Year : 2007 | Volume
: 25 | Issue : 3 | Page : 148--151
Melanotic neuroectodermal tumour of infancy: A case report
N Retna Kumari, S Sreedharan, D Balachandran
Department of Pedodontics, Government Dental College, Trivandrum - 695 011, India
N Retna Kumari
Department of Pedodontics, Govt. Dental College, Trivandrum
A case of melanotic neuroectodermal tumor of infancy occurring in the maxilla in a 13 day old neonate is described. Computed tomography and histopathology confirmed the diagnosis and a submucosal excision was carried out when the infant was 30 days old. But three weeks later the patient reported back with a recurrence and a wide surgical excision was performed. The recurrence may have been caused by incomplete removal of the tumor cells and the initial surgical procedure may have stimulated tumour cell proliferation. Fortunately, 6 month follow up of the patient showed no recurrence.
|How to cite this article:|
Retna Kumari N, Sreedharan S, Balachandran D. Melanotic neuroectodermal tumour of infancy: A case report.J Indian Soc Pedod Prev Dent 2007;25:148-151
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Retna Kumari N, Sreedharan S, Balachandran D. Melanotic neuroectodermal tumour of infancy: A case report. J Indian Soc Pedod Prev Dent [serial online] 2007 [cited 2020 Nov 29 ];25:148-151
Available from: https://www.jisppd.com/text.asp?2007/25/3/148/36568
Melanotic neuroectodermal tumour of infancy (MNTI) is a relatively uncommon osteolytic - pigmented neoplasm that primarily affects the jaws of newborn infants. It is a locally aggressive, benign lesion of neural crest origin that occurs exclusively in infants younger than one year of age. More than 90% of the tumors occur in the head and neck region, most commonly on the anterior part of the maxillary ridge. Other common sites include the skull, the mandible, the epididymus and the brain.
So far approximately 250 cases have been reported in the medical literature. Although classified as a benign lesion, it often grows very rapidly and invades bone marrow diffusely without forming a fibrous capsule. In addition it may present as a multilocular lesion.
The average recurrence rate is 15-20% but it may be as high as 50% among cases treated without wide resection. In very rare instances malignant transformation are also reported. ,,,,, Hence careful, long term follow up is imperative for limiting disability and to ensure the survival of the affected infant.
This case report presents the serious clinical nature of the lesion, emphasizing the need for an early diagnosis and appropriate treatment, as the clinical appearance of the lesion could initially be mistaken for a lesion with a lesser threat like an eruption cyst. It also supports the neuroectodermal theory of pathogenesis and describes a programme for the management of difficult long term problems associated with growth, development and functions produced by surgical excision of such a lesion in the maxilla.
A 13 day old male neonate presented with a firm, non-tender pigmented mass on the left side of anterior maxillary ridge near the midline. Swelling was noticed soon after birth by the pediatrician and the child was referred to the Dept. of Pedodontics, Govt. Dental College, Trivandrum for dental consultation.
The pregnancy and delivery had been normal and there was no history of medication during pregnancy. Growth and development of the neonate were adequate for his age.
The smooth swelling (1 cm x 1 cm) was firm and non-tender on the left side of the anterior maxilla in the region of primary central incisor [Figure 1]. Overlying mucosa was intact and stretched. No pulse/thrill was elicited. On palpation it was hard and non-fluctuant.
An intra oral periapical radiograph was advised and radiographic appearance revealed an unilocular osteolytic lesion in the region of upper left primary central incisor [Figure 2] with the developing tooth bud being pushed out.
Plain radiographs of the skull (postero-anterior and lateral projection) and plain and contrast computed tomography (CT) of maxillofacial region were taken. Results of routine laboratory studies were within normal limits. The skull radiographs revealed an osteolytic area in the region of left maxillary primary central incisor.
Plain computed tomography (CT) of the maxillofacial region showed a small cystic lesion (9 x 12 mm) in the upper anterior alveolar arch. The primary tooth was displaced anteriorly bulging into the gingivolabial sulcus [Figure 3],[Figure 4]. Contrast CT showed a similar picture [Figure 5],[Figure 6].
FNAC revealed round neuroblast like cells and a provisional diagnosis of MNTI was made. The patient was referred to the Dept. of Pediatric Surgery, Govt. Medical College, Trivandrum for surgical intervention.
The patient was admitted for surgery when 27 days old and surgical excision of the lesion was carried out under General Anesthesia when the baby was 30 days old.
A submucosal excision was performed since a wide surgical resection which was usually necessary to eradicate an MNTI, would significantly damage normal growth centers of the maxilla. Developing primary left central incisor was excised since it was stained black. The area was thoroughly curetted down to normal bone. The mucosal flaps were then replaced and sutured. The infant tolerated the anesthetic and surgical procedures well and was discharged in good condition on the 5 th post operative day.
Histopathologic examination of the excised tissue revealed the characteristic biphasic pattern of cell distribution: 1) cell population consisting of larger cells with eosinophilic cytoplasm containing brownish pigments and vesicular nuclei clustered in alveolar pattern, and 2) lesional cell - smaller round cells with scanty cytoplasm and hyperchromatic nuclei with fibrillar background. The histopathologic examination of the tissue confirmed the diagnosis of MNTI [Figure 7],[Figure 8].
The postoperative course was uneventful. Three weeks after surgery, the patient reported back with a firm bluish swelling exactly over the previous surgical site. It rapidly increased in size during the next week.
Plain and Contrast CT scan of maxillofacial region were repeated. CT revealed irregularity and sclerosis in the site of upper left primary central incisor. Soft tissue density lesion noted in the position of the previous mass, suggestive of recurrence (13 x 11 x 9 mm). It bulged out anteriorly into the gingivolabial sulcus and hanged down from the alveolar arch. The margins of lesion which bulged out appeared well defined. The developing teeth within the dental capsule were seen in the remaining maxilla and mandible [Figure 9],[Figure 10].
A wide surgical excision was performed. Many pigmented spots in the surrounding bone were meticulously trimmed until no pigmentation was seen along with a 5 mm margin of healthy tissue. Developing primary lateral incisors, canine and first molar and adjacent bone were sacrificed as they were within the borders of the lesion. The patient made an uneventful recovery and was discharged on the 15 th postoperative day and the excised specimen was sent for histopathologic examination. Histopathologic report was quite similar to the previous one confirming the recurrence of MNTI. Neoplasm was seen infiltrating in between bone and extending up to the deeper margin of resection. A repeat CT scan of the maxillofacial region was taken when the infant was 2 ½ months old [Figure 11],[Figure 12]. CT showed evidence of wide excision extending from the midline upto the deciduous 2 nd molar. A thick rim of soft tissue density was seen in the position of excised bony parts. Review of the patient after 6 months showed no abnormal findings.
Krompecher first described the tumor in 1918,  since then only 250 cases have been reported in the literature. For the next 5 decades, the lesion was reported under a variety of names such as pigmented ameloblastoma, ,, retinal anlage tumor,  melanotic prognoma  or pigmented epulis. These terms reflected theories of suspected origin from the odontogenic apparatus, pigmented anlage of the retina or the sensory neuroectodermal tissues.
In 1966, Borello and Gorlin  reported a case with high urinary excretion of vanillyl mandelic acid (VMA) suggesting a neural crest origin and they proposed the term melanotic neuroectodermal tumor of infancy. However the presence of urinary VMA is not diagnostic for MNTI. ,
The preoperative distinction of this tumor from other small round cell tumors of infancy like rhabdomyoma, neuroblastoma is essential in order to plan the complete resection of the lesion. The many interesting aspects of the lesion, requires further consideration. These are the clinical behaviour patterns of the tumor, its tissue of origin and clinical management of the patient from infancy through adulthood. 
Clinical behaviour of MNTI has 2 features that make it a serious and difficult lesion to manage.
Though classified as a benign lesion, it is often clinically aggressive because of its rapid onset and alarming local growth rate.An increased propensity for recurrence.
The significant feature of MNTI is that recurrences have been observed in maxilla and mandible. No recurrence has been reported from a tumor of extraoral origin. Recurrence of MNTI may occur either as a development from cells of the original tumor that were missed at the initial surgery or that were seeded into intra trabecular spaces during excision of the original lesion. A prediction related to the location of the recurrence produced is that recurrence would consistently develop along the path of least resistance. It would first occupy the void in the bone left by removal of original tumor and make its first clinical appearance at this location.
Borello and Gorlin  have postulated that the maxillary and mandibular varieties of MNTI may arise from neural crest elements which develop in association with odontoblasts that eventually form dentin of primary anterior teeth. MNTI tumor cells, lying in close association with normal odontoblasts may be induced to activity by the normal functioning of their neighboring odontoblasts.
Steinberg et al.  reported that inadequate removal of all tumor cells may be a reason for the relatively high recurrence rate of MNTI, rather than incomplete removal of the primary lesion.
Aggressive surgery is usually not advocated for MNTI because the lesion is benign and also considering the young age of the patient. However, initial incomplete removal of the lesion deep within the bone resulted in a rapid recurrence and a wide surgical excision was carried out. The surgery necessary to eradicate an MNTI can be significantly damaging to normal growth and development of the child. It is therefore important to prepare and execute a plan to guide the child's growth and development through maturity to adulthood so that the effects of surgery can be reduced to a minimum.
Dr. Ajay Kumar, Professor of Pediatric Surgery, Govt. Medical College, Trivandrum.
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