curettage

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Figure 4: Histological section demonstrating both small neuroblastic cells and large melanin-containing epithelial cells – biphasic nature (H and E, ×10)

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The diagnosis was confirmed by immunohistochemistry which showed central round cells positive for synaptophysin and CD56 and peripheral cells positive for PanCK and HMB-45 [Figure 5]. The postoperative course was uneventful, and there was no recurrence at 6-month follow-up of patient [Figure 6].
Figure 5: Immunohistochemical confirmation showing synaptophysin receptor positivity

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Figure 6: Postoperative follow-up at 1 month

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   Discussion Top

Krompecher was the first to describe MNTI in 1918.[4] Since then, 365 cases have been reported in literature. Borello and Gorlin believed that this was of a neural crest origin. Furthermore, because this tumor is common in infancy, they recommended the term MNTI.[5] MNTI clinically presents as a rapidly growing, painless, expansile, unencapsulated partly pigmented mass, typically in the maxillary region in children aged <1 year old.[6] It tends to occur as a single lesion. However, multiple lesions have also been reported.

Oral and maxillofacial tumors rarely occur in the pediatric population compared with the adult population. Most jaw swellings that occur in infants are usually benign odontogenic cysts or tumors including Langerhans cell histiocytosis. However, some, like that reported here, though benign, tend to have alarming growth rates. There is, therefore, the tendency to misdiagnose them clinically as malignant lesions. MNTI should be differentiated from other small, round cell tumors occurring in infancy, such as rhabdomyosarcoma, neuroblastoma, melanoma, and Burkitt's lymphoma.[7] The diagnosis can be especially difficult, given the rarity of MNTI.

The histological profile of MNTI demonstrates a biphasic cell population within a stroma of moderately vascularized fibrous tissue. Large, epithelioid, melanin-containing cells are arranged in alveolar or tubular formations around clusters of small neuroblastic cells.[3]

Immunohistochemical stains can greatly assist in establishing the diagnosis. Larger epithelioid cells stain positive most frequently with cytokeratin, epithelial membrane antigen, vimentin, and HMB-45, reflecting epithelial and melanocytic differentiation.[8] These larger cells are usually nonreactive with S-100 protein, aiding in differentiation from tumors such as melanoma.[3],[8] The nests of smaller cells in MNTI are often positive for neurogenic markers such as synaptophysin, neuron-specific enolase, and glial fibrillar acidic protein.[8] The variability seen in staining results likely represents the multiphenotypical characteristic of this tumor.

Conventional radiographs of bony lesions usually show radiolucency with or without irregular margins. It is typical of CT scans to reveal hyperdense masses, but hypodense variants have been reported as well. CT can accurately define the extent of the lesion and thus provides a good basis for surgical planning.[9]

Surgical excision remains the mainstay of treatment for patients with MNTI, as the only modality with proven efficacy. Given the benign nature of most tumors, 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.[10] Efforts should be directed toward the preservation of vital structures and organs. Adjuvant chemotherapy and radiation are ineffective in controlling recurrences where total excision has not been achieved, and their role is therefore extremely limited.[10] The authors have advocated their role in metastatic disease and in cases of recurrence after a second resection, although there remains little evidence of added benefit.[10]

   Conclusion Top

In view of the rapid growth of MNTIs, malignant potential, and their high rate of recurrence, it is essential to diagnose this type of tumor at an early stage. Pediatricians and pediatric dentists should be aware of this disease and direct patients to prompt oral surgery treatment to minimize mutilating surgeries. These patients should also be under regular follow-up after treatment.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

   References Top

Agarwal P, Agarwal V, Raina VK. Melanotic neuroectodermal tumor of infancy: Case report of an unusual tumor. Indian J Plastic Surg 2003;36:123-5.  Back to cited text no. 1
Lurie HI. Congential melanocarcinoma, melanotic adamantinoma, retinal anlage tumor, progonoma, and pigmented epulis of infancy. Summary and review of the literature and report of the first case in an adult. Cancer 1961;14:1090-108.  Back to cited text no. 2
Metwaly H, Cheng J, Maruyama S, Ohshiro K, Suzuki I, Hoshina Y, et al. Establishment and characterization of new cell lines derived from melanotic neuroectodermal tumor of infancy arising in the mandible. Pathol Int 2005;55:331-42.  Back to cited text no. 3
Krompecher E. Zur Histogenese, Morphologic des. Adamantinome und sonstiger Kifergeschwulste. Beit Path Anat 1918;64:165.  Back to cited text no. 4
Borello ED, Gorlin RJ. Melanotic neuroectodermal tumor of infancy – A neoplasm of neural crest origin. Report of a case associated with high urinary excretion of vanilmandelic acid. Cancer 1966;19:196-206.  Back to cited text no. 5
Bangi BB, Tejasvi ML. Melanotic neuroectodermal tumor of infancy: A rare case report with differential diagnosis and review of the literature. Contemp Clin Dent 2012;3:108-12.  Back to cited text no. 6
[PUBMED]  [Full text]  
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.  Back to cited text no. 7
[PUBMED]  [Full text]  
Barrett AW, Morgan M, Ramsay AD, Farthing PM, Newman L, Speight PM. A clinicopathologic and immunohistochemical analysis of melanotic neuroectodermal tumor of infancy. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2002;93:688-98.  Back to cited text no. 8
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. 9
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. 10


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]


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