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Journal of Indian Association of Pediatric Surgeons
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Year : 2021  |  Volume : 26  |  Issue : 4  |  Page : 265-267

Melanotic neuroectodermal tumor of infancy: Does enucleation alone suffice?

Department of Pediatric Surgery, Narayana Health, Bengaluru, Karnataka, India

Date of Submission01-May-2020
Date of Decision20-Oct-2020
Date of Acceptance27-Nov-2020
Date of Web Publication12-Jul-2021

Correspondence Address:
Dr. Sanjay Rao
Department of Pediatric Surgery, Mazumdar Shaw Multispeciality Building, 258/A, Bommasandra Industrial Area, Anekal Taluk, Hosur Road, Bengaluru - 560 099, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jiaps.JIAPS_135_20

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Melanotic neuroectodermal tumor of infancy (MNTI) is a rare melanin-containing mesenchymal tumor of neural crest origin. We present a case of MNTI in a 1-year-old girl. It was managed successfully with conservative excision (enucleation).

Keywords: Enucleation, exophytic growth, melanotic neuroectodermal tumor

How to cite this article:
Zameer M M, Rao S, Chandrashekar V, D’Cruz A. Melanotic neuroectodermal tumor of infancy: Does enucleation alone suffice?. J Indian Assoc Pediatr Surg 2021;26:265-7

How to cite this URL:
Zameer M M, Rao S, Chandrashekar V, D’Cruz A. Melanotic neuroectodermal tumor of infancy: Does enucleation alone suffice?. J Indian Assoc Pediatr Surg [serial online] 2021 [cited 2023 Jan 28];26:265-7. Available from: https://www.jiaps.com/text.asp?2021/26/4/265/321192

   Introduction Top

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare melanin-containing mesenchymal tumor of neural crest origin. We present a case of MNTI which was managed successfully with conservative excision (enucleation).

   Case Report Top

A 1-year-old girl presented with a large growth protruding from her oral cavity, gradually increasing in size since 5 months of age. Her feeding was poor. On examination, she weighed 3.6 kg (<3rd centile). A lobulated, spherical mass, 8 cm in diameter, was seen protruding from the oral cavity and seemed to arise from the maxilla. The mass was bony hard, immobile, nontender, and pigmented. It was an exophytic mass of bone with desiccated mucosa covering it. X-ray suggested dense calcification within the mass. Computed tomography (CT) scan revealed a large bone forming exophytic mass arising from the maxilla [Figure 1]. The initial plan was to amputate the exophytic portion for tissue diagnosis and to have satisfactory mouth closure. As intubation was difficult, a tracheostomy was done to secure the airway. At surgery, the mass was found to be made of extremely dense bone and required a powered instrument to amputate. A central area within the mass had a grayish soft fleshy tissue speckled with pigment. The solid mass was enucleated and sent for biopsy. A palatal flap was used to cover the bony defect. Oral feeding was rapidly re-established and tracheostomy was removed after 2 weeks. The biopsy showed a biphasic tumor with tubular or alveolar pattern with large melanin-containing cells with abundant cytoplasm and pale nuclei suggestive of MNTI. As the lesion seemed to arise from the right maxilla, a maxillectomy was planned and the reconstruction post resection was a challenge. The poor nutritional status, risks, and potential for poor cosmetic outcomes led to significant delays. During this time, the baby did really well. A review 3 months later showed no progression of the lesion. A plain X-ray showed a remarkable degree of bone remodeling toward normal. A CT scan 6 months later showed completely normal facial bones with no recurrence [Figure 2]a. The child was counseled regarding the risk of recurrence and was kept on close follow-up. The child has now been followed up for over 7 years and has grown well with no signs or symptoms (except of no growth of the upper two incisors). The X-ray at 7 years follow-up shows normal facial growth [Figure 2]b. The child did not require any further surgeries.
Figure 1: Preoperative child with computed tomography scan revealing a large bone forming exophytic mass arising from the maxilla

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Figure 2: Clinical pic and imaging of child on follow-up: Six months (a) and 7 years (b)

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   Discussion Top

MNTI is an unusual, benign osteolytic neoplasm of neural crest origin[1] and is classified as a congenital anomaly of the face and neck according to the WHO ICD-10. MNTI was first described by Krompecker in 1918 as congenital melanocarcinoma. Earlier, it was thought to arise from odontogenic or retinal rests. However, based on immunohistochemical, ultrastructural, and electron microscope studies and on occasional high urinary excretion of vanillylmandelic acid, it is now known to originate 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.

MNTI is a fast-growing but benign tumor. It may be locally aggressive in 15%–36% of cases; 3%–7% of tumors have an overtly malignant behavior with distant metastases.[2] The average recurrence rate after surgery is about 20%. MNTI usually (95%) occurs in children <1 year of age, peaking between 2 and 6 months with no distinct sex predilection.[2]

The tumor usually occurs in the head-and-neck region. Anterior maxilla (premaxilla) is the most common site of origin, occurring in almost 71% of cases.[3] They can occasionally arise in the skull (10.8%), mandible (5.8%), or brain (4.3%).[4] Other sites can be affected by the condition less frequently, including the retina, femur, epididymis, ovaries, uterus, and mediastinum. In the skull, the anterior fontanelle is the most common site; epididymis is the most common extracranial site.[4]

The classic clinical presentation is 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. This mass may have a rubbery consistency and nonulcerated surface, containing prematurely erupted or displaced primary teeth.[5] It results in facial asymmetry, dentition displacement, and difficulty in feeding.

Radiographs and CT images reveal tissue destruction, poorly demarcated radiolucency, hypodense underlying bone, and occasional faint “sunburst” appearance resulting from mild calcifications along vessels radiating from the center of the tumor.[6] MNTIs are biphasic tumors composed of small-cell and large-cell components that are arranged in a nest or cord arrangements set in a vascularized fibrous stroma.[6],[7]

The extent of surgical excision is debatable in the literature. The conservative surgical approach consists of local excision and curettage and prevents aesthetic sequelae with reduced manipulation of the lesion. Management by wide surgical excision is generally preferred (with at least 5 mm of healthy margins) and it seems essential to reduce the possibility of local recurrence rates to approximately 10%–15%[8] and usually leads to a good prognosis.[9] In the absence of free margins, the recurrence rate is increased by five times, most of which occur within 4 months and local radiotherapy with combination chemotherapy is then recommended.[8] In our case, we had initially resected/enucleated the exophytic lesion with the primary idea of getting a pathological diagnosis. However, the follow-up X-ray and CT scan showed excellent remodeling of the bone, and hence, no further resection was needed, thereby preserving normal cosmesis and facial growth. As delayed recurrences are known, close follow-up is essential. The risk of malignant transformation is 6.6%.[10]

   Conclusion Top

MNTI, although rare, has a distinctive clinicopathological and imaging features and it should be considered in the differential diagnosis of all patients presenting with head-and-neck masses, particularly if present in the maxillary region. Treatment is wide excision with consequent facial cosmetic sequelae. However, in this child, an enucleation resulted in complete facial remodeling and normal facial growth.

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

Nelson ZL, Newman L, Loukota RA, Williams DM. Melanotic neuroectodermal tumour of infancy: An immunohistochemical and ultrastructural study. Br J Oral Maxillofac Surg 1995;33:375-80.  Back to cited text no. 1
Kruse-Losler B, Gaertner C, Burger 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. 2
Tan O, Atik B, Ugras S. Melanotic neuroectodermal tumor in a newborn. Int J Pediatr Otorhinolaryngol 2005;69:1441-4.  Back to cited text no. 3
Haque S, McCarville MB, Sebire N, McHugh K. Melanotic neuroectodermal tumour of infancy: CT and MR findings. Pediatric Radiology 2012;42:699-705.  Back to cited text no. 4
Selim H, Shaheen S, Barakat K, Selim AA. Melanotic neuroectodermal tumor of infancy: Review of literature and case report. J Pediatr Surg 2008;43:25-9.  Back to cited text no. 5
Suzuki C, Maeda M, Matsushima N, Takamura M, Matsubara T, Taki W, et al. Melanotic neuroectodermal tumor of infancy in the skull: CT and MRI features. J Neuroradiol 2007;34:212-3.  Back to cited text no. 6
Magliocca KR, Pfeifle RM, Bhattacharyya I, Cohen DM. Melanotic neuroectodermal tumor of infancy. Pediatric Dermatology 2012;29:633-6.  Back to cited text no. 7
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. 8
Araújo PP, Tincani AJ, Paiva VP, Neta IS, Cardinalle IA, De Cassia Pereira R, et al. Melanotic neuroectodermal tumor of infancy (progonoma) Clinical, radiological, pathological features and literature review. Int J Pediatric Otorhinol Extra 2007;2:111-5.  Back to cited text no. 9
Béogo R, Nikiéma Z, Traoré SS, Bouletreau P. Maxillary melanotic neuroectodermal tumor of infancy management: Is conservative surgery the best approach? J Craniofac Surg 2013;24:e338-40.  Back to cited text no. 10


  [Figure 1], [Figure 2]


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